Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology PDF

Textbook of
Oral Medicine, Oral Diagnosis

and Oral Radiology
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Textbook of
Oral Medicine, Oral Diagnosis
and Oral Radiology
Second Edition
Editors
Ravikiran Ongole BDS, MDS
Professor
Department of Oral Medicine and Radiology
Manipal College of Dental Sciences, Manipal University
Mangalore, India
Praveen BN BDS, MDS

Professor and Head
KLE Society’s Institute of Dental Sciences
Bengaluru, India
ELSEVIER
A division of
Reed Elsevier India Private Limited
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Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology, 2/e
Ongole and Praveen
ELSEVIER
A division of
Reed Elsevier India Private Limited
Mosby, Saunders, Churchill Livingstone, Butterworth Heinemann and

Hanley & Belfus are the Health Science imprints of Elsevier.
© 2013 Elsevier
First Edition 2010
Second Edition 2013
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any
means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission of the publisher.
ISBN: 978-81-312-3091-6
Medical knowledge is constantly changing. As new information becomes available, changes in treatment, procedures, equipment and
the use of drugs become necessary. The authors, editors, contributors and the publisher have, as far as it is possible, taken care to ensure
that the information given in this text is accurate and up-to-date. However, readers are strongly advised to confirm that information,
especially with regard to drug dose/usage, complies with current legislation and standards of practice. Please consult full prescribing
information before issuing prescriptions for any product mentioned in the publication.
Published by Elsevier, A division of Reed Elsevier India Private Limited.
Registered Office: 305, Rohit House, 3, Tolstoy Marg, New Delhi – 110 001.
Corporate Office: 14th Floor, Building No. 10B, DLF Cyber City, Phase-II, Gurgaon, Haryana – 122 002.
Publishing Manager: Ritu Sharma

Sr. Commissioning Editor: Nimisha Goswami
Managing Editor: Anand K Jha
Copy–Editor: Saroj K Sahoo
Publishing–Operations Manager: Sunil Kumar
Production Manager: NC Pant
Typeset by Olympus Premedia Pvt. Ltd. (formerly Olympus Infotech Pvt. Ltd.), Chennai, India.
www.olympus.co.in
Printed and bound at
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Dedicated to
My Wife, Parents and Brother

—Ravikiran Ongole
My Parents, Wife and Daughter (Aadya)

—Praveen BN
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Foreword I
The current academic scenario in our country has witnessed an abundance of postgraduate courses and postgraduate
teachers. An offshoot of this has been the burgeoning demand for reliable sources of knowledge for undergraduates,
postgraduates and the dental practitioners.
The mushrooming number of textbooks is a welcome sign of enterprise and effort on the part of our teachers. However
all are not of acceptable quality. One needs to separate the wheat from the chaff and restrict one’s interest to the textbooks
with quality content.
Once such gem is the Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology brought out by Dr Ravikiran Ongole
and Dr Praveen BN, committed academicians with a number of scientific publications and a previous textbook to their
credit; they have succeeded in creating a tome of oral medicine and radiology. It is a matter of great pride to me personally
since Dr Ravikiran is a faculty member and a gifted teacher in my institution.
Their task has been a Herculean one since they have not only contributed their own knowledge to this book; they have
also used the combined experience of other dental and medical professionals from diverse fields.
I am happy that the first edition of the book was received well and I am confident that the second edition of the book
with an additional section on dental radiology would help in imparting further knowledge to students in the field of
Oral Medicine and Radiology.
A brief overview of the book reveals the amount of effort put in by the editors and their contributors. I wish them all
the best in their endeavor to spread knowledge in Oral Diagnosis and Radiology and whole heartedly recommend this
book to all those who seek the same.
Prof (Dr) V Surendra Shetty

Pro Vice Chancellor
Manipal University, Mangalore
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Foreword II
Dentistry has always been an evolving science and every effort is to be made to keep up pace with the advancement. This
textbook has received wide recognition for its contribution to the practice of dentistry. I would like to congratulate the
editors in their continuing effort to present the second edition with attention to detail, written principally for a student
in an attempt to penetrate some depth towards newer research.
The edition has maintained its firm commitment in assuring a thorough and complete text. The ‘examination of a case’
confronts every clinician. I think this book gives a well-reasoned explanation of fundamental aspects, emphasized on
clinical scenario with illustrations, which are of value to a practicing dentist.
Launching a subsequent edition of a warmly received text is more of a challenge. I whole heartedly congratulate their
feat for their truly admirable skills and the contributors for this splendid work of knowledge.
Prof (Dr) Srivatsa G

Principal
Bengaluru
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List of Contributors
Carol M Stewart MS, DDS, MS Sadhana Shenoy S BDS, MDS

Professor, Oral Medicine Reader
Department of Oral and Maxillofacial Surgery and Dept of Oral Pathology
Diagnostic Sciences Oxford Dental College
University of Florida College of Dentistry Bengaluru, India
Gainesville, Florida, USA
Karen Boaz BDS, MDS
Madhu K Nair BDS, DMD, MS, Lic Odont (Sweden), PhD Professor and Head
Associate Professor Department of Oral Pathology
Oral and Maxillofacial Radiology Manipal College of Dental Sciences, Manipal University
Department of Oral and Maxillofacial Surgery and Mangalore, India
Diagnostic Sciences
University of Florida College of Dentistry Ajit Auluck MDS, PhD
Associate Professor, Radiology Clinician Scientist
UF College of Medicine BC Cancer Agency
Gainesville, Florida, USA Simon Fraser University
Canada
Syed Vaseemuddin BDS, MDS
Formerly Assistant Professor Manuel Thomas BDS, MDS
Department of Oral Medicine and Radiology Associate Professor
KLE Society’s Institute of Dental Sciences Department of Conservative Dentistry
Bengaluru, India Manipal College of Dental Sciences
Mangalore, India
Francisco Lopez Sanchez DDS, MDS Ajay G Nayak BDS, MDS
Professor Reader
Periodontics and Oral Pathology Department of Oral Medicine and Radiology
UMSNH College of Dentistry Mahatma Gandhi Vidyamandir’s
Professor, Endodontic surgery, Endo-periodontology and
Karmaveer Bhausaheb Hiray Dental College and Hospital
Ortho-periodontolgy Nashik, India
CUEPI Morelia
Michoacan, México Joanna Baptist BDS, MDS
Assistant Professor
Sara Carolina Rodriguez Peña DDS, MDS Department of Oral and Maxillofacial Surgery
Orthodontist, Military College of Dentistry Manipal College of Dental Sciences
UNITEC, México Manipal University, Mangalore, India
Nandita Shenoy BDS, MDS Thomas Zachariah BDS, MDS

Reader Reader
Department of Oral Medicine and Radiology Department of Oral and Maxillofacial Surgery
Manipal College of Dental Sciences, Manipal University Meenakshi Ammal Dental College and Hospital
Mangalore, India Chennai, India
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Ashok I Lingappa BDS, MDS James C Pettigrew DMD

Professor and Head Associate Professor
Department of Oral Medicine and Radiology Oral and Maxillofacial Radiology
Bapuji Dental College and Hospital Department of Oral and Maxillofacial Surgery and
Davangere, India Diagnostic Sciences
University of Florida College of Dentistry
Nagamani Narayana DMD, MS Associate Professor, Radiology, UF College of Medicine
Assistant Professor Gainesville, Florida, USA
Department of Oral Biology
Seunghee Cha DDS, PhD
College of Dentistry
Assistant Professor
University of Nebraska Medical Center
Department of Oral and Maxillofacial Surgery
Lincoln, USA
and Diagnostic Sciences
University of Florida College of Dentistry
Keerthilatha M Pai BDS, MDS Gainesville, Florida, USA
Associate Dean, Professor and Head
Department of Oral Medicine and Radiology Joseph Katz DMD
Manipal College of Dental Sciences Professor
Manipal, India Department of Oral and Maxillofacial Surgery and
Diagnostic Sciences
Braz Campos Durso DDS, MSD University of Florida College of Dentistry
Professor of Oral Diagnostic and Gainesville, Florida, USA
Orofacial Pain Department Sunanda C BDS, MDS
School of Dental Medicine Professor
Porto Velho, RO Department of Oral Medicine and Radiology
Brazil Dr Syamala Reddy Dental College and Hospital
Bengaluru, India
K Srinivas BDS, MDS
Professor NVS Sekhar Reddy BDS, MDS
Department of Oral Medicine and Radiology Professor
AECS Maruti College of Dental Sciences and Department of Oral and Maxillofacial Surgery
Research Centre Dr Syamala Reddy Dental College and Hospital
Bengaluru, India Bengaluru, India
Mala Kamboj BDS, MDS
Sarita Dimri MD Associate Professor
Formely Assistant Professor Department of Oral Pathology and Microbiology
Department of Dermatology Career Institute of Dental Sciences and Hospital
Kasturba Medical College and Hospital Lucknow, India
Mangalore, India
SV Kumaraswamy BDS, MDS
Shibu Thomas BDS, MDS Formerly Professor and Head
Reader Department of Oral and Maxillofacial Surgery
Department of Oral Medicine and Radiology VS Dental College and Hospital
Indira Gandhi Institute of Dental Sciences Bengaluru, India
Nellikuzhi, Kothamangalam Jeevan Prakash V BDS, MDS
Kerala, India Professor and Head
Department of Oral and Maxillofacial Surgery
Indraneel Bhattacharyya DDS, MSD Jodhpur Dental College and General Hospital
Assistant Professor Jodhpur, Rajasthan, India
Oral and Maxillofacial Pathology
Department of Oral and Maxillofacial Surgery Vivekananda Pai BDS, MDS
and Diagnostic Sciences Professor and Head
University of Florida College of Dentistry Department of Conservative Dentistry
Gainesville, Florida, USA Manipal College of Dental Sciences, Manipal University
Mangalore, India
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Jeeth Rai BDS, MDS Veena KM BDS, MDS

Assistant Professor Professor
Department of Periodontology Department of Oral Medicine and Radiology
KLE Society’s Institute of Dental Sciences Yenepoya Dental College and Hospital
Bengaluru, India Mangalore, India
Sumati Nagappa Baddannavar BDS, MDS Manish Juneja BDS, MDS, DNB
Reader Associate Professor
Department of Oral Medicine and Radiology Department of Oral Pathology and Microbiology
KLE Society’s Institute of Dental Sciences PDM Dental College and Research Institute
Bengaluru, India Haryana, India
Syeda Arshiya Ara BDS, MDS Balaji Rao B BDS, MDS

Professor Formerly Principal, Professor Emeritus
Department of Oral Medicine and Radiology Department of Oral Medicine and Radiology
Al-badar Rural Gulbarga Dental College and Hospital KLE Society’s Institute of Dental Sciences
Gulbarga, India Bengaluru, India
Ceena Denny E BDS, MDS Sumanth KN BDS, MDS

Associate Professor Department of Oral Diagnosis, Oral Medicine and
Department of Oral Medicine and Radiology Oral Pathology
Manipal College of Dental Sciences, Manipal University Faculty of Dentistry, Melaka Manipal Medical College
Mangalore, India Melaka, Malaysia
Gajendra Veeraraghavan BDS, MDS, MFDS RCPS Adel Kauzman DMD, MSc, FRCD(c)
Reader Associate Professor, Department of Stomatology
Department of Oral Medicine and Radiology Faculty of Dentistry
Sree Mookambika Institute of Dental Sciences Université de Montréal, Quebec, Canada
Kulasekharam, Tamil Nadu, India
Iona Leong BDS, MSc, FRCD(c)
Seema Patil BDS, MDS Department of Oral Pathology and Oral Medicine
Reader, Department of Oral Medicine and Radiology Faculty of Dentistry
DAPM RV Dental College University of Toronto, Ontario, Canada
Bengaluru, India Head, Department of Dentistry
Division of Oral Pathology and Oral Medicine
Vishwananth R BDS, MDS Mount Sinai Hospital
Professor and Head
Department of Oral Medicine and Radiology Nagaraj A BDS, MDS
Indira Gandhi Institute of Dental Sciences Reader
Puducherry, India Department of Oral Pathology
Sarat Gummadapu BDS, MDS, MFDS RCPSG Bengaluru, India
Associate Professor
Department of Oral Medicine and Radiology Shubha Sairam BDS, MDS
Drs Sudha and Nageswara Rao Siddhartha Assistant Professor
Institute of Dental Sciences Department of Oral Medicine and Radiology
Krishna (Dt), Andhra Pradesh, India KLE Society’s Institute of Dental Sciences
Bengaluru, India
Srikant N BDS, MDS
Associate Professor Ashith B Acharya BDS, GDFO
Department of Oral Pathology and Microbiology Associate Professor and Head of Forensic Odontology
Manipal College of Dental Sciences, Manipal University SDM College of Dental Sciences and Hospital
Mangalore, India Dharwad, India
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Foluso J Owotade BChD, FWACS Gail F Williamson RDH, MS

Associate Professor and Head Faculty Fellow of Faculty Advancement Initiatives
Department of Oral and Maxillofacial Surgery and Professor of Dental Diagnostic Sciences
Oral Pathology Oral Pathology, Medicine and Radiology
College of Health Sciences Indiana University School of Dentistry
Obafemi Awolowo University Indianapolis, USA
Ile-Ife, Nigeria
Muralidhar Mupparapu D MD
Eyitope O Ogunbodede BSc, BChD, MPH, DDPH, RCS Director, Oral and Maxillofacial Radiology
Head of the Department of Preventive Dentistry University of Pennsylvania School of Dental Medicine
Faculty of Dentistry, Obafemi Awolowo University Philadelphia, USA
Ile-Ife, Nigeria
Suman Jai Sanghar BDS, MDS
Ranganath D Rattehalli MBBS, MRCPsych, M Med Sc, Professor
CCT(UK), DPM, PG Diploma (Cli Psy) Department of Oral Medicine and Radiology
Consultant Psychiatrist, Leeds Partnerships NHS Trust, KSR Institute of Dental Sciences and Research
Seacroft Hospital, Leeds, UK Tiruchengode, Tamil Nadu, India
Sandip Deshpande MBBS, MD, DPM, MRCPsych, Jai Sanghar N BDS, MDS
CCT (UK), PG Diploma (Sex and Reln. therapy) Professor and Head
Consultant Psychiatrist, Sexual and Relationship therapist Department of Oral Medicine and Radiology
Manipal Northside Hospital, Bengaluru Rajah Muthiah Dental College and Hospital
Ex-consultant, NHS, Leeds, UK Annamalai University, Chidambaram
Tamil Nadu, India
Raghavendra Kini BDS, MDS
Professor Medha Babshet BDS, MDS
Department of Oral Medicine and Radiology Assistant Professor
AJ Institute of Dental Sciences Department of Oral Medicine and Radiology
Mangalore, India Hasanamba Dental College and Hospital
Hassan, Karnataka, India
Anshul Mehra BDS, MDS
Reader Rinky Nyachhyon BDS, MDS
Babu Banrasi Das College of Dental Sciences Assistant Professor
Faizabad Road, Lucknow Department of Oral Medicine and Radiology,
Uttar Pradesh, India Peoples Dental College and Hospital
Kathmandu, Nepal
Thomas George BDS, MDS
Faculty of Dentistry Apeksha Mainali BDS, MDS
University of Malaya, Kuala Lumpur, Malaysia Assistant Professor
Shubhasini AR BDS, MDS Kantipur Dental College
Reader, Department of Oral Medicine and Radiology, kathmandu, Nepal
KLE Society’s Institute of Dental Sciences,
Bengaluru, India Praveen BN BDS, MDS
Professor and Head
Bhanushree R BDS, MDS Department of Oral Medicine and Radiology
Lecturer, Department of Oral Medicine and Radiology, KLE Society’s Institute of Dental Sciences
KLE Society’s Institute of Dental Sciences, Bengaluru, India
Bengaluru, India
Ravikiran Ongole BDS, MDS
Kıvanç Kamburoğlu DDS, MSc, PhD Professor
Associate Professor Department of Oral Medicine and Radiology
Department of Dentomaxillofacial Radiology Manipal College of Dental Sciences
Faculty of Dentistry, Ankara University, Ankara, Turkey Mangalore, India
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Preface to the
Second Edition
The first edition of the book was published two years ago. Since then we have received very encouraging feedback from
students and faculty alike. It was also heartening to note that this book was included by various health universities in their
recommended list of textbooks for undergraduate and postgraduate students.
We also received suggestions for including a section on dental radiology with a special request to include chapters on
implant imaging and cone beam computed tomography. Taking these views into consideration, we included a section on
radiology that covers varied topics from radiation physics to specialized imaging techniques. The oral medicine section has
also been updated with the latest concepts in the diagnosis and medical management of various orofacial disorders.
We wish this comprehensive Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology gains wider popularity
amongst the student community and the faculty and continues to cater to the needs of students pursuing the specialty of
Oral Medicine and Radiology.
Ravikiran Ongole
Praveen BN
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Preface to the
First Edition
Oral Medicine in India is still in its nascent stages of growth. Consequently, there is not much literature that is easily
available to the student community. While there are many excellent textbooks available outside the Indian shores, in the
editors’ opinion, there is not much emphasis on diseases that tend to occur in the Indian subcontinent. Further, content
prescribed by the Dental Council of India, which is very useful to undergraduate and postgraduate students of dentistry,
is rarely available.
Many popular textbooks that are available to students of dentistry in India are either exam-oriented (not sufficient
background material; not suitable for reference beyond a particular examination) or focus only on oral medicine or on
oral radiology.
Our goal was two-fold: publish content that would appeal to undergraduate students because the content corresponded
to DCI curriculum; to postgraduate students and practitioners to serve as reference for diseases that seem to occur very
frequently in India. We have tried our best to combine oral medicine, diagnosis and radiological aspects of various orofacial
diseases and oral manifestations of systemic disorders. Chapters such as maxillofacial trauma, Lab investigations, Mental
illness and Syndromes of the head and neck have been specially written for postgraduate students.
We have tried our best to provide up to date references. Another unique feature of this book is the contributions made
from more than 60 authors from various dental colleges all over India and from countries such as USA, England, Canada,
Mexico, Brazil and Nigeria. We have made a conscious effort to tap into the expertise of authors from various fields of
medicine such as plastic surgery, dermatology and psychiatry and from various dental specialties apart from oral medicine
such as oral pathology, oral surgery, conservative dentistry, orthodontics, prosthodontics and periodontics.
We sincerely hope that this textbook will stimulate minds and satiate the intellectual appetite of the students of oral
medicine, diagnosis and radiology.
Ravikiran Ongole
Praveen BN
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Acknowledgments
The editors would whole heartedly like to acknowledge contributions from various individuals, especially the authors to
this book.
We would like to acknowledge the encouragement, guidance and support rendered by Dr V Surendra Shetty, Pro Vice
Chancellor, Manipal University, Dr Dilip G. Nayak, Dean, Manipal College of Dental Sciences, Manipal University, Mangalore
and Dr Srivatsa G, Principal, KLE Society’s Institute of Dental Sciences, Bengaluru. We would also like to thank them for
graciously permitting us to use photographs from the department archives.
We would like to thank the teaching faculty of the Departments of Oral Medicine and Radiology, MCODS (Mangalore)
and KLE Society’s Institute of Dental Sciences (Bengaluru) for their help and suggestions provided during the preparation
of this book.
Our heartfelt thanks to Dr Saranya B, Dr Richa Gaba, Dr Bijina, Dr Sumsum P Sunny, Dr Sushma CN and Dr Pramila
Mendonca who helped us at every step during the preparation of the book.
Our sincere sense of gratitude go out to Mr Mark Dirlam, Supervisor and Graphic Artist and Mr Timothy Centers,
Photographer, Department of Illustrations, Indiana University School of Dentistry, Indianapolis, Indiana, USA and
Dr Jaideep Shekhar for helping us in preparing illustrations for the book.
We are extremely indebted to Dr John O’ Keefe, Editor, Journal of the Canadian Dental Association, Dr Foluso Owotade
and Dr Carol Stewart for their words of strength and encouragement and for lending us photographs.
We appreciate the support and constant encouragement received from the ever enthusiastic publishing team of Elsevier
India, especially Ms Ritu Sharma, Ms Nimisha Goswami and Mr Anand K Jha.
Our sincere appreciation and gratitude to our patients, some of who were terminally ill, for enduring pain and discomfort
in the hope of relief and cure. We hope that this textbook will in some small way alleviate their sufferings.
Above all our deepest gratitude to our families for their affection, unconditional support and encouragement.
We sincerely apologize to individuals whose names have been inadvertently not mentioned.
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Contents
Dedication v
Foreword I vii
Foreword II ix
List of Contributors xi
Preface to the Second Edition xv
Preface to the First Edition xvii
Acknowledgments xix
Part I—Oral Medicine and Oral Diagnosis
SECTION I INTRODUCTION AND APPROACH TO DIAGNOSIS
Chapter 1 History and Scope of Oral Medicine and Oral Radiology 5

History of Oral Medicine • Oral and Maxillofacial Radiology • Oral Medicine and Radiology in India
SECTION II ORAL AND MAXILLOFACIAL DISTURBANCES
Chapter 2 Developmental Disturbances 11

Fordyce’s Granules (Fordyce’s Spots/Disease) • Lingual Tonsils • Leukoedema • Retrocuspid Papillae • Prominent Palatal
Rugae • Circumvallate Papillae or Vallate Papillae • Parotid Papilla • Racial Pigmentation/Physiological Pigmentation
• Mandibular and Maxillary Tori • Developmental Disorders Affecting Tongue • Developmental Disorders Affecting
the Lip • Developmental Disorders Affecting Buccal Mucosa and Gingiva • Developmental Disturbances of the Jaws
• Developmental Disturbances Affecting Teeth
Chapter 3 Orofacial Pigmentation Disorders 61

Pigmented Lesions of Oral Mucosa • Pigmentation of Teeth • Dental Fluorosis
Chapter 4 Bacterial, Viral and Fungal Infections 82

Scarlet Fever • Diphtheria • Tularemia (Rabbit Fever, Deer-fly Fever, Francis’ Disease, Tick-Borne Disease, Ohara’s
Disease) • Erysipelas • Impetigo • Melioidosis • Tetanus • Actinomycosis • Noma (Cancrum Oris, Gangrenous or Necrotizing
Stomatitis) • Botryomycosis (Bacterial Pseudomycosis) • Rhinoscleroma (Respiratory Scleroma) • Cat-scratch Disease •
Infectious Mononucleosis (Monoglandular Fever, Kissing Disease) • Acute Lymphonodular Pharyngitis • Measles (Rubeola)
• German Measles (Rubella) • HIV and AIDS • Acquired Immune Deficiency Syndrome • Sinusitis • Histoplasmosis •
Blastomycosis (Gilchrist Disease) • Mucormycosis (Zygomycosis, Phycomycosis) • Aspergillosis • Cryptococcosis (European
Blastomycosis, Torulosis, Busse-Buschke Disease)
Chapter 5 Orofacial Pain 111

Pain Physiology • Classification of Orofacial Pain • Clinical Assessment of Pain • Pain from Orodental Structures
• Barodontalgia • Paranasal Sinus-related Pain • Myofascial Pain • Neuralgias • Atypical Odontalgia • Atypical Facial
Pain • Burning Mouth Syndrome
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Contents
SECTION III MUCOCUTANEOUS DISORDERS
Chapter 6 Red and White Lesions 133

Description of Red and White Lesions • Etiologic Classification of Red and White Lesions • White Lesions of the Oral
Cavity • Red Lesions of the Oral Cavity • Red Lesions of the Tongue
Chapter 7 Vesiculobullous Disorders 174

Classification of Vesiculobullous Lesions • Predominantly Vesicular Lesions • Herpes Simplex Virus (HSV) Infections
• Herpetic Whitlow and Herpes Gladiatorum • Recurrent Herpes Infections • Varicella Zoster Infections • Hand, Foot and
Mouth Disease • Herpangina • Dermatitis Herpetiformis • Predominantly Bullous Lesions • Bullous Lichen Planus
• Erythema Multiforme • Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis (Lyell’s Syndrome) • Bullous
Impetigo • Epidermolysis Bullosa
Chapter 8 Oral Ulcerative Diseases 196

Classification of Oral Ulcers • Traumatic Ulcers • Primary Herpetic Gingivostomatitis • Recurrent Herpes Infection
• Varicella Zoster Infection • Acute Necrotizing Ulcerative Gingivitis (Trench Mouth, Vincent’s Disease, Vincent’s
Gingivostomatitis) • Tuberculosis • Syphilis • Deep Fungal Infections • Drug-induced Oral Ulcers • Erythema Multiforme
• Blood Disorders Causing Oral Ulcers • Immunologic Disorders • Dermatological Disorders • Pemphigoid • Gastrointestinal
Disorders Associated with Oral Ulcers • Neoplastic Ulcers • Ulcers of Unknown Etiology • Syndromes Associated with
Oral Ulcers • Diagnostic Protocol
Chapter 9 Dermatological Diseases 218

Lichen Planus • Epidermolysis Bullosa • Psoriasis • Ectodermal Dysplasia • Ehlers–Danlos Syndrome • Pachyonychia
Congenita • Dyskeratosis Congenita • Pityriasis Rosea • Xeroderma Pigmentosum • Acanthosis Nigricans • Goltz–Gorlin
Syndrome • Acrodermatitis Enteropathica • Hailey–Hailey Disease (Familial Benign Chronic Pemphigus) • Darier’s
Disease (Keratosis Follicularis) • Reiter’s Syndrome • Incontinentia Pigmenti (Bloch–Sulzberger Syndrome) • Kawasaki
Disease (Mucocutaneous Lymph Node Syndrome) • Tuberous Sclerosis Complex (Epiloia, Bourneville’s Disease)
• Graft-versus-Host Disease
SECTION IV DISEASES OF SPECIFIC STRUCTURES
Chapter 10 Temporomandibular Disorders 239

Components of Temporomandibular Joint • Clinical Evaluation of Temporomandibular Joint • Clinical Evaluation of
Muscles of Mastication and Accessory/Cervical Muscles • Disorders Associated with Deviation/Alteration in the Form of
Articular Surfaces • Articular Disk Defects • Inflammatory Joint Disorders • Degenerative Joint Diseases • TMJ Ankylosis
• Masticatory Muscle Disorders • Congenital, Developmental and Acquired Disorders of the TMJ • Neoplasms Affecting
the TMJ • Condylar Fractures
Chapter 11 Diseases of Salivary Glands 265

Developmental Disturbances • Saliva, Xerostomia, Hyposalivation and Sialorrhea • Inflammatory Conditions of Salivary
Glands • Viral-induced Salivary Gland Pathology • Non-inflammatory Conditions of Salivary Glands • Salivary Gland
Tumors • Benign Tumors • Malignant Tumors
SECTION V CYSTS AND TUMORS OF OROFACIAL REGION
Chapter 12 Cysts of Orofacial Region 303

Classification of Cysts of Orofacial Region • Theories of Cyst Expansion • Odontogenic Cysts • Odontogenic Keratocyst
• Gingival Cysts of Adults • Calcifying Epithelial Odontogenic Cyst (Gorlin Cyst) • Glandular Odontogenic Cyst • Non-
odontogenic Cysts • Nasolabial Cyst • Mid-palatal Raphe Cyst of Infants • Cysts of Maxillary Antrum and Salivary
Glands • Inflammatory Cysts • Pseudocysts • Cysts of Soft Tissues of Mouth, Face and Neck • Nasopharyngeal Cysts
• Thyroglossal Duct Cysts • Lymphoepithelial Cysts (Branchial Cleft Cysts) • Cystic Hygroma • Dermoid, Epidermoid and
Teratoid Cysts • Parasitic Cysts
Chapter 13 Tumors of Orofacial Region 331

Benign Odontogenic Tumors • Odontogenic Carcinomas • Odontogenic Sarcomas • Epithelial Malignant Tumors
• Connective Tissue Malignant Tumors
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Contents
Chapter 14 Oral Cancer 380

Incidence of Cancer of Head and Neck • Etiology and Risk Factors for Oral and Maxillofacial Cancer • Tobacco • Alcohol
• Systemic Health • Molecular Basis of Cancer • Clinical Signs of Cancer • Clinical Examination of a Patient with
Suspected Malignancy • TNM Staging • Nodal Metastasis • Diagnosis of Oral Cancer
SECTION VI TEETH AND PERIODONTIUM
Chapter 15 Dental Caries, Pulp and Periapical Lesions 405

Definition and Etiology • Contributory Factors in Dental Caries • Classification of Dental Caries • Microbiology of
Dental Caries • Fascial Space Infection • Ludwig’s Angina • Osteomyelitis
Chapter 16 Gingival and Periodontal Diseases 440

Classification System in Periodontal Disease • Gingival Diseases • Host–Microbial Interaction • Periodontal Diseases
• Syndromes • Sex Hormones • Stress and Psychosomatic Factors • Nutritional Factors • Radiographic Evaluation of
Periodontal Diseases
Chapter 17 Regressive Alterations of Teeth 458

Classification of Regressive Alterations Affecting Teeth • Tooth Surface Loss • Attrition • Abrasion • Erosion (Corrosion)
• Abfraction • Classification of Tooth Wear • Resorption of Teeth
SECTION VII SYSTEM REVIEW
Chapter 18 Systemic Disorders and their Clinical Implications 473

Symptoms Suggestive of Cardiovascular Disease • Common Cardiovascular Disorders and their Dental Considerations
• Ischemic or Coronary Heart Disease • Myocardial Infarction • Congenital Heart Disease • Rheumatic Fever • Infective
(Bacterial) Endocarditis • Heart Failure • Red Blood Cell Disorders • Polycythemia • Anemia • Thalassemia • White Blood
Cell Disorders • Qualitative Disorders • Non-neoplastic Disorders • Neoplastic Disorders • Bleeding Disorders • Vascular
Disorders (Vessel Wall) • Platelet Disorders • Thrombocytopathic Disorders • Thrombocytopenic Disorders • Disorders of
Coagulation • Inherited Coagulation Disorders • Acquired Coagulation Disorders • Upper Respiratory Tract Infections
• Lower Respiratory Tract Infections • Granulomatous Diseases • Malignant Disorders • Other Respiratory Diseases
Renal Diseases • Gastroesophageal Reflux Disease • Inflammatory Bowel Disease • Ulcerative Colitis • Crohn’s Disease
• Hiatal Hernia • Peptic Ulcer Disease • Eating Disorders • Liver Diseases • Bell’s Palsy • Epilepsy • Parkinsonism •
Multiple Sclerosis • Muscular Dystrophy • Oromandibular Dystonia • Myasthenia Gravis • Growth Hormone • Thyroid
Gland • Parathyroid Glands • Hypothalamus–Pituitary–Adrenal Axis • Pregnancy • Saliva and Monitoring of Hormone
Levels • Interesting Interface between ‘Dentistry’ and ‘Psychiatry’ • Management of Psychiatric Disorders
Chapter 19 Bone Diseases and Fibro-osseous Lesions 568

Fibro-osseous Lesions • Bone Diseases
Chapter 20 Autoimmune Disorders 590

Concepts of Immunity and Autoimmunity • Pemphigus • Epidermolysis Bullosa Acquisita • Systemic Lupus Erythematosus
• Autoimmune Polyendocrinopathy–Candidiasis–Ectodermal Dystrophy • Diabetes Mellitus Type I (IDDM) • Systemic
Sclerosis • Myasthenia Gravis
Chapter 21 Granulomatous Diseases 605

Tuberculosis • Leprosy (Hansen’s Disease) • Syphilis • Deep Fungal Infections • Foreign Body Granulomas • Wegener’s
Granulomatosis • Sarcoidosis • Orofacial Granulomatosis • Crohn’s Disease (Regional Ileitis, Regional Enteritis)
Chapter 22 Sexually Transmitted Diseases 625

Fellatio Syndrome • Traumatic Lesions of Lingual Frenum • Syphilis or Lues • Human Immunodeficiency Virus Infection
• Intraoral Molluscum Contagiosum • Condyloma Acuminatum • Oropharyngeal Gonorrhea • Oropharyngeal Chlamydial
Infection • Oropharyngeal Trichomonal Infection
Chapter 23 Nutritional and Metabolic Disorders 633

Nutritional Requirements of Indians • Carbohydrates • Proteins • Lipids • Vitamins • Metabolic Disorders • Lipid
Reticuloendothelioses
xxiii
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Contents
SECTION VIII FORENSIC DENTISTRY
Chapter 24 Clinical and Radiological Perspective 653

Introduction, History and Relevance • Dental Identification • Challenges in Postmortem Examination • Postmortem
Alterations to the Teeth and Oral Tissues • Social Profiling • Identifying the Edentulous • Craniofacial Identification
• Facial Approximation • Age Estimation Methods • Bite Mark Procedures • Lip Print Investigation
Part II—Oral Radiology
SECTION IX BASICS OF RADIOLOGY
Chapter 25 Basics of Radiation Physics 685

Pioneers in Dental Radiology • Fundamentals of Radiation Physics • Intraoral X-Ray Units • Interaction of X-Rays with
Matter
Chapter 26 Radiation Biology 697

Effects on Living Systems • Molecular and Cellular Radiobiology • Deterministic and Stochastic Effects • Sources of
Radiation • Dose and Risk in Radiography • Radiation Detection and Measurement • Film Exposure and Processing
SECTION X RADIOGRAPHIC METHODOLOGY
Chapter 27 Radiographic Films and Accessories 719

Intraoral Films • Extraoral Films
Chapter 28 Radiographic Techniques 724

Conventional Imaging • Intraoral Radiography • Extraoral Radiography • Posteroanterior Projection • Standard
Occipitomental view • 30ⴗ Occipitomental • Parietoacanthial View (Waters’ View) • Parietoacanthial View (Open Mouth
Waters’ View) • Modified Parietoacanthial Projection (Modified Waters’ View) • Acanthoparietal Projection (Reverse
Waters’ Method) • Submentovertex View (Base or Full Axial Projection, Schuller Method) • Lateral View • Lateral
Cephalometry • AP Axial Projection (Townes’ Method) • Reverse Townes’ View • Lateral Oblique View • TMJ Radiography
• Transcranial View • Transpharyngeal View (Parma Projection, Macqueen-Dell Technique) • Transorbital View
• Lesser Known/Forgotten Extraoral Radiographic Techniques • Panoramic Radiology • Specialized Imaging • Computed
Tomography • Dentomaxillofacial Cone-Beam Computed Tomography • Magnetic Resonance Imaging • Nuclear Medicine
• Ultrasonography • Sialography • Arthrography • Thermography (Thermal Imaging or Infrared Imaging)
SECTION XI PROCESSING OF RADIOGRAPHS AND RADIOGRAPHIC INTERPRETATION
Chapter 29 Latent Image Formation 801

Formation of Latent Image
Chapter 30 Processing of Radiographic Films 803

Manual Processing of Films • Automatic Processing
Chapter 31 Radiographic Faults 812

Errors in Film Storage and Handling • Errors in Film Placement and Projection Technique • Errors in Exposure Parameters
and Processing Technique • Artifacts
SECTION XII RADIOGRAPHIC LANDMARKS
Chapter 32 Intraoral Radiographic Anatomical Landmarks 825

Landmarks Common to both the Maxillary and Mandibular Radiographs • Landmarks Unique to the Maxillary Intraoral
Periapical Radiograph • Landmarks Unique to the Mandibular Intraoral Periapical Radiograph
Chapter 33 Extraoral Radiographic Landmarks 837
Chapter 34 Site Selection, Evaluation and Imaging for Dental Implants 842
xxiv
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Contents
SECTION XIII APPENDICES
Appendix 1 Terminologies 861

Appendix 2 Summary of Radiographic Pathology 867
Appendix 3 Characteristics of Ideal Radiograph 871
Appendix 4 Indications for Intraoral Radiography 872
Appendix 5 Patient Position for Extraoral Radiography 873
References 879
Index 885
Contents on Website (http://www.manthan.info)
I. A B C of Drugs Used in Dentistry e1

II. Syndromes of the Head and Neck e23
III. Laboratory Diagnostic Procedures e78
IV. Halitosis e147
V. Occupational Hazards in Dentistry e154
References e236
xxv
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PART Oral Medicine and
I Oral Diagnosis
Chapter-01.indd 1 10/4/2012 2:59:42 PM


SECTION Introduction
I and Approach
to Diagnosis
1 History and Scope of Oral Medicine and Oral Radiology 5
Chapter-01.indd 3 10/4/2012 2:59:44 PM


CHAPTER
History and Scope of Oral
Medicine and Oral Radiology
Carol Stewart, Madhu K Nair,
Syed Vaseemuddin, Ravikiran Ongole
1
➧ History of Oral Medicine ➧ Oral Medicine and Radiology in India
Mission Course and Curriculum of Oral Medicine and Radiology
Training and Scope of Practice Undergraduate Student Competencies as per Dental
Future Council of India Regulations
➧ Oral and Maxillofacial Radiology Scope of Oral Medicine and Radiology in India
HISTORY OF ORAL MEDICINE ‘Report on Dental Education in the United States and
Canada’. He believed that dental students’ education should
Carol Stewart be more similar to that of medical students. He stressed
the importance of biomedical sciences and research in the
In the British literature, Sir Jonathan Hutchinson (1828– dental school curriculum. His report suggested that oral
1900), a surgeon at the London Hospital, is regarded as the medicine be included in a dental curriculum. Another pio-
Father of Oral Medicine. He described dental manifesta- neer of great intellect and vision was Dr Lester Burket,
tions of congenital syphilis, intraoral pigmentation and professor of Oral Medicine at Pennsylvania School of Dental
perioral pigmentation associated with intestinal polyposis, Medicine. He also promoted the integration of medicine into
later described by Peutz and Jegher. Much of the early dental education and the role of oral health in reflecting sys-
description of oral mucosal diseases was found in derma- temic health. He is considered by many as the Father of
tology textbooks, as documented in the works of Oral Medicine and published one of the first definitive
Dr Erasmus Wilson. textbooks devoted to oral medicine in 1946.
Sir William Osler recognized the importance of the oral The American Academy of Oral Medicine (AAOM) was
cavity and believed that the tongue and oral mucosa acted organized in 1945 as the American Academy of Dental
as mirrors reflecting the state of health of a patient. He Medicine, and its founder was Samuel Charles Miller,
studied medicine at McGill University and in 1884, moved Professor and Chairman of the Department of Periodontics
to Philadelphia and became the Chair of Clinical Medicine at New York University College of Dentistry. In 1966, the
at the University of Pennsylvania. Ultimately, he became name was changed to the American Academy of Oral Medi-
the Chief of Staff at Johns Hopkins University and Hospital. cine. The academy was founded to broaden understanding
The early influence of Dr Kurt Thoma is internationally and knowledge of oral disease and to integrate dentistry
recognized as well. Dr Thoma was a Swiss-born oral sur- with medicine to provide more complete patient care. The
geon who produced significant textbooks in the 1920s and memberships include an internationally recognized group
1930s on oral surgery and oral pathology. His work proof professionals.
moted these disciplines, oral diagnosis, oral medicine and
oral pathology, to have greater prominence in dental
Mission
schools.
The study of oral medicine in the United States has The mission of the AAOM is stated in Box 1.
a unique history among medical/dental specialties. In the The AAOM is the heart and pulse of oral medicine in
United States, the roots of considering oral medicine as the United States and is also internationally recognized
a distinct area of study began with Dr William Gies of through the excellent educational training programs and
Columbia University. Dr Gies, a professor of biological scientific symposia hosted, and the educational literature
chemistry, became interested in dental research. In 1926, and treatment guidelines published for medically complex
the Carnegie Foundation sponsored Dr Gies’ work entitled patients.
5
Section I – Introduction and Approach to Diagnosis
Box 1 Mission of the American Academy of Oral Medicine

and sexually transmitted diseases as well. Skills mastered
include completion of a comprehensive physical eval-
• To foster excellence in education, research and patient care in the uation and medical risk assessment, selection of appro-
field of oral medicine priate diagnostic and laboratory procedures including
• To promote the study and dissemination of knowledge regarding blood studies, cytology, culture, biopsy techniques, and
the medical aspects of dentistry while serving the best interests delivery of appropriate care and assessment of treatment
of the public outcomes. Critical to completing an oral medicine curricu-
• To promote the highest standard of care in the diagnosis and lum is the acquisition of skills necessary to communicate
treatment of oral conditions that are not responsive to conventional
effectively with patients regarding the nature, rationale,
dental, oral or maxillofacial surgical procedures
advantages, disadvantages, risks and benefits of a recom-
• To provide an avenue of referral for dental practitioners who have
mended treatment.
patients with severe, life-threatening medical disorders or complex
diagnostic problems involving the oral and maxillofacial region that
American Academy of Oral Medicine is a sponsor of the
require ongoing non-surgical management American Board of Oral Medicine, the body responsible
• To improve the quality of life in patients with medically-related oral for examining and certifying candidates who have received
disease approved post doctoral training. The board examination is
• To foster increased understanding and cooperation between the offered during September/October each year.
medical and dental professions
Future
Future research will move further into genetics and pro-
Healthy collaborations have been developed between
teomics to define etiopathogenesis of these complex dis-
AAOM and the European Academy of Oral Medicine
eases as well as find targeted treatment approaches. The
(EAOM). EAOM was founded in 1998 based on representa-
potential for using saliva as a diagnostic tool is growing as
tion from European countries including Austria, Croatia,
well. As the life span of patients continues to lengthen due
Denmark, England, Estonia, Finland, France, Germany,
to polypharmacy, advancement in diagnostic technologies
Greece, Hungary, Iceland, Ireland, Israel, Italy, Latvia,
and treatment strategies, the demand for the oral medicine
Netherlands, Norway, Portugal, Romania, Scotland, Serbia,
specialist, both as a primary provider and consultant, will
Slovenia, Spain and Sweden.
grow exponentially. Medicine and dentistry are coming
closer together as they gain an appreciation of what each
Training and Scope of Practice can provide to enhance patient care. The quality of patient
care will continue to improve in parallel with these syner-
As defined on the website of the AAOM, oral medicine is gistic interactions.
the specialty of dentistry concerned with the oral health-
care of medically complex patients and the diagnosis and
non-surgical management of medically related disorders ORAL AND MAXILLOFACIAL RADIOLOGY
or conditions affecting the oral and maxillofacial regions.
These conditions include oral mucosal diseases, oral man-
Madhu K Nair
ifestations of systemic conditions and oral sequelae of
medical treatments. In addition, practitioners have special Oral and Maxillofacial Radiology is the newest dental
expertise in the management of neuropathic pain condi- specialty recognized by the American Dental Association.
tions involving the head and neck area and in pharmaco- The prospects of this discipline are excellent, especially in
therapy and drug interactions. light of the increased use of advanced imaging techniques
Currently, 10 certified 2-year programs are thriving in dentistry. Two-dimensional imaging that was used in
throughout the United States and Canada. The curriculum clinical dentistry is being replaced by newer image acqui-
consists of clinical sciences, biomedical sciences and clin- sition modalities that can generate information in 3D.
ical care interacting with all medical specialties. The mate- Digital radiography is fast replacing film-based imaging,
rial presented in the biomedical sciences provides the bringing with it numerous advantages. These include among
scientific basis needed to understand and carry out the others, the ability to reduce doses (in comparison with
diagnostic and therapeutic skills required by the clinical, older generation films and the use of round collimation),
academic and research aspects of oral medicine. A distinct faster image generation, the capability to post-process
curriculum in internal medicine allows the oral medicine images based on the diagnostic task and also carry out
specialist to gain unique expertise in the management specific image processing to enhance the signal-to-noise
of patients with endocrine, neuromuscular, hematopoietic, ratio, as well as the ability to archive, transmit and retrieve
immune and autoimmune, mucocutaneous, cardiovascu- images using dedicated Picture Archiving and Commu-
lar, renal, respiratory, musculoskeletal, gastrointestinal nications System (PACS).
6
Chapter 1 – History and Scope of Oral Medicine and Oral Radiology
In addition, the introduction and increased use of once they successfully challenge a very rigorous written and
advanced imaging modalities such as cone beam volumetric oral examination lasting several days, upon completion of
computed tomography (CBVCT) and MRI in dentistry has an accredited oral and maxillofacial radiology residency/
resulted in the need for these images to be interpreted by graduate program (http://www.aaomr.org/abomr_contact.
a board certified maxillofacial radiologists to rule out the php). The Board comprises a Board of Directors elected by
presence of other incidental pathology, and patients the Diplomates.
referred for specialized techniques including contrast imag-
ing or functional studies in selected cases. Integration of
services with Neuroradiology/Head and Neck Imaging at ORAL MEDICINE AND RADIOLOGY
hospitals is thus essential for ensuring optimal patient care. IN INDIA
Most medical radiologists are not trained in the interpreta-
tion of oral and maxillofacial pathology, anatomic varia-
Syed Vaseemuddin, Ravikiran Ongole
tions or anomalies and treatment modalities in dentistry to
the extent a maxillofacial radiologist is. The maxillofacial Oral Medicine was introduced in the curriculum and syl-
radiologist understands the diagnostic needs of the dental labus of the Bachelor of Dental Surgery (BDS) course in
patient both from a dental and general maxillofacial per- India about 37 years ago. The Government Dental College,
spective and can cater to the needs of the dental commu- Bangalore affiliated to Bangalore University was the
nity more efficiently based on a specific diagnostic task. first dental school in India to teach Oral Medicine. Dental
Most specialty areas within dentistry including Oral and radiology was only a minor subject being merged with
Maxillofacial Surgery, Orthodontics, Periodontics, Prostho- subjects like Conservative Dentistry, Periodontia and Oral
dontics, Pedodontics, Endodontics, etc. use advanced imag- Surgery. However, its importance was appreciated in the
ing for a variety of diagnostic tasks and treatment planning, late 1950s and Master of Dental Surgery (MDS) in radiol-
as also for follow-up evaluations. Some instances include ogy, a 2-year program, was introduced by Bombay Univer-
trauma, facial and other developmental anomalies such sity in 1959. However, oral medicine was still not included
as isolated clefts or defects, syndromes, obstructive sleep in the BDS curriculum.
apnea, temporomandibular joint disorders, implant surgery, Under the expert guidance of WHO advisors, ‘Oral Medi-
localization of impacted teeth and root canals, image-guided cine, Diagnosis and Radiology’ was introduced as a sepa-
surgical applications, and orthodontic evaluation and treat- rate subject by Bangalore University in 1966. It was taught
ment planning. When an advanced imaging study is done, during the third and final year BDS course in Government
it is expected that the entire volume of data is interpreted Dental College, Bangalore. However, without qualified and
to prevent any incidental pathology from being missed. trained teachers in this specialty, the training of BDS stu-
Some examples include life-threatening arteriovenous or dents remained inadequate. Government Dental College,
lymphovenous malformations, malignancies, aggressive Bangalore was the first institute to start MDS course with
benign tumors, intracranial lesions and cystic lesions of 2-year duration in ‘Oral Medicine, Diagnosis and Radiol-
jaws in patients imaged for dental purposes. ogy’ in 1970. As a part of the training program the first
The future of Oral and Maxillofacial Radiology is bright. orthopantomograph X-ray unit, the first in India was
Medical radiology went through the same phases of evolu- installed and commissioned in Government Dental College
tion in the 1990s as many hospitals transitioned to a film- in 1970—a gift from the WHO. The subject was introduced
less, paper-less environment and improved their workflow in the southern universities in both BDS and MDS courses
by incorporating digital imaging and PACS on an enterprise- a few years later. Bombay University followed, changing
wide basis. Currently, radiology is one of the highly ranked the MDS course in Dental Radiology to MDS course in Oral
and fiercely competitive residencies in medicine. The evo- Medicine, Diagnosis and Radiology in the 1970s.
lution of radiology informatics as a separate discipline The growth and development of the subject oral medi-
within radiology indicates the significance of information cine would have remained incomplete without an organi-
technology resources and computer applications in the zation for the specialists to meet, discuss and propagate
continued development and growth of radiology. Oral and the acquired knowledge. Hence, the specialists mostly of
maxillofacial radiology is headed down the same path. Karnataka and a few from Andhra Pradesh and Tamil Nadu
Medical radiology textbooks acknowledge the fact that met on 20th June 1985 in Bangalore and unanimously
interpretation of oral and maxillofacial pathology on the resolved to form an organization called Indian Academy of
need for maxillofacial radiologists in optimizing patient Oral Medicine. Dr BK Venkataraman and Dr Ramachandra
care. Currently, several post-doctoral and residency pro- Reddy were the founder members of this academy. The
grams exist in the United States and Canada for dental society was registered under Karnataka Societies Registra-
students to specialize in the discipline (http://www.aaomr. tion Act of 1960 at Bangalore. During the fifth national
org/adv_edu_prog.php). The American Board of Oral and conference in Chennai, the Academy was renamed as
Maxillofacial Radiology grants Diplomate status to residents Indian Academy of Oral Medicine and Radiology.
7
Section I – Introduction and Approach to Diagnosis
The Indian Academy of Oral Medicine and Radiology, Scope and Future of Oral Medicine and
launched its official publication Journal of Indian Academy Radiology in India
of Oral Medicine and Radiology in the year 1986. Presently,
the Academy has 800 members. Oral Medicine and Radiology specialists in India are usu-
ally full-time academicians in teaching hospitals. Some of
the specialists engage in specialty practice that includes
Course and Curriculum of Oral Medicine diagnosing orofacial diseases, recognizing the oral mani-
and Radiology festations of systemic diseases and assessing oral health of
medically compromised patients and medically managing
In India, the specialty of Oral Medicine, Diagnosis and these patients.
Radiology is taught to undergraduate students in the third Oral medicine specialists can further pursue a PhD in the
and final year of the BDS program. Following the BDS area of their interest as a part-time program or a full-time
degree a student can pursue a 3-year program in the spe- program.
cialty that leads to an MDS degree in Oral Medicine, Diag- In spite of remarkable progress made by the subject of
nosis and Radiology. Admission to the MDS program is Oral Medicine and Radiology during the last two decades
gained through competitive exams. many teachers and clinicians feel that there is plenty of scope
The syllabus in Oral Medicine and Radiology is divided for improvement in teaching methodology and research.
into Diagnosis, Diagnostic Methods, Oral Medicine and Presently across all dental schools in India, the Depart-
Oral Radiology. ment of Oral Medicine and Radiology functions more like a
source of patient referral to various dental specialties rather
Undergraduate Student Competencies as per than a specialty in itself. Following the registration for-
malities, patients are sent to the Oral Medicine Department,
Dental Council of India Regulations
wherein patients are interviewed, examined, diagnosed and
The student should be able to recognize various diseases referred to various specialties for the necessary treatment.
affecting the oral and paraoral structures and identify pre- The authors instead feel that the patients should be
cancerous and cancerous lesions of the oral cavity and refer directed from the reception/registration counter to the nec-
the patient to the concerned specialist for necessary man- essary department based on the patient’s need.
agement. They should have adequate knowledge about Those specialties in turn can refer patients for an expert
common laboratory investigations and interpretations of opinion and further management of specific problems such
their results. as orofacial pain, temporomandibular disorders, oral precan-
The student should have adequate knowledge about cers and cancers, salivary gland disorders, cysts and tumors
medical complications that can arise while treating system- of the head and neck and syndromes or patients with var-
ically compromised patients and take prior precautions/ ious underlying systemic illnesses that may require modi-
consent from the concerned medical specialist. He or she fications in the treatment plan and maxillofacial imaging.
should have adequate knowledge about radiation health Another area that needs a lot of improvement is oral and
hazards, radiation safety and protection. maxillofacial radiology. Although radiology is an integral
Students should be competent to take intraoral radio- part of the course and curriculum of both the undergraduate
graphs and interpret the radiographic findings. They should and postgraduate training in oral medicine and radiology,
gain adequate knowledge of various extraoral radio- it is mainly confined to extensive training in conventional
graphic procedures, TMJ radiography and sialography. The radiographic techniques.
student should be aware of the importance of intra- and Though specialized imaging techniques such as com-
extraoral radiographs in forensic identification and age puted tomography, magnetic resonance imaging, ultraso-
estimation. They should be familiar with jurisprudence, nography, sialography and arthrography are theoretically
ethics and understand the significance of dental records taught, the practical exposure to the technique and inter-
with respect to law. pretation is far from adequate.
8
SECTION Oral and
II Maxillofacial
Disturbances
2 Developmental Disturbances 11
3 Orofacial Pigmentation Disorders 61
4 Bacterial, Viral and Fungal Infections 82
5 Orofacial Pain 111
Chapter-02.indd 9 10/3/2012 2:17:26 PM


CHAPTER
Developmental Disturbances
Sadhana Shenoy, Karen Boaz, Sara Carolina
Rodriguez Peña, Francisco Lopez Sanchez,
Ravikiran Ongole
2
NORMAL ANATOMIC VARIATIONS Lingual Varices
➧ Fordyce’s Granules Lingual Thyroid
➧ Lingual Tonsils ➧ Developmental Disorders Affecting the Lip
Paramedian Lip Pits/Commissural Pits
➧ Fissured Tongue
Double Lip
➧ Leukoedema
Cleft Lip and Palate
➧ Retrocuspid Papillae
➧ Developmental Disorders Affecting Buccal
➧ Prominent Palatal Rugae Mucosa and Gingiva
➧ Lingual Varices Oral Melanotic Macule
➧ Circumvallate Papillae Fordyce’s Granules
➧ Parotid Papilla Fibromatosis Gingivae
➧ Physiological Pigmentation Hard Tissue Disturbances (Jaws)
➧ Hairy Tongue ➧ Developmental Disorders of the Jaws
➧ Exostoses/Tori Agnathia, Micrognathia, Macrognathia
Hemifacial Hyperplasia, Atrophy
DEVELOPMENTAL DISTURBANCES Condylar Aplasia, Hypoplasia, Hyperplasia
Soft Tissue Disturbances Bifid/Trifid Condyle
➧ Developmental Disorders Affecting Tongue Coronoid Hyperplasia
Aglossia Exostoses, Tori
Microglossia/Hypoglossia ➧ Developmental Disorders of the Teeth
Macroglossia Disturbances Affecting Size of Teeth
Ankyloglossia Disturbances Affecting Shape of the Teeth
Cleft Tongue Disturbances Affecting Number of Teeth
Fissured Tongue Disturbances Affecting Eruption of Teeth
Geographic Tongue Disturbances Affecting Structure of Teeth
Identification of normal anatomical structures forms the FORDYCE’S GRANULES

basis for diagnosis. The skill and knowledge to differenti- (Fordyce’s Spots/Disease)
ate normal from abnormal plays a decisive role in effec-
tive management of the disease process. It is a known fact Fordyce’s granules are named after an American derma-
that normal anatomic structures can have various clinical tologist, John Addison Fordyce. This is an ectopic/hetero-
presentations within the same species. topic collection of sebaceous glands seen in more than
However, one should realize that even the slightest of 80% of the normal population.
change in the appearance of the structure of organs and They are considered ectopic because sebaceous glands
tissues makes the recognition of pathological conditions are typically appendages of the skin. When they are pres-
challenging. On the other hand, normal oral anatomic ent in the oral mucosa they seldom have hair follicles.
structures can induce cancerophobia in many individuals. This condition is characterized by the presence of
These individuals have to be educated regarding the abso- multiple discrete minute yellow colored ‘dots’, ‘spots’ or
lutely benign nature of these anatomical variations. ‘granules’ involving various sites in the oral cavity such as
11
Section II – Oral and Maxillofacial Disturbances
Figure 1 Figure 2
Duct
opening
into
epithelium
Sebaceous
glands
Multiple yellowish ‘dots’ on the buccal mucosa suggestive of

Fordyce’s granules. Courtesy: Department of Oral Medicine The acinar lobules of the sebaceous glands below
and Radiology, MCODS, Mangalore the epithelium with a central duct opening into the
epithelium. Courtesy: Department of Oral Pathology,
MCODS, Mangalore
the buccal mucosa (opposite the molar teeth), lips, oral

commissures, retromolar pad, faucial pillars and the palate
(Figure 1). Some authors have described the appearance as
‘rice-like’, white or yellow white papules. The sebum pro- Anatomically, the arterial supply to the lingual tonsils
duced by these sebaceous glands imparts the yellow color is via the lingual branches of the external carotid arteries.
to these spots. Each of these granules can vary in size from The venous drainage is through the lingual vein that
1 to 3 mm. The condition is asymptomatic and usually finally empties into the internal jugular vein. The lingual
prominent in adults and generally persist throughout life. tonsils are mainly innervated by the lingual branch of the
Histopathologically acinar lobules are seen below the glossopharyngeal nerve and to some extent from the superior
surface epithelium. These acinar lobules usually communi- laryngeal branch of the vagus nerve.
cate with the epithelial surface via a central duct (Figure 2). Lymphatic vessels of the lingual tonsil drain into the
The ducts may show keratin plugging. The sebaceous cells superior deep cervical or jugular lymph nodes.
are roughly polygonal in shape with a central nucleus. The The lingual tonsil is generally not evident on routine
cells contain copious amounts of foamy cytoplasm. clinical examination. It may appear as solitary pink col-
Unless these granules are esthetically disturbing in an ored papule or nodule with a glossy yellowish-pink sur-
individual, no surgical intervention is recommended. face. However, it becomes clinically evident when inflamed
Patient should be educated regarding the harmless and and enlarged. Persistent inflammation of these tonsils has
persistent nature of the condition. Ocampo-Candiani et al also been referred to as lingual tonsillitis. Lingual tonsil-
(2003) showed that CO2 superpulsed laser can be used as litis which is a painful condition characterized by the
an effective treatment modality for patients who desire symptoms of sore throat and cough. The tonsils are ery-
treatment for cosmetic reasons. Sebaceous gland adenoma thematous and spongy or soft on palpation, mimicking
may occur at times. lesions of erythroplakia or oral cancer. However, the bilat-
eral presence of these nodules will help in differentiating
it from carcinoma.
LINGUAL TONSILS Lingual tonsils do not require any active management.
The patient should be reassured and told to report any
Lingual tonsils are the lymphoid aggregates present in the change in size or presence of symptoms. Lingual tonsilli-
oral cavity that are part of the Waldeyer’s ring. It can occur tis can be managed with antibiotics. Extensive enlarge-
unilaterally or bilaterally on the posterolateral border of ment of the lymphoid aggregates can impede intubation.
the base of the tongue. Lymphoid follicles of the lingual Histopathological evaluation is advised when the lym-
tonsil are irregular in shape and size and vary in number phoid aggregate enlarges rapidly in size or exhibits sur-
from 30 to 100. face ulceration.
12
Chapter 2 – Developmental Disturbances
Figure 3
A B
(A) Fissured tongue with central median groove. (B) Fissured tongue with multiple grooves in irregular fashion.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Fissured Tongue Management

Fissured tongue has been described by various names in Patients should be counseled regarding the benign nature
literature such as scrotal tongue or lingua scrotalis, lingua of the condition. They should be motivated to follow opti-
plicata, lingua fissurata, cerebriform tongue and furrowed mum oral hygiene practices. They can be encouraged to
tongue. The tongue in this relatively common malforma- use a soft bristled toothbrush to cleanse the grooves of
tion exhibits multiple grooves or furrows of 2–6 mm depth food debris. In symptomatic patients, topical application
and varied pattern on the dorsal surface. Heredity, aging, of clotrimazole is recommended.
chronic trauma and vitamin deficiency have been proposed
as the causes for this condition.
The exact etiology for this condition is still unknown. LEUKOEDEMA
However, a polygenic mode of inheritance is suspected
because it is seen clustering in families who are affected. Leukoedema is a common alteration of the oral mucosa,
Based on the geographic location and population studies, which appears as a diffuse grayish white opalescent area on
the prevalence of fissured tongue ranges from about 2% the oral mucosa. It is believed that the intercellular edema
to 21%. Males are said to be slightly more predisposed to of the superficial epithelial cells and the parakeratinized
exhibiting this condition. epithelium produces the typical grayish white appearance
The fissures are seen on the dorsal surface of the tongue in this condition.
sometimes extending to the lateral margins. The fissures Axell et al (1981) studied the influence of tobacco hab-
can have various morphological patterns; a single promi- its and leukoedema among 20,333 individuals in Sweden.
nent groove in the midline of the tongue, multiple horizon- In his study 48.9% of the individuals exhibited leuko-
tal groves radiating from the median groove and multiple edema. Males were more commonly affected. Leukoedema
grooves in an irregular fashion (Figure 3A, B). was more commonly found in the second and third decades
The tongue fissures can be seen in childhood. However, of life. The condition was significantly more prevalent in
the fissures are usually prominent with advancing age. individuals with any form of tobacco habit (60%) and rel-
Fissured tongue is usually associated with other conditions atively less common in individuals without any tobacco
such as geographic tongue (see Figure 21), Melkersson– habit (36.3%).
Rosenthal syndrome and Down syndrome. Van Wyk (1985) studied the association between leuko-
The condition is generally asymptomatic. However, the edema and smoking. He examined 1996 high school students.
deep fissures provide an excellent site for lodgment of He concluded that smoking does not cause leukoedema
food debris and candidal colonization. These patients may but may aggravate it. He also suggested that the etiology
complain of burning sensation in the tongue. for leukoedema is multifactorial.
13
Clinical features They proposed that the vacuolation in the cell cyto-
plasm represents a reversible form of cellular degeneration
Leukoedema presents as an asymptomatic, grayish white
resulting from cell damage. They believed that the vacu-
diffuse opalescent region on the buccal mucosa and occa-
olation was caused because of reduced mitochondrial
sionally extending into the vestibule, floor of the mouth
function. The superficial ‘ballooning’ cells are degenerated
and soft palate. The surface appears spongy and usually
cells. They stated that the presence of a compact layer of
comprises folds or grooves. It has been shown that it is
vacuolated cells, keratohyalin granules and keratohyalin-
more common in blacks (almost 90%) compared to whites
like structures in the superficial cells are features of an
(Figure 4).
aborted form of keratinization.
Clinically, this condition can be differentiated from
other white lesions occurring in the oral cavity by stretch-
Treatment
ing the mucosa. Grayish white areas of leukoedema usu-
ally disappear at least partially on stretching. Some authors No active treatment is necessary as it is considered as a
call this technique the ‘stretch test’. variation in the normal anatomy of the oral cavity. How-
ever, it is believed that the condition becomes less promi-
Histopathology and ultrastructural features nent with the cessation of tobacco habit.
Histopathological studies reveal increased epithelial thick-

ness. Parakeratinized epithelium with broad and elongated
RETROCUSPID PAPILLAE
rete pegs are typically seen. Intracellular edema within the
spinous layer is a characteristic feature in leukoedema. The
The retrocuspid papilla is a normal variation in anatomy
edematous/vacuolated cells are large with a pyknotic nuclei.
characterized by the presence of pink colored, soft to
Histopathologically it is believed to mimic lesions of white
firm, generally sessile papule or nodule (Figure 5), located
sponge nevus.
on the gingiva on the lingual surface of the mandibular
Van Wyk and Ambrosio (1983) studied the ultrastruc-
canines (cuspid).
tural and histochemical features of leukoedema in 12 indi-
The size of the nodule may vary in size from 1 to 5 mm in
viduals and compared it with normal buccal mucosa. They
diameter. It is very commonly seen in children and believed
concluded that the ‘intracellular edema’ of the epithelial
to regress with age.
cells in leukoedema is due to vacuolation in the cytoplasm
D’Aoust et al (1991) studied the distribution of retrocus-
of cells. Toward the surface of the epithelium, the vacuo-
pid papillae among three groups (Ecuador, Honduras and
lated cells collapsed into a compact layer of flattened cells.
Nicaragua) of Latin American patients. He found that the
The outer cells of this layer abruptly swelled again to form
retrocuspid papilla was most prevalent in children below the
the characteristic superficial layer of ‘ballooning’ cells of
age of 5 years. He also found a significantly higher female
leukoedema.
Figure 4 Figure 5
Opalescent grayish white hue on the buccal

mucosa characteristic of leukoedema. Retrocuspid papilla. Courtesy: Department of
Courtesy: Dr Ajit Auluck Oral Medicine and Radiology, MCODS, Mangalore
14
predilection in the Honduras group of Latin Americans and more number of palatine rugae and the left side of the pal-
unilateral presentation was more common. Bilateral presence ate shows slightly more number of rugae in both males
of retrocuspid papillae was seen in the Nicaragua group. and females. No bilateral symmetry is seen in the number
Retrocuspid papilla was seen involving the attached gingiva of rugae. As age advances, the length of the rugae and the
more frequently than the free marginal gingiva. Brannon transverse palatal rugal region width increases.
et al (2003) also showed that retrocuspid papillae are com- Luke (1988) studied the development of palatal rugae in
mon in females and young children. In the 51 cases that were mice. He showed that the rugae develop as localized regions
evaluated, bilateral retrocuspid papillae was more common. of epithelial proliferation and thickening prior to the ele-
vation of the palatal shelves. Later on, the fibroblasts and
Histological features collagen fibers accumulate within the connective tissue
beneath the thickened epithelium and then assume a char-
Buchner et al (1990), histopathologically analyzed 30 speci- acteristic orientation. The direction of the collagen fibers
mens of retrocuspid papillae. They showed that almost 80% running across the base of the palatine rugae determines
of the specimens showed loosely arranged delicate fibrous their orientation.
connective tissue with stellate and multinucleated fibroblasts. In the human embryos the palatal rugae are usually
They also showed that significant number of specimens prominent and present throughout the length of the palatal
exhibited elongated rete ridges and/or increased vascularity. shelves at the time of their elevation. At about the 550 mm
These papillae do not require any form of treatment. stage of the embryo there are about five to seven symmetri-
The need for treatment may arise only during the fabrication cal ridges. The anterior ridges originate at the midpalatine
of prosthetic appliances. raphe. Other ridges are seen laterally.
However, toward the end of the intrauterine life, the
posterior ridges almost disappear completely and the ante-
PROMINENT PALATAL RUGAE rior ridges become compressed and prominent.
It is believed that the role of rugae in humans is more or
Palatal rugae have also been referred to as rugae palatinae less vestigial. However, in animals palatine rugae help in
and plicae palatinae transversae. suckling and feeding.
The word ruga (plural: rugae) is a Latin word meaning
ridge, wrinkle or fold. For descriptive purposes palatine
Classification of Palatine Rugae Based on Length
rugae are anatomical ridges, folds or wrinkles in the ante-
rior part of the palatal mucosa. The rugae are present on Kapali et al (1997) in their study to evaluate the palatal rugae
either side of the median palatal raphe behind the incisive patterns in Australian Aborigines and Caucasians used the
papilla. Approximately four to seven rugae are seen on following clinical classification:
either side of the midpalatine raphe (Figure 6).
1. Primary rugae: (A—5 to 10 mm; B—10 mm or more)
Based on the shape of the rugae they have been catego-
2. Secondary rugae: 3 to 5 mm
rized as curved, wavy, straight and circular. Individual
3. Fragmentary rugae: Less than 3 mm.
studies have shown that males generally have slightly
On histological examination palatine rugae are stratified
squamous (predominantly parakeratinized) epithelium on a
Figure 6 connective tissue base, which is similar to the adjacent
palatal tissue.
Palatal rugae pattern analysis has been employed suc-
cessfully in positive human identification. Coslet et al
(1980) reported that the palatal rugae returned back in
several months after its surgical removal.
Many studies have shown that although minor changes
in the morphology of the rugae occur due to orthodontic
tooth movement, extractions, aging and palatal expansion,
these do not significantly alter the rugal morphology
enough to hamper identification.
Lingual Varices
Lingual varices are characterized by the presence of tortu-
Palatal rugae. Courtesy: Department of Oral Medicine and ous dilated veins on the ventral surface of the tongue. It is
Radiology, MCODS, Mangalore
estimated that approximately 10% of the patients in the
15
4th decade and above exhibit lingual varicosities and the

Figure 7
extent of the varicosities increases with advancing age.
Lingual varices have been referred to by various other
names in literature such as phlebectasia linguae, caviar
lesions and linguae varicosities.
Etiopathogenesis
Various theories have been proposed to explain the occur-
rence of these varices. Ettinger et al (1974) showed that the
incidence of varicosities increases with age. Koscard et al
(1970) reported that the elastic support to capillaries signifi-
cantly diminishes with advancing age. This reduction in
the elastic support of the connective tissue supporting the
blood vessels leads to dilation of capillaries and formation of
varicosities. It is also suggested that these are due to abnor-
mally dilated and tortuous veins (varices), as they are not
protected by surrounding tissues against hydrostatic pressure.
Eddy et al (1977) studied the role of vitamin C in 22
elderly vegetarians. He showed that vegetarians had high
levels of ascorbic acid values in plasma (10.2 ⫾ 0.4 mg/l),
compared to other elderly individuals. He concluded that
there was a lower incidence of sublingual petechiae and Sublingual varices. Courtesy: Department of Oral Medicine
varicosities in the vegetarian group. and Radiology, MCODS, Mangalore
Clinical features
In this condition multiple, bluish-purple papular blebs are Figure 8
seen on the ventral and lateral borders of the tongue and
occasionally seen on the lips and buccal mucosa. Ventral
surface of tongue reveals the presence of tortuous and
dilated veins (Figure 7). Susmita et al (2006) reported a
case with palatal varicosities. These are usually seen in the
elderly and are not symptomatic unless the varices are
thrombosed. Jassar et al (2000) reported a symptomatic
case of sublingual varices in a patient with portal hyper-
tension secondary to liver cirrhosis.
Histopathologic features
Microscopically, dilated veins are seen with little smooth
muscle and elastic tissue. Thrombosis may be seen as
concentric zones of platelets and erythrocytes (lines of
Zahn). Older thrombi show dystrophic calcification and/or
phleboliths. Mushroom-shaped circumvallate papillae.
Courtesy: Department of Oral Medicine and Radiology,
Management MCODS, Mangalore
Treatment is usually unnecessary. Surgical treatment is

indicated for cosmetic purposes or when there is thrombosis. project out onto the surface of the tongue. They are sur-
rounded by a marginal groove. Each of the papillae are
attached to the tongue via their slender bases.
CIRCUMVALLATE PAPILLAE OR VALLATE Roughly eight to 12 large (3–5 mm in diameter) mush-
PAPILLAE room shaped papillae are arranged all along the V-shaped
sulcus terminalis that divides the tongue into the body and
The word vallate is derived from the Latin word vallatus, base (Figure 8). However, occasionally they tend to grow
which means walled. Hence circumvallate papillae do not in size when inflamed.
16
Figure 9 Figure 10
Keratinized
stratified
squamous
epithelium
Non-
keratinized
epithelium
Circular
trench
Taste bud
Von Ebner
gland
Circumvallate papillae. The picture reveals keratinized

stratified squamous epithelium, circular trough, taste bud on
the lateral surface of the papilla and von Ebner gland.
A small nodule on the buccal mucosa corresponding to the
Courtesy: Department of Oral Pathology,
first and second molars characteristic of the parotid papilla.
MCODS, Mangalore
MCODS, Mangalore
Histologically, the surface of the vallate papillae exhibits

secondary papillae that are covered by stratified squamous
epithelium. The epithelium lining the lateral surface of the PAROTID PAPILLA
papillae shows numerous taste buds (Figure 9). The minute
ducts of the von Ebner’s glands pours its serous secretions
Stensen’s duct of the parotid salivary gland opens into the
into the base of the circular depression (trough). It is
oral cavity in the buccal mucosa opposite to the maxillary
believed that the secretions of the von Ebner’s glands is the
first and second molars.
primary source of salivary lipase and it also cleanses the
In many instances the orifice is hardly noticeable.
trough so that the taste buds can respond to various stimuli
However in some individuals a small, triangular raised
instantaneously.
pink to red color papule or nodule is readily visible. This
Sbarbati et al (1999) suggest that the concept of
flap of tissue covering the orifice of the Stensen’s duct is
von Ebner’s gland described as ancillary to the taste buds
called parotid papilla (Figure 10). Parotid papillae are usu-
(washing the trough around the vallate papillae or in peri-
ally seen bilaterally. As a result of their anatomic location
receptorial events) should be discarded. In their laboratory
adjacent to the occlusal plane, the parotid papillae are
studies they have found that the von Ebner’s gland and the
common sites for formation of traumatic ulcers.
vallate papillae form a single functional unit. They suggest
this unit be termed ‘circumvallate papilla/von Ebner’s gland
(CP/VEG) complex’. They believe that the CP/VEG com-
plex represented an important enzyme- and pheromone- RACIAL PIGMENTATION/PHYSIOLOGICAL
producing system composed of a sensitive (taste buds) and PIGMENTATION
an effectory (VEG) branch linked by feedback mechanisms
of control. They hypothesize that the taste buds located in Pigmentation of the oral mucosa can occur due to a wide
the distal portion of the VEG ductal system can be consid- variety of endogenous and exogenous agents. Most of these
ered similar to the chemoreceptor cells located in other are due to five basic pigments, namely, melanin, melanoid,
parts of the digestive apparatus such as pancreatic and bile oxyhemoglobin, reduced hemoglobin and carotene.
ducts. Hence, they concluded that the CP/VEG complex Physiologic oral pigmentations are genetically deter-
represented a rare example of chemoreceptor-secretory mined. Various stimuli, such as trauma, hormonal changes,
organ. medication and radiation may result in an increased pro-
Though, circumvallate papillae are usually not readily duction of melanin. An age-related increase of oral mela-
evident on the surface of the tongue, some patients tend to nocytes has also been observed.
panic when they notice these mushroom-shaped papillae In dark-skinned people, oral pigmentation increases,
while brushing their teeth. Patients need to be educated but there is no difference in the number of melanocytes
about their normal presence. between fair-skinned and dark-skinned individuals.
17
Figure 11 Figure 12
Physiologic black colored pigmentation of the gingiva.

Courtesy: Dr Ajit Auluck
Physiologic black pigmentation of the dorsum of the tongue.
MCODS, Mangalore
The variation is related to the differences in the activity of
melanocytes.
breakdown the melanosomes and the melanin. Albinic
Clinical features individuals exhibit very minimal or no presence of
Physiologic pigmentation, which is common in African, melanin. Though all human beings have almost similar
Asian and Mediterranean populations is probably to the concentration of melanocytes, based on the race of the
greater melanocyte activity. Oral physiological pigmenta- individual, melanin producing genes stimulate the pro-
tion is present in all races and seen equally in males and duction of melanin. Melanin is normally found in the skin,
females. The pigmentation develops during the first produced by melanocytes, its functions include absorption
decade of life but the patient may not be aware of it. The of ultraviolet light and scavenging of some cytotoxic
color depends on the amount of melanin produced and compounds.
the site where it is deposited, which may vary from brown There are typically three forms of melanin; namely,
to black. pheomelanin, eumelanin and neuromelanin. Eumelanin is
It is clinically manifested as multifocal or diffuse mela- found in abundant quantities. It is present in the skin and
nin pigmentation. The attached gingiva is the most com- hair. It can produce colors ranging from black to gray
mon intraoral site of such pigmentation (Figure 11), where and brown to yellow. Pheomelanin is abundantly found in
it appears as a bilateral, well-demarcated, ribbon-like, women. It can produce colors ranging from pink to red.
dark brown band that usually spares the marginal gingiva. Neuromelanin is responsible for the pigmentation of four
Other sites of involvement are the tongue (Figure 12), lips, nuclei of the brain, namely, substantia nigra, locus ceruleus,
buccal and labial mucosa and perioral skin. median raphe nucleus of pons and dorsal motor nucleus of
the vagus nerve.
Pathophyisology
Management
The pigmentation of the skin depends on the natural pig-
ment produced in the body called melanin. Melanin is Physiological pigmentation is treated only when esthetics
formed by melanocytes, which are located at the stratum is the concern. Dermabrasion, use of chemical bleach, free
basale of the epidermis. Head and neck region is the first gingival graft placement and cryosurgery are some of the
site of the body where melanocytes appear after approxi- surgical techniques to lighten the pigmentation.
mately 10 weeks of gestation. These melanocytes produce
melanin from tyrosine by the action of tyrosinase. The
Hairy Tongue
melanin that is formed is transported in the form of mela-
nosomes to keratinocytes via the dendritic processes of the This condition has also been referred to as black hairy
melanocytes. tongue. Etiology is unknown, however certain predisposing
Melanosomes persist in dark complexioned individuals. factors include poor oral hygiene, frequent use of mouth-
However in fair skinned individuals the cells in the skin washes, smoking and alcohol consumption, radiation therapy
18
Figure 13 MANDIBULAR AND MAXILLARY TORI
Exostoses
Exostoses or hyperostoses are non-pathologic, benign
bony growths projecting outward from the cortical plate.
Though exostoses are developmental disturbances they are
usually noticed only in adulthood and may enlarge with
age. They are asymptomatic and self-limiting. It is esti-
mated that the prevalence rate of tori is approximately 27
in every 1,000 individuals. Eskimos, American Indians and
Asians and more specifically Koreans are said to have a
higher incidence of tori.
Etiopathogenesis
According to some authors, micro-damage and inflamma-
tion in periodontal tissue in genetically susceptible individu-
als causes exostoses. Abnormal loads during mastication
have also been implicated in inducing exostoses. Buccal
exostoses may result from lateral pressure of the adjacent
teeth.
Yellowish-brown colored hairy tongue. Palatal tori may be a result of chronic periosteal isch-
Courtesy: Department of Oral Medicine and Radiology, emia due to nasal septum pressure. The torquing action of
MCODS, Mangalore the arch of the mandible during mandibular movements is
said to produce mandibular tori.
Clinical classification
for head and neck cancers, fungal infections, topical or
systemic antibiotics and corticosteroids and debilitating Although a formal classification system does not exist,
systemic illness. based on the clinical occurrence, exostoses may be catego-
rized as those that are commonly seen and the rarer forms.
Clinical features
Commonly occurring exostoses
The condition is characterized by the hypertrophy of ❍ Palatal torus
the filiform papillae on the dorsum, producing a hairy ❍ Mandibular torus
appearance. This condition results from inadequate des-
quamation or increased keratinization of the papillae. The Rarer forms of exostoses
filiform papillae are usually about 1 mm in length. How- ❍ Buccal exostoses
ever in hairy tongue these papillae may elongate to about ❍ Palatal exostoses
10 mm. ❍ Subpontine exostoses
Though the condition has historically been referred to
as black hairy tongue, the color can vary from yellow
to brown to black (Figure 13). The coloration depends on Torus/Tori
the chromogenic bacteria, consumption of beverages like The word torus is derived from Latin, which means a
tea and coffee and tobacco use. This condition is usually swelling or bulge. Although etiology is unknown, a hered-
asymptomatic. However some individuals complain of itary basis is suspected. A torus located along the midline
altered taste sensation, halitosis and occasionally gagging of the hard palate is called a palatal torus, or torus palati-
sensation. nus, and a torus in the lingual aspect of the mandible is
Histologically hyperkeratosis of the filiform papillae is called a mandibular torus, or torus mandibularis.
seen.
Patients should be advised to avoid excessive consump-
Clinical classification of tori based on
tion of beverages like tea and coffee. They should also be
morphology
advised to discontinue the use of tobacco. They can be
motivated to use a medium bristled brush over the dorsal Flat torus: Occurs as a slightly convex bony protuber-
surface of the tongue. ance with a smooth surface for mandibular tori. However
19
Figure 14 Figure 15
Palatal torus. Courtesy: Department of Oral Medicine and

Radiology, MCODS, Mangalore Bilateral mandibular tori. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
in the palatal region it extends symmetrically on either

sides of the palate.
Figure 16
Lobular torus: Occurs as a pedunculated or a sessile lob-
ular mass on either the mandible or palate. It arises from a
single base.
Nodular torus: These are seen as multiple bony protuber-
ances each arising from individual bases. As they enlarge
these may coalesce forming grooves between them.
Spindle torus: Occurs along the length of the midpalatal
raphe region for palatal tori. Elongated tori are evident
bilaterally in the mandible.
Torus palatinus
This condition exhibits exostoses in the midline of the
hard palate. It may be inherited as an autosomal dominant
trait. In individual studies the incidence of palatal tori Buccal exostoses. Courtesy: Dr Evelyne Verweij
among the population of United States has been reported
as high as 20–35%. It is believed to be twice as prevalent
in females as in males. female predilection is reported. The mandibular tori usually
The tori in the palatal vault may vary in size from a few occur in a symmetric fashion on the lingual surface
centimeters to larger sized lesions and are usually lobu- of the mandible above the level of the mylohyoid ridge
lated and dome shaped with a smooth surface (Figure 14). (Figure 15). The tori are usually located near the canine
These exostoses generally tend to enlarge with age. His- and premolar teeth. Occasionally the bilateral tori are so
topathologically smaller lesions are composed of compact extensive that they meet at the midline. These tori are
bone. However larger tori may exhibit a central core of termed ‘kissing tori’.
cancellous bone covered by compact bone.
Buccal exostoses
Torus mandibularis
Buccal exostoses are benign, broad-based surface masses
Torus mandibularis is the term used to describe exostoses of the outer or facial aspect of the maxilla (Figure 16)
occurring on the lingual surface of the mandible. It is and less commonly, the mandible (5.1:1). They begin to
estimated that 7–10% of the population of United States develop in early adulthood and may very slowly enlarge
exhibit torus mandibularis. However no specific male or over the years.
20
Figure 17 Figure 18
Occlusal radiograph with palatal torus marked. Mandibular occlusal radiograph showing radiopaque
Courtesy: Department of Oral Medicine and Radiology, masses extending from the lingual cortical plate, bilaterally.
MCODS, Mangalore Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
Palatal Exostoses
They appear as bilateral bony nodular protuberances aris- show excessive periosteal activity. The bone may show
ing on the palatal cortical plate usually corresponding to large lacunae with pyknotic osteocytes, indicative of bone
the maxillary tuberosities. ischemia.
Clinical considerations
Reactive Subpontine Exostoses
Exostoses may interfere with normal speech and other
It has also been referred to as subpontic osseous hyperpla-
functions. Occasionally these bony overgrowths may pre-
sia. It is a reactive exostoses arising from the crest of the
dispose to gingival and periodontal diseases by way of
alveolar ridge beneath the pontic of a fixed partial denture
forcing food toward the gingival margins during mastica-
presenting as nodule or protruberance of crestal bone. It
tion. Large overgrowths may be covered by relatively thin
was first reported in 1971 by Calman et al.
mucosa that ulcerates even by routine activities such as
It may have a genetic origin similar to other exostoses.
mastication and brushing.
However some authors believe that a chronic gingival irri-
They also interfere with the preparation and insertion
tation leads to the subpontic bone proliferation.
of prosthetic appliances and film placement in intraoral
radiography. Some authors have reported obstructive sleep
Radiographic findings
apnea in these patients. Difficulty in endotracheal intuba-
Exostoses and tori appear as well-defined radiopacities tion was also reported in these patients.
superimposed on the roots of the teeth. Palatal tori may Tori may be used for harvesting bone for alveolar ridge
not be appreciated in periapical radiographs. augmentation and as source of autogenous cortical bone
However occlusal radiographs may show a faint radi- in periodontal surgery.
opaque shadow over the midpalatal region (Figure 17).
Mandibular tori are readily visualized as radiopaque areas Management and prognosis
projecting from the lingual cortical plate on mandibular
Bony exostosis generally will not require any form of treat-
occlusal radiographs (Figure 18).
ment. The indications for treatment include functional
disturbance (difficulty in mastication), inability to place
the denture or repeated history of traumatic ulcerations.
Exostoses show dense cortical plate with a laminated pat- These bony outgrowths can be surgically contoured
tern. The laminar bone reveals scattered osteocytes. The or removed. Brunsvold et al (1995) reported recurrence
minute bone marrow spaces may show presence of a fatty of mandibular tori after surgical removal. The author fol-
marrow or a loose fibrovascular stroma. Generally mini- lowed up one patient for 11 years and the other for
mal osteoblastic activity is seen. However some lesions 14 years.
21
SOFT TISSUE DISTURBANCES Figure 19
DEVELOPMENTAL DISORDERS AFFECTING

TONGUE
Aglossia/Lingual Agenesis/Aglossia
Congenita
Failure in the embryogenesis of lateral lingual swellings
during intrauterine life leads to this rare and highly fatal
condition of absence of tongue. However literature review
reveals reports of individuals living a relatively normal
life. Khalil et al (1995) reported aglossia in a 30-year-old
man. It may occur alone or in association with other
deformities including micrognathia; microsomia; congen-
ital absence of mandibular incisors; collapse of the man-
dibular arch; sagittal band between the floor of the mouth Macroglossia. Courtesy: Dr Sumanth
and the palate; and situs inversus (also called situs
transversus—congenital condition where all major visceral
organs such as the heart, stomach, spleen, liver, etc. are Table 1 Classification of macroglossia
placed reversed or mirrored from their normal position). Pseudo macroglossia
Functional thyroid disorder may develop because of the
Tongue posture
embryological association between development of tongue Habitual (tongue thrust)
and thyroid gland. The absence of the lingual muscular Poor neuromuscular control
stimulus generally affects the development of jaws and Edentulousness
results in malocclusion of teeth. These individuals have Maxillofacial skeletal deficiencies
impaired speech and difficulty in swallowing. Highasi Shallow palatal vault
et al (1996) reported conductive deafness, esophageal atre- Deficient maxilla and mandible
sia, hypoplastic epiglottis and ptosis of the eyelid in an Effects of surrounding structures
individual with aglossia. Enlarged adenoids
Cysts, tumors, space infections displacing the tongue
Microglossia/Hypoglossia True macroglossia
This rare developmental condition is characterized by an Congenital causes
abnormally small tongue. It may occur as an isolated anom- Muscular hypertrophy
aly or, more commonly, as part of oromandibular limb hypo- Vascular malformations (hemangioma, lymphangioma)
Down syndrome
genesis syndrome (hypoglossia-hypodactylia syndrome) or
Beckwith–Wiedemann syndrome
in association with micrognathia, hypodontia, situs inver-
Mucopolysaccharidoses I and II
sus, asplenia, absence of lower incisors, or enamel hypo- Congenital hypothyroidism
plasia. Prognosis depends on nature and severity of the Behmel syndrome
condition. Treatment is directed toward improvement of Transient neonatal diabetes mellitus
oral function with an understanding of the changes in the Trisomy 22
mechanisms of oral suction, mastication, swallowing, speech, Laband syndrome
as well as dental occlusion. Lethal dwarfism of Blomstrand
Skeletal dysplasia of Urbach
Tollner syndrome
Macroglossia
Ganglioside storage disease type I
An abnormally enlarged tongue is one that protrudes beyond Lipoid proteinosis
the teeth or the alveolar ridge in the resting position Acquired causes
(Figure 19). Macroglossia can be broadly categorized as true Endocrinal disturbances
macroglossia and pseudo macroglossia (Table 1). In true Acquired hypothyroidism
Acromegaly
macroglossia enlarged tongue is associated with histological
Pituitary gigantism
abnormalities. In pseudo macroglossia (relative macroglossia)
Myxedema
the enlargement is apparent; though histology does not
(Contd...)
provide a pathologic explanation (e.g. Down’s syndrome).
22
Table 1 Continued Figure 20

Infections
Tuberculosis
Actinomycosis
Traumatic injuries
Self-inflicted (self-harm, injury during epileptic seizure)
Presurgical (intubation)/surgical trauma/postsurgical (anesthesia/
hemorrhage)
Neoplasms
Lymphangioma
Hemangioma
Carcinoma
Sarcoma
Solitary plasmacytoma
Neurofibroma
Granular cell tumor
Nutritional and metabolic disorders
Amyloidosis
Ankyloglossia. Courtesy: Department of Oral Medicine
Scurvy
and Radiology, MCODS, Mangalore
Pellagra
Autoimmune disorders
Sarcoidosis
Giant cell arteritis
Miscellaneous tip (Figure 20). Messner (2000) reported that the incidence
Angioneurotic edema of ankyloglossia ranged from 0.02% to 4.8% in newborns.
Modified from Richard D Thrasher III (2007)
Classification of ankyloglossia
I. Based on anatomical appearance
Clinical features
Type 1: Frenulum attaches to tip of tongue in front of
Clinical features include, crenated lateral border of tongue, alveolar ridge in low lip sulcus
open bite, mandibular prognathism and airway obstruction. Type 2: Attaches 2–4 mm behind tongue tip and attaches
There may be ulceration, secondary infection and necrosis. on alveolar ridge
In infants it leads to lisping speech, noisy breathing, drool- Type 3: Attaches to mid-tongue and middle of floor of
ing and difficulty in eating. The tongue has a pebbly sur- the mouth, usually tighter and less elastic. The
face with multiple vesicle like blebs in lymphangioma; in tip of the tongue may appear ‘heart-shaped’
hypothyroidism there is diffuse smooth enlargement; the Type 4: Attaches against base of tongue, is shiny, and
tongue is multinodular in amyloidosis and neurofibromato- is very inelastic
sis, papillary appearance of the tongue is seen in MEN type
II and tongue is fissured in Down’s syndrome. A unilateral II. Classification of ankyloglossia based on distance of the
enlargement of tongue is seen in hemihyperplasia. insertion of the lingual frenum to the tip of the tongue
Management
This classification was suggested by Kotlow (2004)
Macroglossia, unless causing a functional disturbance Normal: 16 mm
need not be corrected. It is treated with surgical techniques Class I (Mild): 12–16 mm
chosen in accordance with the functional results that one Class II (Moderate): 8–12 mm
wants to achieve. It must be the most conservative tech- Class III (Severe): 4–8 mm
nique to preserve the vascular nerve bundle. Speech ther- Class IV (Complete): 0–4 mm
apy may be required in some cases. Tracheostomy is indicated
in cases of airway obstruction.
Clinical significance
Ankyloglossia may lead to difficulties in breast feeding,
Ankyloglossia/Tongue-tie
articulation problems, gingival recession, open bite and
Ankyloglossia or tongue-tie, is the result of a short, tight, abnormal facial development. Frenotomy and frenuloplasty
thick, lingual frenulum causing tethering of the tongue have been effective treatments for ankyloglossia.
23
Intraoral radiography may be difficult in some patients reported that the tongue changes were the severest on the
owing to the limited space available to position the film. 17th day of the menstrual cycle.
Marks and Simon (1979) showed a definitive associa-
tion between geographic tongue and atopy.
Cleft Tongue
Many authors postulate that psoriasis manifests orally
Cleft tongue or bifid tongue is caused due to lack of merg- as geographic tongue. Gonzaga (1996) in his investiga-
ing of lateral lingual swellings of tongue. Partial cleft is tions showed a significant association of Cw6 with both
more common than the total cleft and is characterized by psoriasis and benign migratory glossitis. This antigen was
a deep groove in the midline of the dorsal surface. It is often found in 59.1% of the patients with psoriasis and 43.8%
found as one of the features of orofacial digital syndrome of the patients with benign migratory glossitis. Zargari
with thick fibrous bands in the lower anterior mucobuccal (2006) in a study including 306 patients with psoriasis
fold and clefting of hypoplastic mandibular alveolar pro- concluded that geographic tongue is more common in
cess. Food and microorganisms may collect in the base of early onset psoriasis and may be an indicator of the sever-
the cleft and cause irritation. ity of psoriasis.
Yarom et al (2004) found a strong correlation between
the occurrence of geographic tongue and fissured tongue.
Fissured Tongue
Described on page 13. Clinical features
Geographic tongue is commonly seen in the 2nd decade
Geographic Tongue of life.
The common sites of involvement are the tip of the
Geographic tongue has also been referred to as benign migra- tongue, lateral margins and dorsum of the tongue. How-
tory glossitis, wandering rash of the tongue, annulus migrans, ever some lesions tend to extend to the ventral surface.
stomatitis areata migrans and erythema areata migrans. It The appearance of the lesion typically mimics geographic
is a common benign condition that is seen in almost 3% of outlines on a map, hence the name geographic tongue
the population. Geographic tongue is seen in males and (Figure 21). It appears as circinate irregular erythematous
females equally. However, few articles in literature describe patches which represent the atrophic filiform papillae,
a slightly higher female predilection (2:1). However, the bound by keratotic white bands or lines which represent
tongue changes are more prominent in adults compared to the regenerating filiform papillae (Figure 22).
children. These erythematous patches occur in multiple sites on
the tongue. Very rarely a single site of involvement may be
Etiopathogenesis and predisposing factors seen. In this condition the filiform papillae regenerate in the
The etiopathogenesis of the condition is still not understood. atrophic site in a few days and a new site begins to reveal
Histologically it is said to be an inflammatory condition atrophy. Patients typically report this as ‘migrating rash’.
associated with human leukocyte antigen (HLA)-DR5, Many patients are unaware of the condition as it is
HLA-DRW6, and HLA-Cw6. Eidelman et al (1976) reported generally asymptomatic. However, some report of inability
that many of the parents and siblings of individuals with to consume spicy food owing to burning sensation. Some
geographic tongue also presented with the condition. This authors believe that the association of fissured tongue with
substantiated the possibility of heredity being an etiologi- superimposed candidal infection causes the burning sen-
cal factor. sation. The condition is seldom painful.
Guimaraes et al (2007) in their investigations found that Though this condition is typically seen involving the
the polymorphism⫹3954 interleukin (IL)-1B is associated tongue, it can occur on other sites in the oral mucosa.
with an increased risk of developing geographic tongue. Some authors use terms such as ectopic geographic tongue,
Redman et al (1966) and Bánóczy (1975) et al suggested geographic stomatitis, erythema migrans, erythema areata
that emotional stress was associated with the occurrence migrans, and stomatitis areata to refer to such a finding.
and severity of geographic tongue. The common ectopic sites include the buccal mucosa, labial
There are many studies with regard to the association mucosa (Figure 23), gingiva, floor of mouth and less com-
of geographic tongue and diabetes mellitus. Wysocky et al monly the soft palate and uvula.
(1987) reported a four-fold increase in the presence of
geographic tongue in diabetics. However, Guggenheimer
et al (2000) reported no significant correlation between Biopsy specimens should ideally be obtained from a site
geographic tongue and insulin-dependent diabetes mellitus. which includes the keratotic serpiginous margin and the
Waltimo (1991) in his study on the severity of geo- atrophic area. On histopathological examination hyperker-
graphic tongue in a patient taking oral contraceptive pills atosis, acanthosis and slender, elongated rete pegs are seen.
24
Figure 21 Figure 23
Geographic lip. Courtesy: Dr Foluso Owotade
Geographic tongue. Courtesy: Department of Oral Medicine

Management
and Radiology, MCODS, Mangalore All patients of geographic tongue need to be reassured of the
harmless nature of the condition. In symptomatic patients
palliative therapy with topical or systemic antihistaminics
have proved beneficial due to their local anesthetic effect
Figure 22 (Sigal et al, 1992). Presence of associated fissured tongue
with superimposed candidal infection can be effectively
managed with topical clotrimazole. Benzydamine hydro-
chloride mouthrinse can be used to manage burning sen-
sation. Gibson et al (1990) in their study showed that zinc
supplements proved effective for managing geographic
tongue.
Lingual Varices
Described on page 15.
Lingual Thyroid
Failure of the thyroid tissue to descend from its develop-
mental origin at the foramen caecum to its normal pretra-
cheal location leads to its presence in the tongue at the
foramen caecum. Van Der Gaag et al (1985) postulated
that maternal antithyroid immunoglobulins may arrest the
descent of the thyroid in some individuals.
Geographic tongue on the dorsum of the tongue. Thyroid tissue may be deposited ectopically along this
Courtesy: Department of Oral Medicine and Radiology, early thyroglossal tract. Apart from tongue, ectopic thy-
MCODS, Mangalore roid tissue has also been reported at other midline loca-
tions of the neck such as below the level of hyoid bone,
larynx and trachea, mediastinum and esophagus.
It is estimated that the incidence of lingual thyroid varies
Neutrophil infiltrations are seen in the thick layer of kera- between 1:3,000 and 1:100,000 (Williams et al, 1989).
tin and to a lesser degree in other portions of the epithe- The lingual thyroid is seen as a nodular mass in the
lium. These infiltrations produce microabscess (Munro’s midline about 2–3 cm in diameter with a smooth surface.
abscess) in the keratin and spinous layers. It appears erythematous when highly vascular. Depending
25
Figure 24 Figure 25
Lingual pits on the dorsum surface of the tongue. Commisural lip pit. Courtesy: Department of Oral Medicine
Courtesy: Department of Oral Medicine and Radiology, and Radiology, MCODS, Mangalore
MCODS, Mangalore
These can extend to a depth of 1.5 cm. On squeezing they

on the size, it may produce symptoms of pain, feeling of may express salivary secretions. These lip pits may be a part
fullness in the throat, dysphagia, dysphonia and dyspnea. of Van der Woude syndrome or popliteal pterygium syn-
The diagnostic test is thyroid scan with iodine isotopes drome. Histopathologically, tracts lined by stratified squa-
or technetium-99m, CT scan or MRI helps in delineating the mous epithelium are seen; minor salivary glands may be seen
size and extent of the lesion. Biopsy is avoided as there is communicating with the sinus. Surrounding connective tissue
risk of hemorrhage and it could be the only thyroid tissue is often infiltrated by chronic inflammatory cells. Excision
(in about 70% cases). Histopathologically it resembles nor- of these pits may be performed for cosmetic reasons.
mal thyroid tissue. If it has to be excised, it should be done
after confirming the presence of a functioning thyroid in
the normal position. If it is not so, then the excised tissue Commissural Lip Pits
is transplanted in a muscle with adequate vascularity. These small mucosal invaginations occur at the corners
of the mouth on the vermillion border (unilaterally or
bilaterally), possibly due to failure of fusion of embryonal
Tongue Pits
maxillary and mandibular processes (Figure 25). Though
The authors report a case of lingual pits on the dorsal sur- considered congenital, these often develop later in life—
face of the tongue in a 24-year-old male (Figure 24). The prevalence in children (0.2–0.7%) being much lower than
patient was asymptomatic and reported that these pits in adults (12–20%). Males are more often affected than
were present since childhood. Review of literature shows females. An autosomal dominant pattern of transmission is
no mention of this condition. noted in some families. In most cases, they are asymptom-
atic and are discovered on routine examination; they may
express saliva on squeezing. The depth of these pits range
DEVELOPMENTAL DISORDERS AFFECTING from 1 mm to 4 mm. Associated preauricular pits may be
THE LIP seen in some. They are generally not associated with facial
or palatal clefts. Histopathologically they are similar to
paramedian lip pits.
Paramedian Lip Pits (Congenital Lip Pits) No treatment is necessary. However excision is indi-
These congenital invaginations of the lower lip arise from cated if there is excessive salivary secretion or secondary
persistent lateral sulci on the embryonic mandibular arch. infection.
They are seen as bilateral (occasionally unilateral), sym-
metrical pits on the vermillion border on either side of
Double Lip
the midline ranging from subtle depressions to prominent
humps. This trait may be transmitted genetically to the Here a mucosal fold appears on the mucosal side of the lip,
offspring. commonly the upper lip. The congenital form is due to
26
Table 2 Syndromes associated with cleft lip Figure 26

Achondroplasia
Beckwith–Wiedemann syndrome
DiGeorge syndrome
Fetal alcohol syndrome
Goldenhar syndrome
Gorlin syndrome
Treacher Collins syndrome
Van der Woude syndrome
Waardenburg syndrome
Table 3 Syndromes associated with cleft palate

Achondroplasia
Beckwith–Wiedemann syndrome Unilateral cleft lip extending into the floor of the nose.
Courtesy: Dr Foluso Owotade
Cleidocranial dysplasia
Crouzon syndrome (craniofacial dysostosis)
Ehlers–Danlos syndrome
Fetal alcohol syndrome
Figure 27
Gorlin syndrome (basal cell nevus syndrome)
Marfan syndrome
Pierre Robin syndrome
Rubinstein–Taybi syndrome
Treacher Collins syndrome (mandibulofacial dysostosis)
Van der Woude syndrome
persistence of sulcus between the pars glabrosa and pars

villosa of the lip. The acquired form may be due to trauma,
repeated sucking on the lip or as part of Ascher’s syn-
drome (double lip, blepharochalasis and non-toxic thyroid
enlargement). In most cases, it becomes visible only when
the lip is tense or while smiling. Histopathologically nor-
mal structures are seen with a slight abundance of minor
salivary glands. Excision is the treatment for esthetic
reasons.
Bilateral cleft lip. Courtesy: Department of Oral and
Cleft Lip and Cleft Palate Maxillofacial Surgery, MCODS, Mangalore
Cleft lip results from failure of merging of epithelial groove

between the medial and lateral nasal process by penetration Cleft lip
of mesodermal cells. Cleft palate is the result of epithelial
breakdown with in growth failure of mesodermal tissue and ❍ Class I: Unilateral notching of vermillion border that
lack of lateral palatal segment fusion. Most cleft cases are does not extend into the lip
polygenic but the 5% of cleft cases associated with syn- ❍ Class II: Unilateral notching of the vermillion extending
dromes are said to be monogenic (Tables 2 and 3). Environ- into the lip but not involving the floor of the nose
mental factors like nutritional deficiencies, stress, infections, ❍ Class III: Unilateral clefts of the vermillion border
alcohol, drugs, toxins and ischemia may cause clefts. extending through the lip into the floor of the nose
Veau system of classification is generally used where (Figure 26)
the emphasis is on the extent to which the clefting is seen. ❍ Class IV: Any bilateral cleft of the lip exhibiting incom-
Submucosal clefts are not included here. plete notching or a complete cleft (Figure 27).
27
Figure 28 Figure 29
Orthopantomograph showing cleft alveolus on the right

maxillary lateral incisor region. Courtesy: Department of
Figure 30
Complete cleft palate in an adult.

Courtesy: Dr Prakash Chhajlani
Cleft palate (Figure 28)

❍ Class I: Cleft limited to soft palate
❍ Class II: Defects of both hard and soft palate. They
extend till the incisive foramen
Cleft palate can either be complete or incomplete.
Complete cleft includes cleft of soft and hard palate Lateral cleft lip. Courtesy: Dr Prakash Chhajlani
to the incisive foramen.
Incomplete cleft involves the velum and a portion
of the hard palate. the fusion of the midline structures is complete by the
❍ Class III: These are complete unilateral clefts extending 8th week, the position of the head is not readily accessible
from the uvula to the incisive foramen in the midline for adequate imaging. It is also believed that the head is
and the alveolar process unilaterally (Figure 29). relatively smaller compared to the size of the transducer
❍ Class IV: Complete bilateral clefts involving the soft in the first 15 weeks of gestation.
and hard palate and alveolar process on both sides of Classification of clefts based on ultrasound imaging
premaxilla leaving it free and often mobile.
Rarely, a lateral cleft lip can be seen due to non-fusion Type 1: Cleft lip
of the maxillary and mandibular process (Figure 30). Type 2: Unilateral cleft lip and palate
Type 3: Bilateral cleft lip and palate
Cleft of soft palate including submucosal clefts are associ- Type 4: Midline clefts
ated with eustachian tube dysfunction, recurrent otitis Type 5: Facial clefts associated with amniotic bands or
media and hearing deficits. limb-body wall complex.
Dental anomalies include congenitally missing lateral
incisor, supernumerary teeth, delayed tooth formation, Treatment of clefts requires a multidisciplinary team includ-
enamel hypoplasia, micro/macrodontia and fusion. ing dental, medical and surgical specialists with assistance
of allied health professionals in social services, child devel-
opment and hearing and speech therapy. Cleft lip repair is
Prenatal Diagnosis of Clefts
done when the child weighs 10 pounds, has hemoglobin
The facial structures of the fetus are best imaged after the level of 10 mg/dl and older than 10 weeks. Cheiloplasty is
15th week in utero using ultrasound (Figure 31). Though required later in life. By 1 year, closure of soft palate with
28
Figure 31 Figure 32
In utero diagnosis of bilateral cleft lip at 30 weeks pregnancy Surgically closed cleft palate.
using ultrasound. Courtesy: Dr Sudheer Gokhle, Courtesy: Dr Prakash Chhajlani
Ultrasonologist, Indore, India
pharyngeal flaps is recommended to promote normal speech Figure 33

development (Figure 32).
Palatal obturators are used when there are feeding
problems in cases of cleft palate (Figure 33).
DEVELOPMENTAL DISORDERS AFFECTING

BUCCAL MUCOSA AND GINGIVA
Oral Melanotic Macule

Labial melanotic macule is an otherwise asymptomatic,
usually solitary, small (generally ⬍ 5 mm), flat, brown to
brownish-black lesion found on the vermillion border of
the lower lip near the midline. It can occur at any age. Oral
melanotic macules usually occur on the gingiva, buccal
mucosa and soft palate. Histopathologically there is an
increased amount of melanin in the basal cells with a Feeding palate for cleft lip and palate neonates.
dropout of melanin from these basal cells into the macro- Courtesy: Dr Prakash Chhajlani
phages of connective tissue. This is called melanin inconti-
nence. Unlike in actinic lentigo the rete ridges here are not
elongated. Excision biopsy is done to rule out melanoma
and other pigmented lesions. Excision of these macules may Fibromatosis Gingivae
be undertaken for esthetic reasons. This is an autosomal dominant disorder affecting the
gingiva of one or both arches and characterized by non-
inflamed, non-painful smooth or nodular diffuse over-
Fordyce’s Granules (Fordyce’s Disease/Spots)
growth (Figure 34). The overgrowth may prevent eruption
Described on page 11. of teeth or may be seen covering a large portion of the
29
Figure 34 Figure 35
Diffuse non-inflammatory gingival enlargement in

fibromatosis gingivae. Courtesy: Department of Oral Medicine
Micrognathia
crown in erupted teeth. It may be associated with hyper-

(e.g. missing premaxilla) or mandible (e.g. missing ramus).
trichosis, corneal dystrophy, craniofacial deformities, nail
Partial absence of mandible is more common. When there
defects, deafness, epilepsy and mental retardation. Histo-
is unilateral absence of mandibular ramus, the ear too may
pathologically dense non-inflamed collagenous connective
be deformed or absent.
tissue with the overlying epithelium showing elongated
rete ridges are seen. Gingivectomy is the treatment of
choice. Recurrences may be seen but can be prevented by Micrognathia
extraction of teeth.
Congenital micrognathia is usually seen in association
with other congenital abnormalities like congenital heart
disease and Pierre Robin syndrome. Occasionally, they
HARD TISSUE DISTURBANCES (JAWS) may follow a hereditary pattern. Micrognathia of maxilla
is usually due to deficiency in the premaxillary area.
DEVELOPMENTAL DISTURBANCES OF THE The middle third of face appears retracted. Associated
JAWS maldevelopment of nasal and nasopharyngeal structures
can predispose to mouth breathing. Mandibular micro-
These include complete absence or diminished or exces- gnathia is commonly due to agenesis of the condyles.
sive development of the jaws. Abnormally small and large Normal growth of mandible depends on the development
jaws are called micrognathia or macrognathia respectively. of condyles as well as muscle function. If there is ankylo-
Apparent under-development or over-development due sis of the joint due to trauma, infection of mastoid, middle
to skeletal malpositioning of the jaws (e.g. retrognathia ear or the joint, it causes acquired micrognathia of man-
or prognathia) is called pseudo-micrognathia or pseudo- dible. Clinically, severe retrusion of chin, a steep man-
macrognathia. Disorders of development of facial skeleton dibular angle, and a deficient chin button are observed
may also affect the jaws. In hemifacial hyperplasia one (Figure 35). Table 4 lists few of the syndromes associated
side of the facial skeleton is abnormally large and in hemi- with micrognathia.
facial atrophy, it is under-developed.
Macrognathia
Agnathia
Only the mandible, both maxilla and mandible or the entire
Though the term agnathia refers to absence of jaws, skeleton may be abnormally large. Mandibular macrogna-
usually there is incomplete development of either maxilla thia may be due to increased height of ramus (Figure 36),
30
increased mandibular body length, increased gonial angle, It may also be associated with malformation syndromes
anterior positioning of glenoid fossa or prominent chin like Beckwith–Wiedemann syndrome, McCune–Albright’s
button. syndrome and neurofibromatosis.
Both the jaws are affected in Paget’s disease of bone,
acromegaly, leontiasis ossea (a form of fibrous dysplasia). Etiology and clinical features
Pituitary gigantism leads to generalized increase in size of
Etiologic factors suggested include vascular or lymphatic
entire skeleton. In addition to treating the underlying
abnormalities, central nervous system disturbances, endo-
cause, the length of the mandible may be reduced by
crine dysfunction, aberrant twinning mechanisms and
ostectomy.
chromosomal anomalies.
Women are afflicted twice as many as men. In them,
Hemifacial Hyperplasia either side is equally affected, while in males, right side is
more commonly affected. About 15–20% have enlarge-
Although this is known more commonly as hemifacial ment of cerebrum and mental retardation. Malignancies of
hypertrophy, there is actually hyperplasia of the tissues. adrenal cortex, liver and kidney (e.g. Wilms’ tumor) are
Some degree of facial asymmetry is common. Hemifacial more common in them. On the affected side, the skin is
hyperplasia refers to significant unilateral enlargement of thick and coarse, hair is thick and abundant and sebaceous
the face including eyes, ears, nose and intraoral tissues and sweat gland secretions are excessive. Excessive pig-
(Figure 37). It is often noted at birth and sometimes at mentation may also be evident.
puberty. The disproportionate growth continues until the Lee et al (2001) described three cases with hemifacial
patient’s overall growth ceases, resulting in permanent hyperplasia of the muscles of facial expression with no
asymmetry. other organ system involvement. They proposed to name
this condition with unique characteristics as ‘hemifacial
myohyperplasia’.
Table 4 Syndromes associated with micrognathia
Cohen syndrome Intraoral findings
DiGeorge syndrome Malocclusion is common. Unilateral macroglossia with
Fetal alcohol syndrome prominent papillae are characteristic.
Pierre Robin syndrome
Potter syndrome Radiographic features
Rubinstein–Taybi syndrome
The facial skull bones (mandible, maxilla, zygomatic, tempo-
Treacher Collins syndrome
ral and frontal) are enlarged on the affected side (Figure 38).
Figure 36
A B
(A) Lateral cephalogram showing mandibular macrognathia. (B) Orthopantomograph showing increased ramal length and
mandibular body in macrognathia
31
Figure 37
A B C
(A) A child with hemifacial hyperplasia affecting the right side. (B) Right side of the tongue showing enlargement.
(C) PA view showing enlargement of the mandible and soft tissues on the right side. (D) Enlarged body and
ramus of the mandible on the right side
The roots and crown of the teeth particularly the perma-

Figure 38
nent teeth are often enlarged and may erupt prematurely.
Primary teeth are shed prematurely on the affected side.
The mandibular canal can appear enlarged.
Management
Functional and cosmetic improvements may be achieved
through orthodontic tooth alignment and serial staged
surgeries. Because enlargement is related to all tissue lev-
els, perfect symmetry cannot be obtained.
Hemifacial Atrophy (Parry Romberg Syndrome,

Romberg Syndrome)
This condition is characterized by atrophic changes of
Photograph showing atrophic changes of the tissues in one tissues on one side of the face including eyes, ears,
side of the face. Courtesy: Department of Oral Medicine and nose and intraoral tissues. Trophic malfunction of the
Radiology, KLE Society’s Institute of Dental Sciences, Bangalore
cervical sympathetic nervous system, viral or borrelia
32
infection, trauma, genetic factors, peripheral trigeminal injuries sustained during the growth and development of
neuritis, etc. have been speculated as the causes. Many the mandible, therapeutic radiation and arthritis. This con-
investigators believe that it represents a localized form of dition is managed using grafts followed by orthognathic
scleroderma. surgery and orthodontic correction of malocclusion.
It commonly affects females in the first two decades of
life. The condition begins as a localized area of atrophy of
skin and subcutaneous structures. This atrophy progresses Condylar Hyperplasia
at a variable rate and affects the dermatome of one or more
branches of the trigeminal nerve. Osseous hypoplasia may Condylar hyperplasia refers to a unilateral enlargement of
occur when the condition begins during the first decade. the mandibular condyle. Though the etiopathogenesis is
The overlying skin often exhibits dark pigments (Figure 38). still unclear, various causes have been proposed such as
Some patients have a sharp line of demarcation resembling trauma, endocrinal disturbances and local deficiency of
a large linear scar between normal and abnormal skin near circulation.
the midline of the forehead known as coup de sabre (strike
of the sword). Atrophy of upper lip may expose the maxil- Clinical features
lary teeth. Unilateral posterior open bite develops as a result
of mandibular hypoplasia and delayed eruption of the teeth. Condylar hyperplasia is usually encountered in the second
Teeth on affected side may show deficient root develop- and third decades of life. Patients generally complain of pro-
ment or root resorption. Atrophy progresses for several gressive facial asymmetry. On clinical examination unusu-
years and then becomes stable. Cosmetic surgery and orth- ally large condylar head can give arise to a clinically evident
odontic therapy may help in correcting deformity and swelling in the temporomandibular joint region. The man-
malocclusion respectively. dible is deflected to the normal side. Open bite on the
affected side is a characteristic feature.
The progressive facial asymmetry can be confirmed
Condylar Aplasia by comparing photographs of the patient from childhood
through adolescence to adulthood.
The complete failure of development of the mandibular Orthopantomograph (OPG) is a good scout radiograph
condyle either unilaterally or bilaterally is referred to as for comparing the relative increase in size of the condyle
condylar aplasia. It is estimated that the incidence of con- with respect to the unaffected side. Other radiographic views
dylar aplasia is around 1 in 5,600 births. that could prove beneficial are the open and closed TMJ
The developmental defect is rarely seen in isolation. views, TMJ views (transcranial, transpharyngeal and trans-
It may usually be associated with hemifacial microsomia, orbital), posteroanterior skull view, submentovertex base
Goldenhaar’s syndrome and Treacher Collins syndrome. of skull view and computed tomography.
Lesser known associations of condylar malformations Histopathologically the condition shows normal fea-
and agenesis are with Proteus syndrome, Morquio syn- tures after completion of the growth. However prolifera-
drome and auriculocondylar syndrome. Santos et al (2007) tion of the condylar cartilage is seen during the periods of
reported a case of condylar aplasia without any syndrome active growth.
association. The condition is generally self-limiting. However unusu-
In unilateral aplasia obvious facial asymmetry and shift- ally large condyles causing functional and esthetic distur-
ing of the mandible to the affected side on mouth opening bances can be managed by surgical recontouring. In some
are common findings. cases condylectomy may be required.
Condylar Hypoplasia Bifid/Trifid Condyle

Condylar hypoplasia results from congenital or develop- Hrdlicka in 1941, was the first to describe bifid condyles in
mental disturbances or due to acquired causes. In this con- skull specimens. Schier in 1948 described bifid condyles in
dition the condyle usually retains its shape but appears a living person based on radiographic findings. Artvinli
smaller. Unilateral involvement of the condyles is more et al (2003) and Cagirankaya et al (2005) reported trifid
common than bilateral involvement. mandibular condyle.
Most of these patients also present with a proportion- Most of the cases reported were involving individuals
ately smaller ramus and body of the mandible. A promi- over 20 years of age. Males and females are equally
nent antigonial notch may be seen. The acquired causes affected. Literature reveals the left condyle being twice
that result in hypoplasia of the condyle include traumatic more affected than the right side.
33
Figure 39 Figure 40
Cropped orthopantomograph showing bifid condyle. Cropped orthopantomograph showing trifid condyle.
Courtesy: Department of Oral Medicine and Radiology, Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore MCODS, Mangalore
The etiology for the condition is unknown. Some No treatment is required for this condition as it is
authors believe that the condylar cartilage, during the asymptomatic.
early stages of its development, is divided by well-vascu-
larized fibrous septa and the persistence of such a septum,
Coronoid Hyperplasia
within the growing cartilage might give rise to the bifid
condyle. It is also believed that the rupture of the septal Hyperplasia of the coronoid process is an uncommon
vessels secondary to trauma can give rise to the bifid con- developmental disturbance affecting the jaws. The hyper-
dyle. Various other factors that have been implicated are plasia of the coronoid process can either be unilateral or
exposure to teratogenic substances, endocrinal disturbances, bilateral.
infections, exposure to therapeutic radiation and nutritional This condition is characterized by a progressive limita-
deficiencies. tion in mandibular movement, due to impingement of the
It is believed that bifid mandibular condyle resulting elongated coronoid processes on the posterior surface of
from trauma will exhibit one glenoid fossa, whereas those the zygomas. The shape of the coronoid process usually
of developmental origin exhibit separate glenoid fossa for does not change, however it only increases in size.
each of the two parts of the bifid condyle. It is also postu- On clinical examination there is no apparent facial
lated that the anteroposterior (sagittal) form of bifid con- asymmetry or pain. It usually begins at puberty. Males
dyle results from facial trauma during childhood, and the are more commonly affected than females (5:1). Apart
mediolateral form (coronal) with persistence of the fibrous from genetic inheritance other causes for coronoid hyper-
septa at the condylar cartilage. plasia to occur have been proposed such as trauma,
Bifid or trifid condyles (Figures 39 and 40) are usually increased activity of the temporalis muscle and endocrinal
found incidentally on radiographic investigations. Though stimulus.
commonly an orthopantomograph can reveal the possibil- Waters’ view and orthopantomograph are usually suf-
ity of a bifid or trifid condyle the best imaging modality to ficient to evaluate coronoid hyperplasia. It is believed
visualize the condyle for this condition is coronal computed that the projection of the tips of the coronoid processes
tomography. Moreover, a 3D reconstruction helps in accu- at least 1 cm over the inferior rim of the zygomatic arch is
rately assessing the morphology of the condyles. pathognomonic of coronoid hyperplasia. The impingement
34
of the elongated coronoid process over the zygomas can

Figure 41
be best appreciated by an axial CT image with the mouth
open.
Literature reveals reports of coronoid hyperplasia in
syndromes such as trismus-pseudocamptodactyly syndrome
(Yamashita et al, 1979) and nevoid basal cell carcinoma
syndrome (Leonardi et al, 2001).
The condition can be managed by surgically contour-
ing the coronoid process or by coronoidectomy followed
by a rigorous regimen of physiotherapy.
Exostoses/Tori
Described on page 19.
DEVELOPMENTAL DISTURBANCES Unusually elongated root in premolar.

AFFECTING TEETH Courtesy: Department of Oral Pathology,
MCODS, Mangalore
These include disorders of size, shape, number, structure
and eruption of teeth.
Figure 42
Developmental Disturbances Affecting
Size of Teeth
These include either decreased or increased size of the teeth.
Localized microdontia is fairly common, especially that
affecting maxillary lateral incisor (peg lateral) and the third
molar. When only the roots are long/elongated, the condi-
tion is called rhizomegaly or radiculomegaly (Figure 41).
Similarly, rhizomicry or root dwarfism is the term used to
refer to teeth with short roots (Figure 42).
Microdontia In this condition the teeth are smaller than
usual (microdonts). Apparently small teeth due to abnor-
mally large jaws (pseudomicrodontia) are not included
here. Microdontia can either be localized or generalized
(Figure 43).
In generalized microdontia all the teeth are smaller than
normal. It may be seen in pituitary dwarfism and Down
syndrome (Figure 44A, B). Other conditions that are asso-
ciated with microdontia are Axenfeld–Rieger syndrome
(O’Dwyer et al, 2005), Fanconi anemia (Tekcicek et al, 2007),
Bardet–Biedl syndrome (Drugowick et al, 2007) and oculo- Stunted root length in a maxillary central incisor.
dentodigital dysplasia (Vasconcellos et al, 2005). Remmers Courtesy: Department of Oral Pathology,
et al (2005) reported microdontia as a late effect of chemo- MCODS, Mangalore
therapy on dental development in a patient treated for
neuroblastoma during the early years of life.
Localized microdontia is a rather common condition. It Macrodontia It refers to teeth that are larger than nor-
affects most often the maxillary lateral incisor and third mal. However, the term macrodontia should not be applied
molar. The common form which affects maxillary lateral to teeth that appear large due to fusion or gemination.
incisor is called peg lateral (Figure 45). Localized micro- Localized macrodontia is seen in hemihyperplasia. All the
dontia can be seen in hemihyperplasia. teeth are larger than normal in generalized macrodontia.
35
Figure 43 Figure 45
Microdontic tooth specimen. Courtesy: Department of

Oral Pathology, MCODS, Mangalore
Peg-shaped maxillary lateral incisor. Courtesy: Department of

Figure 44
A
et al, 2006), Ekman–Westborg and Julin syndrome (Nemes
et al, 2006), Cockayne’s syndrome (Arenas–Sordo Mde et al,
2006), Dubowitz syndrome (Chan et al, 2005) and Schinzel–
Giedion syndrome (Cooke et al, 2002).
Developmental Disturbances Affecting the

Shape of the Teeth (Gemination, Fusion and
Concrescence)
Gemination and fusion have been referred to by various
names in literature such as double teeth, double forma-
tions, joined teeth, fused teeth and dental twinning.
B In gemination, a single tooth bud divides and results in
the formation of teeth with a bifid crown and a common
root and root canal. The crown may be clearly bifid or there
may just be a groove (Figures 46 and 47). It is seen com-
monly in the maxillary anterior regions.
Aquilo et al (1999) classified gemination into four types
based on the morphology.
Type I: Bifid crown with a single root
Crown is larger than normal with a notch on the incisal
edge and a bifid pulp chamber. The root and pulp chamber
are of normal size.
Type II: Large crown with a large root
Generalized microdontia. Courtesy: Department of Crown is larger than normal and has no groove or
Oral Medicine and Radiology, MCODS, Mangalore notch. The pulp chamber is single and large. The root is
wider than normal and has one large root canal.
Type III: Two fused crowns with a single root
There are two crowns with a vertical groove. The cervi-
It is associated with pituitary gigantism but is extremely cal portion of both crowns may be joined. The pulp cham-
rare. Various other syndromes are associated with either ber may be separate. The root is conical shaped and larger
generalized or partial macrodontia such as Berardinelli–Seip than normal.
syndrome (Solanki et al, 2008), KBG syndrome (Brancati Type IV: Two fused crowns with two fused roots
36
Figure 46 Figure 48
Fusion of the central and lateral mandibular incisors.

Gemination of the lower anterior tooth exhibiting a subtle
KLE Society’s Institute of Dental Sciences, Bangalore
groove on the incisal edge. Courtesy: Department of
Oral Medicine and Radiology, KLE Society’s Institute of
Dental Sciences, Bangalore
Figure 49
Figure 47
Fusion of the crown and roots. Courtesy: Department of

with separate or fused root canals (Figures 48–50). Fusion

may occur between normal tooth and a supernumerary
Intraoral periapical radiograph showing a bifid crown with a tooth like mesiodens or distomolar.
common root and root canal. Courtesy: Department of Oral The most reliable method of distinguishing between
Medicine and Radiology, MCODS, Mangalore gemination and fusion is by counting the number of teeth
in the arch. In gemination, the count of teeth remains nor-
mal, whereas in fusion the tooth count will reveal a miss-
ing tooth.
There are two crowns with a vertical groove. The cervi- Concrescence is the union of two adjacent teeth by
cal portion of both crowns is joined along with the pulp cementum alone without confluence of underlying dentin
chambers. (Figures 51 and 52). The common teeth that may show
Fusion occurs when there is union of two tooth buds concrescence are the maxillary molars, especially third
with the confluence of dentin, resulting in single tooth molar and a supernumerary tooth.
37
Figure 50 Figure 52
Tooth specimen showing fusion of the roots. Courtesy: Radiograph showing concrescence. Courtesy: Department of
Department of Oral Pathology, MCODS, Mangalore Oral Medicine and Radiology, MCODS, Mangalore
Figure 51
this may have to be resolved by radiography. Some authors
have reported abnormal patterns in concrescence such as
concrescence between an impacted third molar and an
erupted second molar (Romito, 2004), concrescence between
a third molar and a supernumerary fourth molar in the
mandible (Gunduz et al, 2006) and concrescence of the
crown of an impacted tooth and the roots of the erupted
tooth as a result of the deposition of acellular cementum
on the crown (Sugiyama et al, 2007).
Presence of geminated and fused teeth in deciduous
dentition can cause crowding, abnormal spacing, delayed
or ectopic eruption of underlying permanent teeth. Extrac-
tion may be necessary to prevent an abnormality in erup-
tion. If the permanent teeth are affected, treatment of choice
is determined by patient’s needs. Surgical division followed
by endodontic treatment may be done. Selective shaping is
done with/without placement of full crowns. In some cases,
surgical removal of tooth with prosthetic replacement is
done.
Concrescence (fusion of the teeth via the cementum). Dilaceration

Courtesy: Department of Oral Pathology, MCODS, Mangalore
It refers to a sharp bend or curve anywhere along the length
of the tooth, most commonly at the root (Figures 53–55).
Unlike fusion and gemination, concrescence may be Literature review reveals interesting names that have
post inflammatory too (e.g. trauma, crowding with resorp- been used to describe dilaceration such as ‘scorpion tooth’
tion of interdental bone, caries, etc.). In these conditions, (Moreau, 1985) and ‘hand of a traffic policeman’ (Stewart,
there is discrepancy in the number of teeth and roots, and 1978).
38
Figure 53 Figure 55
Intraoral periapical radiograph reveals dilacerated root of

the mandibular third molar. Courtesy: Department of
teeth can cause delayed eruption of permanent teeth, and

Dilacerated roots of a molar. Courtesy: Department of may require extraction.
Oral Pathology, MCODS, Mangalore Malcic et al (2006) assessed the prevalence of dilacera-
tion in Caucasian patients. Nine hundred and fifty-three
periapical intraoral radiographs and 488 panoramic radio-
Figure 54 graphs were evaluated. The results of the study showed
that dilacerations were found more commonly in the max-
illa and posterior teeth were the highest affected. The
prevalence of the root dilacerations were mandibular third
molars (24.1%), maxillary first molars (15.3%), maxillary
second molars (11.4%) and maxillary third molars (8.1%).
Hamasha et al (2002) studied the prevalence of dilac-
eration in Jordanian adults. 4,655 teeth from 814 dental
records were evaluated. The results of the study showed
that the mandibular third molar teeth were the most com-
monly affected teeth followed by the mandibular first
molars. The maxillary anterior teeth and mandibular inci-
sors were least affected. Almost two-thirds of the dilacera-
tions were in the mandible and approximately 5% were in
anterior teeth.
Radiographically when the roots are dilacerated toward
the buccal or lingual directions, a round opaque area with
a dark shadow in its central region, cast by the apical fora-
men along with the root canal surrounded by a radiolucent
halo formed by the periodontal ligament space is typically
seen. This finding has been termed ‘bull’s eye’ appearance.
Extraction may be difficult and result in root fracture
Dilacerated root of a premolar. Courtesy: Department of
on removal. Perforation of roots is a complication associated
with root canal treatment of the dilacerated teeth.
Factors which affect the normal development of the

Talon Cusp
tooth, including trauma, cyst, tumor, etc., can cause this con-
dition. Dilaceration most commonly affects the anteriors Mitchell in 1892 first described talon cusp as a prominent
and the teeth may be non-vital. Dilaceration of deciduous accessory cusp on the lingual surface of a maxillary incisor.
39
or palatal/lingual surface of an anterior tooth and extends

Figure 56
at least half the distance from the cementoenamel junction
to the incisal edge.
Type 2 (minor talon): A morphologically well-defined
additional cusp that projects from the facial or palatal/
lingual surface of an anterior tooth and extends more than
one fourth, but less than half the distance from the cemen-
toenamel junction to the incisal edge.
Type 3 (trace talon): Enlarged or prominent cingula and
their variations, which occupy less than one-fourth the
distance from the cementoenamel junction to the incisal
edge.
Talon cusp can either occur as an isolated dental
anomaly or found in association with other developmental
disturbances like supernumerary teeth, microdont and
Intraoral photograph showing talon cusp in relation to the macrodonts. Literature reveals the association of talon
maxillary right lateral incisor. Courtesy: Department of cusp with Mohr syndrome (oral-facial-digital syndrome,
Oral Medicine and Radiology. MCODS, Mangalore type II), incontinenta pigmentii achromians (Bloch–
Sulzberger syndrome), Rubinstein–Taybi syndrome, Ellis–
van Creveld syndrome, Sturge–Weber syndrome and
Talon cusp was later defined by Gorlin and Goldman as a Alagille’s syndrome.
very high accessory cusp, which may meet the incisal edge Radiographically, the talon cusp is seen as a radiopaque
of the tooth to give rise to a T- or a Y-shaped configuration. structure, in which the enamel, dentin and occasionally
The name talon was used because this dental anomaly the pulp can be seen. Typically the cusp resembles a
resembled the claw of an eagle (Figure 56). V-shaped structure superimposed over the normal image
Talon cusp predominantly affects the permanent denti- of the crown.
tion. However there are reports of involvement of the The common problems associated with talon cusps are
deciduous dentition. Almost 90% of the talon cusps occur increased susceptibility to caries, occlusal interferences and
in the maxilla. The teeth commonly affected are the lateral esthetic concerns. Other associated problems are speech
incisors, central incisors and rarely the canines. Some disturbances, tongue irritation, accidental cuspal fracture
authors have reported the presence of labial or facial talon and periodontal problems due to excessive occlusal forces.
cusps and very few reports mention the presence of facial This accessory cusp can be removed via periodic selective
and lingual talon cusp occurring on the same tooth. Males grinding, such that adequate time is allowed for deposition
and females are equally affected. of tertiary dentin and pulpal recession.
Etiopathogenesis
Dens Invaginatus
A polygenetic component along with various external
Dens invaginatus has also been referred to as dens in dente
influences is proposed to be the cause for the formation of
and gestant odontome.
the talon cusp.
Dens invaginatus is an enamel-lined surface invagi-
It is believed that the compression of the lateral incisor
nation of the crown or root. Based on the site the condi-
tooth germ between the central incisors and canine during
tion may be subdivided into coronal and radicular forms.
the morphodifferentiation stage will either produce infold-
Cementum-lined invaginations of the root are considered
ing of the dental lamina (dens invaginatus) or an outfolding
variations in the root morphology and are not a type of
(dens evaginatus/talon cusp).
invaginatus.
The coronal form is formed by the infolding of the
Clinical classification of talon cusp
enamel organ into the dental papilla. The radicular form of
Hattab et al classified talon cusps as Type 1 talon, Type 2 dens invaginatus is produced because of the invagination
semi talon and Type 3 trace talon. However this classifica- of the Hertwig’s epithelial root sheath.
tion did not take into consideration the presence of the The invagination can be of varying lengths. It may be
talon cusp on the facial aspect of the tooth. Later on this restricted to the coronal portion or it may extend into the
classification was modified by Stephen-Ying et al, as root. When it extends into the root, it may or may not com-
major, minor and trace talon. municate with the pulp. If the invagination is too exten-
Type 1 (major talon): A morphologically well-delineated sive it gives the appearance of a tooth within a tooth (dens
additional cusp that prominently projects from the facial in dente) (Figure 57). Some of the invaginations can get
40
Figure 57 Figure 58
Coronal dens in dente
Type I Type II Type III
The three stages of dens in dente based on

Oehlers classification. Type I dens invaginatus,
Type II dens invaginatus, Type III dens invaginatus.
Courtesy: Dr Ravikiran Ongole
Intraoral radiograph showing dens in dente appearance.

MCODS, Mangalore Dens Evaginatus
Dens evaginatus has also been referred to as occlusal
enamel pearl, Leong’s premolar, tuberculated cusp, acces-
sory tubercle, occlusal tuberculated premolar and evagi-
dilated, with dystrophic enamel at the base and these are
natus odontomas.
referred to as dilated odontomas. Such teeth usually show
Dens evaginatus is a rare developmental anomaly char-
abnormal crowns. Others may perforate the root and cause
acterized by the presence of an accessory cusp or enamel
inflammatory lesions in spite of the vital pulp.
pearl on the occlusal surface of the premolars occurring
Oehlers in 1957 classified coronal dens invaginatus
between the cusps. The tubercle consists of enamel, dentin
into three types, depending on the depth of invagination
and pulp. The condition occurs as a result of proliferation and
(Figure 58).
evagination of an area of the inner enamel epithelium and
Type I: Invagination ends in a blind sac, limited to the
the underlying dental papilla into the enamel organ during
dental crown.
early stages of tooth development.
Type II: Invagination extends to the cementoenamel
It is believed that the prevalence of this condition
junction, also ending in a blind sac.
ranges between 1 and 4%. It occurs most commonly in the
Type III: Invagination extends to the interior of the root,
Mongoloids, Chinese, Thai and Caucasians. The premolars
providing an opening to the periodontium, sometimes pre-
are most commonly affected followed by the molars,
senting another foramen in the apical region of the tooth.
canines and incisors. The mandibular teeth are five times
The maxillary teeth are more commonly affected. Dens
more frequently affected than the maxillary teeth.
invaginatus most commonly affects the maxillary lateral
Presence of the occlusal tubercle can lead to occlusal
incisor followed by the central incisors, premolars, canines disharmony, attrition and tendency to fracture thereby
and the molars.
resulting in pulpal exposure. The involved tooth can turn
Radiographically dens invaginatus is evident as a loop-
non-vital. Some authors have reported fascial space infec-
like or pear-shaped defect lined by a radiopaque line with
tions and osteomyelitis.
density equal to that of enamel, resembling a tooth within
Grinding of the accessory cusp along with indirect pulp
a tooth. In severe forms of invagination, the crown is mal-
capping is recommended. Non-vital teeth are best treated
formed and an open apex is present.
endodontically.
The point of invagination is caries prone and as a pre-
ventive measure, restoration is recommended. If the tooth
Shovel-shaped incisors
is carious and the invagination is close to the pulp then
indirect pulp capping is done. However pulpally involved Shovel-shaped incisors may be seen in association with dens
invagination is best managed by endodontic treatment. evaginatus. Marginal ridges of the incisors are prominent
41
and lingual surface is hollowed resulting in a shovel shape.

Figure 59
At the cingulum a deep pit or fissure or in some cases dens
invaginatus may be seen. These are caries-prone and hence
should be restored soon after eruption.
Taurodontism
Taurodontism originated from the Greek words ‘tauros’
meaning bull and ‘odontos’ meaning tooth. Like the name A B C D
suggests the tooth resembles the tooth of a bull. The term
taurodontism was first used by Keith in 1913. He defined Shaw’s classification of the subtypes of taurodontism.
taurodontism as ‘a tendency for the body of the tooth to (A) Normal tooth. (B) Mild taurodontism (hypotaurodont).
enlarge at the expense of the roots. According to Witkop (C) Moderate taurodontism (mesotaurodont).
‘taurodont teeth have pulp chambers in which the bifurca- (D) Severe taurodontism (hypertaurodont).
tion or trifurcation is displaced apically, so that the chamber Courtesy: Dr Ravikiran Ongole
has a greater apico-occlusal height than in cynodont teeth
and lacks a constriction at the level of the cementoenamel
junction. The distance from the bifurcation or trifurcation
of the roots to the cementoenamel junction is greater than Figure 60
the occlusal-cervical distance’.
In this condition the body of the tooth (usually the
molars) is enlarged and rectangular in shape at the expense
of the roots. The teeth thus resemble bull’s teeth. The pulp
chamber is large, with a greater apico-occlusal height. It
also lacks the constriction at the cervix. Shaw in 1928 clas-
sified the affected tooth into three subtypes based on the
degree of apical displacement of the pulpal floor, namely
hypotaurodontism, mesotaurodontism and hypertaur-
odontism (Figure 59). Orthopantomograph showing taurodontism in
Taurodontism is more frequently seen in permanent deciduous dentition. Courtesy: Department of
dentition compared to deciduous dentition (Figure 60). The Oral Medicine and Radiology, MCODS, Mangalore
first molar is the most frequently affected followed by the
second and the third molar (Figure 61). Premolars may
also be affected (Figure 62).
Taurodontism may be associated with multiple syn- Figure 61
dromes. A few of the syndrome associated with this condi-
tion have been listed in Table 5.
The condition needs no specific dental management.
However there is difficulty in locating, instrumenting and
obturating pulp canals during endodontic treatment.
Ectopic Enamel
It is a term used to describe the enamel present in unusual
locations like the root. Enamel pearls and cervical enamel
extensions are two types of ectopic enamel seen (Figure 63).
Enamel Pearl (Enameloma)

It is an ectopic mass of enamel which can occur anywhere
on the roots of teeth but is usually found at the furcation Intraoral periapical radiograph showing
area of roots. Maxillary molars are more frequently affected taurodontic maxillary permanent first molar.
Courtesy: Department of Oral Medicine and
than the mandibular molars. Premolars and incisors are
rarely affected. These radiopaque droplets may also contain
42
Figure 62 Figure 63
A B C
Types of ectopic enamel. (A) Normal cervical enamel.

(B) Extension of cervical enamel. (C) Enamel pearl in the
furcation area. Courtesy: Dr Ravikiran Ongole
Intraoral periapical radiograph showing taurodontic

mandibular permanent second premolar. Figure 64
Table 5 Taurodontism and syndrome association

Aarskog syndrome (Dayal et al, 1990)
Amelogenesis imperfecta, severe form (Pavlic et al, 2007)
Amelo onycho hypohidrotic syndrome (Herrerias et al, 1987)
Chondroectodermal dysplasia/Ellis–van Creveld syndrome
(Hunter et al, 1998)
Down syndrome (Moraes et al, 2007)
Goltz–Gorlin syndrome (McNamara et al, 1996)
Hypophosphatemic vitamin D rickets (Goodman et al, 1998)
Hypohidrotic ectodermal dysplasia (Glavina et al, 2001)
Klinefelter’s syndrome (Schulman et al, 2005) Intraoral periapical radiograph showing radiopaque
Lowe syndrome (Tsai et al, 1997) enamel pearl in the furcation area of the mandibular
first molar. Courtesy: Department of Oral Medicine
Microcephalic dwarfism (Sauk Jr, 1973)
Seckel syndrome (Seymen et al, 2002)
Smith–Magenis syndrome (Tomona et al, 2006)
Tricho-dento-osseous syndrome (Wright et al, 2008) The complications associated with the presence of the ecto-
Trisomy 18 (Ribiero et al, 2006) pic enamel in deciduous teeth are delayed exfoliation of
von Gierke’s disease/glycogen storage disease type IA the primary tooth and deviation in the eruption path of
(Avsar, 2007) the succedaneous tooth.
Wolf–Hirschhorn syndrome (Johnston et al, 2006)
XXXXY syndrome (Hata et al, 2006) Cervical Enamel Extensions
(Cervical Enamel Projections)
small amounts of dentin and pulp (Figure 64). Removal of In this condition, enamel extends from the CEJ toward the
the lesion may lead root caries, external resorption and pul- bifurcation of molars forming a triangular extension on
pitis, as the pearl may contain vital pulp tissue. Hence they the buccal surface. Localized loss of periodontal attach-
are not generally treated; maintenance of good oral hygiene ment may be seen with furcation involvement. This may
is recommended. Literature review shows reports of enamel lead to formation of buccal bifurcation cyst. Flattening or
pearls occurring in the furcation area of deciduous teeth. removing the enamel in combination with an excisional
43
new attachment procedure and furcation plasty is done to surface of the crown of the mandibular molar. The proto-
achieve a durable attachment. stylid is usually seen on the first or third permanent molars
or in deciduous lower second molars. It is seen in almost
40% of the population. The appearance can vary from a
Supernumerary Roots/Accessory Roots simple pit in the buccal groove to a furrow or a prominent
This is characterized by an increase in the number of roots, cusp (Figure 69).
commonly in the third molars, mandibular canines and pre- The Uto-Aztecan upper premolar is known to occur only
molars (Figure 65). These supernumerary roots may occur in native Americans, with its highest frequency in Arizona.
due to the disturbances of the Hertwig’s epithelial root sheath It occurs in the permanent upper first premolar. In this
during root formation. During extraction it is important to trait the buccal cusp may bulge out to the buccal aspect
make sure that all the roots are removed. This condition with a marked fossa in its distal shoulder (Figure 70).
may also pose problems during endodontic procedures.
Figure 66
Supernumerary Cusps/Accessory Cusps
The common accessory cusps that are seen are talon cusp,
dens evaginatus, cusp of Carabelli and protostylid of lower
molars. Other rare alterations in morphology are the 6th cusp,
7th cusp, Uto-Aztecan upper premolar, deflecting wrinkle and
sinodonty. These alterations in tooth morphology are race
specific. Occasionally the accessory cusps can be located on
non-specific sites on the tooth surface (Figures 66 and 67).
The cusp of Carabelli was first described in 1841 by Georg
Carabelli, who was a court dentist to the Austrian Emperor
Franz. It was also referred to as Carabelli’s tubercle and
tuberculus anomalus of Georg Carabelli.
This accessory cusp is located on the palatal aspect of
the mesiopalatal cusp of the maxillary molar. Though this
trait is commonly seen on the first molar it may also be
seen involving the second and the third molar. Permanent
Accessory cusp on the lingual surface of the mandibular molar.
and deciduous dentitions may exhibit this trait (Figure 68).
Protostylid was first described and extensively studied by
Dahlberg (1945). Protostylid is a feature on the mesiobuccal
Figure 67
Figure 65
Accessory cusps in relation to the buccal aspect of the

maxillary first molar. Courtesy: Department of Oral Medicine
Tooth specimen showing six roots in a mandibular molar. and Radiology, KLE Society’s Institute of Dental Sciences,
Courtesy: Department of Oral Pathology, MCODS, Mangalore Bangalore
44
Developmental Disturbances in Number of observed. Hypodontia may cause abnormal spacing of teeth,
Teeth delayed tooth formation, delayed deciduous tooth exfolia-
tion and late permanent tooth eruption. Prosthetic replace-
This includes total absence of teeth (anodontia), reduced ment of teeth may be needed.
number of teeth (hypodontia and oligodontia), and increased
number of teeth (hyperdontia/supernumerary teeth). Hyperdontia
Hypodontia and oligodontia Hyperdontia results from development of excess dental

lamina and additional tooth germ formation. This may
Hypodontia is lack of development of one or more teeth. show a hereditary pattern (autosomal dominant) and may be
Oligodontia refers to lack of development of six or more a part of Apert syndrome, cleidocranial dysplasia, Ehlers–
teeth. Anodontia is rare and most cases occur in the pres- Danlos syndrome, Gardner syndrome and Sturge–Weber
ence of ectodermal dysplasia. syndrome. These teeth are referred to as supernumerary
Damage to dental lamina before tooth formation can teeth.
result in hypodontia. Causes of hypodontia include genetic Supernumerary teeth are commonly unilateral. Macro-
(autosomal dominant, recessive or sex-linked patterns), dontia and male predominance are seen. Most develop dur-
trauma, endocrine disturbances, infection, radiation and ing first two decades of life. Single tooth hyperdontia is
chemotherapeutic medications.
Hypodontia may also occur in hereditary syndromes such
as Crouzon syndrome, Down’s syndrome, ectodermal dys- Figure 70
plasia, Hurler syndrome and Turner syndrome (Figure 71).
Hypodontia is more common in females. It usually Lingual
affects permanent third molars, second premolars and lat-
eral incisors in that order. Associated microdontia may be
Figure 68
Mesial Distal
Carabelli cusp
Distal Mesial Buccal
The location of cusp of Carabelli on the The Uto-Aztecan feature in the maxillary first premolar.
maxillary permanent first molar. Courtesy: Dr Ravikiran Ongole
Courtesy: Dr Ravikiran Ongole
Figure 71
Figure 69
Buccal
Mesial Distal
Lingual
Orthopantomograph of a patient suffering from ectodermal
Location of protostylid on the mandibular first permanent dysplasia showing hypodontia. Courtesy: Department of
molar. Courtesy: Dr Ravikiran Ongole Oral Medicine and Radiology, MCODS, Mangalore
45
Figure 72 Figure 73
Double mesiodens in the maxillary incisor region.

Courtesy: Department of Oral Medicine and Radiology, Mesiodens. Courtesy: Department of Oral Medicine and
MCODS, Mangalore Radiology, MCODS, Mangalore
seen more in permanent dentition and approximately 90%

present in maxilla with a strong predilection for anterior Figure 74
region. Most common sites are maxillary incisors, maxillary
fourth molars, mandibular fourth molars, premolars, canines
and lateral incisors in that order. Non-syndrome multiple
supernumerary teeth occur most frequently in mandible and
in premolar region. Supernumerary teeth may be seen in
gingiva, maxillary tuberosity, soft palate, maxillary sinus,
sphenomaxillary fissure, nasal cavity, and between the
orbit and brain.
Several terms have been used to describe supernumerary
teeth according to their location.
Mesiodens: Maxillary anterior incisor region (Figures 72
and 73).
Paramolar: Lingually or buccally to a molar tooth
(Figure 74).
Distomolar/distodens: Distal to third molar (Figure 75).
Supernumerary teeth may predispose the area to subacute
pericoronitis, gingivitis, periodontitis, abscess formation, A buccally placed paramolar. Courtesy: Department of
odontogenic cysts and tumors. Early removal of accessory Oral Medicine and Radiology, MCODS, Mangalore
tooth is recommended.
Supplemental teeth are supernumerary teeth. However
they resemble the morphology of the tooth they are asso-
teeth found in infants at birth is called natal teeth and
ciated with (Figure 76).
those erupting within 30 days after birth called neonatal
teeth. Sponge and Feasby further subdivided these teeth
Disturbances of Eruption of Teeth into mature or immature. It is believed that the incidence
of natal and neonatal teeth is approximately between 0.3%
Premature eruption
and 0.5%.
Deciduous or permanent teeth may erupt prematurely. This These teeth generally erupt in the same position as that
may involve single tooth or the entire dentition. Prema- of deciduous teeth in the arch. These teeth are commonly
turely erupted teeth were earlier known by various other seen in the mandibular incisor region. Based on the reports
names such as ‘congenital teeth’, ‘fetal teeth’ and dentitio of various authors, the site of eruption of natal and neonatal
praecox. teeth are: mandibular incisor region (85%), maxillary incisor
Massler and Savara suggested the terms natal and neo- region (11%), mandibular canine region (3%) and maxillary
natal teeth. According the new terminology deciduous canine and molar regions (1%).
46
Histopathological evaluation of ground sections show

Figure 75
hypomineralized enamel and loss of normal architecture
of the enamel rods. The normal architecture of dentinal
tubules is lost. The teeth have large pulpal chambers.
The presence of these prematurely erupted teeth in
infants can cause difficulty in suckling. Riga–Fede disease
is a well-recognized lesion associated with natal and neo-
natal teeth. It is characterized by the presence of a trau-
matic ulcer on the lingual surface of the tongue or lingual
frenum. The ulcer is formed secondary to the trauma caused
by these teeth during suckling.
Delayed eruption
Grossly delayed eruption of teeth may affect both deciduous
Intraoral radiograph showing a ‘microdontic’ distomolar and permanent dentition and can be localized or generalized.
placed paramolar. Courtesy: Department of Oral Medicine and Local causes include retained deciduous tooth and gingi-
Radiology, MCODS, Mangalore val hyperplasia. Systemic causes include rickets, cretinism
and cleidocranial dysplasia. In most cases the cause may
be difficult to ascertain. One such factor may be the lack
of eruptive force. Treatment of local factors or primary
Figure 76
conditions would lead to eruption of the affected teeth.
Eruption sequestrum
When a molar erupts, the bone overlying the cusps is
resorbed earlier, leaving a tiny irregular spicule of bone,
or sometimes a complex odontoma in the central occlusal
fossa. This may be asymptomatic or cause slight soreness.
It appears as a tiny irregular radiopacity overlying the cen-
tral occlusal fossa but separated from tooth itself. The bone
would ultimately disappear by sequestration during the
eruption. If it remains in the soft tissue it can be removed.
Transposition of teeth
Supplemental teeth bilaterally on the lingual aspect of the
mandibular premolars. Courtesy: Dr Sumanth Peck et al described dental transposition as the positional
interchange of two adjacent teeth, or the development or
eruption of a tooth in a position normally occupied by a
non-adjacent tooth. The transposition can either be com-
Etiopathogenesis The etiology for the occurrence of
plete (crowns and the roots of the involved teeth exchange
these teeth is still not known. Various theories have been
positions in the dental arch) and incomplete (crowns are
proposed to explain the occurrence of these teeth. Some
transposed, but the roots remain in their original positions).
authors believe that excessive development during the ini-
It is estimated that approximately 1% of the population
tiation and proliferation stage will produce these teeth.
exhibit transposition of teeth. It can be seen in the maxil-
Another theory put forth suggests that there could be
lary or mandibular teeth. Transposition can either be unilat-
hyperactivity of osteoblastic cells within the tooth germ. It
eral or bilateral. The common teeth that show transposition
is also believed that a superficial positioning of tooth germs
are canine-first premolar in the maxilla (Figure 77) and
during developmental period may cause premature erup-
the mandibular canine-lateral incisor. Though literature
tion of these teeth.
review reveals almost all cases of transpositions affecting
Endocrine disturbances may also be an etiologic factor.
the permanent dentition, Duncan et al (1996) reported the
When the entire dentition is affected, hyperthyroidism
fusion and transposition of the maxillary right central and
should be considered.
lateral primary incisors.
The prematurely erupted teeth are well-formed and nor-
mal in all respects, except that they may be somewhat Etiology Some authors believe that dental transposition
mobile. occurs because of interchange of developing tooth buds.
47
Figure 77 Classification of transmigrated canines

Type 1: The canine is impacted mesioangularly across the
midline, labial, or lingual to the anterior teeth with
the crown portion of the tooth crossing the midline.
Type 2: The canine is horizontally impacted near the infe-
rior border of the mandible below the apices of the
incisors.
Type 3: The canine has erupted either mesial or distal to
the opposite canine.
Type 4: The canine is horizontally impacted near the infe-
rior border of the mandible below the apices of
either premolars or molars on the opposite side.
Orthopantomograph showing bilateral transposition of
Type 5: The canine is positioned vertically in the midline
canine—first premolar in the maxillary arch. with the long axis of the tooth crossing the midline.
Over retained deciduous canines or missing permanent
canines in a patient can be radiographically evaluated to
assess for impacted or transmigrated teeth.
Several theories have been proposed to account for den-
tal transposition, including the interchange of developing
tooth buds, inherited condition, altered eruption paths, Impacted Teeth
trauma and the presence of retained primary teeth. It is Impaction of a tooth occurs when its eruption is impeded
also believed that root dilacerations of the adjacent teeth by a physical barrier. Impaction of deciduous teeth is rare;
may be a potential etiological factor of canine-premolar it generally involves the second molars, probably due to
transpositions. ankylosis. In the permanent dentition, third molars are
Peck and Peck classified dental transpositions based on impacted most frequently (mandibular commoner than
the teeth involved as: maxillary). This is followed by maxillary cuspids and
❍ Maxillary canine-first premolar (Mx.C.P1) mandibular premolars. In permanent teeth, the causes for
❍ Maxillary canine-lateral incisor (Mx.C.I2) impaction include insufficient maxillofacial development,
❍ Maxillary canine to first molar site (Mx.C to M1) overlying cysts or tumors, trauma, thickened overlying
❍ Maxillary lateral incisor-central incisor (Mx.I2.I1) bone or soft tissue. Impacted teeth may erupt partially or
❍ Maxillary canine to central incisor site (Mx.C to I1) completely encased within the bone. The former may be
❍ Mandibular lateral incisor-canine (Mnd.I2.C). associated with pericoronitis and the latter may cause
resorption of roots of adjacent tooth, periodic pain or tris-
Since they are asymptomatic no active management is mus; dentigerous cysts and adenomatoid odontogenic
necessary. tumors are common in these. Multiple impacted teeth may
be related to syndromes and metabolic disorders such as
Transmigration
cleidocranial dysostosis, Gardner syndrome, Yunis–Varon
The term transmigration is used to describe the pre- syndrome, tricho-dento-osseous syndrome, GAPO [growth
eruptive migration of a tooth across the midline of the jaw. retardation, alopecia, pseudoanodontia (failure of tooth
It is believed that transmigration is unique to the man- eruption) and progressive optic atrophy] syndrome and
dibular permanent canines. However Aras et al (2008) and mucopolysaccharidoses. However literature review also
Aydin et al (2003) reported transmigration of maxillary shows reports of multiple impacted teeth in non-syndromic
canines. individuals (Figure 78).
Various authors have proposed diagnostic criteria for Impaction may be classified according to angulation of
transmigration. Some believe that an impacted mandibu- tooth in relationship to the remaining dentition: mesioan-
lar canine that has crossed the midline more than half of gular impaction (Figure 79), distoangular, vertical (Figure
its length should be considered as transmigrated. Others 80) and horizontal (Figure 81). There can be variation of
suggest that the tendency of a canine to cross the man- angulation in sagittal plane, the impacted third molars
dibular midline is a more important consideration than the may be deflected buccally or lingually (Figure 82). When
distance of migration after crossing the midline. the crown points toward the inferior border of mandible or
Mupparapu (2002) proposed a classification system to when it is completely within the ramus of mandible it is
trace the pathway of transmigration. However, this classifi- referred to as inverted impaction (Figure 83). Occasionally
cation system is presently being improved upon by many interesting patterns of impacted teeth are seen such as ‘sleep-
authors to accommodate various patterns of transmigration. ing molars’ (Figure 84) and ‘kissing molars’ (Figure 85).
48
Figure 78 Figure 80
Orthopantomograph showing multiple impacted teeth in

a non-syndromic individual. Courtesy: Department of
Figure 79
Cropped orthopantomograph showing vertically impacted

mandibular third molar. Courtesy: Department of
Figure 81
Orthopantomograph showing mesioangularly impacted

mandibular third molars bilaterally. Courtesy: Department of
Impacted teeth are usually considered for removal when

they are symptomatic or causing resorption of adjacent and Orthopantomograph showing horizontally impacted
undergoing a cystic degeneration. mandibular third molars bilaterally. Courtesy: Department of
Ankylosed Deciduous Teeth (Submerged Teeth)

In this condition, there is fusion of cementum or dentin with Figure 82
the alveolar bone preventing shedding of the deciduous
teeth. Proposed causes include changes in local metabolism,
trauma, infection and genetic influence. These are commonly
seen in mandibular first molars. The affected teeth lie well
below the occlusal plane; a sharp solid sound is heard on
percussion in contrast to the dull sound of the normal
teeth. Radiographically partial absence of periodontal lig-
ament is observed. The adjacent teeth often incline toward
affected tooth, leading to occlusal and periodontal problems
(Figure 86). Supra-eruption of the opposing tooth may
also be seen. Impaction of underlying permanent tooth Orthopantomograph showing buccoversion of an impacted
may also occur. Since forceps extraction is not possible, mandibular third molar. Courtesy: Department of Oral
Medicine and Radiology, MCODS, Mangalore
surgical removal is recommended.
49
Figure 83 Developmental Disturbances in Structure of

Teeth
Developmental defects affecting the structure of
enamel
Overview of amelogenesis The development of enamel
occurs in two phases:
1. The secretory phase
2. The maturation phase
In the first phase, ameloblasts secrete enamel matrix by
the secretion of enamel proteins like amelogenins and enam-
elins, and also aid in its immediate partial mineralization.
The second phase, the maturation of enamel takes place
by introduction of more mineral that is accommodated by
the removal of water and organic matter. The defects that
Orthopantomograph showing the crown of the third
arise in the structure of enamel may be traced back to
molar directed toward the lower border of the mandible.
occurring at any of these phases. Accordingly, most sys-
MCODS, Mangalore tems of classification of developmental defects of enamel
relied on this convenient yet simplistic view of enamel for-
mation. The defects in enamel may thus be broadly classified
as follows:
1. Hypoplastic enamel, a quantitative defect of enamel
Figure 84 (as a result of defective/deficient enamel matrix for-
mation), and
2. Hypomineralized enamel, a qualitative defect of enamel
(as a result of defective mineralization/maturation of
enamel).
The entire process of amelogenesis is influenced by a num-
ber of environmental influences and genetic mutations,
causing aberrant enamel formation. The further categori-
zation of enamel hypoplasia is done on the basis of the
cause as follows:
1. Hereditary enamel hypoplasia (amelogenesis imperfecta)
Orthopantomograph showing bilateral sleeping molars.
2. Environmental enamel hypoplasia.
MCODS, Mangalore
Figure 86
Figure 85
Orthopantomograph showing submerged mandibular

right side first molar. The radiograph also shows adjacent
Orthopantomograph showing kissing molars on the left side. teeth tipping over the submerged tooth. Multiple impacted
Courtesy: Department of Oral Medicine and Radiology, teeth are also seen. Courtesy: Department of
MCODS, Mangalore Oral Medicine and Radiology, MCODS, Mangalore
50
Genes associated with amelogenesis is the significant time when crowns of all permanent
teeth (except those of the third molars) develop. Hypo-
Numerous genes are expressed in an orderly fashion dur-
plasia of the primary teeth and the cusp tips of the
ing the various stages of the formation and maturation of
first molars would reflect ameloblast damage in utero.
enamel. The protein products of these genes regulate gene
Once teeth have fully calcified, they are immune to
expression and ameloblast function. More than a thousand
these external factors, namely,
genes may potentially be involved in odontogenesis with
a. Ingestion of fluoride
hundreds involved in amelogenesis alone. With such a highly
b. Chemicals
regulated process orchestrated by so many genes, it is not
c. Nutritional deficiencies
surprising that 25–80% of the general population suffers
d. Exanthematous diseases/infections
from enamel defects. Below are mentioned a few candidate
e. Birth-related injuries
genes for the causation of structural defects in enamel.
f. Metabolic disorders
AMELX: Tooth-specific gene expressed in preamelo-
g. Celiac disease.
blasts, ameloblasts and in remnants of the epithelial root
sheath. Its product amelogenin forms the bulk of the pro-
Clinical features
tein in developing enamel and is crucial for the regulation
of the size and shape of enamel crystallites. While the appearance of the hypoplastic enamel varies
ENAM: Expressed predominantly by the enamel organ. depending upon the specific etiology, mostly teeth affected
Its gene product is enamelin that may interact with amelo- by enamel hypoplasia appear to have deficient thickness
genin to determine the length of enamel crystallites. of enamel. This may manifest as any/all of the following:
KLK4: Kallikrein-4 codes for a calcium-independent
1. Affecting a single tooth, a group of teeth, or all of the
serine protease that is secreted during the maturative
teeth.
phase of enamel development. Mutation of KLK-4 results
2. Pits or grooves on the enamel surface (Figure 87).
in poorly mineralized enamel.
3. An overall reduction in enamel thickness.
MMP-20: A gene that codes for a calcium-dependent
4. White in color and opaque (lacking the translucency
proteinase that is a member of the MMP family. Its product,
of normal enamel), later changing color after eruption
enamelysin is a proteinase that cleaves amelogenin and
to yellowish–brown.
plays a major role in processing the enamel matrix proteins.
DLX3: A homeobox gene that is homologous to the
Local infection or trauma
distal-less (Dll) gene of drosophila and is critical for cranio-
facial, tooth, brain, hair and neural development. Local trauma or infection (e.g. abscess formation) can
Ameloblastin (product of AMBN), Tuftelin (product of affect ameloblasts overlying a developing crown, resulting
TUFT1), Amelotin (product of AMELOTIN) and Dentin in enamel hypoplasia or hypocalcification. The affected
Sialophosphoprotein (DSPP) are a few other gene products permanent tooth is also known as ‘Turner’s tooth’ and the
that are involved in amelogenesis. condition referred to as ‘Turner’s hypoplasia’.
Environmental Enamel Hypoplasia

Figure 87
Ameloblasts are sensitive to various forms of injury and
insults, either local or systemic, during the process of amelo-
genesis. The effect of the external factors (like metabolic and
genetic conditions) leaves a lasting impression on the formed
enamel that cannot be erased as enamel does not ‘heal’ unlike
other tissues in the body. With knowledge of the chronology
of various stages in the formation of a tooth and a well-
elicited history from the patient, a good clinician will be able
to trace the event that led to the hypoplasia of enamel.
The known environmental factors influencing ameloblast
function during amelogenesis are:
1. Localized causes:
a. Local infection or trauma
b. Irradiation
2. Systemic causes: Systemic causes may have an effect Grooves and pitting defects in enamel hypoplasia.
on permanent teeth if they occur between the time
MCODS, Mangalore
soon after birth and before the age of 6 years, as this
51
Most commonly seen to affect permanent maxillary

Figure 88
incisors or maxillary or mandibular premolars, as a result
of periapical infection of the deciduous predecessor or
trauma involving the deciduous tooth being forced into
the follicle of the underlying permanent tooth.
The affected tooth exhibits an irregular surface with
pits and most often has a smaller crown than normal. The
tooth may show a mild brownish discoloration.
The severity of the hypoplasia depends upon the sever-
ity of the infection and the stage of development of the
underlying permanent tooth.
Irradiation Radiation in the region of the developing
teeth and jaws results in discoloration of enamel besides
anodontia and microdontia.
Ingestion of fluoride The ingestion of excessive amounts
of fluoride (more than one part per million) results in a Yellowish-brown intrinsic staining of teeth associated with
characteristic form of enamel hypoplasia called as ‘mot- dental fluorosis. Courtesy: Department of Oral Medicine and
tled enamel’, first described by GV Black and Frederick Radiology, MCODS, Mangalore
McKay in 1916.
Mottled enamel is mostly endemic in areas where the occurrence of exanthematous diseases and nutritional
drinking water contains excessive amounts of naturally deficiencies tends to principally affect the permanent inci-
occurring fluoride.
sors, canines and first molars as they form within the first
The severity of mottling however varies even in an endemic 18 months of life. Thus, the type of enamel hypoplasia
area and may depend on the duration of exposure, timing that they cause can also be termed ‘chronological hypo-
of exposure relative to enamel formation and the frequency plasias’. The enamel appears pitted and may even show a
of exposure to high concentrations of fluoride in water. horizontal band of hypoplasia.
Mild-to-moderate fluorosis manifests as occasional
white flecks or enamel spots to white opaque, lusterless Congenital syphilis Dental abnormalities as a result of
areas that cover larger areas of the crown. Enamel opaci- congenital syphilis are fortunately rarely seen nowadays.
ties are seen to occur symmetrically around the dental arch. Hutchinson’s incisor was the term used to denote a char-
Severe fluorosis manifests as pitted, irregular, brown- acteristic appearance of the anterior teeth in congenital
stained teeth that may have a corroded appearance syphilis, where the crowns of the teeth tapered toward the
(Figure 88). incisal edge, giving it a ‘screw-driver’ appearance. The central
Histologically, fluorosed enamel shows a well-calcified portion of the incisal edge was deficient as a result of
surface layer on subsurface areas of diffuse hypomineral- hypoplasia of the middle labial lobe of the incisor.
ization. The perichymata are accentuated. Mulberry molars is the term used to describe the molar
Generally, the teeth affected by endemic fluorosis are teeth affected by congenital syphilis that have smaller
relatively caries-resistant. The patient’s only concern is than normal occlusal tables and a nodular occlusal surface
likely to be esthetic. that resembles the surface of a mulberry. They have also
been referred to as Moon’s molars or Fournier’s molars.
Chemicals Certain therapeutic agents like anticancer
drugs, tetracycline, thalidomide, vitamin D as well as com- Birth-related injuries Hypoxia, premature birth, pro-
pounds like lead, mercury and fluorine are known to cause longed labor are some of the birth-related conditions that
enamel hypoplasia in addition to other defects like micro- may result in enamel hypoplasia.
dontia and hypodontia. It has been noted that the average width of the neonatal
line in primary tooth enamel is wider in children born after
Nutritional deficiencies Rickets is the most common difficult labor, and is narrower in children born through
known cause of enamel hypoplasia. cesarean section. (The neonatal line is a zone of hypocal-
Generalized malnutrition and deficiencies of vitamins cification that serves as a histologic landmark correspond-
A, C and D are known to cause enamel hypoplasia. The ing to the event of birth and denotes the transition from
affected enamel appears pitted. intra-uterine to extra-uterine environment).
Metabolic disorders Enamel hypoplasia of varying sever-
Exanthematous diseases/infections
ity is seen in general systemic conditions like hypocalce-
These include measles, chicken pox, scarlet fever, CMV, mia, hypothyroidism, maternal diabetes and toxemia of
rubella, congenital syphilis, respiratory infections. The pregnancy.
52
Celiac disease A genetically influenced immune-mediated Figure 89

disorder characterized by damage to the small bowel mucosa
as a result of contact with the protein gluten.
The cause of enamel defects seen in celiac disease is
not known, but it is postulated that the gluten-induced
immunological process that occurs between the age of
6 months and 7 years results in malabsorption with atten-
dant hypocalcemia, and damage to the delicate enamel
organ. Aine has classified the specific enamel defects seen
in children with celiac disease and categorized them from
grades I to IV.
Amelogenesis Imperfecta
Amelogenesis imperfecta (AI) represents a group of inher-
Generalized yellowish-brown discoloration of
ited, congenital defects that primarily affect only enamel
teeth in amelogenesis imperfecta.
formation and are not accompanied by morphologic or Courtesy: Department of Oral Medicine and
metabolic defects in other body systems other than tooth Radiology, MCODS, Mangalore
form or eruption.
Amelogenesis imperfecta has also been known as hered-
itary enamel dysplasia; hereditary brown enamel and
hereditary brown opalescent teeth.
Figure 90
Etiology
The trait of AI can be transmitted by either an autosomal
dominant, autosomal recessive, or X-linked mode of
inheritance.
Mutations in the amelogenin gene (AMELX) and enam-
elin gene (ENAM) are believed to cause X-linked AI and
autosomal inherited forms of AI respectively.
Recent reports have mentioned the presence of mutations
in the KLK4, MMP-20 and DLX3 genes in the etiology
of AI.
Clinical features Yellowish-brown intrinsic staining of teeth associated

with pitting defects in the enamel. Courtesy:
The incidence varies between 1:718 (Sweden) and 1:14,000 Department of Oral Medicine and Radiology,
(Michigan, USA); the data varies widely depending on the MCODS, Mangalore
geographical clustering of patients.
Affects both dentitions (deciduous and permanent);
sometimes only a part of the dentition may be affected.
Teeth exhibit a yellow to dark brown discoloration (Figure Revision of the concepts of inheritance/
89); the consistency varying from cheesy to hard. The genetics
teeth exhibit pits and grooves and in some cases, enamel
may be entirely absent (Figure 90). In an autosomal recessive mode of inheritance, both par-
ents carry a single mutated (defective) gene but are pro-
tected by the presence of a normal gene which is generally
Classification
sufficient for normal function. Two defective copies of the
Amelogenesis imperfecta has been subjected to numerous gene are required to produce a disorder (i.e. both copies
classifications but the one proposed by Witkop Jr (1989) is of the gene in each cell are altered). Each child has a 50%
the most popular and widely used classification to date. chance of being a carrier like both parents and a 25% risk
It is based on the predominant clinical and radiographic of inheriting the disorder.
appearance of the defect and on the mode of inheritance In an autosomal dominant mode of inheritance, the
of the trait (Table 6). affected parent has a single mutated gene which dominates
53
Table 6 Amelogenesis imperfecta: classification

Other systems of classification applied to AI include:
Type Clinical appearance Mode of inheritance

1. Weinmann et al (1945); based on phenotype: hypo-
Type I Hypoplastic
plastic and hypocalcified.
Type IA Hypoplastic, pitted Autosomal dominant 2. Darling (1956); five phenotypes based on clinical,
Type IB Hypoplastic, local Autosomal dominant
microradiographic and histopathological findings.
Type IC Hypoplastic, local Autosomal recessive
3. Witkop (1957); five types based on phenotype.
Type ID Hypoplastic, smooth Autosomal dominant
Type IE Hypoplastic, smooth X-linked dominant
4. Schulze (1970); based on phenotype and mode of
Type IF Hypoplastic, rough Autosomal dominant inheritance.
Type IG Enamel agenesis Autosomal recessive 5. Witkop and Rao (1971); based on phenotype and
Type II Hypomaturation
mode of inheritance.
6. Winter and Brook (1975); based primarily on
Type IIA Hypomaturation, pigmented Autosomal recessive
phenotype with mode of inheritance as system for
Type IIB Hypomaturation X-linked recessive
Type IIC Snow-capped teeth X-linked
subclassification.
Type IID Snow-capped teeth Autosomal dominant 7. Witkop and Sauk (1976); based on phenotype and
mode of inheritance.
Type III Hypocalcified
8. Sundell and Koch (1985); based only on phenotype.
Type IIIA Hypocalcified Autosomal dominant 9. Witkop (1989); based on phenotype and mode of
Type IIIB Hypocalcified Autosomal recessive
inheritance; the most widely followed system of
Type IV Hypomaturation-hypoplastic classification of AI.
with taurodontism 10. Aldred and Crawford (1995); based on molecular
Type IVA Hypomaturation-hypoplastic with Autosomal dominant defect, mode of inheritance, biochemical defect and
taurodontism phenotype.
Type IVB Hypoplastic-hypomaturation with Autosomal dominant 11. Hart et al (2002); subclassification based on the
taurodontism
molecular defect associated with AMELX.
12. Aldred et al (2003); based on mode of inheritance,
phenotype (clinical and radiographic), molecular
defect and biochemical result.
its normal counterpart. Each child has a 50% risk of inher-
iting the faulty gene and the disorder. In other words, one
copy of the altered gene in each cell is able to cause the Type I/Hypoplastic AI
disorder. Clinical features There is a deficiency of enamel matrix
In an X-linked disorder, one normal copy of a gene on with subsequent normal mineralization.
the X chromosome is generally sufficient for normal func- The enamel does not develop to normal thickness; at
tion. Women who have a defective gene on one of their places the enamel is so thin that crowns do not meet at
two X chromosomes are protected by the normal copy of contact points. The decreased crown height leads to ante-
the same gene on the second chromosome. This benefit is rior open bite (in about 50% of cases).
not available to men since they have one X and one Y Enamel matrix defects vary from pinpoint to pinhead
chromosome. Each male child of a mother who carries the size pits arranged in rows or columns on labial or buccal
defect has a 50% risk of inheriting the faulty gene and the surfaces of permanent teeth. Sometimes pits and grooves
disorder. Each female child has a 50% chance of being a of hypoplastic enamel are seen in a horizontal fashion
carrier like her mother. Males with an X-linked form of across the middle-third of teeth.
disease are generally more severely affected than females In type IE, the carrier females have alternating bands
with similar mutations. of normal thick and abnormal thin enamel (Lyonization
effect).
The Lyonization effect/Lyon hypothesis/X chromosome
Enamel agenesis is seen in Type IG where the tooth has
inactivation Since the female has two X chromosomes,
a rough granular surface and has no contact with adjacent
a mechanism exists to ensure that only one of them
teeth.
(even if both are normal) will remain active for function-
ing, and the other X chromosome gets ‘switched-off’ or Radiographic features The enamel, although thin, shows
‘inactivated’. normal contrast from dentin. Square-shaped crowns.
A recessive disorder is thought to arise from a defect in
genes that code for enzymes, while a dominant disorder Type II/Hypomaturation AI
results from mutation in a gene that codes for a structural Clinical features Enamel is of normal thickness but has
protein. a mottled appearance. It is slightly softer than normal
54
reveals a family history, presents with all teeth similarly

Figure 91
affected, reports a relevant medical history that resulted in
metabolic dysfunction at the time of enamel formation or
if the clinician can identify a chronological distribution to
the appearance of teeth.
Differential diagnosis
❍ Dentinogenesis imperfecta: Increasing attrition leads
to deposition of secondary dentin resulting in the
obliteration of the pulp chamber in AI. DI may be
excluded from the diagnosis as it exhibits bulbous
crowns, narrow roots, obliteration of pulp chambers in
the absence of attrition and normal radio density of
Orthopantomograph showing enamel having the same
enamel.
radiodensity as that of dentin. Courtesy: Department of
❍ Dental fluorosis: Type II C (snow-capped teeth) may be
mistaken for fluorosis but does not have the accentu-
ated perichymata seen in fluorosis. If fluorosis is sus-
pected, usually the premolars and second permanent
enamel, and is easily penetrated by the point of a probe molars may be spared and the history will reveal
and chips away from the crown. excessive fluoride intake.
Enamel appears clear to cloudy, mottled yellow to brown. ❍ Tricho-dento-osseous syndrome: Type IV A of AI is
Enamel in Type II B has random alternating vertical bands differentiated from tricho-dento-osseous syndrome in
of either opaque white or opaque yellow enamel with the absence of nail, hair and bone changes.
bands of translucent normal enamel. ❍ Widespread enamel defects similar to those seen in
The snow-capped teeth (Type II C) have opaque white AI may also be seen in conditions like epidermolysis
enamel on the incisal or occlusal thirds of the crowns of teeth. bullosa, tuberous sclerosis and oculo-dento-osseous
Radiographic features The enamel has approximately the dysplasia.
same radio density as dentin (Figure 91). ❍ Molar-incisor hypoplasia: This condition (also called
‘cheese molars’) peculiarly affects the first permanent
Type III/Hypocalcified Type AI molars and the permanent incisors, with no symmetri-
Clinical features Enamel initially develops with normal cal affliction to strongly suggest a chronological dis-
thickness, is orange yellow in color at the time of eruption. turbance. It has been suggested that the changes seen
It is friable and soft and consists of poorly calcified matrix are due to a combination of genetic predisposition and
which is rapidly lost by attrition leaving cores of dentin. environmental insult.
The teeth are sensitive to temperature changes. Cervical
enamel is better calcified. Anterior open bite is frequently Management
seen.
The three principles of management of patients with AI
Radiographic features Enamel is less radiopaque than den- involve:
tin. Crowns have a moth-eaten appearance with a radiopaque
line representing calcified enamel in the cervical area. 1. Alleviation of pain and anxiety
2. Restoration and maintenance of the remaining dentition
Type IV This is a combination type wherein features of with regard to esthetics
the hypoplastic and hypomaturation types of AI are seen 3. Maintenance/restoration of the occlusal vertical height.
in combination with taurodontism.
The management of young patients with AI is preferably
Histological features of AI Light microscopic studies of done in three phases.
enamel have their limitations and ground sections of teeth
1. The temporary phase, undertaken during the primary
affected by AI show a reduction in enamel thickness.
or mixed dentition. Posterior teeth in the primary and
Ultrastructural study of enamel in hypoplastic AI shows
early mixed dentition are generally restored with stain-
increased enamel porosity and changes in prism morphol-
less steel crowns, while anterior teeth in the primary
ogy and crystallite organization.
dentition may be restored with composites or poly-
carbonate crowns. (Since the enamel prisms in teeth
Diagnosis
affected by AI are irregular, the affected enamel may
Identification of AI is made primarily by clinical examina- be pre-treated by sodium hypochlorite solution to
tion. A differential diagnosis may be made if the patient enhance bonding).
55
2. The transitional phase, when all permanent teeth have Developmental Defects Affecting the
erupted; this phase continues till adulthood. The anterior Structure of Dentin
teeth in adolescents may be restored using porcelain
veneers. An overview of dentinogenesis
3. The permanent phase, which lasts through adulthood. At the late bell stage of tooth development, the cells of the
The anterior teeth may now be restored with porcelain inner enamel epithelium induce the adjacent cells of the
jacket crowns. The anterior open bite may at times dental papilla to differentiate into odontoblasts, which are
require surgical management. the cells responsible for the formation and mineralization
of the most abundant dental tissue, the dentin. The process
The treatment of AI generally extends over many years
of dentinogenesis involves the deposition of predentin
and requires the patient’s commitment to regular restorative
that subsequently mineralizes to form dentin. The first
procedures and meticulous oral hygiene.
layer of predentin that is deposited acts as a signal to the
Frequent topical fluoride applications and dietary con-
overlying inner enamel epithelial cells to differentiate into
trol are needed to prevent caries. The roughened enamel
ameloblasts and begin secreting the enamel matrix. Seventy
surfaces may retain plaque. The exposed dentin may be
percent of dentin is mineralized, while the remainder is
sensitive.
made up of organic material and water (20% and 10% by
Association of AI with syndromes/conditions weight respectively). The organic component is made up by
collagen (mainly type I with small amounts of types III and
It is worth recalling the proposal made by Witkop (1989) V) and non-collagenous matrix proteins (mainly dentin
that ‘the term amelogenesis imperfecta be limited to those phosphoprotein/phosphophoryn, dentin sialoprotein, dentin
inherited, congenital defects that primarily affect only matrix protein, osteonectin, osteocalcin, etc).
enamel formation and are not accompanied by morphologic The process of dentinogenesis thus involves:
or metabolic defects in other body systems other than tooth
form or eruption’. Not withstanding the classification of AI 1. The differentiation of odontoblasts from ectomesenchy-
proposed by Witkop in 1989 based on this premise, there mal cells of the dental papilla following an organizing
are a number of conditions where enamel defects resem- influence of the inner enamel epithelium.
bling AI do occur, but have no place in Witkop’s classifi- 2. Formation of organic matrix
cation. Aldred et al (2003) have argued that the definition 3. Mineralization of the formed matrix to the extent of
of AI should be changed to incorporate the statement that nearly 70%.
the enamel defects may be associated with morphologic or
biochemical changes elsewhere in the body’. Dentinogenesis Imperfecta
The reported occurrence of autosomal recessive AI
Dentinogenesis imperfecta (DI) is a hereditary develop-
(hypoplastic AI with delayed eruption/failure of eruption
mental defect of dentin formation resulting in the appear-
of permanent teeth) in some cases of nephrocalcinosis
ance of opalescent teeth and occurring in the absence
has raised the issue of AI being considered an indicator
of any systemic disorder. It is the most common disorder
for renal examination (ultrasound), as unrecognized and
affecting the structure of dentin.
untreated nephrocalcinosis is associated with significant
Dentinogenesis imperfecta has also been referred to as
morbidity.
Capdepont’s teeth and hereditary opalescent dentin.
Occurrences of hypoplastic/hypomineralized AI in asso-
ciation with cone rod dystrophy (autosomal recessive Etiopathogenesis
inherited) have been reported. Most of the reported cases
are seen to have occurred in close-knit communities that ❍ Hereditary (autosomal dominant trait) with variable
practice consanguineous relationships. The ocular symp- expressivity.
toms include photophobia, nystagmus and reduced central ❍ Mutations in the DSPP gene (that encodes for dentin
vision, with a gradual loss of night vision. sialophosphoprotein, needed for normal dentin forma-
Kohlschutter–Tonz syndrome is a CNS degenerative dis- tion) mapped to chromosome 4q, resulting in abnor-
ease with convulsions, dementia, epilepsy, spasticity and mally soft dentin.
characteristic ‘yellow teeth’ that are consistent with hypo- ❍ Mutations in the COL1A1 and COL1A2 genes that
calcified AI. regulate the structure of Type I collagen that is a com-
Usher syndrome is a genetically inherited disorder ponent of several soft (skin, joint ligaments) and min-
characterized by progressive hearing loss and retinitis pig- eralized tissues (bone, dentin).
mentosa with progressive loss of vision. A few cases have
Classification
been reported of patients with Usher syndrome having
enamel defects (thin enamel in permanent teeth) that The original classification by Shields, Bixler and El-Kafrawy
resemble the hypoplastic AI. (1973) proposed three types of DI which correlates with
56
Table 7 Dentinogenesis imperfecta: classification Figure 92

Classification by Classification by Witkop Gene mutation
Shields et al
DI type I Dentinogenesis imperfecta Mutation in
COL1A1 and
COL1A2 genes
DI type II Classical hereditary opalescent Mutation in DSPP
dentin gene at 4q 21.3
DI type III DI found in the Brandywine Mutation in DSPP
tri-racial isolate in Maryland gene
Orthopantomograph showing bulbous crowns, short and
blunt roots Courtesy: Department of Oral Medicine and
the classification given by Witkop (1979) as depicted in
Table 7.
[Subsequently, investigators were unable to satisfactorily Figure 93
differentiate clinically and histologically between types II
and III when seen in adults. Many researchers now con-
clude that types II and III are essentially the same with the
latter being merely a variation of type II].
Shields type I was initially known as ‘DI occurring in
association with osteogenesis imperfecta’, but recently, the
term for ‘dentinogenesis imperfecta’ is reserved for similar
cases seen in association with osteogenesis imperfecta. The
preferred term for only the dental defect in the absence of
systemic disease is ‘hereditary opalescent dentin’. However,
in the absence of a universal consensus and for reasons of
convenience, the terms ‘hereditary opalescent dentin’ and
‘dentinogenesis imperfecta’ will be used synonymously.
Clinical features Intraoral periapical radiograph showing short and

blunt roots of maxillary posterior teeth. Courtesy:
Affects approximately 1 in 6,000 to 1 in 8,000 children, Department of Oral Medicine and Radiology,
with a predilection for occurrence in whites. Teeth exhibit MCODS, Mangalore
an unusual translucency and opalescence, and the color of
the affected teeth varies from yellowish brown to bluish
gray. Affected teeth have bulbous crowns with prominent Shell teeth are so called because they appear like thin shells
cervical constriction, and narrow roots. Deciduous teeth are of dentin covering large pulps. The enamel may be of nor-
affected more severely, while the permanent second and mal thickness. The deficiency in thickness of dentin is due
third molars that develop later are least affected in DI. to insufficient or defective dentin formation and it may
Enamel fractures easily or rapidly undergoes attrition extend to involve even the crown.
exposing the underlying dentin (this may be due to the den-
tinoenamel junction being straight instead of scalloped, Histological features
thus rendering it unable to withstand shearing forces).
Affected teeth are less susceptible to decay (as a result of Other than a thin layer of mantle dentin, the rest of the den-
defective dentin providing fewer pathways for invasion of tin has fewer tubules, which are wide and irregular. Areas of
dental caries). atubular dentin are also seen partly or totally obliterating
the pulp chamber and root canals. The dentinoenamel
junction is smooth instead of being scalloped. Vascular
Radiographic appearance
inclusions are sometimes seen in the dentin (probably rem-
Short, blunt roots with partial or total obliteration of the nants of pulp tissue). Biochemical analysis of dentin shows
pulp chambers and root canals. Crowns of teeth appear increased water content and a decreased mineral content
bulbous with cervical constriction (Figures 92–94). when compared to normal dentin. Following the loss of
Shell teeth are a rare radiographic variant of DI first enamel, the defective dentin rapidly wears away because
seen in the Brandywine tri-racial isolate in Maryland, USA. of the low micro hardness.
57
A defect in Hertwig’s epithelial root sheath causes it to

Figure 94
fragment and these fragments get incorporated into the
dental papilla where they induce the formation of globules
of dysplastic dentin.
Clinical features
Type I/radicular dentin dysplasia The crowns of affected
teeth in both dentitions are normal in shape, size and
color. Premature exfoliation of erupted affected teeth (due
to their short roots). Affected teeth may be more resistant
to dental caries. Pattern of tooth eruption is normal.
Type II/coronal dentin dysplasia Rarer than Type I. The
crowns of affected teeth in the primary dentition appear
opalescent yellow, brown or bluish, whereas the perma-
nent teeth appear normal in color. Periapical lesions are
Intraoral periapical radiograph of mandibular posterior infrequent when compared to Type I.
teeth showing bulbous crowns, short and blunt roots
with total obliteration of the pulp chambers and root canals. Radiographic appearance
Type I/radicular dentin dysplasia The roots are extremely
MCODS, Mangalore
short, blunt and tapering or may be absent in both
dentitions.
Primary teeth show total pulp obliteration, while the per-
Management manent teeth show pre-eruptive pulpal obliteration resulting
in crescent-shaped pulpal remnants (chevrons of pulp)
The rapid loss of enamel and wearing away of dentin has parallel to the cementoenamel junction.
to be compensated in order to maintain vertical dimension Periapical radiolucencies (representing chronic granu-
for function. However, like many other structural defects lomas, cysts or abscesses. These lesions may have devel-
in teeth, it is increasingly difficult to retain the affected oped as a result of microscopic communication between
teeth. The risk of pulp exposure is high in such teeth, and the the residual pulp and the oral cavity in the absence of
cervical constriction renders it liable to cervical fracture clinically apparent dental caries) may be associated with
when teeth are covered by crowns or used as abutments. teeth that appear to be vital.
Type II/coronal dentin dysplasia Root length is normal
Dentin Dysplasia in both dentitions.
The primary teeth show obliteration of pulp chambers.
The term ‘dentin dysplasia’ was given by Rushton to
Permanent teeth exhibit enlarged pulp chambers (thistle-
describe a rare hereditary condition affecting dentin for-
tube shape in anterior teeth and premolars or flame-shaped
mation that was first described by Ballschmiede in 1920 as
in molars) with multiple foci of radiopacity/pulp stones.
‘rootless teeth’. The enamel is normal, while the dentin is
atypical and there is abnormal pulpal morphology. It has
Histological features
also been referred to as rootless teeth.
Dentin dysplasia is of two types; Type I/radicular den- Type I/radicular dentin dysplasia Enamel, mantle dentin
tin dysplasia (rootless teeth) is so called because the and most of the coronal dentin appear normal.
crowns of the primary and permanent teeth seem normal Deeper layers of dentin and all of the root dentin are
while the roots appear short/stunted. Type II/coronal dentin dysplastic and shows atypical, amorphous, tubular pat-
dysplasia shows a defect in the crowns of primary teeth, terns. Numerous calcified, spherical bodies (denticles?) dis-
but the lengths of the roots seem normal. rupt the tubular course of dentin formation with the result
that dentinal tubules are displaced giving rise to the classic
Etiopathogenesis ‘lava flowing around boulders’/‘water streaming around
boulders’/‘cascading waterfall’ appearance.
Hereditary (autosomal dominant trait).
Mutations in the DSPP gene (that encodes for dentin Type II/coronal dentin dysplasia The primary teeth show
sialophosphoprotein) may be responsible for some cases of altered dentin, resembling the changes seen in DI.
type II dentin dysplasia. (This is similar to the mutation The permanent teeth have normal enamel and coronal
seen in DI type II). dentin, while the roots exhibit atubular, amorphous dentin
58
and large areas of interglobular dentin. (The atypical

severe, DGI-II intermediate, and the DGI-III phenotype
dentin seen in coronal dentin dysplasia has a large amount
the most severe form of the disease. While not studied in
of Type III collagen).
as much depth and interest as the DSPP, the DMP1 (den-
tin matrix acid phosphoprotein gene) is another candi-
Differential diagnosis date gene for these dentin defects. DI type I is the dental
Teeth affected by DI may resemble those of dentin dyspla- phenotype associated with osteogenesis imperfecta and
sia. The differentiating features include: people with this disorder may best be categorized as hav-
ing osteogenesis imperfecta with DI.
Dentin dysplasia Dentinogenesis
imperfecta
Color Yellow to brown to Yellow to brown to
bluish bluish
Regional Odontodysplasia
Crown morphology Normal, shape and Bulbous crown with Regional odontodysplasia is a rare, localized, developmen-
size of crown cervical constriction tal disorder of dental origin with affected teeth having
Obliteration of pulp Occurs 5–6 years Occurs before tooth characteristic clinical and radiographic appearances. This
chamber after eruption of the eruption condition has been described under various names by dif-
tooth (type II) ferent authors, but Zegarelli (1963) is credited with coin-
Thistle tube-shaped Mostly present Absent ing the term ‘odontodysplasia’ to describe a ‘peculiar dental
pulp chamber anomaly of unknown cause’. Later, Pindborg (1970) pre-
Root morphology Normal appearing Short, narrow roots ferred the term ‘regional odontodysplasia’ to describe the
roots (type II) segmental and localized nature of this condition.
No roots (type I) Regional odontodysplasia has also been termed as ghost
teeth, odontogenesis imperfecta, odontogenic dysplasia, uni-
Management lateral dental malformation, AI non-hereditaria segmentalis
and localized hypoplasia (Turner teeth).
Meticulous maintenance of oral hygiene is the key to pro- The term ‘regional odontodysplasia’ best describes the
longed retention of teeth affected by dentin dysplasia, segmental and localized nature of this condition that is of
although the presence of short roots and periapical lesions unknown etiology and affects enamel, dentin and pulp.
may make it seem a daunting task for the dentist. Endodontic
and periodontal treatment is accomplished reasonably well Etiology
in teeth affected by coronal dentin dysplasia.
The cause of this rare dental developmental disorder is not
precisely known, but a few theories have been proposed.
Making Sense of It All! Is it DGI? Or is it DD?
1. A somatic mutation in early tooth development (prob-
Or are they all the same?
ably in the developing dental lamina) that disrupts
This is no doubt puzzling as for more than 30 years, the odontogenesis. This may explain the involvement of
two entities were classified using clinical, radiographic, both deciduous and permanent dentitions.
and histopathologic features. This nosology seemed to 2. A disturbance in vascular supply that creates local
suit everyone fine, even though Shields et al (1973) made ischemia, thereby affecting odontogenesis. Many cases
a mention that ‘the heritable dentin defects can be of odontodyplasia have been reported to be associated
viewed as a continuum’. Recent research has focused on with hemangiomas or vascular nevi adjacent to the
the genetic mutations that underlie these non-syndromic affected teeth. (Some researchers have even proposed
heritable defects of dentin. Mutations in the DSPP gene that the irregularities in the dental hard tissues are a
(dentin sialophosphoprotein gene) cause varied defects result of the ‘blood overflow from vessels’ that affects
in dentin and the defects are seen to correlate with the the local concentration of magnesium and sodium
severity of the mutation. Accordingly, a new concept is around the crystals).
emerging of referring to DI and dentin dysplasia as 3. Activation of a latent viral infection of the tooth germ
‘DSPP-associated dentin defects’. Beattie et al (2006) rec- during odontogenesis.
ommend the setting-up of a gene-based classification
Other theories that have been proposed but are unsatisfac-
system that would group all of the DD-II, DGI-II, and
tory to explain the occurrence of ghost teeth are a heredi-
DGI-III cases that are associated with DSPP defects into
tary basis, medications taken during pregnancy, irradiation,
a single category, and then make distinctions based upon
and failure of migration of neural crest cells, systemic
severity. According to the same designations as are cur-
disorders, Rh incompatibility, nutritional and metabolic
rently in use, the DD-II phenotype would be the least
disorders.
59
Clinical features Figure 95

Age at presentation is variable (4–23 years); the condition
is often diagnosed at the time of eruption of primary and
permanent teeth. Affects both primary and permanent
dentitions.
Generally, the disturbance is localized to one arch, most
often the maxillary left quadrant (incisors and canines).
Females are affected more often than males (1.7:1); there
is no racial predilection observed.
Pulp or periapical pathology may be associated with
the affected teeth even in the absence of gross dental car-
ies (the pathogens may gain access to the pulp through the
Orthopantomograph showing irregularly shaped
clefts in the defective enamel and dentin). Patient may teeth with incomplete root formation in regional
present with delayed/incomplete eruption of teeth and odontodysplasia. Courtesy: Department of
unesthetic appearance of affected teeth. Oral Medicine and Radiology, MCODS, Mangalore
Affected teeth appear hypoplastic and small, discolored
yellow to brown with the enamel surface having shallow
furrows to severe pits and grooves. The gingiva surround-
ing the affected teeth may be enlarged.
Regional odontodysplasia Dentinogenesis
The affected teeth have a ‘ghost-like’ appearance because (RO) imperfecta
of a decreased radiopacity and lack of distinction between Hereditary basis Absent Present
the enamel and dentin.
The teeth have an irregular shape with hypoplastic Enamel hypoplasia Present Absent
crowns. Affected teeth have reduced thickness of dentin with Involvement of Isolated segment of the All primary teeth
large pulp chambers, presence of pulp calcifications, incom- teeth arch in either dentition/ involved
plete root formation and wide open apices (Figure 95). few teeth involved
Unerupted teeth may be surrounded by a pericoronal
radiolucency (representing an enlarged follicle) with abnor- The teeth affected by RO may also be mistaken for odont-
mal foci of calcification interspersed. The trabeculae of the omes or as being involved by dental caries.
alveolar bone adjacent to the affected teeth may show rar-
efaction. Periapical abscesses may be seen in relation to Management
the affected teeth.
Depending upon the vitality of the pulp, pulpotomy or
apexification may be performed to stimulate root forma-
tion. Affected primary teeth may be treated by pulpotomy
Undecalcified ground sections of the affected tooth have and restored with steel crowns.
thin, pitted enamel with disorganized/altered prismatic However, most dentists prefer to extract the affected
structure. The dentin is thinner than normal, and contains teeth for esthetic reasons and rehabilitate the patient with
large areas of interglobular dentin, containing clefts that prosthesis. This seems a safer option as the longer the
may communicate with the pulp. Areas of amorphous den- affected teeth are retained; the higher is the risk of devel-
tin and cellular dentin may also be seen with occasional opment of pathology. However, the clinician must weigh
capillaries. The dentin has fewer tubules than normal. the choice against the psychological effects of a youngster
The predentin zone is very wide. The pulp chambers are having to remove teeth with a resultant decrease in alveo-
enlarged and contain pulp stones. Dental follicles are often lar bone height, potentially decreasing the ability to opti-
enlarged and contain epithelial rests and calcifications. mally restore/rehabilitate the affected area. The possibility
Scanning electron microscopy reveals thin enamel with of the future use of implants may be discussed with the
irregular enamel prisms and irregular crystalline aggregates. patient.
60
CHAPTER
Orofacial Pigmentation
Disorders
Ajit Auluck, Manuel Thomas
3
➧ Pigmented Lesions of Oral Mucosa ➧ Pigmentation of Teeth
Molecular and Pathologic Correlation of Pigmentation Extrinsic Discoloration
Metal Pigmentation Intrinsic Discoloration
Diffuse and Multiple Pigmented Lesions
Focal/Localized Pigmented Lesions
PIGMENTED LESIONS OF ORAL MUCOSA tooth. Amalgam particles can be left behind in the oral tissues
after root canal treatment or retrograde amalgam filling. The
Oral mucosa can have a variety of discolorations like blue, silver particles from the embedded amalgam restorations
black, brown or yellow because of endogenous pigments slowly leach out and stain reticulum fibers causing gray/
or exogenous materials. Recognition and diagnosis of pig- black discoloration of mucosa which is called as amalgam
mented lesions of the oral mucosa is an important aspect tattoo.
of clinical dental practice because it may be suggestive of an Lead pencils contain graphite which is another source of
underlying systemic or metabolic disorder, or it can be an exogenous pigmentation which usually occurs in the palate.
initial stage of malignancy. Therefore, dentists must deter- Rarely when the graphite fragment gets embedded in the
mine the cause of pigmentation, ask relevant questions in palatal tissues following trauma, the mucosa covering this
the history to determine the cause and request appropriate fractured fragment gets discolored and appears as a bluish
investigations for early diagnosis and prompt treatment. black focal pigmented area.
In heavy metal poisoning also we can observe oral
mucosal pigmentation because of formation of metal sul-
Molecular and Pathologic Correlation of fides which get precipitated in inflamed areas of gingiva
causing bluish black discoloration. Table 1 summarizes the
Pigmentation
type of exogenous substances that can cause oral mucosal
A pigment is any organic or inorganic coloring substance. pigmentation.
A pigmented lesion can be defined as an area of altered col- Oral mucosal pigmentation can also occur due to variety
oration of the oral mucosa either because of physiologic or of endogenous pigments. Each of these endogenous pigments
pathologic process because of deposition of endogenous or can result in a distinct oral discoloration suggestive of var-
exogenous pigments or embedded foreign material in the ious disease processes which are described in Table 2. Blue,
tissues. bluish red and purple type of oral pigmentation generally
Exogenous substances cause oral mucosal pigmentation occur as a consequence of blood or vascular disorders. Black,
because these substances are embedded in the oral tissues brown or gray discoloration of the oral mucosa is because
either by direct trauma or iatrogenic implantation, amalgam of melanin or hemosiderin pigments. Yellow discoloration
being the most common. Cavity preparation of a tooth with of oral mucosa is because of bilirubin deposition or inges-
an existing restoration (secondary caries) by a rotary bur can tion of large amount of beta-carotene. The pigmented
forcefully entrap some fine amalgam particles into the oral lesions can either be localized or diffuse depending upon
tissues. Amalgam can also enter oral tissues while removing their etiology or disease process which they manifest.
old or fractured restorations. It can also accidentally enter Blood pigments can cause red, bluish red or brown pig-
into an extraction socket during extraction of a restored mentation of oral mucosa. These pigments are deposited into
61
the connective tissues after lysis of erythrocytes causing capacity of cells to store melanin pigment is exceeded and
extravasation of hemoglobin. The extravasation of blood then melanin spills into the underlying connective tissues
into soft tissues is attributed to trauma, capillary fragility, into the adjacent basilar keratinocytes. This process is called
platelet defects, or clotting disorders. This extravasated as basilar melanosis and melanin incontinence.
hemoglobin is acted upon by the enzymes to form hemo- In the oral cavity we can observe melanosis over the healed
siderin, which is further broken down into bilirubin and areas which had an earlier injury. When the traumatized
biliverdin, all of which cause oral pigmentation. Therefore, oral epithelium regenerates, usually there is overproduction
the same endogenous pigments may result in different of melanin by the melanocytes that repopulate this region
types of oral pigmentations over a period of time. An early causing post inflammatory pigmentation.
hematoma is bluish red in color while a late hematoma Certain hormones and drugs can also influence produc-
becomes dark brown in color. Blood pigments are usually tion of melanin. The adrenal cortical–hypothalamic axis is
cleared from the skin or mucosa within 2 weeks. affected by hypofunction of the adrenal cortex. As serum
Melanin causes blue or blue black discoloration of oral corticosteroid levels decreases, adrenocorticotropic hormone
mucosa. Melanin is produced from an amino acid tyrosine (ACTH) production by the posterior pituitary increases. ACTH
by the melanocytes within the membrane-bound organ- also has a melanocyte stimulatory function so melanin
elles called as melanosomes. These melanocytes are found production increases and it causes diffuse melanosis of the
in the basal layers of the oral epithelium. Melanin is also oral tissues. Therefore oral mucosal pigmentation can also
produced by the nevus cells, which originate from the neu- occur in endocrine disorders. Certain drugs like minocy-
ral crest cells and are found in the skin and mucosa. Color cline can also increase the production of melanin within
of pigmented lesions caused by melanin deposition may the cells causing diffuse pigmentation of oral mucosa and
be brown, blue, gray or black, depending on the amount of is called as drug-induced melanosis.
melanin produced as well as on the location of melanin Oral pigmented lesions can also occur due to the prolif-
within the tissues. eration of melanocytes as observed in the cases of nevi and
Oral lesions can occur either due to excessive production melanoma. Melanocytic nevi develop during childhood and
of melanin pigment or due to proliferation of melanocytes. rarely arise in adult life. Most nevi originate from the mela-
Sometimes there is no increase in the number of melano- nocytes in the basal layer of epithelium and proliferate along
cytes but there is only an increase in the synthesis or pro- the junction with the connective tissue. These are called as
duction of melanin pigment. Over a period of time the junctional nevi. Later these melanocytes drop off into the
connective tissue to form islands of cells and are called as
compound nevi. Later all these cells are completely
detached from basal epithelium and form clusters in the
Table 1 Type of exogenous foreign materials causing pigmen-
dermis or submucosa and are called as intradermal nevi.
tation of oral mucosa
Rarely do we observe blue nevi in oral cavity and they are
Source Color Etiology comprised of spindle-shaped melanocytes which synthe-
Silver, amalgam Gray, black Iatrogenic implantation, size copious amounts of melanin pigment.
trauma Occasionally, bluish discoloration of oral mucosa can be
Graphite Gray, black Tattoo, trauma due to optical phenomenon as a consequence of accumula-
tion of fluid within the epithelial layers. For example a ran-
Lead, mercury, Gray Ingestion of paints,
bismuth medicines, poisoning
ula appears blue in color because the mucous absorbs most
of the visible wavelength except blue.
Chromogenic Black, brown, Superficial colonization
In albinism the gene for tyrosinase is mutated. In vitiligo,
bacteria green
depigmented patches are caused by diminished number of
Table 2 Type of endogenous pigments, the discoloration caused by them and disease process they indicate
Pigment Color Disease process
Hemoglobin Blue, red, purple Varix, hemangioma, Kaposi sarcoma, angiosarcoma, hereditary hemorrhagic telangiectasia
Hemosiderin Brown Ecchymosis, petechiae, thrombosed vein, hemorrhagic mucocele, hemochromatosis
Melanin Brown/black/gray Melanotic macule, nevus, melanoma, hormonal imbalance, drugs
Bilirubin Yellow Jaundice/liver disorder
Carotene Yellow Precursor of vitamin A
62
Chapter 3 – Orofacial Pigmentation Disorders
melanocytes. However, these entities are rarely seen in the in the submucosa. Histopathologically, we may observe a
oral cavity. giant cell reaction surrounding these amalgam particles.
Mercury poisoning
Metal Pigmentation
Mercury poisoning can be because of acute or chronic
Pigmentation of the oral cavity caused by foreign materi- exposure of mercury vapors. Clinical features include gas-
als or metals is called exogenous pigmentation. The impor- tric disturbances, diarrhea, excitability, headache and men-
tance of recognizing oral mucosal pigmentation caused by tal depression. Patients complain of gastric disturbances,
heavy metals lies primarily in the identification and treat- diarrhea, excitability, headaches and mental depression.
ment of the cause to avoid severe systemic toxic effects. Patient may have tremors of lips and extremities, dermati-
Depending on the type of metal implicated, a number of tis and nephritis.
systemic signs and symptoms may be associated with Oral manifestations include increased salivation (pty-
chronic exposure of metals. alism) as mercury is excreted in saliva. Tongue may be
enlarged and painful (glossodynia). There may be hyperemia
Amalgam tattoo and swelling of gingiva, ulcerative stomatitis, loosening and
exfoliation of teeth.
Amalgam tattoo is the most common solitary focal pigmen-
Acrodynia (Swift disease, Pink disease) is idiosyncratic
tation lesion of the oral mucosa. These lesions are gener-
reaction to large doses of amalgam. It is an uncommon
ally less than 1 cm (rarely amalgam tattoos can be large)
mercurial toxicity reaction in which skin is also involved.
and appear as flat, gray-black to blue-black color macules.
It usually occurs after ingestion of mercury from powder,
They are usually found in close approximation to a resto-
ammoniated mercury ointment, calomel lotion. It is mainly
ration. Majority of the lesions are located on the buccal
seen in the children below the age of 5–6 years. Manifesta-
mucosa, gingiva and alveolar mucosa with mandibular
tions are widespread involving the hand, feet, nose and
region being more affected as compared to the maxillary
cheeks which become red or pink in color, cold and
region (Figure 1). All these lesions are asymptomatic and
clammy like a raw beef. Skin may have maculopapular
are discovered during routine dental examination. If par-
rashes with pruritis. Patients may complain of irritability,
ticles are large enough they can be viewed with help of a
photophobia and muscular weakness. Children may be
radiograph (taken with reduced exposure parameters for soft
able to remove their hair in patches.
tissues). The amalgam granules and fragments are found
Oral manifestations include profuse salivation, gingiva
mainly in the lamina propria but were sometimes also seen
is painful and ulcerated, teeth may become loose and exfo-
liate and bruxism is a common finding.
Figure 1 Arsenic
Arsenic in both organic and inorganic forms may produce
acute or chronic symptoms. Oral manifestations may include
increased salivation, gingivitis, and oral ulcerations. Expo-
sure produces severe edema of the eyelids, gastrointestinal
irritation, and both central and peripheral neuropathies.
Arsenic levels can be assessed by complete blood
count, urine analysis and hair and finger nail clippings.
The condition can be managed by removing the offending
agent followed by gastric lavage and chelation therapy
with d-penicillamine.
Bismuth
Bismuth is used in treatment of syphilis and dermatological
disorders. Patients usually have systemic complaints and it
is characterized by ‘bismuth grip’, muscular cramps in the
Amalgam tattoo on the left buccal mucosa in close vicinity abdomen.
to the restoration. The lesion also shows faint white Orally, we usually see bismuth line which is a bluish
keratotic striae indicative of a lichenoid reaction.
black line in marginal gingiva confined to gingival papilla.
Bismuth may react with hydrogen sulfide produced by
63
the bacteria to form bismuth sulfide that gets precipitated

Figure 2
around periphery of an ulcer or erupting molar. Patient
may also complain of burning sensation and metallic taste
in the mouth.
Lead
Lead (plumbism) is an occupational hazard seen in plumb-
ers due to acute or chronic exposure because of inhalation
of lead vapors or dust. It is also seen among the children who
chew wood painted with lead.
Clinical features include gastrointestinal symptoms like
nausea, vomiting and constipation. Patients may have enceph-
alitis or peripheral neuritis characterized by wrist drop or foot
drop. Patients may have hypochromic anemia with baso-
philic stippling in red blood cells.
Oral manifestations include linear bluish black discol-
oration seen in the gingival margin called Burtonian line. Grayish black pigmentation of the oral mucosa characteristic
Gingivitis, ulcerative stomatitis, excessive salivation, metal- of physiologic pigmentation. Courtesy: Department of Oral
lic taste, or rarely bismuth may get deposited in the decid- Medicine and Radiology, MCODS, Mangalore
uous teeth too.
Silver
Racial pigmentation mainly occurs in the childhood.
Argyria (silver poisoning) is caused due to chronic exposure However during early years it may not attract patient’s
due to occupational hazard by silver nitrate. It can result attention and may be observed after puberty. Racial pig-
in pigmentation of both the skin as well as the mucous mentation is asymptomatic and does not require any
membrane. Exposure to silver causes a violet marginal line, treatment.
often is accompanied by a diffuse bluish-gray discoloration
throughout the oral mucosa. It can also be associated with Drug-induced pigmentation
neurologic and hearing damage. Histologically we can
observe silver particles staining the reticular fibers. Many medications when taken over a long period of time can
cause oral mucosal pigmentation. Drug-induced pigmenta-
tion can be due to increased synthesis and accumulation of
Diffuse and Multiple Pigmented Lesions melanin pigments, deposition of the drug or its metabolites
into the oral tissues or deposition of iron after damage to
Racial/physiologic pigmentation
the dermal vessels (Table 3).
Physiologic pigmentation of the oral tissues is clinically Chloroquine and other quinine derivatives which are
manifested as multifocal or diffuse melanin pigmentation usually used in the treatment of malaria and cardiac arrhyth-
with variable prevalence in different ethnic groups. Gingiva mia can cause pigmentation of oral tissues due to a direct
appears darker in color among Africans, Asians, Caucasians stimulating effect on the melanocytes (Figure 3). According
and Hispanics and other dark skinned people. The variabil- to some of the studies these drugs usually cause pigmenta-
ity in the color of the oral tissues is not due to the differ- tion of the palatal tissues.
ence in the number of melanocytes is same but this color Minocycline is another drug causing pigmentation of
difference is due to the difference in the activity of the oral tissues. It is a synthetic tetracycline that is commonly
melanocytes. In dark skinned people melanocytes are used in the treatment of acne vulgaris. Tetracycline causes
more active as compared to the fair skinned people. pigmentation of only the bones and teeth but minocycline
Physiologic pigmentation is seen as diffuse macular pig- can also cause pigmentation of soft tissues. It usually causes
mentations that may be brown, gray or black in color and diffuse brownish discoloration of the hard palate, gingiva,
can appear anywhere in the mouth with buccal surface and mucous membranes and tongue.
gingiva being most commonly involved. On the gingiva it Oral pigmentation can also be due to intake of birth con-
appears as well-demarcated, ribbon-like, dark brown control pills. Chloasma is the term which is used to describe
tinuous band that does not extend to involve the marginal perioral and periorbital pigmentation in such patients
gingiva. Occasionally pigmentation may also be seen on the (Figure 4). The pigmentation usually occurs as a diffuse
tongue, lips and lingual gingiva as diffuse brown patches with brown macular pigmentation which is asymptomatic and
ill-defined or diffuse borders (Figure 2). lesions resolve upon cessation of drug intake. These lesions
64
Table 3 List of drugs that can induce oral pigmentation Figure 4

Bleomycin
Busulphan
Clofazimine
Chloroquine
Chlorpromazine
Cyclophosphamide
Doxorubicin
Estrogen
5-Fluorouracil
Gold
Hydroxychloroquine
Ketoconazole
Minocycline Perioral and periorbital pigmentation. Courtesy: Department
Tetracycline of Oral Medicine and Radiology, KLE Society’s Institute of
Quinacrine hydrochloride
Zidovudine
Figure 5
Figure 3
Smoker’s melanosis of the buccal mucosa associated with

leukoplakia. Courtesy: Dr Ajit Auluck
Brownish-black pigmentation of the lips and buccal mucosa
caused by chloroquine. Courtesy: Dr Ajit Auluck accentuate the pigmentation in dark-skinned patients.
Clinically, there may be multiple diffuse brown pigmented
macules of less than 1 cm in diameter. These lesions can
usually occur due to the hormonal changes which influ-
occur anywhere in the mouth but may be usually localized
ence melanocyte stimulation.
on the attached labial anterior gingiva and the interdental
papillae of the mandible. These lesions are completely
Smoker’s melanosis
asymptomatic and benign in nature (Figure 5).
Tobacco smokers have more intense pigmentation of oral Microscopically there is evidence of basilar melanosis
mucosa as compared to non-smokers. Smoker’s melanosis but there is no melanocyte proliferation. The increased
is more common in females. Women are more commonly melanin production may be a biologic defense mechanism
affected than men because of synergistic effect between against the noxious agents present in the tobacco smoke.
the female sex hormones and smoking. Smoking may The intensity of the oral pigmentation is directly related to
cause oral pigmentation in light-skinned individuals and the duration and amount of smoking. Smoker’s melanosis
65
Figure 6 Figure 7
Diffuse pigmentation of palate in a patient with Addison’s

disease. Courtesy: Dr Ajit Auluck
Perioral pigmentation of the lip in Peutz–Jeghers syndrome.
usually disappears within 3 years of smoking cessation.

Biopsy must be advised whenever there is a surface eleva- Melanin pigment can be seen in the basal layer of the epi-
tion or increase in the pigment intensity or rapid increase thelium. Management involves treatment of the underly-
in the size of the lesion. ing cause and corticosteroid replacement therapy.
A tumor of the posterior pituitary or certain small cell
Endocrine disorders carcinomas can also secrete excessive amounts of ACTH
Adrenal cortical insufficiency can occur secondary to which can cause pigmentation of oral tissues. In ACTH
pathologic processes such as neoplasms that may cause secreting tumors (paraneoplastic syndromes) the patient
damage to the adrenal glands. Due to the deficiency of the manifests features of the Cushing’s syndrome. In both these
adrenocortical hormones in the blood there is an increased conditions there occurs diffuse pigmentation of oral tissues
production of ACTH by the anterior pituitary gland. But along with associated systemic features.
ACTH stimulates the production of melanocyte-stimulating
Café au lait pigmentation
hormone which results in diffuse pigmentation of all the
tissues. As a consequence the skin darkens and becomes It manifests as bronze or tan diffuse multifocal macular
bronzed. Also multifocal diffuse pigmentations appear in pigmentations that appear on the skin as well as the oral
the mucous membranes of the oral cavity, conjunctiva, and mucosa. These pale brown macules may vary considerably
genital regions. in size. They have widespread distribution and can occur on
In the oral cavity pigmentation may appear as diffuse the face, neck or the oral cavity. Because of the pale brown
brown patches on the gingiva, buccal mucosa, palate and color these lesions are called as café au lait spots. It is usu-
tongue (Figure 6). This may resemble physiologic pigmen- ally associated with neurofibromatosis (von Recklinghausen’s
tation. Physiologic pigmentation can be differentiated from syndrome), Albright’s syndrome (polyostotic fibrous dys-
pigmentation caused by Addison’s disease as the later plasia) and Peutz–Jeghers syndrome.
develops and progresses during adult life and not present Café au lait pigmentations in neurofibromatosis have
since birth. It is also accompanied by systemic manifesta- smooth borders and are associated with axillary freckling.
tions like weakness, nausea and vomiting, abdominal It is an autosomal dominant inherited disease with multi-
pain, constipation or diarrhea, weight loss and hypotension. ple skin nodules.
Oral pigmentation may be the first sign of Addison’s dis- In Peutz–Jeghers syndrome patients have intestinal poly-
ease. Therefore complete examination must be done for posis along with oral macular pigmentations that appear
patients with diffuse pigmentation of oral cavity and associ- around the mouth (Figure 7) and on the fingers. When
ated with systemic signs and symptoms. Patients presenting such lesions are observed, a detailed history of the patient
with such features should be sent for medical evaluation should be taken about gastrointestinal complaints as well
and laboratory tests to assess levels of ACTH, plasma cor- as the family history for intestinal polyps. These pig-
tisol and serum electrolytes. mented melanotic spots do not require any treatment and
A biopsy of these oral lesions shows acanthosis with silver- are not associated with any risk for malignant transforma-
positive granules in the cells of the stratum germinativum. tion. However, the patient should be monitored for the
66
Figure 8 Figure 9
Post inflammatory pigmentation of tongue with lichen planus.

Figure 10
Diffuse pigmentation of tongue in HIV patient.
development of internal malignancies especially of gas-

trointestinal tract.
Similar pigmented spots are also associated with McCune–
Albright’s syndrome that is characterized by polyostotic
fibrous dysplasia and precocious puberty. Café au lait
spots associated with it have regular borders in contrast to
neurofibromatosis.
HIV infection
In patients infected with human immunodeficiency virus
(HIV), progressive hyperpigmentation of the skin, oral
mucosa, fingernails, and toenails is reported. Such pigmen-
tation is related to primary adrenocortical deficiency and Post inflammatory pigmentation of buccal mucosa following
to antiretroviral therapy like zidovudine (azidothymidine) traumatic ulceration. Courtesy: Dr Ajit Auluck
therapy. Clinically, oral pigmentation appears as irregular
macules with brown or dark brown color. The tongue,
buccal mucosa, and palate are the most commonly affected of oral cavity can be involved but the buccal mucosa and
sites (Figure 8). the attached gingiva are the most frequently involved sites.
Hemochromatosis Post inflammatory pigmentation

Hemochromatosis is also called bronze diabetes. It is a Long-standing inflammatory mucosal diseases like lichen
chronic disease characterized by the deposition of excess planus can cause diffuse pigmentation of the oral cavity
iron (ferritin and hemosiderin) in the body tissues. Deposi- (Figure 9). Most commonly post inflammatory pigmentation
tion of these can result in fibrosis and functional insuffi- is seen more frequently among the dark-skinned individu-
ciency of the involved organs. Hyperpigmentation may als. Clinically, multiple brown-black pigmented areas are
appear both in the skin as well as of the mucous mem- observed adjacent to the reticular or erosive lesions of
branes of oral cavity and conjunctiva. The oral mucosa lichen planus. Post inflammatory pigmentation can also
shows diffuse homogeneous pigmentation which may vary occur following periodontal surgery and biopsy (Figure 10).
from gray-brown to deep brown in color. Although any part Histologically, there is increased production of melanin by
67
Figure 11 Figure 12
Yellowish discoloration of sclera in a patient with jaundice.

Figure 13
Yellowish discoloration of palate in a patient with jaundice.

the melanocytes and accumulation of melanin laden mac-

rophages in the superficial connective tissue.
Cyanosis
Rarely generalized discoloration of oral mucosa can also
be associated with cyanosis in which entire oral mucosa may
become blue black in color when reduced hemoglobin level
reaches above the critical value of 5 g/100 ml.
Beta carotene
Hemangioma of the tongue. Courtesy: Dr Ajit Auluck
Yellowish discoloration of oral mucosa can also be due to
consumption of large amounts of beta carotene in patients
having metabolic disorders that impairs conversion of malformation starts at birth but hemangioma resolves with
beta carotene to vitamin A. age while vascular malformation does not resolve with age.
If hemangiomas are associated with seizures then skull
Liver disease radiographs should be advised which may reveal tram line
Yellowish discoloration of oral mucosa can also be due to calcifications suggestive of Sturge–Weber syndrome.
liver diseases (jaundice) when bilirubin level increases Hemangiomas may appear as flat or slightly raised. The
more than 2–3 mg/dl (Figure 11). To distinguish between color can range from red to bluish purple depending on the
the two conditions we must examine the sclera of the eyes. type of vessels involved (capillaries, veins or arteries) and
In jaundice sclera will also be yellow in color as bilirubin also the depth of the lesion in the tissues (Figure 13). If the
pigments stain the reticular fibers of sclera whereas beta- lesions are superficial to the overlying epithelium they are
carotene will not stain the sclera (Figure 12). reddish blue in color and if they are deep then they are
blue in color.
Hemangiomas can be diagnosed with diascopy test
Focal/Localized Pigmented Lesions which they usually blanch on pressure. Diascopy test is
performed by pressing glass side gently on the lesion. A
Hemangioma and vascular malformations
positive diascopy result is indicated by blanching and sug-
Hemangiomas occur due to proliferation of cells lining the gests that blood is present within vascular spaces and is
blood vessels and are tumor like hamartomas which usually displaced out of the lesion by application of pressure.
occur in children. Vascular malformations occur due to However, lack of blanching does not exclude the possibility
structural defects in the vessels without the proliferation of a vascular lesion as some cutaneous hemangiomas may
of endothelial cells. Both hemangioma as well as vascular not respond positively to diascopy test.
68
Figure 14 Figure 15
Ecchymosis of the palate. Courtesy: Dr Ajit Auluck
Dilated vessels are also seen in hereditary hemorrhagic

telangiectasia (Rendu–Osler–Weber syndrome) which is
Traumatic hematoma of the buccal mucosa. genetically transmitted as an autosomal dominant disease
Courtesy: Dr Ajit Auluck and cause microaneurysms due to weakening of the
adventitial coat of blood vessels.
Rarely, there may be vascular neoplasms that may
appear as pigmented lesions or masses in the oral mucosa.
Lingual varices are usually seen in adults on the ventral
Angiosarcoma is a malignant vascular neoplasm that
surface of the tongue. These are pathologic dilatations of
presents as nodular tumor and arises from the pericytes of
veins and venules. They become more pronounced with age.
the blood vessels.
These are painless and can rupture leading to uncontrolled
Kaposi’s sarcoma is also a tumor of vascular origin
hemorrhage/bleeding. Varices resemble hemangiomas but
mainly seen in HIV patients. Presence of Kaposi’s sarcoma
can be easily distinguished as hemangiomas resolve with age
in HIV patients is diagnostic of AIDS. It is a malignancy
while varices become more pronounced with age. Varix has
which never metastasizes. A human herpesvirus (HHV-8,
finite growth potential while hemangiomas may grow
also called Kaposi’s sarcoma-associated herpesvirus) has
larger in size. Hemangiomas on palpation may have bruit
been implicated in its etiology. Kaposi’s sarcoma is usually
and thrill which is absent in varices.
seen on the palate, gingiva or tongue. Initially lesions will
Hematomas can also cause various types of discoloration
appear as flat or slightly elevated brown to purple lesions
of oral mucosa. Early hematoma is superficial and can cause
while later lesions may become dark red or purple that
bluish swelling of mucosa. They are slightly elevated, often
may ulcerate, bleed or undergo necrosis. Therefore for
fluctuant and rubbery in consistency. Blood cannot be
establishing a definitive diagnosis we must advice a biopsy
evacuated from hematomas with digital pressure. But late
which may show proliferation of spindle-shaped cells sur-
hematomas are blue-black in color as hemoglobin breaks
rounding poorly formed vascular spaces with numerous
down to form hemosiderin (Figure 14). Eruption hematoma
extravasated red blood cells.
is a dome-shaped blue swelling that appears around a devel-
oping tooth. None of these hematomas have pain or any Graphite
other associated complaints.
Petechiae are minute pin point macules that occur due Graphite is commonly introduced into the oral mucosa fol-
to erythrocyte extravasation, lysis of RBCs and subsequent lowing an accidental injury with a graphite pencil. The
breakdown of pigments. When these areas of discoloration lesion appears as an irregular gray to black macule mainly
are larger than 2 cm then they are called ecchymosis (Figure seen in the anterior palatal region of children. A history of
15). Usually petechiae and ecchymosis are seen at the injury confirms the diagnosis.
junction of soft and hard palate. In normal patients we do
Melanotic macule
not see them but they are seen in patients with bleeding
disorders (hemophilia), clotting disorders, vessel wall They appear as an asymptomatic macule on the vermillion
defects, platelet disorders, vomiting, coughing or fellatio. border of the lower lip. However, they can also occur on the
69
Figure 16 Table 4 Warning signs in a mole suggestive of early melanoma
A—Asymmetry: One half of the lesion does not match the other half
B—Border irregularity: The edges are ragged, notched, or blurred
C—Color variegation: Pigmentation is not uniform and may display
shades of tan, brown, or black; white, reddish, or blue discoloration is
of particular concern
D—Diameter: A diameter greater than 6 mm is characteristic, although
some melanomas may have smaller diameters; any growth in a nevus
warrants an evaluation
E—Evolving: Changes in the lesion over time are characteristic; this
factor is critical for nodular or amelanotic (non-pigmented) melanoma,
which may not exhibit the classic criteria above
Histologically, only increased melanin pigmentation is

seen in the basal cell layer without an increase in the number
Melanotic macule of the buccal mucosa.
of melanocytes.
Pigmented nevi
These are relatively uncommon cause of oral mucosal pig-
Figure 17 mentation and may appear as blue or black focal pigmented
areas. Histologically, there is increase in the number of
nevus cells in the epithelium, connective tissue or both.
Accordingly, nevi are classified as junctional nevi, intra-
dermal nevi or intramucosal or compound nevi.
Usually melanocytic nevi develop during childhood.
Most nevi originate from the basal layer of melanocytes and
proliferate in the epithelium along the junction with the
connective tissue and are called as junctional nevi. Later,
these melanocytes drop off into the connective tissue to
form islands or cluster of melanocytes and are called as
compound nevi. Eventually all the cells leave the surface
epithelium and reside in the dermis or submucosa so called
as intradermal or intramucosal nevi.
Blue nevi arise from dermal or mucosal melanocytes that
persist in the connective tissue during embryonic neural
crest migration and therefore do not arise from junctional
Melanotic macules on the gingiva. Courtesy: Dr Ajit Auluck activity at the epithelium and connective tissue interface
(Figure 18). In blue nevi there are spindle-shaped melano-
cytes which synthesize huge amounts of melanin pigment.
gingiva, buccal mucosa and the palate (Figures 16 and 17). Blue nevi can also occur along with ocular pigmentation
The color may be gray, brown, blue, black or a combination along the distribution of trigeminal nerve and is called
of all these. Oral melanotic macules are formed due to focal nevus of Ota (Figure 19).
increase in the melanin production. There is no increase
in the number of melanocytes but there is only increase in Malignant melanoma
the amount of melanin production.
Described in Chapter 13 on page 368.
Oral melanotic macules are usually smaller than 1 cm in
size. Usually they occur as solitary lesions, which are homog-
Clinical evaluation of pigmented lesions
enous in color and have smooth borders. They are benign
in nature and do not transform into malignancy. If there is When a patient presents with a pigmented oral lesion a
a sudden increase in size of the lesion, change in color or detailed medical and dental history must be recorded.
size increases more than 1 cm, a biopsy must be advised to A complete extraoral and intraoral examination should be
exclude possibility of melanoma (Table 4). done and relevant laboratory tests must be advised.
70
carefully recorded as they may help to ascertain the nature

Figure 18
of the lesion.
Usually benign pigmented lesions are small, symmetric,
uniform in color and show regular borders. Occasionally
benign lesions can be slightly elevated. But if the lesions
have irregular borders, color variation or surface ulceration
then it is most likely to be a malignancy and biopsy must
be advised in such cases. Surface characteristics and mor-
phological appearance of the lesions may not point toward
the true nature of the lesion so if there is any pigmented lesion
seen in oral cavity whose etiology cannot be ascertained, we
must advice a biopsy.
Clinical tests such as diascopy or appropriate laboratory
investigations such as blood tests or radiographs may be
advised to confirm a clinical impression and reach a defini-
tive diagnosis.
Flowcharts 1 and 2 summarize the approach to the diag-
Extensive blue nevus of the buccal mucosa. nosis and differential diagnosis of pigmented oral lesions.
PIGMENTATION OF TEETH
Figure 19
Discoloration of the tooth is one of the most frequent rea-
sons why a patient seeks dental care. Tooth discolorations
are usually esthetically displeasing and psychologically
traumatizing. There are many factors that could steal the
sparkle from a smile. An understanding of the etiology
of tooth discoloration is important to a dentist in order to
come to the correct diagnosis, which allows the dental
practitioner to explain to the patient the exact nature of
the condition. In some instances, the mechanism of stain-
ing may have an effect on the outcome of treatment and
influence the treatment options the dentist will be able to
offer to patients.
The causes for tooth discoloration can be classified
according to the location of the stains, either as extrinsic
or intrinsic. Extrinsic discoloration lies on the tooth sur-
face or in the acquired pellicle. The intrinsic discoloration
occurs when the chromogens are deposited within the bulk
of the tooth, which may be of local or systemic origin.
The coronal aspect of the tooth consists of enamel, den-
tin and pulp. Any change to these structures during odon-
Extraoral photograph showing oculodermal
togenesis or post eruption can cause an alteration in the
pigmentation. Courtesy: Dr Ajit Auluck outward appearance of the tooth because of the change in
the light transmitting and reflecting properties.
The history should include details like the onset and

Extrinsic Discoloration
duration of the lesion, the presence of associated skin
lesions, associated systemic signs and symptoms, history of Extrinsic discolorations are defined as discolorations located
intake of medications and habits. During extraoral exami- on the outer surface of the tooth structure and caused by
nation record the pigmented lesions on the face, perioral topical or extrinsic agents. This can be divided into two
skin as well as the lips. A complete intraoral examination groups; direct staining by the compounds incorporated into
of oral cavity should be done. The number, distribution, the pellicle layer and producing the stain as a result of the
size, shape and color of all pigmented lesions should be basic color of the chromogen, and indirect staining were
71
Flowchart 1
Any discoloration of the oral mucosa Heavy metal poisoning
Amalgam/graphite tattoo
Exclude extrinsic causes of pigmentation Drugs like minocycline, oral contraceptives
Stains due to chromogenic bacteria

Observe the lesion and note the
color and other clinical characteristics Tobacco and food stains
Blue red/brown pigmentation Blue/black melanin

due to blood pigments or iron pigmentation
Blanch on Do not blanch Tumor masses Diffuse Focal

pressure on pressure pigmentation pigmentation
• Hemangioma • Petechiae • Neuroectodermal • Physiologic • Melanotic macule

• Varices • Ecchymosis tumor of infancy pigmentation • Nevi
• Early hematoma • Hemochromatosis • Malignant • Endocrine disorders • Amalgam tattoo
• Telangiectasia melanoma • Smoker’s melanosis
• Kaposi’s sarcoma • Drug-induced
pigmentation
• Post inflammatory
pigmentation
• HIV infection
Clinical approach for diagnosis of pigmented lesions in the oral cavity
there is chemical interaction at the tooth surface with Nathoo type 1: The chromogen binds to the tooth sur-
another compound that produces the stain. face. The color of the chromogen is similar to that of den-
Direct staining has multifactorial etiology with chromo- tal stains caused by tea, coffee, bacteria, and metals.
gens derived from dietary sources or habitually placed in the Nathoo type 2: The colored material changes color after
mouth. It is the polyphenolic compounds found in the food binding to the tooth. The stains actually are Nathoo type 1
that are thought to give rise to the color of the stains. food stains that darken with time.
Indirect extrinsic tooth staining is associated with Nathoo type 3: The colorless material or prechromogen
cationic antiseptics and metal salts. The agent is without binds to the tooth and undergoes a chemical reaction to
color or a different color from the stain produced on the cause a stain. These stains are caused by carbohydrate-rich
tooth surface. foods, stannous fluoride, and chlorhexidine.
Traditionally, extrinsic tooth discoloration has been
classified according to its origin, whether metallic or Factors responsible for extrinsic discoloration
non-metallic.
Table 5 summarizes the extrinsic causes of tooth Diet factors Deposition of tannins found in tea, coffee,
discoloration. and other beverages cause brown stains on the surface
of the teeth. Commercially available soft drinks and food
products containing permitted synthetic food colors (red
color: Ponceau 4R, carmoisine, erythrosine, yellow color:
Classification of Extrinsic Stains
tartrazine pyrazolone, sunset yellow FCF, blue color:
Nathoo (1997) proposed the Nathoo classification system indigo carmine, brilliant blue FCF, green color: fast green
of extrinsic dental stains. According to this classification FCF) and additives can temporarily cause discoloration of
system three categories of extrinsic stains are described. the teeth and the oral mucosa (Figure 20).
72
Flowchart 2
Bluish/black discoloration of oral mucosa
Usually due to metals/melanin pigment
Localized pigmentation Diffuse pigmentation
Exclude pigmentation due to metals

Physiological pigmentation
like amalgam, graphite, lead etc.
Present since birth
In oral cavity, usually amalgam tattoos
are seen in close vicinity of restorations
Smoker’s melanosis
Melanotic macule History of smoking
Small size, mostly on lips, increase

in melanin synthesis Endocrine disorders like Addison’s
disease/Cushing’s syndrome
Nevi Look for systemic signs and symptoms
Increased proliferation of
melanocytes, usually from birth HIV associated melanosis
Advice ELISA for HIV

Malignant melanoma
Associated with syndromes like
Dark, irregular borders, asymmetric
Albright’s syndrome, Peutz–Jeghers
and rapid growth
syndrome
Post inflammatory pigmentation due to General examination to find

healing of lesions like lichen planus other associated features
Algorithm for differential diagnosis of pigmented lesions in the mouth with characteristic features which help in diagnosis
*Biopsy must be advised if there is increase in size, change in color or any proliferative changes associated in
pigmented lesions
Oral hygiene related factors Accumulations of dental Chewing of pan (a combination of betel nut of the areca
plaque, calculus and food particles cause brown or black palm, betel leaf, and lime) results in the production of red
stains. Chromogenic bacteria have been suggested as an colored substance that causes red-black stain on the teeth,
etiologic factor in the production of stains typically at the gingiva, and oral mucosal surfaces (Figure 24).
gingival margin of the tooth (Figure 21). The most com- Drug related Cationic antiseptics such as chlorhexidine,
mon is a black stain caused by Actinomyces species. The cetylpyridinium chloride and other mouth washes (the
stain is composed of ferric sulfide and is formed by the essential oil/phenolic mouthrinse ‘Listerine’, delmopinol
reaction between hydrogen sulfide produced by bacterial mouthrinses) can cause staining after prolonged use.
action and iron in the saliva and gingival exudates (Figure Chlorhexidine, for example, produces brown to black dis-
22). Green stains are attributed to fluorescent bacteria and coloration (Figure 25). Most evidence indicates that the
fungi such as Penicillium and Aspergillus species. Orange likely cause of staining is the precipitation of anionic
stain is less common than green or brown stains and is dietary chromogens onto the adsorbed cations.
caused by chromogenic bacteria such as Serratia marce- Some systemic medications (e.g. minocycline, doxycy-
scens and Flavobacterium lutescens. cline, co-amoxiclav, linezolid) have also shown to cause
Habit related Tobacco from cigarettes, cigars, pipes, and extrinsic staining.
chewing tobacco causes tenacious dark brown and black Metallic compounds are also implicated in dental dis-
stains that cover the cervical one-third to midway on the colorations because of the interaction of the metals with
tooth toward the gingival margin (Figure 23). dental plaque to produce surface stains. Iron-containing
73
Table 5 Extrinsic causes for tooth discoloration
Classification Factors responsible Examples Color

Non-metallic stains Diet Tea, coffee and other foods Brown to black
Direct stains Oral hygiene Dental plaque, calculus and food particles Yellow/brown
Chromogenic bacteria Brown/black/green, orange
Habits Tobacco smoking/chewing Dark brown/black
Pan chewing Red-black
Non-metallic stains Medications Cationic antiseptics (e.g. chlorhexidine) Yellow brown
Indirect stains Essential oils/phenolic mouthrinse Yellow
Systemic antibiotics (e.g. minocycline) Green-gray
Metallic stains Medications Iron containing oral solutions Black
Indirect stains Copper salts in mouthrinse Green
Potassium permanganate in mouthrinse Violet to black
Stannous fluoride Golden brown
Silver nitrate Gray
Occupation Exposure to iron, manganese, silver Black
Exposure to mercury and lead dust Blue green
Copper and nickel Green
Chromic acid fumes Deep orange
Figure 20 Figure 21
Accumulation of calculus and food particles at the cervical

margin of the tooth causing yellowish brown stains.
MCODS, Mangalore
oral solutions used for treatment of iron deficiency anemia

cause black stains (Figure 26). Green discoloration can
result from the use of mouthrinses containing copper salts.
Potassium permanganate mouthwash (violet-black stain),
silver nitrate (black stain), and stannous fluoride (brown
stain) also can induce dental discolorations.
Ice cream candy color staining of the oral mucosa. Occupation related Industrial exposure to iron, manga-
Courtesy: Dr Prem Prakash Kar, Dr Snehal Thatte,
nese, and silver may stain the teeth black. Mercury and
Dr Shomshukla Bhowmick
lead dust can cause a blue-green stain; copper and nickel,
74
Figure 22 Figure 24
Black stains caused by chromogenic bacteria.

MCODS, Mangalore
Brown and black staining of teeth and orange-red staining of

the buccal mucosa in a patient with a habit of chewing ‘pan’.
Figure 23 Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
Figure 25
Tenacious brown stains on the cervical margin of the teeth in

a tobacco chewer. Courtesy: Department of Oral Medicine
Extrinsic stains due to prolonged use of chlorhexidine
mouthrinse
green-to-blue-green stain and chromic acid fumes may

cause deep orange stain.
Since saliva plays a major role in the physical removal
Predisposing factors
of food debris and dental plaque from the outer and inter-
Factors that predispose children and adults to extrinsic proximal tooth surfaces, diminished salivary secretion can
stains include enamel defects, salivary dysfunction, and lead to extrinsic discoloration. Decreased output may be
poor oral hygiene. caused by local disease (e.g. salivary obstructions and
Microscopic pits, fissures, and defects on the outer sur- infections), systemic disease (e.g. Sjögren syndrome), head
face of the enamel are susceptible to the accumulation of and neck radiation therapy for cancer, chemotherapy, and
stain-producing food, beverages, tobacco, and other topical multiple medications (e.g. anticholinergics, antihyperten-
agents. sives, antipsychotics, antihistamines).
75
Intrinsic Discoloration incorporation of bilirubin into developing teeth, produc-

ing jaundice like yellow-green tint within the dental hard
There are several causes of intrinsic tooth discolorations tissue known as chlorodontia. These diseases include sickle
which have either an endogenous or exogenous origin. cell anemia; thalassemia; hemolytic disease of the new-
These changes may occur during or after odontogenesis. born due to Rhesus factor, ABO, or other erythrocyte anti-
During odontogenesis, teeth may become discolored from gen incompatibility (erythroblastosis fetalis/icterus gravis
the changes in the quality or quantity of enamel or dentin, neonatorum); biliary atresia; bile duct obstruction; biliary
or from the incorporation of discoloring agent into the hypoplasia; and cholestasis associated with sepsis.
hard tissues. Post-eruption discolorations occur when the Congenital erythropoietic porphyria (Günther’s disease) is
discoloring agent enter the hard tissues. They may origi- a rare, autosomal recessive disorder of porphyrin metabolism,
nate from the pulp cavity or the tooth space. resulting in an increase in the formation and excretion or
Pre-eruptive causes of intrinsic porphyrins. The porphyrin pigments have an affinity for cal-
discoloration (Table 6) cium phosphate and are incorporated into teeth during den-
tal formation and this causes a characteristic reddish-brown
Metabolic causes The diseases that have the potential discoloration of the teeth, called erythrodontia. The affected
to cause neonatal hyperbilirubinemia may cause the tooth shows a red fluorescence under ultraviolet light.
Alkaptonuria, also known as phenylketonuria or ochro-
Figure 26 nosis is an inborn error of metabolism of tyrosin and phe-
nylalanine causing a build-up of homogentisic acid. This
results in a brown discoloration of the permanent dentition.
Disturbance during development of a tooth Enamel hypo-
plasia may result due to the disturbance of the developing
tooth germ following trauma, infection or nutritional defi-
ciency giving rise to localized or generalized enamel defects
(Figure 27).
Periapical odontogenic infections of the primary teeth
can disrupt normal amelogenesis of the underlying perma-
nent successors and can cause localized enamel hypopla-
sia. Trauma to developing, yet unerupted, teeth can also disturb
Black colored extrinsic stains due to use of iron tonic. amelogenesis and may result in enamel hypoplasia, which
is visualized as a localized opacity on the erupted tooth.
MCODS, Mangalore
Such teeth commonly are referred to as Turner’s teeth.
Table 6 Intrinsic causes for tooth discoloration (pre-eruptive)

Pre-eruptive Metabolic disorders Hyperbilirubinemia Yellow-green
Porphyria Reddish brown
Alkaptonuria Brown
Disturbance of tooth germ Localized
Turner tooth
Generalized White to
Infection (maternal or childhood) yellow to
Nutritional deficiency brownish
Molar incisor hypomineralization (MIH)
Genetic disorder Amelogenesis imperfecta Yellow brown
Dentinogenesis imperfecta Blue brown
Dentin dysplasia Yellow
Systemic syndrome Yellow
e.g. epidermolysis bullosa
Medication Tetracycline Yellow, brown, blue or greenish
Minocycline Blue-green
Ciprofloxacin Greenish
Fluoride Chalky white to brown/black
76
Figure 27 Figure 28
Generalized yellowish discoloration of teeth in amelogenesis

imperfecta. Courtesy: Department of
Generalized chalky white opacifications of the teeth in enamel Oral Medicine and Radiology, MCODS, Mangalore
hypoplasia. Courtesy: Department of Oral Medicine and
borders with normal enamel as opposed to smooth borders

Crown formation begins in utero; therefore, the poten-
with hypoplastic lesions. The suggestions as to the possi-
tial for extensive intrinsic discoloration of the primary
ble etiologies, including environmental changes during
dentition may be present throughout pregnancy. Although
the limited time period, infections during the early child-
rare, maternal rubella or cytomegalovirus infection, mater-
hood, dioxin in breast milk and genetic factors, have yet
nal vitamin D deficiency, drug intake during pregnancy
to be eliminated from the possible causes.
and toxemia of pregnancy can lead to tooth discoloration,
which generally manifests as a focal opaque band of
Genetic defects and hereditary diseases
enamel hypoplasia. Such defects will be chronologically
laid down in the teeth depending on the state of develop- Genetic defects in enamel or dentin formation include
ment at the time of interference and the effect is directly amelogenesis imperfecta (AI), dentinogenesis imperfecta
related to the degree of systemic upset. There may be pit- (DI), and dentinal dysplasia (DD). These hereditary diseases
ting or grooving which predisposes to extrinsic staining of can be associated with intrinsic tooth discoloration.
the enamel in the region of tooth disturbed, often then Amelogenesis imperfecta is a hereditary disorder which
becoming internalized. affects the enamel formation in both primary and permanent
Crown formation of the permanent dentition occurs until dentition. Type I is hypoplastic AI. The teeth typically
the child is aged approximately 8 years. Systemic postnatal have an abnormally thin enamel layer that reveals the yel-
infections (e.g. measles, chicken pox, streptococcal infections, low color of dentin beneath the enamel (Figure 28).
scarlet fever) can also cause enamel hypoplasia. The band Hypoplastic teeth with rough or pitted enamel surfaces are
like discolorations on the tooth are visualized where the also at a greater risk for extrinsic staining. Type II is hypo-
enamel layer has variable thickness and becomes extrinsi- calcified AI. The enamel in the hypocalcified type is yellow
cally stained after tooth eruption. to orange, soft, and lost soon after eruption. Therefore,
Vitamins C and D, calcium, and phosphate are required for hypocalcified teeth develop dark stains and are at high
healthy tooth formation. Deficiencies can result in exposure- risk for dental caries. Type III is hypomaturation AI. Teeth
related or dose-related enamel hypoplasia. with hypomaturation have soft enamel with a mottled
Molar-incisor hypomineralization (MIH) is an idio- opaque white, yellow, or brown discoloration. Type IV AI
pathic condition characterized by severe hypomineralized involves hypomaturation or hypoplastic dentition with
enamel affecting incisors and permanent first molars. taurodontism.
The enamel defects can vary from white to yellow to Dentinogenesis imperfecta, an inherited disorder, is
brownish areas but they always show a sharp demarcation classified into three types. DI type I is associated with
between sound and affected enamel. The nature of the osteogenesis imperfecta and is characterized by opalescent
enamel is porous and brittle, breaking down shortly after primary teeth. DI type II (hereditary opalescent dentin)
eruption under masticatory forces, often resembling affects both the dentition. The pulp chambers often become
enamel hypoplasia, but distinguished by having irregular obliterated and the dentin undergoes rapid wear once the
77
enamel has chipped away. The teeth have a typical amber-

Figure 29
like translucency or opalescence against reflected light,
and there color varies from different shades of yellow to
bluish-brown. DI type III (brandywine isolate hereditary
opalescent dentin) is a very rare autosomal dominant dis-
order which occurs in a racial segregate in Maryland,
United States. It is similar in appearance to type I and II
but with radiographic appearance of shell teeth with mul-
tiple pulpal exposure in the primary dentition.
Dentin dysplasia occurs in two types. In type I DD the
primary and permanent dentition are of normal shape and
form but may have an amber translucency. Teeth with
type II DD are characterized by thistle shaped pulp cham-
ber and pulp stones with brown tooth discoloration.
Defects in enamel formation may also occur in a num-
ber of systemically involved clinical syndromes such as
vitamin D dependent rickets, epidermolysis bullosa, Ehlers– Yellowish discoloration of teeth due to use of tetracycline
Danlos syndrome and pseudohypoparathyroidism. Patients
with epidermolysis bullosa may have enamel hypoplasia
and pitting, which produce a yellowish tint and the
patients are at risk for caries.
Figure 30
Medications
Tetracycline, a broad spectrum antibiotic, is known to
cause intrinsic discoloration when prescribed during tooth
development. Tetracycline staining results from systemic
administration of the drug, which chelates with the cal-
cium ions on the surface of the hydroxyapatite crystals as
a stable orthophosphate complex. Dentin has been shown
to be more heavily stained than enamel. The severity of
the discoloration produced depends on the type of tetracy-
cline used, the dosage and the period of time it was taken
for, as well as the age at the time of administration.
Tetracycline should be avoided from 29 weeks in utero
until full term, in breast feeding mothers and in children Bluish discoloration of teeth due to minocycline.
up to the age of 12 years to avoid discoloration of the Courtesy: Department of Oral Medicine and Radiology,
developing teeth. It has been shown that the discoloration MCODS, Mangalore
occurs with the greatest frequency in the developing den-
tition when total administration is over 3 g, or the treat-
ment exceeds 10 days. The various analogs of tetracycline
produce different color changes, for instance, chlortetra- Degree I: There is minimal expression of tetracycline
cycline produces a slate gray color and oxytetracycline stain uniformly confined to the incisal three-quarters of
causes a creamy discoloration. the crown and is light yellow in color.
Teeth affected by tetracycline have a yellowish or brown- Degree II: There is more variability of staining ranging
gray appearance which is worse on eruption and dimin- from a highly uniform deep yellow to gray banded discol-
ishes with time (Figure 29). The affected teeth also fluoresce oration in which a distinctive difference in discoloration is
under ultraviolet light, giving off a bright yellow color. noted between the cervical region and the incisal four-
Exposure to the sunlight can change the color to brown; the fifths of the crown.
anterior teeth are particularly susceptible to this photo- Degree III: There is very dark blue or gray uniform
oxidation induced color change. In severe cases of tetracy- discoloration.
cline involvement enamel hypoplasia may result. Minocycline is a semi-synthetic derivative of tetracycline.
Tetracycline discoloration has been classified according It is commonly used to treat acne vulgaris. The ingestion of
to the extent, degree and the location of the tetracycline minocycline can lead to a green-gray or blue-gray intrinsic
involvement (Jordan and Boksman). staining of teeth (Figure 30). Unlike with other tetracyclines,
78
staining occurs during and after the complete formation

Figure 31
and eruption of teeth. Staining of the adult dentition appears
to occur in 3–6% of patients taking long-term minocycline
at more than 100 mg daily. The staining caused by mino-
cycline is different from that caused by tetracycline. The
onset of discoloration can occur any time from 1 month to
many years after the initiation of the treatment.
Four theories have been put forward to explain the
possible mechanism by which minocycline causes tooth
discoloration. The first is the extrinsic theory, where it is
thought that minocycline attaches to the glycoprotein in
acquired pellicle. It oxidizes on exposure to air or as a result
of bacterial activity, and so causes degradation of the aro-
matic ring forming insoluble black complex. The pigmen-
tation may be incorporated into the dentin and is possibly
Yellowish brown intrinsic staining of teeth in fluorosis.
a demineralization/remineralization phenomenon related Courtesy: Department of Oral Medicine and Radiology,
to the high local levels of the drug. The second is the MCODS, Mangalore
intrinsic theory, where the minocycline bound to the
plasma proteins is deposited in collagen-rich tissues, such
as the teeth. This then oxidizes slowly over time with expo-
sure to light. The third possibility is that the drug chelates
with iron to form an insoluble complex. The fourth sug- Post-eruptive Causes (Table 7)
gestion is that minocycline could be deposited in dentin
Dental conditions and caries
during dentinogenesis, and the process of secondary den-
tinogenesis can be accelerated in bruxists. Tooth wear is the progressive loss of enamel and dentin
Doxycycline has recently been reported to cause extrin- due to attrition, abrasion and erosion. As the enamel thins
sic staining of teeth, possibly by binding to glycoproteins the teeth become darker as the color of the dentin becomes
in the dental pellicle in patients with poor oral hygiene in more apparent. Once the dentin is exposed the potential of
whom oxidation occurs (e.g. sunlight exposure, bacterial) chromogens to enter the body of the tooth increases.
or via mechanisms similar to those for minocycline. The various stages of carious process can be recognized
Ciprofloxacin, a quinolone given intravenously to infants by the change in color as the disease progresses. The
at dosages of 10 to 40 mg/kg/day to treat infections with pathogenesis of dental caries begins with an incipient
Klebsiella, has been associated with greenish discoloration lesion confined to the enamel layer. Incipient carious
of the teeth when they erupted. lesions are associated with plaque accumulation and man-
ifest as chalky white areas of discoloration secondary to
hypocalcification. As caries progresses into the dentin, the
DENTAL FLUOROSIS overlying translucent enamel reveals the color of the
underlying caries and appears yellowish brown. Extensive
Dental fluorosis is characterized by enamel discoloration caries that involve destruction of both enamel and dentin
resulting from subsurface hypomineralization due to the produce a color that ranges from light brown, to dark
excessive ingestion of fluoride during the early maturation brown or almost black (Figure 32). The brown color is
stage of enamel formation. Fluoride sources are numerous attributed to the formation of Maillard pigments (reaction
and include naturally or artificially fluoridated drinking between proteins and small aldehydes produced by cario-
water, commercially available beverages, foods prepared genic bacteria), melanins, lipofuscins, and uptake of vari-
in fluoridated water, chewable vitamins, oral healthcare ous food colors and bacterial pigments. In some patients,
products (e.g. toothpastes, mouthrinses, oral fluoride sup- the caries process can self-arrest, and remineralization
plements), and professional fluoride products prescribed may occur; however, the brown discolorations usually
by dentists. The severity is related to age and dose, with remain.
the primary and permanent dentitions both being affected The natural darkening and the yellowing of the teeth and
by endemic fluorosis. The enamel is often affected and may the change in their light transmission properties with age
vary from areas of flecking to diffuse opaque mottling are due to the combination of the factors involving both
superimposed on to chalky white or dark brown/black enamel and dentin. The enamel undergoes both thinning
appearance (Figure 31). The dark discoloration thought to and textural change, while the deposition of secondary
be post-eruptive by a process of internalization of the and tertiary dentin and pulp stones all contribute to the
extrinsic stains into the porous enamel. darkening process of aging.
79
Table 7 Intrinsic causes for tooth discoloration (post-eruptive)

Post-eruptive Dental conditions Dental caries
• Incipient Chalky white
• Active Yellowish brown
• Arrested Dark brown to black
Tooth wear Yellowish
Aging Yellowish
Pulpal causes Pulpal trauma with hemorrhage Gray-brown
Calcific metamorphosis Yellowish to yellowish brown
Internal resorption Pinkish
Dental materials Amalgam Blue-gray
Composite glass ionomer cement (GIC) Yellowish brown
Intracanal medicaments (e.g. iodoform, ledermix) Brownish gray
Obturating materials and sealers Grayish
Figure 32 Figure 33
Discolored upper central incisors following trauma.

Grayish black discoloration in the proximal aspect of the Courtesy: Department of Oral Medicine and Radiology,
tooth indicative of dental caries. Courtesy: Department of MCODS, Mangalore
non-infected traumatized teeth is the accumulation of the

Pulpal causes
hemoglobin molecule or other hematin molecules. In the
Bacterial, mechanical, or chemical irritation to the pulp may absence of infection, the release of iron from the protopor-
result in tissue necrosis and the release of disintegration phyrin ring is unlikely.
by-products that might penetrate the tubules and discolor Excessive formation of irregular dentin in the pulp
the surrounding dentin. Trauma that occurs to erupted teeth chamber and along the canal walls may occur following
also causes discoloration. After acute trauma, intrapulpal hem- certain traumatic injuries. This is known as calcific meta-
orrhage will give the tooth a reddish tinge. Occasionally, in morphosis. As a result of this, the translucency of the
younger patients, the color may return to normal as the crown gradually decreases, giving rise to yellowish or yel-
inflammation subsides. More often, the discoloration changes lowish brown discoloration (Figure 33).
to gray-brown in a matter of days as the pulp becomes Root resorption following trauma often presents as a
necrotic. Hemolysis of the red blood cells would follow and pink spot lesion at the cementoenamel junction in an
release the heme group to combine with the putrefying otherwise symptomless tooth, known as the ‘pink tooth of
pulpal tissue to form black iron sulfide. In vitro studies Mummery’. The resorption may be internal, being of pulpal
have recently shown that the major cause of discoloration of origin or external of periodontal origin.
80
Dental Materials Clinical examination

Dental restorations most commonly cause intrinsic dis- The scratch test is usually used to distinguish between
coloration. Amalgam restorations can generate corrosion extrinsic and intrinsic discoloration. Discolored tooth
products, leaving a blue-gray color in the tooth, especially surfaces are scratched with care by using a dental explorer,
in large cavity preparations with undermined enamel scaler, or similar sharp instrument to assess surface tex-
known as amalgam blue. ture. Light scratching with a dental instrument removes
Metal pins and prefabricated posts are sometimes used weakly adherent plaque that causes extrinsic discolor-
to reinforce composite restoration on the anterior dentition. ation. If the discoloration requires removal with a sharp
Discoloration from inappropriately placed pins and posts dental scaler, the discoloration is considered to be tena-
is caused by the metal seen through the composite or the cious. Intrinsic discoloration cannot be removed by using
tooth structure. the scratch test.
Microleakage around composite or glass ionomer resto- Extrinsic staining of a single tooth is unusual. The dis-
ration causes staining. Open margins may allow chemicals tribution is usually generalized. The stains are usually found
to enter between the restoration and the tooth structure on surfaces with poor toothbrush accessibility. Whereas in
and discolor the underlying dentin. case of intrinsic discoloration distribution is either gener-
Several intracanal medicaments are liable to cause alized to all teeth or localized to certain teeth or tooth
internal staining of the dentin. Phenols or iodoform based surfaces. An intrinsic etiology usually exists when a single
medicaments sealed in the root canal and the chamber tooth is discolored.
are in direct contact with dentin, allowing penetration and Regarding other physical findings, teeth with extrinsic
oxidation. These compounds discolor dentin gradually. tooth discoloration usually demonstrate no signs of pulpal
Tetracyclines (e.g. ledermix-triamcinolone acetonide and disease. Teeth with intrinsic discoloration may demonstrate
demethylchlortetracycline) used within the tooth for end- signs of pulpal disease. A single discolored tooth with a
odontic therapy may also cause dark gray-brown dis- history of trauma will usually be non-vital. Radiographs
coloration. may reveal periapical pathology.
Obturating materials are frequent cause for single tooth Under ultraviolet light, teeth with tetracycline staining
discoloration. Incomplete removal of obturating material and congenital porphyria may fluoresce yellow or red,
and sealer remnants in the pulp chamber, mainly those respectively.
containing metallic components, often result in dark
discoloration. Management
The treatment of tooth discoloration consists of identify-
Diagnosis ing the etiology and implementing the required therapy.
History The patient’s history of tooth discoloration pro- Scaling and polishing of the teeth remove many extrinsic
vides useful information regarding the etiology. The his- stains. For more stubborn extrinsic discoloration and
tory includes the following: intrinsic stain, various bleaching techniques may be
attempted. Tooth bleaching can be performed externally,
❍ Dental history: Previous dental treatment, oral hygiene termed night guard bleaching or vital tooth bleaching, or
practices, use of mouthwash, amount and scheduling intracoronally in root-filled teeth, called non-vital tooth
of fluoride intake, history of dental trauma. bleaching.
❍ Medical history: History of maternal or childhood dis- Teeth discolored by dental caries or dental materials
eases, use of medications. require the removal of the caries or restorative materials,
❍ Family history: Genetic disorders. followed by proper restoration of the tooth. Partial (e.g.
❍ Diet history: Nutritional deficiencies, diet that can laminate veneers) or full-coverage dental restorations may
cause staining of the teeth. be used to treat generalized intrinsic tooth discoloration in
❍ Social history and personal history: Occupational which bleaching is not indicated or in which the esthetic
exposure to metals and use of tobacco. results of bleaching fail to meet the patient’s expectations.
81
CHAPTER
Bacterial, Viral and Fungal
4 Infections
Ravikiran Ongole, Praveen BN
Bacterial Infections Viral Infections

➧ Scarlet Fever ➧ Infectious Mononucleosis
➧ Diphtheria ➧ Acute Lymphonodular Pharyngitis
➧ Tularemia ➧ Measles (Rubeola)
➧ Erysipelas ➧ German Measles (Rubella)
➧ Impetigo ➧ HIV and AIDS
➧ Melioidosis ➧ Sinusitis
➧ Tetanus Fungal Infections and Protozoal Diseases
➧ Actinomycosis ➧ Histoplasmosis
➧ Noma ➧ Blastomycosis
➧ Botryomycosis ➧ Mucormycosis
➧ Rhinoscleroma ➧ Aspergillosis
➧ Cat-scratch Disease ➧ Cryptococcosis
Many of the systemic diseases are caused by a wide range of within the first 2 days of the infection. The fever and rash
bacteria, viruses and fungal organisms. Some of these micro- usually resolve in about 7 days. Other associated clinical
bial diseases can exhibit oral manifestations. However, some features include headache, tonsillitis, pharyngitis and
of the microbial diseases such as dental caries and periodon- lymphadenopathy.
tal diseases are localized to the oral cavity. Systemic micro- The scarlet fever rash appears bright red and mimics a
bial diseases with oral manifestations are discussed in this sunburn appearance. Normal pin head-sized areas of the skin
chapter. may project through the rash giving rise to a sandpaper
like surface texture, which is popularly referred to as ‘sand-
paper rash’ or ‘sunburn with goose bumps or goose pimples’.
BACTERIAL INFECTIONS The skin rashes are commonly seen over the trunk,
extremities, neck, groin and specifically along Pastia’s lines
SCARLET FEVER (darkening of the normal skin fold/creases, such as the
axillary crease, anticubital crease).
Scarlet fever is also known by the names scarlatina, scarla- Once the rash resolves, a 3–8 months phase of desqua-
tinella and scarlatiniform rash. It is caused by an infection mation begins, which is characterized by peeling away of
with a pyogenic exotoxin-producing group A -hemolytic the skin in large flakes.
streptococci. The upper respiratory tract is the usual portal
of entry. Most cases occur between 1 and 10 years of age Oral manifestations
and may occasionally be seen in adults. Patients may present with circumoral pallor. The soft pal-
The organism expresses an erythrogenic toxin that acts ate, pharynx, tonsillar region and the tongue are commonly
on the blood vessels to produce the typical skin rash. affected. The oral cavity appears extensively erythematous
and edematous (stomatitis scarlatina). Occasionally a yel-
Clinical features
lowish-white exudate may be seen in the tonsillar crypts.
The incubation period of the bacteria ranges from 1 day During the first 2 days of the infection the dorsal surface
up to a week. Patients will present with fever and skin rashes of the tongue exhibits a white coat through which the
82
Chapter 4 – Bacterial, Viral and Fungal Infections
fungiform papillae are visible. Such an appearance is dead cells, leukocytes and bacteria) initially forms on the
referred to as ‘white strawberry tongue’. After about 4 days tonsils and subsequently spreads to involve the larynx,
the white coat on the tongue desquamates to reveal an pharynx, uvula, soft palate and occasionally the gingiva.
erythematous dorsal surface with hyperplastic fungiform Removal of this mildly adherent membrane leaves a raw
papillae, which is referred to as ‘red strawberry tongue’. bleeding surface. Some patients may exhibit transient
The complications of untreated scarlet fever may occur paralysis of the soft palate.
either due to streptococcal toxin (myocarditis), or bacterial Extensive involvement of the respiratory tract may lead
invasion (septic arthritis, meningitis, osteomyelitis) or by to respiratory obstruction. The other serious complications
an allergic reaction (rheumatic fever, glomerulonephritis). include effects of the toxin on the cardiovascular system,
The diagnosis can be made based on the characteristic nervous system and renal system to cause myocarditis,
clinical presentations, culture of the secretions from the polyneuritis and acute interstitial nephritis.
pharynx or tonsillar regions and detection of antigens spe-
cific for group A -hemolytic streptococci. Prevention and management
Management Immunization of the infants with DPT vaccine (weakened

form of diphtheria toxin) along with booster doses every
A single episode of scarlet fever will usually confer perma- 10 years, throughout life will effectively prevent the occur-
nent antitoxin immunity. However, recurrences are not rence of diphtheria.
unusual. This is due to the fact that toxin produced by other Diphtheria is treated with antitoxin along with antibi-
strains is not neutralized and the bacterial immunity is otics such as intravenous penicillin or erythromycin.
temporary.
Penicillin is the drug of choice. Erythromycin may be used
in patients who are allergic to penicillin. Acetaminophen TULAREMIA (Rabbit Fever, Deer-fly Fever,
or ibuprofen may be used to alleviate pain and manage
Francis’ Disease, Tick-Borne Disease,
fever. Analgesic mouthrinses (benzydamine hydrochlo-
ride) may be used for stomatitis.
Ohara’s Disease)
Tularemia is caused by gram-negative pleomorphic bacte-

rium, Francisella tularensis (named after the extensive
DIPHTHERIA work on tularemia done by Dr Edward Francis).
It is transmitted to men from animals (chiefly rabbits
Diphtheria is an acute infectious disease caused by toxin- and also by muskrats and squirrels) by contact with dis-
producing Corynebacterium diphtheriae or Klebs-Löffler eased or dead animals, by the bites of deer flies, fleas, and
bacillus (after Klebs who discovered the bacillus and Löffler ticks; by contact with contaminated animals or their prod-
who isolated the bacillus in pure culture). ucts; by ingestion of contaminated food or water or by
The bacteria reside in the upper respiratory tract of the inhalation of aerosolized bacteria. Young and middle-aged
infected individual and cause local infection of the upper individuals who are actively involved in outdoor activities
respiratory tract and occasionally the skin and the heart, are more susceptible to the disease.
kidneys, and peripheral nerves.
It spreads through droplet infection and direct contact. Clinical manifestations
The incubation period lasts for a few days, following
which the bacterium expresses an exotoxin that causes The incubation period of tularemia usually varies from 3 to
tissue necrosis that subsequently spreads peripherally. 4 days. The initial symptoms include fever, chills, myalgias
and malaise. Disseminated form of the disease can cause
General clinical and oral manifestations tularemic meningitis, pericarditis, peritonitis, endocarditis
and osteomyelitis. Occasionally a severe form of tulare-
Tonsillitis is usually the first clinical finding. Patients
mia, typhoidal tularemia may be seen.
present with fever, malaise, headache, sore throat, foul
Based on the type of the tularemia, specific clinical man-
taste/breath and cervical lymphadenopathy. The exotoxin
ifestations are seen (Table 1).
causes necrosis of the soft tissues producing a thick, gray-
ish white membrane. As the infection progresses patients
Oral manifestations
may complain of difficulty in speech, swallowing and
breathing. In the initial stages solitary nodular masses may be appre-
Diphtheria involving the skin causes excoriation of the ciated which may eventually form abscesses or ulcerate.
skin of the nasal and perinasal regions. Generalized stomatitis along with extremely painful necrotic
The grayish pseudo membrane (diphtheritic membrane— ulcers may be seen affecting any part of the oropharynx.
covers necrotic ulcerated areas of the mucosa and contains Regional lymphadenopathy is usually a prominent feature.
83
Table 1 Clinical manifestations and types of tularemia
Type of tularemia Mode of transmission Clinical features

Ulceroglandular tularemia Bite of infected insects and animals (ticks, rabbits) Ulcers at the site of inoculation (fingers, hands, feet)
Inflammation of the regional glands. Tender nodes
Glandular tularemia Similar features as that of the ulceroglandular variety except
that there is no evidence of ulcer
Pneumonic tularemia Inhaling of airborne bacteria from soil or inhalation Pneumonia is the characteristic feature. Other symptoms
of the bacteria by healthcare workers include dry cough, dyspnea, and pleuritic chest pain
Oropharyngeal tularemia Eating undercooked meat of an infected animal or Vomiting, diarrhea and other digestive problems
drinking contaminated water
Oculo-glandular form Occurs following exposure of the conjunctiva to The affected eye is tender and erythematous. Occasionally
infected blood purulent exudate may be seen along with inflamed regional
glands
Management IMPETIGO
Tularemia responds well to antibiotics. The drugs of choice
include aminoglycosides (gentamicin, streptomycin) fluoro- Impetigo is a highly contagious superficial skin infection
quinolones (ciprofloxacin, gatifloxacin) and chloramphenicol. caused by -hemolytic streptococci and Staphylococcus
aureus. Impetigo is considered as the most common bacterial
dermal infection in children. It is more common in chil-
ERYSIPELAS dren receiving dialysis.
Erysipelas is an acute inflammation of the skin, with marked Clinical features

involvement of cutaneous lymphatic vessels. It is usually Impetigo usually affects children in the age group of 2–6
caused by -hemolytic Streptococcus pyogenes of group A. years. It spreads via direct skin contact. The incidence of
Erysipelas has to a lesser extent been caused by group B, C, or impetigo is greatest in the summer months, and the infec-
G streptococci, and occasionally by Staphylococcus aureus. tion most often occurs in areas with poor hygiene and in
It has also been referred to as St. Anthony’s fire after crowded living conditions.
the name of the Egyptian monk who is believed to have had There are two types of impetigo: non-bullous (impetigo
the powers to cure this disease. contagiosa) and bullous.
The host response to the infection results in the non-
Clinical features bullous type of impetigo. On the other hand, the bullous
The typical lesion of erysipelas is evident as an erythema- form is not dependent on the host response but results
tous, well defined area that may be warm to touch. Occa- from the direct action of the staphylococcal toxin.
sionally a butterfly rash mimicking lupus erythematosus Non-bullous impetigo The skin of the face and the
may be seen when the bridge of the nose is involved. Fever hands and legs are commonly affected sites. The infection
of sudden onset is a typical feature. Erysipelas is usually begins as a solitary red macule or papule that almost
seen in young or old patients and in systemically compro- immediately turns into a vesicle. The vesicle ruptures to
mised patients. form an erosion, and the contents dry to form characteris-
The typical rash of erysipelas can affect skin on any tic honey-colored crusts that may be pruritic. These lesions
part of the body. However previous sites of trauma are the may spread to surrounding areas by autoinoculation.
most commonly affected. The common sites affected
include the legs, cheeks, eyelids and bridge of the nose. Bullous impetigo Bullous impetigo is described in detail
The infection causes destruction of the cutaneous lym- in Chapter 7 on Vesiculobullous Disorders.
phatic vessels. This in turn increases the susceptibility for
future recurrences (30% recurrence rate).
MELIOIDOSIS
Management
Long-term antibiotic therapy with narrow spectrum peni- Meliodosis is a potentially fatal bacterial infection caused
cillins (benzylpenicillin) or macrolides may help in prevent- by exposure to soil or water contaminated with the bacte-
ing recurrent erysipelas infection. rial species Burkholderia pseudomallei. The causative
84
organism, Burkholderia pseudomallei, was thought to be a TETANUS

member of the Pseudomonas genus and was previously
known as Pseudomonas pseudomallei. The word tetanus comes from the Greek tetanos, which is
Alfred Whitmore, a pathologist and his assistant derived from the term teinein, meaning to stretch. Tetanus,
CS Krishnaswami in 1911, first described melioidosis as a commonly called lockjaw, is an acute neurologic disease
‘glanders-like’ disease among morphia addicts in Rangoon, that results from wound contamination with Clostridium
Myanmar (formerly Burma). tetani, an anaerobic, gram-positive, motile, spore-forming
Stanton and Fletcher in 1932 renamed this disease as rod characterized by generalized muscle rigidity and spasm,
meliodosis, which is a derivative from the Greek words sometimes associated with autonomic dysfunction.
‘melis’ (distemper of asses) and ‘eidos’ (resemblance).
Melioidosis is regarded as endemic to southeast Asia Pathophysiology
and northern Australia. In the Indian subcontinent a survey
conducted by Kang et al (1996) revealed a seroprevalence of It is believed that although most wounds may be con-
7% in a rural rice-growing area near Vellore. taminated with the spores of Clostridium tetani, the germi-
nation and toxin production occurs only in wounds with
Risk factors low oxidation reduction potential, such as those with devi-
talized tissue, foreign bodies, or active infection. The bac-
Diabetes, thalassemia, excessive consumption of alcohol, teria produce two exotoxins: tetanolysin (role still unclear)
renal disease, and frequent history of occupational or rec- and tetanospasmin. Tetanospasmin is the neurotoxin respon-
reational exposure to mud or pooled surface water are sible for the clinical manifestations of the disease. The toxin
common risk factors resulting in melioidosis. spreads hematogenously to the peripheral nerves and trav-
els in a retrograde fashion along the nerve fibers to reach
Clinical features the central nervous system where it blocks the release of
Melioidosis can present in an acute or chronic form. gamma-aminobutyric acid (GABA) from presynaptic
inhibitory neurons. This loss of inhibitory impulses results
The average incubation period of acute melioidosis is
in the cardinal clinical manifestations of reflex irritability
about 9 days. However patients can exhibit a period of
and autonomic hyperactivity.
latency. Such patients do not present any clinical symp-
toms. Literature review reveals that the longest duration Clinical features
between presumed exposure and clinical presentation was
62 years. Such prolonged periods of incubation were rec- Tetanus is usually seen in young adults who are prone to
ognized in American soldiers involved in the Vietnam traumatic injuries. Four types of tetanus are recognized
War, and was referred to as the ‘Vietnamese time-bomb’. based on the clinical presentation: localized, generalized,
The typical clinical presentations of acute melioidosis cephalic and neonatal.
usually include pain and fever. Other clinical findings are The localized form of tetanus is characterized by a lim-
cough or pleuritic chest pain suggestive of pneumonia, ited area of muscular spasm that is confined to the area of
bone or joint pain suggestive of osteomyelitis or septic the entry of the bacilli.
arthritis, or cellulitis, thyroid, lymph node and scrotal The cephalic subtype of tetanus is characterized by cra-
abscess and ocular infection. Parotid abscess has been nial nerve palsies that often precede trismus. Patients pres-
reported specifically in Thai children. ent with cranial nerve palsy (usually facial nerve is
The chronic form of melioidosis is seen in about 10% of affected). Approximately two thirds of cases progress to
the patients. A chronic form is characterized by the presence generalized tetanus. The incidence of cephalic tetanus
of symptoms for longer than 2 months. Patients may pres- ranges from 0.9 to 3.0%. The cephalic form results from
ent with chronic pneumonia, ulcers on the skin surface and injuries sustained to the head and neck region, tooth
chronic dermal infections. Since the chronic form closely extraction or chronic tympanitis. Some patients may pres-
ent with incomplete Bell’s palsy.
resembles tuberculosis, some authors term chronic melioi-
Generalized tetanus is the most common form of the
dosis as ‘Vietnamese tuberculosis’.
disease and carries the highest mortality. Incubation peri-
ods for the generalized form range from a few hours to
Diagnosis and management
greater than 1 month.
The bacterium can be cultured from the tissue fluid from Trismus or lockjaw is the initial presentation in 75% of
the abscesses and the patient’s blood and sputum. The cases. Facial muscle spasm may cause the classic sneering
drugs of choice include antibiotics such as trimethoprim– grin of risus sardonicus. Motor findings progress to involve
sulfamethoxazole and ceftazidime orally. However serious the neck, trunk and extremities, eventually leading to abdom-
infections are best managed with intravenous tetracycline, inal rigidity and opisthotonus. The muscle spasms may be
chloramphenicol, and trimethoprim-sulfamethoxazole. sustained or paroxysmal. In severe cases the spasm of
85
intercostal, diaphragmatic and pharyngeal muscles leads booster at school-entry age (4–7 years), in adolescence
to breathing difficulties which may eventually lead to death. (12–15 years), and in early adulthood.
Neonatal tetanus is a fatal yet preventable disease that
accounts for 14% of annual neonatal deaths worldwide. The
neonatal form of tetanus has a high mortality rate. It results
ACTINOMYCOSIS
from unhygienic delivery practices, application of harmful
substances on the umbilical cord, and lack of maternal TT
Actinomycosis is subacute to chronic, suppurative granu-
immunization. Risk for contamination and subsequent occur-
lomatous disease that tends to produce draining sinus tracts.
rence of neonatal tetanus remains high for several days after
It is caused by anaeroboic gram-positive, non-acid fast
delivery until the baby’s cord wound heals. The incubation
bacilli. The common isolates in humans include Actinomyces
period varies from 3 to 10 days. Neonate’s failure to suckle
naeslundii, A. israelii, A. meyeri, A. viscosus and rarely
is often the first sign of infection in the neonate, and typi-
A. odontolyticus. Kalfas et al (2001) identified a new spe-
cally occurs between the 3rd and 10th day of life. In spite
cies, Actinomyces radicidentis that was isolated from apical
of efforts by the infant, spasms of the masseter muscle impede
periodontitis.
feeding. The newborn becomes irritable and cries con-
Von Langenbeck noted the first case of human actino-
stantly. Exhaustion may subsequently bring about cessa-
mycosis in 1845. Bollinger and Harz in 1877, named the
tion of audible crying. Sometimes the lips are pursed as if
genus Actinomyces when they described the etiologic
to whistle. Variable degree of muscle spasm develops lead-
agent of bovine actinomycosis (‘lumpy jaw’) and called it
ing to asphyxia.
Actinomyces bovis.
Clinical features
Fascial space infections causing trismus, dystonic reac-
Actinomycosis is mostly found in young adults. Women
tions induced by such drugs as phenothiazines and meto-
are less frequently affected than men. Based on the site of
clorpropramide, hypocalcemia, meningitis, encephalitis,
involvement, actinomycosis can be grouped into the cer-
rabies and strychnine poisoning can be considered in the
vicofacial (55%), pulmonary (15%), abdominal and pelvic
differential diagnosis for tetanus.
(25%) and cutaneous and genitourinary actinomycosis
(5%). Cutaneous actinomycosis is extremely rare and these
Diagnosis are said to arise from wounds contaminated with saliva or
A simple bed side test may be used effectively to diagnose as a consequence of hematogenous dissemination follow-
tetanus. The spatula test is said to have a sensitivity of 94% ing a dental procedure. However primary cutaneous acti-
and specificity of 100%. The posterior pharyngeal wall is nomycosis have also been reported. The genitourinary
touched with a spatula. The test is considered positive if form has been reported in patients using intrauterine con-
there is a reflex spasm of masseter and negative if there is traceptive devices.
a normal gag reflex. The presenting symptoms of pulmonary actinomycosis
Enzyme immunoassays for antitoxin levels can also be are fever, cough, thoracic pain and dyspnea. The sputum is
used. A level of 0.01 IU/ml or greater is considered protec- mucopurulent or even sanguineous. With the appearance
tive, making the diagnosis of tetanus less likely. of fistulae, the disease spreads to the mediastinum, the peri-
cardium, and finally to the skin of the chest.
Actinomycosis is believed to be acquired by endoge-
Prevention and management
nous implantation into deep tissues where anaerobic con-
The first step should include rapid-sequence intubation ditions prevail. Actinomyces israelii is an anaerobic normal
with midazolam and succinylcholine. Passive immuniza- inhabitant of the mouth, especially in the teeth and tonsils.
tion with human tetanus immune globulin (5,000 IU) will In the cervicofacial region, puncture wounds, dental
help to neutralize free tetanospasmin. It should be given extractions, or compound fractures are some of the routes
after airway control and before wound debridement. of infection. The cervicofacial variant is characterized by
Finally the source wound should be thoroughly debrided, the appearance of solid sub- or supramandibular nodules
with removal of all foreign bodies and devitalized tissue. or swellings and the overlying skin becoming purple to
Metronidazole, 500 mg IV every 6 hours, is recommended violet.
as the first-line antibiotic. Clinical presentation of cervicofacial actinomycosis is
As a part of the supportive measures, sympathetic over- characterized by the presence of suppurative or ‘wooden’
activity can be managed with a labetalol infusion at 0.25– indurated mass with discharging sinuses. Pus from the dis-
1 mg/min. Diazepam may be used to sedate the patient. charging sinuses contains tiny yellow sulfur granules.
The WHO recommends that an individual should receive Common initial symptoms of infection including pain,
3 doses of DTP in infancy, followed by a TT-containing fever, erythema, edema, and suppuration may be absent.
86
Actinomycosis often involves lymphatic nodes but by Noma is considered to represent the ‘face of poverty’
the direct extension of a primary lesion. Occasionally, the because many of the risk factors that are associated
masticatory muscles and tongue may be involved result- with poverty. The World Health Organization (1998) has
ing in trismus and dysphagia. reported an estimated worldwide incidence of 140,000
cases per year.
Radiographic features
Clinical features
Actinomycotic osteomyelitis affecting the maxilla and
mandible have been reported. Radiographs reveal ill-defined Noma is usually seen in children between the age of 3 and
radiolucencies with a radiopaque periphery. 12 years mainly in the developing countries especially
Periapical actinomycosis is believed to be a non-resolv- sub-Saharan Africa. Children at risk for noma have been
ing periapical lesion associated with actinomycotic infec- seen to have low plasma concentrations of zinc, retinol,
tion and has been suggested as a contributing factor in the ascorbate, and essential amino acids with increased plasma
perpetuation of periapical radiolucencies after root canal and saliva levels of free cortisol.
treatment. A diagnosis is usually made by identifying the Many authors believe that noma, occurs secondary to
typical actinomycotic colonies in a surgical specimen. the extension of necrotizing ulcerative gingivitis.
Occasionally, the periapical actinomycotic lesion may In the initial stages ulcerative areas from the gingiva
appear radiopaque mimicking condensing osteitis. extend to involve the adjacent soft tissues. Subsequently
the necrotic areas spread both into deeper tissue planes
Investigations and superficially. The overlying skin turns deep blue to
black and eventually sloughs away. Extensive necrosis can
Sinus tracts may reveal the presence of yellowish granules lead to exposure of bone and osteomyelitis. Patient may
(1–6 mm in diameter) referred to as ‘sulfur granules’. On present with pain, fever, malaise, foul odor and regional
histological examination, the sulfur granules consist of a lymphadenopathy. The differential diagnosis for noma
central tangled mass of gram-positive mycelia surrounded must include mucocutaneous leishmaniasis, lupus erythe-
at the periphery by gram-negative, club-shaped rods. matosus, leprosy, agranulocytic ulcerations, injuries asso-
The hematoxylin and eosin stained specimen shows the ciated with physical trauma (including burns), syphilis,
‘ray phenomenon’. The periphery of the granule shows fila- oral cancer and yaws.
ments that are radially oriented and embedded in eosino-
philic material.
Other variants of Noma
Management ‘Noma neonatorum’ is characterized by gangrenous process

of the nose, oral cavity, eyelids, and perineum usually seen
The sinus tracts have to surgically excised and abscess in premature infants at births or within the first month of
drainage should be facilitated. Long-term antibiotic ther- life. The causative organism for noma neonatorum is usu-
apy with penicillin or tetracycline is recommended. ally Pseudomonas.
Noma pudendi is the term used for noma affecting the
anogenital area and causing necrosis of the genitalia.
NOMA (Cancrum Oris, Gangrenous or
Necrotizing Stomatitis) Complications and management
Extensive necrosis can cause premature loss of deciduous
The word noma is derived from the Greek word ‘nomein’ teeth, damage to the permanent tooth buds, sequestration
that means ‘to devour’. It is rapidly progressive opportu- of the jaws, trismus, and bony or fibrous ankylosis of the
nistic infection which is caused primarily by Fusobacterium temporomandibular joint. Occasionally, infection from the
necrophorum, Fusobacterium nucleatum and Prevotella oral cavity can extend to other parts of the body causing
intermedia. Other reported organisms isolated from the systemic complications such as toxemia, dehydration and
Noma lesions include -hemolytic Streptococci, Actinomyces bronchopneumonia. Untimely intervention can lead to
spp., Peptostreptococcus micros, Veillonella parvula, Staphylo- death.
coccus aureus, Corynebacterium pyogenes, Bacteroides Local wound care along with restoration of the hydra-
fragilis, Bacillus cereus and Pseudomonas species. tion, nutritional and electrolyte imbalance should be given
The predisposing and/or risk factors for noma include adequate importance.
poverty, malnutrition, immunosuppression (including HIV Penicillin along with metronidazole are the antibiotics of
infection), poor oral hygiene, unsanitary environment, choice in the management of noma. However, clindamycin
leukemia, and infectious diseases caused by measles and and gentamicin are the drugs of choice in the management
herpesviridae. of neonatal noma.
87
BOTRYOMYCOSIS (Bacterial Management

Pseudomycosis) Surgical resection along with antibiotic therapy is the treat-
ment of choice.
Botryomycosis arises from chronic infections produced by
low-virulence organisms in an altered host environment.
Staphylococci have been the most common organisms
implicated, but various other bacteria have also been identi-
RHINOSCLEROMA (Respiratory
fied in lesions of human botryomycosis. Scleroma)
The disease, later referred to as botryomycosis, was first
described involving the lung of a horse by Bollinger in Rhinoscleroma is an endemic, chronic, slowly progressive
1870. Sebastiano Rivolta in 1884 coined the term botryo- granulomatous disease caused by Klebsiella rhinosclero-
mycosis (‘botryos’ from Greek for bunch of grapes) after matis (a gram-negative rod-shaped bacteria, 2.5 m in
he found globular granules in a tumor from the cut sper- length).
matic cord of a horse. In 1882, von Frisch identified K. rhinoscleromatis as
the etiologic agent. In 1870 Ferdinando Von Hebra, a der-
matologist described the disease for the first time. It was
Clinical types and features
later named respiratory scleroma. The word ‘skleroma’ in
Winslow (1959) categorized botryomycosis into two types Greek meaning hard tumefaction, was adopted in 1932 at
based on their site of involvement: integumental and the International Clinical Otorhinolaryngology Conference
visceral. (in Madrid), emphasizing involvement of upper and lower
The integumental botryomycosis affects the exposed airways.
body surfaces such as the hands, feet or the head. It occurs Rhinoscleroma is contracted by direct inhalation of
in the site of a contaminated wound, foreign body or droplets or contaminated material.
trauma and manifests as a localized granulomatous infec-
tion. Occasionally it causes osteomyelitis. Clinical stages
The visceral form is relatively rare. It is usually seen in
immunocompromised individuals. It affects the lung, liver, Humans are the only identified host of K. rhinoscleromatis.
kidney, spleen, brain, prostate, bowel and the lymphatic It usually affects individuals in the 2nd to 4th decades of
tissues (tonsil, lymph node). life. It affects people living in crowded conditions with poor
The typical botryomycotic lesions are indurated fibrotic hygienic and nutritional conditions (including iron deficiency
masses that may form draining sinuses and fistulas. anemia). Rhinoscleroma affects women more commonly
Literature review reveals a few reports of botryomycosis than men (13:1).
affecting the orofacial region. Small and Kobernick (1967) The nose is the most common site of infection, although
reported a patient with botryomycosis of the tongue. Rawal the nasopharynx, paranasal sinuses, and pharynx may also
and Rawal reported a patient with gingival botryomycosis be involved. Other affected organs include the paranasal
and Alavandar (1979) reported botryomycosis affecting sinuses, eustachian tubes, middle ear, orbital tissues and
the mandible. the brain.
Rhinoscleroma occurs in three overlapping stages:
catarrhal-atrophic (sometimes called ozaena), granuloma-
Histopathologic features tous (proliferative or nodular) and sclerotic (cicatricial or
Hematoxylin and eosin stained specimens show chronic fibrotic).
suppurative and granulomatous inflammation with giant In the catarrhal stage, patient may complain of foul
cells, epithelioid macrophages and scattered microab- smelling purulent nasal discharge and nasal obstruction. On
scesses. clinical examination atrophy and crusting of the nasal
Within the areas of the purulent inflammation, Bollinger’s mucosa or hyperemia and exudates in the respiratory tract
granules are seen. These are relatively small, but fre- mucosa are evident.
quently microscopically visible, pale yellow or yellow-white In the granulomatous stage, epistaxis, nasal deformity
granules consisting of irregular aggregates or colonizations and destruction of the nasal cartilage (Hebra nose), hoarse-
of gram-positive cocci, usually staphylococci. ness of voice, anosmia and anesthesia of the soft palate
Bollinger’s granules are surrounded by an amorphous, are common signs and symptoms. Clinical examination
eosinophilic, refringent matrix called the Splendore-Hoeppli may reveal a bluish red and rubbery granulomatous lesion.
phenomenon (this phenomenon is also seen around colo- These lesions over a period of time turn into a pale hard
nies of certain bacteria, fungi, helminthes, actinomyces, granulomatous mass.
mycetoma, nasofacial and subcutaneous phycomycosis and In the sclerotic stage, clinical examination shows gran-
around silk sutures). ulomatous lesions surrounded by dense fibrotic tissue.
88
Diagnosis couple of months. The lymph nodes can enlarge to a size

of about 10 cm.
The diagnosis of rhinoscleroma is made by the bacterial
Other systemic manifestations include parotitis, osteo-
isolation by culture on blood or MacConkey agar. Histopa-
myelitis, hepatosplenomegaly, neurological conditions (sei-
thological specimens can be stained with periodic acid- zures, altered behavior or consciousness, peripheral facial
Schiff, Giemsa and Warthin–Starry stain. The presence of nerve paresis, myeloradiculitis) and hematological condi-
Mikulicz cells (clear cytoplasm vacuolated histiocytes tions (hemolytic anemia, thrombocytopenia and eosinophilia).
containing the bacillus) and degenerated plasmocytes in When a pet’s saliva contaminated (as the cat constantly
Russel bodies are diagnostic of rhinoscleroma. licks its fur) fur is groomed, organisms from the fur are
The hypertrophic stage of rhinoscleroma has character- transferred to the individual’s hand. Such contaminated
istic mild to marked high signal intensity on both T1- and fingers, when used to rub the eyes might cause self-inoc-
T2-weighted MR images. ulation of the organisms to the conjunctiva. Conjunctival
involvement will result in preauricular lymphadenopathy.
Management This association of conjunctival involvement and preau-
Nasal or pharyngeal obstruction is best managed surgi- ricular lymphadenopathy in cat-scratch fever is referred to
cally along with antibiotic therapy. Many authors recom- as ‘Parinaud oculoglandular syndrome’.
mend the use of cephalosporins and clindamycin. Shaer Several skin reactions have been reported, including
et al (1981) have shown that the topical application of erythema nodosum, erythema marginatum and erythema
2% acriflavine solution is an effective and safe treatment multiforme.
option for rhinoscleroma. Tracheostomy may be required B. henselae and B. quinata are implicated in the causa-
if laryngeal scarring causes airway obstruction. tion of bacillary angiomatosis and peliosis in immuno-
compromised hosts such as in AIDS. Orally these lesions
mimic Kaposi’s sarcoma and appear as vascular lesions
and within bone may cause alveolar bone loss.
CAT-SCRATCH DISEASE
Diagnosis
Cat-scratch disease arises from the inoculation of the
gram-negative bacillus Bartonella henselae following a Neville and coworkers enumerated certain criteria for the
cat’s scratch, lick or bite. It is estimated that almost 40% diagnosis of cat-scratch fever. Evidence of at least three of
of the domestic cats may have an asymptomatic B. hense- the following four criteria is considered to be positive for
lae infection accompanied by bacteremia, which can per- cat-scratch disease:
sist for more than a year. It is believed that domestic cats 1. Contact with a cat, presence of a scratch or a primary
tend to rapidly develop antibodies and therefore appear dermal or ocular lesion.
healthy in spite of the bacteremia. 2. Positive Hangar–Rose skin test (cat-scratch disease
The first description in the literature of cat-scratch skin test).
disease is credited to Henri Parinaud in 1889. Dr Robert This skin test was first developed by Hanger and Rose
Debré in 1931 described a case of a boy with a cat-scratch in 1946. Aspirated material from a lymph node of a
on his hand associated with a suppurative epitrochlear patient with known cat-scratch disease is pasteurized,
lymph node. He is credited with recognizing the cat as standardized, and tested for sterility. It is then injected
the vector of this illness and coined the term ‘cat- subdermally and skin reaction is noted.
scratch disease’. Regnery and coworkers (1992) identified
B. henselae as the causative organism for cat-scratch 3. Unidentifiable cause for lymphadenopathy.
disease. 4. Presence of pleomorphic bacilli with Warthin–Starry
Cat-scratch disease is considered as a self-limiting method or Brown–Hopps method.
granulomatous condition characterized by suppurative However, serological test (ELISA for IgM antibodies to
regional lymphadenitis. B. henselae) is the gold standard for the diagnosis of
cat-scratch disease. Elevated serum titers are seen 1–3 weeks
Clinical features after the onset of the disease process.
The incubation period varies from 7 to 15 days. It usually
Management
affects individuals in the second decade of life. Patients
may present with mild fever, fatigue and malaise. The ini- The condition is self-limiting and usually resolves in about
tial lesion occurs as a pustule or papule seen at the site of 6 months duration. Suppurative nodes may be aspirated to
the trauma (cat-scratch or bite). These initial lesions are evacuate the pus. The recommended surgical procedure to
followed by regional lymphadenopathy that lasts for a evacuate the pus is to introduce the needle into the skin
89
1 to 2 cm away from the swelling and then burrow beneath Warwick et al (2003) reported a patient with IM, rup-
the surface of the skin to reach the affected node. The tech- tured spleen and Cullen’s sign (periumblical ecchymosis).
nique helps to hasten the healing and prevent formation of They also suggest that the presence of abdominal pain is an
a persistent sinus. uncommon symptom in infectious mononucleosis and its
Antibiotics such as gentamicin, penicillin, ciprofloxa- occurrence is therefore a danger sign that may forewarn a
cin and rifampin may be used when systemic involvement potentially life threatening complication of ruptured spleen.
is apparent. The complications of IM include myocarditis and car-
Other bacterial infections with oral manifestations such diac conduction abnormalities, neurologic abnormalities,
as tuberculosis, syphilis, leprosy and gonorrhea have been meningitis, encephalitis, cranial nerve palsies, retrobulbar
described in Chapters 8, 21 and 22. neuritis, acute interstitial nephritis, hemolytic anemia,
thrombocytopenia and upper airway obstruction.
Syndrome association
VIRAL INFECTIONS
Chronic fatigue syndrome and Lemierre’s syndrome have
been reportedly associated with IM. The association of IM
INFECTIOUS MONONUCLEOSIS
and chronic fatigue syndrome is still very questionable
(Monoglandular Fever, Kissing Disease) and debatable.
Infectious mononucleosis (IM) is a clinical syndrome caused Diagnosis

by Epstein–Barr virus-4 (EBV, human herpes virus-4).
EBV replicates primarily in beta-lymphocytes but also The Paul-Bunnell test is a serological test that detects het-
may replicate in the epithelial cells of the pharynx and erophile antibodies by agglutination of sheep or horse red
parotid duct. blood cells. However, in the 1st week of infection, the false-
Children and young adults are usually affected. The virus negative rate is as high as 25%.
is transmitted via intimate contact. Children may acquire VCA-IgG and VCA-IgM tests are useful in diagnosing
the virus through sharing of saliva contaminated fingers, patients who have highly suggestive clinical features but
toys and serving spoons. Direct transfer of contaminated negative heterophile antibody test results.
saliva may occur in adults following kissing (hence the Antibody to Epstein–Barr nuclear antigen (EBNA),
name kissing disease) or sharing of straws. The incubation while typically not detectable until 6–8 weeks after the
period is 4–8 weeks. onset of symptoms, can help distinguish between acute
and previous infections.
Elevated hepatic transaminase levels may be seen in
Clinical features
about 50% of the IM patients.
The characteristic clinical features of IM include, malaise,
fatigue and anorexia. These symptoms are immediately Management
followed by high fever (about 104F) which lasts for almost
Infectious mononucleosis usually resolves in about 6 weeks.
2 weeks.
IM is best managed with extensive palliative and supportive
The most striking feature of IM is the presence of
healthcare. Patient should be adequately hydrated. Fever
lymphadenopathy. Any or all lymphatic chains may be
and malaise may be managed with acetaminophen and
enlarged. Lymphadenopathy is always bilateral and sym-
NSAIDs. Though steroids have been used frequently, they
metrical in all patients. Bilateral posterior and anterior
are best used only in an emergency to relieve the patient
cervical lymphadenopathy is highly suggestive of EBV
of respiratory compromise secondary to pharyngeal edema.
infectious mononucleosis.
Antibiotics like penicillins are best avoided as these
Other clinical features include the presence of tonsillar
patients have a higher risk of developing a morbilliform
enlargement, hepatosplenomegaly, jaundice, rhinitis and
skin rash.
pharyngitis.
Hoagland’s criteria (1975) for the diagnosis of IM
include: at least 50% lymphocytes and at least 10% atypi-
cal lymphocytes in the presence of fever, pharyngitis, and ACUTE LYMPHONODULAR PHARYNGITIS
adenopathy, and confirmed by a positive serologic test.
Oral manifestations include hard and soft palate Acute lymphonodular pharyngitis is caused by Coxsackie
petechiae, necrotizing ulcerative mucositis, necrotizing virus A10. It is characterized by prodromal fever, anorexia,
ulcerative gingivitis and pericoronitis. Occasionally, the headache and sore throat. Like most viral infections, the
parotid gland may be affected along with facial nerve condition is self-limiting and generally resolves in about
palsy. 2 weeks.
90
Clinical features convulsions and subacute sclerosing panencephalitis may

develop.
Acute lymphonodular pharyngitis is usually seen in chil-
dren and young adults. Clinically, the hyperplastic lym-
Diagnosis
phoid aggregates are evident as discrete yellow to dark
pink colored nodules or white to yellow papules surrounded Measles can be diagnosed based on the characteristic skin
by an erythematous ring, generally 1–5 in number. These rash and the Koplik’s spots. The diagnosis can be con-
lesions are typically found on the tonsillar pillars, uvula, firmed by the measles virus sandwich-capture immuno-
soft palate and oropharynx. globulin M (IgM) antibody assay.
Histopathological evaluation of these nodules may reveal
epithelial necrosis and inclusion bodies (intranuclear or Prevention and management
cytoplasmic) and multinucleated cells.
Infants below 1 year of age are protected by the maternal
antibodies. Live attenuated measles vaccination given to a
Management
child older than 1 year provides immunity.
The condition is self-limiting and the management is The measles-mumps-rubella (MMR) vaccine is given in
aimed at supportive care. Non-aspirin containing analge- two doses. The first dose is given to children at the age of
sics are beneficial. 1 year. The second dose is given usually between 4 and
6 years of age.
Supplementation by vitamin A during an acute attack,
MEASLES (Rubeola) minimizes the morbidity and mortality risks.
Measles is caused by an RNA virus, paramyxovirus.

Measles is an acute and highly communicable disease and GERMAN MEASLES (Rubella)
infection confers lifelong immunity. The disease process
is marked by the presence of prodromal fever, cough, con- Rubella is caused by Rubivirus, an RNA virus of the
junctivitis and coryza (profuse discharge from the nasal Togaviridae family. Rubella runs a milder course and it
mucous membrane). affects the skin, lymphatic system and the joints. However,
Measles may often occur as an epidemic. The virus is infection of the mother during pregnancy may lead to
transmitted via direct contact or by droplet infection and serious complications for the developing infant.
the respiratory tract forms the portal of entry. The disease spreads via the respiratory route and mainly
Measles is usually considered as a serious disease in by droplet infection and to a lesser extent by direct con-
malnourished, immunocompromised or vitamin deficient tact with contaminated throat or nasal secretions.
individuals.
Clinical features
Clinical features
The incubation period lasts for approximately 18 days. It
Following a 14-day incubation period skin rashes begin to typically affects children and to some extent young adults.
appear. About 2–3 days before the typical skin rash The initial prodromal symptom period is followed by
appears the patient may exhibit prodromal upper respira- lymphadenopathy. Suboccipital and posterior auricular
tory symptoms and conjunctivitis along with lymphade- nodes are typically enlarged. The other clinical findings
nopathy. The skin rash fades away in about 7 days. Pale include skin rashes and pharyngitis. The most important
skinned individuals may exhibit areas of altered pigmen- complication of rubella is the congenital rubella syn-
tation following resolution of the skin rash. drome. The maculopapular rash appears pink and appears
During this stage of the infection the pathognomonic first on the face and then spreads rapidly downwards to
Koplik’s spots begin to appear on the buccal mucosa. Koplik’s involve the trunk and the extremities. These eruptions are
spots are small, irregular, red spots with a minute bluish generally transitory and persist only for about 3 days, thus
white speck in the center of each seen on the buccal accounting for the famous synonym ‘3-day measles’.
mucosa and lingual mucosa. They are named after Henry
Koplik (1858–1927), an American pediatrician who first
Oral manifestations
described them in 1896.
Patients are usually irritable and photophobic at this The common oral manifestation is the presence of minute
stage. These spots represent sites of virus replication and red macules and petechiae on the soft palate and uvula
sites of inflammation of the mucous glands. (Forchheimer spots: non-specific for rubella) of pinpoint
As the disease progresses, bacterial superinfection red macules and petechiae can be seen over the soft palate
may lead to diarrhea, bacterial pneumonia, cancrum oris, and the uvula just before or with the exanthem.
91
Mothers suffering from rubella during the first trimes- that measures antibodies to HIV that are the body’s immune
ter are likely to have children who exhibit pitting and response to the HIV. This blood test remains the best
enamel hypoplasia and rarely complete aplasia. Eruption method for diagnosing HIV infection. American researcher
of teeth may be retarded. Some authors have reported cleft Robert Gallo and French scientist Luc Montagnier are both
lip and palate in some of these children. credited for discovering HIV.
In the later parts of 1986 in India, first cases of HIV
Prevention and management were diagnosed among sex workers in Chennai, Tamil Nadu.
It was noted that contact with foreign visitors had played
Rubella is best prevented by mumps-measles and rubella
a role in initial infections among sex workers.
vaccination. The disease is self-limiting. As individuals are
In 1987 a National AIDS Control Program was launched
highly contagious in the first 1 week, following appear-
to co-ordinate national responses. Its activities covered
ance of the skin rash, they should ideally abstain from
surveillance, blood screening, and health education. By
group activities during this period. Maintenance of hydra-
the end of 1987, out of 52,907 who had been tested, around
tion and bed rest is generally sufficient. Non-aspirin con-
135 people were found to be HIV positive and 14 had
taining NSAIDs may be given for managing the prodromal
AIDS. Most of these initial cases had occurred through
symptoms.
heterosexual sex, but at the end of the 1980s a rapid
spread of HIV was observed among injecting drug users in
the three north-eastern states of India, namely Manipur,
HIV AND AIDS Mizoram and Nagaland. In 1992 the government set up
NACO (the National AIDS Control Organization), to over-
Human immunodeficiency virus (HIV) is a lentivirus (a see the formulation of policies, prevention work and con-
member of the retrovirus family) that can lead to acquired trol programs relating to HIV and AIDS.
immunodeficiency syndrome (AIDS). HIV infects cells of In 1982, AIDS was originally defined by US Center for
the human immune system and destroys them or prevents Disease Control as ‘the disease, at least moderately predic-
them from functioning. Such individuals whose immune tive of a defect in a cell mediated immunity occurring in a
system is defective are much more vulnerable to opportu- person with no known cause for diminished resistance to
nistic infections. that disease’.
Retroviruses have an inherent advantage of both muta- HIV infection in children was first recognized in 1983.
tion (typical of RNA viruses) and latency (typical of DNA Pediatric AIDS can be defined as disease occurring in chil-
viruses). dren less than 13 years of age.
The etiologic agents of AIDS and HIV infections belong
to the family of human retroviruses and the subfamily of
Discovery of HIV
lentiviruses.
Disease syndromes similar to the clinical manifestation of The four recognized human retroviruses belong to two
AIDS have been described in the ancient Ayurvedic litera- distinct groups:
ture. Sushrutha in 800 BC and later Charaka and Vagbhatta
i. The human T lymphotropic viruses [HTLV-1 and 2]
describes a conditions with ‘loss of muscle mass, fever, skin
ii. The human immunodeficiency virus [HIV-1 and 2]
eruptions and ulcers, complexion changes, neurological
disorders, exhaustion, coma and death, and stated that in The most common cause of HIV disease throughout the
irremediable stages treatment should be given up’. world is HIV-1, which was identified in May 1983, and
In 1981, homosexual men with symptoms of a disease HIV-2 was first identified in 1986 in west African patients
that now are considered typical of the acquired immuno- and was originally confined to west Africa. The major dif-
deficiency syndrome (AIDS) were first described in Los ference in the genomes of HIV-1 and 2 is that the HIV-2
Angeles and New York. The men had an unusual type of lacks the vpu gene. HIV is more closely related to an SIV
lung infection (pneumonia) called Pneumocystis carinii (simian immunodeficiency virus) isolated from chimpan-
(now known as Pneumocystis jiroveci) pneumonia (PCP) zees in 1990. HIV-2 is much more closely related phyloge-
and rare skin tumors called Kaposi’s sarcoma. The patients netically to the simian immunodeficiency virus found in
were noted to have a severe reduction in CD4 cells. These cells, sooty mangabees.
often referred to as T cells, help the body fight infections.
Shortly thereafter, this disease was recognized throughout
Structure of the HIV-1 Virion
the United States, western Europe, and Africa. In 1983,
researchers in the United States and France described the HIV-1 virion is spherical in shape and contains an electron
virus that causes AIDS, now known as the human immuno- dense core surrounded by a lipid envelope derived from
deficiency virus (HIV) and belonging to the group of viruses the host cell membrane during budding of the virus from
called retroviruses. In 1985, a blood test became available the infected cell.
92
The virus core contains several core proteins, two

Table 2 Predominant types of HIV-1 in various parts of the
strands of genomic RNA, and the enzyme reverse tran-
world
scriptase that is characteristic of all retroviruses.
The viral envelope is studded with two viral glycopro- Region Predominant type Other subtypes
teins, gp120 and gp41. The gp41 projects outward and is
South America B
important for the fusion of the virus to its target cells and
Australia B C, F
gp120 helps in binding of host cell CD4 receptor.
The virion is approximately 10 kb in length, and it con- Western Europe B A, C, D
tains the gag, pol and env genes, that code for the core Eastern Europe B, C, G, D F
protein, reverse transcriptase and envelope proteins Africa A, B, D, C, E, F, G, H, O
respectively. Uganda A, B, C, D, G
Each structural region comprises three structural proteins/
Zaire A, D, H
antigens. Env gene makes gp160, gp120 and gp41 glyco-
Kenya A
proteins. Gag gene is responsible for p55, p24, and p17
antigens and pol for p31, p51, p66 antigens. Nigeria, Gabon G, H
Cameroon A, B, E, G, H, F
Central Africa A, C, D, E
Subtypes HIV-1 Virus Zambia, Malawi C
The strains of HIV-1 can be classified into three groups: (Southeast Africa)
the ‘major’ group M, the ‘outlier’ group O and the ‘new’ China Bb C
group N. These three groups may represent three separate Southeast Thailand, Bb, E
introductions of simian immunodeficiency virus into Myanmar
humans. Indonesia E
More than 90% of HIV-1 infections belong to HIV-1 India C A, B, F
group M. Group O is believed to be restricted to west-
central Africa and group N (discovered in 1998) is seen in
Cameroon is extremely rare. nucleic acids in the reverse direction, i.e. DNA from RNA
Within group M there are known to be at least nine and hence termed ‘reverse transcriptase’ and the virus as
genetically distinct subtypes (or clades) of HIV-1. These retroviruses.
are subtypes A, B, C, D, F, G, H, J and K (Table 2).
Occasionally, two viruses of different subtypes can
meet in the cell of an infected person and mix together Basic Genetics of HIV
their genetic material to create a new hybrid virus (a pro-
HIV contains nine genes made of 9,749 base pairs.
cess similar to sexual reproduction, and sometimes called
Gag (codes for internal structural proteins and capsid pro-
‘viral sex’).
teins using about 2,000 base pairs), pol (codes for the three
Many of these new strains do not survive for long, but
enzymes necessary for replication using about 2,900 bp) and
those that infect more than one person are known as ‘cir-
env (codes for the surface proteins gp120 and gp41 that
culating recombinant forms’ or CRFs. For example, CRF
protrude from the lipid envelope and attach to cellular
A/B which is a mixture of subtypes A and B.
receptors using about 1,800 bp).
Other genes are tat (transactivator protein), rev (regula-
Lentivirus tor of expression of virus protein), vif (virus infectivity
HIV-1
factor), nef (misnamed negative regulator factor, but really
HIV-2
an enhancing factor), vpr (virus protein R), and vpu (virus
protein U).
Group M Group N Group O
Stages of Infection (Figures 1–17)
Clades A, B, C, D, F, F2, G, H, J, K Step 1: Entry of the HIV virion into the host T-cell, is trig-
gered by the binding of membrane proteins to receptors
on the T-cell surface.
The hallmark of the life cycle of HIV infection is the reverse Step 2: The membrane proteins on the virus are units
transcription of genomic RNA to DNA by the enzyme reverse called gp120. These gp120 unit binds to the receptors
transcriptase. Crispian Scully (1997) stated that HIV is an called CD4 on the T-cell. The union of gp120 and CD4
RNA virus containing an enzyme, which can transcribe initiates another event.
93
Figure 1 Figure 3
gag p17
gag p24
HIV RNA
4
CD
gp 40
gp 120 Envelopment of
T-cell virus into host cell
Co-receptor CCR5 combines with CD4
Figure 4
Binding of HIV virion into host T cell
Figure 2
HIV Endocytosis
T-cell
Complete envelopment of virus

4
CD
5
Figure 5
T-cell
CCR
Capsid
gp120 unit binding to CD4 receptor

HIV
Step 3: CD4 changes its shape so that another membrane T-cell

protein of the T-cell called CCR5 fits. CCR5 is a co-recep-
tor that is essential in enabling the virus to enter the cell. Capsid within host cell
Patients with defective co-receptors do not show signs of
HIV infection in spite of repeat exposures.
Step 4: These events lead to the envelopment of the virus Step 7: Shedding of the capsid is necessary for the exposure
into its host cell. of viral RNA and enzymes. In HIV, the viral genome is in
Step 5: This process is known as endocytosis. the form of RNA. There are two strands of RNA connected
Step 6: Once inside the host cell capsid begins to shed its at one end. The capsid also contains important enzymes for
coat. the events leading to infection of the host cell.
94
Figure 6 Figure 8
Enzymes
Double stranded
viral DNA
RNA
Capsid (shedding the capsid)
Shedding of capsid
Formation of double stranded DNA

Figure 7
Figure 9
Viral RNA
Viral DNA
Reverse
transcriptase
Viral DNA
Host cell nuclear
envelope
Reverse transcription
Host DNA
Step 8: The first event is called reverse transcription. From

a strand of RNA, the enzyme reverse transcriptase cata-
Viral DNA entering host cell nucleus
lyzes the formation of viral DNA.
Step 9: Reverse transcriptase also helps in the formation
of the second strand of viral DNA.
Step 10: The viral genetic material is ready to enter the
host cell nucleus and become integrated into the host DNA. Figure 10
Step 11: The viral DNA is integrated with the help of
another enzyme called retroviral integrase. The integrated
DNA is now called a provirus. Viral DNA
Step 12: The provirus serves as a template for the synthe-
sis of viral RNA. Upon completion, the newly formed viral
RNA moves out into the cytoplasm.
Step 13: The viral RNA carries information that codes for Host DNA
viral proteins and enzymes. Therefore, it is also called
messenger RNA. The codes on the viral RNA are translated
into proteins and enzymes in the form of a long chain Retroviral integrase
called a polypeptide chain. [PROVIRUS]
Step 14: The polypeptide chain includes important pro-
Viral DNA integrating with host DNA
teins for the envelope, capsid, enzymes, and other parts of
95
Figure 11 Figure 13
Ribosome
Viral RNA
Synthesis of viral RNA
Protease breaking polypeptide chain
Figure 12
Figure 14
Viral RNA
Host cell
T-cell nucleus Capsid begins to form

Polypeptide chain
Early stages of capsid formation
Formation of polypeptide chain

Figure 15
the new virus to be formed. The enzyme protease helps to

cut this chain into individual components.
Step 15: With all the components ready to leave the host Membrane proteins
cell the capsid forms to envelop these pieces.
Step 16: Assembly of the capsid initiates the insertion of
membrane proteins on the host cell’s plasma membrane.
Step 17: As the new virus leaves or buds from the surface
of the host cell, it forms an envelope with the inserted
membrane proteins. Host cell
Step 18: Numerous new viruses can be formed from one
T-cell and move on to infect other T-cells in various parts
Formation of membrane proteins on host cell
of the body.
96
and with the frequency of anal receptive intercourse,

Figure 16
which predisposes to rectal trauma in the receptive part-
Plasma membrane ner. It is believed that the virus is carried in lymphocytes
present in the semen and enters the recipient’s body
through abrasions in rectal mucosa. HIV-1 has been found
in vaginal and cervical secretions and in monocytes and
endothelial cells within the submucosa of the uterine cer-
vix of infected individuals through which the transmission
to the male partners takes place.
Zagury D Bernard and Leibwitch et al (1984) stated that
HIV spreads to women from men through the semen and
Budding
from cervical secretions of infected woman to men. This
was based on the isolation of HIV from semen and cervical
Host cell secretions, which makes it biologically possible for HIV to
spread heterosexually from men to women and women
Newly formed virion leaving host cell
to men.
Faltz, Mc Clure and Doughaty et al (1986) stated that
infection can occur without trauma to the vagina. This
was substantiated by experimental vaginal HIV infection
Figure 17 of a chimpanzee and from the case reports of infection
through artificial insemination of the virus.
Pandian et al (1987) reported a relatively higher risk for
those engaged in anal intercourse than those having only
vaginal intercourse but emphasized that anal intercourse
was not always necessary for transmission.
Greenblatt et al (1987) stated that a factor that may
influence transmission is genital ulceration. He stated that
an African study found an increased risk of being infected
on those with a history of genital ulceration.
New HIV molecule Mayer and De Glottal (1987) stated that anecdotal cases
of HIV transmission by oral sexual activity have been
Host cell reported but has never been convincingly demonstrated.
Nobut Gilmole (1992) reported that among the sex-
ual modes of HIV transmission, intercourse is the most
predominant factor, as more than 60% of HIV transmis-
Newly formed HIV molecule sion worldwide were attributed to vaginal intercourse. He
also stated that everybody is susceptible to HIV infection
although some people have been reportedly exposed to
Step 19: The T-cells are eventually destroyed, leaving the the virus and inexplicably they have not been infected.
body with a compromised immune system and susceptible He stated that susceptibility to HIV may need to be facili-
to attack by foreign bodies. tated by mucosal injury caused by trauma, sexually trans-
mitted diseases or genital ulcers.
Transmission Anne M Johnson (1988) stated that there is some evi-
dence that the risk of transmission increase both with the
Transmission of HIV-1 occurs mainly through one or more duration of the sexual relationship and the frequency of
of three routes: sexual contact.
1. Sexual contact WHO (1997) stated that individuals vary on their sus-
2. Passage of the virus from infected mothers to the ceptibility to infection for reasons that are not known.
newborn Through sexual intercourse some may get infected by a
3. Through blood and parenteral inoculation. single exposure from infected partners while others may
not become infected even after repeated exposures. The
receptive partner is at greater risk than the insertive partner
Sexual contact
in both vaginal and anal intercourse. Male to female
It is the predominant mode of transmission. The rate of transmission was higher than transmission from female to
infection is increased with the number of sexual partners males. This was substantiated on 5-year follow-up study
97
at San Francisco where 20% of female partners of HIV Ramos-Gomez (1997) stated that HIV can be transmitted
positive men got infected whereas only one case of female perinatally from mother to newborn infant in three
to male transmission was observed. As per their observa- ways:
tion they stated that anal sex was more hazardous than
vaginal sex because there was greater degree of trauma in 1. Transplacentally during pregnancy
anal intercourse as anal mucosa is thin and vascular. So 2. During delivery as the infant passes through the birth
the highest risk is for person who is passive or receptive canal
partner in anal intercourse. They also opined that oral sex, 3. Postnatally during breast feeding
deep kissing may carry a risk of infection and risk of
An estimated 94% of pediatric HIV infection is acquired
acquiring infection is much higher in those suffering from
perinatally and around half of these reported perinatal
sexually transmitted disease especially so in the presence
infection occurred when the infant through the birth
of ulcerative lesions of the genitalia.
canal.
WHO and NACO (1997) in their publication stated that
Passage of the virus from infected mothers most of the children acquire HIV from their mothers through
to the newborns the three possible routes: transplacentally, intrapartum
Carrew, Jaffe, Hardy A Metal (1988) stated that the pro- and breast feeding.
portion of HIV-infected pregnant women whose infants are The other routes like blood transfusion and role of
subsequently found to be infected ranges from 16 to 33%, contaminated syringes also contributed in a small way.
but the specific determining perinatal infection remains They stated that transplacental is a major source of infec-
unexplained. tion and parental transmission accounting for 80% of
Oxdely (1988) stated that a rare case of transmission all AIDS cases. Frequency of transmission from mother
through breast milk has been documented. Philiop A to child was around 25% and transmission was more in
Piczzo and Karina M Balter (1991) stated that the exact mothers with HIV symptomatic group than asymptomatic
time during pregnancy or delivery when HIV transmission group. They stated that intrapartum transmission was due
occurs is not well established. Although the presence of to contact of child with maternal blood during delivery.
virus had been documented in fetal tissues after 8–15 They also stated that the transmission can occur through
weeks’ gestation, HIV was found predominantly during breast feeding, as the virus has been isolated in breast
intrapartum period. This might occur because of ingested milk.
maternal blood or because of maternal-fetal transmission The mother to child transmission can be now pre-
during labor and delivery. vented by the recent use of antiretroviral drugs. These are
said to prevent the virus transmission from mother to
Goryle, Selik and Chersov (1990) stated that the most
child if the virus status of the mother is detected in early
common means of transmission of HIV to a child from an
stages of pregnancy and prompt use of antiretroviral
infected mother is either during pregnancy or at birth.
therapy.
They stated that although the vertical transmission of HIV
from an infected mother to an infant is unique, few cases
may represent a blood borne transmission. Through blood and parenteral inoculation
In 1996, Indian Council of Medical Research stated
about the selected factors associated with mother to child Volken Wah, Hans H Kramer and Thomas et al (1986)
transmission of HIV-1. It was classified into: reported a case of HIV infection that appeared to have
occurred through horizontal transmission with two sib-
1. Proved or possible modes of transmission lings. They stated even minor bites by HIV infected chil-
❍ Maternal immune deficiency dren may carry the risk of transmitting the virus. They
❍ Chorioamnionitis cautioned the parents and teachers and other people
❍ Breast feeding responsible for HIV infected children should be aware of
❍ Vaginal delivery this possibility and try to prevent spread of virus by this
❍ Low maternal serum vitamin A concentration route.
❍ Instrumental injury during labor and/or delivery Fashy, Schmith and Sash et al (1989) stated that risk of
(episiotomy, forceps, etc.) HIV transmission to healthcare workers by accidental par-
❍ Premature rupture of membranes enteral exposure to infected blood has been estimated to
❍ Prematurity be 0.36% and 0.41% of such injuries.
❍ Prolonged labor Gershon, Valhov and Nelson (1990) stated that in a
2. Controversies study of more than 1,300 dental healthcare workers 94%
❍ Maternal immune response against HIV had sustained an accidental inoculation type injury with a
❍ Viral strain characteristics median number of three parenteral injuries per year.
98
However the risk of HIV infection in this study was ACQUIRED IMMUNE DEFICIENCY SYNDROME
only 0.08%.
Desjarlair and Friedman (1995) stated that transmission Centers for Disease Control and Prevention (CDC) in 1993
by parenteral exposure to infected blood such as through had defined acquired immunodeficiency syndrome (AIDS)
the sharing of uncleaned injection equipment during as the occurrence of one or more group of life-threatening
injecting drug appeared to be the most efficient means of opportunistic infections, malignancies, neurologic dis-
HIV transmission. They also stated that the prevalence of eases, and other specific illnesses in patients with HIV
HIV infection has risen rapidly among equipment sharing infection and/or with CD4 counts less than 200/mm3. CDC
during drug abuse in many countries, emphasizing the stated that this definition was a surveillance definition that
spread by the HIV by this mode. Mast and Grebreding was established to track the incidence of this disease and
(1995) stated that volume of blood involved in percutane- the relative occurrence of diseases that are likely to occur
ous exposure may prove an important determinant of risk. in severely immunosuppressed individuals. It is stated that
The type of gauge of needle used, the depth of penetration in that part of the world where CD4 enumeration is not as
and glove use, effect the amount of blood transferred dur- readily available, clinical diagnoses, in conjunction with
ing needle prick injuries. They also stated that the titer of serologic tests for HIV, could be used to define patients
virus in the contaminant is hypothesized to be an impor- with AIDS and to track the spread of this epidemic.
tant variable affecting the infection. They stated that mean
circulating viral titer in patients with AIDS was 100–1,000 Revised Classification System of HIV Disease
times higher than in patients with HIV infection who are (CDC, 1993)
asymptomatic and exposure of blood from patients with
AIDS was associated with higher risk of transmission. The CDC disease staging system assesses the severity of
HIV disease by CD4 cell counts and by the presence of
HIV and Saliva specific HIV-related conditions.
The definition of AIDS includes all HIV-infected indi-
Malamad et al (1997) in their review article ‘Inhibition of viduals with CD4 counts of 200 cells/l (or CD4 per-
HIV infectivity by saliva’ stated that viral inhibitory fac- centage 14%) as well as those with certain HIV-related
tors are present in a variety of human saliva. They conditions and symptoms.
proposed that the inhibitory factors are produced within
the salivary glands probably in greater concentration in CD4 (mm3) A B C
submandibular saliva and may indicate the presence of 500 A1 B1 C1
multiple factors. They concluded from their study that 200 to 400 A2 B2 C2
saliva does not lyse HIV.
200 A3 B3 C3
Shugars and Wahi (1998) in their article ‘How oral cav-
ity resists transmission of HIV’ stated that the thick epithe-
lial layer of intact oral mucosa is a barrier to infection. Category A
They stated that saliva lubricates the mucosa, dilutes the ❍ Asymptomatic HIV infection
microbial burden and also washes microbes into the gas- ❍ Persistent generalized lymphadenopathy
trointestinal tract where they are destroyed. When trauma ❍ Acute retroviral syndrome
or disease ruptures the physical barrier of the mucosa, HIV
can potentially enter susceptible cells. They stated that Category B
rare cases of transmission have been reported through
blood, blood contaminated oral mucosa or saliva. Further ❍ Bacillary angiomatosis
they stated that saliva inhibits HIV by several endogenous ❍ Candidiasis
inhibitors and oral secretion can neutralize HIV via virus ❍ Cervical dysplasia
specific antibody response. Various salivary components ❍ Constitutional symptoms (fever, diarrhea 1 month)
like lactoferrin, lysosome, lactoperoxidase exert antimi- ❍ Oral hairy leukoplakia
❍ Herpes zoster
crobial effects. Mucins and thrombospondin can entrap
❍ Idiopathic thrombocytopenic purpura
viruses. Defensins—SLP-I and thrombospondin work to
❍ Listeriosis
inhibit HIV from entering susceptible cells. They conclude
❍ Pelvic inflammatory disease
that mouth is made resistant to HIV transmission by
❍ Peripheral neuropathy
the combination of a thick epithelial layer, low number of
CD4 bearing target cells and variety of endogenous inhib-
Category C (AIDS defining conditions)
itors antiviral antibodies. They also stated that these
inhibitory mechanisms may fail when mucosal surface is ❍ CD4 count less than 200 cells/l
not intact. ❍ Candidiasis (pulmonary, esophageal)
99
❍ Cervical cancer Diarrhea (continuous or Progressive headache

❍ Coccidiodomycosis intermittent more than
❍ Cryptosporidiosis 1 month)
❍ Cytomegalovrius Generalized extrainguinal Drug reactions (previously
❍ Encephalopathy lymphadenopathy not seen)
❍ Herpes simplex (chronic, esophageal) Skin infection (severe or Failure to thrive fever
❍ Histoplasmosis recurrent) (continuous/intermittent
❍ Isosporiasis Cough for more than than 1 month)
❍ Kaposi’s sarcoma 1 month dermatitis Generalized
❍ Lymphoma lymphadenopathy
❍ Mycobacterium avium/Mycobacterium kansasii
❍ Pneumocystis carinii Stages in HIV disease
❍ Recurrent pneumonia
❍ Progressive multifocal leukemia. Stages CD4 count range Duration
Acute infection 1,000–750 1–4 weeks
WHO (1997) suggested the criteria for HIV infection. This
criterion was based on the clinical diseases and was clas- Asymptomatic 750–200 2–15 weeks
sified into cardinal findings, characteristic findings and Early symptomatic 500–100 1–5 years
associated findings. Late symptomatic 50–200 1–4 years
Advanced disease 50–0 0–2 years
Cardinal findings
Adults Children Correlation of CD4 cell count and AIDS

complications
Kaposi’s sarcoma Kaposi’s sarcoma (rare in
children) CD4 Strata Complication
Pneumocystis carinii Pneumocystis carinii 500/mm 3 Persistent generalized lymphadenopathy
pneumonia pneumonia Candida vaginitis
Toxoplasma encephalitis Lymphoid interstitial Guillain–Barre syndrome
pneumonitis Polymyositis
Esophageal candidiasis Esophageal candidasis Aseptic meningitis
Cytomegalovirus retinitis Pneumococcal pneumonia
200–500/mm3 Pulmonary tuberculosis
Characteristic findings Thrush
Zoster
Adults Children Cryptosporidiosis, self-limited
Cervical intraepithelial neoplasia
Oral thrush Severe prurigo (itching Kaposi’s sarcoma
without lesion) B cell lymphoma
Oral hairy leukoplakia Non-Hodgkin’s lymphoma Anemia
Miliary, extrapulmonary or Recurrent bacterial/viral Idiopathic thrombocytopenic purpura
infections 200/m3 Mononeuritis multiplex
Non-cavity pulmonary Herpes zoster, past or (usually Oral hairy leukoplakia
tuberculosis present 100/m3) P. carinii pneumonia
Cryptococcal meningitis Progressive neurological Disseminated or chronic herpes simplex
disease Miliary/extrapulmonary
Non-cavity pulmonary Herpes zoster: Tuberculosis
tuberculosis multidermatomal Candida esophagitis
CNS lymphoma
Associated findings Wasting
HIV-associated dementia
Adults Children Peripheral neuropathy
Cryptococcosis
Weight loss more than 10% Neurologic findings Disseminated histoplasmosis and
(dementia)
50/mm3 microsporidiosis
Fever (continuous or Focal motor deficits Disseminated/chronic herpes simplex
intermittent more Disseminated M. avium
than 1 month) CMV retinitis
100
Tuberculosis and HIV b. Squamous cell carcinoma

c. Non-Hodgkin’s lymphoma
It is suggested that the clinical presentation of tuberculosis
5. Neurologic disturbances
is dependent on the degree of immune suppression and
a. Trigeminal neuropathy
those patients with CD4 cell counts above 200/mm3 more
b. Facial palsy
likely to have classical upper lobe disease and cavitary
6. Unknown cause
changes characteristic of reactivation disease. Extrapul-
a. Recurrent aphthous ulceration
monary tuberculosis frequently occurs with HIV infection
b. Progressive necrotizing ulceration
and usually seen in 70% of patients with CD4 cell count
c. Toxic epidermolysis
less than 100/mm3. WHO (1997) stated that in India, 60%
d. Delayed wound healing
of AIDS patients have tuberculosis infection.
e. Idiopathic thrombocytopenia
f. Salivary gland enlargement
Oral manifestations g. Xerostomia
h. Melanotic hyperpigmentation
In 1986 the European community took the initiative to
establish a classification system for oral manifestations
in HIV infection. The classification was presented by Diagnostic criteria
Pindborg. He classified the oral manifestations into six
In 1989, WHO Collaboration Center for Oral Manifestation
categories based on associated agents like fungal, bacteria,
of the HIV under ‘WHO Global Program on AIDS’ and
viral, neoplasm, neurological and unknown cause.
‘European Clearing House of Oral Problems related to HIV’
1. Fungal infection defined the diagnostic criteria for oral manifestations.
a. Candidiasis These diagnostic criteria were proposed for epidemiologi-
i. Pseudomembranous cal surveys. The diagnostic criteria were given for the most
ii. Erythematous common oral manifestations. Following are the diagnostic
iii. Hyperplastic criteria suggested.
iv. Angular cheilitis
b. Histoplasmosis
1. Candidiasis
c. Cryptococcosis
d. Geotrichosis i. Pseudomembranous: The pseudomembranous is pre-
2. Bacterial infection sented as a white or yellow removable plaque leaving
a. HIV Necrotizing gingivitis a red surface. Pseudomembranous may be located in
b. HIV Gingivitis all parts of the oral cavity.
c. HIV—Periodontitis ii. Erythematous: It is defined as red area without
Caused by: Mycobacterium avium intercellulare, removable plaques often located on palate, dorsum
Klebsiella pneumoniae, Enterobacterium cloacae, of the tongue and buccal mucosa. Smears from red
Escherichia coli area must be positive for candida hyphae on PAS
d. Actinomycosis staining.
e. Cat-scratch disease iii. Angular: Fiery and commissures. Smears from red area
f. Sinusitis must be positive for candida on PAS staining.
g. Exacerbation of apical periodontitis
h. Submandibular cellulitis
2. Periodontal disease
3. Viral infection
a. Herpes simplex a. Gingivitis: They defined gingivitis as the disease char-
b. Cytomegalovirus acterized by fiery red edematous attached gingiva and
c. Epstein–Barr may affect the alveolar mucosa. No ulceration must be
Hairy leukoplakia present.
d. Varicella zoster b. Necrotizing gingivitis: This is characterized by gingi-
i. Herpes zoster val pain, swelling, ulcerations, necrosis or as distribu-
ii. Varicella tion of interdental papillae covered with a fibrous
e. Human papilloma virus slough. The patient suffers from fever and halitosis
i. Verruca vulgaris may be present.
ii. Condyloma acuminatum c. Periodontitis: This is characterized by aggressive
iii. Focal epithelial hyperplasia irregular bone destruction. Any infection that gives
4. Neoplasms the impression of affecting periodontal structure other
a. Kaposi’s sarcoma than gingiva.
101
3. Hairy leukoplakia – Focal epithelial hyperplasia

– Verruca vulgaris
Hairy leukoplakia presents as a white, non-removable
❍ Varicella zoster virus
lesion on margin of the tongue. The surface is corrugated,
❍ Herpes zoster
but might be non-corrugated if it is seen on the inferior
❍ Varicella
surface of the tongue or on the buccal mucosa. To estab-
lish a reliable diagnosis, a biopsy must be performed. Group 3: Lesions seen in HIV infection
Biopsy from hairy leukoplakia shows hair-like projections,
❍ Bacterial infections
hyperparakeratosis, koilocytic like cells and no inflamma-
– Actinomyces israelii
tion. The surface layer of the epithelium shows numerous
– Escherichia coli
hyphae of candida.
– Klebsiella pneumoniae
❍ Cat-scratch disease
4. Oral Kaposi’s sarcoma
❍ Drug reactions (ulcerative, erythema multiforme, lichen-
A characteristic macroscopic appearance of either erythema- oid, toxic epidermolysis)
tous or violaceous plaque-like lesions, or a bulky tumor ❍ Epithelioid (bacillary) angiomatosis
predominantly seen in palate or on the gingiva. ❍ Fungal infections other than candidiasis
– Cryptococcus neoformans
Revised classification by the European Community – Geotrichum candidum
Clearing House (1992) – Histoplasma capsulatum
– Mucoraceae (mucormycosis/zygomycosis)
European Community Clearing House in 1992 revised the
– Aspergillus flavus
classification of oral lesions associated with HIV infection.
❍ Neurologic disturbances
The manifestations were divided into three main groups:
– Facial palsy
i. Lesions strongly associated with HIV infection – Trigeminal neuralgia
ii. Lesions less commonly associated with HIV infection ❍ Recurrent aphthous stomatitis
iii. Lesions seen in HIV infection ❍ Viral infections
– Cytomegalovirus
Group 1: Lesions strongly associated with HIV infection
– Molluscum contagiosum
❍ Candidiasis: Erythematous
Pseudomembranous Laboratory diagnosis
❍ Hairy leukoplakia
There are two main approaches for the diagnosis of HIV
❍ Kaposi’s sarcoma
infection, the direct and the indirect.
❍ Non-Hodgkin’s lymphoma
The direct method seeks information on the presence of
❍ Periodontal disease
virus itself by classical isolation method and identifying the
– Linear gingival erythema
presence of virus specific genes by molecular biology tech-
– Necrotizing (ulcerative) gingivitis
niques. George Babu in his review states that the classical
– Necrotizing (ulcerative) periodontitis
method of isolation for HIV is time consuming, expensive,
Group 2: Lesions less commonly associated with HIV requires special containment laboratory and highly trained
infection personnel.
He also states that molecular biology technique such as
❍ Bacterial infections
polymerase chain reaction (PCR) is expensive and should
– Mycobacterium avium intercellulare
be done by highly trained personnel but it is less expen-
– Mycobacterium tuberculosis
sive than classical virus isolation and less time consum-
❍ Melanotic hyperpigmentation
ing. He further states that PCR finds application under
❍ Necrotizing (ulcerative) stomatitis
special circumstances such as the presence of early stages
❍ Salivary gland disease
of infection.
– Dry mouth due to decreased salivary flow rate, uni-
The indirect methods are based upon the observation
lateral or bilateral swelling of the major salivary
that infected persons make antibody eventually. In most
glands
cases, the IgG class of antibody can be detected on the
❍ Thrombocytopenic purpura
serum 6–8 weeks after infection.
❍ Ulceration NOS (not otherwise specified)
❍ Viral infections ELISA Test
– Herpes simplex virus 1. The first system to have been introduced is ELISA
– Human papilloma virus (warty-like lesions) (enzyme linked immunosorbent assay) for screening
– Condyloma acuminatum and western blot test for confirmation of HIV antibody.
102
ELISA is done in a 96-well polystyrene microtiter HAART may be associated with adverse side effects such
plates, the plates being coated with HIV specific anti- as IRIS (immune reconstitution inflammatory syndrome,
gen and allowed to react with patients serum. which is characterized by worsening of the already exist-
2. The other rapid tests include DOT tests (e.g. tridot insti, ing opportunistic infection, appearance of new opportu-
HIV—check and agglutination tests (e.g. Combaids, nistic infections and autoimmune disorders.
Immunocomb). Other side effects of HAART include: bone marrow
3. Western blot test is used to detect the presence of suppression, bleeding disorders, liver and renal toxicity,
nine antibodies to the nine HIV specific antigens. hypersensitivity reactions and severe forms of erythema
In asymptomatic stage, the presence of one antibody multiforme.
(gp160, gp120 or gp41) one from core region (p17, Charles Barr (1995) recommended the following ther-
p24 or p55) and one from polymerase region (p31, p51 apy for oral disease associated with HIV/AIDS.
or p66) is required. In a symptomatic person, the pres-
ence of gp41 with one band from any of the two I. Fungal disease
regions is adequate.
Candida albicans
Management 1. Nystatin vaginal tablets: 100,000 IU/tablet to dissolve
one tablet in the mouth 3 times daily for 1–2 weeks.
The management of HIV disease is two pronged: one aimed 2. Mycostatin pastilles: (Nystatin) 200,000 IU/pastille.
at managing the conditions arising out of the immunosup- One pastille dissolved in mouth to 5 times daily for
pression and opportunistic infections and the other tar- 1–2 weeks.
geted at the virus itself. 3. Mycelex troches (Clotrimazole): 10 mg troche one troche
I. Management of the viral infection dissolved in the mouth 5 times daily for at least 14 days.
Antiviral therapy is instituted in patients with AIDS 4. Nizoral (Ketaconazole): 2,000 mg/tablet one tablet
regardless of the CD4 count and in patients who are daily with food until lesions disappear if response is
asymptomatic but with CD4 count less than 200 cells/ poor—2 tablets daily.
mm3 (Table 3).
Four groups of drugs can be used to combat the virus. 1. Zovirax (acyclovir)—200 mg tablets. Two tablets 4–5
A combination of these drugs may be used in the man- times daily. Recurrence is frequently noted after ther-
agement of HIV infection. These regimens are referred to apy is discontinued.
as highly active antiretroviral therapy (HAART). 2. Podophyllin resin—25% solution. Dab solution on
The commonly employed regimens used include: lesions with cotton applicator 2–3 times daily. To
rinse mouth with plain water for 30–60 seconds fol-
a. 1 NNRTI 2 NRTI lowing last application last application. Allow 1 week
b. 1 or 2 PI 2 NRTI for beneficial effect. Second application may be nec-
c. Triple NRTI. essary depending on thickness of lesion.
3. Retin A (tretinoin)—topical retinoid solution 0.05%
apply to involved area for 1–2 minutes daily for sev-
Table 3 Antiviral therapy for management of HIV infection
eral days.
Drug Examples Mechanism of action
Herpes simplex virus-1 Zovirax (acyclovir) 200 mg
Fusion inhibitors Enfuvirtide It is an anti-HIV peptide tablets 2 tablets 4–5 times daily until healing occurs.
that inhibits entry of the
virus into host cells Varicella zoster virus Zovirax (acyclovir) 200 mg tablet
Nucleoside reverse Abacavir They terminate the or 800 mg tablets: to take 4 g/day.
transcriptase Didanosine elongation of the
inhibitors (NRTIs) Lamivudine growing DNA chain and
Cytomegalovirus Cytovene (ganciclovir sodium), intra-
Stavudine reduce or prevent venous 7.5–15 mg/kg/day for 10–14 days.
Zalcitabine replication of HIV in
Human papilloma virus Surgical excision by scalpel.
Zidovudine infected cells
Cryotherapy, CO2 base, or electrocoagulation.
Non-nucleoside Nevirapine They inhibit a vital step in
reverse transcriptase Efavirenz the transcription of RNA
inhibitors (NNRTIs) Delavirdine genome into double II. Bacterial disease
stranded viral DNA
A. Linear erythematous banding
Protease inhibitors Indinavir They inhibit the cleavage 1. Irrigation with 10% povidone iodine solution
(PIs) Ritonavir of viral proteins 2. Prophylaxis
Atazanavir
3. Antifungal therapy (if suspect candiasis)
103
4. 125 chlorhexidine gluconate Sterilization

5. Frequent systemic follow-up.
Steam: Autoclave instruments at a temperature of 121C,
B. Necrotizing (ulcerative) gingivitis and periodontitis at 15 lb/sq in pressure for 15–20 minutes on specially
1. Irrigation with 1% povidone iodine solution modified cooker.
2. Debridement of necrotic gingival tissue, scaling, Flame: Heating with flame until red-hot to sterilize instru-
root planing ments such as knives and other skin piercing instruments.
3. Antibiotic therapy
4. Hydrogen peroxide—water rinses Disinfection
5. 12% chlorhexidine gluconate rinse
6. Frequent systemic follow-up of the case. a. Boiling: Completely immerse instruments in water, for
about 20 minutes, WHO states that boiling is suffi-
III. Neoplasia cient to inactivate (destroy) HIV.
b. Chemical: HIV is highly fragile and easily inactivated
Kaposi’s sarcoma
by the following chemicals:
1. Chemotherapy: Wet Velban (Vinblastine sulfate) 0.1 ml
1. Ethanol—70%
of a 0.2 mg/ml solution for each 0.5 cm lesion. Drug is
2. Glutaraldehyde—2%
injected 0.2 intralesionally after local anesthesia.
3. Household bleach—1% solution
2. Localized radiation therapy: Fractionated radiother-
4. Formaldehyde—8% (dilute formalin 1:5)
apy of approximately 800 to 1,500 cGy
5. Chlorine sodium—10% solution
3. Intro A (Interferon): 3–5 million IU injected into lesion
6. Isopropyl alcohol—3.5% solution
3 times per week
4. Sotradecol (sodium tetradecyl sulfate): Intralesional WHO also recommends that the needle, syringe should not
injection of a 3% solution at 0.2 cc/cm be recapped, bent or broken by hand to avoid needle prick
5. CO2 laser. injuries or skin puncture. They suggested that it should be
collected in a container with bleach solution and destroyed.
VI. Aphthous ulcers For reusable syringe and needles, they suggested that these
should be decontaminated by soaking in 0.1% sodium
1. Lidex (Fluocinonide) ointment 0.05% or clobetasol
hypochloride solution for 20–30 minutes. They also stated
ointment. Apply to lesion
that when autoclaving is not possible, sterilization can
2. Dexamethasone 0.5 mg/5 ml swish 1–2 teaspoonful
be achieved by specially modified cooker at 15 Ibs/sq inch
around mouth for at least 1 minute and expectorate, pressure at 121C temperature for 15–20 minutes or by
4 times daily until lesion disappears boiling in water for 20 minutes.
3. Prednisone–10 mg tablets. One tablet up to 6 times Diana Shin Flemming and John C Fahley (1996) have
daily for 1 week depending on healing mentioned about CDC classification of dental instruments
4. Thalidomide 200 mg every 12 hours for 5 days into three categories on their risk of transmitting infection:
5. Tetracycline oral suspension–125 mg/5 ml swish and
expectorate 1. Critical: These are surgical and other instruments
6. Levamisol: 50 mg every 8 hours for 3 years. used to penetrate soft tissue or bone which should be
sterilized after each use. These devices include for-
Universal precautions ceps, scalpels, bone chisels, scalers and burs.
2. Semicritical: These instruments are those which do
WHO (1997) recommended ‘Universal Safety Precautions’ no penetrate soft tissue or bone but contact oral tissues
for preventing the spread of HIV infection. They stated which should be sterilized after each use; if not feasible
that ‘Universal Safety Precaution’ means that all body flu- it should receive high level disinfection (e.g. Mouth
ids and blood of patients should be considered as infec- Mirrors, Amalgam condensers).
tions and all precautions should be taken since it is not 3. Non-critical: These are the instruments or medical
known who is infected with HIV. services such as external components of X-ray heads
The universal precaution starts with: that come contact only with skin; these could be dis-
❍ Hand washing. infected by detergent or washing depending upon the
❍ Creating appropriate barrier by use of gloves, masks, nature and degree of contamination.
gowns, eye protectors.
❍ Careful handling of sharp objects.
❍ Proper sterilization and disinfection. SINUSITIS
❍ Disposal of instruments after use/decontamination of
instruments including syringes, needles and equipment. Inflammation of the paranasal sinus mucosal lining is
❍ Proper disposal of infected waste. referred to as sinusitis. When all the paranasal sinuses
104
(frontal, maxillary, sphenoid and ethmoid) are inflamed flow), referred tooth pain, halitosis and reduced smell and
the term pansinusitis may be used. Based on the duration taste.
of the clinical presentation sinus diseases can be catego- Usually acute sinusitis when untreated will result in a
rized into: medically irreversible chronic phase of sinusitis. In rare
❍ Acute: defined as disease lasting less than 1 month. instances, acute sinusitis can trigger an asthmatic attack,
❍ Subacute: presence of disease for 1 to 3 months. may spread to the brain to cause meningitis and can cause
❍ Chronic: is longer than 3 months duration. visual disturbances.
Chronic Sinusitis
Acute Sinusitis
Chronic maxillary sinusitis is defined as sinusitis lasting
Sinusitis occurs when the mucous membranes of the upper
longer than 12 weeks. Patients may report of chronic
respiratory tract namely the nares, pharynx, sinuses and
facial pressure in the maxillary region, headache, rhinor-
larynx become inflamed. The swelling obstructs the sinus
rhea, postnasal drip, light headedness, decreased sense of
openings and prevents mucus from draining normally.
smell, or toothache.
This creates a moist environment which aids in harboring
In children, the symptoms of sinusitis are less specific
infection.
than in adults. Symptoms include persistent nasal conges-
The most common cause for sinusitis is a viral infec-
tion and cough lasting for more than 10 days, high fever
tion. However sinusitis can also occur as a result of a bac-
and purulent nasal discharge. Children are less likely to
terial or fungal (secondary to aspergillosis, mucormycosis,
present with facial pain or headache.
candidiasis, histoplasmosis and coccidiomycosis) infec-
tion. When the upper respiratory tract infection persists
Clinical and radiographic diagnosis
for longer than 2 weeks, a bacterial etiology is more likely.
Other causes for sinusitis include allergies, deviated nasal Apart from history, clinical examination of the sinuses
septum, nasal polyps, antroliths (Figure 18) and as compli- will help in diagnosing sinusitis. In many individuals the
cations of systemic conditions such as cystic fibrosis, gas- sinuses are tender in palpation. Another simple chair side
troesophageal reflux or immunodeficiency diseases. The test that can be employed is transillumination to assess the
severity of sinusitis may exacerbate when exposed to pol- maxillary and frontal sinuses. The paranasal sinuses can
luted air or when an individual smokes. be assessed using plain radiographs such as Waters’ view
(facilitates viewing of the maxillary, frontal sinuses and
Clinical features sphenoid sinuses), Caldwell view (visualization of the
Patients suffering from sinusitis include facial pain (espe- frontal and ethmoid sinuses), lateral view (to visualize the
cially in the periorbital region, maxillary sinus region and sphenoid sinus and the posterior frontal sinus wall) and
forehead), fever, fatigue, nausea, nasal congestion, ery- the submentovertex view (sphenoid sinuses are visualized).
thema over the sinus region (owing to increased blood The typical radiographic findings in a paranasal
sinus view include: localized thickening of the mucosal
lining at the base of the sinus, generalized thickening of
Figure 18 the mucosal lining (Figure 19), partial filling of the sinus
(fluid level appearance) and a complete cloudy or hazy
sinus (Figure 20).
CT of the osteomeatal complex has been used to assess
pansinusitis. It has been frequently reported that sinus CT
scanning has a high sensitivity but a low specificity for
demonstrating acute sinusitis. CT scanning may be used to
assess significant mucosal thickening, air-fluid levels
(Figure 21), osteomeatal complex obstruction, polyposis
(Figure 22), or calcification suggestive of fungal sinusitis.
Management
Acute sinusitis is best managed medically using nasal
decongestants and systemic antihistamines. Patients
should be instructed to use steam inhalation. In most
Intraoral periapical radiograph showing antrolith. infections amoxicillin is sufficient to neutralize pathogens
such as S. pneumoniae, H. influenzae and M. catarrhalis.
Manipal College of Dental Sciences, Mangalore
However when the infection does not subside in a week’s
105
time, following culture, the antibiotic coverage can be FUNGAL INFECTIONS AND
widened to include clavulanic acid. PROTOZOAL DISEASES
In extreme cases when the medical line of management
fails to provide adequate drainage of the sinus, surgical
HISTOPLASMOSIS
drainage may be undertaken. Surgical treatment includes
antral lavage, middle meatal antrostomy and endoscopic
enlargement of the osteomeatal complex. Histoplasmosis was first described by an American physi-
Herpes simplex virus infections, varicella zoster infec- cian, Samuel Darling in 1906. Histoplasmosis is caused by
tions, herpangina, herpangina, hand, foot and mouth dis- the dimorphic fungus (present as yeast at body tempera-
ease, mumps, condyloma acuminatum and molluscum ture and as mold in the natural environment) Histoplasma
contagiosum are described in Chapters 7, 8, 11 and 22. capsulatum.
The mold typically inhabits soil in humid areas con-
taining bat or bird excrement. When humans inhale these
Figure 19
Figure 21
CT scan showing thickening of the mucosal lining of the

ethmoid sinus CT scan showing fluid level appearance in the right
maxillary sinus
Figure 20
Figure 22
Paranasal sinus view showing complete haziness of the

left maxillary sinus CT scan showing a large polyp in the right maxillary sinus
106
air borne spores, they travel to the lungs. It is primarily a Chronic Pulmonary Histoplasmosis
lung disease. There are two varieties of H. capsulatum that
are pathogenic to humans, H. capsulatum var capsulatum It is usually seen in elderly white males who have a pre-
and H. capsulatum var duboisii. Histoplasmosis is an existing lung disease such as emphysema. Patients present
endemic infection in most of the United States. with malaise, productive cough, fever, and night sweats
mimicking the clinical features of tuberculosis. The form
Pathogenesis of histoplasmosis causes necrosis and loss of lung tissue.
Chest radiograph may reveal cavitation and infiltration
Following deposition of the spores in the alveoli at the in the upper lobe of the lungs.
normal body temperature the spores germinate into the Progressive thickening of cavity walls and retraction of
yeast form. The yeast form of the organism is promptly adjacent lung tissue occur over time. Radiographs typi-
ingested by pulmonary macrophages. The yeasts become cally reveal the absence of adenopathy.
parasitic, multiply within these cells, and migrate to hilar
and mediastinal lymph nodes. They subsequently gain
Disseminated Histoplasmosis
access to the blood circulation and eventually disseminate
to various organs. The disseminated form of histoplasmosis is a rare disease
Macrophages throughout the reticuloendothelial system and occurs primarily in immunocompromised persons
ingest and sequester the organism. (patients with HIV infection, lymphoreticular neoplasms,
In an immunocompetent host, in about 2 weeks after corticosteroid therapy, cytotoxic therapy and immunosup-
exposure, cellular immunity develops, and the macro- pressive agents).
phages become fungicidal and eliminate the infection. The The spectrum of illness in disseminated disease ranges
sites infected (such as the lung, liver, spleen, bone marrow from a chronic, intermittent course in immunocompetent per-
and lymph nodes) show evidence of necrosis, leading to sons to an acute and rapidly fatal infection that usually occurs
caseation, fibrous encapsulation, calcium deposition and in infants and severely immunosuppressed persons. Fever
within a few years of the initial exposure, calcified granu- is the most common symptom; however, headache, anorexia,
lomas are formed. cough, weight loss, and malaise are frequent complaints.
However, in an immunocompromised host due to defect Hepatosplenomegaly, lymphadenopathy and oropharyn-
in the cellular immunity, a lethal progressive disseminated geal ulcerations are typically encountered.
form of infection is seen. The buccal mucosa, tongue and palate are the most
common sites affected. Solitary painful ulcers are seen.
Clinical features These erythematous ulcers exhibit firm rolled-out margins
that resemble malignant ulcers. The disseminated form of
There are three clinical forms of histoplasmosis: acute pul-
the disease also affects the nervous system, gastrointesti-
monary, chronic pulmonary and disseminated form.
nal system and the renal system.
Most of the infected individuals are either asymptom-
atic or present very mild illness. Only less than 1% of the Diagnosis
individuals exhibit symptoms.
Histoplasmosis can be diagnosed by culture, fungal stains,
serologic tests for antibodies, and antigen detection.
Acute Pulmonary Histoplasmosis In order to demonstrate the yeast of H. capsulatum spe-
Patients may present with malaise, fever, headache, chills, cial stains such as PAS and Grocott-Gomori methenamine
weight loss, myalgias, sweating, non-productive cough silver have to be used.
and pleuritic chest pain.
Prognosis and management
However, when large number of spores are inhaled
patients may present with dyspnea and hypoxia. Occa- Histoplasmosis is a self-limiting disease. However the
sionally, hepatosplenomegaly, adenopathy, erythema nodo- untreated disseminated form may result in death in almost
sum, and erythema multiforme may be seen. 90% of the individuals. Analgesics and antipyretics may be
Acute pulmonary infection may lead to mediastinal used to manage fever and myalgias. Intravenous amphoteri-
granuloma, pericarditis and arthritis (symmetrical and mul- cin B is used as the drug of choice. Other drugs that have been
tiple joints are involved). used with good results are ketoconazole and itraconazole.
Chest radiographs may reveal solitary or multiple patches
of air space, especially in the lower zones of the lung. Severe
infections may reveal presence of hilar and mediastinal BLASTOMYCOSIS (Gilchrist Disease)
adenopathy and small diffuse pulmonary nodules.
The symptoms of the acute form of the disease resolve Blastomycosis was first described in America by the
spontaneously. American dermatologist, Thomas Caspar Gilchrist in 1894.
107
It is caused by Blastomyces dermatitidis, a dimorphic fun- The hallmarks of disease with these organisms are
gus. It is believed to thrive in organic matter and soil angioinvasion, thrombosis, infarction and necrosis of the
which have high moisture content. involved tissue.
Following inhalation of the spores, three forms of the
disease are manifested: acute, chronic and a disseminated Predisposing factors
form. The predisposing factors for mucormycosis include diabetes,
hematological malignancies (leukemia, lymphoma), bone
Clinical manifestations marrow or organ transplant, prolonged use of steroids,
Blastomycosis is typically seen in men engaged in outdoor patients on deferoxamine therapy (Rhizopus species thrive
activities usually in the 3rd and 4th decades of life. The well in iron rich environment), severe and prolonged neu-
acute form of blastomycosis is characterized by fever, tropenia, deficient T cell immunity and low birth weight.
malaise, myalgias, weight loss, cough, and pleuritic chest Diabetes with ketoacidosis is a very potent predisposing
pain. factor. Mucormycosis thrives very well in acid pH and glu-
The chronic form is much more commonly seen than cose rich medium. Hyperglycemia enhances fungal growth
the acute form. It is characterized by symptoms mimicking and impairs neutrophil chemotaxis, while lactic acidosis
those of tuberculosis such as night sweats, low-grade fever, decreases phagocytosis.
productive cough and weight loss.
Clinical features
Rarely, dissemination to other body sites such as the
skin, bone, CNS, genitourinary system and oral cavity may Mucormycosis may manifest as a rhinomaxillary form or
be seen. Cutaneous lesions may appear as erythematous a more severe rhinocerebral form. It also manifests as pul-
nodules that subsequently ulcerate. monary, gastrointestinal, cutaneous or a disseminated form.
In the early stages of the disease, patients exhibit facial
Oral features cellulitis, anesthesia, nasal discharge, epistaxis, fever, head-
ache and lethargy.
Intraoral lesions of blastomycosis appear as irregular pink Involved tissues become red, then violaceous and
or white areas. The ulcers may be painful and appear finally black as vessels are thrombosed and tissues undergo
irregular with rolled borders. necrosis. Extension into the orbital region can lead to peri-
orbital edema, proptosis, tearing and ocular or optic nerve
Histologic findings involvement spreading along the cribriform plate can
On histological section blastomyces can be easily discern- result in intracranial involvement.
ible with PAS stan, H and E stain, PAP and methenamine
silver stains. It can be cultured using Sabouraud’s agar. Oral manifestations and radiographic features
The most common oral sign of mucormycosis is ulceration
Management of the palate, which results from necrosis due to invasion of
For mild cases itraconazole and ketoconazole can be used a palatal vessel. It characteristically causes denudation of
effectively. Patients who are refractory to ketoconazole underlying bone, occasionally forming an oroantral fis-
can be treated with amphotericin B. tula. Ulcers have also been reported on the lip, gingiva and
alveolar ridge. Gingiva has a peculiar erythematous hyper-
plasia and is termed strawberry gingivitis.
Paranasal sinus radiographs may reveal thickening of the
MUCORMYCOSIS (Zygomycosis, sinus mucosal lining and occasionally air-fluid levels. In
Phycomycosis) severe forms of the disease bone destruction may be seen.
Mucormycosis is an opportunistic deep fungal infection Diagnosis

caused by ‘bread mold fungi’ of the genera Mucor, Absidia,
Special stains like calcofluor white stain; Gomori methe-
Rhizopus and Cunninghamella, also collectively known as
namine silver stain, periodic acid Schiff and Papanicolau
Phycomycetes.
stains are required. Cytological specimens may show rib-
Zygomycosis was first described by Platauf in 1885 as
bon like, wide, aseptate hyphal elements exhibiting angio-
‘Mycosis mucorina’. The class ‘Zygomycetes’ is subdivided
invasion and necrotic debris.
into two orders, which contain the agents of human
Zygomycosis, the Mucorales and the Entomophthorales.
Management
Among the Mucorales, Rhizopus (most common), Mucor,
Absidia, Rhizomucor, Cunninghamella, Saksenaea, Coker- Underlying predisposing factors should be corrected.
omyces and Apophysomyces have been implicated in The necrotic lesions have to be surgically debrided.
causing human disease. Amphotericin B is the first line drug of choice for most
108
cases of zygomycosis. Some authors suggest the use of droppings, rotting wood and soil. Otto Busse, a pathologist,
hyperbaric oxygen therapy as it may aid in inhibiting the in 1894, isolated C. neoformans from the tibia of a 31-year-
growth of fungal spores. old woman. Abraham Buschke also isolated C. neoformans
from the same patient, thus giving rise to the Busse–
Buschke disease in recognition for their discovery.
ASPERGILLOSIS Cryptococcosis is an acute, subacute, or chronic infec-
tion by C. neoformans, chiefly affecting the central ner-
Aspergillosis was first described in 1729 by a priest bota- vous system, causing a pulmonary, disseminated, or
nist, Micheli. In 1893, Morrel Mackenzie published the meningeal mycosis.
first case of aspergillosis affecting the maxillary sinus. It is The risk factors for developing serious forms of cryptococ-
a saprophyte that belongs to the class of myocetes. cosis include patients with AIDS, post organ transplantation,
Aspergillus flavus, A. niger and A. fumigatus are reticuloendothelial malignancy, long-term corticosteroid
pathogenic to humans. It is believed that aspergillosis is treatment and individuals suffering from sarcoidosis.
the second most common opportunistic infection to affect Cryptococcus neoformans is the second most common
immunocompromised individuals. cause of opportunistic fungal infection in patients with
Aspergillus is usually present in decaying matter, soil AIDS. It is considered as a sentinel infection that signals
and water. It is contracted via inhalation. Aspergillus may the perturbation of the host’s immune status.
cause hypersensitivity reactions and in some individuals it
exhibits direct angioinvasion. Aspergillus primarily affects
the lungs resulting in allergic bronchopulmonary aspergil- Types
losis, chronic necrotizing Aspergillus pneumoniae, asper- There are two varieties of C. neoformans: C. neoformans
gilloma (fungus ball/mycetoma) and invasive aspergillosis. var neoformans consisting of serotypes A and D (causes
However, in immunocompromised host the fungi is no disease in immunocompromised hosts) and C. neoformans
longer restricted to the lung. This disseminated form causes var gatti consisting of serotypes B and C (causes disease in
endophthalmitis, endocarditis, and abscesses in the myo- normal hosts).
cardium, kidney, liver, spleen, soft tissue, and bone and The yeast is typically found in pigeon feces. Some of
oral involvement. these organisms are restricted to tropical and subtropical
areas, and are isolated from certain species of eucalyptus
Oral findings trees and the air beneath them. Infections occur through
Involvement of the oral cavity may be seen in the dissemi- inhalation of yeast like organisms which enter smaller respi-
nated form of the disease. The tongue, soft and hard palate ratory passages and then remain dormant depending on the
and an occasional report of pulp and periodontal tissue host reaction. Organisms are then reactivated from such
involvement have been described in literature. Involvement previous dormant infections in the lung or lymph node.
of the maxillary sinus may result in an extension to the
adjacent structures and the palate to form a very painful Clinical features
ulcer surrounded by a zone of necrotic black tissue.
Cryptococcosis is seen more commonly in men due to
their occupational exposure or a lack of estrogens. The
Diagnosis
infection usually affects the respiratory system and the
Aspergillus may be identified in branched septate hyphae nervous system. However a wide dissemination into mul-
about 4 m in diameter in potassium hydroxide prepara- tiple body sites may also occur.
tions. Aspergillus may be cultured in Sabouraud’s agar It is estimated that 40–85% of the patients present
media. The hyphae demonstrate a tendency to invade with involvement of the brain parenchyma. Patients may
adjacent blood vessels. initially complain of headache, fever and nuchal pain sug-
gesting meningeal irritation. As the disease progresses,
Management stupor, coma and dementia may be seen. Papilloedema may
be seen. Complications of CNS involvement include inter-
The ulcerated lesion should be debrided, systemic ampho-
nal hydrocephalus, focal motor deficits, and symptoms of
tericin B therapy is the drug of choice.
raised intracranial pressure.
When the lung is involved, a primary pulmonary
complex, similar to TB may be seen. Symptoms of acute
CRYPTOCOCCOSIS (European Blastomycosis, pneumonia with cough, fever, and lobar pulmonary infil-
Torulosis, Busse-Buschke Disease) trates affecting alveoli may be seen. Pleural effusion with-
out parenchymal lesions are rare.
Cryptococcosis is an opportunistic fungal infection caused Cutaneous involvement results in the formation of pap-
by Cryptococcus neoformans. It is usually present in bird ules, abscesses, cellulitis, acneiform lesions, draining sinuses,
109
or subcutaneous swellings. Ocular involvement is seen as be detected in cerebrospinal fluid. The organism grows in
choroidal infection and endophthalmitis. Direct traumatic blood and chocolate agar within 3–5 days.
inoculation into the bone may result in osteomyelitis and Histopathological examination requires the uses of Pap
arthritis. stain, India ink preparation, alcian blue, Mayer mucicarmine
stain and Masson-Fontana silver stain. These stains help
Oral features in the detection of the polysaccharide capsule of the yeast.
Oral lesions of cryptococcosis appear as nodular or granu- India ink preparation is useful when greater than 10 colony
lomatous lesions which subsequently ulcerate. These forming units (CFU)/ml of yeasts are present.
ulcers typically reveal indurated borders and rolled-out Alternatively, cryptococcal antigen in cerebrospinal fluid
edges. The common sites that are affected include the hard can be detected by latex agglutination.
and soft palate, tongue, gingiva and the tonsillar pillars.
Cryptococcus infection has also been reported involving Management
extraction sockets. Amphotericin B is the drug of choice. However other drugs
that have fewer side effects compared to amphotericin such
Diagnosis
as flucytosine, fluconazole and itraconazole can be used.
The organism can be isolated in culture, recognized histo- Oral candidiasis and paracoccidiodomycosis are described
pathologically and its polysaccharide capsular antigen can in Chapters 6 and 8.
110
CHAPTER
Orofacial Pain
Joanna Baptist, Ajay Nayak, Ravikiran Ongole 5
➧ Pain Physiology ➧ Myofascial Pain
Common Terminologies Associated with Pain ➧ Neuralgias
Properties of Pain Trigeminal Neuralgia
Pain Pathways Glossopharyngeal Neuralgia
Gate Control Theory Postherpetic Neuralgia
➧ Classification of Orofacial Pain Geniculate Neuralgia
➧ Clinical Assessment of Pain Occipital Neuralgia
➧ Pain from Orodental Structures ➧ Atypical Odontalgia
➧ Barodontalgia ➧ Atypical Facial Pain
➧ Paranasal Sinus-related Pain ➧ Burning Mouth Syndrome
PAIN PHYSIOLOGY 2. Nociception: It is defined as a noxious stimulus or has

potential to turn noxious over a period of time.
The International Association for Study of Pain (IASP) in 3. Allodynia: Pain that is produced by a stimulus that is
1994 defined pain as the subject’s conscious perception of not normally painful.
modulated nociceptive impulses that generate an unpleas- 4. Hyperalgesia: Increased sensitivity to painful stimuli.
ant sensory and emotional experience associated with actual 5. Hypoesthesia: Reduced sensation in response to
damage or described in terms of such damage. stimulus.
The experience of pain is usually a protective mechanism 6. Anesthesia: Absence of sensation in response to a
of the body. On a short-term basis, pain warns the indi- stimulus.
vidual that he or she is in danger so that one can alter the 7. Causalgia: Persistent burning pain caused by deaffer-
situation. For example when a person accidentally touches entation of sensory innervation.
something hot he or she will alter the situation by sponta- 8. Neuralgia: It is the pain that is experienced in the
neously withdrawing from the source of injury. Long-term tissues along the distribution of the nerve.
pain will result in immobilization of the affected part such
that the individual can recover from the injury faster (for Properties of Pain
example, muscle spasm).
Pain at times can be non-beneficial, such as pain associ- Weber and Fechner’s law
ated with cancer, psychogenic pains and neuralgias which Weber and Fechner proposed that gradation of stimulus
only adds to the misery of the patient. However, interestingly strength is discriminated approximately in proportion to
these pains help the physician in diagnosis. the logarithm of stimulus strength.
This law can be mathematically expressed as
Common Terminologies Associated with Pain R ⫽ ␣ log S
1. Nociceptors: These are receptors that are sensitive to where R ⫽ intensity of the reaction (i.e. the pain perceived),
painful stimulus and are responsible for initiating the ␣ ⫽ constant and
generation of pain. S ⫽ the intensity of the stimulus.
111
According to this law, exponential increase in the 1. A␦ fibers (wrapped in Schwann cells)
intensity of stimulus does not cause exponential increase 2. C fibers
in pain perceived. Though the pain perceived increases
with increase in stimulus; the pain experienced is only in A␦ fiber C fiber
terms of log of the intensity of the stimulus. 1–6 ␮m diameter 1.5 ␮m diameter
For example, if a pin prick (1 unit of tissue damage) of Myelinated Non-myelinated
1 unit of intensity of stimulus results in 1 unit of pain per- 5–30 m/s conduction velocity 0.5–2 m/s conduction velocity
ception; a musculocutaneous laceration (100 units of tissue
Carries the first pain that is Carries steady dull pain
damage) causing 100 units of intensity of stimulus results in
experienced—sharp
2 units of pain perception and not 100 units. Similarly, a
crushing injury (1,000 units of tissue damage) causing
1,000 units of intensity of stimulus results in 3 units of Stimulation of nociceptors
pain perception and not 1,000 units.
This law ensures that an individual would perceive pain Mechanical, chemical and thermal stimuli excite pain
of all intensities but at the same time will not cause sig- receptors. Fast pain is conducted by A␦ fibers which is
nificant morbidity due to pain. elicited by the mechanical and thermal stimuli. The slow
dull pain is conducted by the C fibers which are elicited by
all three types of stimuli. It is almost always caused by
Adaptation
release of chemicals liberated by the injured tissue. These
As long as nociceptors (pain receptors) are stimulated, pain are endogenous chemicals called algogenic (pain produc-
continues to be perceived as these receptors have very little ing) substances. Algogenic substances stimulate nocicep-
or no property of adaptation. It is interesting to note that tors to produce pain.
the inability to adapt to pain is beneficial in terms that a per- These chemicals are pain producing peptides, bradyki-
son is appraised of the injurious stimulus that causes pain as nins, serotonin (5 HT), potassium ions, prostaglandins,
long as it persists. acetylcholine and proteolytic enzymes.
For example, olfactory receptors get adapted to a It is interesting to note that commonly used NSAIDs sup-
particular smell when stimulated for a long period of time presses pain by inhibiting prostaglandin synthesis. These
thereby the smell is not perceived any longer. prostaglandins by themselves cannot excite the nociceptors,
Though pain is not adaptable, the brain itself along with i.e. prostaglandins are not algogenic but they enhance the
the spinal cord can suppress the input of pain signals to the sensitivity of the nociceptors toward the algogenic power of
nervous system by activating a pain control system called bradykinins and other chemical mediators of pain.
‘analgesia system’.
Sequelae of Pain
Pain localization
Apart from nociception there are various other sequelae of
Pain is poorly localized. It is said that superficial somatic
pain that alter other systems of the body. Pain affects an
pain is more localized than deep visceral pain. However
individual psychologically as well as physically.
this is not exactly true as the superficial injury not only
excites the nociceptors but also the tactile receptors, thus
Psychological sequelae
helping in localizing the pain.
If the superficial nociceptors were alone to be stimulated, An uninhibited individual would react to acute pain by
it would still cause quite poorly localized pain. mourning and crying as seen in young children and ani-
mals. However, most individuals develop frustration, men-
tal irritation or depression in response to long standing
Nociceptors pain. A patient with long standing atypical facial pain will
most often come to the physician with a frowning face
Pain receptors are called nociceptors (from Latin, nocere—to
and would often get irritated with prolonged history taking.
hurt). All nociceptors are free nerve endings (however
Thus such patients should be treated both by medicines
other cutaneous receptors when stimulated excessively
and psychological counseling.
can result in pain). Either present in skin or any other tis-
sues. They are more concentrated in superficial layers of
Muscular sequelae
skin, periosteum, arterial walls, joint surfaces, the falx and
the tentorium of the cranial vault. Deep tissues are sparsely Injury or disease causing pain results in spasm of skeletal
supplied with nociceptors. muscle in the vicinity of the affected region. This is protec-
Two general types of nociceptors are characterized by the tive as it immobilizes the affected region and thereby puts
neurons associated with them. it to forcible rest which is most essential for rapid healing.
112
Chapter 5 – Orofacial Pain
But this spasm also causes ischemia of the muscles which

Figure 1
aggravates muscular pain.
Patients with internal derangement of temporomandibular
joint (TMJ) usually exhibit spasm of the masticatory mus-
cles of the affected side thereby preventing further damage
to the affected TMJ. In such conditions apart from treating
the affected site of injury, muscular spasms should be man- Thalamus
aged with physiotherapy, muscle relaxants, massage, etc.
Neospino- Hypothalamus
thalamic
Autonomic nervous system sequelae Reticular
tract
formation
Patients with somatic pain generally present with increased
blood pressure, pupillary dilatation and tachycardia which Paleospino-
are all the signs of sympathetic over activity. However vis- thalamic
tract
ceral pain is associated with fall in blood pressure and
Gasserian ganglion
vomiting.
Motor nucleus (v)
Reflex Sensory nucleus (v)

Spinal tract nucleus
Withdrawal from painful stimuli is the reflex action exhib- (v)
ited by the individual. It is an important sequela of pain
that protects the individual from further injury.
For example, biting on a stone during mastication Illustration showing the pain pathway
would immediately initiate a reflex action to keep the
mouth open until an individual realizes the presence of the
injurious agent (stone). Such reflex action would protect
further injury to the periodontium. of the neospinothalamic tract. These fibers cross immedi-
ately to the opposite side through the anterior commissure
Dual Pain Pathways and then travel upward in the anterolateral column of the
spinal cord.
Peripheral pain fibers are of two types, the fast conducting Few of these second order neurons terminate in the
(A␦ fibers) and slow (C fibers) conducting. The fast pain is reticular areas of the brain stem (the reticular areas when
felt within 0.1 second of the application of the noxious stim- stimulated causes excessive alertness and increases an
ulus. But it takes 1 second or more for slow pain to begin. individual’s sense perception), while most of the others
Occasionally slow pain may take over a few minutes to travel up to the thalamus terminating in the ventrobasal
begin after application of the noxious stimulus. complex along with the dorsal column—medial lemniscal
Though both fast and slow conducting fibers have free tract. The remaining second order neurons terminate in
nerve endings as their receptors, they travel via two distinct the posterior nuclear group of the thalamus.
pathways for transmitting pain signals to the central nervous From these areas third order neurons relay signals to other
system. Because of this dual pain pathway a single painful basal areas of the brain and to the somatic sensory cortex.
stimulus would often give a first sharp electrical pain that is
generally followed by a slow dull pain. This ‘double’ pain sen- Paleospinothalamic tract (for slow pain)
sation initially would cause an individual to react immedi-
This pathway transmits pain, which is carried via the
ately to safeguard himself/herself. Whereas the slow pain
peripheral slow conducting C pain fibers (it also transmits
tends to become increasingly painful over a period of time.
very few A␦ fibers). These peripheral fibers terminate in
Once these fibers (A␦ and C) enter the spinal cord, the
the laminas I and II (together called substantia gelatinosa)
pain signals take two pathways to the brain. They are the
of the dorsal horns. Most of the signals then pass through
neospinothalamic tract and the paleospinothalamic tract
one or more additional short fiber neurons within the dorsal
(Figure 1).
horns themselves before entering laminas V through VIII,
also in the dorsal horn.
Neospinothalamic tract (for fast pain)
The next series of neurons gives rise to long axons that
Sensations of mechanical and acute thermal pain are mostly join the fibers from the neospinothalamic tract,
brought to the spinal cord by the first order neurons. They passing first through the anterior commissure to the oppo-
terminate in the lamina I (lamina marginalis) in the dorsal site side of the spinal cord and then upward to the brain in
horns where they synapse to excite the second order neurons the same pathway.
113
Analgesia system or pain inhibitory pathway Figure 2

The analgesia system comprises three major components, Dorsal horn
namely,
Spinal
1. The periaqueductal gray and periventricular areas of cord
the mesencephalon and upper pons
2. Raphe magnus nucleus and nucleus reticularis paragi- Aα or Aβ fiber
(Non-nociceptive)
gantocellularis
3. Pain inhibitory complex located in the dorsal horns of C fiber
(nociceptive)
spinal cord
+
The electrical stimulation in the periaqueductal gray area or − Interneuron
in the raphe magnus nucleus can almost completely suppress + −
Projection
many strong pain signals entering the dorsal spinal roots. + neuron
Stimulation of areas at still higher levels in the brain that in To spinothalamic tract
turn excite the periaqueductal gray can also suppress pain.
The nerve fibers originating from periaqueductal gray
and periventricular nuclei secrete enkephalin at their termi- Illustration showing gate control theory
nals. These fibers synapse with the fibers originating from
the raphe magnus nucleus.
The fibers originating from the raphe magnus nucleus Though it activates the projection neuron simultaneous
secrete serotonin at their nerve terminals. Finally these fibers activation of interneuron causes inhibition of projection neu-
terminate at the dorsal horns of the spinal cord. Serotonin ron thus resulting in lack of pain sensation.
(secreted by the fibers originating from the raphe magnus When C fibers are activated they again cause two effects:
nucleus) causes the cord neurons to secrete enkephalins. (i) inhibition of interneuron and (ii) activation of projection
Enkephalin causes presynaptic inhibition and post syn- neuron, therefore resulting in pain perception.
aptic inhibition of A␦ and C pain fibers at the site of synapse When both non-nociceptive mechanoreceptors and
of the dorsal horns. This inhibition is caused by the blocking nociceptors are simultaneously activated, the mechanore-
of calcium channels of nerve membrane at their terminals. ceptors being myelinated fast conducting fibers, the sig-
nals from these fibers reach interneuron and projection
neuron first before C fibers can conduct signals. In these
Gate Control Theory instances the A␣ and A␤ fibers stimulate the interneuron
This theory explaining pain modulation was proposed by before C fibers can inhibit the same. Thus the net effect is
Ronald Melzack and Patrick David Wall in 1965. no pain.
They hypothesized that pain perception was not just solely This would explain the benefits of physiotherapy and mas-
due to activation of nociceptors but due to interaction between sage in the effective management of painful conditions.
pain conducting and non-pain conducting neurons. Non- It is interesting to note that free nerve endings of unmyelin-
pain conducting nerve fibers interfere with the pain conduc- ated nerve fibers when relatively mildly stimulated produce
tion thus altering pain perception. itch and tickle.
There have been various modifications of the original Unlike pain, itching occurs in superficial tissues and not in
gate control theory. A revised version of the gate control deep structures like viscera.
hypothesis proposes that the projection neuron of the spi- It is common knowledge that scratching relieves itching
nothalamic pathway when activated results in sensation as the fast conducting mechanoceptive fibers activated by
of pain (Figure 2). scratching suppress the itching sensation.
The projection neuron synapses with both non-nociceptive Studies indicate that the C fiber system responsible for
mechanoreceptors (A␣ and A␤ fibers) and the nociceptive itching may not be the same responsible for pain. Surpris-
C fibers, hence activated by both. The interneuron is spon- ingly tickling sensation in general is regarded as pleasur-
taneously active and this activation of interneuron causes able whereas itching and pain are regarded as unpleasant
inhibition of projection neuron; thus inhibiting pain per- sensations.
ception.
In turn, the interneuron synapses with the projection neu-
Concept of Referred Pain
ron, C fibers, A␣ and A␤ fibers. When the non-nociceptive
mechanoreceptors (myelinated and fast conducting A␣ and Referred pain is a spontaneous heterotopic pain that is felt
A␤ fibers) are stimulated they cause two effects: (i) activation in an area innervated by a different nerve from the one that
of interneuron and (ii) activation of projection neuron. mediates the primary pain.
114
Characteristics of referred pain Bell (1989) has classified orofacial pain as follows:
1. Referred pain usually occurs within a single nerve root, Axis I (Physical conditions)
passing from one branch to the other.
2. Referred pain in the trigeminal area rarely crosses the 1. Somatic pain
midline unless it originates at the midline. – Superficial somatic pain (cutaneous, mucogin-
3. Usually if the referred pain is felt outside the nerve that gival)
mediates the pain, it is generally felt cephalad to the – Deep somatic pain
nerve (upward and toward the head) and not caudally. – Musculoskeletal pain (muscle, TMJ, osseous and
However in severe pain the excitatory effects are felt periosteal, soft connective tissue, periodontal)
caudal to the site of initiating input. – Visceral pain (pulpal, vascular, neurovascular,
visceral mucosal, glandular, ocular and auric-
ular)
2. Neuropathic pain
CLASSIFICATION OF OROFACIAL PAIN
– Episodic (trigeminal, glossopharyngeal, genicu-
late, nervous intermedius neuralgias and neu-
The American Academy of Orofacial Pain has classified
rovascular pains)
orofacial pain as follows:
– Continuous (neuritis, deafferentation pain and
sympathetically maintained pain)
1. Intracranial structures Axis II (Psychologic conditions)
– Neoplasm
– Aneurysm 1. Mood disorders
– Hematoma 2. Anxiety disorders
– Hemorrhage 3. Somatoform disorders
– Abscess 4. Other conditions
– Edema
2. Extracranial structures
– Teeth Types of Pain
– Ears
– Eyes Acute pain
– Nose It is generally a physiologic response to an injury. It per-
– Throat sists as long as the noxious stimulus is present. Acute pain
– Sinuses almost always subsides within the time period required for
– Tongue the process of normal healing.
– Glands
3. Musculoskeletal disorders Chronic pain
– TMJ disorders
– Masticatory muscle disorders Merskey and Bogduk (1994) described chronic pain as a
– Fibromyalgia persistent pain that is not amenable, as a rule, to treatments
– Cervical disorders based on specific remedies, or to the routine methods of pain
– Generalized polyarthritides control such as non-narcotic analgesics.
4. Neurovascular disorders In common parlance chronic pain is regarded as pain
– Migraine headaches that persists way beyond the normal time required for the
– Cluster headaches process of normal healing. Pain is said to be chronic in nature
– Tension type headaches when it lasts over 3 months. It is generally associated/
– Cranial arteritis influenced by psychological, emotional, social and cultural
5. Neurologic disorders factors.
– Paroxysmal neuralgias
• Trigeminal neuralgia
• Glossopharyngeal neuralgia
CLINICAL ASSESSMENT OF PAIN
– Continuous neuralgias
• Atypical odontalgia
The diagnosis and management of orofacial pain begins
• Traumatic neuroma
with a comprehensive patient history. The most expedi-
• Neuritis
tious way to obtain such a history is via a detailed patient
• Postherpetic neuralgia
questionnaire and detailed patient–physician interview.
115
Figure 3 Figure 4
None Mild Moderate Severe

(0) (1) (2) (3)
Throbbing
Shooting
Stabbing
Sharp
Cramping
Gnawing
Hot–burning
Aching
Heavy
Tender
Splitting
Tiring–exhausting
Sickening
Punishing–cruel
McGill pain questionnaire (short version)

Illustration showing the pain diagram. Patient is
encouraged to mark areas on the picture that
represent the painful sites in the patient
Figure 5
The interview is followed by a complete oral and head 0 10
and neck examination, appropriate chair side investigations,
radiographic imaging and lab studies. No Worst
Assessment of pain can be achieved by certain subjective pain possible
and objective methods. Some widely accepted subjective pain
methods include the use of McGill Pain Questionnaire (long/
short), visual analog pain scale (VAS), brief pain inventory. Visual analog scale
The faces pain scale and the pain diagram.
During the patient interview the clinician should obtain
the following data: mode of onset, duration, location of the intensity can be categorized as mild (score 1–3), moderate
pain, quality or character of the pain, intensity of the pain, (score 4–6) and severe for scores 7–10 (Figure 5).
frequency of the painful episodes, aggravating and reliev- However the ‘Faces Pain Scale’ can be used in child patients
ing factors if any, radiation or referral patterns, any other for assessing the intensity of pain (Figure 6).
associated symptoms and history of any medical consulta- Following the history, physical examination should be
tions/use of medications for the same. undertaken. A thorough physical examination of the head
The location of pain and referral patterns can be identified and neck, including the TMJ, maxillary sinus, masticatory
by the patient using a pain diagram (Figure 3). muscles along with the accessory muscles, salivary glands
The pain quality or character can be expressed using and the oral cavity should be performed.
the widely accepted McGill Pain Questionnaire (Figure 4). Intraoral examination should include the evaluation of
This questionnaire will help the patient to describe how teeth, periodontium and the oral mucosa.
exactly he/she feels about the pain by selecting an appropri-
ate adjective from a list in the questionnaire.
The intensity of pain can be quantified using the visual PAIN FROM ORODENTAL STRUCTURES
analog scale (VAS). The VAS is a 10 cm long line with 0
marked on one end (represents no pain) and 10 at the other
Pulpal Pain
end (represents worst possible pain). The linear scale has
markings from 0 to 10 at 1 cm intervals. The patient is Dental pain is the single-most common cause why a patient
encouraged to mark a point along this scale that correlates visits a dental clinician, a fact that will be vouched for by any
with the intensity of pain experienced. For convenience pain practicing clinician. Pulpal pain forms a major component of
116
Figure 6
0 2 4 6 8 10
Faces pain scale. Score the chosen face 0, 2, 4, 6, 8 or 10, counting left to right, so ‘0’ = ‘no pain’ and ‘10’ = ‘very much pain’.
Do not use words like ‘happy’ and ‘sad’. This scale is intended to measure how children feel inside, not how their face looks.
From PAIN, 2001, 93, 173–183 ‘The Faces Pain Scale–Revised: toward a Common Metric in Pediatric Pain Measurement’,
by CL Hicks, CL von Baeyer, PA Spafford, I van Korlaar and B Goodenough. Reprinted with permission
of the International Association for the study of Pain®.
this dental pain and needs to be comprehensively understood situation, while the pulp can be salvaged in the reversible
for making accurate diagnosis and according the right type. As the inflammation is totally confined within the pulp
treatment options. Pulpal pain is classified as a visceral space, no tenderness is elicited on percussion of the tooth.
type of pain and manifests features characteristic to such Pulpal pain rarely remains unchanged for long periods of
a type of pain. The pulp responds to any form of noxious time. It either resolves by itself, a rare scenario, or progresses
stimuli by way of inflammation. Pulpal pain can be to chronicity and/or pulpal necrosis. Once the pulp under-
brought about by a variety of causes, the most common goes necrosis, no painful stimuli are transmitted and leads
being, dental caries, trauma, chemical irritants and even to relief from all symptoms. This may give rise to a false
rarely, anachoresis. feeling of cure to the patient and leads to neglect. During
the process of pulpal necrosis, thermal stimuli may evoke
Clinical features differences in response. Pain may be aggravated by both
heat and cold when both the A␦ and C nerve fibers are
The patient manifesting pulpal pain describes it as an aching
intact. As the condition progresses, heat will cause the
sensation that is not localizable to the patient. The patient
pain to occur while cold may actually relieve it.
will point out to a region of the jaw and mention about the
pain, but when asked to specifically point out the tooth, will
Diagnosis
be unable to do so. The absence of proprioception in the
pulp leads to such a scenario. On examination of the Pulpal pain is of prime suspect when there is poor local-
region, some clinical finding of deep caries, pulpal involve- ization of the pain by the patient. At times, the patient
ment by caries or fracture of tooth, or deep cervical abra- may not even be able to differentiate if the origin is max-
sion may be seen. The underlying inflammation of the pulp illary or mandibular jaw. Definite clinical lesions like den-
shows typical sign of pain on movement of the part, espe- tal caries, faulty restorations, fractured teeth or others may
cially since the pulp is tightly enclosed in a chamber of point to the source. Thermal, mechanical, chemical and
dentin. This is evidenced by the history of postural changes electric stimulation of a suspected tooth may provide the
in pain that may be reported by the patient. The pain is noxious stimulation and induce the pain that the patient
usually intermittent and aggravated by contact of the can then identify. Vitality tests are not always reliable,
tooth with sweets, cold and/or hot food stuff. The sweets because they indicate the nervous response rather than the
cause a change in the fluid dynamics within dentin due to intactness of the vascular supply and the presence of healthy
their hygroscopic nature and the thermal changes get pulp in one root canal and necrotic pulp in another can
mediated via the A␦ and C nerve fibers, leading to a cross- provide conflicting interpretations. Sequential analgesic
ing beyond the pain threshold of the pulpal receptors. blocking can usually definitively identify the source tooth.
Reversible pulpitis gives a typical finding of pain occur- However if the pain is found to occur in a particular region
rence on application of the noxious stimuli that characteris- of the jaw, but exact localization is not possible clinically,
tically disappears immediately on withdrawal of the then waiting for the natural pain localization to occur by
stimulus. Irreversible pulpitis, on the other hand, shows extension into the periodontal ligament may be another
persistence of pain for some time, even after removal of the option. In rare situations, a direct exploration of the tooth
noxious stimulus. This differentiation is quite necessary as may be justified in severely painful conditions wherein
the treatment options for both vary considerably, as the pulp the patient is unwilling to wait for natural localization of
and/or the tooth need to be sacrificed in the irreversible the pain. This would seem better than a radical option of
117
extracting the tooth. Radiographic examination will not without a breach of lamina dura and altered trabecular pat-
yield any direct characteristic signs of pulpal inflamma- tern are a definitive indication of periodontal pathology.
tion. However extension of any pathology into the pulp,
such as caries, restorations, etc. may be seen as a possible Dental pain Dental hypersensitivity
causative agent and help in diagnosis. Caused due to noxious Caused by normal physiologic
stimulation of the nerve endings stimulation of nerve endings
Periodontal Pain May be intermittent or Usually always transient

continuous, depending upon the
Pulpal pathology leading to necrosis of the pulp after a while stage of pathology
spills into the periodontium via the apical foramen and/or May manifest at any age from Usually seen in the elderly
the accessory foramina. The discussion here pertains to the youngsters to elderly population
apical portion of the periodontium via the pulp being Not associated with any particular Parafunctional oral habits play a
affected rather than the progressive effects of the gingival habits role in its occurrence
or periodontal diseases. The periodontal ligament behaves May or may not respond to cold Always occurs in response to a
and responds to noxious stimulation in a manner similar test cold test
to other ligaments of the body. Therefore periodontal pain
is felt as a deep somatic pain of the musculoskeletal type.
The receptors of the periodontal ligament can precisely locate
stimulus due to the property of proprioception. The causes BARODONTALGIA
of pain originating in the periodontal ligament are as varied
for pulpal pain. Inflammatory reaction, trauma, endodontic Barodontalgia is the pain or injury associated with teeth as a
procedures, improperly contoured restorations, faulty occlu- result of alteration in the pressure gradients. Historically, the
sal contacts, orthodontic appliances and surgical proce- term aerodontalgia was used to describe pain experienced by
dures are the most common. Parafunctional oral habits air crew in flight. Over a period of time it was noticed that
may also be a contributing factor. deep sea divers also suffered tooth pain due to pressure
changes, prompting the use of the term barodontalgia.
Clinical features Barodontalgia can be explained by Boyle’s law, which
states that ‘at a given temperature, the volume of a gas is
The periodontal ligament pain is described by patients as inversely proportional to the ambient pressure’. As an indi-
sharp jabs or pricking type of pain. The pain is usually vidual descends deeper and deeper below the water surface,
intermittent in nature and occurs whenever there is load- pressure exerted by water on the diver increases and
ing of the ligament, such as during chewing. The func- reduces the volume of gases in enclosed spaces such as
tional action causes movement of the tooth that compresses teeth and the paranasal sinuses.
the ligament, leading to displacement of the edema fluid Similarly during a high altitude flight the atmospheric
and irritation of the free nerve endings causing pain. This pressure outside the aircraft decreases thereby permitting the
feature can be observed clinically by percussing the sus- volume of gases to increase. These changes in pressure affect
pect tooth, which simulates the functional action. Any air crew and passengers of a non-pressurized aircraft.
tenderness if present during percussion indicates peri- Barodontalgia arises when gases that are confined within
odontal ligament involvement. The patient will usually closed spaces are unable to contract to adjust the internal
precisely identify the tooth and mention it to be sore or a pressure to correspond to the outside pressure. The phe-
feeling of elongation being present. Clinically the tooth nomenon begins to occur at an altitude of approximately
may be seen to be slightly out of occlusion. There may be 3,000 m and at a water depth of 10 m where the ambient
associated tenderness in the vestibule and at times a frank pressures are 0.75 and 1 atm, respectively.
swelling may be present in the gingival region or in the Individuals may experience a simple sharp or squeezing
vestibule. tooth pain. Occasionally the pressure change may cause the
rupture of the alveolar mucosa.
Diagnosis
Strohaver (1972) categorized barodontalgia into direct
The exact location being pointed out by the patient is the and indirect types. In the direct type, reduced atmospheric
first clue in suspecting periodontal ligament involvement. pressure contributes to a direct effect on a given tooth.
Clinical test of percussion along with presence of any of the However in the indirect type, dental pain is secondary to
contributing factors further helps in diagnosis. Vitality test- stimulation of the superior alveolar nerves by a maxillary
ing can be of help if the cause is related to pulpal pathology, barosinusitis.
because a necrotic or dead pulp definitely favors a diagnosis Direct barodontalgia is generally manifested by moder-
of apical periodontal pathology of some degree. Radiographic ate to severe pain, which usually develops during ascent, is
findings of a widening of the periodontal space with or well localized, and the patient can frequently identify the
118
involved tooth; indirect barodontalgia is a dull, poorly defined procedures such as pulpectomy and capping of an exposed
pain that generally involves the posterior maxillary teeth and pulp. In these individuals endodontic treatment is recom-
develops during descent. mended.
Etiology
Kollmann (1933) proposed three hypotheses to explain the PARANASAL SINUS-RELATED PAIN
occurrence of barodontalgia: expansion of trapped air
bubbles under a root filling or against dentin that activates The paranasal sinuses are placed in close proximity to the
nociceptors; stimulation of nociceptors in the maxillary oral cavity, especially the maxillary sinus and have some
sinuses, with pain referred to the teeth; and stimulation of common innervations. This can cause pathologies in and
nerve endings in a chronically inflamed pulp. about the sinus to be referred to the dental structures and vice
In most cases of barodontalgia the individual’s tooth is versa. Therefore a careful consideration of their close rela-
already affected by some sort of pathology such as acute or tionship must be made in chronic painful conditions. Primary
chronic periapical infection, caries, deep restorations, resid- pain that occurs at the opening of the sinuses into the
ual dental cysts, sinusitis and a history of recent surgery. nasal cavity has secondary reference zones in the maxillary
The term barosinusitis has been used in literature to posterior teeth of the same side and some areas of the face.
describe pain in the tooth caused by congestion of the It should however be noted that the sinus lining mucosa is
maxillary sinus and most often the pain is experienced actually insensitive to pain, but the junction of the sinus
during descent. Whereas barodontalgia is usually experi- lining with the nasal mucosa is richly innervated and when
enced during ascent and the tooth is affected usually by stimulated causes intense pain. Hence any exudate present
periapical pathology. within the sinus causes a feeling of fullness or pressure in
that sinus rather than pain or headache. When the patient
bends forward, the exudates can wash over the sensitive
Underwater Diving ostium causing pain that may radiate to the maxillary teeth.
Many a times, sepsis from the maxillary teeth may in itself
During underwater diving the air from the pressurized tanks
be a cause for the sinus inflammation. If the teeth are sec-
may be forced into the tooth via the carious lesions or defec-
ondarily involved by extension of prior sinus disease, the
tive margins of restorations. With the fall in atmospheric
dental pain will be of the periodontal type due to the effect
pressure during ascent, trapped gases may expand and enter
on the periodontal ligament with almost no features of
dentin tubules, thereby stimulating nociceptors in the pulp
pulpal pain.
or causing the movement of pulp chamber contents through
Pain of maxillary sinus origin will be usually felt as a
the apex of the tooth, resulting in pain.
constant, dull non-pulsatile ache in the maxillary teeth of
Calder and Ramsey (1983) suggest that the physical
the affected side and sometimes on the face. There may be
properties of the gas mixture used during deep sea diving
accompanying ear pain, malaise, nasal congestion and nasal
may contribute to barodontalgia. In scuba tanks, oxygen’s
discharge. Inflammation of the sinus will usually cause
natural diluent gas, nitrogen, is replaced by helium, result-
radiographic findings of haziness or cloudiness of the sinus
ing in a gas of lower viscosity. This gas can enter tissues,
and/or hyperplasia of the sinus lining.
including teeth, and can sometimes become trapped in
closed spaces, such as the pulp chamber and root canal.
There are two mechanisms by which gases can be trapped
in spaces: if there is a space between a tooth and its resto- MYOFASCIAL PAIN
ration, gas may be forced into it during an increase in
pressure; and dissolved gas may diffuse from tissues into Pains of muscular origin are one of the frequent sources of
spaces as pressure decreases. chronic pain anywhere in the body and the head and neck
Occasionally, the trapped gas will expand and the result- region is not bereft from it. The pain originating from the
ing stress may cause tooth fracture. This process has been skeletal muscles, tendons and fascia surrounding these con-
called odontecrexis (Greek word meaning ‘tooth explosion’). stitute the term myofascial pain (MFP). The intimate rela-
The Fédération Dentaire Internationale (FDI) recom- tion of the muscles in and around the oral cavity, both
mends an annual check-up for divers and pilots, with oral anatomically and functionally accords the orofacial muscu-
hygiene instructions. Also it recommends that the patients lature an important consideration in the differential diag-
should not dive or fly in non-pressurized cabins within nosis of chronic orofacial pain. Pathology originating in the
24 hours of a dental treatment requiring anesthetic or for dental structures can cause effects manifested in the muscu-
a week following a surgical treatment. lature. The group of muscles involved, their number and their
Some authors suggest that when treating people who relative location determine the severity of pain felt by the
are subjected to large pressure changes it is best to avoid patient. According to Bell (1989), ‘it is a good rule to follow
119
in diagnosing pains about the face and mouth is initially to stretching exercises and analgesic injection into the trigger
assume that the pain is dental until proven otherwise, then points are most commonly employed. The details of all
muscular until proven otherwise’. modalities used in management of MFP are beyond of the
Myofascial pain is a regional muscle pain disorder that scope of discussion of this book.
manifests characteristic local areas of hypersensitive
bands of muscle tissue known as ‘trigger points’. The exact
nature of a trigger point is not known, but it has been
hypothesized that certain pain inducing events sensitize
NEURALGIAS
the free nerve endings in the muscles that cause the occur-
rence of the hypersensitive areas. Some investigators Neuralgia is a clinical condition involving a pain of a severe
believe that the individual muscle spindles manifesting the intensity, with a throbbing or stabbing character in the course
trigger point are innervated by the sympathetic nervous or distribution of a specific nerve.
system which may be the cause of muscle pain in emo-
tional situations. The trigger point has an ability to cause
referred pain in a definite anatomical area when stimulated. Trigeminal Neuralgia
Each trigger point is thought to be less than 1–2 mm in Trigeminal neuralgia (TN) is frequently encountered neu-
diameter. The complete range of etiologic factors for MFP ropathy in dental practice affecting the fifth cranial nerve.
is difficult to determine, but both local and systemic causes Though it has been known by various names in the litera-
have been consistently identified in most patients, such as ture such as tic douloureux, trifacial neuralgia, Fothergill’s
trauma, emotional stress, fatigue, hypovitaminosis and disease, the currently accepted terminology is ‘trigeminal
infections especially of the upper respiratory tract. neuralgia’. It is a well-recognized disorder characterized by
a severe, paroxysmal burst of pain in one or more branches of
Clinical features the trigeminal nerve often induced by touching trigger points,
A patient manifesting MFP will complain of increased in or about the oral cavity. TN is diagnosed primarily on
fatigue, stiffness and pain in a particular group of muscles. the basis of the history of characteristically unilateral pain
The patient may describe a feeling of subjective weakness attacks that are consistent with specific clinical criteria for
and restriction in the normal range of muscle motion. The the diagnosis. However the typical sequence of events that
pain typically increases as the day progresses and is par- occur in a patient manifesting TN begins with the initial
ticularly more following a stressful day. The clinical pres- symptoms of pain that may be misdiagnosed as pain of
ence of the characteristic trigger point is the hallmark of odontogenic origin. A careful history-taking is of paramount
this condition. When such a trigger point is detected and importance stressing upon the onset (whether insidious or
palpated the patient gives a typical behavioral reaction, sudden), the duration and frequency of painful episodes, and
acknowledging the tenderness felt in the area of pain ref- other associated symptoms.
erence, known as the ‘jump sign’. When the painful muscle The exact cause of TN pain attacks is not known in
is stretched it is found to be restricted in motion and the spite of accurate description of the condition over the
patient may adopt a faulty posture to obtain relief from years by several researchers. However, an interesting find-
the pain. In some situations, multiple trigger points may ing, that some benign tumors in the brain and/or vascular
induce referred pain in the same region or a single trigger anomalies leading to compression and possible demyelin-
point may cause radiating pain in two or more muscles. If ation of the trigeminal nerve root at the entry into the
the muscle soreness persists for a long time, a cyclic pain- pons, produce symptoms clinically characteristic of classic
spasm-pain pattern may form. Therefore accurately locat- TN. This finding strongly implies that injury to the nerve
ing the source of pain, the trigger point, and the zone of root may be an important initiating factor. When such a
reference is equally important. There may be accompanying pathologic component is present, it is known as symptom-
tingling or numbness of the associated area. Patient may atic TN, but in the absence of any such causative factor,
complain of pain in the TMJ region, the ear, scalp or the the condition is known as idiopathic TN. Oral causes, such
neck. Rarely in some patients, autonomic changes may as periodontal lesions and traumatic lesions have been
be present, such as pallor, sweating, flushing, lacrimation, long discarded.
nasal congestion, increased salivation and nausea.
Clinical features
Management
Trigeminal neuralgia has most often a sudden onset and
Management of MFP depends upon the identification of progresses to become a chronic condition. Although no
the trigger point and the referred zone. Conservative mortality is associated with the condition, the quality of
methods based upon physiotherapy principles should be life of the sufferer is severely affected. TN does not show
applied that can be supplemented with drugs. Basic muscle any specific racial predilection and is known to occur
120
throughout the world in more or less the same frequency 5. The clinical neurologic (sensory) examination is
ranges irrespective of the ethnicity, geographic location or normal.
climatic conditions. It manifests in both males and females,
The IHS has defined classic TN as ‘a unilateral disorder char-
with a slight female preponderance (2:3 males to females).
acterized by brief electric shock-like pains, abrupt in onset
The condition usually occurs above the age of 50 years,
and termination, limited to the distribution of one or more
mostly in the 60–70 years range. When this condition
divisions of the trigeminal nerve. Pain is commonly evoked
manifests in adolescents or young adults, a demyelinating
by trivial stimuli including washing, shaving, smoking,
lesion in the pons should be ruled out as that is one of the
talking and/or brushing the teeth (trigger factors) and fre-
most common occurrences.
quently occurs spontaneously. Small areas in the nasola-
The patient gives a typical history of paroxysms of lan-
bial fold and/or chin may be particularly susceptible to the
cinating painful episodes involving the face that are strik-
precipitation of pain (trigger areas). The pains usually remit
ingly unilateral. The pain may be described like being
for variable periods’. This definition covers most essential
stabbed by a knife or an electric shock-like kind of pain.
clinical presentations of TN.
The patient is usually able to precisely point out the area
affected by the pain and charts out an area supplied by the
branches of the trigeminal nerve on the face. The most
commonly affected divisions are the maxillary and man- There are few other conditions that can mimic such a char-
dibular divisions, either singly or in combination. The oph- acteristic condition. However, in some cases, especially in
thalmic division may also be involved, though with less the initial stages, may need other conditions to be ruled out.
frequency. There has been an unexplained finding of pre- Chief among these are odontogenic causes, TMJ-related dis-
dominant right side involvement in most studies. The orders, atypical facial pain, glossopharyngeal neuralgia,
attacks in each patient occur with same intensity and same multiple sclerosis.
areas of the face, brought about by routine activities such The specific age group and sex distribution usually helps
as chewing, smiling, talking, etc. The patient may point differentiate TN from other causes like atypical facial pain
out to an exact location on the skin, called as trigger point, and TMDs that usually begin at a much younger age. A valu-
the stimulation of which causes the occurrence of an attack. able clue to the diagnosis of TN is the occurrence of the
These trigger points are located most commonly around the pain attack with certain activities, such as rubbing the face
mouth, ala of the nose and periorbital or molar regions. or shaving a trigger area, whereas in other facial pain syn-
Majority of the patients complain of lancinating pain dromes, they massage the face or apply heat or ice, which
shooting from the corner of the mouth to the angle of the is strikingly missing out in the history reported by TN
jaw. There may also be a history of jolts of pain from the patients.
upper lip or canine teeth to the eye and eyebrow, without
actual involvement of the orbit itself. The painful episode Investigations
is usually brief lasting from a few seconds up to 2 minutes.
Usually all laboratory values are seen to be within normal
Though the episode may be brief, some patients can experi-
limits. Any kind of electrophysiological testing in the
ence volleys of multiple attacks within a short span of
affected area of the face also usually does not reveal any
time. When an attack might get precipitated in the dental
profound abnormalities. However some clinicians insist
office, the patient is seen clutching the face, especially the
on an elective MRI scan of the brain to detect any missed
region affected and show an expression of deathly pain
out clues, intracranially, and only then proceed with the
(indicates the severity). Several patients, in the past, have
treatment.
been reported to have suicidal tendencies to rid themselves
of the pain. The severe pain as it fades away may give rise
Treatment
to a burning ache that may last for a prolonged time after
the attack. Given the older age manifestation of TN, medical therapy
White and Sweet (1955) described a set of criteria for is usually better tolerated than any form of surgical interven-
diagnosing TN popularly known as ‘Sweet criteria’ and tion, which can be attempted only if and when the pharma-
currently accepted by the International Association for the cologic therapy fails.
Study of Pain (IASP) and the International Headache Two broad groups of pharmacologic agents, anticonvul-
Society (IHS): sants and skeletal muscle relaxants, are usually employed
in the management of TN. Among the anticonvulsants,
1. The pain is paroxysmal and sudden in nature. carbamazepine is the drug of choice for TN. A 100-mg
2. Light touch to the face on the trigger zones provokes tablet usually accords substantial relief within 2 hours, and
the pain. therefore it is used as a first choice agent. So predictable
3. The pain is confined to the trigeminal nerve distribution. and powerful is the relief that if the patient does not respond
4. The pain is unilateral in manifestation. at least partially to carbamazepine, it should lead to a
121
reconsideration for the diagnosis of idiopathic TN. If the ninth cranial nerve. Harris (1926) coined the term glossopha-
initial dosage does not relieve the discomfort adequately, ryngeal neuralgia. This condition though less frequently
administer a higher dose by increments of 100 mg (200 mg/ seen in comparison to TN, is nonetheless equally painful
day) up to a maximum dosage of 1,200 mg/day. This drug and causes a great deal of deterioration in the quality of
may cause bone marrow depression, drowsiness, dizziness, life in affected persons. The exact cause of this condition
blurred vision, vertigo, subjective feeling of dryness in the is unknown as yet, with some reports of nerve compression
mouth as a spectrum of its side effects. Therefore monitor- leading to the condition. However, this still remains to be
ing the bone marrow activity by obtaining a complete ascertained as the definitive cause of GPN.
blood count prior to initiating therapy and routinely
thereafter is indicated. The main disadvantage of this drug Clinical features
is that, though very effective, it does not address the etiol-
Glossopharyngeal neuralgia characteristically manifests in
ogy of the neuralgia and needs to be prescribed on a long-
the older age group usually beyond 60 years of age. It does
term basis for symptomatic relief. Baclofen is the skeletal
not exhibit any specific gender predilection. A patient suf-
muscle relaxant employed in treating TN based upon the
fering from GPN will give a history of severe shooting
CNS depressant properties. Baclofen is most often used
pain occurring in episodes. The attacks have no specific
after therapy with carbamazepine has been initiated as the
pattern and may vary in frequency from a few per day to
effects are synergistic with those of carbamazepine permit-
rarely once in few months. The severity too can oscillate
ting a reduction in the dosing of carbamazepine, thereby
from between intolerable to relatively milder attacks. The
reducing any of its adverse effects. Baclofen is introduced
pain is usually localized to areas around the posterior
as 5 mg t.i.d. for 3 days, followed by increase to 10 and
parts of the oral cavity and the pharynx in a typical uni-
20 mg/day every 3 days, up to a maximum dosage of
lateral manifestation. The pain attacks may be initiated by
80 mg/day. Several other drugs have been tried in the
some specific functions like speech, swallowing, yawning
management of refractory TN, but their role has been
or coughing (associated with tongue movements).
unclear and not supported by strong enough evidence to
Clinical examination usually yields no specific informa-
be routinely employed. Another drug, pimozide has also
tion, but careful probing may reveal certain sensitive areas
been used in the doses of 2–12 mg/day in managing TN.
or trigger points, that are usually located in the oropharyn-
This drug is however recommended only for very severe
geal walls or the peritonsillar area (including the soft pal-
and intractable cases given the higher rate of adverse side
ate). Thus palpation of the lateral aspect of the throat can
effects. Drugs like gabapentin, lamotrigine, topiramate
easily provoke an attack confirming the diagnosis. Rarely,
have been used with some success in few clinical trials,
the pain from GPN may be felt in the ear region only, caused
but their usefulness remains yet to be ascertained at this
due to involvement of the tympanic plexus of the ninth
point in time.
cranial nerve. The close proximity of the glossopharyngeal
Over time, the drugs used for the treatment of TN often
nerve to the vagus nerve may cause accompanying vagal
lose effectiveness as patients experience pain in spite of the
manifestations like syncope, arrhythmia and at times,
routine medications. For such patients, in whom medical
even cardiac arrest.
therapy has failed, surgery is a viable and effective option.
There are various surgical options that may be explored as
options by the neurosurgeon, the description of which is
beyond the scope of this book (Flowchart 1). Since the trigger zones of GPN lie around areas of jaw
usage, the clinician may easily confuse it with odontogenic
Prognosis causes, TMJ-related problems or masticatory muscle dis-
orders. Masticatory pain may be easily differentiated by
Trigeminal neuralgia is not a life-threatening condition. avoiding movements of the mandible and allowing free
The disease manifests typically as clusters of attacks that movements of the tongue. GPN will not be prevented as
wax and wane in frequency. Exacerbations most com- tongue movements would precipitate the attack. Another
monly occur in fall and spring seasons. After an initial definitive method is to apply a topical anesthetic on to the
attack, the disorder may remit for months or even years. pharyngeal mucosa that would completely stop the pain
Thereafter the attacks may become more frequent, more impulses arising from the stimulation of trigger zones. Any
easily triggered, disabling, and may require long-term relief by this method would certainly point to a diagnosis
medication. of GPN.
Eagle’s syndrome, associated with an elongated styloid
process compressing the glossopharyngeal nerve may pro-
Glossopharyngeal Neuralgia
duce symptoms similar to those of GPN. A radiographic
Glossopharyngeal neuralgia (GPN) is a neuropathy similar examination of the neck can help rule out calcific enlarge-
to TN, but occurs due to affliction in the distribution of the ment of the styloid process. Also pain on rotation of the head
122
Flowchart 1
Trigeminal neuralgia
New case Refractory cases
Resistance
Carbamazepine Phenytoin sodium 1. Percutaneous radiofrequency
(Mazetol, Tegretol) (Eptoin, Dilantin) thermocoagulation
100 mg b.i.d. 100 mg 2. Microvascular decompression
Increase the dose of Sustained release

carbamazepine up to carbamazepine
600 mg b.i.d./day (Mazetol SR, C-Lep SR)
400 mg b.i.d./day
1. Combination therapy carbamazepine Second line of drugs

(half the last dose) + phenytoin sodium 100 mg (no established efficacy – cochrane)
2. Oxycarbazepine 300 mg (Oxep, Oxetol) b.i.d./day 1. Lamotrigine 50 mg/day (Lyzin)
up to 1,200 mg/day 2. Pimozide 2 mg b.i.d./day
3. Gabapentin 300 mg (Gabantin, Gabapin) 1st day (Mozep, Pimojet)
300 mg, 2nd day 300 mg b.i.d., 3rd day 300 mg t.i.d. 3. Baclofen 25 mg b.i.d./day
and then maintain the dosage. Can be given up to (Spinofen, Lioresal)
2,400 mg/day
Note: All available medications have been enlisted. however, the physician should
choose the appropriate mode of treatment/drug based on the clinical situations
Management of trigeminal neuralgia
to one side (causing impingement of the nerve) is a feature may be performed to detect any intracranial or extracranial
more suggestive of Eagle’s syndrome rather than GPN. tumors or any vascular anomalies.
Another situation that may compound the diagnosis of A panoramic radiograph may be obtained to check for
GPN is its rare but simultaneous occurrence with TN. This the status of the styloid process calcification.
situation may necessitate ruling out any brain lesions
causing these conditions. Again topically anesthetizing the Treatment
pharyngeal mucosa will cause partial relief and the patient
The medical management of GPN is similar to that of TN. The
will point out to the persistent pain in the areas of the tri-
anticonvulsant, carbamazepine, is the drug of choice,
geminal nerve distribution.
according relief to most of the affected persons. Baclofen
When GPN manifests exclusively in the tympanic
also aids in the management by providing a synergistic
plexus, the excruciating ear pain may be confused with
action along with carbamazepine. The dosage schedules
geniculate neuralgia, ear-related or TMJ-related disorders.
used in managing TN are the same when applied to GPN.
Failure of medical therapy necessitates considering surgi-
Investigations
cal options that have been known to provide relief, but
Laboratory tests do not exhibit any abnormality. In case of come with associated risks accompanying any surgical
concurrent GPN and TN, an elective MRI scan of the brain intervention modality.
123
The occurrence of severe pain during swallowing may states leads to manifestation of zoster and consequently
lead to the avoidance of any food and drinks by the patient. PHN. Generally it is not seen in patients below 40 years
This can adversely impact the general health of the patient and the incidence between 40 and 60 years is quite less as
due to a significant lack of nutrition. Also the older age compared to in those patients above 60 years. No specific
manifestation may predispose to more severe effects of mal- gender predilection is noted and it manifests in both male
nutrition in GPN patients. Therefore adequate dietary adjust- and female patients. The patient provides a typical history of
ments need to be made and counseling must be provided unilateral pain or burning sensation that affects a region in
regarding proper diet schedules. a dermatomal pattern. Clinical examination will reveal either
healing or already healed lesions of zoster infection. In rare
Prognosis instances, subclinical reactivation of herpes zoster can occur
(without clinical manifestation of the rashes) known as zos-
Glossopharyngeal neuralgia is not a life-threatening condi- ter sine herpete. If PHN occurs in conjunction with such a
tion. The disease produces episodic pain attacks that vary in situation, the diagnosis may be slightly confusing.
frequency. Some patients may suffer only few attacks with
natural remissions for prolonged periods. However frequent Management
pain in most cases requires long-term medication.
The condition is never fatal, but patients may experience
significant pain for a prolonged period of time thereby
Postherpetic Neuralgia decreasing their quality of life. The main goal of therapy is
to achieve relief from the constant gnawing pain the
The virus herpes virus varicellae or human herpes virus-3 patient experiences. Though most of the times complete
(HHV-3) causes the infections varicella and herpes zoster resolution is not achieved, some degree of reduction can be
in man. The primary infection with this virus leads to the hoped for, sufficient enough for the patient to bear while
clinical manifestation of varicella or chicken pox. Once performing routine daily activities.
the primary infection resolves, the virus enters into the The various drugs that have been employed in manag-
sensory nervous system and can remain latent there for ing PHN are: the tricyclic antidepressants (amitriptyline,
many years. In the head and neck, the trigeminal ganglion nortriptyline), corticosteroids (dexamethasone, prednisone,
is the preferred site that harbors the virus during its and methylprednisolone), and anticonvulsants (gabapen-
latency. Due to some reason, activation of such latent viral tin). The antiviral drugs (famciclovir) that are used to treat
particles causes the secondary infection called herpes the zoster infection have shown to decrease the incidence of
zoster. PHN as compared to patients who have not received antivi-
Spontaneous pain, pain provoked by trivial stimuli, and ral therapy. Topical therapy with capsaicin and lignocaine
altered sensation accompany herpes zoster and which may has been effective in few patients.
continue long after its characteristic rash has healed is Those patients who do not respond to the medications
known as postherpetic neuralgia (PHN). Herpes zoster infec- within the first year are unlikely to have any relief from the
tion by itself is a painful condition and the pain continues pain and are labeled as refractory. These patients need to
up to a month in most patients. But when the lesions have be educated and counseled about the condition.
clinically healed and the pain still persists beyond a period
of 3 months, the diagnosis points to PHN. The exact cause
of PHN is not known, as it does not occur in every patient Nervus Intermedius (Geniculate) Neuralgia
who manifests zoster infection. The sensory alterations
and pain that occur in PHN have been thought to occur as Nervus intermedius is the sensory branch of the VII cranial
a result of both peripheral and central disturbances in the (facial) nerve that supplies the external auditory meatus,
nervous pathways. Many risk factors have been noted in ear auricle and regions around it. The neuralgia occurring
clinical studies that predispose for development of PHN, in this nerve is referred to as nervus intermedius neuralgia
such as advancing age, the site of zoster rashes (a particu- or geniculate neuralgia or VII nerve neuralgia. This condi-
larly higher risk for trigeminal manifestation), a severe tion is very rare as compared with the other neuralgias in
prodromal pain before the zoster manifestation and a very the head and neck region. It may be associated with herpes
severe degree of rashes. zoster infection of the geniculate ganglion (Ramsay Hunt
syndrome).
The patient describes paroxysmal pain occurring in the
Clinical features
external auditory meatus or the walls of auditory canal.
Postherpetic neuralgia usually affects the elderly group of Rarely the pain may be felt only in the tympanic mem-
patients over 60 years. As the age advances, the risk for brane or within the middle ear. The patient may describe
developing PHN increases. The general debilitation of the a feeling of a hot stick inserted within the ear. Rarely the
health and/or any accompanying immunocompromised pain may be felt in the soft palate, tongue or even within
124
the facial musculature. However careful examination will thought, that too has not been validated, has believed that
reveal that the pain occurs when the ear auricle is stimu- some form of trauma has to be involved that may lead to
lated and cause radiation of pain to the structures men- some unexplained changes in either transmission or rec-
tioned before. ognition of the pain impulse. The association of AO with
Management of the condition is similar to trigeminal neu- psychological disorders has led to some belief that the pain
ralgia with the use of anticonvulsants like carbamazepine may be a component of the spectrum of the psychosomatic
in doses of up to 1,200 mg/day. In Ramsay Hunt syndrome disorders.
a short course (2–3 weeks) of high-dose steroid therapy has
been found to be particularly useful. Clinical features
Atypical odontalgia is a very frequently encountered clin-
Occipital Neuralgia ical situation and patients present with symptoms of
toothache. The patient is usually a middle aged lady in her
Paroxysmal sharp pain occurring in the region supplied by early forties but may manifest in significantly older
the greater and lesser occipital nerves constitutes occipital women too. The exact reason for this age and gender pre-
neuralgia. The pain is felt in the posterior region of the skull dilection has not been understood. The patient usually
up to the vertex. Rarely the patient may manifest temporal points out specifically to a single tooth or very rarely a
or at times even retro-orbital pain. Usually this condition group of adjacent teeth. The teeth most commonly affected
arises due to the compression of the nerves, following trauma, are the premolars and molars of the maxillary jaw, AO is
infection or neoplasms. Given the frequency of musculoskel- very rarely reported in the mandibular teeth. On examina-
etal pains occurring in the same region the clinician needs tion, there is no abnormality detected in the complaint
to carefully differentiate these from each other. The presence tooth. Many a times, the tooth may have been treated end-
of trigger points in the cervical region causing radiating odontically, but does not otherwise show up any cause for
pain up to the occipital region favors a musculoskeletal disor- concern. The tooth may have been extracted and then in
der. Local anesthetic blocking of the taut trigger points such cases, the patients insist that the procedure has not
helps in easy differentiation. No satisfactory management alleviated the pain and the pain still persists, as if the tooth
strategies have been outlined in literature due to the pau- is still present, giving rise to the term, phantom pain of the
city of true occipital neuralgia cases. tooth. The pain described is of a dull, aching, throbbing or
burning and persistent type. The patient will usually
describe a feeling of unpleasant sensation being present
ATYPICAL ODONTALGIA throughout the day that does not increase during functions
such as eating or swallowing. AO does not cause any dis-
Atypical odontalgia (AO) is a clinical condition that poses turbance in sleep and patients report of occurrence of pain
quite a challenge to the dental clinician. As the name sug- immediately after awakening. The presence of the pain is
gests it is toothache that cannot be attributed to any cause usually not altered by local provocation such as during
that are usually suspected. It is therefore also known as pulp testing, percussion, etc. Very rarely the pain may
phantom pain. Various other terminologies have been spread to the adjoining structures within the oral cavity,
applied to this condition in the past, such as, atypical such as jaw bones and even rarely may involve the broader
facial neuralgia, migrainous neuralgia, idiopathic tooth- facial region.
ache, etc. that have only added to confusion rather than Graff-Radford and Solberg have outlined the following
clear many of the impending doubts regarding this condi- criteria that may help in identifying AO:
tion. Therefore the use of these alternative terminologies is 1. Pain in a tooth or tooth site
best avoided. AO is a frequently encountered condition in 2. Continuous or almost continuous pain
clinical practice. Patients usually end up getting various 3. Pain persisting for more than 4 months
dental treatment procedures performed for the problem 4. No sign of local or referred pain
and often seek exacting therapy from the clinician. The 5. Inconsistent results of a somatic nerve block
multiple procedures performed previously may at times All investigative modalities such as laboratory or radio-
mislead the dentist into assuming the diagnosis provided graphic tests turn up with negative results. The patient usu-
by the previous clinician. It is still a poorly understood ally undergoes a lot of attempted therapies without having
phenomenon with unclear pathophysiology and as a con- accorded a definitive diagnosis.
sequence diagnostic confirmation is achieved by exclud-
ing other known causes or those with a somatic etiology.
Some authors have suggested a deafferentation pain model
for explaining the clinical behavior, however till date, Other pathologies manifesting similar symptoms need
this has not been conclusively proven. Another school of to be ruled out before arriving at a diagnosis of AO, as
125
the diagnosis is primarily based upon exclusion. ‘Common be of no value in causing any pain relief. The patients usu-
pathologies are commoner than thought to be, while rare ally agree to this after having suffered through numerous
ones are rarer than thought to be’. This statement applies procedures not having provided relief. Any psychologic
accurately in disorders of the oral cavity too and therefore association if suspected should be dealt with in conjunc-
it is necessary for the clinician to emphatically rule out the tion with a psychologist.
common causes of toothache such as pain of pulpal, perio- Atypical odontalgia does not respond to opioid as well
dontal or myofascial origin and pain referred from con- as non-opioid analgesics. The tricyclic antidepressants are
tiguous structures. the most favored drug employed in managing AO. The
Atypical odontalgia can be differentiated from pulpal exact mode of action of these medications is not known,
pain by the following features: patient cannot usually but it has been suggested that the analgesic properties of
point out to a specific tooth with pulpal pain because of the these drugs are more likely acting than their antidepressant
absence of propioceptive ability of the pulp. Pulpal pain effects. Amitriptyline is prescribed in doses ranging from
tends to worsen over a period of time, while AO is more or 25 to 75 mg per day initially and if needed may be used up
less persistent and of the same intensity. Local provocation to 200 mg per day. The beneficial effect of the drug needs
of heat, cold or electricity will give altered results from the to be carefully titrated against the risk of adverse effects
corresponding or opposing teeth in pulpal-related pain, such as dizziness, drowsiness, headache, xerostomia, con-
but not so in AO. Anesthetic blocks cause instantaneous stipation, appetite and weight changes, nausea, weakness,
relief from pulpal pain. Finally the attempted dental thera- hypotension. But patients not achieving relief with high
pies should have alleviated pulpal pathology, but AO mani- doses are unlikely to have any satisfactory remission of the
fests with persistence of the pain. Similarly periodontal pain condition. In those cases that do achieve relief, complete
will show up clinical findings of periodontal disease and elimination of pain is rare and therapy needs to be contin-
also some radiographic evidence of periodontal destruction. ued for a prolonged duration, a minimum of 3 months.
Anesthesia usually relieves periodontal pain but not AO. Other tricyclic antidepressants such as imipramine, nor-
Myofascial pain usually involves the broader orofacial triptyline, and dothiepin have also been used. However,
region with the major component being involvement of their effects are not very different from amitriptyline.
the musculature as opposed to the single tooth or tooth Phenothiazines have also been used with some success in
site of AO. The presence of trigger points leading to either some clinical trials, however, their role is more supplemen-
spontaneous pain or pain on palpation is not a feature of tary to the tricyclic drugs. Tardive dyskinesia is a major
AO and movements of the musculoskeletal system such as potential side effect of phenothiazines that has not allowed
during talking, chewing, smiling cause exacerbation of for a major role of these drugs in AO. Once pain control of
myofascial pain, again not encountered in AO. a desirable degree is achieved, all these drugs should be
Pain referred from the maxillary sinus may be mistaken tapered off gently and then discontinued.
for AO, therefore needs careful consideration. Sinus pain
usually involves the whole of the maxillary jaw or at least
a quadrant on one side. Application of pressure below the
eyes and over the sinus region, and lowering or bending ATYPICAL FACIAL PAIN
down the head lead to an increase in pain of sinus origin, AO
however usually does not demonstrate any change in the This condition has been the cause of much confusion and
intensity of pain. Imaging studies can usually point out the has led to many contradictions in the literature. The term
sinusitis leading to the pain, but AO will not show up any atypical facial pain (AFP) suggests that it is a painful con-
imaging findings. dition not satisfying certain typical manifestations of
In the rare instances, when AO may involve a region well-known conditions. In the current scenario, AFP has
more than a single tooth, trigeminal neuralgia (TN) may been completely rejected as a diagnostic terminology by
need to be ruled out. TN occurs as lancinating pain in bursts the International Association for the Study of Pain (IASP),
of paroxysms, while AO is a dull ache that is continuous in for the simple reason that it does not describe any defini-
nature. No specific trigger zones exist for AO unlike TN and tive condition and has been used injudiciously in the lit-
TN usually manifests in a much older age group. erature to signify conditions that were not understood or
correctly diagnosed. AFP has been as a ‘wastebasket’ for
dumping any painful condition that has not conformed to
Treatment
the set of criteria for other orofacial pain conditions and
Management of deafferentation pains is usually not easily thus is a diagnosis of exclusion rather than inclusion. AFP
accomplished and AO is no exception. The pharmacologic has thus been encompassing a wide group of facial pain
therapy is usually the best suited strategy and all dental problems.
procedures must be completely avoided once a diagnosis of Although rarely as severe as trigeminal neuralgia, facial
AO has been made, because any somatic treatment would pain is continuous for AFP patients. Recent studies propose
126
that AFP is an early form of trigeminal neuralgia. Indeed, been used with some degree of success. Other alternative
some patients have components of both AFP and TN symp- modalities that have been employed include hot and cold
toms. Earlier literature has linked AFP to ‘psychological compresses and acupressure and/or acupuncture. These
pathology’. Recent studies however have shown no such have been able to provide some degree of relief to majority
link exists. of the patients. Usually there are no associated long-term
consequences due to this condition other than altered qual-
Clinical features ity of life, but the patients usually get used to the pain and
continue with routine activities.
The causes for AFP may be varied but they lead to strik-
ingly similar symptoms. Facial pain, typically manifests in
middle-aged women who often describe some kind of a
vague, intractable, burning, aching or cramping, occurs BURNING MOUTH SYNDROME
on one side of the face, often in the region of the trigemi-
nal nerve that may extend into the upper neck or back of Burning mouth syndrome (BMS) is not a very common
the scalp. The pain is usually of a diffuse pattern and pres- condition, yet can be encountered occasionally by the den-
ents as a continuous manifestation with few, if any periods tal clinician. BMS is a poorly understood phenomenon that
of remission. On examination, however, no frank notice- may present with a dilemma to the attending clinician in
able pathology is detected. The discomfort leads to a defi- both diagnosing and managing such patients. BMS is a
nite decrease in the quality of life. Invariably the patient chronic intraoral painful condition that is not characteris-
will have visited a number of clinicians and undergone tically associated with any clinical lesions. It is known by
numerous dental procedures, in hopes of getting a cure. various other synonyms, like burning tongue syndrome,
Many clinical and laboratory tests would have been per- glossodynia, glossopyrosis, stomatodynia, and stomato-
formed and most of them would have yielded negative pyrosis. However it would be incorrect to label the condi-
results. Though a psychological basis has been often attrib- tion as glossodynia or glossopyrosis when the entire oral
uted to this condition by various authors, the findings of mucosa is involved and in such a scenario BMS would be
various studies have been conflicting and the occurrence the appropriate term. The IASP defines BMS as ‘a burning
of psychogenic findings is not different from that of the pain in the oral mucosa’ and glossodynia as ‘a burning pain
general population. in the tongue only’.
The exact etiologic or precipitating cause of BMS is not
yet known in spite of various proposed hypotheses on the
Diagnosis
matter. Over the years, local, systemic and psychologic
Diagnosing AFP is a challenging task. As mentioned ear- factors have been the broadly suggested causes. Local
lier, the diagnostic path for AFP is usually based upon a causes include candidal infection, denture wear, decreased
process of elimination. When a patient complains of above salivary output, and parafunctional oral habits. Systemic
described symptoms the clinician must first rule out any factors that have been suspected are various nutritional
other conditions. The conditions needing careful consider- deficiency disorders (iron deficiency anemia, vitamin defi-
ations are oral (dental), paranasal sinus-related, myofas- ciencies), hormonal changes like menopause, diabetes,
cial and neurologic causes. Tests to be performed include thyroid and parathyroid disorders and drugs. Patients with
radiographs of the skull, advanced imaging modalities, BMS have been described to have higher levels of anxiety,
especially including the brain and the skull base. A careful depression, cancerophobia and mood disorders as com-
otolaryngologic evaluation and a neurological consulta- pared to control groups. Eli Eliav et al (2007) conducted
tion should be comprehensively obtained. AFP patients are a study to assess the role of chorda tympani in patients
considered complex and end up with a partial or incom- with BMS. The striking feature of their study was the
plete diagnosis, the reason being that the patients are not presence of chorda tympani nerve dysfunction in patients
completely evaluated, but only examined by a given spe- with BMS.
cialist, and what implies in the application of only that They concluded that BMS is a form of neuropathic pain,
specific field of treatment. Thus a multidisciplinary sys- which may be related to chorda tympani nerve dysfunction.
tematic evaluation for patients with suspicion of AFP is an They proposed that the evidence of elevated taste detec-
absolute must. tion threshold levels (via electrogustatory testing) and raised
taste/tingling detection thresholds ratio may help clini-
Management cians diagnose BMS.
Treatment of AFP can be difficult and perplexing for both
Clinical features
the doctor and patient. Pharmacotherapy is usually the first
resorted treatment that may be complemented by surgical The typical BMS patient will describe the problem as a
procedures. Drugs like amitriptyline and gabapentin have burning, scalding or a tingling feeling in the oral mucosa.
127
There may be accompanying persistent bad or altered taste cancer arising from the condition need to be resolved. BMS
sensations. BMS usually affects the elderly, above the age patients often end up visiting many clinicians for getting
of 50 years and with a strong predilection for women. The the problem resolved and often sound dejected. Whether
condition seems to occur between 3 and 12 years of reach- there is an underlying psychologic cause for the pain or
ing menopause and presence of other systemic diseases if the psychologic component is a consequence of the pain-
just adds to the increased risk of developing the condition. ful condition has not been accurately ascertained.
Evaluation of a suspected BMS patient should include Petruzzi et al (2007) described five patients who had
details regarding the onset, location, and character of the burning mouth syndrome along with vulvodynia. They
pain. Any related oral symptoms of salivary changes should termed this condition vulvostomatodynia. The condition
also be noted. The pain and/or burning sensations typi- is characterized by burning sensation in the tongue, lips,
cally start upon awakening and increase in intensity with vestibule and other mucosal sites in menopausal and post
the progression of the day and the condition rarely inter- menopausal women.
feres with sleep. The patient describes the typical sensation
as if the mouth or the tongue were scalded or on fire. The Differential diagnosis
burning sensation may be aggravated by hot and/or spicy
foods but is not specifically caused by them. In some Oral lesions such as local oral infections, oral lichen pla-
patients the symptoms begin in a particular oral location nus and the like should be ruled out. The oral mucosa
and then gradually spread to cover the entire mucosa. The should, therefore, be thoroughly examined for any muco-
tip of the tongue and the inner surface of the lips are the sal lesions that may be a cause for the symptoms. The
most common starting locations. Functions such as eating presence of the burning sensation may be an initial sign of
and drinking can bring about temporary relief. However in the neuralgias; however these will be typically unilateral
majority of cases, no such clinical lesions will be detected. in manifestation. The combined occurrence of burning
The patient may give details of systemic symptoms that sensation and decreased salivation should lead the clini-
might correlate the oral condition with hot flushes of meno- cian to investigate any underlying salivary gland pathol-
pause. The patient’s social and psychologic history should ogy. Common systemic diseases causing burning sensations
also be taken into account and noted if any particular event in the oral mucosa such as diabetes and anemia must be
relates to the onset of the symptoms. Queries related to assessed by appropriate laboratory evaluation.
Flowchart 2
Burning mouth syndrome
Initial visit Chronic cases
Topical:
A. Capsaicin gel 0.025% (Capsain-p) Systemic:
B. Alpha-lipoic acid as mouthrinse A. Amitriptyline 25 mg b.i.d. (Amitone, Amitryn, Amitrol,
b.i.d./1–2 months (ALA-100, Aladin Tadamit) for 15 days, o.d. for 15 days
100 mg) B. Gabapentin 300 mg (Gabantin, Gabapin) 1st day 300 mg,
2nd day 300 mg b.i.d., 3rd day 300 mg t.i.d. and then maintain
the dosage. It can be given to a maximum of 2400 mg/day
C. Alprazolam 0.25 mg b.i.d. (Restyl, Alcalm, Anxit SR-0.5 mg)
Systemic:
for a week and slowly withdraw the drug
A. Multivitamins – B12, methyl
cobalamin and folic acid (MCBM-69, D. Clonazepam 2 mg o.d. at bed time for 15 days (Clozep,
Neurokind-G and Nuvolt-G t.i.d.; Lonazep MD, Melzap)
Nurokind plus, Nuvolt: o.d.) E. Nortriptyline 25 mg o.d. for not more than 3 months (trip)
Note: All available medications have been enlisted. However, the physician should choose
the appropriate mode of treatment/drug based on the clinical situations
Management of burning mouth syndrome
128
Management of cases and even in these rapidly lose effectiveness.

Topical clonazepam when used in doses of 1 mg used for
Any treatable cause of the burning sensations in the oral
3 minutes thrice daily has imparted relief to some patients,
mucosa should be identified and treated comprehensively
yet its routine use has not been validated. Systemic ther-
before labeling any patient to be suffering from BMS.
apies with alpha-lipoic acid, capsaicin, amisulpride and
However, once all such conditions have been ruled out
antidepressants have been tried with inconsistent results.
and a diagnosis of BMS has been arrived at, the situation
Gabapentin that has been effective in other chronic pain
should be explained to the patient and counseling regard-
disorders has demonstrated little or no effect on BMS
ing the benign nature of the condition should be offered.
patients. Cognitive behavioral therapy (to replace patterns
The decrease in the quality of life should be explained as
of negative emotions and thoughts with more realistic
in some patients the conditions undergoes spontaneous
and positive ones) has helped few patients who have had
remission while in the majority of cases it persists as a
underlying psychological causes. However, labeling all cases
chronic condition. Due to an unclear etiopathogenesis,
as psychologically based often leads to inefficient manage-
definitive pharmacologic therapy is not available for the
ment of BMS patients by both dental and medical clini-
condition and all modalities are essentially palliative in
cians. Therefore, no single or sure-shot treatment strategy
nature. Topical, systemic and psychologic therapies have
has been evolved for this enigmatic condition and it would
been used with varying degrees of success. Topical thera-
be wrong to label each case of BMS to suffering from the
pies with anesthetic products such as lignocaine and ben-
same causative mechanism, for therein lies the pitfall of
zydamine hydrochloride seem to be effective in a handful
successfully managing BMS (Flowchart 2).
Trigeminal Pain Pathway

Trigeminal nerve is the primary sensory nerve of the maxillofacial region. Pain is conducted along the afferent fibers
of the branches of the trigeminal nerve into the semilunar or gasserian ganglion. The impulses from the ganglion are
carried to the sensory root of the nerve into the pons. The sensory root either ends directly in the main sensory nucleus
or branches out into the ascending fibers (carry general tactile sensation) and descending fibers (transmit pain and
temperature). Pain impulse, therefore descends from pons, through the medulla down to the level of second cervical
segment and terminates.
The secondary neurons emerge from the spinal nucleus, after which the axons cross the midline and ascend to join
the fibers of mesencephalic nucleus to form spinothalamic tract of the fifth nerve.
These axons continue to ascend and terminate in the posteroventral nucleus of the thalamus. Few of these second
order neurons activate the reticular area of the brain stem causing excessive alertness, increase the individual’s sense
of perception and reaction to pain.
The third order neurons project from the posteroventral nucleus carrying pain impulse to the posterocentral convo-
lutions of the cerebral cortex.
129

SECTION Mucocutaneous
III Disorders
6 Red and White Lesions 133

7 Vesiculobullous Disorders 174
8 Oral Ulcerative Diseases 196
9 Dermatological Diseases 218
131
Chapter-06.indd 131 10/3/2012 5:48:07 PM


CHAPTER
Red and White Lesions

Ashok L 6
Color of Oral Mucosa Hereditary Benign Intraepithelial Dyskeratosis
➧ Description of Red and White Lesions (Witkop’s Disease)
White Lesion Focal Epithelial Hyperplasia (Heck’s Disease)
Red Lesion Dyskeratosis Congenita
➧ Etiologic Classification of Red and White Sponge Nevus
White Lesions ➧ Red Lesions of the Oral Cavity
➧ White Lesions of the Oral Cavity ➧ Red Lesions of the Tongue
Frictional Keratosis/Traumatic Keratosis Migratory Glossitis
Chemical Burns and Thermal Burns Median Rhomboid Glossitis
Leukoplakia Deficiency States
Lichen Planus Foliate Papillitis
Candidiasis Erythroplakia
Oral Submucous Fibrosis Discoid Lupus Erythematosus
Psoriasis
In day-to-day clinical practice oral physicians often encoun- ❍ Formation of pseudomembranes

ter a wide spectrum of oromucosal lesions. These lesions ❍ Pigmentation producing cells like melanocytes.
range from harmless mucosal alterations like change in
the color and texture of the oral mucosa, needing simple
therapeutic remedies and patient counseling to lesions of DESCRIPTION OF RED AND WHITE
a life-threatening nature. Red and white colored alterations LESIONS
of the oral mucosa are commonly seen in dental practice.
Recognizing and differentiating these mucosal alterations White Lesion
from normal anatomic variations is imperative for the
effective management of these lesions. White lesion is a non-specific term used to describe any
abnormal area of the oral mucosa that on clinical exami-
nation appears whiter than the surrounding tissue. It is
usually slightly raised, roughened or of different texture
Color of Oral Mucosa from the adjacent normal mucosa (e.g. linea alba buccalis,
As the oral mucosa is translucent, it reflects the contents frictional keratosis, leukoplakia, chronic hyperplastic can-
of the underlying connective tissues. Normal color of the didiasis etc).
oral mucosa is pink, the intensity being varied in different This normal color of the mucosa may turn into white due
parts of the oral cavity and depends upon various factors to the increased thickness of the epithelium with increased
like, production of keratin (hyperkeratosis) and production of
abnormal keratins and imbibition of fluid by the upper lay-
❍ Thickness of the oral mucosa ers of the mucosa. In situations like coagulation of tissue
❍ Degree of keratinization surface (occurs in burns), results in the formation of white
❍ Amount of vascularity and fibrous content in the con- pseudomembrane, which remains attached to the mucosa
nective tissue but can be scraped off easily. Generally white lesions result
133
Section III – Mucocutaneous Disorders
from various factors like, trauma, infections, immuno-

5. Premalignant lesions
logic injury to the mucosa or other genetically determined
Leukoplakia
factors.
Lichen planus
Lichenoid reactions–drug induced, graft-versus-
Red Lesion host disease
Erythroplakia
Red lesion refers to an area of reddened mucosa that may
Actinic keratoses
appear smooth and atrophic or exhibits a granular, velvety
Discoid lupus erythematosus
texture (e.g. erythroplakia, median rhomboid glossitis,
Chronic hyperplastic candidiasis
erythematous candidiasis, etc). These lesions may occur
6. Premalignant conditions
alone or in combination with a white lesion. Such lesions
Oral submucous fibrosis
may be termed as a mixed lesion or a red and white lesion
Oral psoriasiform lesion
(e.g. speckled leukoplakia, erosive lichen planus, etc).
Dyskeratosis congenita
Individual variations in the color of the oral mucosa are
Syderopenic dysphagia
probably an expression of one or more genetically con-
Syphilitic glossitis
trolled factors.
7. Miscellaneous
Healthy masticatory mucosa (gingiva, palate, dorsal
surface of the tongue) is light pink in color. The lining
mucosa (mucosa over the vestibule, cheeks, lips, floor of Intraoral skin grafts (people of Afro-Asian origin) will
the mouth, and ventral surface of the tongue) is reddish not generally exhibit a white coloration of the skin
pink in color. Palatoglossal arch region is dusky red in graft. The graft will appear black or brown depending
color due to increased vascularity and often misdiagnosed on the extent of melanin pigmentation (Figure 1).
as sore throat.
The histological reason behind the appearance of a red
lesion may be due to dilated blood vessels, influx of new
blood vessels, hemorrhage under the epithelium or a rela- WHITE LESIONS OF THE ORAL CAVITY
tively thin outer epithelium.
White lesions of the oral cavity can be categorized clinically
as keratotic (non-scrapable) or non-keratotic (scrapable)
ETIOLOGIC CLASSIFICATION OF RED lesions. Table 1 gives a list of scrapable and non-scrapable
AND WHITE LESIONS lesions.
1. Normal mucosal variations Frictional Keratosis/Traumatic Keratosis

Leukoedema
Frictional keratosis is defined a white patch with a rough
Fordyce granule
surface which is clearly related to a source of mechanical
Linea alba buccalis
irritation and that will disappear over a period of time
2. Genetically linked white keratotic lesions
with the removal of the stimuli. Lesions belonging to this
Oral genodermatoses
category include linea alba buccalis, and cheek, lip or
White sponge nevus
tongue biting or chewing.
Hereditary benign intraepithelial dyskeratosis
Linea alba buccalis is a non-scrapable white line that is
Pachyonychia congenita
present on the buccal mucosa usually along the plane of
3. Post inflammatory white lesions
occlusion (Figure 2). It may either be seen unilaterally or
Traumatic keratosis
bilaterally. Linea alba occurs due to the constant friction or
Mechanical trauma
irritation of the buccal mucosa by the facial surfaces of teeth.
Thermal burn
It is more pronounced with respect to the posterior teeth
Chemical burn (aspirin burn, uremic stomatitis)
and may have a scalloped architecture. It is asymptomatic
Radiation mucositis
and usually does not require any form of management.
Reactive mucosal hyperplasias (stomatitis nicotina
Chronic cheek, lip, tongue chewing usually presents
palati)
as thickened and shredded whitish areas. Habitual oral
4. White and red lesions due to infections
mucosa chewing can sometimes lead to areas of localized
Syphilis
erosion and ulceration. The lesions may present bilaterally
Measles (Koplik’s spots)
or unilaterally.
Candidiasis
Individuals with chronic cheek biting (morsicatio buc-
Bacterial stomatitis
carum) have either a habit of sucking the cheeks frequently
134
Chapter 6 – Red and White Lesions
Figure 1 Figure 2
Linea alba on the buccal mucosa. Courtesy: Department of

Skin graft. Courtesy: Department of Oral Medicine and

Management
Removal of the etiologic agent will generally result in com-
plete resolution of the lesion within 2 weeks. Biopsy may
Table 1 Non-scrapable and scrapable lesions be considered in lesions that persist for a longer duration,
to rule out any dysplastic changes.
Keratotic lesions Non-keratotic lesions
(non-scrapable) (scrapable)
Linea alba buccalis Chemical burn/thermal burn Chemical Burns and Thermal Burns
Frictional/traumatic keratosis Pseudomembranous candidiasis Chemical trauma to the oral mucosa may be due to
Homogeneous leukoplakia Syphilitic mucous patch improper use of medicaments or the use of harsh chemical
Reticular lichen planus Diphtheritic patch substances such as strong acid or alkali.
Chronic hyperplastic candidiasis Chemical burn usually results from the patient applying
Dyskeratosis congenita analgesics like aspirin or acetaminophen or home reme-
White sponge nevus dies such as clove oil to the mucosa adjacent to a decayed
tooth to alleviate pain. Chemical substances like phenol,
silver nitrate, concentrated hydrogen peroxide, root canal
medicaments can also produce an area of necrosis.
or push the cheek in between teeth with their finger.
Thermal burns are generally caused due to the acciden-
Similarly individuals with chronic lip chewing habit
tal intake of hot food or beverages. These mucosal burns
(morsicatio labiorum) and chronic tongue nibbling habit
are characteristically seen on the anterior one third of the
(morsicatio linguarum) present with macerated appear-
tongue and palate.
ance of the labial mucosa and lateral surface of the tongue
The clinical appearance of these burns in most cases
respectively. The lower labial mucosa is usually affected in
depends upon the severity of the tissue damage. Chronic
lip chewers (Figure 3A, B).
mild burn usually produces keratotic white lesion whereas
Habitual chewing may be associated with individuals
intermediate burn causes localized mucositis and the more
under stress. However most of the individuals are unaware
severe burns coagulates the surface of the tissue and pro-
of the parafunctional habit. The management of these
duces a diffuse white lesion. In such cases the tissue can be
conditions should be targeted at educating the patient
scraped off, leaving a raw bleeding painful surface (Figure 5).
regarding the ill-effects of the parafunctional habit.
Frictional keratosis is also caused by the rough flange
of denture, sharp cusp of a tooth or sharp edge of broken
teeth (Figure 4). Acute pseudomembranous candidiasis and gangrenous
Histologically traumatic keratotic lesions will show stomatitis can be considered in the differential diagnosis
varying degree of hyperkeratosis and acanthosis. for chemical and thermal burns.
135
Figure 3
A B
(A) Whitish macerated appearance of the upper labial mucosa. (B) Whitish thickened and shredded area on the lower labial
mucosa. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Figure 4 Figure 5
Frictional keratosis caused by the sharp cusp of the A chemical burn on the buccal mucosa that is characterized
adjacent molar. Courtesy: Dr Praveen, KLE Institute of by the presence of a white pseudomembrane that reveals
Dental Sciences, Bangalore an underlying erythematous area when scraped off.
Courtesy: Dr Ashok
Management
1. Topical application of anesthetic agent like benzocaine/
occur in individuals who had the habit of smoking tobacco.
lignocaine gel (choline salicylate 8.7%, benzylkonium
This lesion is a reactive hyperplasia to the heat generated
0.01% and lignocaine hydrochloride 2%).
by the tobacco smoke that acts as a chronic irritating
2. Topical application of steroids like triamcinolone ace-
agent. These mucosal changes are mostly seen in reverse
tonide (triamcinolone acetonide oral paste 0.1%).
cigarette/chutta and pipe smokers and relatively lesser in
3. In case of severe pain analgesics can be used.
cigar, cigarette and beedi smokers.
Nicotine Stomatitis Clinical features

(Stomatitis Nicotine Palatinus, Smoker’s Palate)
Smoker’s palate is usually seen in males. However, in the
Thoma in 1941 was the first to use the term stomatitis nic- Indian subcontinent women who have the habit of reverse
otine because he noticed this lesion to almost exclusively chutta smoking also exhibit these mucosal changes. It is
136
Figure 6
A B
(A) Diffuse grayish white pigmentation of the palate with red pinpoint areas characteristic of smoker’s palate.
(B) Multiple papules on the palate with red pinpoint areas in smoker’s palate. Courtesy: Department of
generally asymptomatic. The palatal mucosa appears as a Auluck et al conducted a survey in 10 different dental col-
diffuse grayish white surface or flat topped nodules may leges in India among 153 specialists including oral surgeons,
be seen with red pinpoint areas situated in the center of oral physicians and oral pathologists to check the preva-
each nodule (Figure 6A, B). These red pinpoint areas cor- lence of confusion regarding the definition of leukoplakia
respond to the inflamed orifice of the minor salivary gland and its application. It was found that 33.33% of the special-
ducts. ists preferred to follow WHO definition (1978), while 65.35%
preferred to follow Axell (1984) definition. The authors
Differential diagnosis described the current ambiguity regarding the accepted
definition of oral leukoplakia and emphasized the need for
❍ Palatal papillary hyperplasia
an international collaboration to reach a consensus on the
❍ Focal epithelial hyperplasia (Heck’s disease)
use of the term leukoplakia.
❍ Darier’s disease (follicular keratosis)
WHO definition of leukoplakia (1978) Leukoplakia is a
white patch or plaque that cannot be characterized clinically
Leukoplakia or pathologically as any other disease. The definition indi-
Oral leukoplakia (OL) is the most common precancerous cates that the term leukoplakia does not carry a histologic
lesion of the oral mucosa. The term leukoplakia was first connotation and should be used only in descriptive clinical
used by Schwimmer in 1877 to describe a white lesion on context.
the dorsum of the tongue, which probably represented Axell et al definition of leukoplakia (1984) Leukoplakia
syphilitic glossitis. He proposed the term ‘leukoplakia’ for is a white patch or plaque that cannot be characterized
a diffuse patch on the dorsum of tongue. Since then it has clinically or pathologically as any other disease and not
evolved as a clinicopathologic concept over many years; associated with any physical or chemical causative agent
sometimes representing an innocent hyperkeratosis and except the use of tobacco.
sometimes dysplastic features.
Axell et al definition of leukoplakia (1996) Leukoplakia
Definition as a predominantly white lesion of the oral mucosa that
cannot be characterized as any other definable lesion;
The requirement for a clear definition for oral white some oral leukoplakia will transform into cancer. This def-
lesions has long been recognized. Similar requirements inition is most widely accepted in the western world.
also apply to red lesions or the red component of prepon-
derantly white lesions. The definition of leukoplakia has Pindborg et al definition of leukoplakia (1997) A pre-
often been confusing and controversial. Currently the WHO dominantly white lesion of the oral mucosa that cannot be
definition and definition given by Axell are widely used. characterized as any other definable lesion.
137
Epidemiology P (pathology)
P0 No epithelial dysplasia (includes ‘no or perhaps mild
The frequency of leukoplakia is highly variable among
epithelial dysplasia’)
geographical areas and demographic groups. The preva-
P1 Distinct epithelial dysplasia (includes ‘mild to moder-
lence in the general population varies from less than 1 to
ate’ and ‘moderate to possibly severe’ epithelial dysplasia)
more than 5%. The prevalence of leukoplakia increases to
Px Absence or presence of epithelial dysplasia not
8% in men over the age of 70 and the prevalence in women
specified in the pathology report.
past the age of 70 is approximately 2%.
Mehta et al (1961) reported the prevalence of 3.5% in OLEP (Oral leukoplakia) staging system
4,734 Indian population. Manghi et al (1965) reported a Stage I L1P0
prevalence of 6.5% among 2,004 persons. Smith et al (1975) Stage II L2P0
reported a prevalence of 11.7% among 57,518 persons. Stage III L3P0 or L1L2P1
Yang et al (2001) reported the prevalence of leukoplakia Stage IV L3P1
as 24.4% among the aborigines in southern Taiwan. Chang
et al (2005) reported the prevalence of 7.44%. In a 10-year General guidelines for oral leukoplakia
follow-up study, a random sample of 30,000 villagers in staging system
three areas in India, the annual incidence rates varied 1. If there is doubt concerning the correct L or P category
from 1.1 to 2.4 per 1,000 men and 0.03 to 1.3 per 1,000 to which a particular case should be allotted, then the
women. lower (i.e. less advanced) category should be chosen.
In India the prevalence of oral leukoplakia among betel This will also be reflected in the stage grouping.
quid chewers with tobacco ranged from 0.4 to 1.8% and 2. In case of multiple biopsies of single leukoplakia or biop-
among betel quid chewers without tobacco ranged from sies taken from multiple leukoplakias the highest patho-
0.3 to 0.7%. logical score of the various biopsies should be used.
Table 2 gives the overall impression of the prevalence of 3. For reporting purposes the oral subsite according to
oral leukoplakia with a geographic emphasis. the ICD-DA should be mentioned.
Classification and staging system for The letter ‘E’ in the OLEP abbreviation has been used to
oral leukoplakia prevent confusion with the often used abbreviation for oral
lichen planus.
Van der Waal (2000) designed and proposed the classifica-
Some leukoplakias have a white verrucous texture being
tion and staging system. The present classification and
referred to as verrucous leukoplakia. In the majority of these
staging system is primarily designed for the purpose of
cases no epithelial dysplasia is present. Yet, such lesions
uniform reporting of treatment or management results and
may easily recur (‘proliferative verrucous leukoplakia’) after
requires the availability of a biopsy report. It also could
conservative treatment and may finally lead to the devel-
serve as means for epidemiological studies.
opment of a squamous cell carcinoma.
L (size of the leukoplakia)
L1 Size of single or multiple leukoplakia together 2 cm
Etiopathogenesis
L2 Size of single or multiple leukoplakias together 2–4 cm
L3 Size of single or multiple leukoplakias together 4 cm 1. Tobacco (smoke/smokeless form) Use of tobacco in the
Lx Size not specified form of factory-made cigarettes, beedi, cigars and cheroots
Table 2 Prevalence of leukoplakia
Author and year of study Country Number of persons examined Prevalence (%)
Pindborg et al (1965–1966) India 30,000 1.5–3.3
Gangadharan and Paymaster (1971) India 2,03,249 0.7
Mehta et al (1972) India 1,01,761 0.7
Axel (1976) Sweden 20,333 3.6
Bouquet and Gorlin (1986) USA 23,616 2.9
Hogewind and Van der Waal (1988) Netherlands 1,000 1.4
Banoczy and Rigo (1991) Hungary 7,820 1.3
Ikeda et al (1991) Japan 3,131 2.5
Schepman et al (1996) Netherlands 1,000 0.6
138
and powdered tobacco in pipes or rolled into hand-made Alcohol causes dehydration of the oral mucosa and increases
cigarettes, are the main etiological agents in the causation the ambient temperature of the oral cavity thereby making
of leukoplakia. the oral mucosa more vulnerable to the carcinogenic
Much of the tobacco in the world is consumed in the form effects of tobacco. Alcohol by itself contains known car-
of smokeless tobacco and it is placed in contact with the cinogens such as hydrocarbons and nitrosamines. It also
mucous membrane. In south-east Asian countries tobacco causes liver induced cellular changes in target tissues
is mostly consumed mixed with other ingredients like (increased acetaldehyde content).
areca nut, betel leaf, slaked lime, catechu and condiments.
Smokeless tobacco use is practiced in many forms. 3. Viral infection The possible implication of human
Chewing of tobacco containing products or snuff dipping papilloma virus in the etiology and potential for malignant
habits vary greatly from one part of the world to another. transformation of oral premalignant lesion has been stud-
It is of great importance to quantify the degree of exposure. ied extensively and it was reported that the likelihood of
Over 300 carcinogens have been identified in tobacco detecting HPV was 2–3 times higher in precancerous oral
smoke or in its water-soluble components which can be mucosa and 4–5 times higher in oral squamous cell carci-
expected to leach into saliva. The major and most studied noma than in normal oral epithelium.
among them include aromatic hydrocarbons, benzopyrene The possible viral etiology of OL had been first sug-
and the tobacco specific nitrosamines, N-nitrosonornicotine gested by light microscopic demonstration of HPV sugges-
(NNN), nitrosopyrrolidine (NYPR), nitrosodimethylamine tive changes (Fejerskov, 1977).
(NDMA) and 4-(methylnitrosamine)-1-(3-pyridyl)-1-buta- The first evidence of HPV etiology of oral leukoplakia
none (NNK). Benzopyrene is a powerful carcinogen and is had been provided by Loning (1985), Syrjanen (1986) and
found in amounts of 20–40 mg per cigarette. The main- Naghashfar (1985).
stream smokes of a cigarette contain 310 mg of NNN and In a follow-up study of 20 leukoplakias, Lind (1987)
150 ng of NNK. These agents act locally on keratinocytes, established a significant correlation between the presence
stem cells, and are absorbed and act in many other tissues of HPV antigen and the degree of dysplasia and malignant
in the body. transformation.
Cigarettes can be classified as low or medium, if the tar High prevalence of HPV in PVL had been found by
yield is below 22 mg and as high if tar yield above 22 mg Maitland et al (1987) who found an overall rate of 42%
compared with non-smokers the risk of oral cancer and OL using probes specific for HPV 16. The differences in detec-
for smokers using low to medium tar cigarettes is 8.5%. tion of HPV may be accounted for different sensitiveness
A case control study from Brazil also found an increased of methods used. D’costa et al (1998) in their study detected
risk among cigarette or pipe smokers with a strong dose- HPV 16 in 34% of the potentially malignant lesions and
response relationship between the number of years smoked Mao et al (1996) in 36% of precancerous lesions. In oral
and oral cancer risk (Franco et al, 1989). cavity HPV 16 has been demonstrated in both homoge-
Snuff involves placing moistened, powdered tobacco, neous leukoplakia and in verrucous leukoplakia (Loning,
treated with natron (sodium sesquicarbonate) in the lower 1985; De Villers, 1986; Milde, 1986; Syrjanen, 1988 and
labial, buccal sulcus or the floor of the mouth against the Kashima et al, 1990).
lingual side of the mandible. Snuff has been shown to Studies by Nielsen et al (1996) in the prevalence of HPV
contain carcinogens such as N-nitrosamines which are in oral premalignant lesions found an overall rate of HPV
derived from tobacco. positive lesions to be 40.8% of which five of them were
Many studies have demonstrated that tobacco in any HPV 16. Elamin et al (1998) suggested that the prevalence
form can predispose to OL (Pindborg et al, 1972; Salonen of HPV infection is higher in oral lesions from India. The
et al, 1990; Christian et al, 1992). high prevalence of HPV infection in oral premalignant
cases indicates that HPV infection would be an early event
2. Alcohol Alcohol has also been emerged as a signifi- in the process of malignant transformation of oral epithelial
cant risk factor for OL. In Germany, Wilsch et al (1978) cells.
found that alcohol consumption was greater among peo-
ple with leukoplakia as compared to control. In India 4. Leukoplakia and diabetes A few studies in the litera-
Gupta et al (1984) studied alcohol habits of over 7,000 ture have found a significant association between diabetes
individuals with tobacco habits and found that 31% con- mellitus and OL prevalence (Albert et al, 1992; Ujpal et al,
sumed alcohol regularly, 25% occasionally and 44% did not 2002).
consume alcohol at all. The prevalence of OL was higher Albert et al (1992) reported an OL prevalence of 6.2%
among regular and occasional (3.9%) alcohol drinkers than among diabetics as compared to 2.2% in a control group.
among non-drinkers (2.9%). However the analysis did not adjust for age and gender.
Although alcohol by itself not an important risk factor Diabetes mellitus leads to a number of metabolic and immu-
for leukoplakia, it might produce synergetic effects when nologic changes that affect the oral mucosa and it is associ-
combined with the habit of chewing tobacco or smoking. ated with a variety of oral conditions (Ponte et al, 2001).
139
Thomas et al (2004) have found a significant association

Figure 7
between diabetes mellitus and OL prevalence.
According to weighted model, diabetics were three times
more likely to have OL than non-diabetics. However no
association between diabetes and the incidence of oral
squamous cell carcinoma has been described previously.
Therefore the significance of the apparent association
between diabetes and OL is unclear.
5. Candidiasis and leukoplakia There is a longstanding
discussion whether Candida infection is a cause of leuko-
plakia or if it is a superimposed infection in a pre-existing
lesion. It has been shown that, upon treatment, non-
homogeneous Candida-infected leukoplakias convert into
a homogeneous lesion, and some lesions even regressed.
6. Dietary factors Ramaswamy et al (1996) have shown
that serum vitamins A, B12, C, E, beta-carotene and foliate Commissural leukoplakia. Courtesy: Department of Oral
were decreased in leukoplakic patients as compared to con- Medicine and Radiology, MCODS, Mangalore
trols. Many other studies supporting the protective effects
of carotinoids and vitamin A, C and E have been reviewed
by Enwon et al (1995) and Garewell et al (1999). Figure 8
Only a very few studies have reported a positive asso-
ciation between estrogens and oral leukoplakia. Hashibe
et al (2000) reported an inverse relationship between body
mass index (BMI) and OL prevalence in a large population
in India. The authors hypothesize that higher estrogen lev-
els in people with higher BMI may explain this relation-
ship. Kushlinskill et al (1988) have demonstrated estrogen
receptors in oral squamous cell carcinoma and OL. Thomas
et al (2004) recently have demonstrated a strong protective
effect of estrogen use on OL prevalence in women. In their
study there was no case of OL among women who were
using estrogen.
Clinical features
1. Leukoplakia is seen most frequently in middle-aged
Homogeneous leukoplakia on the buccal mucosa.
and older individuals. Sex distribution is also vari-
Courtesy: Dr Ashok
able. Men are more affected in some countries, while
this is not the case in the western world. Less than 1%
of men below the age of 30 have leukoplakia. The
4. Early or thin leukoplakia appears as a slightly ele-
male-to-female ratio is reported to be about 3:1 to 6:1.
vated grayish-white plaque that may be either well
2. Leukoplakia can be either solitary or multiple.
defined or may gradually blend into the surrounding
3. Leukoplakia may appear on any site of the oral cavity,
normal mucosa (Figure 11). As the lesion progresses, it
the most common sites being: buccal mucosa, alveo-
becomes thicker and whiter, sometimes developing a
lar mucosa, floor of the mouth, lateral border of tongue,
leathery appearance with surface fissures. Some leu-
lips and palate, however the lesions in the floor of the
koplakias develop surface irregularities and are referred
mouth, lateral border of the tongue and lower lip are
to as granular or nodular leukoplakias. Other lesions
most likely to show dysplastic or malignant changes.
develop a papillary surface and are known as verru-
By far the most affected oral sites are the commissures
cous or verruciform leukoplakia.
(Figure 7) and the buccal mucosa (Figure 8) showing
60–90% of the leukoplakia. Next are the lip (3.7%),
Clinical forms of leukoplakia
the alveolar ridge (3.0%) (Figure 9), the tongue (1.4%),
floor of the mouth (1.3%), vestibular mucosa (1.1%) Classically two clinical types of leukoplakia are recognized:
(Figure 10) and the palate (0.9%). homogeneous and non-homogeneous, which can co-exist.
140
Figure 9 Figure 11
Homogeneous leukoplakia on the alveolar ridge and

buccal mucosa. Courtesy: Dr Ashok Early or thin leukoplakia on the buccal mucosa.
Courtesy: Dr Ashok
Figure 10
Figure 12
Keratotic white patch in the labial vestibule due to

placement of tobacco (tobacco pouch keratosis).
MCODS, Mangalore
Homogeneous leukoplakia on the dorsum of the tongue.

Homogeneous leukoplakia is defined as a predominantly Courtesy: Dr Sumanth
white lesion of uniform flat and thin appearance that may
exhibit shallow cracks and that has a smooth, wrinkled or
corrugated surface with a consistent texture throughout
(Figure 12). This type is usually asymptomatic. or discomfort. Proliferative verrucous leukoplakia is an
Non-homogeneous leukoplakia has been defined as a aggressive type of leukoplakia that almost invariably
predominant white or white-and-red lesion (erythroleuko- develops into malignancy. This type is characterized by
plakia) (Figure 13) that may be either irregularly flat, widespread and multifocal appearance, often in patients
nodular (speckled leukoplakia) or exophytic (exophytic without known risk factors. In general, non-homogeneous
or verrucous leukoplakia). These types of leukoplakia are leukoplakia has a higher malignant transformation risk,
often associated with mild complaints of localized pain but oral carcinoma can develop from any leukoplakia.
141
❍ Non-homogeneous type of OL
Figure 13
❍ Presence of Candida albicans
❍ Presence of epithelial dysplasia.
Longitudinal studies of the rate of malignant transforma-

tion in leukoplakia were first reported by Sugar and Banoczy
(1959) in Hungary.
In a study conducted by Gupta et al, 12,212 tobacco
users were followed up annually to assess the malignant
potential of precancerous lesions in Ernakulam district in
Kerala, India. They observed that out of a total of 19 new
oral cancers over period of 8 years, 15 (79%) originated
from leukoplakia.
The location of oral leukoplakia has a significant cor-
relation with the frequency of finding dysplastic or malig-
nant changes at biopsy. In the study by Waldron and
Shafer (1975), the floor of the mouth was the highest risk
site, with 42.9% of leukoplakias showing some degree of
epithelial dysplasia, carcinoma in situ, or unsuspected inva-
sive squamous cell carcinoma. The tongue and lips were also
identified as high risk sites, with dysplasia or carcinoma
Erythroleukoplakia on the buccal mucosa. present in 24.2% and 24% respectively.
Courtesy: Department of Oral Medicine and Radiology, The study by Silverman and colleagues (1984) showed
MCODS, Mangalore an overall malignant transformation of 17.5%. In their
study only 6.5% of homogeneous leukoplakia underwent
malignant change, however, 23.4% of speckled leukopla-
Malignant transformation kia and 36.4% of leukoplakias with microscopic evidence
of dysplastic changes transferred into cancer.
White lesion in the oral cavity were thought to be precancer- Among the different clinical varieties of leukoplakias,
ous as early as 1870 by Paget, who gave them such appella- proliferative verrucous leukoplakia has got highest risk of
tions as ichthyosis, smoker’s patch and leukokeratosis. malignancy. In a follow-up study of 54 cases of proliferative
Leukoplakia is an example for precancerous lesion. verrucous leukoplakia, Silverman and Gorsky found that
The frequency of dysplastic or malignant alterations in 70.3% of the patients subsequently developed squamous
oral leukoplakia has ranged from 15.6 to 39.2% in several cell carcinoma.
studies. Although leukoplakia is more common in men Holmstrup and Besserman (1983) and Silverman (1990)
than women, several studies have shown that women with inferred that proper use of antifungal therapy might result
leukoplakia have a higher risk of developing carcinoma. in a shift from high risk nodular or speckled leukoplakia to
A wide range of rates for the malignant transformation low risk homogeneous leukoplakia. Hernandez et al (2003)
of leukoplakia has been reported from 0.13 to 19.8% but it have suggested that in patients who display oral premalig-
is estimated that the annual transformation rate should nant conditions like leukoplakia, immunosuppression must
not exceed 1%. Up to 10% of leukoplakia may be malig- be considered as an important risk factor for oral cancer.
nant at the time of initial examination. The potential for
malignancy appears higher in certain risk sites like floor of Investigations
the mouth and ventral surface of the tongue, where the
lesion is associated with Candida species, or where the lesion Although an experienced oral physician may be able to
is verrucous or mixed with red lesions (erythroleukoplakia diagnose and manage majority of the leukoplakias, it is
or speckled leukoplakia). always prudent to follow a systematic investigative proto-
Cawson (1966), Einhorn and Wersall (1967) and Banoczy col to diagnose leukoplakia. The investigative procedures
(1977) inferred that certain features of leukoplakia have includes the following:
been reported to be associated with an increased risk of
❍ Toluidine blue staining: Toluidine blue clinically stains
malignant transformation. These are:
malignant lesions, but not normal mucosa. In vivo, the
❍ Gender, particularly women seems to be at risk dye may be taken up by the nuclei of malignant cells
❍ Long duration of the leukoplakia manifesting increased DNA synthesis. Toluidine blue
❍ Leukoplakia in non-smokers (idiopathic leukoplakia) also serves as a guide to biopsy by localizing tumor
❍ Location in the floor of the mouth and or on the tongue cells within the area of the lesion. Toluidine blue
142
Figure 14 Figure 15
Hyperorthokeratosis and basal cell hyperplasia in leukoplakia.

Toluidine blue stained leukoplakic lesion on the gingiva and
buccal vestibule. Courtesy: Dr Ashok
❍ This lesion represents various degrees of epithelial dys-
plasias. Some lesions exhibit carcinoma in situ with top
staining uses a 1% aqueous solution of the dye that is to bottom basilar hyperplasia, loss of polarity, increased
decolorized with 1% acetic acid. The dye binds to dys- mitosis, hyperchromatism, dyskaryosis and alteration
plastic and malignant epithelial cells with a high in nuclear cytoplasmic ratio without any evidence of
degree of accuracy (Figure 14). thickening of epithelial layer or without any evidence
❍ Cytobrush technique: This technique is more accurate of disturbance of keratinization process (Figure 15).
than any other cytologic technique used in the oral ❍ The frequency of epithelial dysplasia in leukoplakia
cavity. The cytobrush technique uses a brush with firm varies between less than 1% and more than 30%. The
bristles that obtains individual cells from the full thick- presence of epithelial dysplasia is generally accepted
ness of the epithelium. as one of the most important predictors of malignant
development in premalignant lesions.
These two techniques are adjuncts and not substitutes for ❍ Markers of proliferation in leukoplakia: There are
an incisional biopsy. markers for determining future cancer development in
❍ Biopsy: When a suspicious lesion is identified, an inci- oral premalignant lesions. These markers are divided
sional biopsy using a scalpel or a biopsy forceps is into genomic markers and differentiation markers.
recommended. When the lesion is very small excisional The genomic markers include DNA aneuploidy, loss of
biopsy is performed as an investigative procedure and heterozygosity and changes in expression of onco-
as a treatment modality. genes and tumor suppressor genes (p53), whereas the
❍ In homogeneous leukoplakia, the value of histological proliferative markers include keratins and carbohydrate
examination to some extent is questioned. The occur- antigens.
rence of epithelial dysplasia is rather low in this type
as is the risk of future malignant transformation. How- Differential diagnosis
ever, even the experienced clinician will occasionally The keratotic lesions that could be considered in the dif-
be surprised by the histopathological findings of a ferential diagnosis of homogeneous leukoplakia includes:
clinically innocent looking homogeneous leukoplakia.
❍ Chronic hyperplastic candidiasis
Therefore a biopsy should be performed in homoge-
❍ Reticular lichen planus
neous leukoplakia. In non-homogeneous leukoplakia,
❍ White sponge nevus.
i.e. usually symptomatic, epithelial dysplasia or even
carcinoma in situ or early squamous cell carcinoma is
Treatment (Flowchart 1)
rather common. The biopsy should be taken at the site
of symptoms, if present, and or a site of redness or General considerations As a standard rule all possible
induration. Biopsies of exophytic, verrucous or papil- agents leading to white keratotic lesions should be eliminated
lary lesions should be taken deep enough to include a such as sharp teeth, candidal infection, etc. so as to rule out
sufficient amount of underlying connective tissue, and other definable lesions. In persisting lesions or in the absence
preferably from the margins. of possible causative factors, a biopsy should be taken to
143
Flowchart 1
Leukoplakia
Topical antifungal agents –

Candid cream (clotrimazole),
thrice/day for a week
No response Reduction in size
Less than 1 cm More

Morethan
thanor or Continue
Continuethe
thetopical
topical
equal
equal to
to 11cm
cm antifungal
antifungalagent
agentfor
for
11month
month
Excisional biopsy Incisional biopsy
Dysplasia present Dysplasia absent
Total
Totalexcision
excisionof
ofthe
the IfIfexcision
excisionisisnot
notpossible
possible–– Retinol –A
Retinol–A
lesion
lesionwith
withgraft
graft A. Capsules of
A. Capsules of lycopene
lycopene 44 mg
mg b.i.d.
bid or (VitaminAAanalogue)
(Vitamin analog)
8ormg
8 mg
ODo.d.
for for 3 months
3 months ointment
ointmentapplication
application
B. Capsules antioxidants
B. Capsules of antioxidants with
with b.i.d./1
b.i.d./1month
month
Follow
Followup
uponce
onceinin selenium bid
selenium b.i.d.
forfor 6 months
6 months
66months
monthsfor
for33years
years C. Topical bleomycin
C. Topical bleomycin 1% 1% w/v
w/v thrice
thrice
a day
day forfor
1515 days
days
Note: All available medications have been enlisted. However, the physician should choose
the appropriate mode of treatment/drug based on the clinical situation
Management of leukoplakia
exclude histologically the presence of a definable lesion and Some carotinoids have antioxidant or anticarcinogenic
to establish the degree of epithelial dysplasia, if present or activities and can block genotoxic activity of oral carcino-
even the presence of carcinoma or carcinoma in situ. gens. Retinoids are the synthetic and natural analogs of
Up to 60% of leukoplakias regress or totally disappear vitamin A. There are naturally occurring retinoid, includ-
if tobacco use is stopped. OL induced by smokeless tobacco ing retinol, retinal, retinoic acid and their metabolites.
may resolve if the habit is stopped. Some candidal leuko- Beta-carotene is a natural precursor of vitamin A. More
plakias respond, at least partially to antifungal drugs (smok- recently etretinate 13-cis retinoic acid and other retinoid
ing should also be stopped) and dysplasia may regress. have been successfully used for the treatment of oral
leukoplakia. Exactly how retinoids may act to inhibit
Medical management of oral leukoplakia
carcinogenesis is unclear, although some retinoids may
Chemoprevention Chemoprevention is the interventional
enhance anti-tumor immune responses. Retinoids have a
use of chemical agents such as minor dietary constitu-
pronounced and essential effect on cell differentiation.
ents and pharmaceuticals to halt or slow the carcinogenic
Retinoids may have an effect by their interaction with
process before invasion. These include natural or synthetic
growth control mechanisms such as transforming growth
compounds, micronutrients or non-nutrients.
factors and also possibly by acting on tumor suppressors
1. Carotinoids and retinoids (-carotine, Vitamin E, either directly or via an interaction with transforming
selenium, canthaxanthin, astaxanthin, phytoene and growth factors. Retinoids may inhibit the transformation
spirulina-dunaliella) mediated by papillomaviruses. Oral leukoplakias have
144
been treated with a range of retinoids and carotinoids. form of photodynamic therapy (PDT) using hematopor-
Leukoplakias have been successfully treated with systemic phyrins has been found to be effective in animal models
13-cis retinoic acid. and has been used to treat head and neck cancers and
Mulay and Urbach (1958) was the first to use vitamin A premalignant lesions in man. PDT was first used in 1990,
therapy for topical therapy of oral leukoplakia. Smith when acridine and light were combined to kill paramecia
(1962) administered 75,000–300,000 IU of vitamin A daily and the first oncologic use of PDT was in 1903, when
for a period of 3–18 months. Seventy two percent of eosin and light were employed in the treatment of skin
hyperkeratosis lesions, 25% of massively keratotic and cancer. PDT involves using specific wavelength of laser
ulcerative forms of leukoplakia improved but the dyskera- light to activate a photosensitizing drug which is adminis-
totic forms were unresponsive. However signs of hyper- tered systemically and is retained selectively in the lesion
vitaminosis were not observed. Silverman et al (1965) and this triggers a cold photochemical reaction resulting
reported that short-term large dose treatment with vitamin A in the generation of reactive products such as singlet oxy-
(300,000–900,000 IU daily) leads to the improvement of gen that damages tissue. Advantages of this type of treat-
leukoplakia. ment as reported by Sciubba (1995) includes inactivation
of clinically subtle or undetectable alteration, sparing of
2. 13-cis retinoic acid 13-cis retinoic acid (CRA) at
normal tissue, minimal morbidity and its use as an adjunc-
1.5 mg/kg per day for 3 months followed by 0.5 mg/kg
tive tool to more traditional modalities. However it was
daily for 9 months resulted in an initial 55% beneficial pointed out that the major disadvantage of PDT is the
response followed by maintenance of effect in 92% of cutaneous photosensitivity which can persist for several
cases. Kaugars and Silverman (1995) in a study of 10 months after administration of the photosensitizer which
patients with leukoplakia using 50 mg of 13-CRA per day can be a major problem in the Indian subcontinent, where
for 3 months showed a 50% reduction in the clinical size oral cancer is most common.
of the lesions in three patients. Treatment of three patients
was discontinued because of elevated serum triglyceride Topical chemotherapy of oral leukoplakia Topical treat-
levels or headache. ment of leukoplakia with podophyllin solution (Kovacs
3. Beta-carotene Many authors have proposed that et al, 1962) or bleomycin (Hammersley et al, 1985;
beta-carotene supplementation can be used for the treat- Malmstrom, 1988; Wong et al, 1989) has induced some
ment of leukoplakia with minimal side effects. Beta-carotene regression or even total resolution of dysplasia and of
alone in a dose of 30 mg daily for 3–6 months also pro- clinical lesions. Hayasaki et al (1977) described the use of
duced a 71% response rate in 24 patients with oral leuko- bleomycin with iontophoresis in the treatment of cancer,
plakias, with no significant toxicity (Garewall et al, 1991). leukoplakia and papillomas of the head and neck region.
The same workers have used beta-carotene 60 mg daily for Their results showed that this method of application was
6 months and report similar benefit (Garewall et al, 1992). not effective for malignant lesions but was effective at
Others have found beta-carotene 90 mg daily to produce removing leukoplakia of the oral mucosa.
benefit in 44% after three cycles of use of 3 months each.
Newer treatments
4. Fenretinide (n-4-hydroxy phenyl retinamide) Gene therapy Patients with head and neck cancer includ-
Fenretinide (synthetic retinoid) 200 mg daily used for 1 ing oral cancer are more susceptible to chromosome dam-
year reduced the relapses and appearance of new oral leu- age when their cells are exposed to mutagens (Schantz et al,
koplakias, compared with controls, with few adverse 1990) and there are a number of genetic changes now
effects in 39 patients having previously had leukoplakias described in oral carcinoma (Scully et al, 1993). Synthetic
surgically excised. antisense oligonucleotides complementary to the start
codons of human papilloma virus (HPV) type 18 E6 and E7
5. Vitamin E Vitamin E has synergistic inhibitory activ- genes can significantly inhibit growth in vitro of oral car-
ity against carcinogenesis in animal models and may have
cinoma cell lines (Steele et al, 1992, 1993).
some beneficial effect in man. Many studies, using vita-
Even though laboratory and animal data for the use of
min E in oral leukoplakias showed a beneficial clinical
gene therapy is very incomplete, many investigators have
response in 46% of 43 patients by 24 weeks, and a histo-
begun clinical trials in human patients. Tests of several
logical response with no serious adverse effects. Another
types of gene therapy have begun in various types of can-
study showed a significant decrease in oral leukoplakia
cer, and for oral cancer; the trials include the testing of
after combined treatment with vitamin E, retinol and
recombinant p53, the expression of suicide genes and the
-carotene (Zaridze et al, 1993). Vitamin E therefore shows
use of conditionally competent adenoviruses. Since the
promise in the control of leukoplakias.
scientific basis for these trials is rather weak, it can hardly
Photodynamic therapy of oral leukoplakia A combined be expected that impressive results are imminent. There
parenteral and locally applied treatment modality in the are as yet no trials in oral potentially malignant lesions
145
aimed at correcting genetic changes or enhancing the Lichen Planus

immune response by gene therapy; indeed the whole field
of gene therapy has been publicly criticized for its rush The word lichen planus is derived from the Greek litera-
to clinical experiments, when the basic studies are still ture; lichen meaning ‘tree moss’ and planus refers to ‘flat’.
incomplete. In 1869 Erasmus Wilson, a dermatologist first reported the
condition. Oral lesions of lichen planus were first described
Other alternative modalities of treatment by Thiebergie. Wickham (1895) described the characteris-
Green tea Tea, the dried leaves of the plant ‘Camellia tic appearance of whitish striae and punctuations that
sinensis’ is a popular beverage consumed worldwide. The develop atop the flat-surfaced papules.
pharmacological actions of green tea includes; anti-
inflammatory action, antioxidant action and anticancer Definitions
action. Tea acts as an inhibitor of initiation of carcinogen-
esis in the following ways: Oral lichen planus (OLP) is a common chronic immuno-
logical inflammatory mucocutaneous disorder that varies
❍ Tea polyphenols are effective in reducing the accumu- in appearance from keratotic (reticular or plaque like) to
lation of free radicals by inducing the production of erythematous and ulcerative.
superoxide dismutase (SOD), a free radical scavenger Eisen (2005) defined OLP as a relatively common chronic
(Das et al, 2002). inflammatory disorder affecting the stratified squamous
❍ Tea inhibits formation of mutagens in a dose depen- epithelia. It is a skin disease common with in the oral cav-
daet manner (Weisburger et al, 2002) and reduces lipid ity, where it appears as either white reticular, plaque, or
peroxidation (Fadhel et al, 2002). erosive lesions with a prominent T-lymphocyte response
❍ In UV induced responses, epigallocatechin gallate in the immediate underlying connective tissue.
(EGCG) prevents the formation of UVB induced cyclo-
butane pyrimidine dimers (Katiyar et al, 1999).
Etiopathogenesis
❍ EGCG is also a strong inducer of the detoxifying
enzyme glutathione-s-transferase. In spite of extensive research, exact etiology is still unknown.
The most accepted and current data suggest that OLP is
These reports strongly point toward antimutagenic activ-
T cell mediated disorder in which there is production of
ity of green tea. The gallated flavonoids (especially gal-
cytokines which leads to apoptosis. Autocytotoxic CD8 and
lated EGCG and the aflavins) act Khafif et al (1998)
T cells trigger apoptosis of oral epithelial cells (Eversole,
reported that green tea extract has been shown to have a
1997; Porter et al, 1997). The immune system is triggered
chemopreventive or inhibitory role in the treatment of oral
due to the interactions among genetic, environmental, and
leukoplakia. Li et al (1999) conducted a double blind, pla-
lifestyle factors.
cebo-controlled trial in 59 patients with oral leukoplakia,
Other possible theories include the genetic background,
and found that oral and topical administration with a
where the weak association between HLA antigen and
black and green tea mixture resulted in a partial regression
lichen planus was found by Powell et al (1986) and
of the lesion in 37.9% of the treated patients compared to
Roston (1994). Dental materials and infectious agents like
placebo control. The treatment reduced cell proliferation
Gram negative aerobic bacillus, spirochetes and increased
and the rate of chromosome aberration in peripheral blood
prevalence of Candida species were suggested by Simon
lymphocytes. Weisburger et al (1999) have proposed that
and Hornstein (1980). Vincent et al (1990), Soto Araya
catechins, in particular EGCG are believed to be responsi-
et al (2004) reported the strong association of psychologi-
ble for the chemopreventive effects of green tea, which act
cal factors like higher level of anxiety, greater depression,
by antioxidant and free radical scavenging activity.
and psychic disorders in patients with erosive lichen
Oral lycopene Oral lycopene in the dose of 8 mg/day is planus.
beneficial in the treatment of oral leukoplakia.
Clinical features
Surgical line of treatment If the lesion is very small, it
should be excised as a part of investigation and as a treat- Lichen planus commonly affects 1–2% of the general popu-
ment option. lation, prevalence rate being 0.1–4%. Forty percent lesions
Any leukoplakia could transform into a carcinoma, occur on both oral and cutaneous surfaces, 35% occur on
even those which did not show epithelial dysplasia ini- cutaneous surfaces alone, and 25% occur on oral mucosa
tially (or in which dysplasia happened to be absent from alone.
the biopsy taken). The main problem is that the malignant Lichen planus commonly affects the adults. However
transformation cannot be reliably predicted yet. Regular it has also been observed in children as recently reported
check-up of these patients is essential both in treated and by Sharma (1999). The age range usually varies from 40 to
untreated patients. 70 years.
146
Figure 16
A B
Violaceous papular lesions of cutaneous lichen planus on the upper and lower extremity. Courtesy: Dr Ashok
There is definite female predilection with ratio of 2:1. Andreasen (1968) divided OLP into six types, namely,
The characteristic manifestation of oral lichen planus is the reticular, papular, plaque like, erosive, atrophic and bul-
appearance of white papules that usually coalesce forming lous type. Oral lesions may manifest in one of the follow-
a network of lines that may intersect or crisscross each ing basic clinical forms; hypertrophic (plaque like, papular,
other forming various patterns. Louis Frederic Wickham reticular), erythematous (atrophic, erosive) and bullous.
described the presense of fine, white or gray lines or dots Reticular form is the most common type. It appears as
seen on the top of the pruritic papular rash on the skin a series of fine, radiant, white striae known as Wickham
in lichen planus. These striae are popularly referred to as striae which may be surrounded by a discrete erythematous
Wickham’s striae. border. The most common site of involvement is the poste-
rior buccal mucosa, tongue and gingiva (Figure 17A–D).
Skin involvement in lichen planus Plaque-like lesions resemble leukoplakia and occur as
Skin lesions are characteristically itchy and violaceous to homogeneous white patches.
brown papules, very frequently distributed over the flexor In 1892 Kaposi first described a distinctive clinical
aspect of the wrist or ankles, extensor aspect of the variant of the disease with blisters, which was termed
lower legs, skin of the lower central back and natal clefts lichen ruber pemphigoides or bullous lichen planus. This
(Figure 16A, B). form is rarer than other forms of oral lichen planus. It
Scalp involvement results in loss of hair (alopecia) appears as a small bullae or vesicle that tends to rupture
which is referred to as lichen planopilaris. Longitudinal easily. The bulla or vesicle range from few millimeter to
ridging and grooves are seen over the nail (onychorrhexis). several centimeters in size.
Other changes seen in the nail includes distal splitting Erosive lichen planus is the second most common type
(onychoschizia), separation of the nail plate from nail bed (Figure 18). Erosions are often extensive, irregular and
(oncycholysis), permanent damaged nail matrix (pteryg- affect the lingual and buccal mucosa and are often associ-
ium) and finally permanent nail loss (anonychia). ated with white lesions. Gingival involvement in erosive
Koebner’s phenomenon is the appearance of the lesions lichen planus produces desquamative gingivitis (Figure 19),
which has greater potential for malignant transformation.
at the site of micro trauma and is well demonstrated in
Occasionally diffuse melanosis may be seen along with ero-
case of skin involvement as well demonstrated in the oral
sive type of lichen planus, which is referred to as pigmented
lesions. Hence any mechanical trauma or irritation such as
lichen planus. The lesion presents as reticular white lesions
sharp filling margins or rough surfaces or ill-fitting den-
(may or may not be associated with erosive areas) sur-
tures should be evaluated and eliminated.
rounded by brownish to grayish black pigmentation (Figure
20A, B). It is believed that the T lymphocytes infiltrate into
Oral manifestations
the basal layers and cause basal cell degeneration thereby
The basic lesion of OLP is papule arranged in the linear or stimulating melanogenesis. Histopathological evaluation
annular forms and crisscrossing each other forming vari- of pigmented lichen planus shows basilar melanosis and
ous patterns like annular and reticular forms. melanin incontinence.
147
Figure 17
A B
C D
(A) Reticular lichen planus on the buccal mucosa. (B) Reticular lichen planus on the buccal mucosa.
(C) Lichen planus on the palate. (D) Reticular lichen planus on the labial mucosa of the upper lip.
Courtesy: Dr Ashok
Atrophic lichen planus presents as diffuse red patches

Figure 18
usually surrounded by white striae. Such striae that radiate
peripherally are usually evident at the margins of atrophic
zones of the lesion (Figure 21A, B).
Reticular forms are usually asymptomatic and rarely
require treatment whereas erosive and atrophic forms are
frequently associated with severe discomfort and burning
sensation. It is an absolute necessity to evaluate periodi-
cally for malignant transformation.
Association of oral lichen planus with systemic

illness
Carrozzo et al (2003), Arrieta et al (2000) and Jubert et al
(1994) found the frequent association of hepatitis C virus
and OLP, and suggested that HCV occasionally replicates
in OLP tissue contributing to the pathogenesis of mucosal
damage. Lichen planus is often associated with immune Erosive lichen planus affecting the tongue.
Courtesy: Dr Sumanth
mediated diseases like alopecia areata, dermatomyositis,
148
Figure 19 Figure 20
Desquamative gingivitis in erosive lichen planus. B

Courtesy: Dr Ashok
lichen sclerosis et atrophicus, morphea, myasthenia gravis,

ulcerative colitis and primary biliary sclerosis.
Syndromes associated with OLP

Grinspan syndrome is the association of OLP with diabetes
mellitus and hypertension. Graham-Little syndrome and
vulvovaginogingival syndrome are other syndromes asso-
ciated with OLP, in which there is mucosal involvement of
gingival and genital region, usually of erosive type.
Differential diagnosis of reticular/annular type of OLP
includes lichenoid drug reaction, electrogalvanic white
lesions and frictional keratosis and leukoplakia. Keratotic
forms of lichen planus can be differentiated from leuko- (A) Brown colored pigmentation on the buccal mucosa in
plakic plaque as the former is usually associated with pigmented lichen planus. Courtesy: Dr Ashok. (B) Grayish
burning sensation and association with etiologic factors black pigmentation on the buccal mucosa bounded by
in the latter. Hypertrophic form also resembles hyperplas- reticular lichen planus. Courtesy: Department of
tic candidiasis. Erosive lichen planus resembles lupus Oral Medicine and Radiology, MCODS, Mangalore
erythematosus.
Oral lichenoid reactions are identical clinically and
histologically with OLP but have an identifiable etiology
differentiated from other forms like pemphigus, pemphi-
like drugs and dental restorative materials including
goid and linear IgA disease.
amalgams, composite resins, cobalt and gold have been
Erythematous lesions of OLP can be excluded from
implicated as the causes of lichenoid reactions. Even
atrophic candidiasis by the presence of whitish striations
flavoring agents and plastics can be important in the
at the periphery.
pathogenesis and management with oral lichenoid reac-
tion. Patch test may be of help but lacks sensitivity and
Investigations
specificity.
Erosive lichen planus has to be differentiated from Diagnosis is generally achieved by the characteristic clini-
lichenoid reaction, graft-versus-host disease, discoid lupus cal presentation and the complete history and the extraoral
erythematosus and speckled leukoplakia. Desquamative manifestations. Sometimes biopsy may be complementary.
gingivitis of gingival erosive lichen planus should be Gingival lichen planus is often difficult to diagnose and
149
Figure 21
A B
(A) Diffuse red patches surrounded by white striae in atrophic lichen planus involving the buccal mucosa.
(B) Diffuse erythematous areas in atrophic lichen planus involving the tongue. Courtesy: Department of
(Max-Joseph space) and, rarely, clinical blistering of the

Figure 22
oral mucosa (bullous lichen planus). Immunofluorescence
of perilesional mucosa shows the fibrin and shaggy fibrin-
ogen in a linear pattern at the basement membrane zone
and cytoids in the absence of deposition of fibrinogen.
Malignant potential of OLP

First report of a gingival cancer diagnosed in patient with
OLP was in 1910 by Hallapeau. The most important com-
plication of OLP is the development of oral squamous cell
carcinoma from the non-keratotic forms. The reported fre-
quency of malignant transformation varies greatly, between
0.4 and over 5%, over periods of observation from 0.5 to
over 20 years.
Saw tooth rete pegs and subepithelial band of chronic
inflammatory cells, characteristic of lichen planus. Courtesy: Prognosis
Department of Oral Pathology. MCODS, Mangalore
Cutaneous lesions are self-limiting and pigmentation may
fade after few years or remain permanent. Complete remission
direct immunofluorescent studies are confirmatory as they occurs in 70% of cases after 1 year. Oral lesions are chronic,
demonstrate presence of autoantibodies. rarely undergo spontaneous remission, furthermore, erosive
Histopathological features include the essential features oral lesions are difficult to palliate. The spontaneous remis-
like superficial band-like infiltrate of T lymphocytes, sion of OLP is much less than 5% of patients over 7.5 years
basal cell liquefaction, degeneration and epithelial matu- follow up. The reticular form of LP has best prognosis because
ration pattern being normal (Figure 22). Additional fea- spontaneous remission occurs in 40% of cases. The erosive
tures include jagged (saw-tooth) and spindly rete ridges, form of the lesion can persist for 15–20 years.
colloid (Civatte, hyaline, cytoid) bodies, and separation of
epithelium from the lamina propria. Degeneration of basal
Management (Flowchart 2)
keratinocytes and disruption of the epithelial basement
membrane and basal keratinocyte anchoring elements in Treatment should be considered after the evaluation of
OLP produce weaknesses at the epithelial-connective tissue clinical type, associated symptoms, and age. Reticular
interface which may result in histological cleft formation lesions are asymptomatic and require no therapy but only
150
Flowchart 2
Oral lichen planus
Relief in
Topical antifungal therapy symptoms
Symptomatic with clotrimazole 1% (Candid) Asymptomatic
cream t.i.d./day for a week
No relief in Regular follow-up

symptoms once in 3 months
New cases Recalcitrant cases
Intralesional steroids:
Topical corticosteroids (increasing order of potency) Injection triamcinolone 0.5 ml
1. Triamcinolone acetonide 0.1% (Tess gel, Ledercort (Amcort) with once/week
ointment) t.i.d./day until symptoms improve for 4 weeks
(maximum of 1 month)
2. Fluocinolone acetonide 0.025% (Fluzone cream)
t.i.d./day for 2 months with tapering dose
Immunomodulation:
3. Clobetasone propionate 0.05% (Clobetol cream,
chloroquine (Lariago, Laquin)
Cosvate gel) b.i.d./day for 2 months
250 mg b.i.d. for 3 months
4. Clobetasone 17-butyrate (Eumosone) 0.05% b.i.d./day
for 1st month, o.d. for 2nd month
5. Mometasone furoate 0.1% (Mosone cream)
Immunosuppression:
Azathioprine (Imuzat, Azoran)
No relief in 1 mg/kg/day (50 – 100 mg) for
symptoms one month. Dosage can be
increased up to 2 mg/kg/day
and should not be given
Combination therapy (systemic + topical) more than 3 months
1. Tab prednisolone 10 mg (Wysolone, Emsolone)
t.i.d. for 2 weeks; 10 mg b.i.d. for 1 week; 10 mg o.d.
for 1 week; 5 mg o.d. for 1 week & 5 mg once in
2 days (thrice) PUVA therapy
2. Topical triamcinolone acetonide 0.1% (Tess gel) (Psoralen ultraviolet A rays)
t.i.d./day till symptoms improve or cyclosporine
solution (Sandimmune) as mouthrinse twice/day
for 15 days Newer drugs like mycophenolite
mofetil 500 mg (Cellmune),
tacrolimus, sirolimus,
pimecrolimus can be tried (long-
term side effects not studied)
Note: All available medications have been enlisted. however, the physician should
Management of oral lichen planus
observation for changes. In general, all treatment should be eliminated. As gingival lesions may exacerbate with the
be aimed at eliminating atrophic and ulcerative lesions, local factors due to Koebner’s phenomenon, an optimal
associated symptoms, and minimize the risk of malignant atraumatic oral hygiene program should be instituted.
transformation. The most commonly employed and useful agents are
All precipitating factors like mechanical irritants (sharp topical corticosteroids and lack of their adherence to mucosa
fillings, metallic restorations, ill-fitting dentures) should has made preferable to use rinses.
151
Topical corticosteroids reduce pain and inflammation. anxious and depressed patients psychotherapy should be
Triamcinolone acetonide 0.1% in Orabase, oral suspension considered.
of triamcinolone, high potency steroid mouthwashes like
betamethasone valerate 0.1%, fluocinolone acetonide 0.1%, Surgery
clobetasol propionate 0.05% have been used effectively.
Excision, C02 laser, cryosurgery and photochemotherapy
Systemic corticosteroids should be reserved for recalci-
have been effective for persistent or dysplastic lesions.
trant erosive or erythematous lichen planus, where the topi-
However surgery may lead to worsening OLP presumably
cal approaches have failed or wide spread involvement of
via a Koebner phenomenon and reportedly causes a high
skin, genital, esophageal or scalp involvement.
rate of recurrence.
Daily dosages of 40–80 mg of prednisolone can be used
for brief period of time, i.e. 5–7 days and stopping abruptly
Miscellaneous treatments
or the dosage should be reduced by 5–10 mg per day grad-
ually over a 2–4 week period. When using most potent Antibiotics like 2% aureomycin mouthwash, antimalarials
steroids, the medication is not indicated for more than like hydroxychloroquine sulfate, azathioprine, dapsone,
15 days. For intractable erosive lesions intralesional injec- interferons in the form of topical (human fibroblast inter-
tions of triamcinolone acetonide (10–20 mg/ml per vial) feron beta and human fibroblast interferon alpha) and sys-
for every 2–4 weeks has been found effective, but results temic (IFN—3–10 million for 3 weeks) have been found
in severe pain. Hydrocortisone, methylprednisolone and effective but lack enough clinical trials. Thalidomide in the
dexamethasone can also be tried. dosage of 100–150 mg per day has shown to be effective
One third of OLP patients who are treated with topical in resolution but not preferred due to serious side effects.
steroids develop secondary candidiasis, which necessitates As no therapy is curative and the goal for symptomatic
treatment with antifungals. patients is palliation. Relief can be achieved in the major-
ity of patients with topical steroid alone or in combination
Immunosuppressive and immunomodulating with other immunomodulatory topical agents. Atrophic/
agents erosive forms of OLP have to be systematically and peri-
odically reviewed for malignant transformation.
Many immunosuppressive agents like cyclosporine 100 mg/ml
may be used as mouthrinse or finger rub application using
Lichenoid drug reaction
very low doses of cyclosporine 48 mg/day in adhesive base
was found to be effective in suppressing T cell cytokine Lichenoid drug reactions and lichen planus exhibit similar
production. clinical and histologic findings. Clinically they demon-
Treatment of OLP with immunomodulators like levami- strate erythematous erosions and ulcerations with focal
sole 150 mg per day for 3 days along with prednisolone areas of radiating lines.
has shown excellent response and long-term remission. The lichenoid reactions are distinguished from lichen
Tacrolimus, a steroid free topical immunosuppressive agent planus by two factors; the association with the adminis-
approved for the treatment of atopic dermatitis is 10–100 tration of a drug, contact with a metal or foodstuff, or sys-
times effective than cyclosporine. Topical tacrolimus 0.1% temic disease, and their resolution when the offending
has shown resolution of erosive lesion in 14% of patients agent is eliminated (Figure 23A, B). The increased preva-
as observed by an independent study, but causes side lence of oral lichenoid drug reactions are perhaps because
effect of burning sensation. The US Food and Drug of increased use of newly introduced drugs and appear-
Administration recently issued a health advisory to inform ance of lesions similar to OLP and spontaneous remission
healthcare providers and patients about a potential cancer after the withdrawal of the drug.
risk from the use of tacrolimus. Diagnosis depends upon establishing the relationship
Topical retinoids like isotretinoin gel 0.1% that act by between the onset and the use of offending agent and res-
down regulation of fibroblast function has been found to olution of the symptoms upon withdrawal of the offend-
be of little use when compared with topical steroids. ing agent A careful drug history is complementary to the
diagnosis. Histology may be beneficial as lichenoid lesions
Other treatment modalities may have a more diffuse lymphocytic infiltrate and contain
eosinophils and plasma cells, and there may be more col-
Psoralens and long wave ultraviolet A (PUVA) therapy with
loid bodies than in classical LP.
8-methoxy psoralen and photochemotherapy have shown
excellent results. To avoid PUVA side effects photosensitizer
Agents causing lichenoid reactions
with topical 0.01% trioxsalen can be used.
But the side effects of therapy include oncogenic The most commonly used drugs that are implicated in
potential, nausea, vomiting, dizziness and headache. The lichenoid reactions include antiarthritics, antihypertensives,
psychological factor should also be considered. In highly antimicrobials, antiparasitics, anxiolytics, non-steroidal
152
Figure 23
A B
(A) Erythematous area on the buccal mucosa in relation to an amalgam restoration. (B) Lichenoid reaction on the buccal
mucosa in relation to a tooth that was previously restored with silver amalgam restoration. Courtesy: Department of
anti-inflammatory drugs, oral hypoglycemic agents, urico- species may also be involved, such as C. tropicalis, C.
suric agents. Lichenoid reaction may develop after months parapsilosis, C. stellatoidea, C. krusei, C. glabrata, C. pseu-
or even years after taking the drug. tropicalis and C. guilliermondii. Candida albicans is a rela-
Dental materials like amalgam compounds, cobalt, tively common inhabitant of the oral cavity, gastrointestinal
gold, acrylic and casting alloys have been known to cause tract and vagina. Candidiasis is the most common oppor-
lichenoid reaction. tunistic infection in the world. Up to 60% of healthy, non-
Some authors report that a low frequency of sensitiza- hospitalized individuals may harbor this pathogen in the
tion to mercury and no beneficial effects from the removal oropharyngeal region. The advent of HIV infection and
of silver amalgam fillings, whereas others suggest that AIDS has resulted in a resurgence of oral candida infec-
sensitization to mercury is an important cause. In cases tions that are usually seen in very young, the very old, and
where patch test negative patients improve with amalgam, the very sick. Its occurrence has increased remarkably since
mercury may be acting as an irritant in the pathogenesis the prevalent use of antibiotics, which destroy the normal
of oral lichenoid reaction. Patch testing and biopsies how- inhibitory bacterial flora, and immunosuppressive drugs,
ever cannot accurately predict the response to removal of particularly corticosteroids and cytotoxic drugs. Oral can-
amalgam fillings with those of other material. didiasis or thrush usually remain as a localized disease, but
on occasion it may show extension to the pharynx or even
Betel quid lichenoid lesion to the lungs.
The genus Candida is a collection of some 150 aspo-
A quid-induced lichenoid oral lesion has been reported
rogenous yeast species. Because of their inability to form
among betel quid users. It resembles OLP but there are
asexual stage, they are most often classified among the
specific differences. It is characterized by the presence of
fungi imperfect in the class Deuteromycetes. C. albicans is
fine, white, wavy parallel lines that do not overlap or criss-
a dimorphic fungus existing both in the blastospore phase
cross, are non-elevated, and in some instances radiate from
and the hyphal or mycelial phase. C. albicans is the most
a central erythematous area. The lesion generally occurs at
dominant species, followed by C. tropicalis, C. glabrata,
the site of placement of the quid. These lesions may regress
C. parapsilosis and C. krusei. Other Candida species and
with decrease in frequency, duration, or change in site of
genera are rare and transient.
placement of the quid. There may be complete regression
of the lesion when the habit is given up.
Epidemiology
Candida is commensal organism and part of the normal oral
Candidiasis
flora in about 30–50% of the population, and is capable of
Candidiasis is a disease caused by infection with a yeast producing opportunistic infections within the oral cavity
like fungus, Candida (Monilia) albicans, although other when appropriate predisposing factors exist.
153
Etiology and pathogenesis ❍ Heavy smoking

❍ Oral epithelial dysplasia
Candida is predominantly an opportunistic infectious agent,
the role of candida as opportunistic invader versus etio-
logic agent in the patients with oral white lesions has not Classification of Candidiasis (Greenberg and Glick, 2003)
clearly been established. However the demonstration of
Pseudomembranous type
the catalytic role of some candida strains in endogenous Atrophic (erythematous)—antibiotic stomatitis
cellular nitrosamine production, the statistically signifi-
Atrophic
cant association of certain strains with dysplastic red and
Denture sore mouth
white lesions, and the hyperplastic effects on epithelium of
Angular cheilitis
candida in vitro, indicate that candida may be a carcino- Median rhomboid glossitis
gen or a promoting agent.
Hypertrophic/hyperplastic
The pathogenesis of different biotypes and strains of
Candida leukoplakia
C. albicans varies. Candida albicans produces an enzyme Papillary hyperplasia of palate
called phospholipase; this enzyme is concentrated at the Median rhomboid glossitis (nodular)
tips of the fungal hyphae and localized in the vicinity of
Multifocal
host cellular compartments where active invasion is occur-
ring. A relationship has been suggested between the adher- Syndrome associated
Familial endocrine neoplasia syndrome
ence of C. albicans to surfaces and its ability to colonize
Myositis (thymoma associated)
and cause the disease. The adhesion of C. albicans to oral
mucosal cells might be due to interaction involving diva- Localized
lent cations. The adsorption of macromolecules onto epi- Generalized
thelial cells onto epithelial cells is believed to occur via
electrostatic interactions involving calcium ions and other
ionic groups. The extent and strength of the adhesion Clinical presentation
depends on the initial surface properties of both the organ- Acute pseudomembranous candidiasis Pseudomembra-
isms and substratum involved and can be influenced by nous candidiasis is the most common form of oral candi-
several factors. diasis. The most common sites include buccal mucosa,
Neville et al have identified three general factors that dorsal tongue and palate. It usually follows antibiotic
may lead to clinically evident oral candidiasis. These factors therapy or immunosuppression. A burning sensation usu-
are: (i) the immune status of the host, (ii) the oral mucosal ally precedes the appearance of as soft, creamy white to
environment, and (iii) the particular strain of C. albicans yellow, elevated plaques, that are easily wiped off from the
(the hyphal form is usually associated with pathogenic affected oral tissues and leave an erythematous, eroded, or
infection). Oral candidiasis has been considered as the dis- ulcerated surface which may be tender (Figure 24). Thrush
ease of the diseased. may be seen in neonates and among terminally ill patients,
The following is a list of specific conditions that may particularly in association with serious underlying condi-
predispose a patient to develop oral candidiasis: tions such as leukemia and other malignancies and in HIV
❍ Factors that alter the immune status of the host disease. A possible complication of oropharyngeal thrush
❍ Blood dyscrasias or advanced malignancy is the involvement of the adjacent mucosa, particularly
❍ Old age/infancy those of the upper respiratory tract and the esophagus. The
❍ Radiation therapy/chemotherapy combination of oral and esophageal candidiasis is particu-
❍ HIV infection or other immunodeficiency disorders larly prevalent in HIV infected patients.
❍ Endocrine abnormalities Any mucosal surface may be involved and erythematous
❍ Diabetes mellitus or white areas often develop beneath the partial or complete
❍ Hypothyroidism or hypoparathyroidism dentures. The lesions may involve the entire oral mucosa
❍ Pregnancy or may relatively localized areas where normal cleansing is
❍ Corticosteroid therapy/hypoadrenalism poor. A prodromal symptom of rapid onset of a bad taste
and the loss of taste discrimination is described in adults.
Factors that alter the oral mucosal environment
Differential diagnosis Differential diagnosis of thrush
❍ Xerostomia
include food debris, habitual cheek biting, burns and
❍ Antibiotic therapy
rarely, a genetically determined epithelial abnormality like
❍ Poor oral or denture hygiene
white sponge nevus.
❍ Malnutrition/gastrointestinal malabsorption
❍ Iron, folic acid, or vitamin deficiencies Chronic hyperplastic candidiasis (candida leukoplakia)
❍ Acidic saliva/carbohydrate-rich diets Hyperplastic candidiasis is seen as chronic, discrete raised
154
Figure 24 Figure 26
White elevated plaques on the buccal mucosa and Erythematous velvety area on the palate in a denture
erythematous areas where the white patches were wearer suggestive of atrophic candidiasis.
scraped off. Courtesy: Dr Ashok Courtesy: Dr Ashok
Figure 25 tongue and other mucosal surfaces. The most common eti-
ology is poor denture hygiene, and/or continuous denture
insertion, but it may also be caused by immunosuppres-
sion, xerostomia, or antibiotic therapy.
The most common appearance is that of a red patch or
velvet textured plaque. When atrophic candidiasis occurs on
the hard palate in association with a denture, it is frequently
associated with papillary hyperplasia. Patient may complain
of a burning sensation associated with this type of candi-
diasis; 15–65% of cases are usually associated with angu-
lar cheilitis. And lesions of chronic atrophic candidiasis
have also been frequently reported in HIV-positive and
AIDS patients. Three progressive clinical stages of denture
sore mouth have been described in the literature.
Median rhomboid glossitis Median rhomboid glossitis is
a form of chronic atrophic candidiasis characterized by an
asymptomatic, elongated, erythematous patch of atrophic
Keratotic white plaques on the dorsum of the tongue in mucosa of the posterior mid-dorsal surface of the tongue
chronic hyperplastic candidiasis. Courtesy: Dr Ashok due to a chronic Candida infection (Figure 27). In the past,
median rhomboid glossitis was thought to be a develop-
mental defect resulting from a failure of the tuberculum
lesions that vary from small, palpable translucent whitish impart to retract before fusion of the lateral processes of
areas to large, dense, opaque plaques, hard and rough to the tongue.
touch (Figure 25). A concurrent ‘kissing lesion’ of the palate is sometimes
The most common sites are the anterior buccal mucosa noted (Figure 28). Specific predisposing etiologic factor(s)
along the occlusal line, and laterodorsal surfaces of the for median rhomboid glossitis have not been clearly
tongue. The most common appearance is that of asymp- established.
tomatic white plaques or papules (sometimes against an
Angular cheilitis (perleche) Clinical appearance is that
erythematous background) that are adherent and do not
of red, eroded, fissured lesions which occur bilaterally in
scrape off.
the commissures of the lips and are frequently irritating
Chronic atrophic (erythematous) candidiasis The most and painful (Figure 29). The most common etiology is loss of
common site is the hard palate under a denture (Figure 26), vertical occlusal dimension, but it may also be associated
but atrophic candidiasis may also be found on the dorsal with immunosuppression.
155
Figure 27 Figure 29
Erythematous fissures in the corners of the mouth bilaterally

as seen in angular cheilitis. Courtesy: Department of
A well defined erythematous area on the mid-dorsum of the Oral Medicine and Radiology, MCODS, Mangalore
tongue roughly rhomboidal in shape suggestive of median
rhomboid glossitis. Courtesy: Dr Ashok
immune defects, in which there is persistent mucocutane-

Figure 28 ous candidiasis that responds poorly to topical antifungal
therapy. The main types of this rare disorders include
familial CMC, diffuse CMC, candidiasis endocrinopathy
syndrome, candidiasis thymoma syndrome.
Role of candida in oral carcinogenesis

Candidal leukoplakias may develop into carcinoma. It is
unclear whether the yeast are involved in the development
or transformation of leukoplakia. The Candida organisms
present in the candidal leukoplakia have higher nitrosa-
tion potential than others, which might indicate a possible
role of specific types in the transformation of some leuko-
plakias. The Candida species may be involved in carcino-
genesis by elaborating nitrosamine compounds, which act
either directly on the oral mucosa or interact with other
chemical carcinogens to activate specified protooncogenes
Erythematous areas on the palate in an individual with and thereby initiate oral carcinoma.
candidal lesion on the dorsum of the tongue. Commonly
referred to as ‘kissing lesion’. Courtesy: Dr Ashok Investigations for candidiasis
The diagnosis of oral candidiasis is most frequently made
on the basis of clinical appearance along with exfoliative
Chronic multifocal oral candidiasis This term has been cytology examination.
given to chronic candidal infection that may be seen in
Smear examination This involves the histologic exami-
multiple oral sites, with various combinations, including
nation of intraoral scrapings which have been smeared on
angular stomatitis, median rhomboid glossitis and palatal
microscope glass slides. A 10–20% potassium hydroxide
lesions. All these lesions will be having 1 month duration
preparation (KOH prep) can be used for immediate micro-
with no history of predisposing factors like systemic dis-
scopic identification of yeast cell forms. Alternatively, the
eases, or patient’s receiving any drugs, or radiotherapy.
slide containing the cytologic smear can be sprayed with
These lesions are most commonly seen in chronic smokers
a cytologic fixative and stained using PAS (periodic acid-
in their 5th or 6th decade of life.
Schiff) stain and other slide with Gram’s stain prior to
Chronic mucocutaneous candidiasis (CMC) It is the term microscopic examination. Yeast cell appears dark blue after
given to the group of rare syndromes, with a definable Gram staining and red or purple in PAS staining.
156
Hematologic investigations Since oral candidiasis is been used. Among these tests, ELISA is the standard and
associated frequently with predisposing factors such as ideal test, which uses the purified cytoplasmic protein of
nutritional deficiencies, blood dyscrasias or HIV infections, C. albicans as antigen. The ELISA has been proven to be a
estimation of hemoglobin lymphocyte and white cell counts, sensitive, cheap, and quick method for the quantification of
blood sugar and serum ferritin are important. antibody titers and would undoubtedly be the laboratory
method of choice.
Biopsy A biopsy of affected tissue may be indicated,
especially when candidiasis is suspected in conjunction
Rationale for treatment
with some concurrent pathology, such as candidal leuko-
Topical versus systemic drugs Topical antifungals are usu-
plakia, epithelial dysplasia, squamous cell carcinoma, or
ally the drug of choice for uncomplicated, localized candi-
lichen planus. The sections should be stained with PAS or
diasis in patients with normal immune function. Systemic
Gridley’s or Gomori methenamine silver (GMS), because
antifungals are usually indicated in cases of disseminated
Candida species stain poorly by hematoxylin and eosin.
disease and/or in immunocompromised patients.
Microbiology It is also possible to culture Candida using Medication should be continued for at least 48 hours
a Sabouraud’s dextrose agar, which is used as a primary after the disappearance of clinical signs of candidiasis along
culture medium to aid in the definitive identification of with complete healing and the absence of mucosal ery-
the fungal organism. To permit the distinction between thema. Some sources recommend drug therapy should be
different yeast species Pagano-Levin medium is useful. continued for 10–14 days regardless of the disappearance
Imprint culture technique This technique uses a sterile of clinical signs of candidiasis.
plastic foam pad of known size (2.52.5 cm) dipped in
Suggested medications for the treatment of candidiasis
Sabouraud’s broth and placed on the suspect mucosal sur-
(Flowchart 3)
face for 60 seconds. Then the plastic foam is placed directly
Topical antifungal medications Nystatin is the first spe-
on Sabouraud’s or Pagano-Levin agar. Candida density at
cific antifungal agent effective in the treatment of candi-
each site is determined by a Gallenkamp colony counter
diasis.
and expressed as colony-forming units (CFU) per mm2.
Nystatin taken systemically may lead to occasional GI
Colony counts in excess of 30 CFU cm2 of mucosa in the
side effects such as nausea, vomiting and diarrhea. It can
dentate and 49 CFU cm2 in denture wearers suggested a
be used in rinse form, oral and vaginal tablets or creams.
candidal infection.
Nystatin oral suspension 100,000 units/ml; 300 ml: rinse
Salivary culture technique This involves patients 2 ml of with one teaspoonful (5 ml) for 2 minutes, use q.i.d. (after
mixed unstimulated saliva into a sterile, universal con- meals, and at bedtime) and spit out.
tainer. The number of Candida expressed as CFU per mm Patient can be directed to rinse and swallow if there is
of saliva is estimated by counting the resultant growth on pharyngeal involvement.
Sabouraud’s agar. Amphotericin B is produced by Streptomyces nidus.
Oral rinse technique Here the patient is asked to rinse It has a broad spectrum of activity in humans and remains
the mouth for 60 seconds with 10 ml of sterile phosphate a cornerstone of therapy for systemic fungal infections.
buffered saline or sterile water. The oral rinse is centri- It is available as cream, lotion and ointment and as an
fuged at 1,700 g for 10 minutes and the deposit resuspended intravenous solution. It is usually fungistatic but fungi-
in 1 ml of sterile PBS. The concentrated oral rinse is now cidal in higher concentrations.
Clotrimazole is the most potent topical agent in azole
inoculated on appropriate media to assess CFU per mm of
group of antifungals. It has got GI and neurological toxicity
rinse sample using a spiral plater prior to incubation. This
clotrimazole troches, 10 mg 70 troches; one troche dis-
technique has advantages over the imprint technique
solves in mouth five times per day for 14 days.
because it is simple to perform and it can be used for the
Systemic antifungal medications include ketoconazole
quantitation of other organisms such as coliforms.
tablets, 200 mg 1 tab q.i.d. with a meal or orange juice for
Immunologic tests Immunity in oral candidiasis is pre- 14 days. Ketoconazole is the drug being used in the treat-
dominantly cell mediated. Cell-mediated immunity to ment of chronic mucocutaneous candidiasis and candidiasis
C. albicans antigens can be demonstrated in most human in immunocompromised patients.
subjects both by the appearance of delayed skin hypersen- Fluconazole tablets, 100 mg, 15 tablets; 2 tablets to start,
sitivity to candida antigens and by in vitro tests of cellular then 1 tablet q.i.d. for 14 days, oral absorption of flucon-
immunity such as inhibition of leukocyte migration or azole is rapid and nearly complete within 2 hours.
stimulation of lymphocyte transformation to candida anti- Itraconazole tablets, 100 mg, 1 tablet b.i.d. with a meal
gens. As test of humoral immunity, the candida agglutinin or orange juice for 14 days. This drug has a long half-life
test, the candida complement fixation test, the candida and fewer side effects than ketoconazole but is expensive.
precipitin test, immunofluorescence, and ELISA test have Its use is contraindicated in liver diseases.
157
Flowchart 3
Oral candidiasis Systemic candidiasis
If such rectification is not Rectify the underlying

possible (AIDS, diabetes) systemic/local cause
Physician opinion
sought
Topical route (denture stomatitis, angular Parenteral route Oral route (pseudomembranous,
cheilitis, median rhomboid glossitis) hyperplastic, acute atrophic)
1. Clotrimazole 1% cream (Candid, Canesten) five 1. Fluconazole 150 mg (Flucole, Candizole)

times/day for 2 weeks b.i.d. for 14 days
2. Clotrimazole 2% gel (Candid V) five times/day 2. Fluconazole 200 mg (Nuforce, Onecan)
for 2 weeks b.i.d. for 14 days
3. Clotrimazole 1% solution (Canesten) five 3. Ketoconazole 200 mg (Ketozole, Fungicide)
times/day for 2 weeks o.d. for 1–4 weeks
4. Nystatin 5 lakh unit tablets (Mycostatin) four 4. Itraconazole 100 mg (Candistat)
times/day for 14 days – crush & mix with water & o.d. for 14 days
use as mouthrinse & swallow
5. Hamycin 2 lakh unit/ml (Hamycin suspension)
2–3 times/day as mouthrinse for 7–10 days
6. Fluconazole dispersible tablet (Nuforce) with
water – to use as mouthrinse 3 times/day for
14 days
Amphotericin B IV infusions 0.3 mg/kg

(Fungisome – 10 mg, 25 mg, 50 mg)
can be infused over 4–8 hrs
Note: All available medications have been enlisted. However, the physician should
Management of candidiasis
All the azole group of drugs are fungistatic, and not Nystatin–triamcinolone acetonide ointment or clotrima-
fungicidal. This is an important consideration when treat- zole cream 1% or miconazole cream 2% or ketoconazole
ing the chronically immunosuppressed, such as those with cream 2% can be applied to affected areas q.i.d. (after
HIV and patients with candidal meningitis. meals, and at bedtime) for 14 days.
Treatment for chronic atropic candidiasis Application Complementary and alternative medicine Garlic cap-
of a thin coat of medicines like nystatin ointment or sules. Garlic may have antifungal and antibacterial prop-
clotrimazole cream 1% or miconazole cream 2%, ketocon- erties. One study found that ajoene, a compound obtained
azole cream 2% to entire inner surface of denture after from garlic, was as effective in treating the fungus that
each meal for 14 days usually results in remission. causes athlete’s foot.
Instruct the patient to leave dentures out at night and Caprylic acid capsules. This fatty acid, derived from
to soak denture in a 1% sodium hypochlorite solution for coconut oil, has been shown to have antifungal properties.
15 minutes with thorough rinsing under running water for These are generally safe, but should not be used in patients
at least 2 minutes, before bedtime. with ulcerative colitis.
158
Oral Submucous Fibrosis pulse rate and they elicit a degree of euphoria by virtue of
their GABA receptor inhibitory properties which contribute
Oral submucous fibrosis (OSMF) is an insidious, chronic dis- to dependence and habituation. There are also broncho-
ease affecting any part of the oral cavity, and sometimes constrictor effects, and evidence for a role in precipitating
the pharynx. Occasionally it is preceded and/or associated and exacerbating asthma and diabetes. This arecoline
with vesicle formation and always associated with a juxta- plays a major role in the pathogenesis of OSMF by causing
epithelial inflammatory reaction followed by progressive an abnormal increase in the collagen production.
hyalinization of the lamina propria. The later subepithelial In genetically predisposed people, betel nut and pan
and submucosal myofibrosis leads to the stiffness of the chewing render the oral mucosa susceptible to chronic
oral mucosa and deeper tissues with progressive limitation inflammatory changes with decreased T-lymphocyte count
in opening of the mouth and protrusion of the tongue. and higher null cell count. Areca nut, chilli and misi are
Oral submucous fibrosis is one of the most prevalent the chief local factors in the production of OSMF.
premalignant condition in India which is easy to diagnose The role of chilli ingestion in the pathogenesis of OSMF
but difficult to manage. At present it is considered as irre- is controversial. A hypersensitivity reaction to chilies is
versible and incurable. believed to contribute to the occurrence of OSMF, explained
According to Pindborg and Sirsat (1966), OSMF is an by some authors as allergen induced eosinophilia due to
insidious chronic disease affecting any part of the oral capsaicin.
cavity and sometimes the pharynx. Although occasionally Misi, a black colored powder containing the substances
preceded by and/or associated with vesicle formation, it is like soda, borax, powdered alum, charcoal of myrobalan and
always associated with juxta-epithelial inflammatory reac- fillers earth in varying proportion is predominantly used
tion followed by a fibroelastic change of the lamina pro- by females in Uttar Pradesh is known to induce OSMF.
pria with epithelial atrophy leading to stiffness of mucosa The flavonoid catechin and tannins from betel nut sta-
and causing trismus and inability to eat. bilizes the collagen fibers and makes them resistant to
degradation by collagenase.
Epidemiology Nutritional deficiency: Malnutrition is more prevalent
The disease occurs mainly in Indians. It affects between in OSMF. Several investigators have reported anemia,
0.2–1.2% of urban population attending dental clinics in vitamin, iron and protein deficiencies among OSMF
India. The disease should be a cause of concern in coun- patients. Iron metabolism seems to be the primary factor
tries with large migrant populations from south-east Asia. and deficiency in folic acid, pyridoxine, and vitamin B12
Worldwide estimates in 1996 indicate that 2.5 million peo- deficiencies are secondary.
ple are affected by the disease. The genetic factors like increased factors like HLA DR 10,
In ancient medicine Shushrutha described a condition, DR3 and DR7 have been reported. Immunological studies
‘Vidari’ under mouth and throat diseases. Schwartz (1952), have shown raised immunoglobulin like A, E and D.
for the first time reported a case of ‘atropia idiopathica Autoantibodies to gastric and parietal cells, as well as
tropica mucosae oris’ occurring in Indians in east Africa. thyroid microsomal, antinuclear, reticulin, and antismooth
Lal and Joshi (1953) first described this condition in India muscle antibodies have been found in 65% of patients
and termed it as OSMF. with the disease.
Increased levels of proinflammatory cytokines and
reduced antifibrotic interferon gamma (IFN-) in patients
Etiopathogenesis
with OSMF, which may be central to the pathogenesis of
Pathogenesis of the disease is still not clear, and it is OSMF, has been demonstrated.
believed to be multifactorial. The exact role of any of the Matrix metalloprotein: The genomic studies have shown
etiologic factor in the development, severity and progress the 5A genotype of MMP3 promoter was associated.
of the disease is not clear as the disease may still progress Studies have shown that six collagen related genes includ-
even if none of these are present. The chewing of betel nut ing COL1A1, COL1A2, COLase, LYOXase, TGF-1, and CST3
has been recognized as one of the most important etio- are found to be located on different chromosomes in OSMF
logical factor for the causation of OSMF. patients.
The chewing of areca nut in various forms and mix- Role of saliva: Trivedi suggested the involvement of
tures is deeply embedded in the social and cultural history trace metal copper in the molecular pathogenesis of OSMF
of India and other south-east Asian countries. Its use as they found the high levels of copper expression in
appears in ancient Sanskrit literature. Areca nut contains saliva could act as initiating factor and stimulation of
potent cholinergic muscarinic alkaloids, notably arecoline fibrogenesis by up regulation of lysyl peroxide.
and guavacoline, with a wide range of parasympathetico- According to the study conducted by Chaturvedi (1991),
mimetic effects, they promote salivation and the passage saliva was found to contain alkaloids, tannins, nitrates,
of wind through the gut, they rise blood pressure and thiocyanins, nitrosamines and have carcinogenic activity.
159
They found that chewing betel nut releases copper into the b. No restriction in mouth opening
saliva that stimulates lysyl oxidase enzyme lead to fibrosis. c. No restriction in tongue protrusion up to mesioincisal
angle of upper central incisor when maximally extended
Molecular pathogenesis of OSMF with mouth wide open.
(Rajalalitha and Vali, 2005) d. Burning sensation only on taking spicy food or hot
liquids.
Collagen is the major structural component of the connec-
tive tissues and its composition within each tissue needs to
be maintained for proper tissue integrity. The synthesis of
collagen is influenced by a variety of mediators, including Figure 30
growth factor, hormones, cytokines and lymphokines. A
prominent mediator is transforming growth factor-beta
(TGF-). The growth factor has also been implicated in the
development of many fibrotic diseases. It causes the depo-
sition of extracellular matrix by increasing the synthesis
of matrix proteins like collagen and decreasing its degra-
dation by stimulating various inhibitory mechanisms. So
transforming growth factor beta signaling pathway might
be critical for pathogenesis of OSMF.
Clinical features
Oral submucous fibrosis is very commonly seen among
the Indians. Indians who have settled in other countries
and to lesser extent in other Asiatic people. Several
Europeans in Europe, India and Africa have also been
mentioned as victims of the disease. Epidemiological sur-
veys of Indian population have revealed that incidence
Pallor or marble-like appearance of the oral mucosa in OSMF.
varied in the range of 0.04–0.4% and in urban population Courtesy: Dr Ashok
0.18–1.2%. Overall prevalence of up to 0.4% in Kerala. The
common age of occurrence varies from 12–62 years with
the mean age being 40 years but there are reports of OSMF
even in as younger as 4-year old. There is female predilec- Figure 31
tion, with the ratio being 3:2.
First and the foremost feature of OSMF is burning sen-
sation and pallor or blanching of oral mucosa. Intraoral
sites of involvement include the buccal mucosa (Figure 30),
retromolar area, followed by soft palate, palatal fauces,
uvula, tongue (Figure 31) and labial mucosa (Figure 32A, B).
There may be stiff and small tongue, blanched and leath-
ery floor of the mouth (Figure 33), fibrotic and depig-
mented gingiva, rubbery soft palate with decreased
mobility and blanched and atrophic tonsils, and shrunken
bud like uvula. Mouth opening may become progressively
reduced (Figure 34).
Other symptoms include increased salivation, change of
gustatory sensation, hearing loss due to stenosis of the
Eustachian tubes, dryness of the mouth, nasal tonality to
the voice. Dysphagia to solids (if the esophagus is involved),
impaired mouth movements (e.g. eating, whistling, blow-
ing, sucking) (Figure 35).
Staging of OSMF
Stage 1: Early OSMF Depapillation and pallor associated with the tongue in OSMF.
Courtesy: Dr Ashok
a. Mild blanching
160
Figure 32
A B
Pallor of the upper and lower labial mucosa in OSMF. Courtesy: Dr Ashok
Figure 33 Figure 34
Pallor of the ventral surface of tongue and floor of mouth in

OSMF. Courtesy: Dr Ashok
Stage 2: Moderate OSMF Difficulty in mouth opening due to the presence of

circumoral fibrous bands in OSMF. Courtesy: Dr Ashok
a. Moderate to severe blanching
b. Mouth opening reduced by 33%, tongue protrusion
reduced by 33% and reduced flexibility d. Thick palpable bands
c. Burning sensation even in the absence of stimuli e. Bilateral lymphadenopathy, definite nutritional com-
d. Presence of palpable bands promise can be established in B complex (angular
e. Lymphadenopathy either unilateral or bilateral cheilitis) and iron deficiency group.
f. Demonstrable anemia on hematological examination.
Khanna and Andrade (1995) grouped OSMF into different
Stage 3: Severe OSMF stages:
a. Burning sensation very severe Group 1: Very early
b. More than 66% reduction in the mouth opening, cheek ❍ Mouth opening is normal
flexibility and tongue protrusion. In much tongue ❍ Burning sensation
may appear fixed ❍ Excessive salivation
c. Ulcers over the buccal mucosa ❍ Acute ulceration and recurrent stomatitis.
161
Figure 35 ❍ Lips circular band felt around the entire mouth

❍ Difficult intraoral examination
Group 5: Advance cases with premalignant and malignant
changes
❍ OSMF and leukoplakia
❍ OSMF, squamous cell carcinoma
Haider (2000) staged OSMF clinically and functionally as

follows:
Clinical Staging
Stage 1: Faucial bands only
Stage 2: Faucial and buccal bands
Stage 3: Faucial and labial bands
Functional Staging
Stage A: Mouth opening 13–20 mm
Stage B: Mouth opening 10–12 mm
Stage C: Mouth opening less than 10 mm.
Inability of the patient to blow his cheeks demonstrating
inelasticity of the oral mucosa in OSMF. Malignant potential and oral submucous fibrosis
Courtesy: Dr Ashok
Various authors in the past have suggested OSMF as a
precancerous condition. According to Pindborg, atrophy
Group 2: Early cases of the epithelium increases the vulnerability of the action
❍ Mouth opening: 26–35 mm (interincisal opening) of carcinogens. Due to irritation by exogenous factors, the
❍ Soft palate and faucial pillars were the areas primarily atrophic epithelium undergoes hyperkeratinization, there
affected is intercellular edema in the prickle cell layers and the
❍ Buccal mucosa appeared mottled and marble like basal cells undergo hyperplasia. After this carcinoma can
where dense pale, depigmented fibrosed areas alter- develop at any stage. Congestion of the blood vessels due
nated with pink normal mucosa to excessive fibrosis in the connective tissue compromises
❍ Red erythematous patches the blood supply. Some have demonstrated abnormal
❍ Widespread sheets of fibrosis. expression of P-53 tumor suppressor gene as detected by
Group 3: Moderately advanced immunohistochemistry in the epithelium of OSMF.
❍ Mouth opening 15–25 mm (interincisal opening)
Other diseases associated with OSMF
❍ Trismus
❍ Vertical fibrous bands could be palpated and firmly There are reports of concomitant occurrence of oral lichen
attached to underlying tissue planus, leukoplakia, pemphigus and squamous cell carci-
❍ Unable to blow out their cheeks and whistle noma in the literature.
❍ Soft palate—the fibrous bands were seen to radiate
from the pterygomandibular raphe Histopathologic changes
❍ Or the anterior faucial pillars in a scar like appearance
Epithelial changes Increase in the clinical severity of the
❍ Lips—atrophy of vermillion border
disease may be accompanied by epithelial hyperplasia or
Unilateral posterior cheek involvement with only ipsilat- atrophy (Figure 36), which is associated with an increased
eral involvement of the faucial pillars and soft palate and tendency for keratinizing metaplasia. Deep invagination
opening reduced to 15–18 mm. of epithelial pegs into underlying lamina propria, lique-
faction degeneration of basal cells have been reported.
Group 4: Advanced cases
❍ Stiffness/inelasticity of oral mucosa Connective tissue changes The connective tissue changes
❍ Trismus include hyalinization, with moderate number of chronic
❍ Mouth opening 2–15 mm (interincisal opening) inflammatory cells.
❍ Fauces thickened, shortened and firm on palpation The most striking feature of connective tissue is the
❍ Uvula was seen to be involved, shrunken, small, and presence of dense collagen bundles, randomly oriented
fibrous band and extending into the underlying striated muscles.
❍ Tongue movement restricted Inflammatory cell infiltration includes lymphocytes,
❍ Papillary atrophy (diffuse) monocytes, plasma cells, and occasionally macrophages.
162
Figure 36 melanocytes, a feature which explains the clinically observ-

able loss of pigment. Absence of fibroblasts within the
hyalinized zones, total loss of epithelial rete pegs, and
extensive degeneration of muscle fibers.
Investigations
Diagnosis of the disease is by clinical findings and con-
firmed by incisional biopsy. Many investigations have been
suggested by various authors, that include hematological,
serological, immunological and biochemical factors. Other
laboratory findings include a raised ESR, slight eosino-
philia, microcytosis and hyperchromic indicative of anemia.
Cytologic smears may be performed.
A neural network-based oral precancer stage detection
method has been proposed. This new technique uses wave-
Atrophic epithelium and the hyalinization and let coefficients from transmission electron micrography
homogenization of collagen fibers. Courtesy: images of subepithelial fibrillar collagen in normal oral
Department of Oral Pathology, MCODS, Mangalore submucosa and in OSF tissues. These wavelet coefficients
are used to choose the feature vector, which, in turn, can be
used to train an artificial neural network. This trained net-
Studies have reported thickened basement membrane work is able to classify normal and oral precancer stages
with marked reduction in the vascularity in the connective (less advanced and advanced) after obtaining the image as
tissue which was inversely proportional to increased density an input. It may be used as an adjunct to hematoxylin and
of collagen which appeared hyalinized. eosin histologic evaluations in the near future.
Histological staging of OSMF Differential diagnosis

Khanna and Andrade (1995) have classified the histologi- Oral manifestations of scleroderma: Scleroderma can be
cal findings of OSMF into four groups. distinguished by other cutaneous, systemic, and charac-
teristic radiographic and laboratory findings.
Group 1: A fine fibrillar collagen network with marked
Anemia: Pale oral mucosa can mimic atrophy and
edema, blood vessels dilated and congested. Large aggre-
fibrosis.
gate of plump, young fibroblasts containing abundant
Amyloidosis: Hyalinized stroma can be distinguished
cytoplasm. The inflammatory cells consist of PMNLs with
from amyloid infiltration by using Congo red and thiofla-
few eosinophils, normal epithelium with some hyperplastic
epithelium. vine T staining under polarized and immunofluorescent
light.
Group 2: The juxta-epithelial area shows early hyalin- Generalized fibromatosis: Although soft tissue masses
ization. Collagen still appears as separate bundles and are not produced in the usual sense, the fibrosis of OSMF
thickened. Plump young fibroblasts are present in moder- may be confused with generalized fibromatosis.
ate numbers. The blood vessels are dilated and congested.
The inflammatory cells are mononuclear lymphocytes, Treatment (Flowchart 4)
eosinophils and occasional plasma cells; flattening and
shortening of the epithelial rete pegs with varying degree Since the exact etiology is unknown, various treatment
of keratinization. modalities have been tried from time to time. The treat-
ments of the condition include avoidance of habits but
Group 3: Juxta-epithelial hyalinization. Thickened col- there is no reversal of fibrosis.
lagen bundles are fairly describable, separated by edema. Management includes the following medications:
Blood vessels constriction, fibrocytes with scanty cyto-
plasm and spindle-shaped nuclei and atrophic epithelium Corticosteroids These agents can be used in pharmaco-
with total loss of rete pegs. Muscle fibers seem to be inter- logic doses for their anti-inflammatory and immunosup-
spersed with thickened and dense collagen fibers. The pressant properties and their effects on blood and lymphatic
degeneration of muscle fibers begin. systems in the palliative treatment. In patients with mod-
erate OSMF, weekly submucosal intralesional injections or
Group 4: Hyalinization of collagen bundles as smooth topical application of steroids may help prevent further
sheet, obliteration of blood vessels and decreased/loss of damage.
163
Dexamethasone (Decadron): Adult dose 4 mg IV/IM. and topical hyaluronidase shows better long-term results
Decreases inflammation by suppressing migration of poly- than either agent used alone.
morphonuclear leukocytes and reducing capillary perme-
ability. Interferon-gamma This plays a role in the treatment of
patients with OSMF because of its immunoregulatory effect.
Placental extracts The rationale for using placental IFN- is a known antifibrotic cytokine. Patients treated with
extract in patients with OSMF derives from its proposed an intralesional injection of IFN- experienced improve-
anti-inflammatory effect, hence, preventing or inhibiting ment of symptoms. IFN-, through its effect of altering col-
mucosal damage. Cessation of areca nut chewing and sub- lagen synthesis, appears to be a key factor to the treatment
mucosal administration of aqueous extract of healthy of patients with OSMF, and intralesional injections of the
human PE (Placentrex) showed marked improvement of cytokine may have a significant therapeutic effect on OSMF.
the condition. Surgical care: Surgical treatment is indicated in patients
Hyaluronidase The use of topical hyaluronidase has with severe trismus and/or biopsy results revealing dys-
been shown to have quicker improvement in symptoms plastic or neoplastic changes. Surgical modalities that have
compared with steroids alone. The combination of steroids been used include the following:
Flowchart 4
OSMF
Stage I Stage II & III Stage IV
Systemic:
1. Iron supplements (Dexorange Syrup, Haemup Syrup/Capsules)
2. Antioxidant capsules b.i.d. for 3 months (Antoxid, Altomin Xl, Lynet, Revup, Oxyace, Oxidix)
Topical: Intralesional injections: No medicinal treatment

1. Benzydamine 0.15% mouth 1. Dexamethasone + can be given
rinse (Tantum) Hyaluronidase 1,500 IU 1. Surgical relieving of
2. Triamcinolone gel (Tess) or (Hylase) + Lignocaine fibrous bands with
crushed dexamethasone 2 ml; multiple site buccal pad of fat
tablets in 20 ml of water & injections once/week covering the wound
use as mouthrinse for 6 weeks 2. Laser surgery
Immunomodulators: 2. Betamethasone +
Levamisole 150 mg o.d. for Hyaluronidase 1,500 IU +
3 days – twice in a month Lignocaine + Placentrex
for 3 months (Vermisol, = 3 ml multiple site
Levazole) injections once/week
for 6 weeks
Newer drugs: Physiotherapy:

1. Pentoxifylline 400 mg b.i.d. for 15 days 1. Mouth opening exercises
(Flexital, Flowpent) 2. Mouth opening appliance
2. Interferon gamma injections 3. Ice cream stick
4. Ultrasound
Note: All available medications have been enlisted. However, the physician should
Management of oral submucous fibrosis
164
Simple excision of the fibrous bands: Excision can result Focal Epithelial Hyperplasia (Heck’s Disease)
in contracture of the tissue exacerbating the condition.
Split-thickness skin grafting following bilateral tempo- Heck’s disease is considered among the most contagious
ralis myotomy or coronoidectomy: Trismus associated of the papillary lesions affecting the oral cavity. Presently
with OSMF may be due to changes in the temporalis ten- the etiology for this condition is said to be a subtype of the
don secondary to OSMF; therefore, skin grafts may relieve human papillomavirus, HPV-13, and possibly HPV-32. It
symptoms. Nasolabial flaps and lingual pedicle flaps: is very commonly seen in Eskimos and American Indians
Surgery to create flaps is performed only in patients with and less commonly in white Europeans. The predisposing
OSF in whom the tongue is not involved. The use of vita- factors associated with this condition are poor hygiene,
min supplements, balanced diet and stretching exercises poverty and communal lifestyle. Presence of this condi-
are aimed at increasing the mouth opening. Regular phys- tion among close communities and family members sug-
ical examinations, biopsy specimen analysis, and cyto- gests an infectious pathogenesis.
logic smear testing should be scheduled to detect oral Dos Santos et al (2004) in their article state the prevalence
dysplasia or carcinoma, especially in patients with severe of this condition affecting the oral mucosa was highest in
OSMF. Patients with surface leukoplakia require close fol- Waimiri-Atroari Indians, reaching 21% with no differ-
low-up monitoring and repeat biopsies. Patients with dys- ences between the sexes or among different age groups.
plasia and carcinomas should receive routine treatment Patients of a younger age group presented with multiple
for these entities. lesions, which were predominantly nodular, whereas older
patients had few or even single lesions, which tended to be
Complications Oral dysplasia and squamous cell carci- flat and papular.
nomas are complications of OSMF. In patients with OSMF,
the risk of developing oral carcinoma is 7–14% over a Clinical features
10-year period.
It is usually seen in children and adults. The condition is
If the palatal and paratubal muscles are involved in
characterized by multiple soft, circumscribed, sessile nod-
patients with OSMF, conductive hearing loss may occur
ules of whitish color or a color mimicking the adjacent
because of functional stenosis of the Eustachian tube.
oral mucosa (Figure 37). The common sites of involvement
Prognosis are the lips, buccal mucosa and the lateral borders of the
tongue (Figure 38).
No treatment is effective in patients with oral submucous
fibrosis, and the condition is irreversible. Recent reports
claim improvement of the condition if the habit is discon-
tinued following diagnosis at an early stage.
Figure 37
Psoriasis
Description is given in Chapter 9 (Dermatological
Diseases).
Hereditary Benign Intraepithelial Dyskeratosis

(Witkop’s Disease)
Witkop-von Sallmann disease It is a rare genetic disor-
der characterized by the oral lesions and bilateral con-
junctival plaques. The oral lesions are similar to white
sponge nevus. They appear as thick corrugated asymptom-
atic white plaque involving the buccal and labial mucosa.
Other intraoral sites include floor of the mouth, lateral
tongue, gingiva and palate. The significant feature of this
disease is the formation of corneal plaques that may lead
to blindness.
Differential diagnosis Well circumscribed, sessile nodules mimicking the color of

White sponge nevus and pachyonychia congenita can be the adjacent oral mucosa in Heck’s Disease. Courtesy:
Prof Braz Campos Durso, Brazil
considered in the differential diagnosis.
165
Figure 38 Figure 39
White sponge nevus involving the buccal mucosa.

Well circumscribed, papular lesions on the labial mucosa in Courtesy: Thomas S Pilak, Marquette University, USA
Heck’s disease. Courtesy: Prof Braz Campos Durso, Brazil
White sponge nevus is also known by other names

which include white folded gingivostomatitis, familial white
The diagnosis of Heck’s disease can be made by clinical folded hypertrophy of the mucous membrane, leukokerato-
examination. However histological evaluation may show sis oris, hereditary leukokeratosis, leukoderma exofoliativum
features of viral infection. The condition is said to be asso- mucosae oris and naevus spongiosus albus mucosae.
ciated with HIV infection. However the association between
these two conditions is yet to be substantiated. Suppression Etiopathogenesis
of the immune system leaves the patient vulnerable to
opportunistic infections, including HPV infections. The basic defect lies in the epithelial cell maturation and des-
quamation. Atypical abundance and aggregation of tonofila-
Management ments and increased intracellular attachment cause piling
up of surface cells and keratin. There is also a decreased
No treatment is necessary as it is self-limiting and regresses shedding of keratin which leads to white sponge nevus.
completely. However some treatment modalities that have
been used include surgical or cryosurgical procedures, Genetic basis
electrocoagulation or treatment with carbon dioxide laser.
Steinhoff et al (2001) have used interferon- for suc- There are mutations in genes controlling keratins, i.e. K4
cessfully managing the condition. However, its efficacy in and K13 on chromosomes 12q13 and 17q21–22. There is
the treatment of oral lesions has yet to be adequately also a K4-3bp deletion. Mutations are also seen in K14 and K19
tested. controlling genes. Rugg et al described mutation in genes
N-60 and Richard et al reported mutations in gene L119P.
Dyskeratosis Congenita Clinical features

Description is given in Chapter 9 (Dermatological Usually presents itself at birth or early childhood. There is
Diseases). no sex predilection. Lesions are seen involving the oral
mucosa and other mucosal sites such as the nasal cavity,
esophagus, larynx, vagina and rectum. The common sites
White Sponge Nevus that are affected include buccal mucosa (Figure 39) fol-
lowed by labial mucosa, alveolar ridge and floor of mouth.
White sponge nevus is a hereditary dyskeratotic hyperpla- Gingiva and tongue are rarely affected. Usually it presents
sia of the mucous membranes that shows an autosomal itself as an asymptomatic gray white folded or corrugated
dominant inheritance pattern with irregular penetrance. It spongy mucosal lesions that often have symmetrical
is characterized by variable and some times severe leuko- weavy pattern (Figure 40). They have soft or spongy tex-
keratosis of the oral mucosa. ture and white opalescent hue. Size of the lesion can vary
Hyde in 1909 gave the first description of this condition. from few millimeters to several centimeters. They present
Subsequently in 1935, Cannon named it white sponge nevus. usually as a symmetrically bilateral lesion.
166
Figure 40 Figure 41
White corrugated spongy mucosal lesions on the labial White sponge nevus. Courtesy: Thomas S Pilak,
mucosa in white sponge nevus. Courtesy: Thomas S Pilak, Marquette University, USA
Marquette University, USA
The color of the oral mucosa depends on the thickness

of the oral epithelium, underlying connective tissue con-
It is usually asymptomatic. However it may become
tents like fibrous tissue, vascularity, and the functional
symptomatic by the irritating stimuli like bacterial or yeast
adaptations of the mucosa toward forces of mastication
infection. Patients can complain of pruritus, burning sensa-
and the inflammatory process.
tion or pain.
Healthy masticatory mucosa (gingival, palate, dorsal
surface of the tongue) is light pink in color. The lining
mucosa (mucosa over the vestibule, cheeks, lips, floor of
Various conditions can be considered in the differential diag- the mouth, and ventral surface of the tongue) is reddish
nosis such as leukoedema, leukoplakia, traumatic kerato- pink in color. Palatoglossal arch region is dusky red in
sis, chemical burn, candidiasis, lichen planus, pachyonychia color due to increased vascularity and often misdiagnosed
congenita, Darier’s disease and dyskeratosis congenita. as sore throat.
Histopathologic features Classification of red lesions—congenital and

acquired
Histopathological sections reveal epithelial thickening
showing both acanthosis and hyperkeratosis. The basal Congenital red lesions Vascular malformations like
layer is intact and the spinous cell layer is continuous till hemangiomas, AV shunts.
the surface and shows intracellular edema and pyknotic Acquired red lesions
nuclei are seen. Parakeratin plugging runs deep into the ❍ Erythroplakia
spinous layer. Mild inflammatory cell infiltrate is seen in ❍ Extravasations of blood (trauma or hemostatic disease)
the submucosa (Figure 41). ❍ Atrophy or erosion of the mucosa
❍ Inflammation secondary to vascular dilatation
Treatment ❍ Infections like (cellulitis, secondary syphilis, candidiasis)
Since it is a benign lesion and usually asymptomatic, no ❍ Inflammatory hyperplasias
treatment is required. However in symptomatic cases tet- ❍ Allergic and autoimmune diseases
racycline mouthrinse and penicillins have shown some ❍ Increase in the hemoglobin pigmentation
good results. Traumatic erythematous macules Mechanical trauma to
the oral mucosa can produce a variety of clinical lesions
depending on the nature and severity and the host response.
RED LESIONS OF THE ORAL CAVITY These may be in the form of erythematic macules, hemor-
rhagic nodules, echymoses, erosions and ulcers in the order
Individual variations in the color of the oral mucosa are of severity. Common causes include self-inflicted injury
probably an expression of one or more genetically controlled like cheek bite or some deleterious habits, sharp margins
factors. of the teeth or restorations and ill-fitting prostheses.
167
The most common site of occurrence of the erythema- Lupus erythematosus Lupus erythematosus is a connec-
tous macules are on the anterior and lateral borders of tive tissue disease of unknown cause in which antibodies
the tongue, floor of the mouth, posterior palate, buccal to nuclear constituents are produced to result in the involve-
mucosa, and the mucosal surface of lips. Clinical configu- ment of various organs. Two forms of the disease include
rations of these depend on the offending agent. This could discoid and systemic lupus erythematosus. In Schiodt’s
be either elicited by the history or by clinical examination. study of 32 patients of lupus erythematosus with oral lesions,
The caustic drugs (aspirin) or hot foods or beverages early lesions were characterized by erythema without the
result in the coagulation necrosis of the superficial tissue striae.
and appear as whitish scrapable membrane over an ery-
thematous base.
Erythematous macules at the junction of hard and soft RED LESIONS OF THE TONGUE
palate should be differentiated from the purpuric macule
of oral sex, palatal bruising because of severe cough or Migratory Glossitis
severe vomiting, allergic manifestations, macular heman-
gioma, atrophic candidiasis, infectious mononucleosis and Description is given in Chapter 2 (Developmental
herpangina. Disturbances).
The lesions usually regress after the removal of the
causative factors. In case of multiple numbers, investiga-
Median Rhomboid Glossitis
tions are needed to rule out the underlying hemostatic
disorders. Description is given in Chapter 2 (Developmental
Disturbances).
Reddish ulcers or ulcers with red halo Ulcerative condi-
tions like recurrent herpes and recurrent aphthous stoma-
titis are first manifested as erythematous macules. Deficiency States
Soft tissue odontogenic infection (cellulitis) Odonto- Certain deficiency states can produce a glossitis of a com-
genic infections may originate in canals and periapex of pletely bald or patchy bald tongue. These include iron
the teeth, gingival and periodontal pockets, and the gingi- deficiency anemias, pernicious anemia, Plummer-Vinson
val operculum over an erupting tooth and may spread to syndrome; sprue and vitamin B complex deficiencies, espe-
the surrounding soft tissues like oral mucosa appear red, cially those of thiamine, riboflavin, nicotinic acid, pyri-
swollen and tender to palpate. doxine, pantothenic acid, and vitamin B12.
Mucositis secondary to systemic diseases Mucosa may

Clinical features
have reddish appearance in case of severe esophagitis and
other gastrointestinal disorders, uremic stomatitis in case Tongue may be intensely red and then becomes smooth as
of end stage renal diseases. the filiform or both the types of papillae atrophy. Symptoms
vary from tender to burning tongue to extreme glossodynia.
Macular hemangiomas and telangiectasias Red macu-
lar hemangiomas occur as both syndromic and non-syn-
dromes associated are readily differentiated from Foliate Papillitis
erythemas by the history of long duration, non-tender-
Sometimes enlarged foliate papillae appear red in color
ness, absence of inflammatory components, characteristic
due to inflammatory enlargement of the lymphoid tissue
emptying of the lesions.
or due to upper respiratory tract infections or mechanical
Polycythemia Polycythemia also called erythremia is a irritation and may be mistaken for erythroplakic lesions
chronic and sustained elevation of erythrocytes and level on the tongue.
of hemoglobin. Primary polycythemia is a neoplastic con-
dition of the hematopoietic system. Secondary polycythemia
Erythroplakia
results from stimulation of bone marrow at high altitudes
or by chronic pulmonary diseases like emphysema. In this Erythroplakia is a precancerous lesion occurring in the
condition the entire oral mucosa appears deep red and oral cavity, The term ‘erythroplakia’ (erythroplasia) was
gingival and soft tissues easily bleed and seen as multiple coined to describe red lesions of the oral mucosa in con-
petechiae over the palate. Laboratory investigations trast to oral leukoplakia.
including elevated levels of erythrocyte count, raised hemo- The term erythroplasia was originally used by Queyrat
globin concentration, hematocrit values quickly establish to describe a red, precancerous lesion of the penis. The term
the diagnosis. erythroplakia is used for a clinically and histopathologically
168
similar process that occurs on the oral mucosa. Erythroplakia In a house-to-house survey in Burma among 6,000 vil-
is a clinical term that refers to a red patch that cannot be lagers over the age of 15 years, five cases of oral erythro-
defined clinically or pathologically as any other condition. plakia were diagnosed, with a prevalence of 0.83%.
This definition excludes inflammatory conditions that Feller et al from South Africa studied 138 cases of oral
may result in a red clinical appearance. precancerous lesions, of which eight were oral erythroplakia.
A recently published case-control study from Kerala,
Definitions India, included 100 cases of erythroplakia among 47,773
controls, with a prevalence of 0.2%.
Over the years several definitions for erythroplakia have
With these few data available it was observed that pres-
been suggested.
ently erythroplakia has a range of prevalence between
Mehta et al, diagnosed erythroplakia ‘when the oral
0.02% and 0.83%.
mucosa was the seat of a well-demarcated, red, often fiery
red patch, which could not be attributed to other causes’.
According to Shafer and Waldron, ‘Erythroplakia of the Classification
oral cavity is a specific disease entity which must be dif- Shear suggested a classification of erythroplakia in 1972. He
ferentiated from other specific or non-specific inflamma- differentiated between clinical and microscopic variations
tory oral lesions, although this can only be done in most and neoplastic from inflammatory changes.
cases by biopsy’. WHO in 1978 defined erythroplakia as
‘any lesion of the oral mucosa that presents as bright red Clinical variations
velvety plaques which cannot be characterized clinically ❍ Homogeneous erythroplakia
or pathologically as any other recognizable condition’. ❍ Erythroplakia interspersed with patches of leukoplakia
This definition was confirmed during an international ❍ Granular or speckled erythroplakia (embracing the
seminar on oral leukoplakia and associated lesions related lesion described as speckled leukoplakia)
to tobacco habits in 1983. In 1994, at another symposium
Microscopic variations
on oral white lesions with special reference to precancer-
❍ Neoplastic
ous and tobacco-related lesions the definition of OE was
– Squamous carcinoma
changed: ‘The term erythroplakia is used analogously to
– Carcinoma in situ (intraepithelial carcinoma) and
leukoplakia to designate lesions of the oral mucosa that
less severe forms of epithelial atypia
present as red areas and cannot be diagnosed as any other
❍ Inflammatory
definable lesion’.
– Candida albicans infections (including denture sto-
An updated definition for erythroplakia was proposed
matitis)
by Bouquot as ‘a chronic red mucosal macule which can-
– Tuberculosis
not be given another specific diagnostic name and cannot
– Histoplasmosis
be attributed to traumatic, vascular, or inflammatory
– Miscellaneous specific, non-specific and non-
causes’.
diagnosable lesions
Erythroplakia is defined as ‘a fiery red patch that can-
not be characterized clinically or pathologically as any
other definable lesion’. This definition is now widely Etiopathogenesis
accepted, although it is based on the principle of diagnosis
While erythroplakia does not seem to have a known geo-
per exclusion.
graphic incidence, studies from India have shown that
erythroplakia may be associated with tobacco smoking
Incidence/prevalence
and chewing habits and that the risk to develop erythro-
It is generally accepted that the erythroplakia is much less plakia was strongly associated with these.
common than oral leukoplakia and most of the prevalence Etiology and pathogenesis of erythroplakia are poorly
figures were derived from studies in South and South-East understood. Predisposing factors are widely unknown, but
Asia and no such figures have been published from other it was suggested that tobacco and alcohol use are probably
geographic areas. involved in most cases.
In a survey of 50,915 Indian individuals, Mehta et al Reports of large case-control study in Kerala, India,
found only nine cases of erythroplakia (0.02%). shed more light on some of the factors involved in the
In 1975 Shafer and Waldron described 58 cases thought etiology of erythroplakia. One of these studies evaluated
to be representative of oral erythroplakia among 64,345 the risk of erythroplakia in relation to chewing tobacco,
biopsies, representing 0.09%. smoking, alcohol drinking, body mass index (BMI), and
Two epidemiological surveys of oral mucosal lesions vegetable, fruit, and vitamin/iron intake. It was concluded
from Malaysia revealed a prevalence of 0.02% for both that tobacco chewing and alcohol drinking are strong risk
studies. factors for erythroplakia in the Indian population.
169
and velvety surface; they may also be seen with other

Figure 42
morphological characteristics, like an irregular, red granular
surface interspersed with white or yellow foci, which may
be described as granular erythroplakia. Erythroplakia is soft
to palpation and does not become indurated or hard until
an invasive carcinoma develops in it.
Erythroplakia is often asymptomatic, although some
patients may complain of a sore or burning sensation.
Malignant transformation
Erythroplakia has the highest risk of malignant transfor-
mation compared to all other oral mucosal premalignant
lesions. The malignant transformation rate for erythroplakia
varies from 14 to 50%.
Erythematous candidiasis, atrophic oral lichen planus and
denture-induced stomatitis can be considered in the differ-
ential diagnosis of erythroplakia.
A red velvety lesion involving the left buccal mucosa
suggestive of erythroplakia. Courtesy: Department of Oral Treatment and recurrence rate
Erythroplakia is a premalignant lesion, which shows high-
est risk for malignant transformation; therefore early treat-
In another study designed to study the risk factors for ment of such lesion is mandatory. Alcohol and tobacco
multiple oral premalignant lesions, suggested that tobacco habits should be avoided as a preliminary measure. Regular
chewing was the most important risk factor for multiple follow-up examination is recommended for the lesion
oral premalignant lesions and therefore may be a major which shows no or moderate epithelial dysplasia. Surgical
source of field cancerization on the oral epithelium in an excision of the lesion is recommended for those lesions
Indian population. which show severe epithelial dysplasia or carcinoma in
situ histologically. Amagasa et al (1985) recorded a recur-
Clinical features rence of erythroplakia in 5 of 7 cases.
Oral erythroplakia occurs most frequently in middle age
and elderly and appears as a red macule or plaque with a Discoid Lupus Erythematosus
soft, velvety texture (Figure 42).
Discoid lupus erythematosus (DLE) is a chronic inflamma-
Among 58 cases reported by Shafer and Waldron 37 cases
tory condition of the skin, connective tissue and specific
(67.8%) occurred in the sixth and seventh decades (19 men,
internal organs that has associated circulating autoanti-
18 women). It occurs most commonly in men. The soft pal-
bodies to DNA and other nuclear and RNA proteins; circu-
ate, the floor of the mouth and the buccal mucosa are the
lar whitish buccomucosal lesions and erythematous rashes
most commonly affected site; the tongue is rarely affected.
of the sun-exposed skin.
Shafer and Waldron, however, observed some differences
Lupus erythematosus is a syndrome whose manifesta-
of location between women and men. The most common
tions range from a localized skin lesion to a destructive
site of occurrence of oral erythroplakia in men was the
systemic disorder without any cutaneous changes.
floor of the mouth, but in women the combined mandibu-
lar alveolar mucosa, mandibular gingiva, and mandibular Classification
sulcus was most commonly affected. In men this combined
site was the least common site of occurrence. The retromo- Gilliam classification of skin lesions associated with LE
lar area in both men and women and the floor of the mouth
in women was the next most common site of involvement. I. LE-Specific skin disease
The typical lesion of oral erythroplakia is less than 1.5 cm A. Acute cutaneous LE
in diameter and half are less than 1 cm, but lesions larger 1. Localized ACLE
than 4 cm have also been observed. Some erythroplakias are 2. Generalized ACLE
smooth and some are granular or nodular. Often there is a B. Subacute cutaneous LE
well-defined margin adjacent to mucosa of normal appear- 1. Annular SCLE
ance, although the erythroplakia lesions may have a smooth 2. Papulosquamous SCLE
170
esterase inhibitor are associated with DLE. Significant

C. Chronic cutaneous LE
increases of HLA B7, B8, DR3 and DQA0102 and a signifi-
1. Classic discoid LE
cant decrease in HLA A2 have been reported for patients
a. Localized DLE
with DLE. It also occurs with increased frequency in female
b. Generalized DLE
carriers of X-linked chronic granulomatous disease.
2. Hypertropic/verrucous DLE
3. Lupus profundus Role of UV light Ultraviolet light is probably the most
4. Mucosal DLE important environmental factor in the induction phase of
a. Oral DLE LE specific skin disease. Early studies demonstrated that
b. Conjunctival DLE cutaneous LE lesions could be provoked in the clinically
5. Chilblain DLE normal skin of the patients with both SLE and cutaneous
6. Lichenoid DLE LE by repeated delivery of high doses of UVB radiation to
II. LE—Non-specific skin disease the same test site. More recent studies argue that UVA
A. Cutaneous vascular disease radiation can also induce cutaneous LE lesions.
1. Vasculitis
2. Vasculopathy Environmental factors The onset of lesions may be pre-
3. Livedo reticularis cipitated by a variety of factors. At a study in Leeds,
4. Thrombophlebitis lesions started with trauma in 11%, with mental stress in
5. Raynaud’s phenomenon 12%, sunburn in 5%, infection in 3%, exposure to cold in
B. Non-scarring alopecia 2% and pregnancy in 1%. Drugs like antibiotics (penicillin,
C. Sclerodactyly streptomycin, sulfonamides, tetracycline), antituberculous
D. Rheumatoid nodules drugs (isoniazid, paraaminosalicylate), antihypertensive
E. Calcinosis cutis drugs (hydralazine and methyldopa), antifungal (griseo-
F. Urticaria fulvin) and antiarthritic (gold) have known to precipitate
G. Erythema multiforme lesions of DLE.
H. Lichen planus
Clinical features
It is usually seen in the 3rd and 4th decades of life. The peak
Alternatively DLE can be classified as:
age of onset is 30 years in females and 40 years in males.
❍ Systemic lupus erythematosus It is predominantly seen in females in the ratio 3:1. Blacks
❍ Bullous form of lupus erythematosus are reportedly more severely affected. The common sites of
❍ Neonatal form of LE involvement include the face, scalp, nose, ears, V area of the
❍ Chronic cutaneous form of LE (CCLE) neck and extensor aspect of the arms. Any area of the face
❍ Subacute cutaneous form (SCLE) including the eyebrows, eyelids, nose and lips can be affected.
❍ Drug-related lupus
Discoid lupus erythematosus (DLE) is named so because Skin lesions
the lesions are disc or coin shaped. DLE is also known by Early lesions The lesion start as patches of erythema,
the name cutaneous lupus erythematosus and chronic dis- occasionally with an urticarial component; later they become
coid lupus erythematosus. papulosquamous and eventuate in elevated reddish edem-
The WHO Collaborating Reference Centre for Oral atous plaques covered with adherent graying scales. The
Precancerous Lesions describes DLE as a benign disorder lesions tend to enlarge peripherally and may coalesce to
of the skin, most frequently involving the face, and char- produce bizarre patterns.
acterized by well-defined red scaly patches of variable
sizes, which heal with atrophy, scarring and pigmentary Chronic lesions Chronic lesions are well defined and cir-
changes. Discoid lupus erythematosus can present as a cular, oval or irregular in shape. Often they have an elevated
localized or generalized form. In the localized form, head erythematous border. The center of the lesion is usually
and neck are most commonly involved, whereas in the depressed. Dilated follicular openings plugged with horny
disseminated form the lesions may occur in a wide spread epithelial plugs are seen. Large areas may be involved.
pattern on the trunk and limbs, or may be localized to Some of the patches may resolve, but residual scarring is
other body sites. more common. The scars are smooth, atrophic, flat and
white. Telangiectasia may be present at the edges of the
scars so called telangiectatic lupus erythematosus is merely
Etiopathogenesis
the morphological form in which telangiectasia predomi-
Genetic factors Genetic deficiencies of the complement nates. Post inflammatory hyperpigmentation also may occur.
components including C2, C3, C4, and C5 as well as the C1 Depigmentation may also be seen. Follicular involvement
171
is a prominent feature. Keratotic plugs accumulate in dilated Discoid lupus erythematosus has negative response for
follicles that soon become devoid of hair. When the adher- LE cell inclusion test usually patients with SLE typically
ent scale is lifted from more advanced lesions, keratotic develop LE cells. This cell or phenomenon consists of a
spikes similar in appearance to carpet tacks can be seen to rosette of neutrophils surrounding a pale nuclear mass
project from the undersurface of the scale (carpet tack sign apparently derived from a lymphocyte. Only rare occasion
or tin tact sign). is the LE cell found in cases of DLE.
Color: Active lesions—red whereas burnt out or scarred
lesions are pink or white. Immunofluorescence studies
Shape is usually round, oval, annular, polycyclic with Immunofluorescence studies reveal a granular or shaggy
irregular borders. pattern of IgG, IgM, IgA, C3 in the basement membrane or
Distribution—scattered discrete lesions. in the dermal-epidermal junction. In comparison to IgM,
Malar rash or the butterfly rash is occasionally seen granular deposits of IgG are extensive at the dermo-
in DLE.
epidermal junction. Such a finding is referred to as lupus
Mucosal or oral lesions Lesions of the mucous mem- band. Lupus band test (LBT) is positive in 90% of active
branes may be limited to these areas, but usually coexist lesions that have not been recently treated with topical
with skin lesions of DLE. Mucous membrane lesions more corticosteroids. However the test is negative in burnt-out
commonly occur during acute systemic episodes of lupus or scarred lesions and in the normal skin (either sun-
erythematosus. They typically consist of gingivitis, mucosal exposed/non-sun-exposed).
hemorrhage, erosion and shallow ulcerations.
❍ Early lesions—mucosal hemorrhage, erosion, superfi-
cial erythematous patches with dilated blood vessels Hyperkeratosis with follicular or keratotic plugging, atro-
on the borders. The center is depressed or superficially phy of the rete pegs, liquefaction degeneration of the basal
ulcerated. layer of cells, perivascular infiltration of lymphocytes and
❍ Chronic lesions—central atrophic areas with small white their collection about dermal appendages, and basophilic
dots, surrounded by a keratinized border composed of degeneration of collagen and elastic fibers with hyaliniza-
radiating white striae. tion, edema and fibrinoid change, particularly prominent
immediately beneath the epithelium.
Different types of DLE
❍ Verrucous DLE: If hyperkeratosis is marked, a warty
lesion with a red slightly raised edge results. 1. Systemic lupus erythematosus (SLE): Systemic mani-
❍ Tumid DLE: The tissues are swollen, brawny, warm and festations are present, LE cells are seen and antinu-
tense. The surface shows a reddish, mottled appear- clear antibodies are present.
ance due to scarring. 2. Polymorphous light eruption (PLE): Absence of anti-
❍ Chilblain lupus: Variant of DLE characterized by nuclear factor from the serum and of dermal-epidermal
purplish-blue, tender chilblain-like (chilblains are ulcers immunoglobulin deposits.
affecting extremities that occur due to exposure to 3. Lupus vulgaris: Lesions usually occur at an early age,
cold and humidity) nodules. and are rarely symmetrical, may be ulcerated and
❍ LE profundus: In this cutaneous infiltrate occurs pri- usually show characteristic apple jelly nodules.
marily in the deeper portions of the dermis and gives 4. Lichen planus: The presence of concentrations of
rise to firm, rubbery, sharply defined nodules varying lymphocytes in the immediate underlying lamina pro-
in size from one to several cm in diameter. pria, deep focal accumulations of lymphocytes with
❍ LE and EM like syndrome (Rowell’s syndrome). germinal centers and perivascular infiltrates of lym-
❍ LE and LP overlap syndrome. phocytes is helpful in differentiating lesions of LE and
❍ Rosacea like syndrome. those of LP. In more chronic lesions the presence of
hyperorthokeratosis and surface depressions contain-
Laboratory findings ing keratin (keratin plugging) suggests LE than LP.
5. Seborrheic dermatitis, actinic keratosis and drug
The laboratory findings for DLE are not specific. Patients
eruptions.
can present with anemia, leukopenia, thrombocytopenia,
elevated ESR levels, elevated serum globulin levels, high The differential diagnosis for oral lesions include lichen
IgG levels, presence of antithyroid antibodies and reduced planus and leukoplakia. The classic oral discoid lesion has
T-cell counts. three outstanding features according to Schiodt which can
Urine examination and blood urea nitrogen has to be be differentiated from other lesions; they are: a central
done to rule out SLE and to know patient’s renal function. atrophic area with small white dots, a slightly elevated
Antinuclear antibodies are rarely present. border zone of irradiating white striae and telangiectasia.
172
Prognosis aureomycin 6–9 mg/day, isotretinoin 20–80 mg/day and

dapsone 100 mg/day.
The untreated skin lesions tend to be persistent, usually
When all of the above have failed in patients with
heals with scarring. Less than 5% of the cases may convert
severe and persistent disease, then the following drugs can
into SLE. Squamous cell and less commonly basal cell car-
be given.
cinomas occasionally occur in scars of DLE, particularly
Pulsed methylprednisolone 500–1,000 mg/day for 2–3
on the scalp, ears, lips and nose.
days or cyclophosphamide 50–200 mg/day or intravenous
pulses of above drugs at 10 mg/kg, at 3–4 weekly intervals.
Management
General measures Patient can be advised against exces- Note: For a long time the terms precancerous lesions
sive exposure to sunlight, UV light and heat. They can and conditions have been used extensively in literature.
be instructed to use umbrellas or broad brimmed hats. However, many authors believed that the use of the
Sunscreen creams or lotions can be used regularly. prefix ‘pre-’ implied that all the lesions and conditions
eventually turned into cancer, which is not true. In
Topical therapy 0.025% fluocinolone cream or 0.1% tri-
order to simplify this understanding and avoid the con-
amcinolone acetonide cream has shown to be effective.
fusion between lesions and conditions, the World
Intralesional corticosteroid injections (triamcinolone ace-
Health Organization in 2005 suggested the use of the
tonide 5–10 mg/ml) at 6 weekly interval are helpful in
term ‘potentially malignant disorders (PMDs)’.
resistant cases.
As per this recommendation, PMDs are the disorders
Oral therapy Oral prednisolone 0.5 mg/kg rapidly tapered in which the risk of malignancy is present in a lesion or
over 6 weeks or hydroxychloroquine, initially 200 mg condition either at the time of initial diagnosis or at a
twice daily, reducing to 200 mg/day after response. later stage.
Alternatively chloroquine sulfate 200 mg twice daily Examples of high-risk PMDs are erythroplakia, leuko-
can be used. Other drugs that have been used include plakia, oral submucous fibrosis and erosive lichen planus.
173
CHAPTER
7 Vesiculobullous Disorders
Nagamani Narayana, Ravikiran Ongole
➧ Classification of Vesiculobullous Lesions Pemphigus Vulgaris

➧ Predominantly Vesicular Lesions Paraneoplastic Pemphigus (Paraneoplastic
Herpes Viruses Autoimmune Multiorgan Syndrome)
Bullous Pemphigoid
➧ Herpes Simplex Virus (HSV) infections
Mucous Membrane Pemphigoid or Cicatricial
➧ Herpetic Whitlow and Herpes Gladiatorum
Pemphigoid
➧ Recurrent Herpes Infections
➧ Bullous Lichen Planus
Herpetic Labialis
➧ Varicella Zoster Infections ➧ Erythema Multiforme
Complications Associated with Herpes Zoster Recurrent Erythema Multiforme
➧ Hand, Foot and Mouth Disease ➧ Stevens–Johnson Syndrome and Toxic

➧ Herpangina Epidermal Necrolysis (Lyell’s Syndrome)
➧ Dermatitis Herpetiformis ➧ Bullous Impetigo

➧ Predominantly Bullous Lesions ➧ Epidermolysis Bullosa
Pemphigus ➧ Linear IgA Disease
There are a variety of oral lesions which clinically present

Herpangina
as vesiculobullous (VB) lesions. Although the lesions start
Dermatitis herpetiformis
as vesicles or bullae they rupture early and appear as ulcer-
ated or erosive areas. As a result they are better called ulcero- 2. Predominantly bullous
vesiculobullous diseases. In this chapter common oral VB Pemphigus vulgaris
lesions will be discussed. Bullous pemphigoid
A vesicle is defined as a fluid-filled elevated lesion, less Benign mucous membrane pemphigoid
than 1 cm in diameter. A bulla is a fluid-filled elevated Bullous lichen planus
lesion greater than 1 cm in diameter. Erythema multiforme
Stevens–Johnson syndrome
Bullous impetigo
CLASSIFICATION OF VESICULOBULLOUS Epidermolysis bullosa
LESIONS Linear IgA disease
III. Histopathological classification

I. Acute and chronic vesiculobullous lesions
(Table 1) Intraepithelial vesiculobullous lesions
HSV infection
II. Based on the clinical presentation
Varicella infection
1. Predominantly vesicular Herpangina
HSV infection Hand, foot and mouth disease
Varicella infection Pemphigus
Hand, foot and mouth disease Familial benign chronic pemphigus
174
Chapter 7 – Vesiculobullous Disorders
Table 1 Comparison between acute and chronic vesiculobul- Table 2 Site of latency of viruses
lous lesions
Name of virus Site of latency
Acute—VB lesions Chronic—VB lesions
HSV 1 & 2, VZV Sensory nerve ganglia
Duration Short Long
CMV Lymphocytes, salivary gland tissue (rarely)
Age Young Middle aged—older
EBV B lymphocytes and salivary gland tissue
Etiology Allergy, burns, viruses Autoimmune
HHV-6, HHV-7 CD4 lymphocytes
Examples Herpes simplex infections Pemphigus
HHV-8 Still unknown but believed to be associated with B
Chicken pox Bullous pemphigoid lymphocytes circulating in hematopoietic system
Herpes zoster Cicatricial pemphigoid
Herpangina Bullous lichen planus
Hand, foot and mouth disease Chronic herpes simplex
It is estimated that out of the 80 known herpes viruses,
Erythema multiforme Linear IgA disease
at least eight are known to infect human beings. The her-
pes viruses that are known to cause infection in humans
are herpes simplex virus (HSV 1 and 2), varicella zoster
virus (VZV), cytomegalovirus (CMV), Epstein-Barr virus
Epidermolysis bullosa (EBV), human herpes virus 6 (HHV-6), human herpes virus 7
Mucosal erythema multiforme (HHV-7) and human herpes virus 8 (HHV-8). These viruses
Subepithelial vesiculobullous lesions are usually transmitted from host to host by direct contact
Bullous pemphigoid or through saliva and genital secretions. Herpes viruses
Cicatricial pemphigoid are shed in the saliva of asymptomatic hosts.
Epidermolysis Bullosa When an individual comes in contact with the virus, pri-
Dermal Erythema multiforme mary infection is seen. Subsequently these viruses estab-
Dermatitis herpetiformis lish latency in the host. The site of latency for each form
Linear IgA disease of herpes virus is different (Table 2).
IV. Based on whether the lesions are infectious

or non-infectious HERPES SIMPLEX VIRUS (HSV) INFECTIONS
Infectious VB lesions
Herpes simplex infections Etiology and pathogenesis
Varicella infections Herpes simplex virus infection is caused by two types of DNA
Herpangina viruses namely HSV 1 and HSV 2. Characteristically, HSV 1
Hand, foot and mouth disease is responsible for infections involving the oropharyngeal
Non-infectious VB lesions regions, dermatitis and meningoencephalitis (infections
Pemphigus above the waist) and HSV 2 is responsible for anogenital
Paraneoplastic pemphigus infections (infections below the waist). However, changing
Bullous pemphigoid sexual practices have shown that these viruses may not
Cicatricial pemphigoid necessarily be responsible for infections of specific sites.
Erythema multiforme In contrast to other viruses HSV needs physical contact
Dermatitis herpetiformis to transfer infection. The incubation period ranges from
Epidermolysis bullosa acquisita 2 days to 3 weeks. After the primary infection the virus
Linear IgA disease hides within the trigeminal ganglia and becomes activated
when the environment is conducive for viral replication.
Approximately 90% of USA population is seropositive for
PREDOMINANTLY VESICULAR LESIONS antibodies to HSV. Of this group only 1% exhibit primary
herpetic infections and 30–40% demonstrate recurrent
infections. Primary HSV infections are usually seen after
Herpes Viruses
6 months of age and peak at about 2–3 years of age.
The word herpes is derived from the Greek word ‘herpein’ Other specific HSV infections include herpetic whitlow,
which literally means to creep. The word signifies the herpetic gladiatorum, herpetic meningoencephalitis, herpetic
creeping or spreading nature of the skin lesions caused by conjunctivitis, herpetic eczema (Kaposi’s varicelliform erup-
many of the herpes viruses. tion) and disseminated herpes simplex of newborn.
175
Clinical features As the lesion progresses, the involved digit reveals multiple
tiny vesicles which are extremely tender. The surrounding
Primary HSV infection Only 1% of the population in
area appears edematous and erythematous. The lesion may
USA exhibit all signs and symptoms of primary infection.
rupture to form shallow ulcers and subsequently heal in
It usually affects young children and adolescents, and occa-
about 10 days time.
sionally young adults. The primary HSV infection seldom
Similar vesicular lesions seen on the bodies of sports-
occurs in the first 6 months of life as the infant is protected
men (especially contact sports) and wrestlers is referred to
by the maternal antibodies that are still circulating in the
as herpes gladiatorum.
newborn.
Primary herpetic gingivostomatitis is characterized by
Investigations
fever, malaise, anorexia, irritability and regional lymphade-
nopathy (especially, submandibular and superficial cervi- Typical clinical features will usually suffice to make a clini-
cal group of nodes are involved). Subsequently the mouth cal diagnosis. However for definitive diagnosis cytological
becomes sore and the individual may complain of burning smears, antibody titers and viral isolation can be used.
sensation in the mouth and difficulty in swallowing. Apart Cytological smear requires deroofing a fresh vesicle,
from the gingiva, the buccal mucosa, palate, tongue, ton- followed by scraping of the base of the lesion and the
sillar and pharyngeal region may be affected. smear made should be stained with Papanicolaou’s, Wright’s
The gingiva is erythematous, boggy and bleeds sponta- or Giemsa’s stain.
neously or on the slightest of provocation. As the disease The characteristic findings include the presence of mul-
advances multiple tiny to moderately large (few mm to tinucleated giant cells and intranuclear viral inclusion bod-
almost a centimeter in size) yellow colored vesicles develop ies such as Lipschütz bodies or Cowdry Type A (ovoid,
that rupture to form shallow painful ulcers that are usually amorphous, eosinophilic bodies that exhibit peri inclusion
covered by a grayish colored membrane. These ulcers are halo that is caused by the peripheral displacement of the
bound by an erythematous halo. nucleolus and the nuclear chromatin). The cells exhibit
Generally in about 2 weeks the lesion heals without ballooning degeneration of the nucleus.
scarring. Immediately after the resolution of the primary On biopsy the microscopic features include an ulcerated
lesion, the virus travels along the nerve pathway and lies epithelium with large keratinocytes showing glassy giant
dormant in the nerve ganglia regional to the site of the marginated nuclei (Tzanck cells) (Figures 1 and 2).
primary infection. Within the regional ganglion the virus Alternatively viral isolation can be done. It is one of the
incorporates its DNA into the host’s DNA thereby estab- most definitive methods of identifying HSV.
lishing the beginning of a life-long affliction. The trigem- Serological tests can be used to detect antibodies in
inal ganglion is usually the site for dormancy for HSV-1 patient suffering from primary HSV. For the test, blood
and HSV-2. sample should be taken in the initial 3 days of an acute
infection. Alternatively a convalescent blood sample can
be obtained 4 weeks after the primary infection. It is
HERPETIC WHITLOW AND HERPES believed that the antibody titer should increase by four
GLADIATORUM times in convalescent serum sample for the diagnosis of
the primary infection.
Herpetic whitlow is an intense painful infection affecting
the fingers or toes of children, adults and healthcare work-
ers. It typically involves the terminal phalanx of the index RECURRENT HERPES INFECTIONS
and thumb fingers of children and healthcare workers. It is
believed that HSV-1 is responsible for 60% of the known Almost 30–40% of the individuals exposed to HSV infec-
cases of herpetic whitlow and 40% are caused by HSV-2. tion will exhibit at least one episode of recurrent herpes
In children, most cases can be attributed to autoinoculation infections. Recurrent infections can present either as
of HSV-1 (infants suffering from acute herpetic gingivo- recurrent herpes labialis or recurrent intraoral herpes.
stomatitis who engage in thumb/finger sucking). In young Various factors have been known to trigger these recur-
adults and elderly, herpetic whitlow is caused by autoin- rent attacks such as stress, trauma, exposure to sunlight
oculation of HSV-2, usually due to digital-genital contact. and menstruation. These recurrent infections usually have
Literature review reveals few reports describing herpetic a milder course.
whitlow affecting the toe. Ozawa et al (2004) reported an
elderly patient who exhibited herpetic whitlow of the toe.
Herpetic Labialis
The lesions of herpetic whitlow are usually preceded by
prodromal symptoms of burning or tingling sensation and This is the most common secondary herpetic lesion,
pruritus of the affected digit and sometimes the whole limb. usually following exposure to UV light or extreme cold.
176
Figure 1 Figure 3
Clusters of vesicles in a 26-year-old female, characteristic

of herpes labialis. Courtesy: Dr Nagamani Narayana,
Nebraska, USA
treatment. These are also present with prodromal symptoms

Photomicrograph of herpetic ulcer. H&E stain (10⫻). such as paresthesia, swelling, burning and tingling.
Arrow points to acantholytic cells and Tzanck cells. Secondary herpetic lesions recur, and the recurrence
Courtesy: Dr Nagamani Narayana, Nebraska, USA
rate is ⬎ 6 times/year. Secondary intraoral herpetic lesions
are seen in patients who are immunocompromised from
HIV/AIDS, pre-transplant and post-transplantation surgery
Figure 2 or post-chemotherapy. Intraorally ulcerations occur on
bound and mobile mucosa.
Yeo et al (2002) reported the presence of herpes labialis
in musicians who play woodwind instruments and brass
instruments such as the flute, saxophone, trumpet and horn.
They suggest that the herpetic lesions are precipitated by
mechanical trauma of the lips and stress suffered by the
musicians preparing for a performance. It is commonly
seen that the woodwind players tend to have lesions on
the lower lip and brass instrument players have herpetic
lesions on the upper lip.
Acute herpetic infections Streptococcal pharyngitis,
erythema multiforme and acute necrotizing ulcerative
gingivostomatitis (ANUG) have to be considered. All these
Photomicrograph (40⫻). Arrow points to a Tzanck cell with lesions will demonstrate systemic signs and symptoms.
large molded nucleus. Courtesy: Dr Nagamani Narayana, Erythema multiforme lesions occur commonly on the lip
Nebraska, USA and less often on the gingiva. Lip lesions are bleeding
and crusty. In ANUG the gingival lesions demonstrate
punched-out interdental papilla with accompanying
Groups of vesicles appear at the vermillion border, and
bleeding and halitosis.
rupture may result in crusty lesions. The lesions are tingly
and painful, normally healing within 2 weeks. The lesions Secondary herpetic infections Consider aphthous ulcers
are highly contagious at the weeping stage. in the differential diagnosis of intraoral recurrent lesions.
Intraoral herpetic lesions are evident as clusters of ves- Aphthous ulcers are found on mobile mucosa and do not
icles (Figure 3). These vesicles rupture, leaving ulcerations go through a vesicular phase. The ulcers are painful and
on bound mucosa, usually within 3 days of recent dental are difficult to differentiate from herpes simplex lesions.
177
Table 3 Treatment regimen for different kinds of HSV infection
Immunocompetent Immunosuppressed
Primary herpetic gingivostomatitis— Symptomatic—no aspirin or NSAIDs Acyclovir capsules 200 mg or valacyclovir
children caplets 500 mg
Primary herpetic gingivostomatitis— Symptomatic, aspirin and NSAIDs, magic mouthwash Acyclovir capsules 200 mg, 5 times daily
adult for 10 days or valacyclovir caplets 500 mg,
2 caplets twice daily for 5 days
Recurrent herpetic labialis Abreva, Denavir 5%, valacyclovir 1,500 mg, single dose Valacyclovir caplets 500 mg, 4 caplets taken
or valacyclovir 750 mg, twice daily for 1 day started within at prodromal symptoms and 4 caplets
1 hour of onset of prodromal signs 12 hours later
Recurrent intraoral herpes Acyclovir 200 mg 5 times a day for 10 days
Treatment and may get secondarily infected. The common sites of

involvement are the trunk, face and extremities. Intraorally,
The aims of managing HSV infections are to inhibit auto-
the buccal mucosa, tongue, palate, gingiva and pharyngeal
inoculation and transmission. The management is targeted
mucosa are affected. Oral mucous membrane may show
at symptomatic relief. Topical and systemic antivirals like
multiple shallow ulcers preceded by thin walled vesicles,
acyclovir can be used (Table 3).
which are usually not very painful.
To be effective all treatment should be started within
In adults varicella may result in complications like
72 hours of initial disease presentation. Patient compli-
pneumonitis, encephalitis, and fetal abnormalities when
ance is shown to be better with fewer and shorter doses
seen in pregnancy.
(Whitley, 2006).
Secondary infection (herpes zoster) Herpes zoster is the
Prognosis reactivated form of the VZV. It is also referred to as shin-
gles. The word shingles is reportedly derived from the Latin
The main question arises should you treat these patients
word ‘cingulum’ which means ‘girdle’. The term girdle is
when they have recurrent lesions. Universal precautions
apt to describe the lesion of herpes zoster as it presents as
including use of gloves enable us to do proceed with treat-
a unilateral rash that can wrap around the waist or torso
ment. If the lesions are oozing and will transfer virus to
like a girdle. The word zoster is Greek word that refers to
other sites and patient has no emergency it is advisable to
a belt like object that is used by warriors to hold the armor
postpone treatment. If in an emergency application of
in place at the waist.
rubber dam may help in decreasing transmission of virus
Secondary varicella zoster infection occurs in adults
to other sites.
with altered immune status or under stress and is unilat-
Herpes simplex virus infections are described in detail
eral. The main distinguishing feature of VZV is unilateral
in Chapter 4 on Bacterial, Viral and Fungal Infections.
and involves one of the branches of the trigeminal nerve.
Clinically tingling and vesiculation similar to herpes sim-
plex are seen. Clinical appearance of these lesions is very
VARICELLA ZOSTER INFECTIONS similar to herpes simplex except for the unilateral location
(Figures 4 and 5). Patients may complain of mild to severe
The primary and secondary infections are caused by VZV pain that may exacerbate on the slightest of touch. The
belonging to Herpedes family. Chicken pox is the primary dermal lesions usually resolve in about a week forming
infection and herpes zoster is caused by reactivation of the a scab.
VZV. This infection occurs due to inhalation and primary
infection occurs in children. Reactivation occurs when the
patient is stressed or immunosuppressed. It usually affects Complications Associated with Herpes Zoster
dermatomes in the trunk, head or neck.
The most common complication associated with HZ is the
development of postherpetic neuralgia. In this condition
Clinical features
the pain continues to persist even after the herpetic lesions
Primary infection (chicken pox) Primary varicella infec- have resolved. The pain is typically presented as sharp or
tion occurring in children is characterized by fever, chills, burning pain. Postherpetic neuralgia is usually seen in
malaise and headache with a quick rash. The rash is pruritic individuals over the 5th decade of life.
178
present with painful inflammatory condition of the eye

Figure 4
along with impaired vision or transient blindness.
Some individuals exhibit only the prodromal symptoms
of pain and paresthesia without the development of visible
cutaneous rash. This phenomenon is referred to as ‘zoster
sine herpete’.
Ramsay Hunt syndrome is one of the rare manifestations
of HZV infection where geniculate ganglion is involved.
The facial and auditory nerves can be involved. The condi-
tion is characterized by facial paralysis, pain and vesicles
in the external auditory canal and pinna of the ear and the
oral cavity. Other associated findings are tinnitus, vertigo
and ipsilateral hearing loss.
Investigations
Cytological smears derived from the vesicles reveal multi-
nucleated giant cells along with intranuclear inclusion bod-
ies. PCR and direct immunofluorescence assay are more
effective in diagnosing HZV infections.
Herpes zoster seen in a 70-year-old male with papular lesions Differential diagnosis
on the left half of his face. Courtesy: Dr Nagamani Narayana,
Nebraska, USA Recurrent herpetic lesions and aphthous ulcers should be
considered in the differential diagnosis of intraoral lesions.
Treatment and prognosis

Figure 5
In healthy patients if the diagnosis occurs within 72 hours
of initiation of the disease a course of acyclovir or valacy-
clovir can be administered. If the patient is seen later dur-
ing the course of the disease symptomatic relief in the form
of magic mouthwash can be prescribed. In immunosup-
pressed patients a prescription of acyclovir or valacyclovir
can be administered. It is some belief that a prescription for
antiviral and corticosteroid therapy prevents postherpetic
neuralgia.
HAND, FOOT AND MOUTH DISEASE
The term hand, foot and mouth (HFM) disease was used for
the first time in 1960 following an outbreak of a relatively
Herpes zoster seen in a 70-year-old male with ulcerations in
mild febrile condition associated with papular and vesicular
the palate, characteristically involving one half of the palate. lesions on the dermis and the oral cavity in Birmingham,
Courtesy: Dr Nagamani Narayana, Nebraska, USA England.
Etiology
Herpes zoster is generally unilateral. However in immu- Enteroviruses are known to cause HFM disease. The
nocompromised individuals or an associated malignancy, enteroviruses that have been implicated are Coxsackie virus
herpes zoster can exhibit a generalized involvement. Other serotype A 16, Coxsackie virus serotype A 4 to 7, 9, 10 and
relatively uncommon complications involve encephalitis, Coxsackie virus serotype B 1 to 3 and 5. Asia Pacific region
peripheral nerve palsies and myelitis. including regions like Singapore, Japan, Indonesia, Malaysia
Zoster ophthalmicus is seen when herpes zoster involves and Taiwan in the 1970s reported severe forms of HFM dis-
the ophthalmic ganglion of the trigeminal nerve. Patients ease resulting in a widespread epidemic. The cause for this
179
epidemic was Enterovirus-71 A, B, C (EV 71). The EV-71 HERPANGINA

apart from causing HFM disease can also cause flaccid
paralysis, myocarditis, pulmonary hemorrhage, encephali- Herpangina was first described by John Zahorsky in 1920.
tis and meningitis. Herpangina is primarily caused by coxsackie virus A 1-10,
Deshpande et al (2003) isolated a strain of EV-71 from 16, or 22. Other viruses that may cause herpangina are
the stool of a child suffering from acute flaccid paralysis coxsackie virus B 1-5, enterovirus 71 and echovirus 3, 6,
following administration of oral polio vaccine. They 9, 11, 16, 17, 22, 25, and 30. Herpangina occurs in epidem-
termed this isolated strain as EV 71 D genotype. ics and usually occurs in the summer months. It usually
Sasidharan et al (2005) studied 81 children (age group affects young children and adolescents. The mode of spread
of 7 months to 8 years) who presented with papulovesicu- is the fecal-oral route. The incubation period varies from
lar exanthems in Calicut, India. In their study 19 children 1 to 10 days and usually lasts 4 days.
showed a significant rise in the titers of IgM antibody
against EV-71. Clinical features
Infection usually occurs by the fecal-oral route, leading
to viremia and invasion of the skin and mucosa. The incu- Compared to hand, foot and mouth disease the disease
bation period is approximately 3–7 days. process is usually mild and patients may not exhibit overt
prodromal symptoms. Patients may present with mild rise
Clinical features in temperature, malaise, anorexia, abdominal pain, sore
throat and cervical lymphadenopathy.
Patients usually present with prodromal symptoms such
as low-grade fever, malaise, anorexia, myalgia, headache, Oral manifestations
cough, rhinorrhea and sore throat. The skin of the hands
and feet along with the oral mucosa are involved. The oral Oral manifestations are very typical of this condition.
mucosa and the hand are almost always affected. Multiple minute vesicles and ulcers roughly measuring
The skin lesions are characterized by the presence of 1–2 mm in diameter are seen. The ulcers are surrounded by
numerous erythematous macules in the initial stage. As an erythematous halo. These lesions are typically confined
the disease progresses vesicles begin to appear in the mac- to the posterior part of the oral cavity such as the faucial
ules which subsequently heal without crusts in about 10 to pillars, tonsillar region, posterior pharyngeal region, soft
14 days. palate and uvula. Very rarely the posterior parts of the
The common sites of involvement are the palms and buccal mucosa and tongue are involved. Patients may
fingers of the hand, and soles and toes of the feet. Other complain of sore throat and difficulty in swallowing.
sites that are less commonly affected are the trunk, exter-
nal genitalia and the buttocks. Management
Herpangina is a self-limiting disease. The fever associated
Oral manifestations with this condition usually subsides within 3–4 days. The
oral ulcers heal within a week. The condition can be man-
The oral lesions usually occur before the skin lesions.
aged symptomatically with antipyretics and analgesic/
Multiple vesicles and ulcers may be seen affecting any
anesthetic mouthrinses.
part of the oral mucosa. However the common sites that
are involved include the tongue, buccal mucosa and hard
palate. Patients usually complain of pain, sore throat and
dysphagia. Oral lesions usually heal without complica-
DERMATITIS HERPETIFORMIS
tions in about 7 days time.
The oral manifestations of HFM disease mimic lesions Dermatitis herpetiformis (DH) is a rare, chronic, pruritic
of herpangina, acute herpetic gingivostomatitis, recurrent immunobullous disorder of the skin that is associated with
herpetiform and minor aphthous ulcerations and erythema gluten hypersensitivity. The condition is characterized by
multiforme. the subepidermal deposition of IgA antibody against the
tissue transglutaminase. Antibody deposition leads to the
formation of dense neutrophilic abscesses, subepidermal
Management
vesicles and bullae. Spurkland et al (1997) described the
The disease is self-limiting and needs to be symptomati- genetic basis for DH. They showed that DH and celiac
cally managed. Systemic antipyretics (paracetamol suspen- disease are associated with class II HLA alleles A1*0501
sion or tablet), topical analgesic mouthrinse (benzydamine and B1*02, which encode the HLA-DQ2 heterodimer.
hydrochloride) and topical anesthetic agents (2% ligno- Salmela et al (2001) showed that there was an upregu-
caine gel) are effective in the management of fever and lation of metalloelastase in both intestinal and lesional
sore mouth. skin.
180
Clinical features Table 4 Antigens targeted by autoantibodies and correspond-

Dermatitis herpetiformis usually affects individuals in the ing forms of pemphigus
2nd and 3rd decade of life. Dermal lesions are character- Antigens Forms of pemphigus
ized by blisters over an erythematous or urticarial base.
Desmoglein 3 Predominantly mucosal
The lesions are pruritic.
pemphigus vulgaris
The common sites of involvement include the knees,
Desmoglein 3, desmoglein 1 and Mucocutaneous pemphigus
elbows, scalp, back and buttocks. It is believed that about
probably desmoglein 4 vulgaris
10% of the patients present with symptoms of celiac dis-
ease. DH is believed to be associated with some of the Desmoglein 1 Pemphigus foliaceous
autoimmune and connective tissue diseases such as Type I Desmoglein 3, Desmoglein 1 and Paraneoplastic pemphigus
diabetes mellitus, pernicious anemia, autoimmune thyroid- plakin proteins
itis, Sjögren’s syndrome, lupus erythematosus, sarcoidosis, Adapted from Ettlin DA, Dent Clin North Am 2005;49:110.
scleroderma and psoriasis. DH has also been frequently asso-
ciated with hypopigmentation.
short lived and easily break down resulting in ulcerations.
Oral manifestations The term ‘pemphigus’ is derived from the Greek word
pemphix which literally means a blister or bubble.
Dermatitis herpetiformis can involve any part of the oral Chidgey (2002) in his update on desmosomes and disease
mucosa. It manifests as multiple vesicles are bullae. These described pemphigus as an autoimmune disorder that
fragile vesicles rupture immediately to form shallow pain- manifests with damage to the desmosomes of epithelial cells
ful ulcers. induced by the activity of antibodies against transmem-
brane desmosomal glycoproteins belonging to the cadherin
Investigations supergene family of desmogleins (Dsg), resulting in intra-
Histopathologically, presence of microabscesses in the epithelial immuno-deposits and breaking of intercellular
dermal papillae and subepithelial blister formation are bridges’. Table 4 summarizes the antigens targeted by auto-
some of the findings associated with DH. The characteris- antibodies in various forms of pemphigus.
tic finding in DH is the presence of eosinophilia.
Direct immunofluorescence test reveals the presence of Types of pemphigus
antibodies IgA, IgM and IgG at the junction of the dermis The three basic types of pemphigus depending upon the
and epidermis. extent of epithelial involvement and clinical presentation
Dietrich et al (1999) demonstrated that the level of include:
immunoglobulin A autoantibodies to endomysium in patients
with DH was significantly elevated. 1. Pemphigus vulgaris
Patients exhibit sensitivity to halogens. Pemphigus vegetans—variant of pemphigus vulgaris
characterized by excessive granulation and crusting
Management 2. Pemphigus foliaceous
Idiopathic pemphigus foliaceous
Dermatitis herpetiformis usually runs a prolonged course Drug induced pemphigus foliaceous
and sometimes persists for life. Patients are advised to Pemphigus erythematosus
avoid gluten in the diet. Dapsone is usually the drug of Endemic pemphigus foliaceous/fogo selvagem
choice. In the initial stages 50 mg/day is given and later 3. Paraneoplastic pemphigus-associated with neoplasms
increased to about 300 mg/day as per the requirement. Pemphigus vulgaris and vegetans—pemphigus foliaceous
and erythematosus
Affects entire epithelium
PREDOMINANTLY BULLOUS LESIONS Involves oral mucosa—affects upper prickle/spinous
layer of epithelium of skin
Pemphigus
Pemphigus is an autoimmune disease characterized by Pemphigus Vulgaris
intraepithelial blister formation due to a breakdown in inter-
Epidemiology
cellular adhesion. This breakdown process is referred to as
primary acantholysis. Prior to the advent of steroids, pem- Pemphigus vulgaris occurs equally in both sexes during the
phigus was fatal due to fluid loss and electrolyte imbal- 4th and 5th decades. Studies have shown a slight increased
ance and bacterial infection. Pemphigus presents itself as incidence in Ashkenazi Jews and individuals with HLA-DR,
clinically characterized vesicles and bullae. The lesions are HLA-A10, HLA-DRB1 phenotypes. Other autoimmune
181
diseases such as myasthenia gravis, lupus eythematosus, nail folds may be involved first, together with the oral
rheumatoid arthritis, Sjögren’s syndrome and Hashimoto’s lesions.
thyroiditis also may be present. ❍ Dermal lesions are characterized by bullae over the skin.
Fluid in the bullae appears clear at first but later it may
Etiopathogenesis become hemorrhagic or even seropurulent. Initially
The underlying mechanism causing the characteristic intra- the bullae are tense, but soon become flaccid and rup-
epithelial lesion of pemphigus vulgaris is the binding of ture to form erosions which ooze and bleed easily. The
specific IgG antibodies to an antigen on the epithelial cell denuded areas sometimes are partially covered with
membrane. crusts with little or no tendency to heal and enlarge by
The stimulus that triggers the abnormal IgG production confluence. The healed lesions usually leave hyperpig-
is unknown. However certain exogenous factors such as mented patches. However in some instances these
medications, dietary components and unknown environ- solitary erosive areas may coalesce and involve exten-
mental factors can induce or perpetuate pemphigus in a sive areas of the skin.
genetically predisposed individual. ❍ Nikolsky’s sign (first described by Pyotr Vasiliyevich
There is evidence that the binding of IgG antibody to Nikolsky in 1896) is positive. The Nikolsky’s sign is posi-
the pemphigus antigen leads to epithelial cell separation tive if slight pressure or rubbing of the skin produces
by triggering either complement activity or the plasminogen- lateral movement of the upper layers of the epidermis.
plasmin system. IgG autoantibodies are said to target two ❍ The Asboe-Hansen sign, or ‘bulla spread phenomenon’,
structural proteins of the desmosomes namely desmoglein 1 is positive in pemphigus. Gentle pressure on an intact
and desmoglein 3. Recently a new pemphigus antigen des- bulla will force the fluid to spread under the skin away
moglein 4 has been discovered and implicated in the from the site of pressure.
pathogenesis of pemphigus vulgaris.
Oral manifestations
Immunogenetic aspects of etiology ❍ Oral lesions usually appear first in this disease. Almost
The increased incidence of pemphigus in Jews leads to the 80–90% of the patients with pemphigus vulgaris develop
proposition that there is a genetic predisposition to the oral lesions sometime during the course of the disease
disease and initial studies examined the frequency of MHC and in 60% of the cases oral lesions occur first.
class I antigens in affected individuals. The first evidence ❍ The typical oral lesion begins as a bulla on a non-
for genetically determined susceptibility was the demon- inflamed base, which almost immediately ruptures to
stration by Krain et al (1973) of an increased frequency of produce shallow ulcer. The margins of the ulcer show
HLA-A10 in Caucasian patients with pemphigus vulgaris, evidence of tissue tags.
especially in those who were Jewish. A strong association ❍ The commonly involved sites are the buccal mucosa
with HLA-A10 was also noted in Japanese patients by (sites of trauma along the occlusal plane), gingiva and
Hashimoto et al (1977). Katz et al (1973) have reported an palate (Figure 6A, B).
association between HLA-A13 and pemphigus vulgaris, ❍ The edges of the shallow ulcers extend peripherally
but this has not been confirmed by others. over a period of weeks until they involve large por-
Subsequent studies have shown that almost all pemphigus tions of the oral mucosa.
vulgaris patients have either HLA-DR4 or DRW6 hapto- ❍ Distal extension from the oral cavity causes involve-
types. Also the disease susceptibility has been linked to an ment of the esophagus, pharynx and larynx, which
HLA-DQB gene. causes hoarseness of voice and dysphagia.
Clinical features Differential diagnosis
❍ Occurs in the 5th and 6th decade of life. Rarely affects Various bullous diseases that pemphigus vulgaris must be
younger individuals. differentiated are epidermolysis bullosa, erythema multi-
❍ Men and women are equally affected. forme, bullous pemphigoid, cicatricial pemphigoid, bul-
❍ It most commonly occurs in Jews, Greeks, east Indians lous drug eruptions and other forms of pemphigus.
and individuals from the orient. The histological presence of suprabasal intraepidermal
❍ Pemphigus vulgaris affects the mucosa and skin, bulla with acantholysis is characteristic of pemphigus and
resulting in superficial blisters and chronic ulceration. usually differentiates it from other similar diseases.
Various mucosal surfaces may be involved such as the
Investigations
oral, ocular, nasal, pharyngeal, laryngeal, upper respi-
ratory and anogenital mucous membranes. Cytology Smears taken from freshly opened vesicles are
❍ The common sites of involvement are the groin, usually preferred. Tzanck cells can be seen. These are epi-
scalp, face, neck, axillae and genitals. However the thelial cells that are free in the vesicular space and are
182
Figure 6
A B
(A) Shallow ulcers on the buccal mucosa in a patient with pemphigus vulgaris.
(B) Shallow ulcers on the palate in a patient with pemphigus vulgaris. Courtesy: Dr Sumanth
characterized particularly by degenerative changes which

Figure 7
include swelling of the nuclei and hyperchromatic stain-
ing. This is also referred to as Tzanck test.
Shklar in 1971 reported that there is a marked increase
in RNA in the cytoplasm of these acantholytic cells as well
as in the epithelial cells at the floor of the vesicles.
Histopathology Tissue specimen for biopsies is obtained
from the active border of a denuded area, as intact blisters
are rarely seen.
The characteristic histological finding is an intraepidermal
bulla resulting from a loss of cell-cell adhesion of kerati-
nocytes. Although the basal cells lose lateral desmosomal
contact with the adjacent cells, hemidesmosomes are intact.
Therefore, attachment to the basement membrane is main-
tained, giving the appearance of a ‘row of tombstones’.
Other pathologic changes seen are acantholysis, cleft
and blister formation in the interdermal areas just above Photomicrograph of the histological section of pemphigus
the basal cell layer and acantholytic cells. The separation vulgaris. Courtesy: Department of Oral Pathology,
of cells in the layers of the stratum spinosum is known as MCODS, Mangalore
acantholysis. The loss of contact between the malphigian
cells begins with the detachment of tonofilaments from
the desmosomes, loss of intercellular cement substances
and cellular degeneration with formation of the interepi- approached 90%. However in the recent times the mortal-
dermal cleft or bulla (Figure 7). ity rate is said to be approximately 10%.
Immunofluorescent studies In pemphigus vulgaris the Topical therapy
antibody will bind the immunoglobulin deposits in the ❍ Eroded and crusted, painful skin lesions and the asso-
intercellular substance and exhibits a positive fluores- ciated foul odor can be effectively managed by bathing
cence under the fluorescent microscope. This is termed the area with 0.01% potassium permanganate solution
‘fish-net’ pattern of binding. or 0.5% silver nitrate solution.
❍ Alternatively the raw surfaces can be sprayed with
Management
corticosteroids or 2% procaine hydrochloride.
Pemphigus was traditionally considered a potentially fatal ❍ Chlorhexidine mouthrinses can be used to alleviate
disease. Before the advent of effective therapies, mortality discomfort and malodor.
183
❍ Painful oral ulcerations can be managed by topical appli- ulcerations and polymorphous skin lesions that progressed
cation of viscous xylocaine especially before food intake. to blisters on the trunk and extremities. It was found that
the autoantibodies from these patients reacted with an anti-
Systemic therapy gen complex composed of desmoplakin I and the 230-kd
❍ Corticosteroids: Systemic administration of cortico- antigen of bullous pemphigoid.
steroids comprises three phases: Paraneoplastic pemphigus is characterized by extensive
– Control phase: Characterized by an initial high dose mucocutaneous erosions associated with a neoplasm such
corticosteroid administration to the point of obvi- as leukemia, lymphoma, sarcomas, thymomas, Castleman’s
ous clinical improvement. Therapy is initiated by disease, Waldenström’s macroglobulinemia (high levels of
giving 60–160 mg of prednisone daily. If there is no macroglobulin [IgM], increased serum viscosity, and lym-
response even after a week, the dosage is doubled. phoplasmacytic infiltrate in the bone marrow) pancreatic
When new lesions cease to form and old lesions carcinoma, bronchogenic squamous cell carcinoma or intra-
heal, the dosage is decreased slowly. Lever (1977) ductal breast carcinoma.
suggests 180–360 mg of prednisone daily for It is believed that almost 80% of the patient’s suffer
6–10 weeks. from either non-Hodgkin’s lymphoma, chronic lympho-
– Consolidation phase: In this phase the dosage of cytic leukemia or Castleman’s disease.
prednisone is reduced over a period of several
weeks. According to Arnold, once the control over Clinical features
the disease is achieved, an attempt to decrease the
steroid dose by transferring the patient to intra- The predominant feature of paraneoplastic pemphigus is
muscular injections of triamcinolone acetonide is painful mucous membrane erosions, of which oral ero-
highly advisable. sions are the first sign of disease in 22.2% of cases. The most
– Maintenance phase: The corticosteroids are gradu- common sites involved are the lips and oral mucosa, with
ally tapered down to alternate day dose and ulti- multiple, severe, persistent erosions. Symptoms of oropha-
mately stopped. However this reduction in dosage ryngeal involvement may include sore throat and dyspha-
is made possible by replacing steroids with immu- gia. Bilateral conjunctival involvement has been noted in
nosuppressive drugs. The dosage of immunosup- up to 72.2% of cases.
pressive drugs is reduced to zero in several months. The skin lesions vary in shape and size, with a conflu-
❍ Immunosuppressive agents: Azathioprine 100–200 mg ent erythema of the trunk, on which blisters and erosions
per day in conjunction with prednisone 150–200 mg form. Erythematous maculopapular lesions with dusky cen-
daily can be used. Fellner et al reported good results ters or central vesicles may arise on the extremities, mimick-
by combining 200 mg of prednisone with 100–200 mg ing target lesions seen in erythema multiforme. Occasionally,
cyclophosphamide. the lesions may be pruritic. Paraneoplastic pemphigus is an
Other agents that have been used with mixed extremely rare entity that has an onset at 60 years or older
results are dapsone, gold sodium thiomalate and and is more common in women than men.
aurothioglucose.
❍ Plasmapheresis: It is particularly useful in patients who Diagnostic criteria
are refractory to corticosteroids. It involves removal of Anhalt in 2004 proposed a four-point minimal criteria for
the circulating antibodies. diagnosing paraneoplastic pemphigus:
❍ Photopheresis: This modality of treatment was described
by Rook et al. It involves administration of 8-methoxy- 1. Painful and progressive stomatitis which preferentially
psoralen followed by exposure of peripheral blood to involves the tongue. This criterion is the most charac-
ultraviolet radiation, causing photoinactivation of WBC. teristic of this condition.
❍ Immunomodulators: Chaffins et al in 1993 reported 2. Histologic findings of acantholysis or lichenoid or
that drugs like levamisole (100 mg/week), combination interface dermatitis.
of nicotinamide and tetracycline and oral prostaglandins 3. Demonstration of antiplakin autoantibodies, which are
are effective in the treatment of pemphigus. the key serological markers for this condition.
4. Demonstration of an underlying lymphoproliferative
neoplasm.
Paraneoplastic Pemphigus
(Paraneoplastic Autoimmune Multiorgan Syndrome) Histopathologic features and immunological
studies
Anhalt and coworkers in 1990 were the first to suggest the
term paraneoplastic pemphigus for a clinically and immu- On histopathologic examination, paraneoplastic pemphi-
nologically distinct condition. They reported five patients gus appears to be a combination of pemphigus vulgaris and
with underlying neoplasms associated with painful mucosal erythema multiforme. There is suprabasilar acantholysis as
184
seen in pemphigus vulgaris, as well as basal cell vacuola- patients have autoantibodies binding to an immunodomi-
tion, lymphocytic exocytosis, and dyskeratotic keratino- nant region of BP180, the non-collagenous 16A domain
cytes typical of erythema multiforme. (NC16A), which is located extracellularly close to the
Paraneoplastic pemphigus is distinguished from the transmembrane domain of the protein. Autoreactive T and
other forms of pemphigus as direct immunofluorescence B cell responses to BP180 have also been found in patients
reveals not only IgG and C3 deposits within the intercel- with BP.
lular spaces but also along the basement membrane zone. Wohl and others (2008) studied the expression of vincu-
In the classic forms of pemphigus, indirect immuno- lin in autoimmune cutaneous diseases. Vinculin is a cyto-
fluorescence is positive only on stratified squamous epi- skeletal protein associated with cell to cell and cell to
thelial substrates. However, in paraneoplastic pemphigus, matrix junctions, where it is thought to function as one of
there is staining of other tissues, including the bladder, heart, several interacting proteins involved in anchoring F-actin
and liver. IgG autoantibodies are directed against desmo- to the membrane.
plakins I and II (components of the cytoplasmic plaque), On semiquantitative immunohistochemistry investiga-
which are present in stratified squamous epithelium and tions they found that the expression and distribution of
these other tissues. vinculin are accentuated in patients with various skin
autoimmune diseases and appear to be stronger in diseases
Management and prognosis involving the basement membrane.
There is little to offer in the treatment of paraneoplastic
pemphigus. If a benign tumor is resected, some patients Clinical features
may go into remission. Unfortunately, the prognosis is
Three distinct clinical types BP have been reported: the
generally poor, and treatment is usually unsuccessful.
commonly known adult form of BP and the relatively rare
Immunosuppressive treatment and plasmapheresis have
forms of BP occurring at infancy and childhood.
not been effective; however, immunophoresis may be a
The adult form of BP typically occurs between the 6th
promising alternative.
and 8th decades of life. Males and females can be equally
Paraneoplastic pemphigus is a rapidly progressive bul-
affected. BP shows no ethnic or racial predilection. Dermal
lous disease that is invariably fatal when associated with a
lesions of BP are polymorphous in nature. Literature is
malignant tumor. When paraneoplastic pemphigus occurs
replete with reports of non-bullous forms of BP. They can
in the context of a benign neoplasm, the mucocutaneous
present as urticarial papules, plaques, vegetating forms,
erosions will usually show gradual resolution after excision
nodular lesions, hyperkeratotic areas and erythematous and
of the tumor. It is important to remember that paraneo-
eczematous lesions.
plastic pemphigus may precede the clinical appearance of
Initial lesions can usually present as urticarial erup-
a neoplasm; therefore, it is mandatory that these patients
tions that progress to bullae over weeks or months. The
receive screening for neoplasms and regular follow-up care.
bullae are pruritic, large and tense. However they gener-
Other forms of pemphigus are described in Chapter 20,
ally persist longer due to their thick wall. Over a few days
Autoimmune Disorders.
the bullae rupture to form large and tender eroded regions.
These bullae can occur in any part of the body such as the
Bullous Pemphigoid axillae, abdomen, legs, forearms and groin.
The bullae may contain clear fluid or at occasions con-
Bullous pemphigoid (BP) was first described as a distinct
tain hemorrhagic content. The erosions heal without scar
clinical entity by Lever in 1953. It is an autoimmune con-
formation.
dition characterized by subepidermal blistering resulting
Involvement of the mucous membrane is relatively
in large, tense bullae involving the skin and rarely the mucous
uncommon in BP compared to pemphigus vulgaris. The
membrane. Autoantibodies are targeted at the components
oral cavity and genital and anal mucosa can be affected.
of the basement membrane.
Oral bullae tend to rupture and form erosions or ulcers due
to the frequent micro trauma sustained during mastication.
Pathophysiology
Childhood BP though rare, is the most common IgG
Kasperkiewicz et al (2007) reviewed the pathophysiology of mediated subepidermal bullous disease in children. It typi-
BP. They described that the autoimmune response in BP is cally affects children below the age of 18 years and involves
directed against two hemidesmosomal proteins within the the skin. The mucous membrane involvement is relatively
dermal-epidermal junction, namely BP180 and BP230 (also more common than the adult from of BP. The histopathologic
known as BPAG1). picture is similar to the adult form consisting of subepider-
BP230 localizes intracellularly and associates with the mal bullae with variable amount of eosinophils and direct
hemidesmosomal plaque. BP180 is a transmembrane immunofluorescent studies show linear deposition of IgG
glycoprotein with an extracellular domain. Most of the and/or C3 at the basement membrane zone.
185
The infantile form of BP is seen in infants in the first Management and prognosis
year of life. The mucous membrane involvement is slightly
The clinical course of BP is limited, usually lasting from a
more common than the adult form of BP. However the
few months to years. It is seldom fatal. Almost 50% of the
characteristic feature is the involvement of hand and feet.
patients exhibit remission in about 6 year duration.
Maria Isabel Martinez-De Pablo and others (2007) suggest
Mild cases of BP can effectively be managed with topi-
that the IgA mediated autoimmune response predominates
cal corticosteroids. Systemic steroids such as prednisolone
in children in contrast to the IgG response that is predomi-
is used for managing BP. Steroids can be tapered off with
nant in adults, due to immunological immaturity or fre-
improvement of the condition. Steroids can be used in
quent exposure to infectious agents and/or vaccines.
combination with immunomodulatory drugs such as dap-
Waisbourd-Zinman and others (2008) compared the
sone, azathioprine and methotrexate.
features of infantile BP with childhood BP. They found
that the number of infantile BP individuals is steadily increas-
ing. The predisposition for acral (extremities like the hands Mucous Membrane Pemphigoid or Cicatricial
and legs) involvement is significantly higher in infants. Pemphigoid
However the genital involvement is very rare in infantile
BP compared to childhood BP. The deposition of IgM at Mucous membrane pemphigoid is an autoimmune chronic
the basement membrane zone was higher in the childhood inflammatory disorder characterized by subepithelial blis-
variety of BP. tering. It affects the skin, oral mucosa and ocular mucosa.
The name cicatricial pemphigoid is attributed to scarring
Oral manifestations of the conjunctival mucosa.
Bullous pemphigoid may or not affect the oral mucosa. Etiology and pathogenesis
The incidence of oral involvement varies from 8 to 45%.
Some reports suggest that the oral lesions precede the skin The etiology of mucous membrane pemphigoid is unknown.
lesions. The pathogenesis is believed to be caused by the reaction
of IgG antibody with BP protein 180 and laminin 5 which
❍ Oral lesions are characterized by small bullae, which are present in lamina lucida of the basement membrane
form slowly and are less painful than those seen in complex resulting in breakdown of the attachment between
pemphigus vulgaris. epithelium and connective tissue. The molecular players
❍ Buccal mucosal gingiva are relatively more commonly responsible for the pathogenesis in blister formation are
involved. still unknown.
❍ Gingival lesions consist of generalized edema, inflam-
mation and desquamation with localized area of
Clinical features
vesicle formation.
❍ Oral lesions of bullous pemphigoid are clearly indis- Cicatricial pemphigoid is characterized by the presence of
tinguishable from oral lesions of cicatricial pemphigoid, vesicles which heal by scarring. These vesicles generally
but remission of bullous pemphigoid is more common. tend to occur on the mucous membranes of the oral cavity
❍ Bullous pemphigoid may coexist with lichen planus and conjunctiva. It has been reported that 90% of the
and referred to as lichen planus pemphigoides. patients have oral involvement, 66% have conjunctival
lesions and approximately 25% have skin involvement.
Histopathological and immunofluorescent studies Erosions and scarring may involve other mucous mem-
branes, including those of the nasopharynx, oropharynx,
Biopsy specimen from the perilesional area will show sub- esophagus, larynx, vagina, penis and rectum. Esophageal
epithelial separation. The epithelium is separated from the involvement can cause dysphagia secondary to fibrous
connective tissue at the level of the basement membrane. adhesions and scarring. Laryngeal erosions, edema and
The typical finding in BP is the presence of eosinophils scarring can be life threatening.
within the content of the bullae. Other inflammatory cells
such as lymphocytes and histiocytes are seen.
Oral lesions
Electron microscopy reveals presence of the bullae within
the lamina lucida of the basement membrane resulting in ❍ Oral lesions can have two clinical presentations—ero-
destruction of the anchoring filaments and desmosomes. sions on the non-keratinized mucosa/keratinized gin-
Generally direct immunofluorescence will be sufficient giva or desquamative gingivitis.
to diagnose BP. However, some patients demonstrate cir- ❍ The common sites of involvement are the facial gin-
culating autoantibodies in the serum. Direct and indirect giva (64%), buccal mucosa (58%), palate (26%), eden-
immunofluorescence reveals deposition of IgG and C3, tulous alveolar ridge, especially under a prosthetic
along the basement membrane zone in a linear fashion. appliance (16%), tongue (15%) and lower lip (15%).
186
Figure 8 Figure 10
Oral erosions with distinct margins on the palate in cicatricial Symblepharon formation in cicatricial pemphigoid.
pemphigoid. Courtesy: Dr Sumanth Courtesy: Dr Sumanth, Department of Oral Medicine and
Figure 9
Ocular findings
❍ Subconjunctival scarring leading to blindness in about
15% of the patients.
❍ Initial lesions may be limited to the upper tarsal con-
junctiva, where they escape detection if the eyelid is
not everted.
❍ Fibrous tracts fuse the scleral and palpebral conjunc-
tiva resulting in symblepharon formation (Figure 10).
Histopathology
The classic histological feature of mucous membrane pem-
phigoid is separation of the epithelium from the under-
lying connective tissue due to subepithelial separation.
A mixed inflammatory infiltrate is present in the underly-
Desquamative gingivitis in cicatricial pemphigoid, ing submucosa (Figure 11). Direct immunofluorescence
Courtesy: Dr Sumanth demonstrates linear deposition of IgG and C3 at the base-
ment membrane (Figures 12 and 13).
❍ Intact vesicles are rarely seen. However according to Differential diagnosis

Martin (1998), it is much more likely to see an intact
Mucous membrane pemphigoid must be differentiated
vesicle in cicatricial pemphigoid than in pemphigus as
from pemphigus vulgaris, BP, lupus erythematosus, lichen
the lesions are thicker walled and they are subepithelial.
planus and paraneoplastic pemphigus. Bullous pemphigoid
❍ The oral ulcers or erosions have a distinct margin. These
is similar to mucous membrane pemphigoid, but BP is com-
ulcers generally may become secondarily infected and
monly seen in older women with circulating antibodies to
eventually heal in 3–4 weeks accompanied by scarring
BP protein 230 in addition to BP antigen 180.
and fibrosis (Figure 8).
❍ Desquamative gingivitis can present as varying degree
of erythematous gingiva either localized or generalized.
Areas of erosion and denudation of the gingival surface There is no single treatment for mucous membrane pem-
may be noticed (Figure 9). phigoid, as it must be tailored to the individual patient sim-
❍ Nikolsky’s sign is positive. ilar to pemphigus vulgaris. If extensive lesions involving
❍ Spontaneous gingival bleeding may be seen. the oral cavity are present, systemic prednisone may be
187
Figure 11 Figure 13
Direct immunofluorescence pattern of benign mucous

membrane pemphigoid with C3 deposited against the
basement membrane. Courtesy: Dr Carol Stewart,
Florida, USA
Intact basal cells and sub-basal separation is seen in benign
mucous membrane pemphigoid. H&E stained section (20⫻).
Courtesy: Dr Nagamani Narayana, Nebraska, USA
If lesions are extensive, immunosuppressive medications
such as azathioprine, mycophenolate and cyclophosphamide
may be necessary to manage symptoms. Also, a combina-
Figure 12 tion of tetracycline and niacinamide (niacin flush free),
500 mg taken three or four times a day for 6 months with
slow tapering, may be helpful in controlling the disease.
This latter combination has no known serious side effects
and is tolerated well by patients. It is important to educate
patients about the disease process and inform them that
treatment does not cure the condition, but only helps to
control the disease. The disease may cycle through periods
of exacerbations and remission over time. These patients
should be instructed to undergo an annual eye examina-
tion to avoid complications such as blindness.
BULLOUS LICHEN PLANUS
Hebra used the term ‘leichen ruber’ to describe lichen pla-

nus. However in 1869, Erasmus Wilson introduced the
Direct immunofluorescence pattern of benign mucous term lichen planus (LP). It is believed that almost 0.5 to 1%
membrane pemphigoid with the autoantibody IgG-1 of the population is affected by lichen planus. Typically
deposited against the basement membrane.
lichen planus affecting the skin presents as violaceous,
Courtesy: Dr Carol Stewart, Florida, USA
polygonal flat topped papules, usually associated with
white striae on their surface.
Andreasen classified oral lichen planus into six types
indicated. Normally a short course of prednisone is pre- namely reticular, plaque-like, erosive, papular, atrophic
scribed (40 mg per day for 7 days without tapering). Topical and bullous lichen planus. It is believed that almost 25%
steroids may be prescribed either alone or in addition of the individuals present with oral manifestations alone
to systemic steroids, as ointments or oral rinse solutions. and about 50% of the patients with skin lesions have oral
188
lesions. Oral lesions are usually seen in women between morphological appearances such as macule, papule, bullae
the 4th and 7th decades of life. and crust, hence the name ‘multiforme’.
Bullous form of lichen planus is the rarest form of the Previously, EM consisted of a disease spectrum that
various varieties of oral lichen planus. Bullous oral lichen ranged from a mild or minor form, severe or major EM,
planus manifests as small vesicles or bullae that rupture Stevens–Johnson syndrome (SJS) and the most severe form
easily to form shallow erosions. The lesions of bullous oral of the disease, toxic epidermal necrosis (TEN) or Lyell’s
lichen planus vary in size from few millimeters to several syndrome.
centimeters in diameter. Patients usually complain of burn- The present understanding of the disease process, groups
ing sensation and pain when the bullae rupture to form EM minor and major into the EM spectrum and SJS and
erosive areas. These erosions mimic the clinical presenta- TEN into another group. EM shows very minimal mucosal
tion of erosive lichen planus. involvement and less than 10% epidermal detachment.
Bullous lichen planus commonly affects the buccal
mucosa (frequently seen adjacent to the second and third Etiopathogenesis
molar teeth) and lateral margins of tongue. The less com-
It is estimated that almost 70 to 80% of the cases of EM are
monly affected sites include the gingiva and labial mucosa.
triggered by HSV 1 and 2 infections. Besides HSV other
The exact etiology of bullous lichen planus is still unclear.
bacterial, viral and fungal infections have been reported to
However it has been suggested that there is an immuno-
trigger EM attacks. Viruses such as CMV, VZV and HIV
logically induced degeneration of the basal cell layer of
have been less frequently associated with EM. In children
the oral mucosa.
Mycoplasma pneumoniae is reportedly a common etiolog-
Histopathological findings ical agent.
Other popular predisposing factors include drugs, chemi-
Histopathologic evaluation is required for identifying bul- cals and food coloring and flavoring agents. Medications
lous lichen planus. The histopathologic features of dermal that have been implicated in triggering EM include non-
lichen planus and bullous lichen planus are the same such steroidal anti-inflammatory drugs, penicillins, phenothia-
as hyperkeratosis and basal cell degeneration, a band of zines, sulfonamides, barbiturates, hydantoins, ciprofloxacin
inflammatory cells at the epithelial connective tissue inter- and metformin. EM has also been reported to occur after
face, and Civatte bodies may be seen in the epidermal or hepatitis B, smallpox and diphtheria-pertussis-tetanus
within the upper dermal cell layers. (DPT) vaccinations.
Gawkrodger et al (1989) stated that the characteristic Aurelian et al (2003) and Kokuba et al (1999) studied the
feature of bullous lichen planus is the subepidermal clefting association of EM with HSV and drug intake respectively.
leading to separation of the epithelium from the basement They reported that the pathogenesis of herpes-associated
membrane zone. However direct or indirect immunofluo- erythema multiforme is akin to a delayed type hypersensi-
rescence labeling is negative. tivity reaction.
Lichen planus pemphigoides, bullous pemphigus and The disease begins with the transport of viral DNA
pemphigus vulgaris have to be differentiated from bullous fragments to distant skin sites by peripheral blood mono-
lichen planus. nuclear cells. HSV genes within DNA fragments are
expressed on keratinocytes, leading to the recruitment of
Management HSV-specific CD4⫹ Th1 cells (helper T cells involved in
Topical or systemic steroids, azathioprine, dapsone, cyclo- cell-mediated immunity). The CD4⫹ cells respond to viral
sporine, mycophenolate (Nousari et al, 1999) and retinoids antigens with production of interferon-gamma, initiating
have all been used to manage bullous lichen planus. Anne- an inflammatory cascade.
Moon van Tuyll van Serooskerken et al (2007) successfully However the drug-associated erythema multiforme lesions
treated bullous lichen planus on the lip with a combina- exhibit positivity for tumor necrosis factor-alpha and not
tion of tretinoin 0.025% and triamcinolone 0.1%. interferon-gamma as in herpes-associated erythema mul-
tiforme lesions.
ERYTHEMA MULTIFORME Clinical features

Erythema multiforme may affect individuals of any age.
Erythema multiforme (EM) is an acute, reactive, self-limiting However, it is more common in young adults. Males and
and recurring mucocutaneous disorder that causes blister- females are equally affected. EM lesions seldom present
ing and ulceration of the skin and mucous membranes. with prodromal symptoms. However mild and non-specific
Hebra in 1866 described EM as a benign condition char- symptoms are reported by some patients such as low-grade
acterized by skin lesions with concentric color changes, fever, malaise, myalgia and diarrhea. The lesions of EM
which were distributed symmetrically. EM has various usually appear within 2–3 days of onset of the disease.
189
Figure 14 Some authors propose that though HSV infection may

not be readily apparent, subclinical attacks of HSV may
play an important role in the pathogenesis of the dis-
ease. Aurelian and others (1998) suggest that most cases
of recurrent EM may be herpes-related.
Sen and Chua (2004) reported that the recurrent
attack usually occurs even before the lesions from the
previous attack have completely resolved. They suggest
that the individuals suffering from recurrent EM report
two or more attacks of erythema multiforme per year,
with each episode lasting for about 2 weeks.
However one should not use the diagnostic term of
recurrent erythema multiforme for individuals with per-
sistent skin lesions lasting weeks to months or a chronic
non-episodic course of the disease. One should not con-
Photograph showing the characteristic target lesion on the
sider recurrent erythema multiforme when an individual
patient’s back. Courtesy: Department of Oral Medicine and exhibits only acute mucosal inflammation without skin
Radiology, KLE Society’s Institute of Dental Sciences, lesions.
Bangalore The presence of the typical skin lesion is indispensable
for the diagnosis of the recurrent form of erythema multi-
forme.
The lesions of EM seldom involve more than 10% of the
surface area of the body. Oral manifestations
Though the lesions of EM may involve any part of the
Mucosal lesions of erythema multiforme are usually
body, they are common in the sites of sun exposure, irrita-
restricted to the oral mucosa. However in the major form
tion or trauma. The face, oral mucosa, dorsal surface of the
of the condition mucosa of the eye and genitalia are fre-
hands, feet, elbows and knees are the sites that are com-
quently affected. It is estimated that approximately 40 to
monly affected. The lesions of EM are usually restricted to
60% of the individuals suffering from erythema multi-
the oral mucosa unlike Stevens–Johnson syndrome.
forme major have oral manifestations. Lips and anterior
The typical dermal lesions of EM are target, iris or bull’s
parts of the oral cavity are frequently affected. The oral
eye lesion. These are asymptomatic, discrete, erythema-
lesions of erythema multiforme are manifested as bullae,
tous macules or papules set in a concentric ring pattern
erosions or ulcers, which may or may not be covered by
usually comprising a central bulla. The iris lesion has three
a pseudomembrane. Target lesions when seen are usu-
concentric zones: a central dusky or darker red area (cen-
ally restricted to the lips. The vermillion border of the
tral bulla or area of necrosis), a paler pink or edematous
lip exhibits a characteristic ‘bloody crusted’ appearance
zone, and a peripheral erythematous zone (Figure 14).
(Figure 15). These lesions generally heal without scar-
The size of the iris lesion varies from a few mm to about
ring. Gingiva is typically uninvolved.
2 cm in diameter. These lesions usually resolve in about
3 to 5 weeks. However recurrence is generally reported
Diagnosis
in EM.
The diagnosis of EM is usually by the typical clinical
Recurrent Erythema Multiforme features. Histopathological features are not specific and
hence not diagnostic.
Schofield et al (1993) studied 65 patients who had recur- Perivascular infiltrate of CD4 and CD8 lymphocytes
rent EM. They observed that the mean number of recur- surrounding swollen blood vessels in the upper dermis
rences was 6 per year and the mean duration of the with papillary dermal edema is seen.
disease was 9.5 years. Vacuolar degeneration of the basal layer, subdermal
Sen and Chua (2004) described that the HSV asso- blister formation and epidermal necrosis of keratino-
ciated EM, mycoplasma associated EM and drug asso- cytes can be appreciated. The severity of the keratino-
ciated EM (repeated readministration) usually present cyte necrosis increases with the advancing lesion. The
as recurrent EM. They state that infectious agents, individual necrotic keratinocytes are surrounded by CD8
commonly viruses (due to reactivation), are likely to be cells termed ‘satellite cell necrosis’. An overproduction
chiefly associated with recurrent EM. It is estimated that of interferon-gamma by CD4⫹ T helper-1 cells is seen in
about 70% of cases have disease precipitated by HSV. the epidermis.
190
Figure 15 STEVENS–JOHNSON SYNDROME AND

TOXIC EPIDERMAL NECROLYSIS
(Lyell’s Syndrome)
Stevens–Johnson syndrome (SJS) and toxic epidermal

necrolysis (TEN) are presently considered as a spectrum of
the same disease process. They can be distinguished from
erythema multiforme, varying only in the area of involve-
ment of skin surface. The acute life-threatening conditions
are characterized by epidermal sloughing and mucositis
secondary to extensive keratinocyte apoptosis.
Etiopathogenesis
Murata and Abe (2007) reported that the keratinocyte apop-
Bloody crusted appearance of the lips in a patient with
tosis is brought about by the interaction of the fas receptor
erythema multiforme. Courtesy: Department of Oral Medicine and fas ligand. They also emphasize the need to evaluate
and Radiology, MCODS, Mangalore other genetic factors that may predispose to TEN and SJS.
Lerner et al (2000) used reverse transcriptase-PCR and
immuniperoxidase staining to study the role of inducible
form of nitric oxide synthetase in generating large amounts
of nitric oxide. They proposed that a large burst of nitric
Ng and others (2003) used nested PCR to detect HSV- oxide in TEN and SJS may cause the epidermal apoptosis
DNA in skin biopsies with histologically proven EM. and necrosis.
Their findings showed that PCR was positive in 60% of In their study, inducible nitric oxide synthase was detected
the patients with HSV-related recurrent EM and PCR by reverse transcription polymerase chain reaction. Strong
was positive in 50% of the patients with idiopathic staining for inducible nitric oxide synthase was observed
recurrent EM. in inflammatory cells in the lower epidermis and upper
dermis.
Differential diagnosis Drugs are considered to be the most common cause of
Dermal lesions may mimic lesions of hypersensitivity SJS/TEN. The drugs that commonly cause SJS/TEN are anti-
reactions, drug eruptions and urticarial lesions. Intra- convulsants, sulfonamides, non-steroidal anti-inflammatory
oral lesions may resemble recurrent aphthous stomati- drugs and antibiotics.
tis, contact stomatitis and ANUG when the gingiva is SJS/TEN is considered as a cytotoxic immune reaction
involved. Other conditions that can be considered are causing destruction of keratinocytes expressing drug-related
pemphigus and varicella infections. antigens. TNF-alpha derived from macrophages and kera-
tinocytes may play an important role in the pathogenesis
Management by inducing apoptosis of epidermal cells or by attracting
cytotoxic effector cells, or both. Drug metabolites such as
Patients with mild EM usually do not require any form hydroxylamines and arene oxides derived from sulfon-
of treatment as the condition is self-limiting. Since EM amides and aromatic anticonvulsants respectively, bind to
is usually associated with HSV, oral acyclovir will mini- cell constituents if they are not rapidly detoxified by epox-
mize the duration of the condition. Topical steroids will ide hydrolase. These metabolites act as haptens and render
provide symptomatic relief. the keratinocytes antigenic by binding to them. A defect
The incidence of recurrent EM drastically falls down in the detoxification system may be the cause of the drug
following treatment with valacyclovir (500–1,000 mg/ eruption.
day) or famciclovir (125–250 mg/day).
Severe recurrent EM can be managed with immuno- Clinical features
suppressive agents. Dapsone or antimalarials (hydroxy-
chloroquine) are the primary drugs in the treatment of Toxic epidermal necrolysis and Stevens–Johnson syndrome
recurrent EM. Azathioprine also helps in suppressing the are usually seen in adults. Both the conditions occur as a
condition, however it has many side effects. Davis et al single episode and associated with drug exposure.
(2002) showed that mycophenolate mofetil can be used Literature review reveals various age groups being
as an effective drug in managing recurrent EM. involved. The involvement of men and women also shows
inconsistency.
191
Devi et al (2005) conducted a retrospective study of University of Florida management guidelines for
41 patients diagnosed as SJS or TEN in India. The young- TEN and SJS
est patient in their study was 12 years old and the oldest
1. Admit patient directly to the burn intensive care unit
was 72. Majority of the patients were in the 2nd to 5th
(BICU) or an intensive care setting
decade of life.
2. Discontinue corticosteroids if they are being used to
Kaur et al (1990) evaluated 30 patients with drug
treat the eruption
induced TEN in India. Their study revealed that the
3. Discontinue unnecessary medications and suspect
peak incidence of TEN was in the 4th to 6th decades
medications
of life.
4. Obtain baseline laboratory tests, such as complete blood
It is believed that the increased incidence in the elderly
count, liver function tests, metabolic panel, chest radio-
is due to their increased medicine intake and altered drug
graphs, and any other appropriate imaging or serologic
metabolism with advancing age.
tests, including an immediate (stat) immunoglobulin
Kaur et al reported that males were slightly more com-
A (IgA) serum level
monly affected. Devi et al showed that males and females
5. Look carefully for evidence of infection
were equally affected.
6. Obtain punch biopsies of skin for diagnosis confirma-
Most of the lesions of SJS or TEN are usually seen
tion. An alternative for rapid diagnosis is removal of
within 2 months of drug intake.
a bulla roof for immediate frozen sections to differen-
However the recurrent lesions tend to appear within a
tiate between TEN and staphylococcal scalded skin
week of a repeat episode of drug intake. The striking fea-
syndrome (SSSS)
ture of SJS and TEN are that they do not recur if the
7. Culture skin, blood, body orifices (as appropriate), and
offending drug is not taken, unlike in EM.
urine daily to monitor for early infection, and keep
Prodromal symptoms such as fever, nausea, vomiting,
abreast of changing antibiotic sensitivities
malaise, sore throat and rhinitis may been seen. This pro-
8. Use systemic antibiotics only for documented infec-
dromal phase is followed by the appearance of macular
tions or signs of sepsis
rashes affecting almost all regions of the body. It is
9. Place large-bore intravenous lines or a central venous
believed that TEN may be considered if the rash involves
line in an area of uninvolved skin to ensure adequate
more than 30% of the surface area of the body.
intravenous access
Severe sloughing of the skin and mucous membrane is
10. If within 48–72 hours of bulla onset, use intravenous
evident. Some authors describe this finding as ‘scalded
immunoglobulin (IVIG), sucrose depleted, 1 g/kg/day for
appearance’. Nikolsky’s sign and target lesions may be evi-
3 days infused over 4 hours. If 72 hours have passed,
dent in some individuals. The oral, ocular and genital
but the patient is still actively progressing with new
mucosae are affected in SJS.
lesions, IVIG may still be useful
The ocular lesions present as corneal ulcerations, con-
11. Monitor fluid and electrolytes closely and begin total
junctival scarring, uveitis and blindness. Genital lesions
parenteral nutrition (TPN) in patients unable to take
are characterized by genital ulcers and phimosis. Extensive
nourishment. Fluid replacement need not be as
sloughing may lead to secondary skin infection, sepsis,
aggressive as for burns of same extent
renal failure and death.
12. Debridement of necrotic and desquamating areas may
be performed
Histopathological and laboratory studies
13. Consult ophthalmologist to assess ocular involvement
Extensive epidermal necrosis is appreciated. Markedly 14. Consult otorhinolaryngologist to evaluate extent of
lowered number of lymphocytes are seen around vessels. upper respiratory tract involvement
Macrophages and dendrocytes are typically found in 15. Further consultations driven by patient condition (i.e.
large numbers in TEN. There is an elevated production of internal medicine to manage comorbidities, pulmo-
tumor necrosis factor-alpha in the epidermis, unlike EM nary medicine for airway involvement, gastroenterol-
(interferon-gamma is elevated in EM). ogy for alimentary involvement, and gynecology or
Inflammatory cells are hardly found in TEN. urology for genitourinary involvement)
Acute phases of TEN may show a temporary phase of 16. Physical therapy daily to preserve limb mobility
reduced number of CD4⫹ T-lymphocytes. Erythrocyte sedi- 17. Pain relief measures, such as patient-controlled anal-
mentation rate may be elevated. gesia (PCA) pump
18. Hydrotherapy (whirlpool) if needed
Management 19. Non-stick dressings to denuded areas, saturated with
0.5% silver nitrate impregnated every 3–8 hour as
The offending drug should be immediately identified and
needed. Pre-impregnated dressings with silver nitrate
discontinued. The following treatment guidelines followed
are an alternative
by University of Florida can be practiced effectively.
192
20. Avoid sulfa-containing topical or systemic preparations cleavage. The exotoxin produced by staphylococci is
21. Oral care with chlorhexidine rinses and white petrola- directed against epidermal desmoglein 1. Itzhak Brook
tum to lips (1980) reported a case of bullous impetigo caused by
22. Air-fluidized bed to minimize shearing force Streptococcus salivarius.
23. Keep room warm to prevent hypothermia Bullous impetigo usually occurs in crowded places and
24. Foley catheter and nasogastric tube placement only unhygienic surroundings. Presence of large families in clus-
when necessary ters will help the disease spread through direct contact.
25. Avoid unnecessary manipulation of skin. Adhesive tape When the condition disseminates to affect various parts
should not be applied directly to involved skin when of the body, it is referred to as staphylococcal scalded skin
possible syndrome. This is usually seen in immunocompromised
26. Baby shampoo for cleansing hairy areas daily patients. The toxin may disseminate hematogenously and
27. Mineral oil or petrolatum for dry skin lead to generalized staphylococcal scalded skin syndrome.
28. Skin substitute grafting (porcine xenografts or artifi- Bullous impetigo can affect any part of the skin and mucosa.
cial skin) based on BICU protocol. Literature review reveals few reports of buccal mucosa
involvement.
The initial lesion appears as small to large, superficial,
BULLOUS IMPETIGO fragile bullae. These bullae usually occur on the extremities,
axille and the trunk. Some patients report history of trauma
Impetigo is a bacterial infection that affects the superficial or infections. These bullae are generally painless. However
areas of the skin. It commonly affects infants and young some patients experience burning or itching sensation.
children in the age group of 2–5 years. However it has The bullae measure about 2 to 5 cm in size and usually do
known to affect individuals of various age groups. It is not rupture easily. They contain a serous clear yellow fluid
believed that impetigo is the most common skin disease to that subsequently turns cloudy and dark yellow. In about
affect children. 3–4 days time the bullae rupture and produce a thin, light
Donovan et al (1992) in their study to evaluate whether brown, varnish-like or honey like crust. A characteristic find-
bullous impetigo in homosexual men is a risk marker for ing in bullous impetigo is a ‘collarette’ of scale surround-
HIV-1 infection, concluded that bullous impetigo if seen ing the blister roof at the periphery of ruptured lesions.
in an adult, it could prove to be a clinical indication that
a person is either infected with HIV-1 or is in close (pos- Management and prognosis
sibly sexual) contact with a person with HIV-1 infection. Impetigo is a self-limiting condition and it usually resolves
It is a highly contagious infection caused primarily by spontaneously in about 2 weeks without scar formation.
Staphylococcus aureus. However some cases have been The topical antibiotics mupirocin and fusidic acid are
reportedly caused by group A beta-hemolytic streptococ- effective. Orally administered systemic antibiotics can be
cus (Streptococcus pyogenes) alone or in tandem with used in severe forms of the condition.
Staphylococcus aureus. The mode of transmission is gen-
erally by direct contact.
EPIDERMOLYSIS BULLOSA
Types
There are two forms of impetigo, namely, bullous impetigo Epidermolysis bullosa (EB) comprises a group of inherited
and non-bullous impetigo (impetigo contagiosa). The basic mucocutaneous disorders characterized by blister forma-
difference between the two is that the non-bullous variety tion due to a defect in collagen metabolism. These blisters
represents the host response to the staphylococcal infection, may arise spontaneously or as a result of mild trauma.
whereas in bullous impetigo the staphylococcal toxin is These inherited forms of epidermolysis bullosa should be
responsible for the condition and not the host response. differentiated from epidermolysis bullosa acquisita which is
an autoimmune disorder. Some authors prefer to use the term
Clinical features hereditary epidermolysis bullosa to refer to the forms that are
generally inherited. EB is a genodermatosis. The pattern of
Bullous impetigo is usually seen in infants. But it is seen
inheritance may either be dominant or recessive. The reces-
to affect children and adults likewise. Unlike the non-
sive forms of EB are relatively more severe and aggressive.
bullous form which may be caused by a combination of
staphylococcus and streptococcus. Bullous forms is always
Types of EB based on ultrastructural features
caused by the toxins produced by Staphylococcus aureus.
Staphylococcus aureus, phage group II type 71 is the pre- Jo-David Fine et al (2008) ‘The classification of inherited
dominant causative organism. This strain of bacteria pro- EB: Report of the Third International Consensus Meeting
duces an exfoliatin toxin that causes subcorneal epidermal on Diagnosis and Classification of EB’
193
Epidermolysis bullosa simplex—intraepidermal cleav-

RDEB, pretibial
age is seen (epidermolytic)
RDEB, pruriginosa
Junctional epidermolysis bullosa—intralamina lucida
RDEB, centripetalis
cleavage (lamina lucidolytic)
RDEB, bullous dermolysis of the newborn
Dystrophic epidermolysis bullosa—sublamina densa
cleavage (dermolytic)
Kindler syndrome—mixed General clinical features and oral manifestations
Epidermolysis bullosa simplex The condition is charac-
terized by the presence of the presence of multiple vesicles
Epidermolysis bullosa simplex or bullae at birth or some time after birth.
There are two basic types of EB simplex The common sites that are involved are the hand and
Suprabasal EB simplex feet. Oral mucosa is seldom affected.
Lethal acantholytic The condition is self-limiting. The vesicles heal in about
Plakophilin deficiency a week to 10 days time without the formation of scars.
EBS superficialis Most of the children are free of this condition at puberty.
Basal EB simplex
Localized (previously called EBS, Weber-Cockayne) Junctional epidermolysis bullosa The junctional variety
EBS, Dowling-Meara of epidermolysis bullosa is a severe form that occurs at
EBS, other generalized (the earlier term EBS, birth and usually death ensues in the first 3 months of life.
Koebner is no more used and presently included in In very severe forms the skin tends to shed increasing the
generalized EBS) risk for superinfection and septicemia.
EBS with mottled pigmentation Children may present with hoarse cry, cough and breath-
EBS with muscular dystrophy ing difficulty. Apart from the skin, oral, ocular, pharyngeal
EBS with pyloric atresia and genitor urinary mucosa may be affected.
EBS, autosomal recessive The oral lesions manifest as bullae and erosions affecting
EBS, Ogna almost any part of the oral cavity. Infants may have dif-
EBS, migratory circinate ficulty in feeding.
Dystrophic epidermolysis bullosa The dominant variety
Junctional epidermolysis bullosa usually occurs at birth and occasionally at adolescence. At
There are two basic types in JEB young age the condition has a generalized involvement.
JEB, Herlitz As the age advances the condition tends to be confined to
no subtypes a particular region.
JEB, other Bullae are seen on the feet, ankles, knees and elbows.
JEB, non-Herlitz, generalized (was previously gen- Nails are typically dystrophic. Other features that may be
eralized atrophic benign EB) seen are palmoplantar hyperkeratosis and hypertrichosis.
JEB, non-Herlitz, localized The bullae rupture and heal with extensive scarring.
JEB with pyloric atresia Milia (tiny keratin filled cysts) may be seen on the face.
JEB, inversa Compared to the recessive form of the condition, the oral
JEB, late onset (was previously known as EB involvement is limited. Occasionally bullae may be seen in
progressiva) the oral cavity. Teeth are seldom affected.
Laryngo-onycho-cutaneous (LOC) syndrome The recessive form of the condition is usually seen at
birth or in neonates. Bullae are seen in the sites predisposed
Dystrophic epidermolysis bullosa
to pressure or mild trauma such as the buttocks, knees,
There are two subtypes
elbows, feet and fingers. The bullae subsequently rupture to
Dominant dystrophic epidermolysis bullosa
reveal raw erosive areas. These heal by extensive scarring.
DDEB, generalized
Scarring of the fingers may result in ‘club like fists’.
DDEB, acral
Like the dystrophic variety, nail may be absent altogether
DDEB, pretibial
or may be defective. Oral manifestations are commonly
DDEB, pruriginosa
seen. Bullae may be seen affecting any part of the oral
DDEB, nails only
mucosa. With the rupture of the bullae, painful erosions are
DDEB, bullous dermolysis of the newborn
seen which heal by scarring. Extensive scarring of the oro-
Recessive dystrophic epidermolysis bullosa
pharyngeal and esophageal tissues may lead to hoarseness
RDEB, severe generalized
of voice and difficulty in feeding and swallowing. Dental
RDEB, generalized other
tissues are usually affected. Congenitally missing teeth,
RDEB, inversa
malformed or hypoplastic teeth are usually seen.
194
Linear IgA Disease lesions are typically bullae which rupture to form tender
erosions or ulcers.
Linear IgA disease has also been referred to as linear IgA Patients complain of burning sensation and discharge
bullous dermatosis (LABD) and linear immunoglobulin A from the eye when the ocular mucosa is affected. Common
dermatosis. It was considered to be a variant of DH or BP. ophthalmic findings are subconjunctival fibrosis, symbleph-
However, it is presently considered as a separate disease aron formation and cicatricial entropion with trichiasis.
entity.
LABD is a chronic subepidermal bullous disease associ- Drug-induced LABD
ated with linear deposition of IgA along the basement
membrane. The disease can be idiopathic or drug-induced. The clinical and histological picture is the same as the idio-
Other provoking factors for LABD are viral infections, pathic form of LABD. However, like the name suggests LABD
immunological disorders and malignancies. develops after drug administration. Literature review reveals
LABD affects both children and adults and the disease various drugs that have known to produce LABD, such as
process is similar in both. However, when the disease occurs vancomycin, diclofenac, lithium, captopril, cyclosporine
in children it has been referred to as chronic bullous der- glibenclamide, interferon-gamma, iodine contrast agent,
matosis of childhood. phenytoin sodium, somatostatin and sulfamethoxazole/
Patients suffering from Linear IgA disease produce IgA trimethoprim.
antibodies against the BP-180 glycoprotein (type VII col-
lagen) which is a component of the hemidesmosomes. Disease associations
Antibody deposition leads to complement activation and
LABD has been reportedly associated with infections, malig-
neutrophil chemotaxis, which results in loss of adhesion at
nancies and other autoimmune disorders. However many
the dermal-epidermal junction, thereby forming a bulla.
of the associations may simply be coincidental in nature.
Marinkovich et al (1996) reported that LAD-1 (a basement
Few of the commonly associated infections with LABD
membrane component) is one of the targets of autoanti-
are typhoid, tuberculosis, varicella, herpes zoster, and
bodies in patients with LABD.
upper respiratory tract infections.
It has been reported that almost 5% of the patients with
Clinical features
LABD may have an associated malignancy such as Hodgkin’s
Dermal lesions mimic lesions of DH or BP. Vesiculobullous disease and non-Hodgkin lymphoma, chronic lymphocytic
rash may be appreciated over a normal or erythematous leukemia, plasmacytoma, multiple myeloma and squamous
dermis. The bullae may contain serous sero-hemorrhagic cell carcinomas.
content. The common sites of involvement include the LABD has also known to occur along with other auto-
extensor surfaces of the hand and feet, lower trunk, peri- immune disorders such as Crohn’s disease, ulcerative coli-
oral region, genital region and buttocks. In adults, the tis, multiple sclerosis, systemic lupus erythematosus and
trunk and the limbs are usually affected. Lesions may not rheumatoid arthritis.
be symmetric. The bullae may arrange in a herpetiform
manner or clusters of discrete bullae, which has been pop- Management and prognosis
ularly termed as ‘jewels sign’. Another characteristic find-
The lesions of LABD generally follow a chronic course with
ing is the presence of vesicles or bullae bordering the
periods of acute exacerbations. Spontaneous remission
annular lesion which is termed ‘string of beads sign’.
may be seen in some patients. However almost all patients
Dermal lesions usually heal without scarring.
exhibit recurrence. Dermal lesions are managed with dap-
sone or sulfapyridine. However mucosal lesions are usu-
Mucosal manifestations
ally refractory to dapsone. Prednisolone along with dapsone
It is estimated that approximately 80% of patients suffer- will usually help in controlling the condition. Lewis et al
ing from LABD exhibit mucosa involvement. Oral, nasal, (2007) successfully treated a patient suffering from LABD
ocular and/or genital mucosa can be involved. Mucosal with oral mucosal involvement using mycophenolate, an
lesions heal with significant scarring. About 60% of these antiproliferative immunosuppressive agent. They reported
patients show involvement of the oral mucosa. that mycophenolate can be used as a useful adjunct to man-
Any part of the oral mucosa can be affected. Gingival age oral lesions of LABD, when other immunosuppressants
involvement can present as desquamative gingivitis. Oral are contraindicated.
195
CHAPTER
8 Oral Ulcerative Diseases

Keerthilatha M Pai, Braz Campos Durso
➧ Classification of Oral Ulcers ➧ Drug-induced Oral Ulcers

➧ Traumatic Ulcers Stomatitis Medicamentosa
➧ Ulcers Associated with Infections Stomatitis Venenata
➧ Erythema Multiforme
➧ Herpes Simplex Infection
➧ Blood Disorders Causing Oral Ulcers
➧ Varicella Zoster Infection
Chicken Pox RBC Disorders
Herpes Zoster WBC Disorders
➧ Immunologic Disorders
➧ Coxsackie Virus Infection
Herpangina Aphthous Ulcers
Hand, Foot and Mouth Disease ➧ Dermatological Disorders
➧ Acute Necrotizing Ulcerative Gingivitis Pemphigus
Bullous Pemphigoid
➧ Tuberculosis
Cicatricial Pemphigoid
➧ Syphilis
➧ Gastrointestinal Disorders Associated with
➧ Deep Fungal Infections Oral Ulcers
Aspergillosis Gastroesophageal Reflux Disease
Histoplasmosis Crohn’s Disease
Mucormycosis Ulcerative Colitis
Cryptococcosis Celiac Disease
North American Blastomycosis
➧ Neoplastic Ulcers
South American Blastomycosis
An ulcer may be described as breach in the continuity of ❍ Ulcers associated with blood dyscrasias
the surface epithelium of the skin or mucous membrane to ❍ Immune mediated
involve the underlying connective tissue as a result of micro ❍ Oral ulcers associated with dermatological disorders
molecular cell death of the surface epithelium or its trau- ❍ Oral ulcers associated with GI disorders
matic removal. ❍ Neoplastic oral ulcers
❍ Ulcers of uncertain etiology.
CLASSIFICATION OF ORAL ULCERS Classification based on mode of onset

and clinical presentation
Ulcerative lesions affecting the oral cavity can be catego- ❍ Single ulcers
rized based on the etiology or based on the mode of onset – Traumatic ulcer
and clinical presentation. – Tuberculous ulcer
– Syphilitic ulcer
Classification based on etiology
– Histoplasmosis
❍ Traumatic – Blastomycosis
❍ Infectious (bacterial, viral and fungal infections) – Mucormycosis
❍ Drug induced – Neoplastic ulcers
196
Chapter 8 – Oral Ulcerative Diseases
❍ Multiple ulcers
Flowchart 1
– Acute multiple ulcers
• Herpes virus infections Recurrent episodes
• Primary herpes simplex virus infections
• Coxsackievirus infections
• Varicella zoster virus infection
• Erythema multiforme Systemic symptoms Trauma
• Contact allergic stomatitis
• Oral ulcers secondary to cancer chemotherapy
and/or radiotherapy Absent Present
• Acute necrotizing ulcerative gingivitis IBD
– Chronic multiple ulcers
• Pemphigus Location
Cyclic neutropenia
• Subepithelial bullous dermatoses
• Herpes simplex virus infection in immunosup-
Sprue
pressed patients Keratinized Non-
❍ Recurring oral ulcers keratinized
Behcet’s
– Recurrent aphthous stomatitis
– Behçet’s syndrome
RAS HIV
– Recurrent herpes simplex virus infection Culture
– Cyclic neutropenia
– Chronic idiopathic neutropenia. + − FAPA
HSV Atypical RAS

Flowcharts 1 and 2 summarize acute ulcers that are recur- HIV Magic
ring in nature and those that occur as an isolated episode. Cyclic neutropenia
Algorithm to evaluate acute oral ulcers that are recurrent

TRAUMATIC ULCERS in nature. (Reprinted with minor modifications from
Bruce AJ (2003), ‘Acute oral ulcers’, Dermatologic Clinics,
Traumatic injuries involving the oral cavity may lead to Vol. 21, p. 2, with permission from Elsevier)
the formation of surface ulcerations. Although the exact
incidence of these ulcerations is not known they are one
of the most common ulcers seen affecting the oral cavity.
Nocturnal parafunctional habits, such as bruxism may be
associated with the development of traumatic ulcers of the
Types of Trauma buccal mucosa, the labial mucosa and the lateral borders of
❍ Mechanical (sharp tooth, overextended denture, ortho- the tongue. Ulcerations may be the result of voluntary, self-
dontic brackets, toothbrush, ill-fitting dental prosthe- induced, and deliberate acts by patients with physical or
sis, etc.) psychological symptoms who suffer from attention seeking
❍ Chemical (aspirin, concentrated clove oil, sodium hypo- behavior. These ulcers are characteristically present over
chlorite, hydrogen peroxide, root canal medicaments, visible surfaces such as the lips, corner of mouth and facial
chemotherapy, etc.) aspects of gingiva (ulcers caused by gingival picking).
❍ Thermal (extremely hot or cold insults such as hot Bulimic individuals may present with nail marks or minute
cheese, hot beverages, popsicle) (Figure 1) pinpoint red spots and/or ulcerations over the palate
❍ Radiation (used in treatment of head and neck cancer) which is brought about by the frequent efforts to vomit.
❍ Self-inflicted (self-harm) Newborns and infants may present with sublingual
❍ Iatrogenic (injuries caused by high speed rotary instru- ulcerations (Riga–Fede disease). These ulcers may occur as
ments, cotton rolls, etc.) a result of chronic mucosal trauma due to adjacent anterior
natal or neonatal teeth. The trauma is often associated with
The oral cavity is prone to injuries from events such breast feeding. The lingual frenum may be ulcerated by
as accidentally biting oneself while talking, sleeping, mas- repeated trauma because of the frenum rubbing against the
tication or as a result of an epileptic seizure. Fractured, mandibular incisors teeth in cunnilingus and in recurrent
malposed, or malformed teeth, as well as the premature coughing episodes.
eruption of teeth, can contribute to the formation of sur- Young children are commonly susceptible to electrical
face ulceration. and/or thermal burns of the lips and commissure areas.
197
Flowchart 2
Single episodes
Infection Drugs Iatrogenic Trauma
Radiation
Bacterial Viral
Chemotherapy
ANUG HSV
Syphilis VZV
Gonorrhea Coxsackie
Tuberculosis Rubeola
Rhinoscleroma EBV
Algorithm to evaluate acute oral ulcers that occur as an isolated episode. (Reprinted with minor modifications from
Bruce AJ (2003), ‘Acute oral ulcers’, Dermatologic Clinics, Vol. 21, p. 2, with permission from Elsevier)
associated with areas of erythema. The ulcer may reveal the

Figure 1
presence of a central yellowish purulent exudate. Occa-
sionally the border of the ulcer is indurated.
Ulcerations can occur throughout the oral cavity. Ulcers
associated with mechanical trauma are often found on the
buccal mucosa, the labial mucosa of the upper and lower
lips, and the lateral border of the tongue (Figure 2). The
mucobuccal folds, gingiva, and palatal mucosa may also
be involved. Most lesions associated with electrical burns
occur in children and involve the lips and commissural
areas. Ulcers formed due to thermal injuries are generally
seen to occur on the posterior regions of the buccal mucosa
and the palate.
Caustic chemical agents can damage any area of the
oral mucous membrane. However, they are commonly seen
A thermal burn on the hard palate secondary to intake of on the gingival margins and buccal vestibular regions of
a hot bread sandwich. Courtesy: Department of the oral cavity. Very frequently these ulcers are covered by
Oral Medicine and Radiology, MCODS, Mangalore a whitish pseudomembrane which when peeled leaves
behind a raw ulcerated surface (Figure 3). Some patients in
the Indian subcontinent use cloves and topical pain balms
Extensive ulcerations with necrosis may develop. Children meant for extraoral application over the gingiva to relieve
in this age group have a tendency to chew their lips tooth pain. Hence, most of the chemical burns are gener-
immediately after surgical removal of teeth under the ally seen adjacent to carious teeth.
influence of local anesthesia.
Diagnosis
Clinical features
The diagnosis of traumatic ulcerations is based on the his-
In most cases, the source of the injury is identified. The tory of trauma or insult (hot/cold or radiation therapy) prior
patient’s usual complaint is pain or a painful ulceration. to onset of ulcer. Mechanical trauma induced ulcers often
Individual lesions usually appear as shallow or deep ulcers have linear configuration. The depth of ulcer depends on
198
Figure 2 Histologic features

Histopathologically an area of surface ulceration covered
by a fibrinopurulent membrane consisting of acute inflam-
matory cells intermixed with fibrin is seen. The stratified
squamous epithelium from the adjacent surface may be
hyperplastic and exhibit areas of reactive squamous atypia.
The ulcer bed is composed of a proliferation of granulation
tissue with areas of edema and an infiltrate of acute and
chronic inflammatory cells.
Management
❍ Consumption of a soft and bland diet.
❍ Removal of traumatic factor (extraction of root stumps,
supraerupted teeth and malposed third molars, sharp
cuspal edges of teeth may be grounded, irritating den-
tures may be corrected, restoration of fractured teeth
and orthodontic correction of malposed teeth) will cause
A traumatic ulcer on the left lateral aspect of the tongue due the resolution of the ulcer in 10–14 days.
to sharp teeth. Courtesy: Department of Oral Medicine and ❍ Patient may be advised to rinse his/her mouth with
Radiology, KLEDC, Bangalore warm saline.
❍ Topical application of antiseptic and analgesic/anesthetic
medication (choline salicylate 8.7%, benzylkonium
0.01% and lignocaine hydrochloride 2%—patient can
Figure 3
be asked to apply the agent over the ulcers 10 minutes
prior to food intake, 3–4 times a day).
❍ In case of multiple ulcers—analgesic/antiseptic mouth-
wash (chlorhexidine gluconate 0.2% or benzydamine
hydrochloride 0.15% mouthrinse—one teaspoon of the
agent can be dissolved into 50 ml of water. This pre-
pared solution is swished in the mouth for 1 minute.
The patient is advised to rinse the mouth 3 times a day,
30 minutes after food intake).
❍ Application of topical corticosteroids (triamcinolone
acetonide oral paste 0.1%). Patient can be advised to
apply the paste 30 minutes prior to food intake, 3 times
a day for 2 weeks.
❍ For extremely large and deep seated ulcers, penicillin
may be administered (Capsule Amoxicillin, 500 mg,
3 times a day for 5 days) to prevent secondary
A chemical burn on the right buccal mucosa extending infections.
into the vestibule in patient who had the habit of applying ❍ Patients presenting with ulcers secondary to self-
pain balm in the vestibule to manage periodontal pain.
inflicted injuries may be referred to a psychiatrist or
psychologist after symptomatically managing the ulcer.
MCODS, Mangalore
Complications
nature of trauma. Generally the area surrounding the ulcer Most traumatic ulcers resolve with the removal of the
is inflamed. A traumatic factor will often be evident in the etiological agent. However, some ulcers may be second-
vicinity of the ulcer (e.g. sharp edge of tooth). arily infected and subsequently heal with extensive scar
formation.
Prognosis
Carcinomatous ulcer, recurrent aphthous ulcerations, ulcers
associated with deep fungal infections can be considered The outcome of traumatic ulcerations is excellent, provided
in the differential diagnosis of traumatic ulcers. the etiological factor is eliminated. Healing of the ulcerated
199
Table 1 Ulcers associated with infections
Viral Bacterial Fungal
Specific Non-specific
Herpes simplex virus Borrelia vincenti and Fusobaterium Infected traumatic ulcer • Histoplasmosis
• Primary herpetic gingivostomatitis • ANUG • Ruptured odontogenic abscess • Mucormycosis
• Recurrent herpes labialis • Cancrum oris/noma presenting as ulcer • Blastomycosis
• Recurrent intraoral herpes • Cryptococcosis
• Coccidiodomycosis
Varicella zoster virus Mycobacterium tuberculosis
• Chicken pox • Tuberculous ulcer
• Herpes zoster • Tuberculosis cutis orificialis
Coxsackie virus Treponema pallidum
• Herpangina • Chancre
• Hand, foot and mouth disease • Snail track ulcer
• Gumma
Epstein–Barr virus Gonococci
• Infectious mononucleosis • Gonococcal stomatitis
Human immunodeficiency virus
• AIDS
mucosa is usually delayed when the lesions overlie the ❍ Prodromal systemic symptoms (fever, malaise, myal-
maxillary or mandibular alveolar process, hard palate and gia) precede oral lesions by 2–3 days.
tip of the tongue. ❍ The skin, mucous membranes, eyes and central ner-
If the ulcer does not resolve within 2 weeks of removal vous system are the most commonly affected sites.
of the etiological agent, biopsy of the ulcer may be consid- ❍ Multiple oral ulcers affecting all parts of the mouth.
ered to rule out malignancy. ❍ Generalized erythema of gingiva usually associated
with multiple vesicles or ulcers (Figure 4).
Ulcers associated with infections ❍ Cervical lymphadenopathy occurs as a rule.
(bacterial, viral and fungal infections) ❍ Food intake becomes difficult and dehydration may
ensue.
Table 1 summarizes ulcers associated with infections.
❍ Self-limiting condition in normal children. However,
it may become disseminated in immunocompromised
children or adults.
PRIMARY HERPETIC GINGIVOSTOMATITIS
Diagnosis
Primary herpetic gingivostomatitis is caused by herpes sim-
Primary herpetic gingivostomatitis can be identified based
plex virus (double-stranded DNA virus which is a member
on history and clinical findings. Cytological (PAP/Tzanck)
of the human herpes virus family). Most orofacial and ocu-
smears of intact or recently broken vesicles may demonstrate
lar infections are caused by HSV-1. Infections involving the
epithelial giant cells containing intranuclear eosinophilic
genitalia and the skin surface of the lower part of the body
viral inclusions, that are typical of herpes viral infections.
are caused by HSV-2. It has been reported that HSV-2 has a
greater virulence. Almost 95% of the cases have a subclini-
cal infection, only about 5% manifest symptoms. The infec-
tion confers resistance against another primary infection Leukemia, erythema multiforme and stomatitis medica-
for lifetime. mentosa can cause lesions that resemble primary herpetic
gingivostomatitis.
Clinical features
Treatment
❍ Primary herpetic gingivostomatitis has an acute onset.
❍ Typically affects children (generally below 6 years of Symptomatic management Ensure that the patient is
age), but this infection also occurs in adults (immuno- adequately hydrated and electrolyte balance is maintained.
compromised). Antipyretic and analgesic medication like acetamino-
❍ Males and females are equally affected. phen, 500 mg given thrice daily is effective (3 times a day).
200
2. Herpes labialis (occurs along external vermillion border

Figure 4
of lips).
Precipitating Factors
❍ Exposure to UV light (sunlight)
❍ Menstruation
❍ Fever
❍ Stress
❍ Decreased immunity
❍ Traveling and change in climatic conditions.
Clinical presentation
❍ Patients present with cluster of tiny fluid filled vesi-
cles (Figure 5A–C) which rupture to form pinpoint
ulcers. These ulcers may coalesce to form larger areas
Gingival erythema, multiple small ulcers and vesicles in the
of ulceration.
attached gingiva in a 36-year-old man suffering from acute
❍ Lesions may be preceded by burning, itching, tingling
herpetic gingivostomatitis. Reproduced with permission from
editor, JCDA. Ajar AH and Chauvin PJ. Acute herpetic
sensation or pain in the region.
gingivostomatitis in adults: a review of 13 cases, including ❍ The lesions last for 5–7 days and subside and subse-
diagnosis and management. J Can Dent Assoc quently recur. Now the frequency of recurrence may
2002;68(4):247–51 be varied.
❍ Occasionally associated with fever and pharyngitis.
❍ As a consequence to healing an area of pigmentation
is noticed at the site of the lesion (however this pig-
Soothening oral rinses (anesthetic mouthwashes like mented area is readily visualized only in fair skinned
dyclonine hydrochloride 0.5%, about 10 ml can be used individuals).
4 times a day as an oral rinse).
Diagnosis
Specific management Antiviral therapy can be instituted
in children and adults. These medications will decrease the Recurrent herpes infection can be identified by their typi-
fever, pain, severity of the lesions and minimize viral cal location and clinical presentation. In addition, biopsy
shedding. and Tzanck smear can be performed.
In children antiviral medications like acyclovir can be
given within first 72 hours in a dosage of 200 mg 5 times Treatment
a day for 10 days. Acyclovir helps to decrease fever, pain, The management of recurrent lesions is generally symp-
lesions and viral shedding. tomatic. Patients can be advised to stay indoors to mini-
Newer drugs like valacyclovir (1 g orally times a day for mize exposure to sunlight. Sunscreen lotion can be used
7 days) and famciclovir (500 mg times a day for 7 days) are when venturing out. However in individuals where there is
more effective. an increased frequency of recurrence, acyclovir therapy
can be instituted as a prophylactic measure (400 mg twice
Prognosis a day for 10 days).
The outcome of primary herpetic gingivostomatitis is good
in otherwise healthy individuals. Complications may occur
in immunocompromised individuals. The virus remains dor- VARICELLA ZOSTER INFECTION
mant in nerve ganglion and may get reactivated later in
life causing secondary infection. Varicella zoster virus causes two distinct clinical entities.
The primary infection by varicella zoster causes chicken
pox, while the reactivated virus causes a secondary infec-
RECURRENT HERPES INFECTION tion termed herpes zoster or shingles.
Recurrent infections of herpes can manifest in two forms: Chicken Pox

1. Intraoral herpes (occurs on highly keratinized mucosa The primary infection by varicella zoster virus usually
of hard palate/gingiva) affects children. It is characterized by the sudden onset of
201
Figure 5 Figure 6
B
Vesicular lesions involving the face and neck in
chicken pox. Courtesy: Dr Ceena Denny
generalized pruritic vesicular rashes affecting the skin. The

incubation period of the virus varies from 10 to 21 days.
Approximately 50% of the affected children present with
prodromal symptoms of fever, malaise, headache, and
abdominal pain, which last for about 1–2 days before the
appearance of the dermal lesions.
Dermal lesions
C ❍ Typical lesions of chicken pox, clinically exhibit four
phases. The maculopapular phase (erythematous macules
are evident), vesicular phase (minute fluid filled vesicles
are seen), ulcerative phase (commonly seen on mucous
membranes of oropharynx, vagina and conjunctiva)
and the phase of healing (Figure 6).
❍ In the maculopapular phase erythematous macules are
seen. In some patients, vesicles develop within the
maculopapular rash giving rise to ‘dewdrop on a rose
petal’ appearance.
❍ The commonly involved sites are the scalp, face and
trunk.
❍ The lesions are often pruritic.
On an average 100–300 new lesions are found at any

given point of time. Lesions heal generally without scar-
ring. Occasionally crusting of the lesions may be seen.
(A) Vesicles on the upper lip in a patient with
However, an area of hypopigmentation may be appreci-
recurrent herpes labialis. (B) Ulcers formed
ated at the site of the healed lesion. These hypopigmented
subsequent to vesicle rupture in a patient with
recurrent herpes labialis. Courtesy: Department of areas fade away with time.
Oral Medicine and Radiology, MCODS, Mangalore.
(C) Healing lesions of herpes labialis. Courtesy: Oral lesions
Department of Oral Medicine and Radiology,
The common intraoral sites affected are the hard and soft
KLEDC, Bangalore
palate, labial and buccal mucosa. Oral lesions of chicken
202
pox are seen as small vesicles that subsequently rupture to

Figure 7
form shallow round ulcers surrounded by an erythematous
halo.
Management
Vaccination against varicella zoster should be given to
infants between 12th and 18th months of life. The manage-
ment of the disease is mainly symptomatic. Acyclovir can
be administered in the early phases of the disease.
Herpes Zoster
Herpes zoster is caused by reactivation of varicella zoster
virus that is inactive in dorsal root or cranial nerve gan-
glion, after primary infection.
It is estimated that only in about 0.3–0.5% of the
population, the virus is reactivated after the primary
infection.
Clinical features
❍ The clinical features depend on the dermatome
involved. Healed lesions of herpes zoster with scab formation.
❍ The nerves that are commonly affected are C-3, T-5, Characteristically, the lesions do not cross the midline and
this patient had involvement of the ophthalmic, maxillary
L-1 and L-2.
and mandibular divisions of the trigeminal nerve.
❍ Manifests in middle age.
❍ When the trigeminal nerve is involved, the ophthalmic
division of the nerve is most commonly involved. About
15–20% of the affected individuals show involvement
of the maxillary or mandibular division. and healing time of the infection and reducing the pain
❍ Painful vesicles and/or ulcers seen both inside and out- associated with the disease.
side the oral cavity, which stop abruptly at the midline Acyclovir (800 mg) is given 5 times a day for a week or
(i.e. lesions do not cross midline) (Figure 7). valacyclovir (1,000 mg) or famciclovir (500 mg) can be
❍ Bilateral lesions may indicate severely immunocom- given 3 times a day for a week.
promised state. Literature review reveals reports of
necrosis of alveolar bone exfoliation of teeth in immu-
nocompromised individuals. Herpangina
❍ Occasionally pain may be present along the course of
Herpangina is caused by coxsackie virus (A 1-10, 16, or 22).
the affected nerve in the absence of vesicles. Such a
It occurs generally in epidemics. It predominantly affects
manifestation is termed zoster sine herpete or zoster
posterior parts of oral cavity.
sine eruption.
❍ Ramsay Hunt syndrome is a symptom complex associ-
Clinical findings
ated with herpes zoster. It is characterized by varicella
zoster infection affecting the geniculate ganglion of ❍ In the early stages erythematous macules are seen.
the facial nerve, unilateral facial paralysis and unilat- As the disease progresses, vesicles with a central ulcer-
eral vesicular eruptions involving the oral mucosa and ation develop, which exhibit an erythematous halo.
external ear. ❍ Generally around 2–10 lesions are seen which measure
less than 5 mm in diameter.
Post-herpetic neuralgia is one of the relatively common
❍ The common sites involved are the posterior pharyn-
sequelae of varicella zoster infection that results from
geal region, uvula, anterior faucial pillars, tonsils and
scarring of the involved nerve.
soft uvula. On occasions ulcers may be present on the
tongue and buccal mucosa corresponding to the pos-
Management
terior regions of the oral cavity.
Antiviral drugs like acyclovir, valacyclovir and famciclo- ❍ The lesions are usually associated with mild fever which
vir have been used successfully in shortening the course subsides within a week.
203
❍ It is a self-limiting condition and generally subsides ❍ Approximately five to ten ulcers are seen.
within a week. ❍ Tongue may be involved in about 45% of the patients.
❍ Marginal gingiva is characteristically unaffected. Tongue may become swollen and tender.
❍ The other sites of involvement are the palate, buccal
mucosa and gingiva.
Hand, Foot and Mouth Disease
Management
Hand, foot and mouth disease is a viral disease caused by
coxsackie viruses (members of the Picornaviridae family). ❍ Topical application of anesthetics like viscous lido-
It is characterized by oral lesions associated with lesions caine and diphenhydramine may be used to manage
involving the extremities. painful oral ulcers. Sucralfate suspension can also be
Infections can occur in a sporadic form (coxsackie virus effectively used.
types A4–A7, A9, A10, B1–B3, and B5) or as epidemics ❍ Fever and arthralgias can be treated using antipyretics
(coxsackie virus A16 or enterovirus 71). The incubation and analgesics.
period ranges from 3 to 6 days. Though the disease runs a ❍ Literature review reveals that there is a reported quicker
mild course and is self-limiting, a severe form of the condi- resolution of symptoms with the use of oral acyclovir.
tion is seen in infants and children. Enteroviral infections ❍ A low intensity laser ablation of the oral ulcers is said
may cause myocarditis, pneumonia, meningoencephalitis to shorten the duration of painful oral ulcers.
and even death. Infection occurring during the first trimes-
ter in pregnant women can result in spontaneous abortion Prognosis
and retarded development of the fetus. The prognosis of the condition is generally excellent
owing to the self limiting nature of the disease. However,
Clinical features complications like meningoencephalitis, myocarditis, pul-
Hand, foot and mouth disease typically affects young chil- monary edema, and death have been reported.
dren below 10 years of age. Males and females are equally
affected. Prodromal symptoms like low-grade fever,
anorexia, malaise, abdominal pain, sore mouth and cough ACUTE NECROTIZING ULCERATIVE
are evident which lasts for 12–36 hours. The condition is GINGIVITIS
self-limiting. Mucosal and cutaneous lesions resolve spon- (Trench Mouth, Vincent’s Disease,
taneously in 5–7 days. Vincent’s Gingivostomatitis)
Literature review reveals that almost 22% of the patients
may present with cervical or submandibular lymphade- Acute necrotizing ulcerative gingivitis (ANUG) is an acute
nopathy. bacterial infection affecting the gingiva. As per the 1999
American Academy of Periodontics classification system,
Skin lesions ANUG is presently classified as necrotizing periodontal
❍ Approximately 65% of the patients demonstrate skin disease that is characterized by rapidly progressive ulcer-
involvement. ation typically starting at the crest of the interdental
❍ The commonly affected sites are the hands (dorsal papilla, spreading along the marginal gingival and subse-
aspect of the hands and sides of the fingers are very quently causing rapid and extensive destruction of the
commonly affected), feet and the buttocks. periodontal tissue.
❍ The characteristic skin lesion is seen as asymptomatic, The anaerobic organisms that are said to cause ANUG
erythematous macule characterized by a central grayish are Fusobacterium necrophorum, Prevotella intermedia,
vesicle. The macule varies in size from 2 to 10 mm. Fusobacterium nucleatum, Porphyromonas gingivalis and
❍ These macules are elliptical with their long axis aligned Treponema species.
parallel to the skin lines.
❍ The lesions subside in about a week’s time. Predisposing factors
Suppressed immunity, malnutrition, stress, poor oral hygiene,
Oral lesions smoking and local trauma predisposes to the development
of ANUG. Literature review reveals that the prevalence of
❍ In the early stages, oral lesions begin as erythematous
ANUG among HIV patients ranged from 4 to 16%.
macules that evolve into 2–3 mm vesicles on an
erythematous base. However, intact vesicles are rarely
Clinical findings
seen as they ulcerate instantaneously.
❍ The ulcers are painful and interfere with chewing and ❍ ANUG is generally seen in young and middle-aged
swallowing. adults. However, in the Indian subcontinent and
204
Figure 8 Table 2 Comparison of acute necrotizing ulcerative gingivitis

and acute herpetic gingivostomatitis
Acute necrotizing ulcerative Acute herpetic
gingivitis gingivostomatitis
Complex bacterial etiology Viral etiology
Ulcers confined to gingiva Ulcers all over mouth
Indefinite clinical course and duration Self-limiting lasts for 7–10 days
Non-contagious Contagious in those with
depressed immunity
No viral antibodies Convalescent sera shows viral
antibodies
Treatment with antibacterial agent No role for antibacterial agents
Necrosis of the interdental papillae in acute necrotizing

ulcerative gingivitis. Reproduced with permission from editor,
JCDA. Mirbod SM, Ahing SI. Tobacco-associated lesions TUBERCULOSIS
of the oral cavity: part I. Non-malignant lesions.
J Can Dent Assoc 2000;66:252–6 Tuberculosis is a chronic infectious granulomatous bacte-
rial disease generally acquired by inhaling droplets con-
taminated by Mycobacterium tuberculosis. However, it can
Africa, it occurs almost exclusively in children of age also be acquired by consuming unpasteurized cow’s milk
group between 3 and 10 years. These children character- that is infected by Mycobacterium bovis or by other atypical
istically belonged to the low socioeconomic groups. mycobacteria.
Hospital-based studies in Nigeria showed a prevalence
rate of about 23% in children less than 10 years of age. Oral manifestations
❍ Gingiva tends to bleed spontaneously or with the
It is hypothesized that tubercle bacilli enter the oral
slightest of provocation.
mucosa through a break in the mucosal surface. Abbot
❍ Marginal and interdental gingiva is erythematous and
et al in an independent study were able to isolate tubercle
edematous.
bacilli from the mouthwashings of 44.9% of the individu-
❍ Crest of the interdental papillae is blunted.
als suffering from pulmonary tuberculosis. This study
❍ Minute punched-out crater like ulcers are seen in the
underlines the importance of intact mucosal lining in pro-
interdental papillae. These ulcers are covered by a gray-
viding protection against tuberculosis affecting the oral
ish white pseudomembrane. The ulcers are extremely
cavity.
painful (Figure 8).
The oral manifestations of tuberculosis are rare. It is
❍ Foul odor.
estimated that approximately 1.4% of the patients suffering
❍ Patients may present with fever, malaise and regional
from tuberculosis exhibit oral manifestations. The tuber-
lymphadenopathy.
culous lesions may present as ulcer, fissure, tuberculoma
Table 2 compares features of acute necrotizing ulcerative or as a generalized enlargement of the tongue. In the initial
gingivitis and acute herpetic gingivostomatitis. stages the lesion can be in the form of a nodule or opales-
cent vesicle which progresses to form an ulcer. The most
Management of ANUG common oral manifestation is ulcer (Figure 9). Oral tuber-
culous lesions typically present with severe, unremitting
❍ Manage underlying factors.
and progressive pain.
❍ Well balanced diet and regular meals.
❍ Gently remove necrotic material with swab.
Sites
❍ 3% hydrogen peroxide diluted with equal amount of
water used to rinse mouth every 2 hours on first day; Soft tissues are more frequently affected than hard tissues.
3–4 times/day for next 4 days. The sites of involvement include the tongue, soft palate,
❍ Penicillin V (oral): 400,000 units/day for 5 days/amox- hard palate, gingiva, lips, floor of the mouth, vestibular
icillin 250–500 mg t.i.d. for 5–7 days. and retromolar regions and recent tooth extraction sock-
❍ Metronidazole 400 mg t.i.d. for 5 days. ets. Maxilla and the mandible have also been affected and
❍ Oral prophylaxis can be undertaken after acute symp- referred to as tuberculous ostemyelitis. Mandible is more
toms subside. commonly involved. Tuberculous osteomyelitis generally
205
Figure 9 Figure 10
A solitary crateriform ulcer with slightly elevated

and indurated borders on the lateral margin of the Caseation necrosis of a tubercular lymph node.
tongue in a patient with tuberculosis. Courtesy: Courtesy: Department of Oral Pathology, MCODS,
Dr Velia Ramírez Amador, Universidad Autónoma Mangalore
Metropolitana-Xochimilco, Mexico
Oral manifestations
occurs due to a hematogeneous spread of the bacilli or All stages of acquired syphilis can present as oral ulcers
through a direct extension through a fresh extraction (chancre, mucous patch and gumma). Usually chancre
socket. and mucous patch are self-limiting and pass on to the
next stage.
Typical lesion Chancre The occurrence of primary syphilis in the oral
The ulcer is irregular with ragged, undermined edges, min- cavity is extremely rare. Almost all chancres are seen in
imal induration and yellowish granular base. The ulcer is the genital region. However with the change in the sexual
surrounded by sentinel tubercles. Primary oral tuberculous practices the oral cavity can also be a site for the presence
lesions are usually associated with caseation of the regional of chancre.
draining group of lymph nodes (Figure 10). The lips, tongue, tonsillar regions and the palate are the
common sites that are involved. In the initial stages a pap-
Differential diagnosis ule may be seen which subsequently erodes. The typical
syphilitic ulcer is punched out, non-tender, indurated and
Infected traumatic ulcer, major aphthous ulcer, syphilitic associated with a yellowish serous discharge. The associ-
ulcer, sarcoidosis, lymphogranuloma venereum, foreign ated nodes are generally firm and non-tender on palpation.
body granuloma, histoplasmosis and malignant ulcer can Chancres are self-limiting and last for a period of 2 weeks.
be considered as differential diagnosis for tuberculous They heal with minimal scar formation.
ulcer.
Mucous patches Generally secondary syphilis appears
Management after a latency period of 6 months after the primary lesions
are noticed. Patients complain of fever, headache, body-
The pain associated with the ulcer can be treated symp- ache and sore throat. Cutaneous maculopapular rashes
tomatically. Antitubercular therapy for 18 to 24 months associated with lymphadenopathy are seen.
should be instituted. Oral lesions are characterized by the appearance of oval
red macules (usually seen on the palate) or papules (usually
seen on the buccal mucosa and commissures) and mucous
SYPHILIS patches. The oral commissures demonstrate split papules.
Mucous patches are seen as raised erosive areas covered by
Syphilis is a sexually transmitted disease that is caused by a grayish white pseudomembrane and surrounded by an
Treponema pallidum. erythematous halo. They usually measure about 1 cm in
206
diameter and seen bilaterally on the movable surfaces in the Oral findings
oral cavity. These small lesions can join together to give
Involvement of the maxillary sinus is rarely seen in
rise to snail track ulcers (serpiginous pattern).
aspergillosis. An extension of the infection from the
A severe and generalized form of secondary syphilis can
sinus can result in involvement of the adjacent soft tis-
result in lues maligna, which is also termed ulceronodular
sue and palate producing blackish or yellowish necrotic
disease. The oral mucosa reveals shallow crater like ulcers.
ulcerations.
The common sites involved are the palate, buccal mucosa,
tongue, lower lip and gingiva.
Gumma Gumma is a highly destructive lesion that is Histoplasmosis
typically seen in tertiary stage of acquired syphilis. The Histoplasmosis is caused by Histoplasma capsulatum. Fol-
gummatous lesion may sometimes occur 8–10 years after lowing inhalation of this dimorphic fungus it can cause an
the initial infection. The hard palate and tongue are com- acute pulmonary histoplasmosis or can assume a dissemi-
mon sites of involvement. However, literature review nated form in immunocompromised individuals.
reveals sites like the parotid salivary gland, soft palate and The typical oral lesion, though very rare appears as an
the lower alveolar ridge being affected. erosion or ulcer commonly involving the tongue, palate
Gumma of the hard palate will result in perforation, and buccal mucosa.
thereby producing an oronasal communication.
Diagnosis and management Mucormycosis

Sensitive tests like VDRL and specific tests like FTA-ABS It is an opportunistic infection caused by a saprophytic
test/TPI assay/ELISA can be used to diagnose syphilis. fungus that is found in the soil, bread mold, animal manure
According to the 1993 Centers for Disease Control and and rotting vegetation. Mucoraceae are fungi that are com-
Prevention (CDC) treatment guidelines for sexually trans- monly found in soil or in rotting vegetation. Rhizopus can
mitted diseases, parenteral penicillin G is the drug of choice be found in moldy bread. Mucormycosis can assume vari-
for managing syphilis. Patients allergic to penicillin can be ous clinical forms based on the site of involvement such as
treated with doxycycline, tetracycline or erythromycin. rhinocerebral form (spores deposited in nasal turbinates),
pulmonary form (spores lodged in the lungs by inhala-
tion), cutaneous form (spores introduced through wounds
DEEP FUNGAL INFECTIONS and cuts on the skin surface), gastrointestinal form (spores
ingested) and a disseminated form.
The rhinocerebral form of mucormycosis characteristi-
Deep fungal infections are relatively uncommon in the
cally involves the paranasal sinuses. An extension of the
oral cavity. These deep seated infections generally have
disease process can result in ulceration of the palate. The
systemic manifestations and rarely involve the oral cavity.
typical lesion is seen as a large, painful ulcer of the palate
The deep fungal infections that produce ulcerative lesions
filled with blackish necrotic slough. Ulcers have also been
in the oral cavity include mucormycosis, blastomycosis,
reported to occur on the gingiva, lip and alveolar ridge.
histoplasmosis, aspergillosis, cryptococcosis and paracoc-
cidiodomycosis. Characteristically, these infections are seen
in immunocompromised individuals suffering from HIV, Cryptococcosis
AIDS or systemically debilitating conditions such as dia-
betes mellitus, malignancies and individuals on long-term Cryptococcosis is an opportunistic fungal infection caused
immunosuppressive therapy. by Cryptococcus neoformans. It is usually present in bird
droppings, rotting wood and soil.
C. neoformans preferentially affects the meninges, basal
Aspergillosis ganglia and cortical gray matter. Oral mucosal lesions appear
as superficial ulcers or as deep ulcers with indurated bor-
Aspergillosis is reportedly the second most commonly seen
ders and rolled out edges.
fungal infection affecting the oral cavity after Candida.
Aspergillosis in humans is caused primarily by Aspergillus
fumigatus and A. niger. The spore is found in rotting veg- North American Blastomycosis or
etation. The transmission of fungal spores to the human
Gilchrist’s Disease
being is by inhalation.
It primarily affects the lungs. Aspergillosis has a ten- Gilchrist’s disease is caused by Blastomyces dermatidis. It
dency for a hematogeneous invasion causing thrombosis is caused by inhalation of the spore which is predominantly
and infarction of perivascular tissues. found in the soil. Like most of the deep fungal infections,
207
blastomycosis has a localized form that affects the respira-

paracoccidioidomycosis is frequently diagnosed because
tory system and a disseminated form. However, unlike other
of mouth lesions. In this sense, the dentist plays a fun-
fungal infections immunosuppression does not increase the
damental role in the diagnosis of this pathology.
risk of disseminated disease.
Oral lesions are usually extensive, can be found on
Blastomycosis is endemic to the Mississippi and Ohio
up to 78.9% of the cases investigated, and frequently
River basins in the United States and certain areas in Africa
affect more than one area of the oral mucosa. The
and India.
mucous and mucocutaneous lesions of paracoccidioi-
Disseminated form of blastomycosis commonly involves
domycosis have a significant clinical diagnostic value,
the skin, CNS and the genitourinary tract. Papulopustular
since they provide direct observation of the lesion and
or verrucous, grayish lesions are seen on the skin surface.
easy access to the anatomopathological and mycologi-
Oral mucosa may exhibit ulcerations.
cal examination, in addition to their high prevalence.
The main clinical feature in the oral lesions is a granu-
Paracoccidiodomycosis or South American lomatous mulberry-like surface and ulceration.
Blastomycosis Because of the chronic character and progressive
extension of the ulcerated oral lesions, they may resem-
Paracoccidiodomycosis is caused by Paracoccidioides brasil- ble other infections and neoplastic diseases. In the clin-
iensis. This disease is commonly seen in Latin American and ical routine, the patients are mistakenly diagnosed as
South American regions (Brazil, Colombia, Argentina and having mainly squamous cell carcinoma, tuberculosis,
Venezuela). sarcoidosis, leishmaniasis, Wegener’s granulomatosis,
histoplasmosis, actinomycosis, lupus erythematosus,
Clinical features lymphoma, or syphilis.
The predilection for male patients, in what seems to
It usually affects men older than 30 years of age. Farmers
be a result of a protective effect of female hormones such
are at a greater risk of acquiring this disease. After inhala-
as ␤-estradiol, which inhibits transformation of the
tion, the fungus usually remains dormant and the indi-
hyphal form of the organism to the pathogenic yeast
vidual is asymptomatic. In an immunocompromised host,
form as well as is a consequence of the professional
the fungus exhibits the clinically active state.
outdoor activities of males. It is postulated that after
Lung is the first site of involvement. It leads to produc-
menopause, women are more susceptible to the disease.
tive cough, chest pain and shortness of breath. Other symp-
However, possible genetic factors associated to the HLA
toms include joint pains and body aches. Cervical and
antigen may facilitate the appearance of mycosis.
axillary lymphadenopathy is seen.
The fungus Paracoccidioides brasiliensis is easily
Painful ulcers involve the mucosa of the oral cavity;
recognized under hematoxylin and eosin (H&E) stain-
gastrointestinal (GI) tract and upper respiratory tract are
ing, inside or outside of giant cells, as birefringent,
common. Oral ulcers are typically seen on the lips, gin-
double-contoured cyst-like structures with a diameter
giva, and tongue.
ranging from 1 to 30 ␮m. Typically, H&E stained slides
reveal epithelial pseudoepitheliomatous hyperplasia,
granulomatous response and microabscesses. The under-
Paracoccidioidomycosis
lying connective tissue displays a chronic granuloma-
The paracoccidioidomycosis is one of the most prevalent tous reaction, with epithelioid cells and giant cells. The
deep systemic mycoses. It is more frequently observed number and organization of granulomas are variable.
in patients from South America or Central America and Disorganized granulomas tend to predominate in the
is an important expression of the spectrum of pathol- oral lesions. The periodic acid-Schiff (PAS) and Grocott-
ogy in Latin America. Brazil accounts for approxi- Gomori methenamine silver are particularly useful to
mately 70% of the cases reported in the literature. highlight the yeast-like structures with multiple bud-
Immigrants from these regions may carry it elsewhere ding that resemble a pilot’s wheel or Mickey Mouse ear.
or people visiting these endemic areas may acquire the Eosinophilic inflammatory infiltrate is a common find-
infection. ing, however it is not directly related to the amount of
Knowing paracoccidioidomycosis is important to fungus or granulomas. Immunocytochemical techniques
the dentist, since the oral mucosa provides an impor- may provide new information on the microscopic aspects
tant substrate to the saprophytic life of the fungus, and of this disease and should be encouraged.
the disease has frequent oral and dermatological mani- The treatment scheme will depend on the severity
festations, usually the first to be detected. In spite of of the disease, and long-term follow-up is mandatory.
that, few cases have been reported in the English lan- Even with supposedly adequate treatment, death is not
guage literature. Although a primarily lung infection, uncommon in both acute and chronic cases.
208
DRUG-INDUCED ORAL ULCERS ulcers are resistant to all treatment modalities and persist
for months.
The oral mucosa is susceptible to insult from both sys-
temically administered drugs and locally applied drugs in Management
the oral cavity. The suspected medication should be replaced by an alter-
The term ‘stomatitis medicamentosa’ is reserved for the native drug wherever possible. Patients can be advised to
effects of systemically administered drugs and ‘stomatitis apply topical anesthetic agents over the ulcer. Tetracycline
venenata’ is the term used for the effects induced by topical mouthwashes and topical steroids are effective in manag-
application of drugs or due to contact (contact stomatitis). ing drug-induced oral ulcers.
Pathogenic basis of drug-induced stomatitis/

oral ulceration Stomatitis Venenata (Contact Stomatitis)
Drug-induced stomatitis or ulcerations are said to be caused Contact stomatitis is relatively rare when compared to
either by immune mediated or non-immune mediated contact dermatitis. The inherent qualities of the oral
pathways. These changes in the oral cavity depend on the mucosa and the oral environment make it more resistant
potential toxicity of the drug, frequency of intake, route to potentially allergic agents than the keratinized skin.
of drug administration and the inherent immunity of the Saliva dilutes and digests or washes away allergic agents.
individual receiving the medication. It also contains a high concentration of epidermal growth
factor which aids in healing of mucosal injuries if any. The
Immune-mediated pathway The medicament or one of oral epithelium, which is non-keratinized in nature, has
its components is thought to trigger an immune response. fewer proteins; as a result there are fewer targets for the
This immune response is targeted at the surface epithelium, allergens. Allergens in contact with the oral mucosa are
thereby forming ulcers. removed very rapidly because of the higher epithelial turn-
Non-immune mediated pathway In this form of reac- over rates of the oral epithelium.
tion there is no evidence of an immune response and it is However, in spite of the inherent protective mechanism
not dependent on antibodies. The medication administered of the oral mucosa, topical application of medicaments can
will directly stimulate mast cells and lymphocytes to release elicit a localized mucosal reaction in some individuals.
cytotoxic chemical mediators that cause stomatitis and oral
ulcerations. Types of contact stomatitis
Allergic contact stomatitis and irritant contact stomatitis
are subtypes of contact stomatitis (Table 3).
Stomatitis Medicamentosa
The agents causing stomatitis are usually dentifrices,
Adverse effects of drugs are generally manifested over the mouthwashes, dental materials, cosmetic agents, food col-
skin surface. However, literature review reveals that the oring and flavoring agents and preservative agents. The
oral cavity can sometimes be the only site of involvement. agents causing contact stomatitis are summarized in the
It is a known fact that no medication is safe and that every Box 1.
drug has the potential to cause damage to the body includ-
ing the oral cavity. These drug reactions affecting the oral Management
mucosa can be in the form of stomatitis, lichenoid reac-
The first step in managing contact stomatitis is by avoiding
tions, erosions and ulcers.
contact with the identified or suspected irritant or allergen.
Medications causing oral ulcers

Table 3 Contact stomatitis: types
Non-steroidal anti-inflammatory drugs (indomethacin,
piroxicam and ibuprofen), anti-depressant (sertraline), COX-2 Irritant contact stomatitis Allergic contact stomatitis
inhibitors (celecoxib, rofecoxib), anticancer chemothera-
No previous history regarding Previous history regarding exposure
peutic agents such (cyclosporine, methotrexate, doxorubi-
exposure to suspected irritant to allergen is required
cin), anti-hypertensives (calcium channel blockers) and
miscellaneous agents like gold salts can cause stomatitis Clinical signs are seen within Clinical manifestations usually seen
minutes to hours of exposure after 48 hours of subsequent exposure
and/or oral ulcerations. Antidepressants cause xerostomia,
to irritant (a type IV hypersensitivity reaction)
which in turn results in stomatitis or ulceration.
Drug-induced oral ulcers are generally solitary and typi- Activation of immunologic Activation of memory T-cells
mediators without involvement
cally seen over the lateral margins of the tongue. These
of memory T-cell function
ulcers are often surrounded by a white halo. Most of the
209
Box 1 Common agents causing contact stomatitis Table 4 Blood disorders causing oral ulcers
Oral cleansing aids RBC disorders WBC disorders

• Dentifrices (contain peppermint, cinnamon) Anemia Quantitative Qualitative
• Mouthrinses (chlorhexidine) disorders disorders
• Dental floss (contains colophony or rosin)
• Iron deficiency • Leukemia Lazy leukocyte
Dental materials • Pernicious • Agranulocytosis syndrome
• Free monomer (in acrylic appliances) • Sickle cell anemia • Cyclic neutropenia
• Nickel (content of orthodontic wires) • Thalassemia • Multiple myeloma
• Mercury (dental amalgam fillings)
• Gold (constituent of crowns, fillings)
• Denture adhesives (contains rosin which is resin obtained from
WBC Disorders
conifers)
• Eugenol (in periodontal packs, cements) WBC disorders can cause oral ulcerations and necrotizing
• Phenol lesions which are commonly seen on the gingiva, floor of
Cosmetics (contain rosin derivatives, propolis and ricinoleic acid) the mouth, buccal mucosa and pharynx. These ulcers are
• Lipsticks characterized by the lack of the inflammatory halo and
• Lip balms generally associated with necrosis and foul smell. Cyclic
• Sunscreens neutropenia is associated with recurring oral ulcers. The
Latex treatment is directed at the cause of the ulceration.
Food substances (containing preservatives, coloring and flavoring
agents, sea food, fruits such as apples, pears, etc.)
IMMUNOLOGIC DISORDERS
Patients are instructed to avoid smoking. Toothpaste and
Aphthous Ulcers (Recurrent Aphthous Stomatitis,
mouthwashes with strong flavoring agents are best avoided
(baking soda can be an effective alternative to tooth pastes). Aphthae, Canker Sores)
Topical triamcinolone acetonide or flucinonide 0.05% gel Recurrent aphthous stomatitis (RAS) is a common con-
can be used. dition characterized by recurring ulcers confined to oral
mucosa in patients with no other signs of disease. Patient
presents with multiple round or ovoid ulcers generally
ERYTHEMA MULTIFORME with well-defined borders and erythematous halo surround-
ing the periphery of the ulcer. Literature review reveals
Described in Chapter 7 on Vesiculobullous Disorders. that approximately 25% of the population is affected with
this condition.
BLOOD DISORDERS CAUSING ORAL ULCERS Etiopathogenesis

Although many theories were proposed to explain the etiol-
Blood disorders are associated with increased risk of oral ogy of RAS, there appears to be no single causative factor.
ulcers. Necrosis is generally a prominent feature of these Various etiological factors have been proposed, such as
ulcers. Other clinical signs and symptoms and a hemo- hereditary, trauma, deficiency states, psychological factors,
gram will help the diagnosis. endocrine disorders, allergic conditions, infections, blood
The red blood cell (RBC) and white blood cell (WBC) dyscrasias, drugs, GI diseases, urological disorders, derma-
disorders that cause oral ulcers are summarized in Table 4. tological disorders and immunologic origin, etc.
Lehner (1964) and Dolly (1969) were of the opinion that
RAS belonged to the group of autoimmune diseases. Lehner
RBC Disorders
in 1969, found elevated levels of IgG and IgA in the sera
Iron deficiency anemia is characterized by the presence of patients with RAS. A fluorescent antibody technique
of glossitis and glossodynia, angular cheilitis and atrophic showed both IgG and IgM were binding by epithelial cells
areas on the tongue due to papillary atrophy. Patients with of the spinous layer of oral mucosa in RAS patients.
pernicious anemia exhibit glossitis, burning tongue, angu- Greenspan et al (1981) implicated antibody dependent
lar cheilitis, papillary atrophy, and recurrent oral ulcer- cellular cytotoxicity as a pathogenic mechanism in RAS.
ations. Sickle cell anemia and thalassemia exhibit oral Thomas and coworkers (1990) showed that T lymphocytes
ulcerations. These patients are relatively more likely to from RAS patients had increased cytotoxicity to oral epi-
develop osteomyelitis. thelial cells.
210
Clinical features ❍ Ulcers are usually seen on the non-keratinized oral

mucosa (commonly on the buccal and labial mucosa).
❍ Individuals may experience burning sensation which
Ulcers are rarely seen in the heavily keratinized palate
may appear 2–48 hours before the ulcer appears.
or gingiva.
During the initial periods, a localized area of erythema
develops within hours, a small white papule forms,
Types of recurrent aphthous stomatitis
ulcerates and gradually enlarges over the next
48–72 hours. Three forms of RAS are clinically recognized, namely,
❍ The ulcers are usually regular and well defined. They minor, major, and herpetiform.
are rimmed by an erythematous halo (Figure 11) and
Minor aphthous ulcers (Mikulicz ulcer) The minor RAS are
the ulcer is covered with a yellowish-gray fibrinous
tiny round to ovoid ulcers that mainly involve the non-
pseudomembrane.
keratinized oral mucosa (such as the buccal mucosa, labial
❍ The number and size of the ulcers vary based on the
mucosa, floor of mouth, ventral surface of the tongue). In
type of RAS.
most individuals one to six ulcers measuring about 2–4 mm
in diameter are present at any given point of time. The ulcers
are surrounded by an erythematous halo (Figure 12A, B).
Figure 11 These ulcers heal in about a week’s time without scarring.
Major aphthous ulcers (Sutton’s ulcers; periadenitis
mucosa necrotica recurrents) Like the name suggests
these ulcers are larger in size (almost up to 1 cm in size)
when compared to minor RAS. Major RAS involve even
the keratinized areas of the oral mucosa (such as the palate
and dorsum of tongue). These ulcers usually occur in very
few numbers ranging from one to six at a time. They per-
sist for a longer duration and heal in about 2–6 weeks
with scarring.
Herpetiform ulcers These are present as crops of tiny
pin head sized ulcers which coalesce together. These may
be present both on the keratinized as well as the non-
keratinized mucosa. These ulcers typically begin as tiny
Aphthous ulcer on the upper labial mucosa. The ulcer is vesicles that subsequently ulcerate. These are extremely
surrounded by an erythematous halo. Courtesy: Department painful and tend to heal in about 10 days and almost
of Oral Medicine and Radiology, MCODS, Mangalore
immediately recur.
Figure 12
A B
(A) A minor aphthous ulcer on the lower labial mucosa. (B) A minor aphthous ulcer on the lateral margin of the tongue.
The ulcer is surrounded by the characteristic erythematous halo. Courtesy: Department of Oral Medicine and
211
Table 5 Comparison of recurrent aphthous ulcer and recur-

foliaceous and erythematoses are the four histopathological
rent intraoral herpes
variants of the disease. Pemphigus vulgaris is the most
severe form.
Recurrent aphthous stomatitis Recurrent intraoral herpes
(RAS) (RIHS) Clinical features
Non-specific etiology Viral etiology
It is commonly seen in Jews and predominantly affects
Wide range of age group Middle age adults with no sex predilection. Bullae of varying sizes are
Freely movable mucosa involved Attached mucosa seen on skin and mucosal surfaces. The bullae on the skin
Single or 2–3 ulcers at a time Multiple pinpoint ulcers are tense; however in the oral cavity they rupture almost
except in herpetiform where crops immediately to form ulcers or erosions (Figure 13A, B). These
of pinpoint ulcers occur ulcers typically have peripheral tissue tags. Nikolsky’s sign
Non-specific findings on H/P Tzanck cells on smear (lateral pressure applied over apparently normal skin or
No antibody titers Convalescent sera show viral mucosa causes sliding of the skin) is positive. Ashboe–
antibodies Hansen sign, also known as bulla spreading sign (pressure
Corticosteroids used Corticosteroids contraindicated applied over a bulla makes it spread) is positive. Desqua-
mative gingivitis is commonly seen in pemphigus vulgaris
(Figure 14).
Table 5 compares clinical features of recurrent aph-
Diagnosis
thous stomatitis and recurrent intraoral herpes.
The diagnosis of pemphigus vulgaris is made based on the
Diagnosis typical clinical signs. Histopathologically, the presence of
intraepithelial bulla is characteristic of pemphigus vulgaris.
The diagnosis of RAS is done by exclusion. Hematological
Immunofluorescent studies exhibit a positive fluorescence
examination can be performed to rule out blood dyscra-
under the fluorescent microscope with the antibody binding
sias. Ocular, genital, skin, or rectal lesions should not be
to the immunoglobulin deposits in the intercellular sub-
present to make a diagnosis of aphthous stomatitis.
stance. Other non-specific findings associated with pem-
phigus vulgaris are a raised ESR and hypoalbuminemia.
Management
The primary goals of therapy for RAS are relief of pain, Management
reduction of ulcer duration, and restoration of normal oral
The mortality associated with untreated pemphigus vulgaris
function. Secondary goals include reduction in the frequency
is about 10%. Systemic corticosteroids are effective in man-
and severity of recurrences and maintenance of remission.
aging the condition. Prednisolone 0.5–1 mg/kg/day or about
Topical medications, such as antimicrobial mouthwashes
40–80 mg/day is administered. Cyclophosphamide is given
and topical corticosteroids, can achieve the primary goals
at an initial dose of 50 mg/day and the dose escalated to
but have not been shown to alter recurrence or remission
100 mg/day. Mouthwashes like benzydamine hydrochloride
rates. Systemic medications can be tried if topical therapy
0.15% will help in managing pain. Antiseptic oral rinses
is ineffective.
such as chlorhexidine gluconate 0.2% can be used.
Levamisole has shown variable efficacy in reducing
ulcer frequency and duration in patients with minor recur-
rent aphthous ulcer (RAU). Dose: 150 mg per day for 3 con-
secutive days followed by a gap of 2 weeks. Then repeat PEMPHIGOID
for 3 days. This is to be done 6 times (total therapy time is
3 months and total number of tablets is 18). Thalidomide Bullous pemphigoid and benign mucous membrane pem-
is effective but, because of its toxicity and cost, should be phigoid cicatricial pemphigoid are the two variants of
used only as an alternative to oral corticosteroids. pemphigoid.
Bullous Pemphigoid
DERMATOLOGICAL DISORDERS
Bullous pemphigoid is commonly seen in women in the 6th
and 7th decades of life. It is characterized by the appear-
Pemphigus
ance of bullae over the skin, which rupture to give rise to
Pemphigus is a chronic, autoimmune skin disorder which is erosive areas, which heal spontaneously.
considered to be the most dreaded, dramatic and devastating Not all cases of bullous pemphigoid involve the oral
of all dermatologic disorders. Pemphigus vulgaris, vegetans, cavity. Intraorally small bullae are seen especially on the
212
Figure 13
A B
(A) Ulcers in the vestibular region in pemphigus vulgaris. (B) Erosions on the hard palate in pemphigus vulgaris.
Ultrastructural studies reveal the loss of attachment of

Figure 14
the epithelium and basement membrane to the underlying
connective tissue or lamina propria.
GASTROINTESTINAL DISORDERS
ASSOCIATED WITH ORAL ULCERS
Gastrointestinal diseases that may be associated with oral

ulcers include gastroesophageal reflux disease (GERD),
inflammatory bowel diseases (Crohn’s disease, ulcerative
colitis) and celiac disease. These patients have symptoms
of gastrointestinal discomfort along with oral ulcers. Gen-
Desquamative gingivitis in pemphigus vulgaris. erally barium meal and gastroscopic examination will con-
Courtesy: Dr Francisco Sánchez, Morelia City, Mexico firm involvement of the GI tract.
gingiva and buccal mucosa. Patients may complain of Gastroesophageal Reflux Disease
mild pain. These bullae rupture over a period of time to
form erosions or ulcers. Histopathologically subepidermal Gastroesophageal reflux disease arises out of the passage
bullae formation is seen. of gastric contents into the esophagus. GERD is consid-
Bullous pemphigoid is treated with systemic steroids. ered the most common upper GI tract disorder with the
Azathioprine and cyclophosphamide have also been used prevalence ranging from 6 to 10% of the population.
successfully. However, heartburn which is the most common symptom
of GERD is reported by 59% of the population. Gastropare-
sis, increased abdominal distention, hiatal hernia, and
Cicatricial Pemphigoid myopathy causing defective GI motility are the common
causes for GERD.
Cicatricial pemphigoid is a chronic subepidermal blistering
autoimmune disease. It is characterized by the presence
Clinical features
of vesicles involving the oral, genital, and conjunctival
mucosa. The oral lesions are generally erosions or shallow ❍ Patients complain of pain burning sensation extend-
ulcers on the facial gingiva, buccal mucosa, palate, tongue, ing from epigastrium to the neck (heart burn) which is
and lower lip. The ulcers exhibit a distinct outline and heal commonly felt after a meal.
with scarring in about one month. ❍ Chest pain that mimics anginal pain.
213
❍ Esophagitis, esophageal ulceration and stricture. Clinical features

❍ Patients may complain of chronic coughing episodes.
❍ Ulcerative colitis may occur at any age. However, it
most commonly affects individuals between the 2nd
Oral manifestations
and 4th decades of life.
❍ Erosion of palatal surfaces of teeth leading to dentinal ❍ Males and females are equally affected.
hypersensitivity and occasional pulpal involvement. ❍ Patients may complain of abdominal pain and diar-
❍ Mucosal atrophy, erythema and ulceration. rhea associated with blood and mucus.
❍ Dysgeusia. ❍ Loss of appetite, dehydration, fatigue and loss of
weight.
❍ Some patients may complain of severe abdominal
Crohn’s Disease cramps and the constant desire to empty bowels.
❍ Handlers in 1999 reported extra intestinal manifesta-
Crohn’s disease is a chronic inflammatory bowel disease
that can affect any part of the GI tract. It causes fissures, tions such as ulcerations on the buttocks, abdomen,
transmural inflammation, and non-caseating granulomas of thighs and face.
the GI tract.
It generally affects males and females equally, however Oral findings
it affects white males more frequently. Oral mucosal involvement is characterized by the presence
of ulcers, angular stomatitis and superficial hemorrhagic
Clinical features ulcers. It is estimated that approximately 5–10% of the
❍ It has a bimodal peak of incidence. Crohn’s disease patients with ulcerative colitis exhibit oral manifestations.
affects individuals in the 2nd and 3rd decades of life
and in the 6th and 7th decades of life. Celiac Disease
❍ Individuals may complain of diarrhea or constipation,
abdominal pain, and intermittent fever. Celiac disease is an autoimmune gluten-dependent enter-
❍ Some patients may present with extra intestinal man- opathy characterized by intestinal malabsorption and sub-
ifestations characterized by arthritis and erythema total or total atrophy of intestinal villi which improves
nodosum. after gluten-free diet.
Oral findings Clinical features

Many patients present with oral manifestations before any 1. Infants present with growth retardation, diarrhea,
systemic manifestations are evident. Halme in 1993 reported vomiting and abdominal distention.
that the severity of oral lesions may be used as a marker 2. Patients who are not treated in time may present with
to judge the severity of the intestinal impairment. On an short stature, pubertal delay, iron and folate defi-
average approximately 8–9% of the patients may exhibit ciency with anemia, and rickets.
oral manifestations prior to intestinal involvement. 3. Young children may be depressed and irritable.
Patients may present with: 4. Adults may report of episodic or nocturnal diarrhea
and weight loss.
❍ Diffuse painful swelling of the lips, gingiva and other 5. Other extra intestinal manifestations reported in
areas of the oral mucosa. literature include infertility, peripheral neuropathy,
❍ Oral ulcers and angular cheilitis. convulsions, psychiatric disturbances and increased
❍ The characteristic feature of Crohn’s disease is the incidence of bone fractures.
cobblestone appearance of the buccal mucosa.
❍ Histopathological finding of non-caseating granulo-
Oral findings
mas is typical of Crohn’s disease.
❍ Difficulty in eating and swallowing due to the presence Patients may present with oral ulceration that may mimic
of ulcers and fissures. ulceration of recurrent aphthous stomatitis.
Ulcerative Colitis NEOPLASTIC ULCERS

Ulcerative colitis is a chronic inflammatory bowel disorder
sharing the similar clinical features of Crohn’s disease Neoplastic lesions that exhibit oral ulcerations are squa-
except that the involvement is restricted to the mucosa mous cell carcinoma, adenocarcinoma, mucoepidermoid
and submucosa of the colon. carcinoma and metastatic carcinoma.
214
Clinically, these ulcers exist for long periods of time, and ❍ MAGIC syndrome (mouth aphthae, genital lesions and
induration is a prominent feature. However, biopsy will interstitial chondritis)
help in diagnosing the nature of the ulcers. The treatment ❍ Behçet’s syndrome
for most of these ulcers is surgery/radiation or combination ❍ Reiter’s syndrome
of both. The prognosis depends upon the stage of diagnosis. ❍ Stevens–Johnson syndrome
❍ Ramsay Hunt syndrome
ULCERS OF UNKNOWN ETIOLOGY

PFAPA Syndrome (Marshall’s Syndrome)
Necrotizing Sialometaplasia In 1987, Marshall et al reported a condition with periodic
fever. This was termed Marshall’s syndrome. However, this
Necrotizing sialometaplasia is a non-neoplastic, self-limit-
condition was later referred to as PFAPA which included
ing, inflammatory condition of the salivary glands. Abrams
periodic fever, aphthous stomatitis, pharyngitis and cervical
et al in 1973 described this condition for the first time. The
adenitis.
clinical and histopathological picture of necrotizing sialo-
metaplasia resembles those of a malignancy, which often
leads to its misdiagnosis. It is proposed that necrotizing Diagnostic criteria for PFAPA syndrome
metaplasia occurs due to vascular ischemia. (proposed by Thomas et al)
1. Regularly recurring fevers with an early age of onset
Clinical features (⬍ 5 years of age)
❍ Literature review reveals that it affects individuals 2. Symptoms in the absence of upper respiratory tract
from an early age of 8 months to 70 years. However, it infection with at least one of the following clinical
is commonly seen to affect individuals in the 4th and signs:
5th decades of life. a. aphthous stomatitis
❍ The minor salivary glands of the palate (75% involve- b. cervical lymphadenitis
ment) are very frequently involved followed by other c. pharyngitis
minor salivary glands in the retromolar pad area, buc- 3. Exclusion of cyclic neutropenia
cal mucosa, tongue, incisive canal, and labial mucosa. 4. Completely asymptomatic interval between episodes
❍ Involvement of the parotid and submandibular sali- 5. Normal growth and development.
vary glands are also reported.
❍ Lesions of necrotizing sialometaplasia can present as
MAGIC Syndrome
ulcers or well-defined submucosal swellings.
❍ The typical ulcer has a crater like form, usually mea- Literature review reveals that the symptom complex of
suring 1–3 cm in diameter. mouth and genital ulcerations associated with inflamed
❍ Erosion of the palatal bone may be appreciated in cartilage (polychondritis) was first described in 1995. Apart
some cases. from the typical ulcers involving the oral cavity and the
genital regions, polychondritis is present. It involves the
Diagnosis auricles, lungs, heart, and the vascular system. Patients may
also complain of associated symptoms such as fatigue, mal-
Based on clinical appearance alone necrotizing sialometa-
aise, and fever. Use of immunosuppressive agents such as
plasia cannot be diagnosed. Generally, a histopathological
azathioprine, methotrexate, or cyclophosphamide is the
evaluation of the lesion is necessary to establish the cor-
modality of choice to manage symptoms and signs associ-
rect diagnosis. Malignant ulcers, Wegener’s granulomato-
ated with MAGIC syndrome.
sis, and extranodal lymphoma can be considered in the
differential diagnosis.
Reiter’s Syndrome
SYNDROMES ASSOCIATED WITH ORAL Reiter’s syndrome is named after Hans Reiter who described
ULCERS a classical triad of arthritis, non-gonococcal urethritis, and
conjunctivitis, in 1916. However, in the recent years the
term ‘Reiter’s syndrome’ is used to refer to peripheral arthri-
The syndromes that are associated with ulcerative lesions
tis lasting longer than one month, associated with urethri-
affecting the oral cavity are:
tis, cervicitis, or diarrhea. It is usually seen following a
❍ PFAPA syndrome (periodic fever, aphthae, pharyngitis, gastrointestinal or genitourinary infection. Reiter’s syn-
adenopathy) drome secondary to gastrointestinal infection is seen in
215
children whereas Reiter’s syndrome secondary to genito- purposes the presence of at least three episodes of ulcers in
urinary infection is seen in adults. a year is required. Painful crops of ulcers are seen which
It is generally seen in individuals in the second and typically heal without scarring.
third decades. Peripheral arthritis is the most characteristic
feature. It usually affects the lower limbs. Mucocutaneous Genital ulcers
lesions of Reiter’s syndrome are seen in adults. Balanitis
and vulvitis are typical of Reiter’s syndrome. Oral ulcers Vulva and vagina in females and scrotum and penis in males
and erosions are seen in some patients. Histopathologi- are the common sites for ulcers. These ulcers are painful
cally it is difficult to distinguish these lesions from and usually heal with scar formation.
psoriasis.
Ocular involvement
Patients complain of increased lacrimation, conjunctival
Behçet’s Syndrome erythema, blurring of vision, eye pain and photophobia.
Behçet’s syndrome is named after Hulusi Behçet, a Turkish
Other clinical findings
dermatologist who described a triad of RAUs, genital ulcer-
ations, and uveitis leading to blindness, in 1937. Patients can present with non-specific pustular skin rashes,
There are two widely used diagnostic criteria for erythema nodosum and folliculitis. Arthralgias and arthri-
Behçet’s disease, namely, the International Study Group tis can be seen. Patients may frequently complain of
Criteria for diagnosis of Behçet’s syndrome and O’Duffy abdominal pain, diarrhea, and melena. Involvement of the
criteria. central nervous system is the most dreaded manifestation
of Behçet’s disease. Patients are also susceptible to deep
International Study Group Criteria for vein thrombosis and arterial disease as a result of vessel
diagnosis of Behçet’s syndrome involvement.
Oral ulcers (major/minor/herpetiform RAUs) occurring at

least thrice in a year and the presence of at least two of the Stevens–Johnson Syndrome
following:
Stevens–Johnson syndrome is characterized by the pres-
❍ Recurrent genital ulcerations ence of minute blisters on the skin. Detachment of the
❍ Recurrent eye lesions (uveitis, retinal vasculitis and skin is limited to about 10% of the body surface area. Skin
cells in the vitreous) detachment involving 30% or more of the body surface
❍ Skin lesions (erythema nodosum/papulopustular lesions/ area associated with epidermal necrosis is referred to as
acneiform nodules) toxic epidermal necrolysis. Clinically another variant is
❍ Positive pathergy test. considered which is characterized by skin detachment
between 10 and 29% of the body surface area and is termed
O’Duffy criteria Stevens–Johnson syndrome—toxic epidermal necrolysis
overlap.
The O’Duffy criteria require the presence of oral ulcers
(recurrent aphthous ulcerations) and the presence of any Clinical findings
two of following:
Patients complain of fever and myalgia. The initial presen-
❍ Genital ulcers tation of the condition is an erythematous rash on the face
❍ Uveitis and trunk that rapidly spreads to involve other parts of the
❍ Cutaneous pustular vasculitis body. Occasionally blisters are seen within the rash.
❍ Synovitis Stevens–Johnson syndrome and toxic epidermal necrol-
❍ Meningoencephalitis. ysis usually begin with fever, headache, cough, and body
The diagnosis requires the exclusion of inflammatory bowel aches, which may last from 1 to 14 days. This is followed
disease, systemic lupus erythematosus (SLE), Reiter’s syn- by the appearance of a flat red rash on the face and trunk,
drome, and herpetic infections. that often spreading later to the rest of the body in an
irregular pattern. The areas of rash enlarge and spread,
often forming painful blisters in the center. The skin over
Oral ulcers
the blisters can be easily slided off.
Oral ulcers are characteristic of this disease. Usually, oral Blisters are seen on oral, ocular and genital mucosa that
ulcerations are the first clinical finding. For diagnostic subsequently ruptures to form ulcers. Patients will complain
216
Table 6 Diagnostic protocols

Acute Chronic
Single Multiple Single Multiple Recurrent
• Traumatic ulcer • Herpetic gingivostomatitis • Infected traumatic ulcer • Multiple major aphthous ulcers • Aphthous ulcers
• Aphthous ulcer • Recurrent intraoral herpes • Major aphthous ulcer • Behçet’s syndrome • Cyclic neutropenia
• Herpangina • Necrotizing sialometaplasia • Reiter’s syndrome • Behçet’s syndrome
• Hand, foot and mouth disease • Tuberculous ulcer • Pemphigus vulgaris • Reiter’s syndrome
• Chicken pox • Syphilitic ulcer • Benign mucous membrane • Lichen planus
• Herpes zoster • Cancrum oris • Pemphigoid • Pemphigus
• Infectious mononucleosis • Fungal ulcer • Erosive lichen planus • Pemphigoid
• HIV infection • Malignant ulcer • Lichenoid reactions
• ANUG
• Stomatitis medicamentosa
• Stomatitis venenata
• Herpetiform aphthous ulcers
• Minor aphthous ulcers
• Leukemia
• Cyclic neutropenia
Table 7 Diagnostic protocols: investigations

Acute Chronic
Single Multiple Single Multiple Recurrent
• Generally no need • Hemogram to rule out • Biopsy • Biopsy for histopathologic • For cyclic
of investigations hematological malignancies • In case of TB, Mantoux examination and DIF neutropenia, TC
• Routine hemogram • Tzanck smear test, ESR, lymphocyte • Indirect IF and DC thrice in a
may be performed • Antibody titers in recurrent count, chest X-ray, PCR • Routine hemogram with week for 6–8 weeks
viral infection • In case of syphilis, VDRL ESR • Hemogram
• Paul Bunnell test for infectious test, FTA-ABS test or TPI • Serum protein level • Pathergy test in
mononucleosis assay • Patch test in case of Behçet’s syndrome
• Bone marrow study for • Special stains in case of suspected lichenoid
leukemia suspected fungal infections reactions
difficulty in eating and swallowing. Increased salivation Step 3: Correlate the history and clinical findings and
may be an associated complaint. draw a differential diagnosis
Step 4: Investigations to establish the diagnosis (Table 7).
DIAGNOSTIC PROTOCOL Step 5: General guidelines for managing oral ulcers:

1. Patient is advised to discontinue oral habits such as
smoking/pan chewing/alcohol.
Step 1: Determine whether ulcers are acute or chronic,
2. In viral ulcers, rest is important and adequate hydration
single or multiple or recurrent (by history) (Table 6).
to be ensured.
Step 2: Note the features of the ulcer(s) and associated 3. The treatment should be directed at the cause.
symptoms: 4. Symptomatic relief with topical anesthetic/analgesic
1. Size, shape, location, surrounding area, tissue tags at preparations.
periphery, tenderness, foul smell, bleeding, induration 5. Antiseptic ointments/gels to prevent secondary infec-
of base, edges, margins, floor tion.
2. Presence of skin lesions (e.g. lichen planus, pemphigus, 6. Topical steroids in ulcers taking longer time to heal
pemphigoid, erythema multiforme, Stevens–Johnson (triamcinolone acetonide in Orabase).
syndrome, etc.) 7. Systemic steroids in case of erythema multiforme,
3. Systemic symptoms (fever, malaise, etc.) seen in viral pemphigus, pemphigoid, erosive or vesiculobullous
infections, ANUG, TB, erythema multiforme, etc. lichen planus and allergic stomatitis.
217
CHAPTER
9 Dermatological Diseases
K Srinivas, Sarita Dimri, Ravikiran Ongole
➧ Lichen Planus ➧ Hailey–Hailey Disease (Familial Benign

➧ Epidermolysis Bullosa Chronic Pemphigus)
➧ Psoriasis ➧ Darier’s Disease (Keratosis Follicularis)
➧ Ectodermal Dysplasia ➧ Reiter’s Syndrome
➧ Ehlers–Danlos Syndrome ➧ Incontinentia Pigmenti (Bloch–Sulzberger
➧ Pachyonychia Congenita Syndrome)
➧ Kawasaki Disease (Mucocutaneous Lymph
➧ Dyskeratosis Congenita
Node Syndrome)
➧ Pityriasis Rosea
➧ Tuberous Sclerosis Complex (Epiloia,
➧ Xeroderma Pigmentosum
Bourneville’s Disease)
➧ Acanthosis Nigricans
➧ Graft-versus-host disease
➧ Goltz–Gorlin Syndrome
➧ Diagnostic Signs in Dermatology
➧ Acrodermatitis Enteropathica
LICHEN PLANUS Etiopathogenesis

An interplay of host, lifestyle, and environmental factors
Lichen planus (LP) has also been known as lichen ruber has been implicated in the etiopathogenesis of LP. It is
planus. It is one of the most common dermatologic, immu- believed that LP is caused due to cell-mediated immunity
nopathological diseases to affect the oral mucous mem- initiated by endogenous or exogenous factors.
brane. The management of oral lichen planus continues to
challenge even the most experienced oral physician.
Clinical features
The term ‘lichen planus’ is derived from a Greek word
lichen which means tree moss and a Latin word planus The onset of LP occurs most commonly during the 5th or
which means flat. The strange name of the condition was 6th decade. No sexual predilection is evident. The typical
provided by the British physician Erasmus Wilson, who cutaneous lesions of LP present as flat topped, purple, polygo-
first described the lesion in 1869. Thibierge first described nal, pruritic papules and plaques most commonly occurring
the oral lesions systematically in 1885. on the flexor surfaces of the arms, wrists, ankles, and legs.
Fitzpatrick et al (1993) described LP as a unique cutane- Oral lesions may be observed in up to 75% of patients
ous entity consisting of an eruption of papules distinct in with cutaneous LP and in approximately 25% of cases it
color and configuration, in patterns and location of appear- can be the only manifestation of the disease. Conversely,
ance and in microscopic as well as gross structure. only 10–20% of patients whose initial presentation is oral
Andreasen categorized oral LP into six types, namely, LP will develop cutaneous LP. The oral lesions have been
reticular, papular, plaque-like, atrophic, erosive, and bul- observed in up to 1–4% of the population.
lous. Oral LP almost invariably occurs as a bilateral disease
Lichen planus has been associated with various diseases and it involves the posterior buccal mucosa followed less
such as hepatitis C, oral cancer, and diabetes mellitus. commonly by the tongue, gingiva, hard palate, and the
218
Chapter 9 – Dermatological Diseases
Unknown antigenic change in oral mucous membrane Figure 1
Focal accumulation of Langerhans cells within

the epithelium
Activated helper/inducer T lymphocyte in

the lamina propria
Expression of ICAM and HLA-DR on the

surface of keratinocytes
Influx of cytotoxic/suppressor T-cells within

the epithelium
Keratinocyte damage
Linear lesions of LP occurring as a result of scratching

Basal cell degeneration characteristic of Koebner’s phenomenon. Courtesy:
MCODS, Mangalore
Pyknotic and shrunken basal cells (Civatte bodies)
Apoptosis of keratinocytes
Atrophic and erosive lesions involving the gingiva results
in desquamative gingivitis which is characterized by bright
red areas involving the full width of attached gingiva.
Failure of phagocytosis of apoptotic cells
Investigations
Colloid bodies (underlying dermis) Biopsy of the lesion should be done to confirm the diag-
nosis. In situations where histopathology does not confirm
the diagnosis then immunofluorescence studies of biopsy
labial mucosa. Although any site can be involved, palatal specimens should be done. Direct immunofluorescence
and sublingual lesions are very uncommon. demonstrates a shaggy band of fibrinogen in the basement
Clinically oral LP appears as radiating white or gray membrane zone in 90–100% of cases. Specimens for immu-
velvety thread like lesion, which consists of papules in lin- nofluorescence should be stored in Michel’s/Bouin’s solu-
ear, annular or retiform arrangement. A tiny white ele- tion or normal saline and then sent to histopathology.
vated dot is present at the intersection of the white lines
known as ‘Wickham’s striae’ or ‘Honiton lace’. Differential diagnosis
An isomorphic response (Koebner’s phenomenon) is com-
Reticular form – Lichenoid reactions
mon occurrence in LP, and develops in areas previously
Plaque form – Leukoplakia, hyperplastic can-
subjected to some type of trauma (Figure 1). Reticular is
didiasis, traumatic keratosis
the most common type, consisting of slightly raised fine
Atrophic form – Speckled leukoplakia, anemic
whitish lines in an interlocking lace like keratotic pattern.
stomatitis, systematic lupus
Papular lesions are small (0.5–1.0 mm) white raised pap-
erythematosus and discord
ules. Plaque type closely resembles leukoplakia with a
lupus erythematosus
reticular surrounding. Atrophic type appears as inflamed
Erosive and bullous form – Vesiculobullous lesions
areas of mucosa covered by thin red appearing epithelium.
Annular form – Erythema circinata migrans.
Erosive type presents with atrophic mucosa with ulcers.
Bullous form is very rare and is characterized by forma-
Management
tion of large thin walled bullae.
Papular, plaque like, atrophic and erosive lesions are The lesions of oral LP appear, regress and reappear in some
very frequently accompanied by reticular lesions. All what unpredictable fashion. Asymptomatic LP need not be
forms of oral LP are generally asymptomatic, but atrophic, treated. The treatment of symptomatic LP is necessary.
erosive and bullous forms are associated with pain and The role of Candida in the causation of oral LP has been
burning sensation. debated, therefore a smear for candida needs to be made and
219
if positive, a topical antifungal—clotrimazole (available as develop vesicles and bulla in response to friction. Oral
Candid gum paint in India) should be given for 14 days. vesicles are mild and small and heal without scarring.
Steroid-resistant cases can be treated using topical
tacrolimus 0.1% (available as Tacroz and Tacrovate), cyclo- Epidermolysis bullosa simplex with muscular dystrophy
sporine rinse and systemic hydroxychloroquine sulfate An autosomal recessive disorder that appears at birth.
200 mg o.d. for 3–6 months (available as tablet HCQS). Multiple bullae are seen frequently involving the oral mucosa.
Other modes of treating oral LP are topical and systemic Extremities develop more bullae which result in scarring that
retinoids, psoralen ultraviolet A (PUVA) therapy, dapsone, eventually leads to muscular dystrophy and deformity.
mesalazine and levamisole. Epidermolysis bullosa atrophicans generalisata graves
Erosive LP is a premalignant condition with a malignant An autosomal recessive disorder that develops in neonates
transformation rate of 0.1–0.3%. It has to be supplemented within hours after birth. Nail beds are usually the first area
with systemic vitamin A preparations for chemoprevention. of involvement with shedding of nails, remaining skin
However most lesions of erosive LP do not respond to surface progressively develops bullae. Many infants die
conventional steroid therapy unless supplemented by intra- within a few months and survivors have nail distortion,
lesional steroids and especially so if ulcerations are present. growth retardation, anemia, scarring and skin lesions.
Treatment of oral lichen planus is given in Flowchart 2 Large fragile vesicles and bullae are frequently seen in the
on page no 153. oral cavity, especially in the posterior hard palate and soft
palate. Enamel hypoplasia and enamel pits leading to
dental caries are usually seen. Another routinely encoun-
EPIDERMOLYSIS BULLOSA tered feature is perioral crusting.
Epidermolysis bullosa dominant dystrophic/hypertrophic
Epidermolysis bullosa (EB) is a diverse group of disorders
form An autosomal dominant form, which is very mild;
that have as a common feature blister formation with tissue
20% of patients develop lesions before 1 year of age.
separation occurring at variable depths in the skin and/or
Vesicles and bullae begin to develop and gradually lessen
mucosa depending on the specific EB type. There may be
with age. Dystrophic nails and scarring is prominent. White
marked oral involvement, potentially creating devastating
mucosal epithelial inclusion cysts may be seen on the
alterations in the soft and hard tissues. Oral tissue fragility
tongue, buccal mucosa and palatal mucosa.
and blistering is common to all EB types.
Scarring epidermolysis bullosa with dermolytic vesicles
Classification An autosomal recessive disorder that appears shortly after
birth. Bullae are seen on the feet, fingers, buttocks, back
❍ Epidermolysis bullosa simplex and the occiput. Oral vesicles prone to scar formation are
❍ Epidermolysis bullosa simplex with muscular dystrophy seen. Hypoplastic enamel and enamel pits are commonly
❍ Epidermolysis bullosa atrophicans genaralisata graves found.
❍ Epidermolysis bullosa dominant dystrophic/hypertrophic
form
❍ Scarring epidermolysis bullosa with dermolytic vesicles. Differential diagnosis
The current classification proposed for epidermolysis Lesions of pemphigus vulgaris, erythema multiforme and
bullosa is mentioned in Chapter 7 on Vesiculobullous dermatitis herpetiformis mimic epidermolysis bullosa. His-
Disorders. topathological and immunofluorescent studies will help in
differentiating these conditions.
Etiopathogenesis
Management
Pertaining to hereditary forms pathogenesis appears to be
related to genetic defects in basal cells, hemidesmosomes Treatment is often frustrating because conventional therapy
or anchoring connective tissue filaments depending on the with corticosteroids and immunosuppressive agents fre-
disease subtype. The acquired non-hereditary type is often quently does not result in significant clinical improvement.
precipitated by exposure to specific drugs, and type VII Various therapeutic modalities have recently been used
collagen antibodies located below the lamina densa are which include cyclosporine, colchicine, plasmapheresis,
found. extracorporeal photochemotherapy and intravenous gamma
globulins.
Although the data are preliminary, they suggest that
Clinical features
intravenous immunoglobulins may be a promising treat-
Epidermolysis bullosa simplex Epidermolysis simplex is ment modality for resistant, non-responsive or refractory
seen in neonates and infants. Nails, feet, hand and neck EB acquisita.
220
Dental management Etiopathogenesis

The majority of individuals with mild EB subtypes may Although the cause of psoriasis is unknown, it is now widely
receive dental treatment with only minor modifications in accepted that psoriasis involves an increase in the rate of
approach. epithelial cell proliferation. It is proposed that the increased
Individuals with EB can retain their dentition using epithelial turnover rate of psoriasis is associated with cell
conventional restorative techniques. With aggressive damage.
preventive interventions and management of developing There also appears to be a strong genetic component to
malocclusions using serial extraction, it is also possible the pathogenesis of psoriasis. Thirty-five percent of patients
to reduce the likelihood of rampant caries, achieve an with psoriasis have a positive family history. Studies on
acceptable occlusion without the need for active tooth the major histocompatibility complex (MHC) have shown
movement or appliance therapy, and allow these indi- a strong association between B13, Bwl7, Bw37, and Cw6
viduals to benefit from maintaining a natural healthy antigens and development of psoriasis.
dentition.
Clinical features
Psoriasis is more common in whites and in women. Approxi-
PSORIASIS mately 10–15% of new cases begin in children younger than
10 years. The median age at onset is 28 years. Typical skin
Psoriasis is a non-contagious skin disorder that most com- lesions of psoriasis appear as well-circumscribed erythem-
monly appears as inflamed, edematous skin lesions covered atous patches with overlying thick silvery scales (Figure
with a silvery white scale. 2A, B). Lesions may occur in any location, but most com-
monly involve the scalp and the anterior hairline, torso,
bony prominences of the extremities, nails, perianal and
Types
perineal areas. The course of the disease is unpredictable
❍ Guttate psoriasis and characterized by spontaneous episodes and relapses.
❍ Pustular psoriasis If the deep scales are removed, one or more tiny bleed-
❍ Inverse psoriasis ing points are disclosed, which is popularly referred to as
❍ Erythrodermic psoriasis. Auspitz’s sign.
Figure 2
A B
Erythematous patches on the forearms along with silvery scales in psoriasis. Courtesy: Department of
221
The oral counterpart of psoriasis is rare. The first descrip- Clinical features
tions of oral lesions of psoriasis have been attributed to
The condition is characterized by hypodontia, hypotricho-
Oppenheim and Thimm (1903).
sis, and hypohidrosis. Neonates exhibit excessive scaling
Van der Waal and Pindborg described four types of oral
of the skin and unexplained pyrexia. Patients present with
psoriatic lesions.
sparse hair and eyebrows (Figure 3A, B).
❍ Well-defined, gray to yellowish white, tiny, round to As the age progresses frontal bossing, saddle nose, sunken
oval lesions. cheeks, thick/everted lips wrinkled and hyperpigmented
❍ Lacy, circinate, white elevated lesions on the oral mucosa
and the tongue paralleling skin lesions.
❍ Fiery red erythema of the oral mucosa including the
tongue seen primarily in the acute form of psoriasis. Figure 3
❍ A geographic tongue that occurs more frequently among
A
patients with psoriasis than without.
Histopathology
Histopathologically, intraepithelial microabscesses (Munro’s
abscesses) are common. However, these are not specific for
the disease.
Management
Oral lesions should be treated with topical corticosteroids.
The topical steroids that are available in India are triam-
cinolone acetonide—0.1% with orabase (Kenacort oral paste,
TESS cream), clobetasol propionate—0.05% (Clobetamil
cream, Tenovate skin cream).
Psoriatic arthritis
B
Psoriatic arthritis is a systemic disorder and inflammatory
condition and this disease may be the major contributing
factor to temporomandibular dysfunction (TMD) symptoms
and signs. TMD signs and symptoms are found more fre-
quent and more severe in the patients with psoriatic arthritis
of other joints than in the patients with psoriasis without
arthritis. TMD signs and symptoms in psoriasis are mainly
caused by the related joint involvement that directly affects
the masticatory system.
ECTODERMAL DYSPLASIA
The ectodermal dysplasias comprise a large and heteroge-

neous group of disorders (about 170) characterized by a
variety of congenital defects in structures of ectodermal
origin including skin, hair, teeth, nails, and sweat glands.
Out of these the most common and best studied disease is
anhidrotic or hypohidrotic ectodermal dysplasia (Christ–
Siemens–Touraine syndrome).
(A) A boy with sparse scalp hair in ectodermal dysplasia.
Etiopathogenesis Courtesy: Dr Sumanth KN. (B) Photograph showing
It is an inherited X-linked recessive trait associated with hair loss of the eyebrows and scalp hair. Courtesy:
the repressed expression of a gene on the X-chromosome
MCODS, Mangalore
in the positions from q13 to q21.
222
Figure 4 Figure 6
Hypoplastic and conical primary maxillary central incisors in Hypoplastic and malformed maxillary second primary molars
ectodermal dysplasia. Courtesy: Dr Sumanth KN as well as underdeveloped alveolar ridges in ectodermal
dysplasia. Courtesy: Dr Sumanth KN
Figure 5 malformed (Figure 6). Orthopantomograph will help to

confirm the absence of teeth (Figure 7).
Distinct condition if all the three components are present.
However, for single component of disease the differential
diagnosis is isolated oligodontia, Witkop tooth nail syn-
drome and trichodental syndrome.
Management
There is no treatment available and the treatment is symp-
tom related. For xerostomia, pilocarpine 5 mg one tab is
given three times a day. Salivary substitutes can be used.
Crowns for malformed teeth and dental implants for eden-
tulous areas can be recommended.
Hypoplastic and malformed mandibular second primary

molars as well as underdeveloped alveolar ridges in EHLERS–DANLOS SYNDROME
ectodermal dysplasia. Courtesy: Dr Sumanth KN
The term Ehlers–Danlos syndrome (EDS) was coined after
the names of a Danish dermatologist, Edvard Ehlers (1901)
and a French dermatologist, Henri Alexandre Danlos
skin around the eyes are seen. Patients may present with
(1908) who reported patients exhibiting thin, hyperplastic
fever of unknown origin because of the inability to sweat.
skin, loose jointedness, and hemorrhagic tendencies.
It is a group of inherited disorders characterized by
Oral manifestations
excessive looseness (laxity) of the joints, hyperelastic skin
Anodontia/oligodontia is often seen. The remaining teeth are that is fragile and bruises easily, and/or easily damaged
usually malformed. Both deciduous and permanent teeth are blood vessels. The diagnosis of EDS encompasses any of
affected. Most common missing teeth are molars. Mal- six types of connective tissue disorders that are hereditary
formed teeth have truncated and conical crowns and short- in nature and exhibit a characteristic defect in collagen
ened roots (Figures 4 and 5). metabolism.
Dry mouth, high palatal arch and cleft palate may be There are six major types of EDS that are characterized
seen in some individuals. The alveolar ridges are usually by distinctive features.
223
Figure 7
Orthopantomograph showing multiple missing teeth in ectodermal dysplasia. Courtesy: Dr Sumanth KN
EDS was initially subdivided into seven types following ❍ Easy scarring and poor wound healing
a meeting of various authors in Berlin, Germany. However ❍ Premature rupture of membranes at birth
owing to its complex nature, another widely accepted clas- ❍ Visual difficulties.
sification consisting of six types of EDS was proposed in
Villefranche, France in 1997. Signs
❍ Excessive joint laxity and hypermobility
Villefranche classification of EDS ❍ Soft, thin, or hyperextensible skin
❍ Mitral valve prolapse
❍ Classical EDS (similar to Berlin Type I [gravis] and ❍ Signs of platelet aggregation failure
Berlin Type II [mitis])—Type V collagen defects. ❍ Rupture of intestines, uterus, or eyeball (seen only in
❍ Hypermobility EDS (similar to Berlin Type III [hyper- vascular EDS, which is rare)
mobile])—unknown collagen defects. ❍ Deformed cornea.
❍ Vascular EDS (similar to Berlin Type IV [arterial-
ecchymotic])—Type III collagen defects.
Oral manifestations
❍ Kyphoscoliosis EDS (similar to Berlin Type VI [ocular-
scoliotic])—defect in lysyl hydroxylase. Scarring on the chin and forehead, a history of repeated
❍ Arthrochalasia EDS (similar to Berlin Type VIIa and luxations of the TMJ, epicanthus, hypertelorism, a narrow
VIIb [arthrochalasis multiplex congenita])—defect in curved nose, sparse hair and hyperelasticity of the skin.
Type I collagen. Fragile mucosa and gingiva—early-onset generalized
❍ Dermatosparaxis EDS (similar to Berlin Type VIIc periodontitis leading to premature loss of deciduous and
[human dermatosparaxis])—defect in procollagen permanent teeth. Tooth mobility is often encountered.
N-peptidase. Hypoplasia of the enamel is commonly seen. Premolar
and molar teeth can present with deep fissures and long
Etiopathogenesis cusps. Microdontia is sometimes present. Irregularities in
Different forms of EDS have different modes of inheritance. the dentin structure and dentinal tubules may also be seen.
Family history is a risk factor in some cases. Various sub- Radiographic examination often reveals pulp stones
types are inherited as autosomal dominant/recessive and and roots that are short and deformed.
X-linked traits. A variety of genetic mutations cause abnor- The tongue is very supple. Approximately, 50% of those
mality in collagen that will result in the disease. with the syndrome can touch the end of their nose with their
tongue (Gorlin’s sign), compared to 8–10% of the normal
Clinical features population and the palate is commonly vaulted.
Symptoms
Investigations
❍ Joint dislocation/subluxation/joint pain, increased
joint mobility, joints popping, early arthritis, double- ❍ Collagen typing performed on a skin biopsy sample
jointedness and flat feet ❍ Collagen gene mutation testing
❍ Easily damaged, bruised, and stretchy skin which is ❍ Lysyl hydroxylase or oxidase activity
very soft and velvety ❍ Echocardiograph (heart ultrasound).
224
Differential diagnosis as hoarseness and occasionally as a life-threatening

respiratory stridor.
Marfan’s syndrome, generalized familial joint hypermobil-
ity syndrome, cutis laxa, pseudoxanthoma elasticum and
Oral manifestations
Larsen’s syndrome.
Oral findings present soon after the birth and may be the
Management earliest sign of the disease. Focal or generalized white
opaque thickening of oral mucosa (oral leukokeratosis) is
There is no specific cure for EDS, so individual problems
commonly seen on the buccal mucosa, tongue and lips.
and symptoms must be evaluated and cared for appropri-
Intermittent angular cheilitis is also seen in some cases.
ately. Genetic counseling is recommended for prospective
Patients sometimes present with natal and prenatal teeth.
parents with a family history of EDS.
Investigations
Dental considerations
Molecular DNA analysis will help in identifying the condition.
❍ Mitral valve prolapse indicates prophylactic antibiotics Biopsy may be performed to differentiate oral leukokerato-
are for relevant procedures. sis from leukoplakia.
❍ Inferior alveolar nerve blocks should be given with
Though no specific treatment exists for the condition, it
great care to avoid causing hematoma. is believed that the clinical manifestations of the condition
❍ Forces used in orthodontic treatment should be
become less severe with advancing age.
lighter than usual, given the fragility of the periodontal
ligament and relapses are common dictating a longer
period of retention.
❍ Ideally, oral surgery should be avoided. It is imperative DYSKERATOSIS CONGENITA
to test blood coagulation values before proceeding
with surgery. Since sutures do not hold well wounds Dyskeratosis congenita, also known as Zinsser–Engman–
should be covered with acrylic dressings. Cole syndrome and Hoyeraal–Hreidarsson syndrome, is a
rare, progressive bone marrow failure syndrome charac-
terized by the triad of reticulated skin hyperpigmentation,
nail dystrophy, and oral leukoplakia.
PACHYONYCHIA CONGENITA
It is a pre-malignant condition. It is genetically hetero-
geneous, with X-linked recessive, autosomal dominant
Pachyonychia congenita (PC) is a rare autosomal domi-
and autosomal recessive subtypes. It is related to telomer-
nant keratin disorder that typically affects the nails and
ase dysfunction. Telomeres are repeat structures found at
palmoplantar skin, and often the oral mucosa, tongue,
the ends of chromosomes that function to stabilize chro-
larynx, teeth, and hair.
mosomes. With each round of cell division, the length of
Müller made the first documented observation in 1904.
telomeres is shortened and the enzyme telomerase com-
Jadassohn and Lewandowsky published the next reports
pensates by maintaining telomere length in germline and
in 1906. In the dermatologic literature, PC is better known
stem cells. Because telomeres function to maintain chro-
as Jadassohn–Lewandowsky syndrome. PC is also known
mosomal stability, telomerase has a critical role in pre-
as Touraine’s polykeratosis congenita, palmoplantar kera-
venting cellular senescence and cancer progression.
toderma.
Rapidly proliferating tissues with the greatest need for
telomere maintenance (e.g. bone marrow) are at greatest
Etiopathogenesis
risk for failure.
The disease results from mutations in the genes encoding
epidermal keratinocyte keratins. The mutation is likely to Clinical features
have a deleterious effect on protein structure as it inter-
Mucocutaneous features The mucocutaneous features are
feres with the assembly of polypeptides forming the keratin
the most consistent features of the disease. Reticulated skin
skeleton of epidermal cells (Bowden, 1995).
hyperpigmentation affecting the neck, face, chest, and
arms is the most common finding occurring in approxi-
Clinical features
mately 90% of patients. Telangiectasia, atrophy (poïkilo-
Onychodystrophy, palmoplantar keratoderma and folli- dermia) dystrophic nails and palmoplantar keratoderma,
cular keratosis in areas of friction are characteristic fea- hyperhidrosis, blepharitis, conjunctivitis, epiphora include
tures. Epidermal inclusion cysts and pilosebaceous cysts other signs which are less frequently seen.
are often seen in PC. Thickened or coarse curly hair is Leukoplakia, which is the third feature of the classic
usually seen. Laryngeal involvement is usually present clinical triad, has been reported in 80% of the affected
225
patients. This can occur on any mucosal surface, but has implicated. Recently herpes like particles have been found
been most frequently reported affecting the oral mucosa. in 71% of PR lesions. Involvement of two herpes viruses,
The specific intraoral sites affected include lingual mucosa, HHV-6 and HHV-7 has been suggested as a cause of erup-
buccal, mucosa and the palate, with the tongue being the tion. There are various reports associating PR like erup-
most frequently affected. The other sites reported include tions with drugs like metronidazole, barbiturates, captopril,
the urethra, glans penis, vagina and anorectal region. clonidine, gold and ketotifen. However, reported epidemio-
Patients have a recognized increased risk of malignancy logical evidence for infectivity includes occasional family
from pre-existing mucosal leukoplakia, reaching an inci- history or household outbreaks, seasonal and year to year
dence of approximately 35% with a peak in the third to fluctuations.
decade of life.
Clinical features
Non-mucocutaneous features The non-mucocutaneous
features include bone marrow failure, pulmonary disease, The first manifestation of the disease is usually the
ophthalmic, skeletal, dental, genitourinary, gastrointesti- appearance of the herald patch which is sharply defined,
nal and neurological abnormalities. One of the most com- bright red, round or oval plaque of 2–5 cm covered with
mon features of this disease is bone marrow failure fine scales usually seen on the thigh or upper arm, trunk
resulting in peripheral cytopenias. or the neck in 50–90% of cases. After an interval of
Bone marrow failure is reported as the principal cause 5–15 days, the general eruptions begin to appear in crops
of death in 70% of patients as a consequence of bleeding at 2–3 days interval as dull pink colored oval small plaques
or opportunistic infections. covered by fine, dry, silvery scales in Christmas tree pat-
tern (long axis of the lesion characteristically follow the
Oral manifestations lines of cleavage parallel to the ribs) on the upper chest
and back.
Manifestations in the oral cavity other than leukoplakia
Involvement of oral mucous membrane is unusual but
include hyperpigmentation of the buccal mucosa, severe
ill-defined red patches with some desquamation or with
periodontal disease, hypocalcified teeth and taurodontism.
punctuate hemorrhages, or bullae may be observed.
Crops of vesicles with patches of white necrotic mucosa
The lesion of PR can be large (PR gigantea), urticarial
infected with Candida are another commonly encountered
(PR urticata), vesicular, pustular, purpuric and erythema
feature.
multiforme like.
The oral lesions can be histopathologically evaluated
for assessing the degree of dysplasia.
Management ❍ Seborrheic dermatitis
❍ Guttate psoriasis
Pancytopenia responds to androgen therapy in about 50%
❍ Secondary syphilis
of patients. Supportive care is important. Bone marrow
❍ Pityriasis lichenoides.
transplant has cured about 25% of a small number of patients.
Oral leukoplakia should be treated conventionally.
Dental considerations Since the disease is asymptomatic and self-limiting, no
treatment is required. Oral erythromycin in a dose of 200 mg
It is imperative to test blood coagulation values before
four times a day has been shown in a study by Sharma
proceeding with surgery in patients with aplastic anemia
et al to hasten the clearance of the lesions. If the itch is
and pancytopenia.
troublesome or the appearance is distressing, a topical ste-
roid or UVB irradiation can be helpful.
The skin lesion commonly fades after 3–6 weeks and
PITYRIASIS ROSEA may leave temporary hypo- or hyperpigmentation. Second
attack of PR occurs in about 2% of cases after an interval
Pityriasis rosea (PR) is an acute self-limiting disease, proba- of few months or many years.
bly infective in origin, affecting mainly children and
young adults, and characterized by a distinctive skin erup-
tion and minimal constitutional symptoms. XERODERMA PIGMENTOSUM
Etiology
Xeroderma pigmentosum (XP) is a rare autosomal reces-
The cause of PR is uncertain, but many epidemiological and sive disease characterized by photosensitivity, pigmentary
clinical features suggest that an infective agent may be changes, premature skin aging, neoplasia and abnormal
226
DNA repair. Some patients with XP also have neurological Diagnosis

complaints.
The unscheduled DNA synthesis assay (following UV irra-
Etiology diation of the cells in culture) is the standard laboratory
method for diagnosis of XP. Prenatal diagnosis by amnio-
Hebra and Kaposi first reported this disorder in 1874 and centesis and molecular genetic techniques allow for earlier
the term ‘xeroderma pigmentosum’ meaning pigmented and more reliable results.
dry skin was introduced in 1882.
Inheritance is autosomal recessive and there are at least Treatment
eight different subtypes that are recognized, designated
complementation groups A–G and XP variant. Patients must be protected from sunlight by every possible
Cleaver first reported in 1968 that fibroblast from most means—by using sunblock creams, sunglasses with side
patients with typical XP lack the normal capacity to repair shields, two layers of clothing and broad brimmed hat.
UV radiation damage to DNA. Epstein et al in 1970 showed Early and adequate excision of all tumors is essential.
that DNA repair was defective in vivo. Approximately, Topical 5-fluorouracil may be useful for early or pre-
80% of patients with XP show a defect in the initiation of malignant lesions. A recent clinical trial by Yarosh et al
DNA excision repair of UV photoproducts in all cell types: used the microbial enzyme T4 endonuclease V applied
epidermal cells, dermal fibroblasts, lymphocytes, conjunc- regularly as a topical liposome lotion for over a period of
tival cells, corneal cells and amniotic fluid cells. 1 year, significantly reduced the onset of both new basal
cell carcinoma and actinic keratoses.
Clinical features The prognosis is poor. Most patients die 30 years earlier
than the normal population, either directly from cutane-
Skin is normal at birth and first symptoms are noted ous malignancy or from complications associated with the
between the 6th month and the 3rd year in 75% of cases treatment of the cancer.
as freckling and increasing dryness on light exposed sur-
faces followed by acute sunburn or more persistent ery-
thema telangiectasia and angiomas on unexposed skin
and on the lingual and buccal mucosa. Superficial ulcers, ACANTHOSIS NIGRICANS
healing with difficulty leave disfiguring scars and con-
tractures may produce ectropion. Keratoacanthomas, Acanthosis nigricans (AN) is an acquired dermatologic
actinic keratoses, small round white atrophic spots and condition characterized by the development of a velvety,
crusted vesiculobullous lesions are also seen. brownish alteration of the skin. The cutaneous lesions itself
Malignant tumors like basal cell carcinoma, squamous is benign, yet it is significant because it represents a cuta-
cell carcinoma may develop as early as the 3rd or 4th year. neous marker for internal malignancy.
The disease is often fatal before the age of 10 years and
worldwide two-thirds die before 20 years of age. Etiology
Acanthosis nigricans has a variety of known causes
Ocular symptoms
whose common mechanism is likely to be stimulation of
The eyes are affected in 80% of cases and presents as photo- tyrosine kinase growth factor receptor signaling pathway
phobia and conjunctivitis as early symptoms and ectropion, in epidermis.
symblepharon, ulceration, vascular pterygium, corneal opac- In insulin resistance syndrome, high levels of circulat-
ities and epitheliomas of lid conjunctiva and cornea may ing insulin directly or indirectly activate the insulin-like
develop later. growth factor 1 receptor (IGF1R), which is a transmem-
brane protein related to the insulin receptor.
Neurological symptoms Tumor-derived growth factors are preserved to be involved
Neurological symptoms occur in 20% of XP patients in malignant AN.
with one or more of the following: mental retardation,
areflexia or hyporeflexia, spasticity, ataxia, sensorineural Clinical features
deafness, dysphasia and abnormal electroencephalographic Earliest changes are pigmentation, dryness and roughness
findings. of the skin which in the affected areas is grayish brown or
black, palpably thickened and covered by small papillo-
Oral manifestations
matous elevations, which give it a velvety texture. As the
Oral manifestations which often occur before 20 years of thickening increases, the skin lines are further accentuated
age, include development of squamous cell carcinoma of and the surface becomes mammillated or rugose and larger
the lower lip and tip of the tongue. warty excrescences develop. Most common sites are axillae,
227
the back and sides of neck, anogenital region and the Skin manifestations
groin and flexures.
As the name suggests there is a focal loss of the dermis
Oral lesions of AN have also been reported and may
characterized by the outpouching or herniation of the sub-
occur in 25–50% of affected patients, especially those with
cutaneous fat. Based on the degree of melanin pigmenta-
malignant forms. The lesions appear as diffuse, finely pap-
tion, the lesion has varied appearances. Erythematous
illary areas of mucosal alteration that most often involve
macules are evident in fair skinned individuals whereas
the tongue or lips, and rarely buccal mucosa.
areas of hypo- or hyperpigmentation are seen in darker
individuals. The lesions are typically confined to the lines
Types of AN
of Blaschko. The common sites that are affected are the
1. Inherited forms of acanthosis nigricans forearms, thighs and cheeks.
2. Benign acquired acanthosis nigricans Some authors describe the presence of raspberry like
3. HAIR-AN syndrome—hyperandrogenism, insulin resis- papillomas. Papillomas are usually present at the skin and
tance and acanthosis nigricans mucosal junction such as the perioral, periocular, perianal
4. Autoimmune acanthosis nigricans and perivulvar regions.
5. Drug-induced acanthosis nigricans
6. Malignancy-associated acanthosis nigricans Facial features
7. Tripe palms Patients may have an asymmetrical face with a pointed chin.
8. Nevoid acanthosis nigricans. The eyes are usually sunken and ears may appear to be
protruded and asymmetric. Colobomas of the iris, choroid,
Management retina, or optic disk are seen in almost 35% of the patients.
Treatment is of the underlying cause or is otherwise symp- Occasionally, hypertelorism and blue sclera may be seen.
tomatic and of little help. Removal of the tumor in the
malignant form may allow some improvement. A case of Oral features
hereditary benign AN improved dramatically with etretinate. Occasionally, cleft lip and palate associated with this syn-
Although AN itself is a harmless process, the patients should drome have been reported. Both the deciduous and permanent
be evaluated to ascertain which form of disease is present. dentition may be affected. Teeth are usually hypoplastic
and microdontic. Oral papillomas may be seen in any part
of the oral mucosa. However, the lips, gingiva, tongue and
GOLTZ–GORLIN SYNDROME buccal mucosa are the common sites.
Other relatively rare findings include split/double lingual
Goltz–Gorlin syndrome is also referred to as focal dermal frenum, high palatal vault and cleft lip/palate.
hypoplasia syndrome or Goltz syndrome. We should be
aware that this syndrome is not the same as Gorlin syn- Radiographic findings
drome or Gorlin–Goltz syndrome, which is basal cell nevus The characteristic finding is the presence of osteopathia stri-
syndrome. ata (longitudinal striations) in the metaphysis of the long
This uncommon genetic condition is transmitted as an bones and the sacral bone. Orthopantomograph may reveal
autosomal dominant trait. It is characterized by typical the presence of multiple taurodonts.
skin defects and widespread involvement of various organ
systems. It affects the eyes, skeletal system, urinary sys- Treatment and prognosis
tem, cardiovascular and central nervous system and the
No definitive treatment is possible for the condition. The
gastrointestinal system.
prognosis and severity of the condition depends on the
organ system that is involved.
Clinical features
Almost 90% of the individuals who present with this syn-
drome are females. When it occurs in males the condition ACRODERMATITIS ENTEROPATHICA
is lethal. The clinical features are usually evident at birth
and the signs and symptoms progress with advancing age. Acrodermatitis enteropathica (AE) is a rare disease trans-
Patients are usually short statured with sparse hair over mitted as an autosomal recessive trait. It was first recog-
the scalp, pubic region, eyebrows and eyelashes. Patients nized in 1936 by Thore Brandt and further investigated by
may complain of hypohidrosis. Other features include nail Danboly and Closs. Although deficiency dermatitis caused
dystrophy and syndactyly, polydactyly and lobster-claw by low dietary zinc has the exact clinical and histological
deformity of the hands. Diffuse cortical cerebellar atrophy features but the term should be reserved only for genetic
and recurrent respiratory infections may be seen. causes of zinc deficiency.
228
Etiology Clinical features

The gene defect appears to involve SLC 39 A4 on The condition usually presents in the third or fourth decade
8q24 which encodes a ZIP protein responsible for the zinc as flaccid vesicopustules, crusted erosions or expanding
transport in enterocytes. Zinc absorption in patients of circinate plaques appearing in areas exposed to friction.
AE is low about 2–3% compared with 27–67% in normal Flexural disease may be hypertrophic and malodorous with
adults. soft, flat, moist vegetations and fissures. Asymptomatic
longitudinal white bands are present in the nails of some
Clinical features patients and fine palmar pits have been documented.
Herpes simplex virus and contact dermatitis, both irri-
The disease typically starts after weaning or earlier if the tant and allergic, may exacerbate Hailey–Hailey disease.
infant is not given breast milk. The child turns peevish,
withdrawn and photophobic and develops a vesicobullous Differential diagnosis
dermatitis on the hands, feet and periorificial areas. The
scalp hair is lost. Diarrhea is often present. Growth is stunted ❍ Pemphigus vegetans
and there is a decreased resistance to infection. Wound ❍ Darier’s disease
healing is poor and skin lesions do not heal. ❍ Impetiginized eczema
❍ Candidal intertrigo.
❍ Atopic dermatitis
❍ Cutaneous candidiasis Loose, cool clothing will reduce friction and sweating.
❍ Epidermolysis bullosa Topical steroids with antibacterial agents may be effective.
❍ Seborrheic dermatitis. Rarely systemic corticosteroids may be required for wide-
spread disease. Oral treatment of herpes simplex should be
Diagnosis considered in patients with unresponsive painful disease.
Long remissions are common and many patients may
Low serum of zinc and alkaline phosphatase (a zinc improve in old age.
dependent enzyme) may aid in the diagnosis of zinc
deficiency.
DARIER’S DISEASE
(Keratosis Follicularis)
Zinc sulfate for AE was introduced between 1973 and
1974. Oral zinc in a dose of about 2 mg/kg per day was Darier’s disease, described independently by White and
found to clear all clinical manifestations. Prolonged ther- Darier in 1889, is an autosomal dominant condition charac-
apy up to adult age is necessary. terized by a persistent eruption of hyperkeratotic papules,
histological examination with a distinctive overlying dys-
keratosis.
HAILEY–HAILEY DISEASE
(Familial Benign Chronic Pemphigus) Etiology
It is caused by mutations in ATP2A2 gene at chromosome
Hailey–Hailey disease is a rare autosomal dominant 12q24.1, which encodes the sarco and endoplasmic reticu-
intraepidermal blistering disease which is characterized by lum calcium ATPase type 2 (SERCA2) which is a member of
recurrent vesicles and erosions usually affecting the neck, a family of ion pumps that maintain high calcium concen-
axilla and groin. The condition was described by Hailey tration in endoplasmic reticulum.
brothers in 1939.
Clinical features
Etiology
Characteristic lesion of Darier’s disease is a firm rough pap-
Hailey–Hailey disease is caused by mutations in ATP2C1, ule, which is skin colored, yellow brown or brown seen on
a gene on chromosome 3q21 that encodes a P-type calcium seborrheic areas which exacerbate on sun exposure,
transport adenosine triphosphate (ATPase). The cellular coalescent papules form irregular warty fissured plaques
process that may be affected by mutation includes gene or papillomatous plaque masses which, in the flexures,
transcription, post-translational modifications and traf- become vegetating and malodorous. On the scalp, heavy
ficking of adhesion proteins and the assembly of adhesion crusting has a characteristic spiny feel on palpation
junctions. with loss of hair. The external auditory meatus may be
229
blocked by keratotic debris. Palms and soles show Table 1 Infectious organisms associated with the onset of
minute pits or, in older subjects, punctuate or filliform Reiter’s disease
keratosis.
Frequency of oral lesions ranges from 15 to 50% but is Urogenital pathogens Enteric pathogens
usually asymptomatic and is discovered on routine exam- Chlamydia trachomatis Shigella flexneri, serotype 2a, 1b
ination. They consist of multiple, normal to white colored C. psittaci Salmonella typhimurium
flat topped papules that if numerous enough to be conflu- C. pneumoniae S. enteritidis
ent, result in cobblestone mucosal appearance. The lesion Ureaplasma urealyticum S. paratyphi
primarily affects the hard palate and alveolar mucosa. Neisseria gonorrhoeae Yersinia enterocolitica
If palate lesions are prominent, the condition may Campylobacter jejuni
resemble inflammatory papillary hyperplasia or nicotine
stomatitis.
Nail changes include red or white longitudinal bands of
varying width, often ending in a pathognomonic notch at Genitourinary tract involvement Urethritis is the most
the free margin of the nail. The nails are brittle. common presentation and manifests as dysuria with
mucoid or mucopurulent discharge. Acute abacterial cys-
Treatment titis with increased micturitional frequency and chronic
Many patients with mild disease require no treatment other prostatitis may occur. In females, persistent urogenital
than emollients, simple hygiene and advice to avoid exces- involvement may manifest as cervicitis, urethritis and
sive sun exposure. In severe disease oral and topical reti- vaginitis.
noids are usually effective. Arthritis The arthropathy is typically an acute asymmet-
ric additive and ascending inflammatory oligoarthritis of
weight bearing joints. Symptoms may range from slight
REITER’S SYNDROME arthralgia with no visible signs to marked erythema,
edema, tenderness and exquisite joint pains with complete
Reiter’s syndrome is characterized by the presence of immobility. Characteristic features of the arthropathy of
a non-suppurative polyarthritis exceeding a duration of reactive arthritis and Reiter’s syndrome are oligoarticular,
1 month associated or preceded closely by a lower uro- predominantly lower limbs, asymmetrical dactylitis,
genital or enteric infection in young men who carry the enthesitis and lower back/buttock pain.
HLA-27 antigen. It was named after Professor Hans Reiter, Cutaneous lesions The classic skin lesions of kerato-
who in 1916 reported a German officer who developed derma blenorrhagica manifests as thickened, heaped up,
urethritis conjunctivitis and arthritis following an episode crusted and yellowish scaly lesions on an erythematous
of bloody diarrhea. base affecting the acral lesion. Circinate balanitis affects
Inflammatory eye involvement and mucocutaneous about 20–40% and occurs in up to 85% of men with
manifestations are common. The classical triad of arthritis, sexually acquired Reiter’s disease has been shown in a
conjunctivitis, and urethritis is observed only in 33% of the study by Martin et al. In uncircumcised males, it appears
cases. The less stringent criteria of American College of as painless, serpiginous, erosive, geographic lesions on the
Rheumatology, comprising of peripheral arthritis of more glans penis and in circumcised males as hyperkeratotic
than 1 month duration, occurring in association with ure- papules.
thritis or cervicitis exhibit a sensitivity of 84.3% and spec- Nail involvement is seen in 15% of cases as yellowish
ificity of 98.2% when compared with other arthropathies. discoloration, subungual hyperkeratosis and onycholysis.
Etiology Ocular involvement Conjunctivitis is the only presenta-

tion and is mild, transient and recurrent. Keratitis and uve-
The etiopathogenesis of Reiter’s disease centers on the itis can occur.
determination of the role of probable infective triggering
agents and the proneness of certain individuals to develop Musculoskeletal features Plantar fasciitis, the classical
the condition due to genetic susceptibility (Table 1). ‘lover’s heel’ occurs in 25% of patients. It is often associ-
ated with calcaneal spur; both are highly suggestive of
Clinical features Reiter’s disease.
The earliest features of Reiter’s disease usually presents
Oral manifestations
within 1–4 weeks of exposure. Fever, constitutional com-
plaints and features of urethritis or enteritis usually pre- The oral lesions which occur in slightly less than 20% of
cede arthritis. the patients are described in various ways. Some reports
230
mention painless erythematous papules distributed on the Teeth and jaw changes are seen in 65–95% of cases
buccal mucosa and palate while other reports describe and are of considerable diagnostic importance because,
shallow, painless ulcers that affect the tongue, buccal in contrast to many dermatologic features, they persist
mucosa, palate and gingiva. Some authors have even through life. The changes are delayed eruption, changes in
described that geographic tongue may be a component of dental contour, hypodontia/microdontia, micrognathia/
Reiter’s syndrome, probably because geographic tongue prognathia.
bears a superficial resemblance to the lesion of circinate Hair are sparse and thin and partial baldness is seen in
balanitis. 35–70%.
Central nervous system changes are seen in 10–40% of
Treatment and prognosis cases as microcephaly, mental retardation, spastic paralysis,
convulsions and epilepsy.
Therapeutic intervention in Reiter’s disease should aim
Structural anomalies are most frequently related to
at suppression of inflammation, optimum joint protection,
neurological system and is seen in 14% of the cases as body
relief of pain, patient education, and when appropriate,
asymmetry, scoliosis, spina bifida, syndactylia, ear anom-
eradication of infection; NSAIDs is the mainstay of
alies, additional ribs and skull malformations.
treatment. Indomethacin (75–150 mg/day), phenylbuta-
Breast anomalies are seen in 1% of the cases as hypopla-
zone (200–600 mg/day), naproxen (375–750 mg/day) and
sia or additional nipples.
diclofenac sodium (100–200 mg/day) can be used. Systemic
Dental defects are frequent—delayed dentition, partial
corticosteroids (40–60 mg) prednisolone are indicated for
anodontia, and cone-or peg-shaped teeth are the most
the patients with florid disease, severe pain, wasting, fever,
usual.
high ESR, posterior uveitis, pericarditis and in those who
have failed to respond to NSAIDs.
Conjunctivitis is self-limiting and requires no treatment. Diagnostic criteria
One percent atropine and steroid drops for iritis and sys- No evidence of IP in a first degree female relative.
temic steroids for posterior uveitis can be given.
Keratoderma blenorrhagica and circinate balanitis require Major criteria
hygiene and topical steroids. Methotrexate and azathioprine Typical neonatal rash
should be considered as second line of therapy. ❍ Erythema
The prognosis and course of individual patient is varied ❍ Vesicles
and unpredictable. Severe disability occurs in less than ❍ Eosinophilia
15% of cases and is usually secondary to debilitating
Typical hyperpigmentation
lower extremity disease, aggressive axial involvement or
❍ Mainly trunk
blindness. Death is rare and is usually attributable to car-
❍ Blaschko’s line
diac complications.
❍ Fading in adolescence
Linear atrophic hairless lesions

INCONTINENTIA PIGMENTI Minor criteria (supportive evidence)
(Bloch–Sulzberger Syndrome) ❍ Dental involvement
❍ Alopecia
Incontinentia pigmenti (IP) is a rare X-linked dominant ❍ Woolly hair/abnormal nails
disease that affect skin, eyes, hair, teeth and central nervous ❍ Retinal disease.
system and manifest itself during early neonatal period. At least one major criterion is necessary to make a firm
The disease was first described by Garrod in 1903, its patho- diagnosis of sporadic IP. The minor criteria, if present, will
genesis in 1926 by Sulzberger and localization of rash in support the diagnosis.
1985 by Happel.
Evidence of IP in a first degree female relative

Clinical features
The evidence of IP is likely in a primary female relative
Four stages are differentiated according to the changes in
of an affected female if any of the following features are
the skin are shown in (Table 2).
demonstrable, alone or in combination.
Nail dysplasia is found in 40–60% of cases. Eye
changes are seen in one-third of the cases as speckled dif- ❍ Suggestive history or evidence of typical rash
fuse hypopigmentation in the retina (a pathognomonic fea- ❍ Skin manifestation of IP
ture), microphthalmia, lenticular hemorrhage, retrolental – Hyperpigmentation
fibroplasia, cataract and atrophy of the optic nerve. – Scarring
231
Table 2 Four stages are differentiated according to the changes in the skin
Stages Clinical features Time of manifestation

Stage 1 vesicular Linear vesicles, pustules, or blisters with erythema along Blaschko’s line During infancy, possible in childhood
Stage 2 verruciform Keratotic papules and plaques At the age of 2–8 weeks
Stage 3 pigmented Hyperpigmented macules along the Blaschko’s line in the mastoid, At the age of 12–40 weeks
axillary, and inguinal sites; the localization of secondary rash may not
coincide with that of the primary rash
Stage 4 depigmented Brownish macules begin to disappear; hyperpigmentation becomes more Continues from infancy to childhood
apparent during examination under Wood’s lamp; in the site of lower
limbs there remains linear hypopigmentation and skin atrophy
– Hairless streaks Clinical features

– Alopecia at vertex
The onset is acute, with a high fever, which lasts for at least
– Anomalous dentition
5–7 days. The mucosa and conjunctiva are injected. In the
❍ Woolly hair
mouth, the lips are dry and fissured, the tongue appears red
❍ Retinal disease
with prominent papillae (strawberry tongue) and the throat
❍ Multiple male miscarriages.
is injected. After 3–4 days there is generalized exanthema.
The area affected on the limbs becomes edematous followed
by scaling. There is accompanying cervical lymphadenitis,
In acute stage of disease, herpes simplex infection, impe- although not always present and may only involve one
tigo, candidosis, epidermolysis bullosa congenita. In later node. Fever resolves in 1–2 weeks.
stage, differentiated from post-inflammatory pigmentation
and hypomelanosis of Ito. Complications
In about one-fourth of cases there is accompanying
myocarditis which may be followed by symptomatic coro-
The vesicles should not be touched and the skin must be kept nary artery disease and in 1–2% by myocardial infarction.
clean to avoid infection. Local anti-inflammatory treat- Other complications include arthralgia, arthritis, severe ery-
ment with steroids may be applied. thema multiforme, iritis, proteinuria, hepatitis and aseptic
Timely diagnosis of IP prior to pregnancy and early meningitis.
genetic consultation of pregnant women with IP to evaluate
the risk of damage for the children of such women are Diagnosis
essential. Abnormalities on investigation includes leukocytosis and
thrombocytosis with a raise ESR. The raised platelet count
is most often seen in the post-acute phase.
KAWASAKI DISEASE
(Mucocutaneous Lymph Node Syndrome) Treatment
Intravenous gammaglobulin in high doses (2 g/kg in a sin-
This condition is usually seen in children, often affecting gle effusion over 10 hours) is very helpful in reducing the
those below 2 years of age, characterized by fever and gen- overall mortality and complication of the disease. Short-
eralized exanthem with lymphadenitis. The disease was term use of aspirin is also helpful in reducing the risk of
first described in 1967 by Kawasaki from Japan. platelet aggregation.
Etiology
Various hypotheses have been advanced to account for its TUBEROUS SCLEROSIS COMPLEX
symptoms. These include rickettsial illness, exposure to (Epiloia, Bourneville’s Disease)
house-dust mite. It has been suggested that bacterial
superantigens cause disease through wide scale activa- Tuberous sclerosis (TSC) is a genetic disorder of hamar-
tion of immune mechanism with cytokine release bringing toma formation in many organs, particularly the skin,
other cell types, including vascular endothelium, into an brain, eye, kidney and heart. The characteristic skin lesions
uncontrolled immunological reaction. are angiofibroma, shagreen patch, periungual fibroma and
232
ash leaf white macules classically, although not invariably Skin lesions
seen in combination with epilepsy and mental retardation.
Skin lesions are found in 60–70% cases. Lesions of four
Sherlock coined the term ‘epiloia’ indicating the diagnos-
types are pathognomonic:
tic clinical triad of epilepsy, low intelligence and adenoma
sebaceum. ❍ Angiofibromas usually appear between the ages of
3–10 years and often become more extensive at
Etiopathogenesis puberty and then remain unchanged. Firm, discrete,
red brown, telangiectatic papules 1–10 mm in diameter,
The inheritance of tuberous sclerosis is determined by sin-
extend from the nasolabial furrows to the cheeks and
gle autosomal dominant gene, with variable expression.
chin.
It is recognized that about half the TSC families are linked
❍ Periungual fibromas (Koenen’s tumors) appear at or
to 9q34 (TSC1) and other half to 16p13 (TSC2). TSC1 gene
after puberty as smooth, firm 5–10 mm in length, flesh
has not yet been coded but TSC2 gene encodes a protein
colored excrescences emerging from nail fold.
named ‘tuberin’ which shows homology to the catalytic
❍ Shagreen patch is an irregularly thickened, slightly
domain of the GTPase activating protein Rap1 which is
elevated, soft skin colored plaque, usually in the lum-
involved in the regulation of cell proliferation and differ-
bosacral region.
entiation.
❍ Ash leaf macules are present at birth or in infancy and
Approximately, 60–70% of TSC cases are thought to
are 3 cm in length, most easily detectable by examina-
be the result of new mutations, but before genetic counsel-
tion under Wood’s light, are frequently present on the
ing of the normal parent of an affected child, both parents
trunk or limbs.
should be fully investigated. A study by Flinter et al
showed that about 30% of normal parents had TSC.
Mental deficiency
Clinical features Mental deficiency is present in 60–70% of cases and may
be progressive, but if mental development has been nor-
Onset before the age of 5 years with cutaneous changes or
mal throughout the childhood subsequent deterioration is
with epilepsy is usual, although the disease may remain
uncommon.
latent until adolescence. Diagnostic criteria determined
Epilepsy is seen in almost all mentally retarded patients
by a committee of the US National Tuberous Sclerosis
and in some 70% of those with average intelligence. It
Association have been modified. A definitive diagnosis of
usually begins in infancy or early childhood, thus often
TSC requires two major features.
preceding the skin lesions by many years.
Major features
❍ Facial angiofibroma or forehead plaque Ocular signs
❍ Non-traumatic ungular or periungual fibroma
Ocular signs occur in 50% of the cases but are hard to
❍ Shagreen patch (connective tissue nevus)
detect. Retinal phacomas, pigmentary and other retinal
❍ Multiple retinal nodular hamartoma
abnormalities and hypopigmented spots on iris can occur.
❍ Cortical tuber
Cardiac and renal tumors, pulmonary changes, gastro-
❍ Subependymal nodule
intestinal tumors and endocrine and other metabolic dis-
❍ Subependymal giant cell astrocytoma
turbances can occur.
❍ Cardiac rhabdomyoma, single or multiple
❍ Lymphangioleiomyomatosis and/or renal angiomyo-
Oral manifestations
lipoma
❍ Hypomelanotic macules (⬎3) Oral manifestations of tuberous sclerosis include develop-
mental enamel pitting on the facial aspect of the anterior
Suggestive features requiring further investigation
permanent dentition in 50–100% of patients. These pits
❍ Multiple randomly distributed pits in dental enamel
are readily appreciated after applying a dental plaque–dis-
❍ Hamartomatous rectal polyps
closing solution to the teeth.
❍ Bone cyst
Multiple fibrous papules affect 11–56% of patients seen
❍ Cerebral white matter radial migration lines
predominately on the anterior gingival mucosa. Diffuse
❍ Gingival fibromas
gingival enlargement and radiolucencies of the jaws that
❍ Non-renal hamartomas
represents dense fibrous tissue proliferation is also seen.
❍ Retinal achromic patch
❍ Confetti skin lesions
❍ Multiple renal cysts
❍ Skin tags The cosmetic appearance may be improved by removing
❍ Positive family history in first degree relatives. angiofibromas with pulsed dye vascular laser (585 nm).
233
Neurosurgery should be considered when epilepsy is con- followed by a faint red maculopapular rash leading to des-
trolled by drugs and there is fixed, circumscribed, electro- quamation or even toxic epidermal necrolysis.
encephalographic focus. The treatment of lesions in other Chronic GVHD occurs 3–14 months after the trans-
organs is unsatisfactory and surgical procedure may be plant. When the condition is localized it occurs as hypopig-
required for relief of symptoms. mented nodular areas which eventually soften and atrophy.
Patients affected by this condition have a slightly reduce Generalized GVHD starts as erythematous rash and
life span compared with the general population, with deaths becomes lichenoid. With advancing time scleroderma like
usually related to CNS or kidney disease. changes appear.
The oral mucosal manifestations of GVHD depend on
the duration and severity of the attack and the target
oral tissue. Sometimes the oral lesions of GVHD are the
GRAFT-VERSUS-HOST DISEASE
only signs of disorder. It is estimated that 33–75% of
patients suffering from acute GVHD and about 80% of
Graft-versus-host disease (GVHD) occurs when immuno- patients suffering from chronic GVHD will have oral
competent cells from a donor recognize and react against involvement. Patients will present with fine reticular net-
‘foreign’ tissue antigen in an immunocompromised host. work of white striae that resembles oral lichen planus
Moderate to severe acute GVHD affects 9–35% of patients involving tongue, labial mucosa and the buccal mucosa.
undergoing standard allogenic bone marrow transplanta- Atrophy, ulceration and xerostomia of the oral mucosa
tion, despite using HLA matched sibling donors and can be present.
immunosuppression after grafting.
Etiology
Prophylactic use of cyclosporine, methotrexate, predniso-
Billingham described the original criteria for development lone combination therapy has reduced incidence of acute
of a GVHD in 1966: GVHD. T-cell depletion by using anti-T cell receptor anti-
❍ Genetically determined histocompatibility difference bodies is successful.
between donor and recipient. Granulocyte colony stimulating factor, used to enhance
❍ Immunocompetent cells in the grafted tissue able to engraftment may reduce the incidence of GVHD. Once the
recognize foreign histocompatibility antigens in the disease is established, treatment with high dose steroids or
host and to react against them. cyclosporine is of value symptomatically and antilympho-
❍ Inability of the host to recognize and react against the cyte globulin may be of additional benefit.
grafted tissue. The prognosis depends on the extent to which the dis-
ease progresses and whether it can be controlled. Mortality
is caused by the disease itself and severe superinfection
Clinical features
due to immunosuppressive therapy. It is believed that about
Acute GVHD occurs within 60 days of bone marrow trans- 55% of the patients with mild GVHD survive compared to
plantation and most often after 7–10 days. Mild fever is the 15% survival rate in severe GVHD.
Diagnostic Signs in Dermatology normal population. Multiple café-au-lait spots especially

more than six in number and exceeding 1.5 cm in diameter are
Antenna sign: This sign is seen in keratosis pilaris. Individual seen then underlying systemic disease is suspected. It is seen in
follicles show a long strand of keratin glinting when examined neurofibromatosis (outline is like the coast of California),
in side light resembles like antenna is known as antenna sign. Albright’s syndrome (outline is like coast of Maine), Fanconi’s
Asbo-Hansen’s phenomenon (bulla spread phenomenon): anemia, Bloom’s syndrome and Cowden’s syndrome.
Refers to the ability to induce peripheral extension of a blister Carpet tacks sign: Usually seen in discoid lupus erythemato-
as a consequence of applying lateral pressure to the border of sus. Removal of the scale shows its undersurface to be covered
intact blister. This is seen in pemphigus. with the horny plugs which filled the follicles, resembling
‘carpet tacks’.
Auspitz’s sign: This sign is seen in plaque type of psoriasis.
When the surface of the typical plaque is lifted from the base, ‘Cerebriform tongue’ sign: The sign is originally described by
punctuate bleeding points occur at the sites of scale removal. Premalatha and coworkers (1981). Oral lesions in pemphigus
vegetans are hyperplastic masses which on the tongue can give
Café-au–lait spots: These are light to dark brown well-
rise to cerebriform appearance.
circumscribed round to oval cutaneous macules, which vary in
size from 1 to 20 cm. These are usually present at birth or Crowe’s sign: The sign is seen in neurofibromatosis. Axillary
appear during infancy. Isolated lesions are found in 10–33% of freckling seen in neurofibromatosis is known as Crowe’s sign.
234
Darier’s sign: This sign is observed in mastocytosis. Gentle Id reaction: It is an allergic manifestation of candidiasis, the
rubbing of the urticaria pigmentosa skin lesion causes local dermatophytoses, and other mycoses characterized by itching
itching, redness and whealing and is known as Darier’s sign. and vesicular lesions that appear in response to circulating
This sign is due to the local histamine release in the lesional antigens at sites that are often far distant from the primary
skin. fungal lesion itself.
Deck-chair sign: This sign is noted in papuloerythroderma of Koebner’s phenomenon: Linear lesions occurring as a result of
Ofuji. Papuloerythroderma of Ofuji is a rare, intensely pruritic external trauma such as scratching is called Koebner’s phe-
eruption of unknown etiology, consisting of wide spread nomenon. This phenomenon can be seen in psoriasis and lichen
coalescing sheets of uniform erythematous papules. The lesion planus.
characteristically spares the compressed abdominal body folds,
Koebner effect: In patients with psoriatic arthritis of TMJ,
and this is known as deck-chair sign.
who have disabling movements of the mandible, surgery is
Dimple sign: This sign is usually seen in dermatofibroma. If indicated and surgery may be complicated by psoriasis
the overlying epidermis is squeezed the dimple will be seen, forming on the surgical scar, which is referred to as Koebner
indicating the tethering of the overlying epidermis to the effect.
underlying lesion.
Leser–Trélat sign: This sign often occurs as a manifestation of
Dubois sign: Usually seen in congenital syphilis. Very short visceral malignancy. Internal malignancy associated with sud-
little finger seen occasionally as late stigmata in congenital den development of numerous seborrheic keratoses, in an erup-
syphilis is known as Dubois sign. tive fashion with or without pruritus, is generally accepted as
the sign of Leser–Trelat.
Flag sign: Commonly seen in kwashiorkor and rarely in severe
ulcerative colitis and after extensive bowel resection. Alternating Nikolsky’s sign: It was named after Pytor Vasilyenich Nikolsky
white and dark bands occur along individual hair is known as who was the first to describe this finding. On applying gentle
flag sign. Intermittent protein malnutrition leads to this sign. mechanical pressure (e.g. blowing air or applying pressure with
a mirror handle) on affected tissue will result in the formation
Forschheimer’s sign: Originally described by Forschheimer, is of a lesion. Nikolsky’s sign is seen both in the skin and oral
observed in rubella. An enanthema is present in up to 20% of mucosa. It is seen in pemphigus, paraneoplastic pemphigus,
patients during the prodromal period or on the first day of the mucous membrane pemphigoid, epidermolysis bullosa, linear
rash, and are confined to the soft palate. This presence of enan- IgA bullous disease, lupus erythematosus, graft-versus-host
thema is known as Forschheimer’s sign. disease and toxic epidermal necrolysis.
Gorlin’s sign: Ability to touch the tip of the nose with the Nose sign: This sign is evident in exfoliative dermatitis. Nose
tongue in patients with Ehlers–Danlos syndrome. and perinasal area is characteristically spared in exfoliative
dermatitis involving the other parts of the face and body is
Groove sign: Originally described by Greenblatt in lympho-
known as nose sign.
granuloma venereum (LGV). Enlargement of lymph nodes above
and below the inguinal ligament may give to bubo a grooved Oil drop sign: This sign is usually seen in psoriasis. Circular
appearance known as groove sign. areas of discoloration of the nail bed and hyponychium may
resemble oil drop below the nail. Histologically there are areas
Headlight sign: Vascular stigmata associated with atopic der-
of psoriatic change below the nail bed and hyponychium.
matitis. Perinasal and periorbital pallor is termed as headlight
sign. Ollendorf’s sign: This sign is observed in secondary syphilis.
In secondary syphilis, the papule is exquisitely tender to the
Hertoghe’s sign: Cutaneous stigmata associated with atopic
touch of a blunt probe, which is termed positive Ollendorf’s
dermatitis. Thinning of the lateral eyebrows is known as
sign.
Hertoghe’s sign.
Osler’s sign: This sign is seen in alkaptonuria. By the third
Higoumenakis’ sign: This sign is commonly seen in congenital
decade of life, the deposition of pigment in alkaptonuria
syphilis. Irregular thickening and enlargement of the sternocla-
becomes apparent. In the early stages, the sclera is pigmented
vicular portion of the clavicle is known as Higoumenakis’
which is termed Osler’s sign.
sign. It is the result of periostitis and usually is unilateral than
bilateral. Psoriasiform lesions: Lesions that have histopathological
similarities to psoriasis are termed as psoriasiform lesions.
Hutchinson’s sign: This sign is observed in subungual malig- Examples of such lesions are Reiter’s syndrome, geographic
nant melanoma as well as herpes zoster ophthalmicus. A lon- tongue and erythema circinata migrans (ectopic geographic
gitudinal melanotic streak accompanied by periungual tongue).
pigmentation in subungual malignant melanoma is known as
Hutchinson’s sign. In ophthalmic nerve zoster, appearance of Romana’s sign: This sign is observed in American trypanoso-
vesicles on the side of the nose indicates the involvement of the miasis also known as Chagas disease. Unilateral edema of the
nasociliary nerve, and this is known as Hutchinson’s sign. eyelids, inflammation of the lacrimal gland associated with the
235
preauricular lymphadenopathy is known as Romana’s sign. This (1886–1954). It is basically a type of exfoliative cytology. In
sign is due to portal of Trypanosoma cruzi in the conjunctiva. pemphigus, acantholytic cells are seen and in herpes multi-
nucleated giant cells with Lipschutz bodies are seen.
Sailor’s skin: Skin damage (dry, wrinkled skin) due to pro-
longed exposure to the sun and wind. Some authors use the Wickham’s striae: It is seen in lichen planus. On skin, there
term ‘farmer’s skin’. Actinic elastosis or solar elastosis is one are flat-topped papules with violaceous hue and the surface of
such dermal condition where sailor’s skin can be appreciated. these papules is covered by characteristic very fine grayish
Thumb sign: This sign is positive in Marfan’s syndrome. white lines called Wickham’s striae. In the oral cavity the dis-
Completely opposed thumb in the clenched hand projects ease appears as radiating white lines. At the intersection of
beyond the ulnar border, known as thumb sign. It is simple these white lines a tiny white elevated dot is present known as
screening test for Marfan’s syndrome. striae of Wickham or Honiton lace.
Tzanck’s test: The examination of fluid from a bulla (a blister) Wimberger’s sign: This sign is observed in early congenital
in search of Tzanck cells characteristic of varicella (chicken syphilis. Radiological examination shows loss of density on
pox), herpes zoster, herpes simplex, and pemphigus vulgaris. the medial side of the upper end of the tibia is known as
It was named after a Russian dermatologist Arnault Tzanck Wimberger’s sign.
236
SECTION Diseases of
IV Specific Structures
10 Temporomandibular Disorders 239

11 Diseases of Salivary Glands 265
237
Chapter-10.indd 237 10/2/2012 1:35:22 PM


CHAPTER
Temporomandibular
Disorders
Shibu Thomas, Joanna Baptist,
Ravikiran Ongole, Thomas Zachariah
10
➧ Components of Temporomandibular Joint ➧ Degenerative Joint Diseases
➧ Clinical Evaluation of Temporomandibular Osteoarthrosis
Joint Osteoarthritis
➧ Clinical Evaluation of Muscles of Mastication Juvenile Idiopathic Arthritis
and Accessory/Cervical Muscles Polyarthritides
Traumatic Arthritis
➧ Disorders Associated with Deviation/
Infectious Arthritis/Septic Arthritis
Alteration in the Form of Articular Surfaces
Rheumatoid Arthritis
➧ Articular Disk Defects Psoriatic Arthritis
Disk Thinning and Perforation Hyperuricemia
Adherence and Adhesions
➧ TMJ Ankylosis
Disk Displacement
➧ Masticatory Muscle Disorders
Disk Displacement with Reduction
Disk Displacement without Reduction Acute Disorders
Displacement of Disk–Condyle Complex Chronic Conditions
(Hypermobility and Dislocation) ➧ Congenital, Developmental and Acquired
➧ Inflammatory Joint Disorders Disorders of the TMJ
Synovitis or Capsulitis ➧ Neoplasms Affecting the TMJ
Retrodiscitis ➧ Condylar Fractures
The temporomandibular joints (TMJs) are two joints bone. The convex articular eminence forms the anterior
between the mobile mandible and the fixed temporal bone. limit of the joint. The glenoid fossa is covered by a thin
Each joint contains two joint spaces which are separated layer of fibrocartilage.
by a fibrocartilaginous articular disk.
The TMJ is a compound joint. It can also be considered as
ginglymodiarthroidal joint (capability of both hinge type and Mandibular Condyle
sliding/gliding movement). The use of the term ‘compound
The condyle is elliptically shaped with its long axis oriented
joint’ is justified based on the fact that the TMJ is composed
mediolaterally. The dimension of the condyle is roughly
of three bones, namely, the mandibular condyle, squa-
20 mm in the mediolateral direction and approximately
mous portion of temporal bone and the non-ossified articular
8–10 mm in the anteroposterior direction. The articulating
disk. The TMJ is the articulation between the condyle of the
surface of the condylar head is covered by fibrocartilage
mandible and the squamous portion of the temporal bone.
(Figure 1).
COMPONENTS OF TEMPOROMANDIBULAR Articular Capsule and Articular Disk

JOINT
The articular capsule is a fibrous membrane that surrounds
Glenoid Fossa and Articular Eminence/ the joint and incorporates into the articular eminence. It
attaches to the articular eminence, the articular disk and
Protuberance
the neck of the mandibular condyle. Superiorly, it is attached
The glenoid fossa or mandibular fossa is a well-defined hol- to the temporal bone and inferiorly to the neck of the con-
low area on the inferior portion of the squamous temporal dyle. The anatomical and functional boundaries of the
239
Section IV – Diseases of Specific Structures
Figure 1 Figure 2
The biconcave articular disk.

Courtesy: Dr Arturo Mann
Skeletal components of TMJ (condylar head and

the glenoid fossa). Courtesy: Dr Arturo Mann
condyle. Both joint spaces have small capacities (passive
volume of the upper joint space is 1.2 ml and the passive
volume of the lower joint space is 0.9 ml).
The condyle articulates with the inferior surface of the
TMJ are defined by the articular capsule. The inner surface disk to form the lower joint, the disk–condyle complex, where
of the capsule is covered by the synovial membrane that only hinge or rotatory movement occurs. Translation or
aids in the secretion of the synovial fluid. The articular sliding movements occur in the upper joint, composed of
capsule confines the synovial fluid to the articulating sur- the disk–condyle complex articulating with the mandibular
faces. Temporomandibular ligaments provide additional fossa of the temporal bone.
reinforcement to the capsule on its lateral wall. During functional movements of the mandible, stability
Articular disk is a biconcave oval structure with a thin within the TMJ is achieved by maintaining the thin inter-
intermediate zone and a thick posterior and anterior border. mediate zone of the disk between the condyle and the
These thick posterior and anterior bands act as wedges, eminence.
giving the disk a self-seating capacity with the condyle The disk also serves as a shock absorber to counteract
functioning against the intermediate zone. The importance the many forces acting on the joint during both function
of this feature cannot be overemphasized; the contour of and parafunctional activity.
the disk with the thick bands helps prevent the displace-
ment of the disk from the condyle during translation. An
absence of blood vessels and nerves in the intermediate
Synovial Fluid
zone of the disk enables this part of the disk to act as a
pressure-bearing area (Figure 2). The main functions of the synovial fluid are to nourish the
The disk is a fibrous, saddle-shaped structure that sepa- avascular articulating cartilage and provide lubrication
rates the condyle and the temporal bone. The meniscus between the articulating surfaces during function.
varies in thickness—the thinner, central intermediate zone Synovial fluid is a clear straw-colored thixotropic fluid
separates thicker portions called the anterior band and the which is composed of mucin with some albumin, fat, epi-
posterior band. Posteriorly, the meniscus is contiguous with thelium, and leukocytes. Synovial fluid also contains lubricin
the posterior attachment tissues called the bilaminar zone. secreted by synovial cells. It is mainly responsible for so-
The bilaminar zone is a vascular, innervated tissue that plays called boundary layer lubrication, which reduces friction
an important role in allowing the condyle to move forward. between opposing surfaces of cartilage. Synovial fluid is
The meniscus and its attachments divide the joint into made of hyaluronic acid and lubricin, proteinases and col-
superior and inferior spaces. The superior joint space is lagenases.
bounded above by the articular fossa and the articular During movement, the synovial fluid held within the
eminence. The inferior joint space is bounded below by the cartilage is squeezed out mechanically to maintain a layer
240
Chapter 10 – Temporomandibular Disorders
of fluid on the cartilage surface (so-called weeping lubri- (Golgi tendon organs) which play an important role as static
cation). Boundary lubrication is a function of water phys- mechanoreceptors for protection of these ligaments around
ically bound to the cartilaginous surface by a glycoprotein. the TMJ.
Accessory ligaments of TMJ include the stylomandibular
and sphenomandibular ligaments.
Discal Ligaments The stylomandibular ligament runs from the styloid
The medial and lateral portions of the articular disk are process downward and forward to the medial surface and
attached to the corresponding poles of the condyles via non- border of the angle of the mandible. The stylomandibular
elastic, short discal ligaments. These ligaments are vascu- ligament functions by limiting excessive mandibular
larized and innervated. protrusion.
These ligaments restrict the movement in the lower joint The sphenomandibular ligament arises from the spine
to hinge or rotatory action when viewed in a sagittal plane. of the sphenoid bone and extends downward and forward
Discal ligaments cause the disk to move passively with to insert on the lingula of the mandible along the lower
the condyle in an anterior and posterior direction during border of the mandibular foramen. The sphenomandibular
condylar translation. These ligaments permit very little ligament restricts protrusive, mediotrusive as well as passive
lateral excursion. jaw opening.
Discomalleolar ligament was described by Pinto in 1962.
This ligamentous structure connects the malleus in the
Posterior Attachment or Retrodiscal Tissue or tympanic cavity and the articular disk and capsule of the
Bilaminar Zone TMJ. It is estimated that only 29% of the TMJs reveal
the presence of this ligament.
The retrodiscal tissue is confined to the space between the This anatomical relationship between the middle ear and
articular disk and the posterior wall of the articular capsule. the TMJ is believed to be one of the explanations for the
It is richly vascularized and well innervated. aural symptoms associated with TMJ dysfunction.
It is attached anteriorly to the posterior band of the artic- Tanaka’s ligament was described in 1986. This ligament
ular disk and posteriorly to the tympanic plate and poste- provides a cord like reinforcement to the medial wall of
rior aspect of the condyle. The retrodiscal tissue contains the articular capsule.
loosely associated collagen fibers and a meshwork of elastic
fibers.
The upper retrodiscal lamina is elastic in nature and Muscles of Mastication
exerts a posterior traction on the disk. The inferior retro-
discal lamina is non-elastic and restricts forward rotation Various mandibular movements such as opening, clos-
of the disk on the condyle. ing, protrusion and retrusion occur under the coordi-
nated movements of the muscles of mastication, namely,
the masseter, temporalis, medial pterygoid and lateral
Ligaments Associated with Temporomandibular pterygoid.
Joint
The ligaments associated with the TMJ have three major Masseter muscle
functions: stabilization, guidance of movement, and move- Masseter muscle arises from the lower border and inner
ment limitation. surface of the anterior two-thirds of the zygomatic arch,
The ligaments that have been associated with the TMJ passes inferiorly and posteriorly, and inserts on the outer
include lateral ligament (temporomandibular ligament), surface of the mandibular ramus (Figure 3). The deep fibers
stylomandibular ligament, sphenomandibular ligament, of the masseter muscle (pars profunda) are vertically oriented
discomalleolar ligament (Pinto’s ligament) and Tanaka’s whereas the superficial fibers (pars superficialis) are more
ligament. oblique. This muscle is responsible for elevating the man-
The lateral ligament is comprised of two parts: a deep dible to aid in jaw closure and for clenching and crushing
part (horizontally oriented) and a superficial part (verti- action.
cally oriented). The horizontally oriented portion of the
lateral ligament limits retrusion and laterotrusion. In this
Temporalis muscle
manner, the sensitive retrodiscal tissue is protected from
injury. The temporalis is a broad, fan-shaped muscle that arises
The vertically oriented portion of the ligament limits from the temporal fossa (superior and inferior lines of the
opening of the jaw. The vertically oriented superficial part temporal bone). Via a strong tendon it inserts into the
of the temporomandibular ligament contains nerve endings upper anterior border and the medial aspect of the coronoid
241
process, and into the anterior border and adjacent medial The muscle fibers extend inferiorly, posteriorly and laterally
surface of the mandibular ramus. to insert into the medial surface of the ramus, approximating
The anterior fibers run obliquely downward and poste- the angle of the mandible. Here it joins with the masseter
riorly (serve as elevators and also maintain the postural to form a muscle sling (Figure 4A, B).
(resting) position of the mandible, with the teeth slightly The medial pterygoid primarily aids in jaw closure. It
apart), the middle part of the temporalis muscle helps also participates in protrusion of the mandible.
in closure of the jaws and the posterior fibers which are
oriented in a horizontal direction help in retrusion of the Lateral pterygoid muscle
mandible and gentle finer movements to achieve a position
The lateral pterygoid muscle comprises two functionally
of intercuspation.
distinct entities: (i) the smaller, superior head arises from
the infratemporal surface of the greater wing of the sphe-
Medial pterygoid muscle noid and runs backward in a nearly horizontal direction;
The medial pterygoid muscle originates from the pterygoid (ii) the larger, inferior head arises from the lateral surface
fossa (medial surface of the lateral pterygoid plate). Some of the lateral pterygoid plate and runs backward in a
of the fibers also originate from the maxillary tuberosity. somewhat oblique upward direction. Both heads merge
posteriorly into a tendon that inserts in the following way:
the upper fibers, which correspond more to the superior
Figure 3 head, insert into the anterior surface of the capsule and
disk; the inferior fibers, which correspond mainly to the
inferior head, attach mostly to a depression (pterygoid fovea)
on the inner side of the anterior surface of the mandible
(Figure 5). Studies on the run and the attachment of the
lateral pterygoid muscle on 41 cadavers by Abe et al (1993)
Temporal muscle
showed the presence of a third intermediate belly of the
lateral pterygoid muscle.
Both the heads of the lateral pterygoid muscle function
as independent antagonistic muscles. Contraction of
the inferior head pulls the condyle forward down the slope
of the articular eminence (opening of the mandible and
Masseter muscle protrusion); whereas the superior head is active during
mandibular closure and contracts in conjunction with
the mandibular elevation muscles. It also exerts a holding
or bracing action on the condyle when the teeth are
Schematic diagram showing the location of the temporalis
held together and during power strokes. The superior head
and masseter muscle
also acts to rotate the disk anteriorly on the condyle
Figure 4
A B Lateral pterygoid muscle
(upper head)
Lateral pterygoid muscle
(lower head)
Temporal muscle
Medial pterygoid muscle
Schematic diagram showing the medial pterygoid muscle
242
Figure 5 Arterial Supply, Venous Drainage and

Sensory Innervation of TMJ
The TMJ and the muscles of mastication are primarily fed
by the maxillary artery and superficial temporal artery.
The blood supply to the condylar head is also derived from
the inferior alveolar artery via the bone marrow.
The venous drainage is via the superficial temporal vein,
maxillary plexus and the pterygoid plexus. The mandibu-
lar branch of the trigeminal nerve is the motor supply to the
muscles of mastication.
The TMJ is innervated predominantly by the articulo-
temporal nerve, masseter and the temporal nerves. Four
types of receptors aid in proprioception: Ruffini mechano-
receptors (type I), pacinian corpuscles (type II), Golgi ten-
don organs (type III) and free nerve endings (type IV). The
joint capsule, lateral ligaments and genu vasculosum in
the bilaminar zone typically contain these receptors. Com-
paratively, the anteromedial portion of the capsule con-
Schematic diagram showing the lateral pterygoid muscle tains very few type IV receptors.
when the disk–condyle complex is moving upward and CLINICAL EVALUATION OF

backward against the eminence. By keeping the disk TEMPOROMANDIBULAR JOINT
between the condyle and eminence, the superior head
of the lateral pterygoid muscle aids in maintaining joint Temporomandibular joints are located about 1.5 cm ante-
stability. rior to the tragus of the ear. The two TMJs, considered
together, compromise only one part of the total articula-
Suprahyoid muscles tion between the lower jaw and the skull–facial skeleton
complex. The other important contribution is made by the
The digastric, mylohyoid, geniohyoid, stylohyoid muscles interdigitations of the mandibular and maxillary dentition,
are grouped under suprahyoid musculature owing to their and function and health of the joint is directly related to
anatomic location. These muscles are considered accessory condition of the teeth.
muscles of mastication and help in jaw opening along with The history of presenting illness should include the onset
the lateral pterygoid muscle. and course of signs and symptoms. Past history should
include the details regarding arthritis, infections, degener-
Mandibular movements ating diseases, parotitis, ear disorders, muscular disorders,
trauma, past dental treatment, diet/nutritional adequacy
Forward movement or protraction The articular disk and habits like clenching, gum chewing, etc. and the indi-
glides forward over the upper articular surface, the head of vidual lifestyle.
the mandible moves along with it. The movement in the
opposite direction is referred to as retraction.
Slight mouth opening The mandible moves on the Examination of Temporomandibular Joint
undersurface of the disk like a hinge. The TMJ is examined by a thorough inspection, palpation,
Wide mouth opening The hinge like movement is fol- and auscultation.
lowed by gliding of the disk and the head of the mandible, The face is inspected for any obvious asymmetry, scars
as in protraction. At the end of this movement the articu- (may be indicative of previous surgeries, trauma), swelling/
lar disk comes and rests against the articular eminence. ulceration/sinus openings in the pre-auricular region. Observe
for deviation/deflection (Figure 6) of mandible on mouth
Chewing movements/lateral excursions In these move- opening.
ments the condylar head of one side glides forward The TMJs can be palpated by extra-auricular and intra-
along the disk but the head of opposite side merely rotates auricular methods. Palpation can be done standing at
on a vertical axis. This moves the chin forward and to 10 o’clock or 11 o’clock positions by the clinician. Intra-
one side. auricular palpation can be achieved by placing the little
243
supra-orbital ridge to above the ear. The patient is asked to

Figure 6
report any discomfort or pain.
Masseter
Masseter is palpated bilaterally in the area overlying the
anterior border of the mandibular ramus. The area of pal-
pation is directly above the attachment of the body of the
mandible.
Pterygoids
Palpation of the pterygoid muscle is difficult because of
the inaccessibility of the muscle.
Medial pterygoid
Medial pterygoid can only be palpated near its insertion
by placement of the index finger laterally and posteriorly
into the floor of the mouth toward the medial surface of
Deflection of the mandible to the left side. the angle of the mandible.
Lateral pterygoid
Tenderness of the lateral pterygoid can occasionally be
detected by indirect application of pressure. The index finger
finger inside the external auditory meatus. During man-
or back end of the handle of an instrument is positioned
dibular movement the posterior poles of the condylar head
distal and posterior to the maxillary tuberosity and poste-
can be palpated with the pulp of the little finger. Intra-
rior pressure is exerted to compress tissue against the muscle
auricular palpation may also be used to elicit capsular
(Figure 7).
tenderness.
Alternatively, the medial and lateral pterygoid muscles
Extra-auricular examination of the TMJ is achieved by
can be assessed by functional evaluation. These can be
placing the index fingers in the pre-auricular region about
achieved by asking the patient to perform simple tasks like
1.5 cm medial to the tragus of the ear. The lateral pole of
opening the mouth against resistance (Figure 8) and clos-
the condyle is accessible during this examination.
ing the mouth against resistance.
Palpatory examination of the TMJ should include
the assessment of mouth opening, range of mandibular
Digastric muscle
movements, joint tenderness, detection of clicks and/or
crepitus. Digastric muscles are palpated with the fingertips aligned
roughly parallel to the inferior border of the mandible in
the submental and submandibular region.
CLINICAL EVALUATION OF MUSCLES OF
MASTICATION AND ACCESSORY/CERVICAL Cervical Examination
MUSCLES
Temporomandibular disorders/myofascial pain disorders
Tenderness of the muscles of mastication results from stress often have musculoskeletal problems in other regions that
and fatigue which are characteristics of temporomandibu- are particularly associated with neck. Check for mobility
lar dysfunction. of the neck and examine for range and symptoms.
The muscles to be examined should include the tempo- Patient is first asked to look to the right and then to the
ralis, masseter, medial and lateral pterygoids, digastric, left. There should be at least 70⬚ rotation in each direction.
cervical and sternocleidomastoid muscles. Next patient is asked to look upward as far as possible
(extension) and then downward (flexion). Any pain is
recorded and any limitation of the movement determines
Temporalis
muscular or vertebral problem. Sternocleidomastoid/tra-
Temporalis is palpated simultaneously with the finger- pezius/posterior cervical muscles are often part of neck
tips aligned in a row from the hairline just above the disorder and may refer pain to face and head.
244
length of the muscle is palpated down to its origin near

Figure 7
the clavicle.
Posterior cervical muscles

Trapezius, longissimus (capitis and cervices) splenius
(capitis and cervices) and levator scapulae.
The posterior cervical muscles do not directly affect
mandibular movements; however, they do become symp-
tomatic during temporomandibular disorders and there-
fore are routinely palpated. They originate at the posterior
occipital area and extend inferiorly along the cervicospi-
nal region. Because they are layered over each other,
sometimes it is difficult to identify them individually.
These muscles can be examined by slipping fingers
behind the patient’s head. Those of the right hand pal-
pate the right occipital area and those of the left hand
palpate the left occipital area, both at the origins of the
Use of the back end of a mouth mirror to elicit muscle (the patient is questioned regarding any discom-
tenderness at the site of the attachment of the lateral
fort). The fingers then move down the length of the neck
pterygoid muscle
muscles through the cervical area and any patient discom-
fort is recorded.
Trapezius
Figure 8 Trapezius, an extremely large muscle of the back, shoulder
and neck, does not directly affect jaw function but is a
common source of headache and is easily palpated. It
commonly has trigger points (TrPs) that refer pain to the face
and hence the purpose of its palpation is to search for active
TrP. The upper part is palpated from behind sternocleido-
mastoid, inferolaterally to the shoulder and any TrP are
recorded.
Splenus capitis
Splenus capitis is palpated for general pain, tenderness,
and TrP. Its attachment to the skull is a small depression
just posterior to the attachment of the sternocleidomastoid.
Palpation is begun at this point and moves inferiorly as
the muscle blends into the other muscles.
Occlusal Evaluation
Examining the dentition and occlusion is an important
part of the physical examination of a TMJ disorder or oro-
facial pain patient. It may provide very useful information
Functional evaluation of lateral pterygoid muscle about the existence of bruxism or other oral habits and
(opening mouth against resistance) their possible effects on the dentition, periodontium or
other oral structures. Such an examination can also deter-
mine whether there has been a progressive change in the
occlusal relationship (midline shift, anterior open bite,
unilateral posterior open bite, etc.) that may indicate the
Sternocleidomastoid
presence of such conditions as unilateral condylar hyper-
Palpation is done bilaterally near its insertion on the outer plasia, rheumatoid arthritis, or neoplasm. Noting the num-
surface of the mastoid fossa behind the ear. The entire ber of missing teeth particularly loss of posterior occlusal
245
support is important since this situation may predispose DISORDERS ASSOCIATED WITH DEVIATION/
the TMJs to degenerative joint disease (osteoarthrosis) ALTERATION IN THE FORM OF ARTICULAR
especially in the presence of bruxism. SURFACES
Imaging Protocol Changes associated with the articular surfaces include those
of the mandibular condyle and glenoid fossa. The changes
Decision should be taken considering the following: that can be appreciated are condylar head flattening
❍ Clinical situation (Figure 9), flattening of the glenoid fossa or bony irregu-
❍ Cost larities over the condylar head.
❍ Radiation dose. Thinning of the articular disk borders and perforations
are common changes associated with change in form of
These depict the osseous structures of the joint with vary- the disk.
ing degrees of bony detail.
❍ Plain films, panoramic radiographs, conventional and Clinical features
computed tomography
Patient is usually asymptomatic. Over a period of time the
CT can be reserved for the evaluation of: patient is accustomed to a new pattern of mouth opening,
thereby avoiding pain during mandibular movements.
❍ Foreign body giant cell reaction to implants
Occasionally, a click may be evident during the opening
❍ Suspected tumors
and closing movements. What is interesting about the
❍ Ankylosis
clicks associated with condition is that the click is evident
❍ Complex facial fractures
at the same point both during opening and closing. Whereas
– MRI is indicated for soft tissues, including disk
in click associated with disk displacement, the opening
position and contour
click is usually evident after 20 mm of mouth opening and
– MRI when contraindicated, arthrography is recom-
the closing click is felt just short of occlusion of teeth.
mended.
This condition can be managed by instructing the
patient to develop a path of mandibular movement that
Disorders of TMJ avoids the interference and to chew on the affected side.
This will minimize the intra-articular pressure in the ipsi-
Dimitroulis in 1998 described ‘temporomandibular disor- lateral joint.
ders’ as a collective term used to describe a number of
related disorders involving the TMJ, masticatory muscles
and occlusion with common symptoms such as pain,
ARTICULAR DISK DEFECTS
restricted movement, muscle tenderness and intermittent
joint sounds.
The disorders of the TMJ may exhibit a wide variety Disk Thinning and Perforation
of symptoms and signs. The symptoms and signs that are It is believed that the disk wears out over a period of time.
frequently associated with temporomandibular disorders Hence, elderly individuals may generally present with
include: pain on mouth opening, limitation of mouth thinning of the disk which may ultimately perforate. The
opening, pain on chewing, joint noises (clicking and/or other causes include excessive occlusal loads from para-
popping, grating), pain in the region of the joint and/or functional habits such as bruxism, clenching and trauma.
muscles, pain around the region of the ear, temporal region The thinnest intermediate portion of the disk may show
and cheeks, subjective hearing loss, occlusal irregularities, a circular hole with irregular or fragmented border. A per-
attrition of teeth, headache (frontal, temporal, suboccipi- forated disk will expose the articular surface of the joint
tal), tinnitus, muscle hypertonicity and hypertrophy of jaw leading to degenerative changes.
muscles, neck pain and difficulty in swallowing.
TMJ disorders may arise from macro trauma such as
Clinical features
in road traffic accident (RTA), excessive mouth opening
(yawning, biting onto a large chunk of food) or from On auscultation of the TMJ, crepitus or grating noises may
repeated micro trauma such as in parafunctional habits be heard. In the early phases of the process pain may be a
(bruxism), uneven occlusal loading (malocclusion, high presenting complaint. Once the disk is perforated occlusion
points in restorations, poorly contoured crowns). Other may be altered when teeth are in maximum intercuspation.
causes include stress, underlying systemic diseases, arthri- Disk changes are readily evident on MRI and arthrography.
tis and developmental abnormalities. See Box 1 for clas- Degenerative changes can be appreciated on traditional
sification of TMJ disorders. imaging modalities and CT.
246
Box 1 Classification of temporomandibular disorders

Cholitgul et al (1990) evaluated 15 patients who
were reported to have disk perforation at arthrography.
I. Disorders of the TMJ Eleven patients reported pain. Clicking and crepitation
Deviation in form was common. Deviation of the mandible at maximum
Articular surface defects mouth opening toward the affected side was seen in nine
Disk thinning and perforation patients. The muscles of the affected side were tender on
Adherence and adhesions
palpation. The disk perforation was located in the poste-
Disk displacement
rior attachment in most joints. An anterior disk displace-
Disk displacement with reduction
Disk displacement without reduction
ment was found in almost all patients. They concluded
Displacement of disk–condyle complex that most joints with disk perforations were osteoarthrotic
Hypermobility and the most severe clinical and radiological findings
Dislocation are associated with an anterior disk displacement without
Inflammatory conditions reduction.
Capsulitis and synovitis
Retrodiscitis
Degenerative diseases Adherence and Adhesions
Osteoarthrosis
Osteoarthritis Adherence refers to a transient phase in which the condy-
Juvenile idiopathic arthritis lar head and the articular disk (inferior joint space) or the
Polyarthritides articular disk and the glenoid fossa (superior joint space)
Ankylosis may adhere together. However, prolonged periods of adhe-
II. Masticatory muscle disorders sion may result in a permanent state of adhesion (true
Acute conditions adhesions).
Reflex muscle splinting The causes for adhesion are long periods of static loading
Myositis of the joint (e.g. jaw clenching during sleep) and hemar-
Muscle spasm throsis caused by macro trauma or surgery.
Chronic conditions
Normally, when the joint is loaded, weeping lubrication
Myofascial pain
is exhausted and boundary lubrication takes over to prevent
Hypertrophy
Fibromyalgia
adhesions. But when the jaw is subjected to long periods
of static loading, the boundary lubrication is not sufficient
III. Congenital, developmental and acquired disorders of
to compensate for the exhaustion of weeping lubrication,
condylar process
resulting in adherence of the disk either with the upper or
Congenital and developmental disorders
Condylar hyperplasia
lower joint compartment.
Condylar hypoplasia
Aplasia Clinical features
Acquired disorders
Patients may complain of a stiff jaw, dull aching pain and
Neoplasms
limited mouth opening, especially if they habitually clench
Fractures
their teeth. However, the limitation in mouth opening
characteristically corrects following a ‘single click’ when
the patient makes attempts to open the mouth.
True adhesions may cause elongation of the collateral
Figure 9 discal ligaments and anterior capsular ligaments. Hence,
during translatory movements the condyle is ahead of the
articular disk thereby appearing that the disk is posteriorly
dislocated. It is thus hypothesized that posterior disk dis-
placements may result from disk adhesions.
Clinically, restriction of the condylar movements to
rotation alone, is typical of adhesions between disk and
superior joint space (mouth opening may be restricted to
about 25 mm). However, when the adhesion occurs between
the disk and the inferior joint compartment, rotational
movement is inhibited and the translatory cycle is normal
(patient can open the mouth to a normal interincisal dis-
Orthopantomograph (OPG) showing flattening of the
tance but experience a jerk or limitation when attempting
condylar head on the right side
to open the mouth to its full extent).
247
Disk Displacement front of the condyle, limiting the condylar translation in the
affected joint results in a ‘closed lock’ (Figure 11).
Disk displacements are also termed as internal derange-
ment. The internal derangements could include disk dis- Clinical features
placement with reduction and disk displacement without
reduction. It is generally a painful condition as the articular capsule,
Anterior disk displacement is common and it usually discal ligaments and posterior attachment are inflamed.
occurs when there is elongation of the disk attachment and Patient may present with pain and severe limitation in mouth
deformation or thinning of the posterior border of the disk, opening (maximum of 25–30 mm). Mandible is deflected
which in turn permits the articular disk to get displaced in to the ipsilateral side on mouth opening. There is limitation
an anterior direction on the surface of the condyle. In nor- in protrusive movements. Chronic cases may present with
mal conditions, when the teeth are in occlusion, the poste- joint crepitus. Lateral excursions are limited.
rior band of the disk ends at the apex of the condyle. In
anterior disk displacement, the posterior band of the artic-
Displacement of Disk–Condyle Complex
ular disk terminates ahead of the condylar apex. The most
common causes for internal derangement include trauma, (Hypermobility and Dislocation)
clenching and biting on hard substances. Occasionally during the terminal phases of the translatory
cycle, as the condyle moves past the articular eminence
it may suddenly move forward to facilitate a wide mouth
Disk Displacement with Reduction opening referred to as subluxation (hypermobility, partial
It is characterized by an anterior or anteromedial displace- dislocation).
ment of the disk upon mouth opening. However, on clos- Hypermobility may occur due to joint laxity seen as a
ing the mouth the disk returns to a more normal position genetic predisposition (Ehlers–Danlos syndrome), following
relative to the condyle on opening (Figure 10). dental procedures that require prolonged mouth opening
(endodontic procedures, third molar extraction), excessive
Clinical features yawning and during endotracheal incubation for general
anesthesia.
Clicking sound may be heard during mandibular opening
and closing. The opening click may be heard during any Clinical features
phase of the translatory cycle and the closing click may be
felt as the disk again becomes displaced. Mandible may be Many patients describe the sudden forward movement as
deviated to the affected side. Muscle splinting may result a feeling of a ‘thud’ sound. This condition is usually pain-
in joint tenderness and limitation of mouth opening. less unless it becomes chronic. Patients may exhibit a tapered/
elongated face. Hypermobility may be distinguished from
anterior disk displacements in which the click is associated
Disk Displacement without Reduction only with wide opening and absence of closing click.
In this condition the condylar head is unable to pass under
the displaced disk. The reasons for the condyle to be trapped
Dislocation (Open Lock)
include: thickening of posterior band, change in shape of
disk from biconcave to biconvex and decrease in tension Dislocation of the condyle is a common condition that
in the posterior attachment. Such a trapping of the disk in may occur in an acute or chronic form. It is characterized
Figure 10
Resting phase Early translatory phase
Late translatory phase
Open click
Phases of anterior disk displacement with reduction
248
by inability to close the mouth with or without pain. Lateral dislocation has been described by Allen and
Dislocation has to be differentiated from subluxation which Young in 1969 in two subgroups: type 1 is the lateral sub-
is a self-reducible condition. When the mouth is opened, luxation and type 2 is a complete dislocation where the
the head of the condyle should not pass beyond the apex condyle if forced laterally and superiorly to the temporal
of articular eminence. In case of laxity of capsular struc- fossa. It is accompanied by the fracture of body of mandible
tures, a wide open position allows the condyle to move pass at symphysis.
the articular eminence which cannot be reduced by the Superior dislocation as described by Zecha in 1977 is
patient. Dislocation can occur in any direction with ante- the dislocation of condyle in the middle cranial fossa and
rior dislocation being the most common one. Various pre- associated with fracture of glenoid fossa. It is said to be
disposing factors have been associated with dislocation most probably due to the small rounded shape of the con-
like muscle fatigue and spasm, the defect in the bony sur- dyle which fails to impinge in the margins which is stronger
face like shallow articular eminence, and laxity of the cap- than the central area.
sular ligament. People with defect in collagen synthesis like
Ehlers–Danlos syndrome, Marfan syndrome are said to be
genetically predisposed to this condition.
INFLAMMATORY JOINT DISORDERS
Clinical features
Synovitis or Capsulitis
The condition is characterized by inability to close the mouth
Synovitis and capsulitis are clinically considered as a sin-
after wide opening. Bilateral dislocation is more common
gle disorder. Synovitis refers to inflammation of the syno-
than unilateral dislocation. However, when the dislocation
vial tissues and inflammation of the capsular ligaments is
is unilateral, the chin is deviated to the contralateral side.
referred to as capsulitis. Various causes have been attrib-
Palpation in the preauricular region reveals an empty joint
uted to these inflammatory conditions such as trauma,
fossa and may reveal the condyle anterior to the joint. The
opening the mouth excessively, chronic condylar displace-
inability to close the mouth is due to the spasm of the
ment in a posterior direction and sometimes from a direct
masticatory muscles. A typical facial expression (elongated
spread of inflammatory products from the surrounding
face) is due to anxiety related to the thought of not being
structures.
able to close the mouth (Figures 12A, B and 13A, B).
Clinical features
Types of dislocation
Continuous pain that exacerbates during function is charac-
Depending upon the position the condyle occupies, Heslop teristic of this condition. Limitation in jaw movements is
in 1956, described the anterior dislocation in which the another common finding. In some individuals malocclusion
condyle moves anterior to the articulating eminence. It is in the posterior teeth is seen due to inferior displacement
one of the most common type of dislocation. It represents of the condyle resulting from the edema.
the pathological forward extension of normal translatory
movement of head of condyle. The anterolateral variant
was described by Morris and Hutton in 1957.
Retrodiscitis
Helmy in 1957 described the posterior variant in which the
head of condyle is displaced posterior to its usual position. Inflammation of the retrodiscal tissues (retrodiscitis) results
It is usually associated with a fracture of base of skull or from a traumatic injury which may indirectly cause the
the anterior wall of bony meatus. condylar head to impinge on the retrodiscal tissues.
Figure 11
Resting phase Early translatory phase Late translatory
Phases of anterior disk displacement without reduction
249
Figure 12
A B
(A) ‘Elongated face’ in bilateral condylar dislocation. (B) OPG showing bilateral condylar dislocation.
Courtesy: Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore
Figure 13
A B
(A) Normal facial appearance following reduction of the dislocated condyles. (B) Normal positions of the condyle in the glenoid
fossa after reduction. Courtesy: Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore
Chronic disk displacement and dislocation may also result However, patient should be instructed to chew on the
in insults to the retrodiscal tissues. affected side as this will relieve occlusal load on the side
affected thereby hastening the healing process.
Clinical features
Inflammation of the retrodiscal tissues may lead to a for- DEGENERATIVE JOINT DISEASES
ward and downward placement of the condyle thereby
producing a same-sided malocclusion of posterior teeth
Osteoarthrosis
and heavy contact in the anterior teeth of the opposite
side. Continuous pain is felt in the TMJ region, which Osteoarthrosis is a non-inflammatory degenerative disorder
exacerbates on clenching. of a joint affecting articular tissues and subchondral bone.
250
Figure 14
Ely cyst in the mandibular condyle. Courtesy: Department of Oral Medicine and Radiology,
It is believed that excessive load on the TMJ leads to In the early stages of osteoarthritis patient may complain
degeneration of the fibrous articular tissue covering the of pain that exacerbates after function and relieved by rest.
condyle. Osteoarthrosis occurs secondary to displacement However, as the condition worsens pain may be present
of the disk. even at rest. TMJs are usually stiff in the early morning
hours and cold climatic conditions may exacerbate the pain.
Clinical and radiographic features On clinical examination, the TMJ may exhibit very lim-
ited range of movement. Mouth opening will result in
Patients will exhibit restricted movement of the mandible.
deviation of the mandible to the same side. Some patients
Mouth opening may be associated with deflection toward
may present with an anterior open bite. Crepitus, myositis
the affected side. Auscultation may reveal crepitus during
and masticatory muscle spasm are other characteristic fea-
opening and closing the mouth. The condition is painless.
tures of osteoarthritis.
Long-standing osteoarthrosis leads to the formation of
multiple cystic areas in the medullary region of the condyle
which in turn may collapse to form erosive areas altering
the condylar morphology. Some authors suggest that osteo- Various radiographic findings may be seen based on the
arthrosis is a ‘burned out’ or resolved phase of osteoarthritis. extent and severity of the degeneration and the simulta-
neous remodeling. Radiographs may reveal a reduction in
Management the size of the joint compartments or a total lack of space,
flattened condylar head, erosion on the articulating sur-
Occlusal irregularities, if any, may be corrected to prevent
face, subchondral sclerosis and osteophytes.
TMJ overloading.
These osteophytes may eventually break off to lie on the
joint space and may be detected on MRI and arthrography
Osteoarthritis as joint mice or loose bodies. Another characteristic finding
is the presence of subchondral bone cysts (Ely cyst). These
Osteoarthritis is characterized by pain secondary to TMJ pseudo cysts (Figure 14) are areas of degeneration, con-
synovial inflammation. It is usually seen in elderly and taining fibrous tissue, granulation tissue and osteoid. In
relatively more frequently in women. severe cases, as the posterior slope of the articular emi-
nence erodes, the glenoid fossa enlarges in size.
Clinical features
Almost all the patients report of a gradual onset of symp-
Juvenile Idiopathic Arthritis
toms. The condition is self-limiting and even in the absence
of active treatment the symptoms subside over a period of The juvenile idiopathic form of arthritis is a chronic, inflam-
time, and the TMJ movements revert back to an acceptable matory, systemic disease typically beginning before 16 years
level. of age. It may affect one or more joints of the body.
251

The condition is characterized by peripheral arthritis. An
immunoinflammatory pathogenesis is considered as the
etiology. Based on the clinical expression at the onset
and the first 6 months of the disease, three types have been
described: oligoarticular (four or fewer involved joints),
polyarticular (five or more involved joints), and systemic
(presence of arthritis and severe systemic involvement).
Patients present with chronic synovitis, arthralgia,
and impaired joint mobility. Chronic inflammation in the
joint results in degenerative changes thereby causing pain,
joint sounds, and limited movement. Alterations in facial
growth, such as micrognathia, retrognathia, facial asym-
metry, and anterior open bite, also occur due to condylar
involvement. Other extra-articular manifestations of dis-
ease include fever, rheumatoid rash, cardiac disease and
chronic uveitis.
Polyarthritides Limitation in mouth opening and deviation of the

jaw in septic arthritis. Courtesy: Department of Oral
Polyarthritides represent a group of disorders characterized Medicine and Radiology, Manipal College of
by inflammation of the articular surfaces of the joint. It Dental Sciences, Mangalore
resembles osteoarthritis as it exhibits degenerative changes
in the articular cartilage and underlying bone along with
inflammation of the capsule and synovial tissues.
Clinically, tenderness may be elicited on TMJ palpation. Swelling, tenderness and rise in local temperature are char-
The TMJ area may exhibit swelling and erythema. The acteristic of septic arthritis. Limitation in mouth opening
patients may have limited function. Crepitus is a character- and deviation of the jaw to the affected side are other find-
istic finding. Patient’s symptoms may aggravate with para- ings (Figure 15). Affected side may reveal tender cervical
functional habits. Radiographs reveal surface changes in lymphadenopathy.
the glenoid fossa and flattening of the articular eminence. Synovial fluid and blood studies will aid in the diagno-
sis. The commonly isolated organisms from a previously
normal TMJ are gonococcal species and from TMJ with
Traumatic Arthritis previous history of arthritis, Staphylococcus aureus has
been isolated. Untreated cases of septic arthritis can lead
Occasionally a major traumatic episode can result in inflam- to brain abscess, ankylosis and osteomyelitis of temporal
matory changes that ultimately lead to articular surface bone. Ankylosis and facial asymmetry may be a common
changes. Patients may complain of restricted mouth open- complication in children.
ing and pain. On palpation a soft end feel is typically
evident.
Rheumatoid Arthritis
Rheumatoid arthritis (RA) is a chronic inflammatory, auto-
Infectious Arthritis/Septic Arthritis
immune disorder, which may involve many of the diar-
This form of arthritis is generally seen in patients with throdial joints (usually in a symmetrical fashion) in the
previously existing joint disease or with underlying sys- body characterized by persistent synovitis. Women are three
temic illness. The highest incidence of septic arthritis is to four times more affected than men. The age of onset
seen in individuals on long-term immunosuppressive drugs varies between 25 and 55 years.
or corticosteroids. Though the exact nature of RA is still unknown, it is
Otherwise sterile articular surfaces and joint spaces believed that the inflammation of the synovial membranes
may become infected secondary to blood borne bacterial extends into the surrounding connective tissues and artic-
infection or extension of infection from adjacent sites such ular surfaces (this reactive macrophage laden fibroblastic
as the molar teeth, middle ear and parotid gland. proliferation from the synovium that extends to the joint
Clinically patient gives a history of constant pain in surface is called pannus), which then become thickened
the TMJ region which typically aggravates with function. and tender. The cells of the synovial membrane express
252
enzymes that cause destruction of the articular surface,

Figure 16
eventually leading to a fibrous ankylosis.
Clinical features
TMJ afflicted with RA may produce pain, joint stiffness,
limited mouth opening, joint sounds and open bite. The
reported prevalence of TMJ involvement by RA varies
widely from 4.7 to 88%.
The American Rheumatism Association (1987) laid down
guidelines for diagnosis of RA. According to these guide-
lines, the patient must present three or four of the follow-
ing symptoms for more than 6 months: morning stiffness
for more than 1 hour; arthritis in three or more joints; arthri-
tis in the hands; symmetrical arthritis; rheumatoid nodes;
presence of rheumatoid factor; radiographic alterations. Pencil-shaped condyle on the right side and condylar head
flattening of the left side. Courtesy: Department of
Laboratory investigations Oral Medicine and Radiology, Manipal College of
Dental Sciences, Mangalore
The level of rheumatoid factor, erythrocyte sedimentation
rate, C-reactive protein, thrombocyte count and plasma
tumor necrosis factor-␣ will aid in the diagnosis. Clinical features
Radiographic features Unlike rheumatoid arthritis, the TMJ symptoms associated
with PA are unilateral. Patients present with pain over the
The characteristic radiographic findings include general- TMJ, limited mandibular movement and deviation of the
ized decreased density of bone, severe erosion of the con- mandible to the affected side. Radiographically, the TMJ
dylar head (occasionally only the neck of the condyle may changes are generally similar to those that are seen in
be remaining), subchondral sclerosis, flattening of the con- rheumatoid arthritis, however in some patients extensive
dylar head, subchondral cysts and osteophyte formation. sclerotic changes may be evident.
In some individuals, the condyle may assume a sharpened
pencil shape owing to the erosion of the anterior and pos-
terior condylar surfaces (Figure 16). Hyperuricemia
A modified grading system for the evaluation of TMJ
abnormalities based on the degree of bony destruction of Gout is considered a true crystal deposition disease. Gout
the mandibular condyle can be summarized as: may be described as a pathological response of the periar-
ticular tissues to the presence of monosodium urate mono-
❍ Grade 0 (normal): well-defined cortical outline of the hydrate crystals. Though a chronic state of hyperuricemia
condyle is required for the development of gout, it alone is not suf-
❍ Grade I (mild): presence of cortical destruction and ficient to predispose to gout. It is estimated that 95%
irregular margin of the condyle of hyperuricemic individuals may not develop gout. Gout
❍ Grade II (moderate): bony destruction or erosion of commonly affects the first metatarsophalangeal joint (50%)
the condyle or evident flattening, with deviation from and other joints such as the ankle, knee, wrist, elbow and
normal joint morphology the TMJ.
❍ Grade III (severe): complete or almost complete destruc-
tion of the condyle. Clinical features
Crystal deposition may be seen in tissues adjacent to the TMJ.
Psoriatic Arthritis Occasionally, monosodium urate crystals may be evident
Psoriatic arthritis (PA) is present in about 5–7% of the in the synovial fluid aspirate.
patients suffering from psoriasis. The diagnosis can be
made when patients present with erosive polyarthritis with
negative rheumatoid factor and psoriatic skin lesions are TMJ ANKYLOSIS
seen long before TMJ is affected. The skin lesions are seen
long. This condition affects the fingers and spine along Ankylosis is an intra-articular condition where there is
with the TMJ. Eighty-five percent of the patients present fusion between the bony surfaces of the joint, the condyle
with pitting of the nails. and the glenoid fossa. The term ‘ankylosis’ is derived from
253
a Greek word which means stiffening of a joint as a result Local infections Otitis media, mastoiditis, osteomyelitis
of a disease process. Ankylosis of the mandible with immo- of temporal bone, parotid abscess, infratemporal or sub-
bility of the joint may be of an osseous, fibro-osseous, or masseteric space or parapharyngeal infections, furuncle,
cartilaginous variety. actinomycosis (the source of infection is contiguous, from
Ankylosis must be distinguished from its counterpart adjacent structures).
pseudoankylosis. Hypomobility of the joint due to coronoid
Systemic conditions Tuberculosis, meningitis, pharyn-
hyperplasia or due to fibrous adhesions between the coro-
gitis, tonsillitis, rubella, varicella, scarlet fever, gonococcal
noid and tuberosity of the maxilla or zygoma (e.g. as in
arthritis, ankylosing spondylitis (the route of spread is
‘V-shaped’ fracture of the zygomatic arch impinging on
hematogeneous).
coronoid leading to fibrous or bony union) are also exam-
The local and systemic infections may pass along as
ples of pseudoankylosis. Even though jaw movement is
septic arthritis which may not always cause ankylosis.
restricted as in bony ankylosis, the pathology is extra-
Staphylococcus species, Streptococcus species, Hemophilus
articular in these cases.
influenzae, Neisseria gonorrhoeae are the most likely
causes of septic arthritis. The infection may take either the
Etiopathology
hematogeneous, contiguous, or by direct inoculation. The
Trauma Most cases of ankylosis result from condylar synovium with its high vascularity and lack of a limiting
injuries sustained before 10 years of age. A unique pattern basement membrane is vulnerable to infection.
of condylar fractures is seen in children. Condylar cortical
Arthritis/inflammatory conditions About 50% cases in
bone in children is thin with a broad condylar neck and
juvenile RA (Still’s disease) also have TMJ involvement
rich subarticular interconnecting vascular plexus. An intra-
along with polyarthritis. Osteoarthritis may also lead to
capsular fracture leads comminution and hemarthrosis of
ankylosis.
the condylar head. This sort of intracapsular burst fracture
is called ‘mushroom fracture’. This results in the organi- Neoplasms Sarcoma, osteoma and condroma may also
zation of a fibro-osseous mass in a highly osteogenic envi- result in ankylosed joint. The pathogenesis of ankylosis is
ronment. Moreover, immobility leads to ossification and generally the same in all the non-traumatic conditions—
consolidation of the mass, resulting in ankylosis. Anky- degenerative, destructive, and inflammatory changes in
losis may also occur in trauma sustained during forceps disk followed by repair leading to ankylosis. Destruction
delivery. of the disk leads to bony contact between the condyle and
Laskin (1978) had outlined various factors that may be glenoid fossa.
implicated in the etiology of ankylosis following trauma:
❍ Age of patient: Younger patients have significantly Classification of ankylosis according to tissues
higher osteogenic potential and rapidity of repair. involved and extent
Moreover, the articular capsule is not as well developed ❍ ‘True’ ankylosis or pseudoankylosis
in younger patients, thus permitting easier condylar ❍ Extra-articular or intra-articular
displacement out of the fossa and thereby damage to ❍ Fibrous, bony, or fibro-osseous
the disk. Finally, there is a greater tendency for pro- ❍ Unilateral or bilateral
longed self-imposed immobilization of the mandible ❍ Partial or complete.
post-traumatically in children.
❍ Type of fracture: The condyle in children has a thinner
Classification of ankylosis by Topazian (1966)
cortex along with a thick neck, which predisposes them
to a higher proportion of intracapsular comminuted ❍ Type I: Fibrous adhesions in or around the joint-
fracture. In contrast, adults have a thinner condylar restricted condylar gliding.
neck which usually fractures at the neck, thus sparing ❍ Type II: Formation of a bony bridge between the
the head of the condyle within the capsule. condyle and glenoid fossa.
❍ Damage to articular disk: The direct contact between a ❍ Type III: Condylar neck is ankylosed to the fossa
comminuted condyle and the glenoid fossa either from completely.
a displaced or torn meniscus is the key factor in the
development of ankylosis. Grading of TMJ ankylosis
❍ Period of immobilization: Prolonged mechanical immo-
Sawhney (1986) graded TMJ ankylosis into four types:
bilization or muscle splinting can promote osteogen-
esis and consolidation to set in an injured condyle. ❍ Type I: Flattening or deformity of condyle with little
Total immobility between articular surfaces after con- joint space on radiograph. There is minimal bony
dylar injury leads to a bony type of fusion, whereas fusion, but extensive fibrous adhesions around joint.
some movement leads to a fibrous type of union. Some movement is possible.
254
❍ Type II: Bony fusion on the outer edge of articular sur-

Figure 17
face, but no fusion on the deeper aspect of the joint.
❍ Type III: A bridge of bone exists between the ramus
and zygomatic arch. The upper articular surface and
the articular disk on the deeper aspect are still intact.
Medially, a displaced atrophic condyle still exists and
which is functional. Type III ankylosis results from a
fracture-displaced condyle, compared to the crushing
types of condylar injuries as in types I and II.
❍ Type IV: Total TMJ obliteration between ramus and
skull by large bony mass. It is the most common type.
Clinical features
The clinical features of ankylosis depends on:
❍ Type of ankylosis: Unilateral versus bilateral, bony
versus fibrous, extent of joint involvement.
❍ Age of onset and duration of ankylosis: The deformity
will be severe if it occurs before the age of 5 years.
Clinical features of unilateral ankylosis

Facial features Bird face deformity. Courtesy: Department of Oral Medicine
and Radiology, Manipal College of Dental Sciences, Mangalore
❍ Obvious facial asymmetry.
❍ Chin receded with hypoplastic mandible on affected
side, resulting in deviation of chin and mandible ❍ Relatively short hyomental distance with tight suprahy-
toward affected side. oid musculature.
❍ Unilateral vertical deficiency on the affected side. ❍ Cervicomental angle may be reduced or completely
❍ Roundness/fullness on affected side; foreshortened absent.
mandible, flatness and elongation on normal side as it ❍ Obstructive sleep apnea may be present due to oro-
grows toward the affected side. pharyngeal airway narrowing in cephalocaudal,
❍ Loss of the normal bilateral symmetrical divergence anteroposterior, and transverse directions.
from the mental region toward the angle.
Intraoral features
❍ The lower border of the mandible on the affected side
has a concavity that ends in a well-defined antegonial ❍ Mouth opening would be less than 5 mm (Figure 18) or
notch. may be nil at times.
❍ Markedly elongated coronoid process. ❍ Generally a class II malocclusion, although class I
occlusion may also be seen.
Intraoral features
❍ Incompetent lips and proclined lower anteriors.
❍ Occlusal cant with deviation of maxillary and man- ❍ Open bite with protrusion of both upper and lower
dibular midlines toward affected side. anteriors resulting from the protrusive action of
❍ Class II Angle malocclusion present on the affected tongue because of decreased tongue space.
side with unilateral crossbite on the opposite side. ❍ Severe crowding, multiple impacted teeth with oral
❍ The mouth opening is restricted: amount of opening health maintenance problems, leading to caries and
depends upon degree of ankylosis. periodontal problems.
The key clinical difference between intra-articular and
Clinical features of bilateral ankylosis
extra-articular ankylosis is the ability or limited ability to
Facial features translate the mandible. If the ankylosis has an extra-
articular origin, translation and anteroposterior movement
❍ Symmetrical defect.
may not be as limited as rotational movement.
❍ Retrognathic mandible with a short ramus and a small
body.
❍ Often microgenia, small chin.
❍ ‘Bird-face deformity’ (Figure 17) or ‘Andy Gump’ facies. In fibrous ankylosis, joint may appear normal or the
❍ Convex profile. articulating surfaces may be irregular. The joint space is
255
Figure 18 Management
The goals of management should include restoration of
mouth opening and joint function, facilitation of condylar
growth, correction of facial profile and to relieve upper
airway obstruction.
Surgical correction of ankylosis is best achieved by
condylectomy, gap arthroplasty, coronoidectomy, interpo-
sitional arthroplasty (with autogenous or alloplastic grafts)
and secondary procedures such as orthognathic surgery
and distraction osteogenesis. Surgical correction should be
followed by active physiotherapy.
When ankylosis is left untreated it may result in abnor-
mal facial growth and development, speech defects, nutri-
tional impairment, respiratory distress syndrome, conditions
related to poor oral hygiene and psychological impact on
the patient.
Restricted mouth opening. Courtesy: Department of

Oral Medicine and Radiology, Manipal College of MASTICATORY MUSCLE DISORDERS
Acute Disorders
Reflex Muscle Splinting
Figure 19 Muscle splinting or protective co-contraction is a CNS

response to actual tissue injury/a threat of injury. Like the
name suggests, in the event of an injury or threat of
potential injury the sequence of muscle activity is modi-
fied such that the tissue is protected from any further
insult. High points in a newly fabricated crown, biting on
hard food substance, etc. may lead to a reflex muscle
splinting.
Clinical features
Patient may report of muscle weakness following the tis-
sue injury. Pain is experienced only during function and is
not evident at rest. Limited mouth opening which can
improve when the patient attempts to open the mouth
gently is another characteristic feature. The affected mus-
cle may feel tight/stretched on palpation.
Management and prognosis

Orthopantomograph showing bony ankylosis in the right side Usually removal of the cause and resting the affected mus-
and elongated coronoid process. Courtesy: Department cle is sufficient. Moist heat fomentation will aid in hasten-
of Oral Medicine and Radiology, Manipal College ing the recovery process. Patient should be advised not to
of Dental Sciences, Mangalore overuse the affected the muscle. Muscle relaxants can be
used for a short period of time.
markedly decreased. In bony ankylosis, the joint space

Myositis
may be obliterated, completely or partly by an osseous
bridge (Figure 19). Other features include deepening of the Inflammation of the muscle that results from a local cause
antegonial notch and compensatory elongation of the cor- is referred to as myositis. The causes of myositis include
onoid process on the affected side. traumatic injuries, muscular strain and orodental infections.
256
However, myositis usually arises out of a long standing, severe in spasm, with increased tension and decreased flexibility.
and neglected muscle splinting and myospasm. It usually presents with regional muscle pain distributed in
one or two quadrants of the body. Taut bands are groups
Clinical features of muscle fibers that are hard and tender on palpation.
Tender spots are specific sites of localized pain.
The involved muscle is painful and usually associated with
a swelling. Like all muscular disorders, the pain exacer-
Predisposing factors
bates when the muscle is under function. Muscular dys-
function occurs secondary to muscle pain and inflammatory Acute muscular injury sustained as a result of macro
exudate as opposed to muscle contraction. Untreated trauma or sudden wide mouth opening or a sustained state
myositis results in a myofibrotic contracture. of muscular contraction such as injuries due to bad pos-
ture, muscular tension and clenching/bruxism can predis-
pose to MFP.
Muscle Spasm (Myospasm)
Myospasms of the muscles of mastication are uncommon. Pathophysiology
It is a CNS-induced tonic muscle contraction. In this con- Precipitating factors of MFP may cause the facilitated
dition, all the motor units of the affected muscle contract release of acetylcholine at motor end plates, sustained
resulting in shortening of the muscle length resulting muscle fiber contractions and local ischemia with release
in an acute spasm. Certain local muscle conditions have of vascular and neuroactive substances, and muscle pain.
known to predispose to myospasms such as muscle fatigue, More acetylcholine may then be released, thus perpetuat-
alteration in the local electrolyte balances and deep pain. ing the muscle pain and spasm. Local muscle fibrosis may
Muscles that have already been overused or those that have occur after a prolonged period of time.
been weakened because of protective splinting more likely
undergo a spasm. Certain tranquilizers may also cause mas- Clinical features
ticatory muscle spasm.
Laskin included the following signs and symptoms for the
Clinical features diagnosis of MFP:
Patient usually presents with limited mouth opening. Pain is ❍ Unilateral, dull pain in the ear or preauricular region,
usually dull and continuous with occasional periods of acute that is worse on awakening
pain. The pain may be referred to the face, temple region ❍ Tenderness in muscle of mastication on palpation
and the ear. Malocclusion is another characteristic feature. (localized TrP)
Analgesics can be prescribed. Moist heat fomentation ❍ Limitation/deviation of mandible on opening.
will help in relaxing the muscle. Local anesthetic without A more comprehensive description of the clinical features
vasoconstrictor can be injected into the affected muscle are described below.
to relieve pain and facilitate speedy recovery. Occlusal
bite guards may be given to counteract the effects of para- Primary finding
functional habits if any.
❍ Patient complains of pain in one or more masticatory
muscle
Chronic Conditions ❍ Tenderness or pain upon manipulation/palpation of
masticatory muscle with replication of chief complaint
Myofascial Pain ❍ Tender site pain can be referred to other orofacial
Costen (1934) first described a symptom complex, ‘TMJ areas (TrP)
pain dysfunction syndrome’, which included facial and ❍ Pain aggravated by mandibular function
head pain and TMJ dysfunction. Laskin (1969) was the ❍ Acute malocclusion.
first to coin the term ‘myofascial pain dysfunction syn-
Secondary findings
drome’. Myofascial pain (MFP) can arise in any skeletal
muscle such as those in the head (commonly the muscles of ❍ Restricted range of motion (ROM)
mastication), neck, lower back and shoulders. ❍ Maximum assisted opening ⬎ maximum unassisted
Traditionally, MFP is believed to arise from TrP (trigger opening ⬎ pain free opening
point) in a muscle. TrPs are minute sensitive areas in a ❍ ROM increases by use of vapocoolants
muscle that spontaneously or upon compression cause ❍ Limited function
pain to a distant region, known as the referred pain zone. ❍ Clinical or behavioral indications of hyperfunction/
In recent times, MFP includes muscle pain from ‘taut parafunction
bands’ (TB) with TrP or ‘tender spots’ (TS). The muscles are ❍ Acute malocclusion.
257
Possible findings muscle in spasm. Alternatively, 0.5% procaine without

epinephrine can be injected into the trigger point.
❍ TMJ pain
❍ Joint sounds Medication NSAIDs and skeletal muscle relaxants (chlo-
❍ Inflammation rzoxazone, methocarbamol) can be used. Patients who are
❍ Restricted range of motion unmodified by assisted anxious can be prescribed 2 mg of diazepam three times
opening/vapocoolant a day and 5 mg at bed time for a 2-week period. This will
❍ Hypertrophy help in controlling the anxiety and relaxing the muscles.
❍ Myalgia secondary to systemic disease. Such individuals should be counseled not to drive and to
Other reported symptoms include: musculoskeletal symp- avoid using heavy machinery.
toms (fatigue, stiff joints and swelling), neurological symp-
Occlusal splint therapy The occlusal splint helps in
toms (tingling, numbness, blurred vision, muscle twitching
maintaining a harmonious relation between the TMJ and
and excessive lacrimation), and otologic symptoms (ear
muscles. It also aids in re-establishing a coordinated pat-
pain, tinnitus, diminished hearing, dizziness and vertigo).
tern of activity in the absence of tooth influence. The most
Other symptoms that patients report of include: dentinal
important function of the splint is that it helps in the reso-
hypersensitivity and cutaneous hypersensitivity.
lution of micro trauma to muscle.
Referral pattern for myofascial pain Other treatment modalities include isokinetic jaw exer-
cises, biofeedback, acupuncture and hypnotherapy.
1. Medial pterygoid muscle: Pain referred to posterior
part of mouth and throat, TMJ and infra-auricular area. Transcutaneous electric nerve stimulation (TENS) The
2. Lateral pterygoid muscle: Inferior head refers pain to TENS temporarily activates afferent nerves, thereby mod-
the TMJ area and the superior head refers to the zygo- ulating pain. The electrical impulses are produced in a
matic area. handheld battery-operated device. TENS has been proven
3. Masseter muscle: Pain is referred to the posterior man- to be useful in controlling masticatory muscle and neuro-
dibular and maxillary teeth, ear and TMJ. genic pains. The impulses generated have a duration of
4. Temporalis muscle: Pain is referred to all maxillary 2 millisecond with an interval of 0.5–1.5 second. The oper-
teeth and upper portion of face. ating voltage is about 4 volts. TENS is believed to have
physiological (rhythmic contractions of muscles increase
Management blood supply), neurological (electrical stimulation inhibits
The management of MFP requires a multi-pronged pain conduction), pharmacological (releases endorphins)
approach. In the short term, the aim is to abolish the TB, and psychological (placebo) effects.
TrP and TS for pain relief. In the long-term, achieving
muscle flexibility and eliminating associated precipitating
factors is the objective. Muscular Hypertrophy
Counseling Patient should be counseled about the nature Enlargement or hypertrophy of the masticatory muscles is
of the condition and encouraged to discontinue parafunc- a relatively rare condition. The hypertrophy is clinically
tional habits and consume soft diet. evident as a localized firm non-tender swelling along the
body of the affected muscle.
Physiotherapy Physical modalities are useful supplemen-
Legg was the first to describe bilateral enlargement of
tary treatments that help in controlling muscle pain and
the masseter and temporalis muscles. However, literature
spasm. Moist heat application to the affected site increases
review reveals the description of muscular hypertrophy of
blood flow and increases vascularity, resolution of inflam-
the masseter, temporalis and the pterygoid.
mation and fibrosis. It also increases flexibility of connec-
Masticatory muscle enlargement can be either congenital
tive tissue and decreases muscle spasm and pain. However
or acquired. Usually, the acquired form is a result of para-
moist heat fomentation provides superficial heat with limited
functional jaw habits such as bruxism or masticatory
subcutaneous penetration. Ultrasound, on the other hand,
hyperfunction (chewing hard food, chewing gum). Some
provides deep heat with higher subcutaneous penetration.
authors believe that emotional stress may result in chronic
Trigger point therapy Spray and stretch technique and forceful clenching of the jaws and bruxism, resulting in
local injections are used. hypertrophy of the muscle (Figure 20).
Refrigerant spray such as ethyl chloride or fluoromethane Muscular hypertrophy should be differentiated from con-
can be sprayed onto the skin surface from a distance of ditions such as lipomatosis, vascular tumors, liposarcoma,
about 18 inches at an angle of 30⬚. The skin surface should rhabdomyosarcoma and infiltrative leukemia and lymphoma.
be stretched manually during the spray. The spray anes- MRI and ultrasound will help in revealing the homogeneous
thetizes the site and facilitates the patient to stretch the enlargement of the muscles.
258
Figure 20 However, one should be careful to distinguish tender

points associated with myofascial pain from those seen in
fibromyalgia. Only tender points associated with myofascial
pain refer pain, produce a ‘jump response’ on palpation
and cause central excitatory response.
Clinical features
Fibromyalgia is more common in women compared to men
(9:1). The peak onset of disease is between 45 and 60 years
of age. Patients have also reported of paresthesia, symp-
toms of irritable bowel syndrome, dysmenorrhea and
Raynaud’s phenomenon.
Official diagnostic criteria for fibromyalgia

The diagnostic criteria were developed by the American
College of Rheumatology in 1990.
Masseteric hypertrophy. Courtesy: Department of
Oral Medicine and Radiology, Manipal College of ❍ History of widespread pain: Pain is considered
Dental Sciences, Mangalore widespread when all of the following are present: pain
in the left side of the body, pain in the right side of the
body, pain above the waist, and pain below the waist.
In addition, axial skeletal pain (cervical spine or ante-
Orthopantomographs may reveal enlarged ramal region rior chest or thoracic spine or low back) must be pres-
and a prominent antigonial notch in masseteric hypertro- ent. In this definition, shoulder and buttock pain is
phy. Anteroposterior radiographs show bone spurs at the considered as pain for each involved side. Low back
mandible angle (it is estimated that 20% of normal popu- pain is considered lower segment pain.
lation may also show bone spurs). Guggenheim and Cohen ❍ Pain in 11 of 18 tender point sites on digital palpation:
reported that bone spurs are caused by periosteal irritation Digital palpation should be performed with an approxi-
and new bone deposition responding to increased forces mate force of 4 kg/cm2. For a tender point to be consid-
exerted by the muscle bundles. ered ‘positive’, the subject must state that the palpation
was painful. ‘Tender’ is not to be considered ‘painful’.
Management
In normal circumstances muscular hypertrophy needs no Note: For classification purposes, patients will be said to
specific treatment. However, when cosmetic compulsions have fibromyalgia if both criteria are satisfied. Widespread
are paramount, various non-surgical and surgical treat- pain must have been present for at least 3 months. The
ment modalities may be considered. presence of a second clinical disorder does not exclude
It has been suggested that cessation of the parafunc- the diagnosis of fibromyalgia.
tional habit such as bruxism may result in muscular atrophy.
Premature contacts, poorly contoured restorations and mal- The 18 tender points are depicted in Figure 21:
occlusions should be corrected. 1, 2 (Occiput): bilateral, at the suboccipital muscle insertions.
Minute doses of botulinum toxin type A may be injected 3, 4 (Low cervical): bilateral, at the anterior aspects of the
into the affected muscle. Surgical options include partial intertransverse spaces at C5–C7.
or complete resection of the enlarged muscle and reduc- 5, 6 (Trapezius): bilateral, at the midpoint of the upper
tion of bone prominence from the mandibular angle. border.
7, 8 (Supraspinatus): bilateral, at origins, above the scap-
ula spine near the medial border.
Fibromyalgia
9, 10 (Second rib): bilateral, at the second costochondral
Fibromyalgia is a syndrome characterized by chronic wide- junctions, just lateral to the junctions on upper surfaces.
spread musculoskeletal pain, stiffness, non-restorative sleep, 11, 12 (Lateral epicondyle): bilateral, 2 cm distal to the
and fatigue. Occasionally, many of the patients may mimic epicondyles.
patients of chronic fatigue syndrome. The characteristic fea- 13, 14 (Gluteal): bilateral, in upper outer quadrants of
ture of fibromyalgia (FM) is the presence of non-inflamma- buttocks in anterior fold of muscle.
tory muscle and connective tissue ‘tender’ points that are 15, 16 (Greater trochanter): bilateral, posterior to the tro-
widespread, involving all four quadrants of the body. chanteric prominence.
259
Figure 21 Figure 22
Back Front
Occiput
Trapezius Lower cervical
Supra- 2nd rib

spinatus
Lateral
Gluteal epicondyle
Greater
trochanter
Knee Unilateral posterior open bite. Courtesy: Department

of Oral Medicine and Radiology, Manipal College
of Dental Sciences, Mangalore
Schematic diagram showing tender points in fibromyalgia

Malignant tumors such as chondrosarcoma, synovial sar-
coma, fibrosarcoma, osteogenic sarcoma and multiple
17, 18 (Knee): bilateral, at the medial fat pad proximal to myeloma have been reported to affect the TMJ structures.
the joint line. Distant metastasis to the TMJ have been reported to occur
from primaries in the breast, lungs, prostate, liver, uterus,
Management pancreas and rectum.
The clinician needs to be aware of these uncommon
Fibromyalgia can be treated effectively with amitriptyline neoplasms as they tend to mimic the clinical features of
(tricyclic antidepressant). Amitriptyline improves the sleep other common temporomandibular disorders such as limited
quality and minimizing pain. NSAIDs and muscle relaxants jaw movements, restricted mouth opening, deviation or
have also been tried successfully. deflection during mouth opening and joint sounds. As the
condition advances it may result in extension of the tumor
mass into the middle cranial fossa or invade posteriorly
CONGENITAL, DEVELOPMENTAL AND through the glenoid fossa and present as a lesion of the
ACQUIRED DISORDERS OF THE TMJ middle ear or external auditory canal. When the tumor mass
extends laterally it can produce a preauricular swelling mim-
Developmental disturbances affecting the condyle such as icking a parotid gland tumor. Cranial nerves may be involved
aplasia, hypoplasia and hyperplasia have been described in if the tumor mass extends into the infratemporal and pter-
Chapter 2 on Developmental Disturbances. ygopalatine fossae.
Clinical features
NEOPLASMS AFFECTING THE TMJ Benign tumors All the benign tumors share the similar
features of slow growth of the tumor mass, gradually
Both benign and malignant tumors can affect the TMJ developing malocclusion (generally unilateral posterior
structures that include the articular disk, synovial mem- open bite, Figure 22), deflection of the mandible to the
brane, articular capsule, glenoid fossa and most impor- unaffected side, facial asymmetry (Figure 23A, B), joint
tantly the condyle. Malignant tumors are rare and reports noises and restriction in jaw movements. Benign tumors
of metastasis to the TMJ from distant sites are very few in are usually painless.
number. Despite this fact, one should also look out for The tumors are usually seen in the 2nd and 3rd decades
locally invasive lesions originating from the middle ear of life. They may radiographically present as a well-
and parotid. defined nodular mass extending from the condylar head,
The common benign tumors that affect the TMJ include: unlike hyperplasia where there is uniform enlargement of
osteoma, chondroma, osteochondroma and osteoblastoma. the condylar head (Figure 24). Another distinguishing
260
Figure 23
A B
Facial asymmetry in patient with osteochondroma of the condyle. Courtesy: Department of

Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore
Figure 24 recurrence rates are common), whereas high-grade tumors

may metastasize through the lymphatics. Many of the
patients complain of a rapidly growing swelling (in the pre-
auricular region) and pain, diminished hearing and
restricted mouth opening.
Synovial chondrosarcoma (SC) may usually originate as
a primary from the synovium or secondarily in an already
existing synovial chondromatosis. These tumors generally
occur in women in the 5th and 7th decades of life. SC pres-
ents as a soft tissue mass resulting in TMJ dysfunction and
pain. Radiographs reveal clumps of calcifications occurring
in a soft tissue mass. The articulating surfaces may show
Orthopantomograph showing uniform enlargement of the
erosion. Joint space may occasionally exhibit freely lying
condylar head on the right side. Courtesy: Department of
Oral Medicine and Radiology, Manipal College
cartilaginous nodules.
of Dental Sciences, Mangalore Synovial sarcomas arise from the pluripotential mesen-
chyme. These tumors originate in close proximity to ten-
dons, tendon sheaths and joint capsule. They are said to
represent about 5% of all soft tissue sarcomas. These tumors
feature between the two is that the hyperplasia occurs
are relatively slow growing and painless and usually present
usually as a reactive lesion to trauma, whereas benign
as mass in the pre-auricular region. Synovial sarcomas are
tumors arise spontaneously. The most common benign
usually present in the 2nd and 4th decades of life. Strikingly
tumor to affect the TMJ is osteoma followed by osteo-
50% of these tumors show calcifications radiographically.
chondroma. Osteomas may occur as single isolated lesion
Recurrences are common and the lung is the most preferred
or as a part of Gardner’s syndrome. Benign osteoblastomas
site for metastasis. Synovial sarcoma is unique in the
though frequently seen in the 2nd decade of life, may occur
sense that it is one sarcoma that metastasizes (20% of the
anywhere in the 1st to 8th decades of life. These benign
cases) to the lymph nodes.
tumors often produce a tender swelling and patients usu-
Osteosarcomas may be seen in any age group ranging
ally give a history of trauma.
from the 2nd to the 6th decade of life. The clinical features
Malignant tumors Chondrosarcomas account for about include mild pain, malocclusion and restricted mouth
1–3% of the sarcomas affecting the facial bones and jaws. opening and other mandibular movements. Radiographs
It is usually seen in women in the 5th decade of life. reveal extensive destruction of bone. Metastasis to the
Low-grade tumors have excellent prognosis (however high lungs is common.
261
Multiple myeloma and occasionally a solitary plasma- Classification of Condylar Fractures

cytoma may affect the TMJ in males over the 6th decade of
life. Patients usually present with pain and limited mouth I. Lindhal’s classification of condylar fractures
opening. Large tumors may exhibit pre-auricular swelling. A detailed and comprehensive classification of mandibu-
Radiographs reveal well-defined punched-out radiolucent lar condylar fractures was proposed by Lindhal in 1977.
areas without a sclerotic rim. Occasionally pathological In order to categorize the condylar fractures it is manda-
fractures may be seen. tory that radiographs be taken in two planes at right
angles to each other.
Metastatic tumors Metastasis to the jaws is rare and the This classification takes into consideration three param-
condyle is almost never a location. However, isolated eters, namely, the level of fracture, relationship of the frac-
reports have described TMJ dysfunction and pathologic tured condylar segment to the mandible and the relationship
fracture as the presentation features. There have been var- of the condylar head to the glenoid fossa.
ious theories suggested to explain the cause for very less
chances of metastasis to the condyle. Some of these include 1. Level of fracture (Figure 26)
lack of abundant red marrow, relatively sluggish pace of a. Intracapsular head or condylar head fracture: Vertical
blood flow, separate vascular supply from the circular pen- fractures (anteroposterior sagittal split), compression
etrating branches of the maxillary and superficial temporal fractures (results in a mushroom-shaped expansion of
arteries and an osseous plate that cuts off the condylar mar- the condylar head) and comminuted fractures are the
row cavity from the spongiosa of the rest of the mandible. subtypes of the fractures of the condylar head.
Synovial chondromatosis is an idiopathic condition, b. Condylar neck fractures: These fractures are seen at
which has been one of the more commonly reported con- the inferior attachment of the capsule.
ditions associated with loose joint bodies. It is thought to c. Subcondylar fractures: These fractures occur inferior to
represent a cartilaginous metaplasia associated with an the condylar neck. The subcondylar region corresponds
abnormal synovium, rather than a true neoplasia. It pres- to the area extending anteriorly from the inferior-most
ents with multiple cartilaginous nodules located within point of the sigmoid notch to the point of maximum
the joint space. Symptoms include joint swelling, pain, curvature of the posterior border of the mandible.
limited movement and joint noise. 2. Relationship of the fractured condylar segment to the
mandible The relationship of the fractured condylar seg-
ment with the remainder of the mandible is categorized
CONDYLAR FRACTURES into: undisplaced (fissure fracture), deviated (angulated
fractured segment without overlap), displaced with medial
Condylar fractures are the most common among fractures overlap of the fractured segment, displaced with lateral
involving the mandible. It is estimated that of all the man- overlap, anterior and posterior overlap and fractured frag-
dibular fractures approximately 25–52% involve the con- ment with no contact with the remainder of the mandible.
dyle (Figure 25).
Lindhal in 1977 described three major sources of con-
dylar injuries:
1. When kinetic injury is imparted to a static individual Figure 25
(such as in interpersonal violence i.e. a blow to the
chin). Usually in such cases, fracture of the parasym-
physis may be seen on the side of trauma along with
condylar neck fracture on the contralateral side.
2. When kinetic injury is imparted to a moving individual
against a static surface (such as in a ‘parade ground’
fracture, or injury sustained when an individual falls
on the floor like in an epileptic fit). In these instances
symphyseal fracture is usually associated with bilat-
eral condylar neck fractures.
3. When a moving individual strikes against another
moving object, a summation of the kinetic energies of
the first two varieties occurs leading to a severe form
of injury (such as in road traffic accidents). Such inju-
ries may cause bilateral parasymphyseal along with
Condylar fracture marked on a dry skull specimen
bilateral condylar neck fractures.
262
Figure 26
Intracapsular Extracapsular Subcondylar

fracture fracture fracture
Schematic diagrams showing various levels of condylar fractures
3. Relationship of the condylar head to the glenoid fossa the ear on the side of fracture (laceration of anterior wall
There are three subtypes under this category, namely, no of the external auditory meatus) may be evident.
displacement (radiographically the joint space appears Ecchymosis of the skin just below the mastoid process on
normal), displacement (increased joint space but the man- the side of fracture may be seen. This is due to the down-
dibular condyle is related to the glenoid fossa and disloca- ward tracking of the hematoma around the fractured con-
tion (condylar fragment is totally out of the glenoid fossa, dyle to an area beneath the external auditory canal. This
usually dislocated in an anteromedial direction). clinical appearance should not be misunderstood as the
Battle’s sign which is a similar sign seen in the base of
II. MacLennan’s classification skull fractures.
Traumatic injuries to the condyle (involvement of the
MacLennan considered the relationship of the fractured
condylar growth center) during the period of mandibular
condylar fragment to the mandible for his classification.
growth may result in stunted and inhibited growth pro-
Four broad categories have been described: (i) no dis-
ducing a hypoplastic mandible. Occasionally, limited joint
placement, (ii) fracture deviation (simple angulation of the
function may be seen secondary to hemorrhage and sub-
condylar fragment in relation of the remainder of the
sequent bone formation within the joint spaces. The higher
mandible as seen in greenstick fractures), (iii) fracture dis-
the fracture is located and greater the degree of displace-
placement (overlap of the condyle and the remainder of
ment of the fragments, the less favorable are the expected
the mandible) and (iv) fracture dislocation (complete dis-
functional results. When the fracture fragments are dis-
ruption of the condylar head from the glenoid fossa).
placed at least by 5 mm and displaced by a minimum of
30⬚, a severely dislocated fracture can be considered.
III. Classification of condylar neck fractures The condylar neck is usually very slender (about 4.9 mm)
Spiessl and Schroll classified condylar neck fractures into and hence very amenable to fractures (Figure 27). The
six different types: condylar neck usually fractures secondary to a blow to the
chin or parasymphyseal region.
1. Type I: Condylar neck fracture without serious dis- When the fracture line runs above the level of insertion
location of the lateral pterygoid muscle within the capsule of the
2. Type II: Deep seated condylar neck fracture with dis- joint, then very minimal bony displacement is evident.
location However, when the fracture occurs below the level of mus-
3. Type III: High condylar neck fracture with dislocation cle insertion, condylar displacement is usually seen in the
4. Type IV: Synovial deep seated condylar neck fracture direction of the pull of the muscle (anteriorly, inferiorly
with luxation and medially directed forces) (Figure 28).
5. Type V: High condylar neck fracture with luxation In severe cases the mandibular condyle may be impacted
6. Type VI: Head or intracapsular fracture. through the glenoid fossa and the patient may not be able
to open the mouth.
On palpation, following trauma, the patient may
Clinical signs and symptoms of condylar
experience severe tenderness over the temporomandibular
fractures
region. Paresthesia of the lower lip may be seen when the
Unilateral condylar fractures may present with a distinct bleeding from the condylar region trickles down across the
swelling over the TMJ region. Occasionally, bleeding from base of the skull to exert pressure over the mandibular
263
Figure 27 Figure 28
Posteroanterior (PA) view showing medial displacement of

condylar neck fracture
Cropped orthopantomograph showing condylar neck fracture
occur in association with symphyseal or parasymphyseal

division of the trigeminal nerve as it exits from the fora- fractures. Long-standing condylar fractures may present
men ovale. with a hollow over the condylar head region. However,
Patient may not be able to protrude the mandible and this hollowing due the medial displacement of the condy-
may have a limited ability to exhibit lateral excursion of the lar fragment may not be appreciated due to the edema
mandible to the opposite side. Unilateral fracture causes following immediate injuries.
shortening of the ramus and molar occlusal gagging on Routine CT studies of suspected closed head injury
the same side. Bilateral condylar fractures usually present encompass the calvaria to the cranial base. It is at the
with all the clinical signs and symptoms of the unilateral cranial base that the glenoid fossa appears. An ‘empty
fractures occurring on both the sides. glenoid fossa’ sign is when there is no condyle associated
Bilateral displacement of the condylar fragments may within the fossa. This is suggestive of a condylar neck
result in an anterior open bite. Condylar fractures usually fracture.
264
CHAPTER
Diseases of Salivary Glands
Carol M Stewart, Indraneel Bhattacharyya,
Madhu K Nair, James C Pettigrew,
Seunghee Cha, Joseph Katz
11
➧ Developmental Disturbances ➧ Non-inflammatory Conditions of
Aplasia/Agenesis and Related Aberrancy of Salivary Glands
Salivary Glands Sialadenosis
Hyperplasia of Minor Salivary Glands Anorexia/Bulimia-related Sialadenosis
➧ Saliva, Xerostomia, Hyposalivation, and Sialadenosis Associated with Alcoholic Cirrhosis
Sialorrhea Diabetes Mellitus
Saliva Medication-induced Sialadenosis
Hypofunction and Xerostomia Orofacial Granulomatosis
Xerostomia and Salivary Gland Hypofunction Sarcoidosis
due to Medications ➧ Salivary Gland Tumors
Xerostomia and Salivary Gland Hypofunction ➧ Benign Tumors
due to Radiation Therapy Pleomorphic Adenoma (Benign Mixed Tumor)
Sjögren’s Syndrome Canalicular Adenoma
Benign Lymphoepithelial Lesion (Mikulicz’s Disease) Basal Cell Adenoma
Sialorrhea Papillary Cystadenoma Lymphomatosum
➧ Inflammatory Conditions of Salivary Glands (Warthin’s Tumor)
Mucocele Oncocytoma (Oxyphilic Adenoma)
Ranula ➧ Malignant Tumors
Sialolithiasis, Salivary Duct Stone, Salivary Calculi Mucoepidermoid Carcinoma
Sialadenitis Intraosseous Mucoepidermoid Carcinoma
Non-specific Sialadenitis Acinic Cell Adenocarcinoma
Bacterial Sialadenitis Malignant Mixed Tumor (Carcinoma Ex Pleomorphic
Subacute Necrotizing Sialadenitis Adenoma)
Cheilitis Glandularis Metastasizing Mixed Tumor
Necrotizing Sialometaplasia Adenoid Cystic Carcinoma
Chronic Sclerosing Sialadenitis (Kuttner Tumor) Polymorphous Low-Grade Adenocarcinoma
➧ Viral-induced Salivary Gland Pathology
Mumps
Human Immunodeficiency Virus (HIV)
Salivary glands and saliva play a critical role in mainte- 4. Non-inflammatory conditions
nance of oral and systemic health. Salivary glands are fre- 5. Tumors.
quently involved in a wide array of conditions which
Many of these conditions result in salivary gland hypo-
result in glandular dysfunction. These may be subdivided
function, enlargement, pain and facial nerve paresthesia
into five categories which include:
that will prompt the patient to seek evaluation and treat-
1. Developmental disturbances ment from the dentist. This chapter will address each of
2. Saliva and salivary flow alterations these five subgroups focusing on the etiology, clinical fea-
3. Inflammatory conditions tures, diagnosis and treatment of these conditions.
265
DEVELOPMENTAL DISTURBANCES prudent. Sometimes, congenital absence of salivary glands

is not noticed until adulthood resulting in unfortunate
Aplasia/Agenesis and Related Aberrancy sequelae. However, clinical suspicion of salivary gland
agenesis should be heightened in the setting of the ‘non-
of Salivary Glands
drooling baby’. Lack of complete development of the sub-
Congenital aplasia or agenesis of the major salivary glands mandibular gland duct has been reported in two infants
is an uncommon finding, characterized by partial or total which presented as unilateral cystic swellings in the floor
lack of development of the gland. Agenesis may involve of the mouth. Both cases responded to simple incision and
one gland, pairs, or multiple glands, and be unilateral or decompression of the fluid-filled ducts. Early treatment is
bilateral. It may be a single independent finding or be important to avoid feeding difficulties and possible later
associated with other developmental anomalies. complications such as ranula or sialadenitis.
Congenital agenesis of major salivary glands was first
reported by Gruber in 1985. A recent search of the world Diagnostic imaging
literature reported 30 documented cases. Three cases of
When a unilateral glandular anomaly is observed clini-
unilateral aplasia of the parotid have been reported. As it
cally, bilateral imaging should be considered to examine
may be asymptomatic and go unnoticed, the true incidence
for possible lesions on the contralateral side. Computed
is unknown. Bilateral agenesis is more common, with
tomography (CT) can be used to demonstrate congenital
10 cases reported in the English literature. Sometimes con-
total or partial absence of salivary glands.
genital absence of one parotid or submandibular gland is
associated with hypertrophy of the contralateral gland.
Management
Aplasia may occur as a single event or with autosomal
dominant inherited developmental conditions such as first Salivary gland aplasia has been associated with rampant
branchial arch anomalies, hemifacial microsomia and man- dental decay in children. Treatment for potential hypo-
dibulofacial dysostosis. Parotid gland aplasia may be asso- salivation resulting from salivary gland agenesis would
ciated with malformations of the lacrimal apparatus as include frequent dental examinations, salivary stimulants
well. A combination of multiple developmental anomalies for glands that might remain, meticulous home care and a
is found in the lacrimo-auriculo-dento-digital (LADD) customized fluoride program.
syndrome (Levy–Hollister syndrome). This is an autosomal-
dominant multiple congenital anomaly disorder character-
Hyperplasia of Minor Salivary Glands
ized by hypoplasia, aplasia or atresia of the lacrimal and
salivary systems, ear anomalies, hearing loss, digital mal- Clinical features
formations and dental alterations. As the parotid gland
Adenomatoid hyperplasia of the minor salivary glands is a
develops during the 4th week of uterine life, and the sub-
rare lesion characterized by localized swelling that clini-
mandibular and sublingual, and minor glands develop
cally mimics a neoplasm. The lesion most often develops
between 6th and 12th weeks, association of salivary gland
on the hard or soft palate, although it has been reported in
aplasia with other congenital abnormalities is easily
other oral minor salivary gland sites. The most common
understood. Bilateral aplasia of the parotid gland has also
presentation is during the 4th to 6th decades of life. The
been reported in a patient with Down’s syndrome. In ecto-
typical presentation is a painless, indolent palatal swelling
dermal dysplasia, aplasia of the submandibular glands and
which may be soft or firm to palpation. The overlying
alterations in salivary gland function have been reported
mucosa is usually normal in color, although some lesions
as well.
are red or bluish in color. The etiology is uncertain. In one
report, the occurrence of adenomatoid hyperplasia on the
Clinical features and diagnosis palate occurred in patients who were tobacco smokers or
denture wearers or both. The authors, Barrett and Speight
Salivary gland agenesis may be asymptomatic if only par-
suggested that chronic, local trauma could be a factor in
tial agenesis occurs or if the condition is isolated to one
the development of the condition.
gland. If more extensive involvement occurs, agenesis can
produce profound hyposalivation in children resulting in
Histopathology
advanced dental caries, candidiasis, ascending sialadeni-
tis, and even laryngitis and pharyngitis. A child with sub- Histopathologic examination demonstrates lobular aggre-
jective xerostomia and functional hyposalivation should gates of normal appearing mucous acini that are greater in
be carefully examined for salivary gland dysfunction to number (hyperplasia) than normally would be found in the
include partial or complete salivary gland agenesis. Famil- area. These glands may appear to be increased in size
ial parotid gland aplasia has been reported, hence exami- (hypertrophy). Chronic inflammation, if present, is usually
nation of the siblings of a child with agenesis, might be mild and localized. Shimoyama et al reported that Ki-67
266
Chapter 11 – Diseases of Salivary Glands
staining for cell proliferative activity demonstrated no sta- stimulation, parotid secretions account for at least half of
tistically significant differences among adenomatoid hyper- the volume of whole saliva. Serous secretions are ‘watery’
plasia and a matched group of normal palatal salivary and contain digestive enzymes, water, salts, and ions. They
glands. The conclusion was that adenomatoid hyperplasia aid in mastication, clearing agents from the oral cavity, and
had limited growth potential. facilitate swallowing and speech. Fluid from the parotid
gland enters the oral cavity through the main excretory
Treatment duct, Stensen’s duct, located in the buccal mucosa next to
the maxillary second molars. In the submandibular gland,
To determine the true nature of a clinical enlargement, a
the acinar components are mixed mucous–serous, but
biopsy is necessary. After the diagnosis of adenomatoid
predominantly serous. At rest, the submandibular gland
hyperplasia has been established via histopathologic exam-
secretions account for approximately two-thirds of the
ination, no further treatment is indicated.
unstimulated saliva production. Wharton’s duct is the
main duct of the submandibular gland and enters the floor
of the mouth on either side of the lingual frenum. The
SALIVA, XEROSTOMIA, HYPOSALIVATION sublingual gland, the smallest of the three major glands, is
AND SIALORRHEA located above the mylohyoid muscle. Acinar elements are
mixed, but predominantly mucous. The mucin produced
Saliva facilitates lubrication and swallowing. The main excretory
duct of the sublingual gland, Bartholin’s duct, may join
Saliva is an essential fluid for maintaining oral and sys- Wharton’s duct resulting in a blending of secretions, or
temic health and a satisfactory quality of life. While saliva open into the oral cavity with a separate sublingual papilla.
is sometimes considered bothersome during restorative Numerous sublingual ducts may join the submandibular
procedures, the alteration in quality and/or quantity of gland duct or open separately into the floor of the mouth.
saliva has serious sequelae for the patient. Saliva is the Minor salivary glands are named according to their
product of three paired major salivary glands (the parotid, location—lingual (anterior and posterior), labial, buccal,
submandibular, and sublingual), and minor glands found palatine and glossopalatine. Table 1 summarizes the nomen-
in the hard and soft palate, lips, tongue, and buccal mucosa clature for the major and minor salivary glands and their
(Figure 1). Major salivary glands are composed of ductal acinar components.
and acinar structures. The acinar cells are the secretory In health, approximately 750 ml of saliva is produced in
components and ductal cells are the branching network a day. The principal control of salivary secretion is medi-
that transports saliva into the oral cavity. The acinar cells ated by sympathetic and parasympathetic innervation. The
of the parotid gland are purely serous elements and upon adrenergic (␣ and ␤) receptors are regulated by the sym-
pathetic nervous system and the muscarinic receptors by
the parasympathetic nervous system. At least five musca-
Figure 1 rinic-cholinergic receptor subtypes exist and it has been
determined that the muscarinic receptor that medicates
secretion of saliva is the M3 subtype. The parasympathetic
Table 1 Major and minor salivary glands and secretion
Glands Type of secretion

Parotid Purely serous
Submandibular Mixed, however
predominantly serous
Sublingual Mixed, however
predominantly mucous
Parotid Lingual minor salivary glands
gland
Sublingual Anterior lingual (glands of Blandin and Primarily mucous
gland Nuhn)
Submandibular Posterior lingual (glands of von Ebner) Purely serous
gland Labial and buccal minor salivary glands Mixed
Glossopalatine and palatine minor salivary Purely mucous
Major salivary glands. Courtesy: Roger Hoover glands (Weber’s glands)
267
nerve stimulation leads to an increased volume of saliva, the ‘mouth mirror test’. The clinician places the mirrored
whereas the sympathetic stimulation has an effect on pro- surface of the mouth mirror over the buccal mucosa and
tein content and salivary composition. attempts to move it. If the mirror glides effortlessly over
Saliva plays a major role in the local and systemic pro- the tissue, one can deduce that salivation is adequate.
tection of the oral cavity, oropharyngeal region, and the The prevalence of xerostomia in the population varies
upper gastrointestinal tract. Normal quantity and protein widely due to the lack of a clear definition for xerostomia.
composition is essential for buffering the acidity of the Some clinicians use the terms ‘xerostomia’ and ‘hyposali-
oral cavity, lubrication of tissues, maintaining the integ- vation’ interchangeably. Hyposalivation or salivary gland
rity of the oral tissues, providing antimicrobial proteins hypofunction results when the salivary gland flow rate is
and digestive enzymes, and remineralization of the teeth. lower than normal. Defining ‘normal’ is problematic due
Lack of saliva, hyposalivation, may increase a patient’s sus- to variations in the literature on collection methods, time
ceptibility to dental decay, oral ulcers, fungal infections, of day collections were performed, and the inability to
and dysphagia or difficulty in swallowing. control for medications. Unstimulated whole salivary
Saliva contains many antimicrobial proteins that help flow rates of less than 0.15 ml/min might provide a good
maintain a normal oral flora. The histatins provide anti- reference for hyposalivation. The determination of hypo-
fungal properties, and proline-rich proteins help reduce salivation and xerostomia do not consistently correlate as
bacterial colonization by modifying the ability of organ- one might expect. Patients with salivary gland hypofunc-
isms to attach to tissues. Mucins are salivary glycoproteins tion are sometimes without complaints of oral dryness, or
that play a role in mucosal lubrication and assist in aggre- xerostomia. Conversely, patients with complaints of ‘dry
gation of oral microorganisms. Statherin and proline-rich mouth’ may not have reduced salivary flow rates. Xerosto-
proteins have calcium-binding properties to assist reminer- mia and salivary hypofunction affect the oral health-related
alization. These proteins combined with the cleansing and quality of life. Reported reasons include the negative impact
flushing ability of saliva constitute significant protective of oral dryness on speaking, eating, and wearing dental
mechanisms for the oral cavity and upper gastrointestinal prostheses. Both xerostomia and salivary gland hypofunc-
system. Of special importance to the dentition is the presence tion require a careful systematic evaluation to determine
of buffers to help maintain a neutral pH and the remineral- causative factors.
izing capacity. The pH range of saliva will vary from 6.7 to Dry mouth has a variety of causes as indicated in Box 1.
7.4 in health. Of concern is pH below 5.5, which will pro- Common causes of salivary gland dysfunction leading to
mote enamel dissolution and increase caries susceptibility. hyposalivation include medications, irradiation to the head
and neck, and autoimmune diseases such as systemic lupus
erythematosus (SLE) and Sjögren’s syndrome (SS). Other
Hypofunction and Xerostomia
systemic conditions affecting salivary glands include sar-
Altered saliva production, both qualitatively and quanti- coidosis, sialadenosis, and viral infections such as HIV and
tatively, creates significant oral health concerns for the hepatitis C.
patient and challenges the dentist to provide appropriate Mouth-breathing, tobacco smoking, alcohol use, and con-
patient education and treatment. Patients frequently pres- sumption of beverages containing caffeine can contribute
ent with the complaint of a ‘dry mouth’. Xerostomia is the to oral dryness as well. Age-related hyposalivation has
subjective sensation of dry mouth and may be determined been reported due to structural changes in salivary glands
by questioning individuals about their perceptions of oral with increasing age. Other studies have reported no age-
dryness. The need to sip water while eating dry foods, dif- dependent decrease in saliva flow rate in healthy elderly
ficulty in swallowing food without liquids, and a feeling populations. The incidence of xerostomia will increase and
of ‘too little’ saliva in the mouth have been correlated with
salivary gland hypofunction. Hyposalivation is the produc-
tion of inadequate or less than normal amount of saliva, Box 1 Possible causes of salivary gland hypofunction
usually from gland hypofunction. A few simple chairside
Medications
assessments could be performed to quickly assess oral dry-
Radiation to head and neck
ness. The lack of salivary pooling in the floor of the mouth
is a basic clinical indicator of hyposalivation. Another Systemic diseases
indicator is the ‘cracker sign’. If a patient states that they Sjögren’s syndrome
Sarcoidosis
cannot eat a cracker without drinking fluids, xerostomia
Systemic lupus erythematosus
and hyposalivation should be suspected. Another indicator
Primary biliary cirrhosis
is the ‘lipstick sign’. Patients with hyposalivation may
Viral infections
have lipstick adhere to the incisal edges of the anterior
HIV
teeth because they lack saliva that would normally pre-
Hepatitis C
vent adherence. Another clinical clue to hypofunction is
268
the oral manifestations will continue to be a challenge for cause subjective complaints of dry mouth and many
the clinician. induce hyposalivation. The drugs most frequently impli-
Treatment of xerostomia is symptomatic and support- cated in dry mouth include the tricyclic antidepressants,
ive, but should be aggressively proactive in prevention of antipsychotics, atropinics, beta blockers and antihistamines.
dental decay associated with salivary hypofunction. Patient In another study as reported by Thomson, the unstimu-
education and patient empowerment so they become par- lated (resting) salivary flow rate was reduced among indi-
ticipants in their oral healthcare are key to successful viduals who were older, female, or taking antidepressants,
outcomes. Hydration, dietary modifications and meticulous and higher among smokers or people who were taking
oral hygiene are especially helpful. Symptomatic treat- hypolipidemic drugs. Other studies have correlated dry
ment is aimed at minimizing the subjective complaints, and mouth with the number of medications taken, rather than
includes drinking water or finely crushed ice chips and specific types of medications. Field et al have demon-
sugar-free fluids. Patients should be advised that caffeine strated that medication is a better predictor of risk status
can contribute to the feeling of oral dryness and conse- for dry mouth than either age or gender. Medications that
quently, caffeine-free and sugar-free drinks should be induce salivary hypofunction are a concern among the
strongly encouraged. Avoiding alcohol, including alcohol- elderly, but xerostomia can also be a concern for young
based mouthrinses, spicy foods, and strong flavorings, adults. Thomson et al in his study involving a large cohort
such as cinnamon and mint, should also minimize oral dis- of 32-year-old subjects, reported that the prevalence of
comfort. Artificial saliva is helpful for many patients due xerostomia was found to be 10%, with no apparent gender
to the coating action, but does not provide long-lasting difference. There was a strong association between xero-
relief. Some patients find relief by chewing sugar-free gum stomia and diminished oral health-related quality of life.
or sucking on sugar-free candies. Lip balms and moistur- Xerostomia was significantly higher among those taking
izers may be helpful as well. antidepressants, iron supplements, or narcotic analgesics,
Because patients with hyposalivation are at increased and those subjects taking antidepressants at both 26 and
risk for dental decay, oral applications of topical fluorides 32 years of age demonstrated 22 times the odds of reporting
should be initiated. The fluoride is incorporated into the xerostomia.
enamel of the teeth to increase resistance to demineraliza- A study of medications and caries among older indi-
tion and decay. Fluoride products are available over-the- viduals by Thomson et al revealed that an adjusted coro-
counter as rinses and as more highly concentrated brush-on nal caries increment (AdjCI) was higher among males and
gels (0.4% stannous fluoride gel). Prescription products those taking a beta blocker or an anti-asthma drug for the
are available which provide 1.1% neutral sodium fluoride previous 5 years. Several classes of drugs have been asso-
treatment in convenient brush-on gels or rinses. These ciated with dry mouth (Box 2). The most common groups
should be used after regular toothbrushing in the evening include those with: (i) anticholinergic effects such as tricy-
just before retiring. Patients should be reminded to avoid clic antidepressants, drugs for urinary retention and over-
rinsing, eating or drinking for 30 minutes following the active bladder, antipsychotics, diuretics, and antihistamines;
fluoride application. Stannous fluoride or neutral sodium (ii) sympathomimetic drugs such as antihypertensives, anti-
fluoride treatments can be provided using fluoride carri- depressants, decongestants, and bronchodilators; (iii) skel-
ers, or custom trays fabricated from a cast of the patient’s etal muscle relaxants; (iv) benzodiazepines; (v) proton pump
mouth. The carriers are particularly helpful in treating inhibitors; and (vi) anti-migraine agents. Dry mouth has
severe hyposalivation resulting from head and neck radia-
tion therapy. Preventive fluoride programs should be cus-
tomized by the dentist to meet the specific needs and Box 2 Drugs associated with xerostomia and hyposalivation
conditions of the patient.
Tricyclic antidepressants
Antipsychotic medications such as phenothiazines
Xerostomia and Salivary Gland Hypofunction
due to Medications Diuretics
Antihistamines
Jacobsen and Chavez in their article describe that indi-
Anti-migraine agents
vidual above 65 years of age comprise 13% of the popula-
Proton pump inhibitors and H2 antagonists
tion, but consume approximately one-third of all drugs
prescribed. Loesche et al and Foc have reported many Anti-HIV drugs such as dideoxyinosine and protease inhibitors
studies that have demonstrated a link between dry mouth Opioids
in the elderly and polypharmacy. Shinkai et al reported Benzodiazepines
that in a group of 1,163 adults (age range 32–81 years), Decongestants
57% reported dry mouth to be the most frequent side
Selective serotonin reuptake inhibitors (SSRIs)
effect of their medications. Hundreds of medications can
269
been reported to be a consequence of cytotoxic drugs such The mucositis will slowly resolve 2–3 weeks after ces-
as 5-fluorouracil. Medications used for treatment of HIV sation of the treatment. A loss of taste has been reported
have also been associated with dry mouth. These include which generally is recovered after 6 months.
didanosine and protease inhibitors. Minimizing the probability of osteoradionecrosis (ORN)
It is important for the dentist to inquire about all medi- includes a dental examination at least 2 weeks prior to the
cations, both prescription and over-the-counter agents, initiation of radiation therapy to address or remove teeth
during the health history review. Sometimes the dentist that have a hopeless long-term prognosis. Daily fluoride
can provide assistance by sharing concerns of medication- treatments in custom carriers and close follow-up aid in
induced hyposalivation with the primary care physician. reducing the incidence of xerostomia-induced dental caries.
In some situations, the dentist might suggest medications Due in part to more efficient radiation techniques, the inci-
such as pilocarpine or cevimeline to promote salivation. dence of ORN has been declining in radiation patients over
These medications will be discussed in the section on the last two decades. Advances in radiation techniques,
Sjögren’s syndrome. including the use of fractionated radiation doses, have
minimized the incidental damage to adjacent tissues. Fur-
Xerostomia and Salivary Gland Hypofunction thermore, use of three-dimensional dosimetric intensity-
modulated radiation therapy (IMRT) has been shown to
due to Radiation Therapy
reduce late salivary toxicity, since the portion of tissue
The role of saliva in oral health and function relates both exposed to low radiation doses has a potential for repair.
to its fluid characteristics and to its specific components. Based on recent publications, the prevention of ORN remains
The water (fluid) phase accounts for 99% of the volume and controversial. A recent report compiled by Chang et al after
the remainder is salts and proteins. Essential functions of reviewing a large series of ORN studies stated that extrac-
these components include flushing, buffering the acidity, tion of teeth with poor prognosis before radiation therapy
and mucosal coating to maintain tissue integrity. Mucosal did not appear to reduce the risk of ORN. The investigation
coating and tissue lubrication are essential for speech, taste of IMRT by Wu et al to achieve sparing of the parotids and
perception, mastication, and swallowing. Cancer patients yet achieve higher tumor control appears to show promise.
undergoing treatment for head and neck disease often Until additional evidence is available to define guidelines,
experience severe difficulties maintaining such functions. a pre-radiation referral for a dental evaluation is necessary.
Salivary function can also be diminished by radioiodine To facilitate prevention of ORN, irradiated dental patients
therapy for thyroid carcinoma, especially if multiple doses should maintain a high level of oral health. Pre- and post-
are administered. Deterioration of oral health and radiation- therapy close collaboration by a multidisciplinary team
induced oral dryness has a significant influence on patients’ can be invaluable for patients receiving head and neck
overall quality of life during and after treatment. radiation therapy.
Together with surgery, radiation therapy is the main
treatment for head and neck tumors. All or part of the
Sjögren’s Syndrome
major and minor salivary glands may be included within
the radiation field due to the site and extension of the Sjögren’s syndrome (SS) is a chronic autoimmune disease
tumor and the lymphatic spread. Exposing the glands to affecting the exocrine glands, primarily the salivary and
radiation often results in severe salivary gland hypofunction lacrimal glands. Patients most commonly complain of a
and changes in saliva composition. A profound decrease subjective persistent feeling of dry mouth (xerostomia)
in salivary flow occurs during the 1st week of radiation and of dry eyes (keratoconjunctivitis sicca). This is due
therapy and continues throughout the course of therapy. to lymphocytic infiltrates and destruction of salivary and
In general, fully irradiated parotid glands exposed to doses lacrimal glands and systemic production of autoantibod-
exceeding 60 Gy sustain permanent damage resulting in ies. In 1933, Henrik Sjögren, a Swedish ophthalmologist,
severe hypofunction, and there is no recovery of gland presented his doctoral thesis entitled ‘Zur Kenntnis der
function over time. Partial salivary flow recovery may Keratoconjunctivitis Sicca’, and described the clinical
occur at lower doses. and histopathological aspects of the disease.
Radiation mucositis may begin during the 2nd week of Sjögren’s syndrome occurs worldwide and while it
therapy. Primary sites are intraoral mucosal surfaces within may occur at any age, the peak incidence is between 40
the direct portals of radiation. A whitish discoloration will and 50 years. Sjögren’s syndrome has one of the highest
appear from keratin accumulation, which is followed by female-to-male ratio (9:1) of any autoimmune rheumatic
sloughing, revealing atrophic erythematous, and friable disease. In addition to ocular and oral dryness, a wide
mucosa. Ulceration then develops producing burning and spectrum of extraglandular manifestations may occur as
pain which is exacerbated by eating and oral hygiene. well. The musculoskeletal, hematological, vascular, pulmo-
Symptomatic support for radiation mucositis is important nary, gastrointestinal, dermatological, renal and nervous
to assist the patient in maintaining adequate nutrition. systems may be involved. Patients with SS have an increased
270
risk of developing lymphoma. Early reports estimated that Box 3 European American Consensus Group criteria for
patients with SS had up to 44 times increased risk of Sjögren’s syndrome
developing lymphoma compared with the general popula-
tion. Chronic fatigue, depression, and a diminished quality Ocular symptoms (must have 1 of 3)
1. Daily dry eyes ⬎ 3 months
of life are also common components of SS.
2. Recurrent sand or gravel sensation in the eyes
3. Use of tear substitutes more than 3 times per day
Classification
Oral symptoms (must have 1 of 3)
Two forms of the disease are recognized. Primary SS is the 1. Feeling of dry mouth for more than 3 months
presence of sicca syndrome, xerostomia, or ‘dry mouth’, 2. Recurrent or persistently swollen salivary glands as an adult
and xerophthalmia, or ‘dry eyes’ together, with no other 3. Need frequent liquids to aid in swallowing dry food
autoimmune disease. Secondary SS is sicca syndrome plus Ocular signs (1 of 2)
another associated autoimmune disease such as rheumatoid 1. Schirmer’s test, performed without anesthesia (ⱕ 5 mm in
arthritis (RA), SLE, or scleroderma. Based upon the classi- 5 minutes)
fication criteria applied, the prevalence of SS may range 2. Rose Bengal score or other ocular dye score (ⱖ 4 according to
from 0.5 to 3.0% of the population. All classification sys- van Blijsterveld’s scoring system)
tems use a combination of both subjective and objective Histopathology in minor salivary gland biopsy
findings in the diagnostic process. The most recent 2002 Focal lymphocytic sialoadenitis, with focus score ⱖ 1 (focus is
criteria include subjective symptoms of dry mouth and dry ⱖ 50 lymphocytes per 4 mm2 tissue adjacent to normal appearing
mucous acini)
eyes, and the following objective tests: ocular signs by
Schirmer’s test and/or Rose Bengal score; focal sialadenitis Salivary gland involvement (1 of 3)
by histopathology; salivary gland involvement by either 1. Unstimulated whole salivary flow (ⱕ 1.5 ml/15 minutes)
2. Parotid sialography showing the presence of diffuse sialectasis
salivary scintigraphy, parotid sialography or unstimulated
(punctuate, cavitary of destructive pattern) without evidence of
salivary flow rate; and autoantibodies of SS-A/Ro and/or
obstruction in the major ducts
SS-B/La specificity. Box 3 presents the 2002 American- 3. Salivary scintigraphy, showing delayed uptake, reduced
European Consensus Group Criteria for SS. concentration and/or delayed excretion of tracer
Autoantibodies
Etiopathogenesis Antibodies to SS-A/Ro or SS-B/La or both
Currently, the etiology of SS is not clearly understood, but For primary SS
appears to be multifactorial. It has been suggested that a. Any 4 of the 6, as long as either item 4 (histopathology) or
environmental agents may trigger SS in genetically pre- item 6 (serology) is positive
disposed individuals. Experimental and clinical evidence b. Presence of any 3 of the 4 objective criteria items
suggest that immune reactivity is modulated by gender. (items 3, 4, 5, 6)
Immune reactivity is higher in females than males, and For secondary SS
lymphocytes and monocytes from female subjects show In the presence of another connective tissue disease, the presence
higher antigen presenting activity and mitogenic responses. of item 1 or 2 plus any 2 from 3, 4, and 5
Taiym et al have found higher prolactin levels in SS Exclusion criteria
patients than controls. Estrogens might be pro- or anti- Past head and neck radiation
inflammatory, based on dose-related metabolite conver- Hepatitis C infection
Acquired immunodeficiency syndrome (AIDS)
sions. The role of sex hormones in rheumatic autoimmune
Pre-existing lymphoma
diseases has yet to be clarified. Potential mechanisms
Sarcoidosis
underlying SS include disturbances in apoptosis, circulat- Graft-vs-host disease
ing autoantibodies against the ribonucleoproteins Ro and Use of anticholinergic drugs (since a time shorter than four-fold
La or cholinergic muscarinic receptors in salivary and lac- half-life of the drug)
rimal glands or cytokines. These processes interfere with Source: Vitali et al. Annals of Rheumatic Diseases 2002;61:554–58.
normal glandular function; and as the mucosal surfaces
become sites of chronic inflammation, the disease appears
to enter a self-perpetuating inflammatory cycle.
While a genetic predisposition to SS appears to exist, diseases and co-association of multiple autoimmune dis-
no simple Mendelian inheritance pattern has been demon- eases has been reported by Becker et al. Reports have
strated. Cases of two or more individuals with SS per fam- also indicated that a SS proband may have relatives with
ily and SS in twins have been described. However, the level other autoimmune diseases in approximately 30–35% of
of genetic contribution is not known. Because large twin the cases. Assessing human leukocyte antigen (HLA)-DR
studies in SS are lacking, the twin concordance rate cannot and HLA-DQ gene segments in patients with SS reveals an
be estimated. Familial clustering of different autoimmune increased use of haplotypes B, Drw52 and DR3. Correlations
271
have been found between presence of HLA-DR haplotype

Figure 2
and the presence of Ro/LA in SS. Gene polymorphisms
have been analyzed, but no clear-cut relationship between
these and primary SS have been identified. Clustering of
non-major histocompatibility complex (MHC) susceptibil-
ity candidate loci in human autoimmune diseases supports
a hypothesis that, in some cases, clinically distinct auto-
immune diseases may be controlled by a common set of
susceptibility genes.
Clinical features and diagnosis

Dry mouth and dry eyes are the most common complaints
of patients with SS. Patients’ ocular concerns may include
a history of dry eyes, a sensation of sand or gravel in the
eyes, and/or frequent (⬎ 3 times per day) use of tear sub-
Xerostomia and fissured tongue.
stitutes. Objective ocular tests for dry eyes include a
Courtesy: Dr Carol Stewart
Schirmer’s test for tear production. Using sterile strips of
filter paper placed just inside the lower lid, tear production
can be assessed by measuring the length of wetness on the
filter paper. Less than 5 mm of wetness in 5 minutes, with-
out local anesthesia is considered a positive test, meeting Figure 3
the 2002 Sjögren’s consensus criteria. A second ocular test
utilizes Rose Bengal staining of the cornea, and measures
areas of increased dye intensity. With the use of a slit-
lamp, the stain will detect devitalized tissue. The ocular
dryness is sufficient to produce disruption in the integrity
of corneal and conjunctival epithelium and dye will accu-
mulate in these areas. The total areas identified are used to
determine the extent of ocular damage. Patients with com-
plaints of ocular dryness, especially if combined with other
symptoms of SS, should be referred to the ophthalmologist
for evaluation. Untreated keratoconjunctivitis sicca can
progress to corneal ulcerations and even blindness.
The most frequent oral signs and symptoms a dentist
will encounter are xerostomia, a subjective sensation of
oral dryness, and hyposalivation, or a diminished salivary
flow rate. These will vary between patients, and the sub- Oral cavity of Sjögren’s syndrome patient showing dental
caries and fissured tongue. Courtesy: Dr Carol Stewart
jective dryness may not directly correlate with objective
measures of hyposalivation. Initial indications of a dimin-
ished salivary output would be a lack of pooling in the floor
of the mouth, thick or frothy saliva, and observing exam- flow rate is diminished in primary SS patients compared
ination gloves sticking to the tongue or buccal mucosa. with controls. In addition to quantitative changes, the pro-
Patients may complain of difficulty chewing and swallow- tein content of the saliva is altered as well. Proteins neces-
ing, difficulty wearing their dentures, and altered taste. sary for buffering the oral acidity, countering fungal and
They will relate the necessity for drinking liquids to aid in microbial organisms may be altered. The lack of adequate
swallowing food or to enhance their ability to speak. They salivary flow and qualitative changes in protein content
may admit to keeping water by their bedside at night or may predispose the patient to dental decay, particularly in
frequently waking with a dry mouth. Patients with SS fre- the cervical area, tooth loss, candidiasis and oral ulcer-
quently carry water with them and often need to sip every ations (Figure 3). The change in saliva is linked to the
10–15 minutes during a consultation appointment. Upon lymphocytic infiltrate in the glands and subsequent dam-
intraoral examination, the tongue may appear fissured, age to the functional units.
slightly erythematous, and sometimes depapillated. If the From one-fourth to two-thirds of patients with pri-
patient complains of a ‘burning tongue’ and dysgeusia, mary SS will have a diffuse enlargement of the major sali-
oral candidiasis should be suspected (Figure 2). Salivary vary glands during the course of their disease (Figure 4).
272
Figure 4 Figure 5
Sjögren’s syndrome patient with enlarged parotid gland. Sialograph of Sjögren’s syndrome.
Courtesy: Dr Carol Stewart Courtesy: Dr James Pettigrew
This swelling may be unilateral or bilateral, intermittent or

Figure 6
constant in nature. If the parotid swelling initially is uni-
lateral, it often becomes bilateral with time. It commonly
produces mild to no discomfort. However, diminished flow
will enhance a patient’s susceptibility to bacterial infection
in the glands and recurrent sialadenitis, which will produce
pain. Although SS is considered primarily a disease of
middle-aged females, it has also been reported in children
and adolescents. Recurrent bilateral parotitis in children
and adolescents should include the possibility of SS in the
differential diagnosis.
Conventional sialography renders useful information
about the gland architecture and changes within it. Water-
based radiopaque dye is injected into the major gland
ducts followed by conventional imaging. Peripheral ducts
within the glands are usually affected first with the inner
ductal structure relatively well preserved. However, punc-
tuate collections of the contrast material may be visible in
the early stages of the disease followed by globular or Sialograph of Sjögren’s syndrome of the submandibular
larger collections as the condition progresses as shown in salivary gland. Courtesy: Dr Ajit Auluck and
Figures 5–7. Once extensive intraglandular destruction has Dr Chandrakant Shetty
taken place and infection has become established, dilata-
tion of central ducts is noted. Abscesses within the gland
may be noted with a uniform distribution, unlike focal appearance, malignant transformation may be suspected.
abscesses caused by other types of infections. However, Foci of calcification within the glands are not uncommon.
sialography is an invasive procedure and other imaging Heterogeneous enhancement may therefore be expected
modalities may be considered. (Figure 9A, B). The glands could eventually appear smaller
Imaging with CT and MRI is common. The glands in size. In MRI, numerous punctuate areas appear within
enlarge with time, assuming a denser appearance on CT. the glands with low signal intensity on T1 and T2 weighted
A honeycomb appearance is not infrequent but this is also images as the disease progresses. This is considered diag-
seen with granulomatous conditions. Bilateral enlarge- nostic of the condition. But, a lymphoma arising in a
ment with cystic and solid lesions is noted (Figure 8A, B). benign lymphoepithelial lesion (BLEL) does not have char-
In the early stage, the parotids appear normal. Multiple acteristic features that would help differentiate it from
cysts appear during the intermediate stage. These eventu- other tumors. However, it may be considered in patients
ally grow with time. If the mass assumes an invasive who present with a parotid mass. Function of the glands is
273
directly correlated with the amount of fat deposition, of lymphadenopathy helps exclude HIV-related lympho-
thereby indicating that a monitoring of this feature may epithelial cyst formation.
be useful in diagnosing the condition. Another imaging Another test to confirm altered salivary gland function
technique that is useful is magnetic resonance sialography. is whole unstimulated sialometry. Sialometry is the mea-
It has been shown to be highly accurate with excellent surement of salivary flow rate. It is instrumental in reach-
sensitivity and specificity. Globular, punctuate or a lytic ing a diagnosis of hyposalivation (below normal salivary
appearance is typical of the condition. If cysts develop flow rate), a common finding in patients with SS. In an ideal
within BLEL, these are detected using CT or MR. The absence situation, patients should not eat, drink, smoke, or brush
their teeth for 90 minutes before the sialometric assess-
ment. The patient is asked to expectorate into a preweighed
Figure 7 container, while sitting upright for 15 minutes. After re-
weighing the tube post-collection, dividing by 15 and apply-
ing the conversion factor (1 g ⫽ 1 ml), a flow rate can be
determined. Unstimulated salivary flow ⱕ 1.5 ml/15 min or
⬍ 0.1 ml/min is considered consistent with Sjögren’s diag-
nosis. After an SS diagnosis has been established, periodic
flow rates (every 3–6 months) may provide meaningful
information to assess disease progression. Subsequent sal-
ivary assessments should be collected at the same time
each sampling, preferably either 9:00 AM–noon or 1:00
PM–3:00 PM, to avoid fluctuations due to circadian rhythm
of salivary secretion and composition. Often, an SS patient
will be prescribed a sialogogue to enhance salivary flow.
Periodic flow rates are helpful to track medication efficacy
as well.
Lymphocytic infiltration in the lacrimal and salivary
glands is a major feature of SS. One of the criteria for clas-
sification of SS includes the biopsy of the labial salivary
Sialograph of Sjögren’s syndrome showing atelectasia.
glands. A 1.5-cm incision is made on normal appearing
Courtesy: Dr James Pettigrew
mucosa of the lower lip lateral to the midline (Figure 10).
Figure 8
A B
Sjögren’s syndrome. CT images showing diffuse sialadenitis affecting the submandibular and parotid glands bilaterally.
Courtesy: Dr Madhu Nair
274
Figure 9
A B
Chronic Sjögren’s syndrome. Contrast CT demonstrating a granular appearance of parotid glands that have been
reduced in size. Courtesy: Dr Madhu Nair
and are found throughout the glands. A finding of more

Figure 10
than one focus within a 4 mm2 area of glandular tissue is
supportive of a SS diagnosis. Manthorpe et al have reported
an interesting fact that the focus scores are lower in SS
patients who are cigarette smokers.
While the labial salivary gland biopsy is widely consid-
ered one of the best diagnostic tools for SS, it is not 100%
reliable. Personal experience has shown that minor sali-
vary gland histopathology may not present the classic pic-
ture, yet be supportive of an SS diagnosis. Some SS patients
may show areas of classic patchy lymphocytic foci with
other areas more consistent with chronic sclerosing sialad-
enitis in confirmed primary SS patients. In addition, sali-
vary gland biopsies of patients with long-standing SS
demonstrate areas of fibrosis more prominently than the
classic criteria allow. In more chronic cases, repeated biopsy
attempts reveal only fibrosis and no viable labial salivary
gland lobules. Sampling and timing of the biopsy may be
more critical than previously considered.
Sjögren’s labial salivary gland biopsy procedure.
Courtesy: Dr Carol Stewart
Laboratory diagnosis values
Patients presenting with signs and symptoms of SS should
have an appropriate laboratory assessment. Common find-
Five or more accessory salivary gland lobules are examined ings include a positive rheumatoid factor (RF). RFs are
histopathologically for the presence of focal chronic autoantibodies against antigenic determinants that are
inflammatory aggregates. A focus is 50 or more lympho- present on the Fc portion of human IgG, and are found in
cytes and some plasma cells within a 4 mm2 field in the sera and saliva of 60–80% of patients with primary SS.
salivary gland biopsy specimen. As noted in Figure 11A, B, A positive RF does not confirm that the patient has rheu-
these aggregates are adjacent to normal-appearing acini matoid arthritis. Positive antinuclear antibodies (ANA) are
275
Figure 11
A B
Sjögren’s labial salivary gland histopathology (5⫻ and 40⫻). Courtesy: Dr Carol Stewart.
(A) Magnification 5⫻; (B) magnification 40⫻
found in most patients, particularly anti-SS-A/anti-Ro and includes minimizing carbonated drinks and avoiding snack-
anti-SS-B/anti-La. Reports have demonstrated that precipi- ing on sticky sugary processed foods. Salivary stimulants
tating autoantibodies to Ro and La occur in patients with such as sugar-free gums and lozenges will assist in enhanc-
SLE, but are more prevalent in primary SS, occurring in ing the salivary flow. If available, xylitol sweetened gums
60–80% (anti-Ro) and 40–60% (anti-La) of SS patients. and lozenges are beneficial due to their reported anticario-
genic effects. Prescription sialogogues, such as pilocarpine
and cevimeline can be very helpful as long as functional
Management
salivary gland tissue remains. However, prescription sialo-
The patient with SS must be treated by a multi-specialty gogues are not recommended for all SS patients. These
team for an optimal outcome. The rheumatologist will should not be used in patients with uncontrolled asthma
monitor the inflammatory components related to core dis- or narrow-angle glaucoma, and should be used with cau-
ease and any extraglandular manifestations. The ophthal- tion with certain types of cardiovascular disease, eye, lung,
mologist will manage the ocular health of the patient on and liver conditions. Consultation with the patient’s rheu-
a routine basis. A variety of treatment modalities might be matologist before prescribing these medications is prudent
appropriate to help maintain ocular secretions, such as to confirm the lack of contraindications. Not only are SS
lachymal duct plugs and laser occlusion. Prescription eye patients more susceptible to dental decay and candida,
drops containing 0.05% cyclosporine emulsion may help their periodontal status should be monitored as well. While
some patients with ocular moisture. Many patients use over- some reports indicate no significant difference between
the-counter artificial tears as well. As many SS patients the periodontal status of SS patients and controls, others
seem to be sensitive to agents in cosmetics and preserva- report more severe periodontitis in SS patients. Patients
tives in over-the-counter products, hypoallergenic products should be given all assistance possible to maintain their
may be preferred. dentition in the optimal condition. As many of these
The dental management for patients with SS is critical patients are taking bisphosphonates for osteoporosis, the
to their long-term oral health. Patients demonstrating option of implant replacements for lost teeth, or implant
hyposalivation may also demonstrate increased suscepti- supported dentures is one that should be approached with
bility to dental decay. Follow-up visits every 4 months utmost care and patient’s informed consent. The potential of
may assist in monitoring and treating active dental caries. bisphosphonate-associated osteonecrosis should be reviewed
Patients should be educated and empowered to assist in with the patient prior to extractions, implants, or any oral
the management of their own oral health. Artificial saliva surgical procedures.
substitutes and oral hygiene products that contain lacto- While SS often follows an indolent course, of critical
peroxidase and lysozyme (e.g. Biotene® toothpaste and importance is the concern for development of lymphoma.
mouthrinse) may be helpful. Meticulous home care with Kassan et al reported that SS patients have 40 times higher
a home fluoride program customized for the patient is risk of development of lymphoma than the normal popu-
essential. Nutritional counseling might be helpful, which lation. Lymphoma has been reported by Voulgarelis et al
276
immunosuppressives are reserved for treatment of severe

Figure 12
extraglandular manifestations of SS. Anti-B-cell therapy
is a new potential therapy for the glandular and extra-
glandular manifestations in addition to the management of
lymphoma associated with SS. Gene-transfer modalities
used in animal models continue to show future promise.
The dentist should be an integral part of the medical man-
agement team, along with the rheumatologist and oph-
thalmologist, for optimal patient care.
Benign Lymphoepithelial Lesion

(Mikulicz’s Disease)
In the late 1800s, Johann von Mikulicz-Radecki described
a patient with an unusual bilateral painless swelling of
the lacrimal glands and all the major and minor salivary
Palatal MALT lymphoma and carious roots in glands. Histopathologic examination showed an intense
Sjögren’s syndrome patient. Courtesy: Dr Carol Stewart
lymphocytic infiltrate, with features that were recognized
as the BLEL. The clinical presentation became known as
Mikulicz’s disease and the term was used to describe cases
in 4.3% of patients with SS. These tumors may arise in the of bilateral parotid and lacrimal enlargement. With time,
salivary gland or within lymph nodes. What was consid- the histopathological diagnosis of these cases proved to
ered a BLEL in the past might currently be diagnosed as a be inconsistent with Mikulicz’s disease. The clinical pic-
low-grade non-Hodgkin’s B-cell lymphoma of the mucosa- ture of symmetrical lacrimal and salivary gland enlarge-
associated lymphoid tissue (MALT) lymphoma. Figure 12 ment was attributed to other diseases such as tuberculosis,
demonstrates a patient with SS demonstrating a bluish sarcoidosis and lymphoma. Consequently, these cases were
area in the hard palate, which was diagnosed as BLEL. In later described as Mikulicz’s syndrome, restricting the
addition, SS patients may develop caries that involve root term Mikulicz’s disease for use with a histopathologic
surfaces. MALT is normally found in Peyer’s patches in the diagnosis of BLELs. Over the years, the use of these terms
ileum of the lower gastrointestinal tract, where it plays a became interchanged and confusing, so their use has been
role in normal humoral immune response. Mononuclear discouraged.
cell lymphocytic infiltrates in the exocrine glands can
develop into marginal zone B cell lesions or acquired Clinical features
MALT. A recent enlargement or persistent parotid enlarge- Mikulicz’s disease is reportedly characterized by symmetric
ment could be a lymphoma. MALT lymphomas have even and persistent swelling of the lacrimal glands and either or
been discovered though SS labial salivary gland biopsies. both of the major salivary glands (parotid and subman-
Patients with SS have an increased risk of developing dibular) and the exclusion of other diseases that may mimic
monoclonal B-cell MALT lymphoma due to perhaps pro- this presentation, such as sarcoidosis, viral infection or
longed autoimmune inflammation or persistent antigenic lymphoproliferative disorders. Many cases of BLELs were
stimulation to virus or bacteria. Occasionally, high-grade a component of SS. The lymphoepithelial lesion was com-
lymphomas develop which can demonstrate aggressive monly found in adults, primarily women, and usually in the
behavior. A patient demonstrating a persistent swelling of parotid gland. The affected gland was diffusely enlarged,
the major salivary glands or lymph nodes, or sudden asymptomatic or mildly painful.
parotid gland enlargement or pain should be evaluated to
rule out possible lymphoma.
Diagnosis
Currently, SS patients suffer from diminution in quality
of life resulting from the sicca complex, extraglandular Microscopic examination demonstrates a heavy lympho-
manifestations, and the depression from disease chronicity cytic infiltrate associated with the destruction of the sali-
and anxiety associated with development of lymphomas. vary acini. The ductal epithelial cells and surrounding
Educating patients and helping them to understand their myoepithelial cells become hyperplastic, and form groups
disease and empowering them with knowledge is an impor- of cells known as ‘epimyoepithelial islands’. These islands,
tant management strategy. The prognosis for SS patients once considered benign, are currently recognized to be
may improve as greater understanding of the pathogenesis indicative of low-grade salivary lymphoma of the MALT.
is achieved through continued research. Currently, systemic A review of Mikulicz’s initial case report concluded that
277
Mikulicz actually reported the first case of MALT lym- Patients reporting to the dental office will present due
phoma, based on the published histopathology. The authors, to concern about the unknown condition which carries
Ihrler and Harrison further urged that the terms Mikulicz’s significant social stigmas. The patient may bring their ‘spit
disease and Mikulicz’s syndrome should no longer be used. cup’ with them and describe their saliva as ‘foamy’. Several
Conversely, a recent report by Yamamoto et al concluded conditions should be considered.
that Mikulicz’s disease is a distinct entity and different Minor sialorrhea may be associated with minor oral
from SS both clinically and histopathologically. Addition- irritations and ill-fitting or new dentures. Episodic sialor-
ally, the authors reported that Mikulicz’s disease was an rhea may be a subtle manifestation of gastroesophageal
IgG4-related systemic disease. reflex disease (GERD). Excessive saliva is produced as a
protective buffering mechanism in patients with GERD.
Treatment This is called ‘water brash’. A similar condition of sialor-
rhea with unknown etiology termed ‘idiopathic paroxys-
The affected gland must be surgically removed. Fortunately, mal sialorrhea’ is reported to consist of episodes of
most MALT lymphomas are low-grade tumors that tend to increased salivary flow occurring 1 or 2 times per week at
remain localized with good survival rates. Occasionally, 2–5 minutes in duration. The episodes are preceded by a
tumors transform to high-grade lymphomas with aggressive prodrome consisting of nausea or epigastric pain, but
behavior. without progression to vomiting. These may be variants of
the same condition. Sialorrhea may be associated with
esophageal obstruction (foreign body, cancer, or stricture
Sialorrhea formation), infection, and nasogastric intubation with
symptomatic sequelae of sialorrhea.
Clinical features
Sialorrhea or ptyalism is a condition characterized by Treatment
increased salivary flow. Sialorrhea can occur with various
neurologic disorders, infections, the secretory phase of Treatment of sialorrhea depends on its etiology. Some
the menstrual cycle, heavy metal poisoning, Wilson dis- causes of mild or transitory sialorrhea may need no treat-
ease, paroxysmal sialorrhea, and rabies. Older aged indi- ment. If the salivation is believed to be due to GERD,
viduals in chronic care facilities and chronically debilitated referral to their physician for evaluation and treatment
with cerebrovascular accident may demonstrate chronic would be appropriate. If the condition is due to inadequate
drooling. motor control, speech therapy might improve the situa-
Parkinson’s disease (PD), amyotrophic lateral sclerosis tion, if the patient is able to cooperate. Botulism toxin
(ALS), and cerebral palsy are neurodegenerative diseases injected into the parotid gland has been reported to have
associated with sialorrhea. The appearance of excess saliva efficacy for ALS and PD as reported by Lagalla et al and
in neuropathologic conditions may be due to excessive Contarino et al. Surgical techniques might be needed to
saliva, but is usually related to impaired cerebral control modify the salivary glands or ductal structures. A recent
of orofacial function. Weakness of the facial and perioral report by McAloney et al showed efficacy and safety from
muscle tone inhibits the normal retention, movement, bilateral submandibular duct relocation and bilateral sub-
and/or swallowing of saliva. Excessive salivation has been lingual gland excision. The prognosis of sialorrhea will
reported in familial dysautonomia (FD) due to subman- vary with the degree to which the causal factors can be
dibular and sublingual salivary gland hyperactivity. These managed.
changes may be the result of ongoing parasympathetic
denervation characteristic in FD. The consequences of
‘drooling’ are not restricted to medical issues, but can INFLAMMATORY CONDITIONS OF
cause major social handicaps. Severe psychosocial conse- SALIVARY GLANDS
quences and social stigmatization may be emotionally
devastating for patients and families. Drug-induced sialor-
Inflammatory conditions are the most common pathol-
rhea has been reported as well. Major medication groups
ogy affecting the salivary glands. Dentists should be famil-
associated with drooling are anti-psychotics, particularly
iar with their clinical manifestations and recommended
clozapine, and direct and indirect cholinergic agonists that
treatment.
are used to treat dementia of the Alzheimer’s type and
myasthenia gravis. Other drugs cited include risperidone,
lithium and digoxin. Heavy metal toxins, such as mercury
Mucocele
and thallium produce sialorrhea as does exposure to irre-
versible acetylcholinesterase inhibitors such as insecticides The mucocele is a common lesion that results from rupture
and nerve agents. of a salivary gland duct and spillage of mucin into the
278
surrounding tissues. For that reason, the term ‘mucus extra- and plasma cells. The lumen is filled with an eosinophilic
vacation’ phenomenon is used to describe this lesion. The coagulum containing inflammatory cells.
rupture of the gland or duct may be due to local trauma,
but many cases develop without a history of trauma. These Treatment
will be found most frequently in the lower lip.
Treatment of the mucous retention phenomenon is exci-
sion. If only incised, the contents will be released, but the
Clinical features
lesion may recur when the area has healed.
Mucoceles typically present as fluctuant, non-ulcerated
dome-shaped mucosal swellings that range from 2 mm to
several centimeters in size. Mucoceles have been reported Ranula
in patients of all ages. The lesions typically have a bluish Clinical features
translucent hue due to the spilled mucin under the tissue
surface (Figure 13). Deep mucoceles may appear normal in ‘Ranula’ is the term used for mucoceles that occur in the
color, and may feel firmer to palpation than superficial floor of the mouth in association with ducts from the sub-
ones. Duration may be days to years. Patients will often mandibular or the sublingual gland. Generally these are
report that the lesion intermittently gets larger, and then larger than mucoceles occurring in other locations and can
shrinks. This history is consistent with the nature of the elevate the tongue. The ranula is usually located lateral
lesion which will enlarge, sometimes during eating, and to the midline and appears as a dome-shaped fluctuant
then spill contents into the surrounding tissue, and even- swelling in the floor of the mouth as seen in Figure 14.
tually shrink in size. The most common location is the The color may be translucent blue or normal in color if
lower lip, but mucoceles may also be found in the buccal deep seated. A rare plunging type that has herniated
mucosa, anterior ventral tongue and floor of the mouth through the mylohyoid muscle has been described by
(ranula). Davison et al.
Histopathology Histopathology
They consist of a circumscribed cavity in the connective The histopathology of a mucocele and ranula are similar,
tissue, producing an elevation of the mucosa with thinning however, the ranula may be a true cyst with an epithelial
of the epithelium. The cavity wall is made up of a lining of lining. Spilled mucin may elicit a granulation tissue
compressed fibrous connective tissue and fibroblasts. The response that contains foamy histiocytes.
connective tissue wall may demonstrate granulation tissue
infiltrated by polymorphonuclear leukocytes, lymphocytes Treatment
Treatment of a ranula includes unroofing the lesion, exci-
sion of the lesion, or removal of the sublingual gland.
Figure 13
Figure 14
Mucocele of the lower lip. Bluish dome-shaped lesion of

short duration on the mucosal aspect of lower lip. Ranula. Courtesy: Dr Praveen, Department of Oral Medicine,
Courtesy: Dr Indraneel Bhattacharyya KLE Institute of Dental Sciences, Bangalore
279
Sialolithiasis, Salivary Duct Stone, Figure 15

Salivary Calculi
Clinical features
Sialoliths are calcified bodies that develop within the sali-
vary gland or ductal system. These are one of the most
common salivary gland conditions. These are believed to
develop from deposition of calcium salts around a focus
of material within the duct lumen. Solitary or multiple sialo-
liths can form. The initiating focus may consist of desqua-
mated epithelial cells, bacteria, foreign bodies, or mucus.
Sialoliths most commonly occur in the submandibular
gland (80–90%), but may develop in the parotid and sub-
lingual gland as well. Multiple stones are more common in
the parotid glands than other major glands. Minor gland
calculi are occasionally seen in labial glands and buccal
mucosa. These usually form in young and middle-aged Occlusal radiograph of sialolith in submandibular gland.
adults, but may develop at any age. The classic presenta- Courtesy: Dr Carol Stewart
tion is an intermittent postprandial salivary gland swell-
ing that gradually subsides over the next 2–3 hours.
Patients report moderately severe pain, just before, during
and after meals due to stimulation of salivary flow and the
pressure produced against the occluded duct. Recurrent Figure 16
and chronic obstruction causes stasis, inflammation, and
infection, which can result in persistent enlargement.
Sialoliths may be round or elongated and measure from
2 mm to 2 or more centimeters in diameter. The involved
duct may contain a single stone or multiple stones. Upon
excision and gross examination, these appear yellow in
color. The cause is uncertain, but sialolith formation can
be promoted by chronic sialadenitis and partial duct
obstruction.
Diagnostic procedures
Conventional radiographs can successfully image most
sialoliths as they appear as radiopaque masses. Smaller
sialoliths that are not fully calcified pose a diagnostic chal-
lenge. Underexposed radiographs can sometimes demon-
strate the presence of the sialolith. Occlusal films help
identify stones in the submandibular gland as shown in
Figures 15 and 16. If a panoramic film is generated, multi-
ple calcifications may be identified as shown in Figure 17. Occlusal radiograph of sialolith in submandibular gland.
It is possible for multiple calcifications to appear superim- Courtesy: Dr James Pettigrew
posed on the mandible and mimic a bony lesion. A sialo-
graph of Wharton’s duct depicts ductal enlargement
proximal to the sialolith in Figure 18. However, Rabinov
and Weber report that up to 20% of salivary calculi are facilitate retrograde spread of any existing bacterial infec-
radiolucent. If not calcified, the stones may not be evident tion as a result of stasis. In addition, sialography can cause
on these films. Non-contrast CT is often considered the increased pain when acute sialadenitis exists. Dormant
best single modality for the diagnosis of calculi. Most stones infections can get worse after sialography; thus necessi-
not seen on conventional radiographs can be detected by tating the use of antibiotics. Contrast CT examination, on
CT. Sialographs are useful in detecting non-calcified sialo- the other hand, can image non-calcified sialoliths if these
liths, but sialography is an invasive procedure with poten- are larger. In Asia and Europe, ultrasound imaging is the
tial to dislodge the stone deeper into the gland, as well as mainstay of imaging.
280
invasive treatment modality that does not require removal of

Figure 17
the gland for sialolithiasis. Commonly associated with sial-
olithiasis is acute or chronic sialadenitis, which is described
in the next section.
Sialadenitis
Sialadenitis or an inflammation of the salivary glands
may arise from a variety of infectious and non-infectious
conditions. Non-infectious causes of salivary gland dys-
function include disorders such as SS, sarcoidosis, radia-
tion therapy, medications, and congenital anomalies were
discussed in the previous sections. The infectious causes,
which will be discussed in the following sections, com-
monly include bacterial and viral infections. Salivary
Panoramic film of multiple sialoliths in parotid gland. stones or sialoliths are also associated with salivary gland
Courtesy: Dr James Pettigrew dysfunction of the major and minor glands. Conditions
that predominately affect the minor salivary glands, such
as cheilitis glandularis and necrotizing sialometaplasia
will be reviewed as well.
Figure 18
Non-specific Sialadenitis
Non-specific sialadenitis may manifest as chronic paroti-
tis, also called chronic recurrent parotitis. The cause of the
parotid gland enlargement may be multifactorial and
include decreased salivation either from decreased pro-
duction or decreased secretion, stasis and an ascending
retrograde infection. Sometimes, no predisposing factor is
identified. This entity is usually unilateral, painful (mild to
severe), and characterized by intermittent exacerbations of
swelling and remission. Massaging or milking the gland
will reveal cloudy or purulent secretions. In a large series,
Bhatty et al reported that the mean age was 46 years,
with mean duration of symptoms of 4.6 years. The swell-
ing may last for several hours or several weeks. The condi-
Sialograph of submandibular gland showing dilatation of tion establishes a cycle of blockage and infection, which
Wharton’s duct proximal to sialolith. Courtesy: becomes self-perpetuating. Fever and malaise may be
Dr Ajit Auluck and Dr Chandrakant Shetty present. Remissions last from weeks to years. The extent of
the gland destruction may increase over time with each
episode as does the pain intensity. The disease tends to
progress and may lead to the formation of a fibrous mass
Histopathology
in the affected gland.
The histopathologic features include a calcified mass that
exhibits concentric laminations surrounding a central focus Diagnostic imaging
of amorphous debris. The associated duct will demonstrate
Sialography will demonstrate the parotid duct system. A
squamous, oncocytic or mucous cell metaplasia.
‘sausage-like’ pattern reflects areas of duct wall dilatations
and stricturing that result from the effects of the ascend-
Treatment
ing bacterial infection. Because sialography may result in
Small stones may be removed by massaging and manipula- retrograde spread of infection into the gland, other imag-
tion to move the stones toward the duct orifice or peripheral/ ing modalities are preferred. CT scan may show increased
transoral ductotomy. Large stones may require excison of density due to the normal parotid gland fat being replaced
gland and/or associated duct. Lithotripsy has been tried with by inflammation and fibrosis. Contrast CT will show
limited success. Sialoendoscopy provides a new minimally enlargement of the gland and its duct in the absence of
281
a causative agent such as a sialolith. Figure 19 shows uni- involvement of the gland with areas of destruction. Acinar
lateral gland and duct enlargement of the right subman- loss and fibrosis will be present in varying amounts.
dibular gland. Figure 20A–C show non-specific sialadenitis
in the right parotid and submandibular glands. Treatment
Treatments include antibiotic therapy during an acute
Histopathology attack, sialogogues, increased fluid intake and parotid
The histopathology is consistent with non-specific sialad- gland massage. When surgical intervention is appropriate,
enitis with marked infiltrate of inflammatory cells. The a superficial parotidectomy has a high success rate with
histology may show mild chronic sialadenitis or widespread minimal long-term complications.
Bacterial Sialadenitis
Figure 19
Bacterial sialadenitis may arise from a bacterial infection
involving any of the salivary glands. Acute bacterial sia-
ladenitis most commonly occurs in children younger than
2 months and in elderly persons who are debilitated by
systemic illness, or who have had surgical procedures. How-
ever, persons of all ages may be affected. The most com-
mon pathogens are Staphylococcus aureus and anaerobic
bacteria.
Clinical features
Acute sialadenitis is seen in the parotid gland and is
bilateral in 10–25% of case. The affected gland is swollen
and painful, and the overlying skin may be erythematous.
The patient may present with a low-grade fever and tris-
mus. Milking the gland may reveal a purulent discharge.
Acute suppurative sialadenitis is most frequently due to
S. aureus, but also may arise from streptococci, including
Unilateral enlargement of the right submandibular Streptococcus pneumoniae and Streptococcus pyogenes and
gland and duct, consistent with low-grade sialadenitis.
gram-negative aerobic bacilli including Escherichia coli.
A study of acute sialadenitis from all three major glands
Figure 20
A B C
Non-specific sialadenitis noted in the right parotid and submandibular glands. Courtesy: Dr Madhu Nair
282
with 47 specimens as reported by Brook revealed a broad necrosis. The ducts tend to be atrophic and do not show
spectrum of microbes present in the purulent fluid. The pre- squamous metaplasia characteristic of necrotizing sialo-
dominant aerobes were S. aureus and Hemophilus influen- metaplasia. Based on the clinical and histopathologic fea-
zae, while anaerobes were gram-negative bacilli, including tures, some prefer to consider SANS a separate non-specific
Prevotella, Porphyromonas, Fusobacterium and Peptostrepto- inflammatory condition of the minor salivary glands with
coccus species. unknown etiology.
Histopathology Treatment
The diagnostic features of acute sialadenitis will include Subacute necrotizing sialadenitis is self-limiting and heals
neutrophils within the ductal system and acini. within 2–3 weeks. It generally requires no treatment.
Diagnosis
Cheilitis Glandularis
A history of preceding events may aid in making the diag-
nosis. Predisposing factors can include dehydration, malnu- The term ‘cheilitis glandularis’ was first used by Volkmann
trition, immunosuppression, dental infection, anticholinergic in 1870 to describe a disorder that presented with a
medications, tracheotomy, sialectasis, ductal obstruction, chronic, suppurative inflammation of the lower lip charac-
neurosurgical, and abdominal surgical procedures. terized by swelling of the mucous glands, dilated open-
ings, and mucopurulent discharge. Cheilitis glandularis is
an uncommon inflammatory condition of the minor sali-
Treatment
vary glands, and most commonly affects the lower lip of
Treatment for acute sialadenitis includes appropriate anti- adult males. The etiology is unknown, however associated
biotic therapy based on culture and sensitivity findings. factors have been suggested. These factors include tobacco,
Rehydration is important to improve salivary flow. If an poor oral hygiene, bacterial infections, possibly heredity,
abscess has formed, surgical drainage may be required. If and actinic damage.
the condition is severe, surgical removal of the gland may
be necessary. In debilitated patients, the condition may be Clinical features
fatal due to spread of infection and sepsis. Prompt recog-
nition by the dentist with appropriate referral otorhino- Cheilitis glandularis most commonly affects the lower lip,
laryngologist for definitive treatment is essential for a but it has been reported in the upper lip, palate, and buccal
good outcome. mucosa. Affected individuals experience swelling and
eversion of the lower lip as a result of hypertrophy and
inflammation of the glands as seen in Figure 21. The open-
Subacute Necrotizing Sialadenitis ings of the minor salivary ducts are inflamed and dilated,
and pressure on the glands may produce mucopurulent
Clinical features secretions emanating from the ductal openings. The con-
Subacute necrotizing sialadenitis (SANS) is a form of sali- dition most frequently occurs in middle-aged men, but
vary inflammation that occurs most commonly in young women and children have been reported as well.
adults and teens. The lesion involves minor salivary glands Historically, cheilitis glandularis has been classified into
of the hard and soft palate, and presents as a localized three types, based on the severity of the disease. These are
palatal swelling covered by erythematous, intact mucosa. (i) simple, (ii) superficial suppurative (Baelz’s disease), and
The nodule may be accompanied by abrupt onset of pain. (iii) deep suppurative (cheilitis glandularis apostematosa).
An infectious or allergic origin has been suggested. Some The superficial suppurative type demonstrates painless
diagnosticians have questioned whether SANS represents crusting, swelling, and induration of the lip with superfi-
a separate entity or part of the spectrum of necrotizing cial and deep ulceration. This type seems to be the result of
sialometaplasia. Unlike necrotizing sialometaplasia, SANS secondary infection of the simple type. The deep suppura-
does not ulcerate or slough necrotic tissue. tive type, also known as cheilitis glandularis apostematosa,
myxadenitis labialis, or cheilitis glandularis suppuritiva
profunda, is a deep-seated infection associated with abscess
Histopathology
formation and spontaneous expression of suppurative mate-
Subacute necrotizing sialadenitis is characterized by a rial from the ducts. The latter two types represent prog-
heavy mixed inflammatory infiltrate consisting of neutro- ressive stages of the diseases with bacterial involvement
phils, lymphocytes, histiocytes, and eosinophils. There is and demonstrate increased inflammation, suppuration, and
an absence of acinar cells and those present may exhibit ulceration of the lip.
283
Figure 21 Figure 22
Necrotizing sialometaplasia of the palate. A well-defined ulcer

that began after 1 week of seating porcelain fused to metal
(PFM) crown on tooth number 3. Patient reported mild
Cheilitis glandularis. Courtesy: Department of Oral Medicine, discomfort and that the lesion was progressively getting
MCODS, Mangalore larger. Courtesy: Dr Indraneel Bhattacharyya
Histopathology as a true malignant condition. Radiation therapy was used

in the past, but is no longer recommended due to associ-
Histopathologic features consist of non-specific chronic ated complications.
inflammation, dilated secretory ducts, dilated ducts con-
taining mucin, areas of fibrosis, and areas of chronic scle-
rosing sialadenitis. The ductal lining may show oncocytic Necrotizing Sialometaplasia
metaplasia and atrophy of the acini. Concomitant dysplas-
Clinical features
tic changes may be seen in the surface epithelium.
Necrotizing sialometaplasia is an uncommon, benign, but
Diagnosis locally aggressive inflammatory lesion of salivary gland
tissue which both clinically and histologically may mimic
The differential diagnosis for cheilitis glandularis would
a salivary gland malignancy. In 1973, it was described by
include orofacial granulomatosis and multiple mucoceles.
Abrams and colleagues as a reactive necrotizing inflam-
Orofacial granulomatosis, which will be described in the
matory process of the minor salivary glands of the hard
next section, is a non-tender, persistent swelling of the lips
palate. While the etiology is unknown, the prevailing the-
usually with oral ulcerations. Histologic findings would
ory is that local ischemia of the salivary tissue leads to
include non-caseating giant cell granulomas. Definitive
local infarction. Most patients are in the 4th or 5th decade,
diagnosis of cheilitis glandularis requires a biopsy along
but the lesion has been reported in all ages, except chil-
with the clinical picture.
dren. In a large series as reported by Brannon et al, males
were affected more often than females, and whites were
Treatment
affected more commonly than African-Americans by a
The treatment for cheilitis glandularis may vary depend- ratio of 5:1. Most lesions occur in the posterior hard pal-
ing on the severity of the condition. It may be treated with ate, usually unilateral (Figure 22). Bilateral and midline
lip balms, sunscreens, topical steroids, intralesional ste- lesions may occur as well. Other intraoral sites such as the
roids, systemic antihistamines, and/or antibiotics. If conser- retromolar pad, buccal mucosa, lower lip, and tongue have
vative therapy fails, surgical resection or vermillionectomy been affected. Early lesions may present as a non-ulcerated
may be indicated. A significant percentage of cases of deep swelling in the posterior palate with or without pain.
suppurative type have been associated with the development Within 2–3 weeks, the necrotic tissue sloughs and leaves
of squamous cell carcinoma of the overlying epithelium. a crater-like ulcer ranging from 1 cm to more than 5 cm.
Because actinic damage has been implicated in many cases of Commonly, patients do not seek treatment until the ulcer
cheilitis glandularis, malignant degeneration could be asso- occurs. They may report a feeling of fullness in the area,
ciated with susceptibility to environmental factors, espe- prior to the ulceration. Pain may or may not be a complaint
cially sun damage versus considering cheilitis glandularis even though the ulcer may be quite large.
284
Diagnostic imaging Diagnostic imaging

A palatal soft tissue attenuation focus with no character- Ultrasound imaging results in the gland appearing
istic appearance may be noted on CT and MRI. Osseous enlarged and lobular with a cirrhotic pattern of numerous
changes are not noted. Lesions in the parotid are also some- hypoechoic regions against a heterogeneous background.
times seen. Ultrasonography may detect multiple foci of The presence of lymph node enlargement is sometimes
hypoechoic nature within the parotids. Contrast CT dem- noted on CT/MRI. Diffuse enlargement of the gland with
onstrates high attenuation areas within the parotids while enhancement may be noted on CT.
MRI can clearly show well-delineated masses that are hypo-
intense on T1 and isointense on T2 weighted contrasted Treatment
images. Findings could resemble those of benign tumors The treatment is surgical removal.
such as pleomorphic adenomas. Alternatively, a more dif-
fuse margin on CT can be misconstrued for a malignant
tumor. The condition can also appear in other locations VIRAL-INDUCED SALIVARY GLAND
within the sinonasal and upper aerodigestive tracts. PATHOLOGY
Histopathology
Mumps
Biopsy is necessary to confirm the diagnosis and rule out
Clinical features
malignant disease. The histopathology includes ulcerated
mucosa, pseudoepitheliomatous hyperplasia of the epithe- Epidemic parotitis or mumps is a moderately infectious
lium, acinar necrosis, and squamous metaplasia of salivary disease caused by a virus of the paramyxovirus group.
ducts. Coagulation necrosis has been seen in early lesions. Generally, mumps is a disease of childhood with an incu-
Inflammatory cells may be found with fibrosis and granu- bation period of 2–3 weeks. The virus may be in the saliva
lation tissue. While mucous cells are necrotic, the lobular of affected persons and dissemination through droplets is
architecture of involved glands is preserved. The lesion common. The patient will complain of fever, fatigue, and
can be misdiagnosed as squamous cell carcinoma or present with a painful unilateral, or more commonly, bilat-
mucoepidermoid carcinoma in the absence of an adequate eral parotid enlargement. The infection may also involve
representative section of the lesion. the submandibular gland. Clinical features include a flu-
like illness with fever, headache, vomiting, and pain below
Treatment the ear. This is followed by firm, somewhat rubbery swell-
ing of the salivary glands, sometimes elevating the ears.
Once the diagnosis has been established, no specific treat- The glands are extremely tender to palpation and milking
ment is indicated. The lesion will heal by secondary inten- them may produce a thick white secretion from Stensen’s
tion with no intervention within 4–10 weeks. ducts. The swelling may last approximately 1 week. Other
organs may be involved which include the testes, ovaries,
Chronic Sclerosing Sialadenitis (Kuttner Tumor) pancreas, and mammary glands.
Clinical features Prognosis and treatment

The Kuttner tumor, a chronic inflammatory condition of When the disease affects the adult male, orchitis, inflam-
the salivary glands, was first described by Kuttner in 1896. mation of the testicles, is a complication approximately
Clinically, the condition cannot be distinguished from a 20% of the time. The orchitis is usually unilateral, but can
true neoplasm. The submandibular gland is affected more occur bilaterally. Even with involvement of both testicles,
commonly than any other salivary gland. It is usually sterility is only a rare complication of orchitis. In adults,
localized to the superficial aspect of the submandibular dyspnea secondary to severe swelling of the salivary gland
gland, but may involve the deep aspect. It manifests as a which required a tracheostomy, has been reported by
firm, enlarged gland. Etiology may be sialolithiasis (about Ishida et al. The mumps vaccine has been available since
one-third of all cases), autoimmune sialadenitis or idio- 1968, which has resulted in a marked decline in the inci-
pathic. Recent reports suggest that pathogenesis probably dence of the disease.
has an immunologic background.
Human Immunodeficiency Virus (HIV)
Histopathology
Clinical features
The histolopathologic features consist of chronic sclerosing
sialadenitis, specifically chronic inflammation and fibro- Benign lymphoepithelial lesions (BLEL) in AIDS or AIDS-
sis. It may be seen with sialolithiasis. related parotid cysts (ARPC) are frequently reported in HIV
285
infected patients, and may develop later in the course of to the increased use of medications to control AIDS-related
the disease. The term ‘ARPC’ is preferred to help differenti- infections. Bilateral enlargement in imaging studies is more
ate between AIDS-related lesions and BLELs. HIV-positive common even in the absence of such clinical findings.
status alone is needed to manifest with BLEL and therefore, On T1 weighted images, cysts may appear with low sig-
the term ‘BLEL-HIV’ is most appropriate. Approximately 5% nal intensities. Inhomogeneity of lesions is noted some-
of patients with HIV-1 develop parotid enlargement. These times, primarily with solid lesions. On T2 weighted images,
cysts can be single or multiple and usually involve the the cysts appear hyperintense and well-defined, as do reac-
superficial lobe of the parotid gland. These are usually bilat- tive lymph nodes.
eral, often cystic, and sometimes accompanied by cervical T1 coronal and axial fast fluid-attenuated inversion-
lymphadenopathy. These may be soft or firm to palpation recovery (FLAIR) MRI images depict bilateral cysts within
and can produce significant cosmetic deformities. Symp- the parotids quite well (Figure 23A, B). On ultrasono-
toms of dry mouth, ocular dryness, and arthralgia can occur, graphic studies, the cystic lesions appear with some inter-
along with normal or reduced salivary flow rates. The nal septations, generating little echo. On the other hand,
pathogenesis of HIV-associated salivary gland disease may solid lesions appear with the same characteristics of solid
include hyperplasia of intra-parotid lymphoid tissue. tumors.
Histopathology Treatment
The AIDS-related swellings reflect the lymphoid hyperpla- Sometimes, patients seek treatment only when the cosmetic
sia or lymphoepithelial cyst formation. The cyst wall may deformity from the enlarged glands becomes very promi-
contain germinal centers and a dense infiltrate of lym- nent. Highly active antiretroviral therapy is an effective
phoid cells. treatment approach. Because HIV-associated salivary gland
disease can clinically resemble SS, the differential diagno-
Diagnostic imaging
sis of bilateral parotid enlargement should include HIV
Contrast enhanced CT or MRI is advised to help delineate infection. While the parotid manifestation is considered to
the internal architectural details. Cystic and solid tumors have a benign course, progression to lymphoma is possi-
can appear within the parotids. Bilateral enlargement is ble. Consequently, periodic monitoring is indicated. Any
noted on advanced imaging modalities with associated change in growth pattern would require biopsy. Rosso et al
cervical and nasopharyngeal lymphadenopathy. Adenoids suggest that annual ultrasonography evaluation should be
and tonsils may appear enlarged as well. However, reac- done to identify possible malignant transformation of
tive adenopathy may not be seen on these studies, owing BLELs in vertically infected children.
Figure 23
A B
Axial and coronal T1 FLAIR MRI images showing cysts in the parotids bilaterally in an HIV-positive individual.
286
NON-INFLAMMATORY CONDITIONS OF Figure 24

SALIVARY GLANDS
Sialadenosis
Sialadenosis (sialosis) is a non-inflammatory, non-neo-
plastic, often recurrent condition which most commonly
manifests as a bilateral enlargement of the parotid and/or
submandibular glands. In a recent report of 65 cases of
sialadenosis as described by Satoh and Yoshirara, age
ranged from 19 to 71 years and the male:female ratio was
5:3. The salivary gland enlargements are usually slow
growing, may be unilateral or bilateral, and are sometimes
associated with pain. The condition is usually associated Perimyolysis. Characteristic pattern of dental erosion of the
with an underlying systemic disorder. These disorders lingual enamel resulting from purging and vomiting
include diabetes, hypothyroidism, pregnancy, alcoholism, associated with bulimia. Courtesy: Dr Carol Stewart
malnutrition, obesity, anorexia nervosa, bulimia, and med-
ications that affect the autonomic nervous system. Treat-
ment depends upon identification of the underlying cause.
As patients with these conditions will commonly present Pathogenesis
to the dental office, an understanding of the causes and
While the specific pathogenesis has not been determined,
effective management strategies is essential for optimal
it is generally accepted that multiple emetic episodes cause
patient care.
an autonomic neuropathy. The enlargement is believed
to affect the autonomic innervation of the salivary acini,
causing disruption of the intracellular secretory cycle.
Anorexia/Bulimia-related Sialadenosis
This may lead to excessive accumulation of secretory
Clinical features granules, with marked enlargement of the acinar cells. Due
to sympathetic nerve dysfunction, zymogen (a precursor
Eating disorders are not uncommon in young women who
of amylase) production and storage may increase. Indi-
seek to be thin or have a more ideal figure. These disorders
vidual acinar cells enlarge because of zymogen granule
include anorexia nervosa and bulimia nervosa. Anorexia
engorgement.
involves deliberate lack of food intake and bulimia includes
binge eating with self-induced purging. It has been esti-
Diagnosis
mated that up to 19% of college-going women have bulimia.
Only 5–10% of people with bulimia are male. Sometimes Because patients with bulimia and anorexia do not fre-
the frequent purging of acidic gastric fluids will produce a quently disclose their condition, a broad medical evalua-
characteristic enamel erosion on the lingual surfaces of tion will be required. The diagnostic assessment would
the maxillary anterior teeth (Figure 24). However, if mea- include tests to rule out SS (ANA, anti-SS-A/Ro, anti-SS-
sures are taken to neutralize the oral pH and maintain oral B/La, rheumatoid factor, hepatitis C virus, complete blood
hygiene, these signs may not be present. count [CBC], serum angiotensin-converting enzyme, immu-
Parotid sialadenosis has been reported to occur in noglobulin disorder [IgA, IgM, albumin, urine Bence Jones
10–66% of people with bulimia. Bilateral parotid gland protein], hepatic disorder [glutamic oxaloacetic transami-
enlargement has been the presenting sign in some cases nase and glutamic pyruvic transaminase], diabetes [fasting
and one report by Mignogna et al also included bilateral blood glucose], and hypokalemia [serum electrolytes]).
palatal minor gland enlargements in addition to the parotid Salivary secretion may be decreased or normal. Sialo-
gland involvement. Schlienger et al reported a case where graphy may be helpful in establishing the diagnosis. Sia-
a 24-year-old female consulted physicians for 3 years for lography may demonstrate a ‘leafless tree’ pattern which is
treatment of a painless parotid swelling which was initially thought to be caused by compression of the finer ducts by
confused with SS. Eventually, bulimia was confirmed. the hypertrophic acinar cells. Splaying of the ducts within
Unilateral parotid gland and submandibular gland swell- the enlarged gland may be seen, with the ducts appearing
ing may also occur in sialadenosis associated with buli- normal. In early stages of the disease however, there may
mia, but less frequently. Bulimics may purge several times be no sialographic changes. CT scan of the parotids may
per day. Emetics, diuretics, and laxatives frequently are show a bilateral enlargement of the parotid glandular
used as adjunctive agents for weight reduction, but may parenchyma with increased glandular density, fibrosis
not be revealed during a routine medical history review. or fatty infiltration that are characteristic of advanced
287
changes. CT is non-specific. The changes could be mis- Treatment

construed for a lipoma; however, the absence of a fibrous
Treatment should focus on the underlying liver disease as
capsule is helpful in differentiating between these condi-
well as the oral complications. When the liver disease is
tions; ultrasound and MRI are useful and non-invasive
managed, the parotid glands may reduce somewhat in
approaches.
size. In addition to sialadenosis, the dentist should also be
aware of salivary changes that increase caries risk suscep-
Histopathology
tibility. The parotid gland flow rate in patients with alco-
The histopathological report of a salivary gland biopsy holic cirrhosis has been reported to be less than flow rates
will reveal hypertrophy of the acinar cells, sometime 2–3 in healthy controls. Diminished buffer capacity and less
times greater than normal size. The nuclei are displaced to attention to oral hygiene will put the patient at increased
the cell base, and the cytoplasm is engorged with zymogen risk for dental decay. In addition, patients who consume
granules. Significant inflammation is not observed. large quantities of wine will have increased risk for dental
erosion due to the erosive potential of wines due to their
Treatment acidity. The pH of wine is reported to range from 3.0 to 3.8
and the pH at which enamel dissolves is reported to be pH
Therapy will include psychological counseling with an
of 5.0 to 5.7. Treatment will include patient education and
eventual goal of cessation of vomiting. This may result in
complete dental evaluation.
a gradual reduction of the salivary gland enlargement.
Pilocarpine hydrochloride drops have shown efficacy in
reducing parotid gland enlargement in bulimic patients. In
Diabetes Mellitus
cases refractory to treatment, parotidectomy may be con-
sidered to improve unacceptable esthetics in patients with Clinical features
bulimia.
Diabetes mellitus is a disorder of carbohydrate metabo-
lism mediated through decreased production of insulin
Sialadenosis Associated with Alcoholic or tissue insensitivity to the effects of insulin, resulting
in hyperglycemia. Two types of presentations are recog-
Cirrhosis
nized, although overlap is seen. Type I is characterized by
Clinical features a lack of insulin production and is commonly diagnosed
during childhood, and Type II may present in obese chil-
Salivary gland enlargement, usually the parotid glands,
dren and adults, due to decreased sensitivity to insulin.
may occur in some patients who ingest large quantities of
The complications include kidney failure, blindness, neu-
alcohol on a long-term basis with resultant liver damage.
ropathies, and atherosclerosis. Oral symptoms may include
The enlargement results from an ethanol-produced periph-
dry or burning mouth, gingival inflammation. Sialade-
eral autonomic neuropathy that causes disordered sali-
nosis has been reported in diabetes mellitus. The sialade-
vary metabolism and secretion. Increased intracytoplasmic
nosis is believed due to an autonomic neuropathy, as in
zymogen granules within the acinar cells are believed the
alcoholism and nutrition-induced sialosis. With auto-
cause of parotid enlargement.
nomic disturbances in sympathetic innervation, alteration
in protein synthesis occurs. Cytoplasmic swelling develops
Diagnosis
from engorgement by intracytoplasmic zymogen gran-
In alcohol associated sialadenosis, 40–60% of the adult ules. As a result, the parotid acini may double in size
patients with hepatic damage presented with parotid (hypertrophy) resulting in clinically obvious parotid gland
enlargement. The histopathology of parotid glands reveals enlargement.
an increase in fatty infiltration, edema and fibrosis without
inflammation. Diagnosis would be assisted by the patient’s Diagnosis
history of alcohol intake. The dentist could request liver
Long-standing, bilateral, painless enlargement of the
enzymes and bilirubin tests to aid in making the diagnosis.
parotid glands may be observed. These are soft in consis-
Elevated levels would prompt the dentist to refer the patient
tency and follow the outline of the parotid glands, with-
to an internal medicine specialist for evaluation and a
out nodularity. Milking the parotid glands may reveal
radiologist for imaging.
fluid of normal volume and consistency. Lower resting
and stimulated saliva flow rates in diabetics versus normal
Diagnostic imaging
controls have been reported as well. Levels of calcium are
On CT, the parotid will demonstrate increased density with increased and salivary magnesium, zinc and potassium
time, as the fat is replaced with increased acinar hypertrophy. are reduced.
288
Diagnostic imaging granulomatous inflammation are found, often surround-

ing vessels. Granulomas contain histiocytes and multinu-
CT scan may show enlarged parotid glands without masses,
cleated giant cells. Special stains for fungal organisms and
but demonstrating decrease in density due to significant
acid-fast bacteria are negative. No foreign bodies or inclu-
fat infiltration of the gland.
sions are identified in the tissue.
Histopathology
The histopathology associated with parotid gland biopsies
Upon receiving the histopathologic findings, the clinician
of diabetic patients when compared with those from alco-
must determine the initiating cause. Treatments have included
hol associated sialadenosis is characterized by an increased
intralesional corticosteroids, radiotherapy, sulfasalazine,
number of lipid intracytoplasmic droplets in the acinar
hydroxychloroquine sulfate, azathioprine, cyclosporine A,
and ductal cells, and abundant adipose infiltration in the
methotrexate, metronidazole, and other antibiotics. Most
stroma.
clinicians treat the lips with intralesional corticosteroids
with variable success. Spontaneous remissions occur as
Treatment
well. The prognosis is variable as there is no clearly effec-
While proper glycemic control should be the treatment goal, tive treatment to date.
this does not result in a significant reduction in the parotid
enlargement. Cosmetic improvement may be obtained with
gland removal, but the risk–benefit ratio must be considered. Sarcoidosis
Sarcoidosis is an immune-mediated multisystem disease
Medication-induced Sialadenosis of unknown origin, characterized by the presence of epi-
thelioid non-caseating granulomas in the involved organs.
Medication-induced sialadenosis has been reported for Sarcoid granulomas are commonly encountered in the
a number of medications that affect the autonomic ner- lungs, with lymph node involvement, but may be seen in
vous system. Antihypertensive agents, psychotropic drugs, many other sites. Sarcoidosis may also affect the heart,
␤-adrenergic agents, and bronchodilators have been impli- liver, spleen, bones, skin, eyes, lymph nodes, parotid glands
cated. Mauz et al reported a case were valproic acid was and the oral cavity and may first manifest in the oral and
associated with bilateral parotid and submandibular gland maxillofacial region. The disease occurs worldwide and
sialadenosis. may affect either gender. Geographically, there appears
to be a higher incidence among central and northern
Europeans. It usually arises in the 2nd to 4th decades of
Orofacial Granulomatosis
life, with a slight female predominance. In the United States,
Clinical features sarcoidosis primarily affects African Americans, who tend
to have more acute symptoms, extrapulmonary manifesta-
Orofacial granulomatosis was first introduced in 1985
tions and higher mortality rate at an earlier age compared
by Wiesenfeld and encompass a spectrum of clinical pre-
to whites. The most prominent manifestation of the dis-
sentations that share common histopathologic findings
ease involves the lungs with acute dyspnea, cough, and
of non-caseating giant cell granulomas. The conditions
chest pain being common symptoms. Chest radiographs
included in this category are Melkersson–Rosenthal syn-
reveal bilateral hilar lymphadenopathy, diffuse parenchy-
drome, cheilitis granulomatosa, Crohn’s disease, sarcoid-
mal infiltrates or both. Less commonly, sarcoidosis devel-
osis, and tuberculosis. Clinical presentation will vary, but
ops slowly over months or years, especially among whites.
the most frequent site affected is the lips. Patients present
In some cases, patients have no symptoms, and the disease
with a non-tender, persistent enlargement of the upper or
is discovered on routine chest radiographs. Mortality rates
upper and lower lips. When this presentation is combined
are similar among races. Approximately, one-fourth of
with fissured tongue and facial paralysis, the clinical con-
those with chronic sarcoidosis die of respiratory failure as
dition is called Melkersson–Rosenthal syndrome. Involve-
reported by Keller. Skin lesions occur nearly 25% of the
ment of the lips alone is called cheilitis granulomatosa.
time. These often appear as chronic, violaceous indurated
Signs and symptoms include edema, erosions, paresthesia
lesions that are termed ‘lupus pernio’ and frequent the nose,
or taste alterations.
ears, lips and face. Symmetric elevated indurated purplish
plaques are also commonly seen on the limbs, back and
Histopathology and diagnosis
buttocks. Scattered, non-specific, tender erythematous nod-
The diagnosis is confirmed upon characteristic histopa- ules known as ‘erythema nodosum’, frequently occur on
thologic findings. Scattered aggregates of non-caseating the lower legs.
289
Enlargement of the major salivary glands may be the for sarcoidosis. Secretion of angiotensin-converting enzyme
first sign of the condition. Diagnosis can be difficult and by granuloma-forming epithelioid cells results in elevated
prolonged due to the non-specific symptoms. Examination serum levels in 80–90% of cases. However, this is support-
may reveal bilateral parotid and submandibular gland ive and non-diagnostic for sarcoidosis. When bone lesions
enlargement of recent onset, asymptomatic or mildly sym- occur, hypercalcemia may be observed. Other laboratory
ptomatic, and firm and sometimes nodular upon palpa- findings in sarcoidosis might include anemia, leucopenia,
tion. Facial nerve involvement at presentation may be thrombocytopenia, and an elevated erythrocyte sedimen-
mistaken for malignancy. Glandular size does not fluctu- tation rate.
ate upon eating. Milking the parotid glands may demon-
strate delayed flow and sialometry may be below normal. Treatment
The infiltration of major salivary glands and subsequent
xerostomia predispose patients to dental decay and fungal Treatment will be determined by the onset and course of
infections. An analysis of 45 published cases as reported the disease. Spontaneous regression is common. In approx-
by Blinder et al revealed an age range between 5 and imately 60% of patients, the symptoms resolve spontane-
69 years, with highest prevalence between 30 and 40 years. ously within 2 years without treatment. Of those affected,
The study reported lesions in the jaws, buccal mucosa, pal- one-fifth can be treated with corticosteroids. Those with
ate, lips, gingiva, and floor of the mouth. Sarcoid lesions refractory disease may be treated with methotrexate, aza-
in the buccal mucosa and tongue present as submucosal thioprine, and cyclophosphamide which will impact dental
nodules and indurations or superficial ulcerations. In gin- care. A recent refractory case was successfully treated with
gival lesions, erythematous swelling is generally seen with infliximab. A small percentage of patients die of pulmo-
some superficial ulcerations in the anterior labial gingiva. nary, cardiac or CNS complications. Consultation with the
Lesions in the floor of the mouth may present as ranulas. physician is essential for quality dental care.
In many cases, the lesions in the buccal mucosa, gingiva
and tongue were the first clinical manifestation of the dis-
ease. Bony jaw lesions appear as diffuse, poorly defined
radiolucencies which may be associated with tooth mobil- SALIVARY GLAND TUMORS
ity. Lesions in maxilla, mandible and condyle have also
been reported. Salivary gland tumors represent 2–3% of all head and
neck neoplasms. Salivary gland neoplasms may be either
benign or malignant. Almost 80% of salivary gland neo-
Histopathology and laboratory
plasms occur in parotid glands; and two-thirds to four-
investigations
fifths of the parotid tumors are benign. Nearly half of the
Because the differential diagnosis of oral dryness and submandibular gland tumors are malignant and up to 80%
bilateral parotid enlargement would include sarcoidosis, of tumors of the minor salivary glands are malignant.
SS, MALT lymphoma, sialadenosis, Warthin’s tumor, and In general, the smaller the salivary gland, the greater is
HIV-associated salivary gland disease, biopsy of the the probability of malignancy. Tumors of the sublingual
enlarged glands is sometimes required. The histopathologic gland are rare and comprise approximately 1% of all sali-
findings from salivary gland biopsy usually demonstrate vary gland neoplasms; however, the majority of sublin-
non-caseating granulomas. Tightly clustered aggregates of gual gland tumors, 70–90%, are malignant. Minor glands
epithelioid histiocytes are present with a surrounding rim are a common site for salivary tumors, and may involve
of lymphocytes, mixed with Langhans giant cells. The gran- the palate, buccal mucosa, and labial mucosal glands.
ulomas often contain laminated basophilic calcifications These tumors comprise approximately 10–20% of salivary
known as Schaumann bodies or stellate inclusions known gland tumors, with nearly half being malignant. Clinical
as asteroid bodies. However, neither is specific for sarcoid- presentation and behavior will vary with each specific type
osis. The differential diagnosis for granulomatous diseases of tumor. Diagnosis will depend on clinical presentation,
would include infectious agents such as mycobacteria, imaging, and the distinctive histopathology. Fine-needle
fungi, bacterial and parasitic organisms, hypersensitivity aspiration (FNA) technique has been used to identify sali-
to environmental agents, and other autoimmune disorders vary gland neoplasms, but with mixed performance. A
such as Wegener’s granulomatosis. retrospective analysis as reported by Hughes et al found
Referral to appropriate physicians to evaluate the false-positive rates with benign lesions and malignant
lungs, heart, central nervous system, eyes, skin and lymph cases with false-negative rates. These were most com-
nodes would be required for a comprehensive diagnostic monly associated with lymphoma, actinic cell carcinoma,
evaluation and management. The serum angiotensin- low-grade mucoepidermoid carcinoma, and adenoid cystic
converting enzyme (sACE) is considered a diagnostic tool carcinoma.
290
BENIGN TUMORS Figure 25
Pleomorphic Adenoma (Benign Mixed Tumor)

Clinical features
The pleomorphic adenoma or benign mixed tumor is the
most common salivary neoplasm and accounts for over
half of the tumors in the parotid and submandibular glands.
The name ‘mixed tumor’ comes from the morphologic
complexity and differentiation of tumor cells which vary
between tumors and within the same tumor. While high in
variability of cell type, the cells are rarely ‘pleomorphic’.
While most common in the parotid gland, pleomorphic
adenomas may occur in any major gland or in the minor
salivary glands. Figure 25 shows a pleomorphic adenoma
of the parotid gland with elevation of the ear lobe. Onset is
commonly between the 4th and 6th decades, but they may
occur in young adults and children. There is a slight female
predilection. Facial nerve involvement demonstrated by
facial nerve paralysis is rare. The tumor presents as a
slow-growing, painless, firm and non-tender mass that is Pleomorphic adenoma of the parotid gland showing elevation
mobile in early stages. The tumor may have intermittent of the ear lobe. Courtesy: Dr Ajit Auluck
growth periods. As size increases, the tumor becomes more
irregular and nodular upon palpation. The overlying skin
seldom ulcerates. Local discomfort is noted with an Figure 26
increase in size. The intraoral site of preference is the hard
palate (Figure 26) and sometimes the lip.
Diagnostic imaging
Sialography demonstrates displacement of the ducts around
the benign tumor which itself is well delineated with
definitive margins (ball-in-hand appearance). CT shows a
similar picture, with a higher degree of attenuation within
the tumor mass that demonstrates homogeneous density.
A lobulated appearance is also not unusual. The margins
may appear vague if there is associated inflammation
or hemorrhage. Differentiation from a malignant lesion
becomes difficult. Occasionally, the mass appears with lower Pleomorphic adenoma. Large dome-shaped firm sessile
swelling of the hard palate. The lesion had eroded palatal
attenuation similar to a cyst. A mixed appearance is also
shelf bone and was fairly destructive. The patient reports
noted if cystic change or necrosis exists. Bleeding within
that the lesion had been present more than 2 years.
the tumor mass can result in the presence of increased Courtesy: Dr Indraneel Bhattacharyya
attenuation foci within the gland. Dystrophic calcifica-
tions are not uncommon. Figure 27A, B demonstrates the
radiographic features of a persistent pleomorphic adenoma the nature of its contents and constitution. Bleeding foci
on contrast CT. Figure 28 demonstrates an axial plain CT appear with higher signal intensity on both types of images.
scan of a pleomorphic adenoma of the parotid gland. Necrosis, on the other hand, appears with low T1 signal
Figure 29 shows a parotid gland pleomorphic adenoma via intensity and high T2 intensity. Larger lesions can be inho-
an axial CT scan heterogeneously enhancing post contrast. mogeneous. A low intensity capsule can be seen on T2
MRI, on the other hand, can clearly demonstrate the benign weighted studies and on fat-suppressed, contrast enhanced
nature of the lesion with distinct margins. Most often, the T1 weighted images. This feature may not be seen with
apparent lobulation noted in CT is not noted, with the recurrences as the capsule may have been penetrated at the
mass appearing solitary. The mass presents with a low T1 time of previous surgical intervention. Presence of calcific
weighted and high T2 weighted intensity consistent with foci is difficult to appreciate. The tumor exhibits moderate
291
Figure 27
A B
Persistent but stable, known pleomorphic adenoma in the left prestyloid parapharyngeal space. Courtesy: Dr Madhu Nair
Figure 28 Figure 29
Axial plain CT scan of pleomorphic adenoma of Axial CT scan showing heterogeneously enhancing
parotid gland. Courtesy: Dr Ajit Auluck and post-contrast pleomorphic adenoma of parotid gland.
Dr Chandrakant Shetty Courtesy: Dr Ajit Auluck and Dr Chandrakant Shetty
solid contrast enhancement. Recurrent pleomorphic ade-

nomas may be multifocal, along with malignant tumors
such as the acinic cell carcinoma and oncocytoma. The pleomorphic adenoma is usually a well-circumscribed,
Referral to a radiologist plays an essential role in diagno- encapsulated tumor, composed of a mix of glandular epi-
sis and localization of salivary gland tumors in the major thelium and myoepithelial cells within a hyalinized, eosin-
glands. Ultrasonography results in generation of hypoechoic ophilic, mesenchymal stroma. The epithelium may form
areas within a well-defined homogeneous mass. Detection ducts, cystic structures, and islands or sheets of cells. Myo-
of deep lobe tumors is difficult using ultrasonography. epithelial cells often comprise a large percentage of the
292
‘carcinoma ex pleomorphic adenoma’. Malignancy will be

Figure 30
further discussed in the section on malignant salivary
gland tumors.
Monomorphic adenoma
The term ‘monomorphic adenoma’ was originally used to
distinguish the pleomorphic adenoma from a group of
benign salivary gland tumors composed of more uniform
cells or a single cell type. Currently, the term is used in
more than one classification scheme with differing inter-
pretations and significance. Basal cell adenomas and can-
alicular cell adenomas have been termed monomorphic
adenomas due to their uniform histopathology. Due to con-
fusion in nomenclature, the term monomorphic adenoma
should be replaced with the specific name of the adenoma
Pleomorphic adenoma, histopathologic microscopic image being discussed. The following sections will describe these
of pleomorphic adenoma stained with hematoxylin and specific types.
eosin (magnification 20⫻). Proliferation of islands, strands
and duct-like formations of myoepithelial cells in a
cartilaginous and fibrous connective tissue stroma. Canalicular Adenoma
Courtesy: Dr Indraneel Bhattacharyya
Clinical features
The canalicular adenoma is an uncommon tumor that
tumor cells with variable morphology. Some demonstrate occurs almost exclusively in the minor salivary glands and
eccentric nuclei and eosinophilic hyalinized cytoplasm has a marked predilection for the upper lip. The buccal
resembling plasma cells, called ‘plasmacytoid’ myoepithe- mucosa is the second most common location. The tumor
lial cells. Areas may include chondroid material and even appears in older adults and demonstrates a female predi-
osteoid adjacent to ductal epithelium and myoepithelial lection. The clinical presentation is a slowly growing pain-
cells (Figure 30). Tumor cells may be found in the connec- less mass that ranges from several millimeters to 2 cm.
tive tissue capsule from incomplete capsule development, It may be firm or fluctuant to palpation. The overlying
capsular penetration, pseudopodia, and satellite nodules. mucosa may be bluish or normal in color. It may be mul-
tifocal and have multiple nodules in the upper lip or
Treatment and prognosis buccal mucosa.
The accepted treatment for pleomorphic adenomas is sur- Histopathology

gical excision. For tumors occurring in the superficial lobe
of the parotid gland, excision of the tumor and superficial The tumor is named so for its histologic pattern which con-
parotidectomy with conservation of the facial nerve is rec- sists of uniform columnar or cuboidal cells forming canal-
ommended. For deep lesions, parotidectomy is usually like ductal structures. The cells may appear in a double row
necessary. Submandibular gland tumors are treated by total and enclose cystic spaces of varying size. The spaces are
removal of the gland with the tumor. Tumors of the hard filled with an eosinophilic coagulum, and the supporting
palate are generally excised, with incision down to the stroma is loose and fibrillar with fine vascularity. Large
periosteum. The tumor and overlying mucosa are removed cystic spaces may be created and the epithelium may dem-
and submitted for histopathological examination. In very onstrate papillary projections into the lumina.
rare situations, the pleomorphic adenoma can be intra-
osseous, arising from ectopic salivary tissue. Clinically and Treatment and prognosis
radiographically, these may resemble lesions of odontogenic Treatment consists of surgical excision and the outcome is
origin, such as a lateral periodontal cyst. With complete very good. Recurrence is rare.
removal of the tumor, prognosis is excellent. Recurrence is
generally associated with incomplete removal and seeding
of the primary tumor. Benign pleomorphic adenomas may Basal Cell Adenoma
become malignant. This transformation may take place in
Clinical features
long-standing untreated tumors or in recurrent ones. In
addition, the malignant component may be present at the The basal cell adenoma was first reported as a distinct
initial excision. These rare malignant lesions are termed lesion by Kleinsasser and Klein in 1967. The basal cell
293
adenoma is an uncommon, benign salivary tumor which Papillary Cystadenoma Lymphomatosum

primarily occurs in the parotid gland. The glands of upper (Warthin’s Tumor)
lip and buccal mucosa are the second most common site.
It usually appears as a firm and mobile slow-growing Clinical features
mass. The tumor may present at any age, but is most com- Warthin’s tumor is a benign neoplasm that occurs
mon in older adult females. It presents as a slowly growing almost exclusively in the parotid gland. It represents the
freely movable mass, with a clinical appearance similar second most common benign parotid tumor. The patho-
to a pleomorphic adenoma and other parotid neoplasms. genesis is unclear. The traditional theory suggests that
A special subtype, the membranous basal cell adenoma they arise from heterotopic salivary gland tissue in parotid
often appears with coexisting dermal cylindromas of the lymph node. Aguirre et al suggested that they develop
scalp, often occurring in combination with skin append- from a proliferation of salivary gland ductal epithelium
age tumors. Multiple bilateral tumors may develop within that is associated with secondary formation of lymphoid
the parotids. tissue. The lesion is a slow-growing, painless nodular
mass of the parotid and may be firm or doughy upon pal-
Diagnostic imaging pation. While uncommon, they have been reported in the
submandibular and minor salivary glands by Fantasia
Basal cell adenomas of the parotid gland often present as
and Miller. The onset is generally in the 6th to 7th decade
small well-marginated tumors and appear as masses with
of life, with males being slightly more commonly affected
central large cysts or solid masses with microcytes on CT
than females. Kotwall reported that smokers have eight
and MRI scans. On 2-phase helical CT scans, the enhance-
times higher risk of developing a Warthin’s tumor than
ment patterns and imaging architecture of basal cell ade-
non-smokers. Klussmann and workers, in a large series
nomas are related to the histologic subtype of the tumor.
of 185 patients with Warthin’s tumor reported that 89%
On the 2-phase CT scan, the histologically solid-looking
of the subjects were smokers and 66% were heavy smok-
tumors show marked contrast enhancement on the early
ers. Bilateral Warthin’s tumor was seen in 17% of these
phase and subsequent decrease in attenuation on the
patients.
delayed phase. Lee et al described that tumors with large
cystic areas showed gradual and additional enhancement
Diagnostic imaging
on the delayed phase and were classified as tubular or
trabecular subtype on histopathologic evaluation. CT will reveal a well-delineated, homogeneous, non-
calcifying mass with a well-defined wall. Cystic appear-
Histopathology ance is quite common as are multiple lesions. Figure 31A, B
demonstrates the cystic appearance of the lesion on con-
The tumor is encapsulated, and it is named so because of trast CT. The intrinsic mass demonstrates no evidence of
basaloid appearance of the tumor cells. The basaloid perineural spread.
hyperchromatic peripheral cells show a palisaded arrange- MRI demonstrates a homogeneous or inhomogeneous
ment, similar microscopically to a basal cell carcinoma. appearance depending on the size of the tumor and pres-
The central cells have paler staining nuclei. Cells may also ence of cystic changes. If solid, the lesions appear with
be in ribbons or cords. The trabecular subtype demon- low intensity on T1 weighted images and high intensity
strates narrow cord-like epithelial strands, and the tubular on T2 weighted images. Radionuclide imaging studies
subtype is characterized by the formation of small round, using technetium pertechnetate studies show accumula-
duct-like structures. The membranous basal cell adenoma tion of the tracer within the tumor, and is considered diag-
is characterized by palisading of peripheral cells and an nostic. Positron emission tomography (PET) also indicates
excessive hyaline basal membrane. Multiple large lobular increased uptake of tracer. The differential diagnosis of
islands of tumor give the appearance of a jigsaw puzzle. Warthin’s tumor on MRI includes other benign cystic
The islands are surrounded by a thick layer of hyaline lesions. However, Warthin’s tumor exhibits nodularity of
material, similar to the microscopic appearance of the the cyst wall, whereas other benign cystic lesions tend to
dermal cylindroma. have smooth walls. Conversely, cystic and necrotic malig-
nant tumors tend to be ill-defined. Warthin’s tumors fail to
Treatment and prognosis enhance, whereas pleomorphic adenomas undergo solid
Treatment consists of surgical removal and recurrence is contrast enhancement.
rare, with one exception. Likely owing to its multifocal
Histopathology
nature, the membranous subtype has a higher recurrence
rate than other subtypes. Malignant basal cell adenomas Histologically, the papillary cystadenoma lymphomato-
(basal cell adenocarcinoma) may arise de novo or from sum is composed of a mixture of ductal epithelium and a
malignant change of pre-existing basal cell adenomas. lymphoid stroma. The epithelium is usually a double row
These have a good prognosis. of oncocytes with surrounding cystic spaces. The lining
294
Figure 31
A B
Warthin’s tumor as noted on a contrast CT study reveals a multiseptated predominantly cystic mass within the tail of the
parotid gland on the right side. It is intrinsic to the gland and well below the main trunk of the facial nerve and its
major branches. Courtesy: Dr Madhu Nair
forms papillary projections into cystic spaces. The epithe- Treatment and prognosis
lium is supported by a lymphoid stroma which may dem-
The treatment of choice is surgical excision. It does not
onstrate germinal centers.
have a high recurrence rate. Malignant transformation is
uncommon.
The preferred treatment for Warthin’s tumor is surgical
removal. Malignant Warthin’s tumors, carcinoma ex papil- MALIGNANT TUMORS
lary cystadenoma lymphomatosum, have been reported, but
are extremely rare.
Adenocarcinomas of salivary gland origin are quite varied
in their histologic features and clinical behavior. These are
Oncocytoma (Oxyphilic Adenoma) diagnosed based on their microscopic features. The parotid
is the most common site, followed by minor salivary glands.
Clinical features In the parotid, the lesions are usually firm to palpation
The oncocytoma is a rare salivary gland tumor which usu- and may produce damage to the facial nerve. The presence
ally occurs in the parotid gland (84%), but has been reported of enlarged lymph nodes raises the suspicion of malig-
in the submandibular gland and minor salivary glands. nancy, but also occurs in inflammatory conditions. The
Clinically, it cannot be distinguished from other benign presence of nodal metastasis is a poor prognostic indica-
salivary gland tumors. The name ‘oncocytoma’ is from the tor. Intraorally, minor salivary glands of the hard palate
resemblance of the tumor cells to normal oncocytes, which are the most common location for adenocarcinomas fol-
are found in salivary glands, respiratory tract, breast, thy- lowed by buccal mucosa, lips and base of tongue.
roid, pancreas, parathyroid, pituitary, liver and stomach.
The oncocytoma occurs in elderly persons and is some- Mucoepidermoid Carcinoma
what more common in women than in men. The tumor usu-
ally measures from 3 to 5 cm in diameter and appears as a Clinical features
discrete encapsulated mass. It is usually asymptomatic. Mucoepidermoid carcinoma is one of the most common
malignant salivary gland tumors. It was first described by
Histopathology
in 1945 by Stewart, Foote and Becker. It occurs in indi-
Histologically, the oncocytoma is a tumor of ductal cells viduals from the second to the seventh decade. While it is
that are enriched with mitochondria, giving the cell a rarely seen in the first decade of life, it is the most com-
swollen eosinophilic appearance. mon malignant salivary gland tumor in children. Some
295
tumors have been associated with a previous history of

Figure 32
radiation therapy to the head and neck region. There are
three grades: low, medium and high. Low-grade tumors
have a limited potential to metastasize to regional lymph
nodes. High-grade tumors tend to be solid rather than
cystic and behave aggressively like a squamous cell carci-
noma. Mucoepidermoid carcinoma most commonly pres-
ents in the parotid gland where it may initially appear as
a painless swelling. Intraorally, tumors are found in the
palate (Figure 32), buccal mucosa, retromolar area, and
base of tongue. These may present as slowly enlarging,
painless masses. The low-grade lesions contain cysts which
may be filled with mucoid material. These intraoral lesions
may resemble a mucocele, especially when located in the
retromolar area. More high-grade tumors grow rapidly and
produce pain early in the process. Facial nerve paralysis is Mucoepidermoid carcinoma in the hard palate. Well
a common feature in high-grade tumors. As these tumors circumscribed raised lesion of 4 months duration on the hard
tend to infiltrate surrounding tissue, they may metastasize palate with large central umbilicated ulceration with rolled
to regional lymph nodes. borders. Courtesy: Dr Indraneel Bhattacharyya
Histopathology
Mucoepidermoid carcinoma is composed of cells from strat- initial treatment. Twenty-one patients (9%) had local
ified squamous lines, mucous cell lines, and a third cell- recurrence only, 12 (5%) demonstrated metastasis and sur-
type, the intermediate cell. The mucous cells have abundant vived, and (11%) died of their disease. Clinical features
foamy cytoplasm that stains positively with mucicarmine associated with metastasis or deaths were more advanced
stain. The epidermoid cells frequently demonstrate features age, tumor size, and preoperative symptoms. In a recent
of squamous epithelium, intercellular bridges, but rarely series of mucoepidermoid carcinomas of the minor salivary
show keratinization. The intermediate cell is basaloid in glands as reported by Triantafillidou et al, immunohisto-
appearance and may be the origin of the other mucous and chemical assay of Ki-67 antigen expression correlated with
epidermoid cells. A lymphocytic infiltrate is commonly seen. tumor grade. Prognosis in this series of 15 tumors was a
Low-grade tumors show all cell types. Prominent cyst for- function of the histological grade, adequacy of excision
mation, a high proportion of mucous cells, and minimal and clinical staging.
atypia are characteristic features. High-grade tumors con-
sist of solid islands of squamous and intermediate cells,
which can show pleomorphism and mitotic activity. Few Intraosseous Mucoepidermoid Carcinoma
mucus producing cells are present, which allow the
lesion to be confused with a squamous cell carcinoma. The Clinical features
intermediate-grade tumor shows features that rank in Intraosseous mucoepidermoid carcinoma most frequently
between the low- and high-grade tumors (Figure 33). appears in the posterior jaws, mandible more frequently
than the maxilla. It has been reported in all ages, from
Treatment 1 to 85 years. Most cases occur in the 4th and 5th decades.
Treatment may vary according to the tumor location, grade,
and staging. Surgical removal of the lobe or gland may be Diagnosis
adequate. High-grade or large tumors merit wider resection, The radiographic picture of central mucoepidermoid car-
similar to that employed for squamous cell carcinoma. For cinoma may resemble an ameloblastoma or odontogenic
patients with evidence of metastatic disease or large high- cyst. Based on radiographic imaging, Inagaki classified
grade tumors, radical neck dissection may be indicated. these lesions into three categories: cystic, rarefying, and
Postoperative radiation therapy may be employed as well. infiltrative. He reported that those with the infiltrative
type were histologically poorly differentiated tumors, and
Prognosis
these patients died of their tumors. Radiographic findings
Patients with low-grade tumors and complete excision correlated with histologic findings and prognosis in this
have a good prognosis. Patients with high-grade tumors limited series. Other salivary lesions that have appeared
have a poorer prognosis. In a large series reported by centrally in the jaws include pleomorphic adenoma, ade-
Goode et al, most patients (75%) were tumor free after the noid cystic carcinoma and malignant mixed tumor.
296
Figure 33 Acinic Cell Adenocarcinoma

Clinical features
Acinic cell adenocarcinoma is a malignant salivary gland
tumor characterized by histologic appearance showing serous
acinar differentiation. The majority of acinic cell carcino-
mas are found in the parotid gland, and only rarely in the
intraoral minor glands. The age range is broad with a peak
incidence in the third decade of life. Similar to many salivary
gland tumors, it may be a slow-growing mass. In a large
series as reported by Ellis and Corio, tumors were usually
less than 3 cm in diameter and were slow growing. Pain
was a common symptom, but not indicative of prognosis.
Histopathology
Mucoepidermoid carcinoma, histopathology: Microscopic The acinic cell adenocarcinoma may be composed of cells
image of mucoepidermoid carcinoma stained with that exhibit varying degrees of differentiation, but resem-
hematoxylin and eosin (magnification 20⫻). Large aggregates ble serous acinar cells. Four growth patterns have been
of intermediate cells interspersed with cells with clear described: solid, papillary cystic, follicular and micro-cystic.
cytoplasm and mucous cells. The cellular aggregates are The micro-cystic variety was most commonly seen in a
arranged in clusters separated by thin fibrous connective large series as described by Ellis and Corio.
tissue septa. The peripheral cells appear more epidermoid
with reduced cytoplasm and hyperchromatic nuclei. Treatment and prognosis
Courtesy: Dr Indraneel Bhattacharyya
Treatment usually consists of parotid gland lobectomy
or parotidectomy. Submandibular tumors are treated by
Variation in presentation on CT is noted depending on removal of the gland. In a recent series as reported by
the degree of differentiation of the tumor mass. Well- Hoffman and coworkers, worse survival was associated
differentiated lesions have well-defined margins and may with high-grade lesions, age more than or equal to 30 years
mimic a benign tumor with or without cystic changes. More and the presence of metastatic disease. Although a better
aggressive types have ill-defined margins and show signs outcome was not statistically demonstrated for combined
of local tissue destruction. Contrast enhanced CT shows an therapy, surgery with irradiation is the most common man-
inhomogeneous enhancement with well-defined margins. agement in the United States for cases with regional metas-
Poorly differentiated lesions show enhancing mass with tases, high-grade, and microscopic positive margins.
irregular margins and lymphadenopathy. MRI shows inho-
mogeneous low to intermediate signal intensities on T1
and T2 weighted images. Nuclear medicine studies do not Malignant Mixed Tumor
reveal any tracer uptake. MRI is the recommended imag- (Carcinoma Ex Pleomorphic Adenoma)
ing modality as perineural spread and deeper involvement
Description and clinical features
are better delineated. Perineural spread is sometimes noted
along cranial nerve VII. Malignant mixed tumors are the malignant counterpart to
the pleomorphic adenoma, or benign mixed tumor. In
Histopathology many cases, there are no obvious differences in clinical
presentation of benign versus malignant pleomorphic ade-
Most centrally occurring mucoepidermoid carcinomas show
nomas. The carcinoma ex pleomorphic adenoma is char-
the same variability and grades as found in salivary gland
acterized by malignant transformation of the epithelial
tumors. Most are low-grade lesions, although high-grade
component of a previously existing benign pleomorphic
lesions have been reported as well.
adenoma. Most of these are seen within the parotid gland,
followed by the submandibular gland. These also occur in
the minor salivary glands and sublingual gland. This lesion
The treatment for intraosseous mucoepidermoid carci- affects an older age group than the benign counterpart,
noma is surgery, sometimes with adjuvant radiation ther- usually occurring around 50–70 years of age. The common
apy. Radical surgical resection provides a better long-term clinical presentation is rapid expansion of a long-standing
prognosis than conservative measures such as curettage. asymptomatic mass, new onset of pain, paresthesia, and/
Metastasis has been reported in a small number of cases. or fixation to the skin. Although pain or recent rapid
297
growth is common, cases may present as a painless mass, neck (43%), and lung (36%). The 5-year disease-free sur-
similar to a benign lesion. The risk for malignant transfor- vival was 50%. Chemotherapy and radiotherapy were of
mation increases with the duration of the tumor. limited value.
Histopathology
Adenoid Cystic Carcinoma
The histolopathologic features will vary widely. In some
lesions, the benign areas will be difficult to locate due to Clinical features
an abundance of malignant areas. In other tumors, the Adenoid cystic carcinoma is a common, well-defined
bulk is benign with small areas demonstrating the malig- malignant salivary gland tumor that may occur in any
nant features. Areas of the benign pleomorphic adenoma salivary gland. In one series as reported by Jones et al,
may be present with areas of malignant changes to the nearly half of the tumors arising in the oral cavity occurred
epithelial component. The changes include hypercellularity, in the hard palate, and the remainder occurred in other
increased abnormal mitotic activity, areas of hyalinization minor glands, the submandibular and parotid glands.
and necrosis. The malignant component will demonstrate Adenoid cystic carcinoma occurs in the 5th, 6th and 7th
extracapsular invasion. Sites of poorly differentiated car- decades of life; rarely before the age of 20. Most series
cinoma may be found as well. show an equal gender distribution. In the major glands, it
commonly presents as a slow-growing swelling or mass.
Treatment and prognosis The patient may be aware of the lesion for months or years.
Carcinoma ex pleomorphic adenoma is treated by wide exci- Pain intensity may grow as the lesion progresses. Fixation
sion, often with local lymph node dissection, and adjuvant to deeper structures occurs in later stages. As with other
radiation therapy. A recent study reported by Chen and malignant salivary gland tumors, involvement of the facial
coworkers showed significant improvement in a 5-year nerve is a serious finding. Adenoid cystic carcinoma of the
local control from 49 to 75% through the use of postop- intraoral minor glands presents as a swelling or mass, usu-
erative radiotherapy. It was also associated with improve- ally in the palate. Eventually, pain and ulceration develop.
ment in survival among patients without evidence of Jones et al described one study that reported a better sur-
cervical lymph node metastasis. vival rate if tumors were in the hard palate versus other
sites in the head and neck. The specific survival rate was
40% at the age of 20 years. Adenoid cystic carcinoma may
Metastasizing Mixed Tumor also appear in the tongue, and centrally in bone.
Clinical features
Histopathology
The metastasizing mixed tumor is also quite rare. Most
Several histologic types are recognized. The classic pattern,
patients have a history of a pleomorphic adenoma which
the cribriform type, is recognized by the arrangement of
was excised many years earlier. The majority of cases orig-
tumor cells in the ‘Swiss cheese’ pattern. Tumor cells have
inate in the parotid gland, but the primary tumor may
deeply basophilic nuclei with scant cytoplasm (Figure 34).
occur in the submandibular gland or minor glands. Metas-
Other patterns include the tubular type and solid type. In
tases are most frequently in the bones, lung or regional
the tubular pattern, tumor cells form multiple small ducts
lymph nodes.
or tubules within a hyaline stroma. The solid variant is
composed of sheets of tumor cells, with cellular pleomor-
Histopathology
phism and mitotic activity, and focal necrosis. It is associ-
Both lesions, the initial benign mixed tumor and the tumor ated with a poorer prognosis than the cribriform type.
at the metastatic site show histopathologic features of the Perineural invasion is a highly characteristic feature of
benign mixed tumor. Microscopic malignant changes are adenoid cystic carcinoma, but perineural invasion is seen
not observed. in other salivary malignancies, particularly the polymor-
phous low-grade adenocarcinoma.
Diagnostic imaging
Treatment consists of surgical excision of the primary tumor
and the metastatic sites. In a recent report of a review of Parotid lesions appear with well-defined margins while
44 cases as reported by Nouraei, most patients had local minor gland masses have ill-defined margins. Retrograde
recurrences before metastasis. The mean presentation-to- spread along facial or mandibular nerve is not uncommon.
metastasis latency was 16 years. Bone was the most com- Contrast MRI is the optimal imaging modality to visualize
mon site for metastases (45%), followed by the head and such extension. Varying degrees of nerve involvement
298
Figure 34 Polymorphous Low-Grade Adenocarcinoma

Clinical features
Polymorphous low-grade adenocarcinoma (PLGA) was first
described in 1983 and termed ‘lobular carcinoma’ due to
its resemblance to lobular carcinoma of the breast. It is a
low-grade salivary gland tumor that occurs primarily in
the minor salivary glands. Common presentation is a pala-
tal mass that may range in size from 0.4 mm to 6 cm. The
average age of occurrence in a large series as reported by
Castle et al was 58 years with a range from 23 to 94 years.
The tumors were infiltrative and characterized by a poly-
morphous growth pattern.
Histopathology
The neoplastic cells are isomorphic with vesicular nuclei.
Individual tumors may demonstrate multiple patterns,
Adenoid cystic carcinoma, histopathology: including solid, ductotubular, cribriform, trabecular, and
Microscopic image of adenoid cystic carcinoma stained single file growth. The histologic features may resemble
with hematoxylin and eosin (magnification 20⫻). Invasive adenoid cystic carcinoma. Neurotropism is frequently
islands of neoplastic epithelial cells forming duct-like identified. Mitotic activity is not prominent.
structures containing a pale mucoid material imparting
the classic ‘Swiss cheese’ appearance. The tumor cells are
Treatment
basaloid in appearance with hyperchromatic basophilic
nuclei and scanty eosinophilic cytoplasm. Wide local excision is the treatment of choice. Local recur-
Courtesy: Dr Indraneel Bhattacharyya rences may occur despite having negative margins after
surgery. Local recurrence may appear 15 years after initial
surgery. Regional nodal metastasis and lung metastasis
have been reported by Pogodzinski et al, 20 years after the
can be imaged using MRI. CT also detects some changes procedure. These occurrences support the need for pro-
related to nerve involvement. Widening of the nerve longed follow-up. Recently, PLGA was reported by Ruiz-
canal and obliteration of fat at the cranial foramina is Godoy et al in the parotid gland in a 60-year-old male.
evident on contrast CT but early subtle changes are not Treatment included a superficial parotidectomy and post-
discernible. operative radiotherapy (46 Gy). Transformation from PLGA
in the palate to a high-grade carcinoma has been reported
Treatment and prognosis as well.
Surgical excision is the treatment of choice with radio-
Prognosis
therapy as it metastasizes to the lymph nodes. Metastatic
spread most commonly involves the lungs and bones. In Conservative, but complete surgical excision is the treat-
addition, metastatic spread to the kidney and liver has ment of choice for these tumors, with a reported 97% sur-
been reported. Regardless of the treatment modality, the vival rate. While these have a tendency to recur, the metastatic
overall prognosis is poor. potential is low.
299

SECTION Cysts and Tumors
V of Orofacial
Region
12 Cysts of Orofacial Region 303

13 Tumors of Orofacial Region 331
14 Oral Cancer 380
301
Chapter-12.indd 301 10/4/2012 12:44:43 PM


CHAPTER
Cysts of Orofacial Region

Ravikiran Ongole, Sunanda C, NVS Sekhar
Reddy, Joanna Baptist, Thomas Zachariah
12
➧ Classification of Cysts of Orofacial Region ➧ Inflammatory Cysts
➧ Theories of Cyst Expansion Periapical Cyst
➧ Odontogenic Cysts Residual Cyst
Gingival Cyst of Infants Paradental Cyst
Eruption Cyst Mandibular Infected Buccal Cyst
Dentigerous Cyst ➧ Pseudocysts
➧ Odontogenic Keratocyst Traumatic Bone Cyst
Orthokeratinized Odontogenic Cyst Aneurysmal Bone Cyst
Stafne’s Bone Cyst
➧ Gingival Cysts of Adults
➧ Cysts of Soft Tissues of Mouth, Face and Neck
Lateral Periodontal Cyst
Botryoid Odontogenic Cyst Anterior Median Lingual Cyst
➧ Nasopharyngeal Cyst
➧ Calcifying Epithelial Odontogenic Cyst
➧ Thyroglossal Duct Cyst
➧ Glandular Odontogenic Cyst
➧ Lymphoepithelial Cyst
➧ Non-odontogenic Cysts
Nasopalatine Duct Cyst ➧ Cystic Hygroma
➧ Nasolabial Cyst ➧ Dermoid, Epidermoid and Teratoid Cysts
➧ Mid-palatal Raphe Cyst of Infants ➧ Parasitic Cysts
➧ Cysts of Maxillary Antrum and Salivary Cysticercosis
Glands Hydatid Cyst
Trichinosis
The word ‘cyst’ is derived from Greek word kystitis mean- displacement of roots of teeth, resorption of roots, pares-
ing bladder. Kramer (1974) defined cyst as a pathological thesia, expansion of the cortical plates and eventually
cavity having fluid, semifluid or gaseous contents and result in pathologic fracture of the jaw.
which is not created by the accumulation of pus. It is fre-
quently, but not always, lined by the epithelium. Cysts may
arise due to trauma, inflammation and degeneration or CLASSIFICATION OF CYSTS OF
retention. They are called true cysts if lined by epithelium OROFACIAL REGION
and, pseudocysts (false) if not lined by epithelium. During
the initial stages, when the cysts are small they are usually 1. True cysts of the oral cavity (odontogenic and non-
asymptomatic. Secondary infection may result in the odontogenic)
formation of abscess, cellulitis, osteomyelitis and subse- 2. Pseudocysts of the oral cavity
quent sinus formation. As the cyst enlarges it may cause 3. Cysts of the associated structures of the orofacial region.
303
Section V – Cysts and Tumors of Orofacial Region
True Cysts of Oral Cavity (Lined by Epithelium) Figure 1
❍ Developmental cysts
– Odontogenic cysts
– Gingival cyst of infants
– Eruption cyst
– Dentigerous cyst
– Odontogenic keratocyst*
– Orthokeratinized odontogenic cyst
– Gingival cyst of adults
– Lateral periodontal cyst and botryoid odontogenic
cyst
– Calcifying odontogenic cyst
– Glandular odontogenic cyst
❍ Non-odontogenic cysts
– Nasopalatine duct cyst Enlargement of the cyst at the peripheries
due to active division of the cells of the epithelium
– Nasolabial cyst
lining the cyst
– Mid-palatal raphe cyst of infants
❍ Inflammatory cysts
– Radicular cyst
– Residual cyst Mural Growth Theory
– Paradental cyst
– Buccal bifurcation cyst The mural growth theory is explained by two propositions.
1. Peripheral cell division: It is proposed that the cyst
Pseudocysts of Oral Cavity (Not Lined by enlarges at the peripheries because of active division of
Epithelium) the cells of the epithelium lining the cyst (Figure 1). It is
believed that the division of these cells is a response to
❍ Aneurysmal bone cyst any irritating stimulus. The theory further suggests that
❍ Stafne’s bone cyst once the stimulus is withdrawn the cyst regresses in size.
❍ Traumatic bone cyst This theory has not found many supporters as cyst regres-
sion will result in irregular inner surface of the cyst as the
Cysts of Associated Structures of Orofacial bone surrounding the cyst offers resistance. One should
Region remember that the contents of the cyst will support the
cystic lining and it can cause rapid resorption of sur-
❍ Cysts of the salivary glands rounding bone to accommodate the enlarging cyst.
❍ Cyst associated with the maxillary antrum
❍ Anterior median lingual cyst 2. Accumulation of cellular contents: This theory was
❍ Nasopharyngeal cyst proposed by Kramer (1974) to explain the enlargement of
❍ Thyroglossal duct cyst OKCs. He proposed that as mural squames are shed off the
❍ Branchial cleft cyst lining epithelium they accumulate, thereby increasing the
❍ Cystic hygroma cyst volume. The sites of expansion and growth are repre-
❍ Parasitic cysts sented by finger-like projections which are zones of active
cell division or proliferation (Figure 2).
*Odontogenic keratocyst (OKC) has been redesignated as keratocystic
It is also believed that these finger-like extensions are
odontogenic tumor by the World Health Organization (WHO). OKC should
not be included in the classification of cysts anymore. formed because keratocysts have very poor bone resorbing
properties. Hence, they proliferate along the cancellous
bone which is less dense than the cortical bone. This explains
the fact why keratocysts very rarely cause expansion or
THEORIES OF CYST EXPANSION resorption of the cortical plates.
Kubota et al (2000) studied the role of interleukin-1
Malcolm Harris (1975) summarized various theories that alpha (IL-␣) and matrix metalloproteinase-9 (MMP-9) in
explain cyst expansion. Mural growth theory, hydrostatic the expansion of odontogenic cysts. They found that an active
enlargement, and role of bone resorbing factors are the form of MMP-9 was present in OKC fluids more frequently
theories that have been proposed by various authors to than dentigerous cyst and radicular cyst fluids. However,
explain enlargement of cysts. proMMP-9 was present in all cyst fluids.
304
Chapter 12 – Cysts of Orofacial Region
enters and accumulates within the cyst thereby aiding its

Figure 2
enlargement.
Role of Bone Resorbing Factors

Harris et al (1973) proposed that cyst tissue (capsule) con-
tains a combination of prostaglandins E2 and E3, which
are considered very potent bone resorbing factors. It is
believed that the leukocytic and bone resorbing factors
aid in bone resorption. It is believed that the keratocyst
secretes comparatively lesser amount of bone resorbing
factors per unit surface area compared to other cysts (peri-
odontal cyst, radicular cyst). This explains the burrowing
nature or intramedullary growth of the keratocyst.
ODONTOGENIC CYSTS
Finger-like projections which are zones of active cell The odontogenic cysts are derived from epithelium associ-
division or proliferation ated with the development of dental apparatus. It is esti-
mated that odontogenic cysts make up approximately
90% of the jaw cysts. These cysts are generally lined by
Kubota et al (2002) in their study showed that interleukin stratified squamous epithelium. However, some develop-
(IL)-1␣ stimulates enzymatic degradation of the extracel- mental or fissural cysts in the maxilla will have respiratory
lular matrices of the bone around the cysts. This induces epithelium as the lining.
expansion of OKC.
Oka et al (2005) studied the effects of positive intra-
cystic fluid pressure in OKCs. They proposed that the pos- Gingival Cyst of Infants
itive intracystic pressure may play a crucial role in OKC Gingival cyst of infants or newborns is an odontogenic cyst
growth via stimulating the expression of IL-1 in epithelial which is developmental in nature. Like the name suggests
cells. these cysts are seen in infants. These cysts are seldom
seen after 3 months of age. They arise from the epithelial
remnants of dental lamina called cell rests of Serres.
Hydrostatic Enlargement of Cysts Clinically, the cyst is seen on the crest of the maxillary and
mandibular dental ridges and appears creamish white.
It has been proposed that accumulation of fluids within
These cysts are usually minute in size and rarely exceed
the cystic cavity will cause expansion of the cyst wall. The
3 mm in diameter and commonly occur on the maxillary
fluid accumulation can occur from secretion by the goblet
alveolar ridge.
cells that line the follicular cysts or by transudation or
Histopathological evaluation reveals a keratin filled cyst
exudation from capsular capillaries in periodontal cysts
lined by parakeratinized epithelium. Gingival cysts in infants
as proposed by Main (1970) and hemorrhage in follicular
need no treatment as they tend to undergo involution and
and periodontal cysts as proposed by Harris and Pannell
disappear. Most cysts tend to rupture spontaneously.
(1973).
James (1926) and Tratman (1939) proposed the osmotic
theory or dialysis to explain hydrostatic enlargement of cysts.
Eruption Cyst
The cyst wall and the capillaries in the cystic capsule are
made up of high molecular weight proteins like fibrin and The eruption cyst is a type of soft tissue cyst associated
␣2-globulins which aid in increasing the permeability of with erupting teeth. It surrounds the crown of a tooth that
the capsular capillaries and the cyst walls. has already erupted through bone but impeded by the
It was found that the mean osmolality of cystic fluid is overlying soft tissue. Kuczek et al (2003) reported a case
10 milliosmoles higher than that of serum. This gradient where a boy developed an eruption cyst who was adminis-
helps in the accumulation of all the shed degenerated cells tered cyclosporin A (potent immunomodulatory agent) subse-
from the lining of the cyst. As the cyst has inadequate quent to a cardiac transplantation. Cyclosporin A was replaced
lymphatic drainage, the fluid from the capsular capillaries with tacrolimus and there was no new cyst formation.
305
The authors proposed that the formation of an eruption

Figure 3
cyst may be an adverse effect of cyclosporin A in children
with erupting teeth.
Clinical features
Eruption cysts are usually seen in childhood. These cysts
are commonly seen in the age groups of 5 and 9 years.
However, Woldenberg (2004) published a case report of
an eruption cyst in the maxilla of a 40-year-old female.
Studies by Anderson (1990), Woldenberg (2004) and
Aguiló (1998) show that these cysts are more prevalent in
the maxillary arch. In a study of 24 cases of eruption cysts
by Bodner et al (2004), it was found that eruption cysts
were associated with natal teeth in two cases, with primary Orthopantomograph (OPG) showing dentigerous cyst
teeth in 10 cases and with permanent teeth in 12 cases. (multilocular) associated with impacted right lower
It was seen that males more commonly presented with third molar and a supernumerary tooth displaced
eruption cysts than females (2:1). The primary mandibular superiorly approximating the sigmoid notch.
central incisors and the permanent first molars were the Courtesy: Dr NVS Sekhar Reddy
most common site involved. Though most of the eruption
cysts are reported as solitary entities, literature review
reveals report of a patient presenting with multiple eruption
cysts. Ramón Boj and García-Godoy (2000) described a Figure 4
case of a 15-month-old child who had six eruption cysts
simultaneously.
On clinical examination, the cyst is visible as a soft
fluctuant mass on the alveolar ridges and may vary in size
from about 1 to 1.5 cm in diameter. It may have the same
coloration of healthy oral mucosa or appear bluish or
bluish black. Eruption cyst histologically mimics dentiger-
ous cyst.
Management
Usually the cysts open up spontaneously. In some children
the overlying soft tissue can be incised to facilitate the tooth
to erupt. Literature reveals that marsupialization is suffi-
cient to manage eruption cysts. Orthopantomograph (OPG) showing dentigerous cyst
(unilocular) associated with the right upper third molar.
Dentigerous Cyst (Follicular Cyst) Radiology, MCODS, Mangalore
It is an odontogenic cyst formed by the accumulation of

fluid between reduced enamel epithelium and enamel sur-
face. It surrounds the crown of an impacted tooth and is relatively more predisposed. It grows aggressively and
attached to its neck. The crown of the involved tooth projects produces facial asymmetry, bony expansion, displacement
into the cyst lumen. It is estimated that 10% of impacted and resorption of teeth. Entire ramus might be hollowed
teeth are associated with dentigerous cysts. Dentigerous cysts if associated with mandibular third molar (Figure 6A, B).
may also be seen associated with supernumerary teeth and In case of maxillary canine, anterior maxilla is expanded.
odontomas. There will be no pain unless secondarily infected. On aspi-
ration, yellow-colored fluid is obtained.
Clinical features
It occurs frequently in association with impacted man-
dibular (Figure 3), maxillary third molars (Figure 4), and Radiographically, dentigerous cysts exhibit three different
maxillary canines (Figure 5). Dentigerous cyst is com- presentations, namely, the central, lateral and circumferential
monly seen in the 2nd and 3rd decades of life. Males are types. In central variety, the cyst symmetrically envelops
306
Figure 5 Figure 6
Orthopantomograph (OPG) showing a dentigerous cyst

associated with the right upper canine.
Radiology, MCODS, Mangalore B
crown. The cyst occurs on lateral aspect of the crown in

lateral variety and envelops crown entirely in circumferen-
tial variety (Figure 7). Dentigerous cyst may appear as well-
defined unilocular or multilocular radiolucency (Figure
8A, B). Occasionally these cysts may cause displacement
and resorption of the adjacent teeth (Figure 9). Large cysts
often cause expansion of the cortical plates.
Histologic features
It has no characteristic microscopic features. It has cyst lin-
ing and connective tissue wall. The connective tissue wall
consists of very loose fibrous connective tissue and sparse
collagenized myxomatous tissues. It has presence of odon- 3D-reconstructed images showing hollowing of the mandible
togenic epithelium (Figure 10). The inflammation leads to on the right side associated with the dentigerous cyst.
presence of Rushton bodies of hematogeneous origin in Courtesy: Dr NVS Sekhar Reddy
the lining epithelium. Cholesterol clefts might be present.
Potential complications
ODONTOGENIC KERATOCYST
The odontogenic cyst has following complications: the devel- Odontogenic keratocyst (OKC) was first described by
opment of mural ameloblastoma (Figure 11), squamous cell Philipsen in 1956. WHO has recently designated OKC as
carcinoma and mucoepidermoid carcinoma. keratocystic odontogenic tumor (KCOT) and is defined as ‘a
benign uni- or multicystic, intraosseous tumor of odonto-
Syndrome association genic origin, with a characteristic lining of parakeratinized
Multiple cysts can be formed in association with Gorlin– stratified squamous epithelium and potential for aggres-
Goltz syndrome, cleidocranial dysplasia and Maroteaux– sive, infiltrative behavior’.
Lamy syndrome. Keratocystic odontogenic tumor is described in Chapter 13
on Tumors of Orofacial Region.
Management
Orthokeratinized Odontogenic Cyst
Surgical removal of cyst and tooth is recommended
(Figure 12). Marsupialization is done in case of very large Philipsen in 1956, in his study of jaw cysts, described a
cysts. The cyst recurs if incompletely removed. distinct form of OKC which showed an orthokeratinized
307
pattern. Wright et al (1981) reported 60 cases of orthokera- Clinical features

tinized odontogenic cyst (OOC) and compared with OKC and
Orthokeratinized odontogenic cyst usually presents as a soli-
found that the OOC appears to be a distinct clinicopatho-
tary cyst in the posterior part of the mandible. It is usually
logic entity. It was suggested that this cyst be called OKC,
seen in males commonly in the 2nd to 5th decade of life.
orthokeratinized variant. Iamaroon et al (2004) compared
In comparison to OKC it is less aggressive and has a very
the proliferation index of the epithelial cells between OKC,
low recurrence rate (2.2%). Literature review reveals that
OOC, dentigerous cyst and ameloblastoma. He concluded
almost two-thirds of the cases of OOC appear like dentiger-
that OKC should be considered a benign tumor rather than
ous cyst on clinical and radiographic examination.
simply an odontogenic cyst and OOC as a non-aggressive
cystic lesion. Histopathologic features
Orthokeratinized odontogenic cyst has a thin epithelial lin-
ing with a luminal surface of orthokeratin. The basal cell
Figure 7 layer contains flattened squamous or cuboidal cells and a
well-developed granular cell layer (Figure 13).
A B
Orthokeratinized odontogenic cyst presents either as a well-
defined unilocular or multilocular radiolucency. Radio-
graphically, OOC may resemble a dentigerous cyst when it
is unilocular and resembles ameloblastoma when it is mul-
Central Lateral tilocular. Occasionally, it may cause cortical plate expan-
sion and thinning of the lower border of the mandible.
C Roots of teeth may be displaced (Figure 14). However,
resorption of roots is seldom seen.
The cyst should be surgically excised (Figure 15).
GINGIVAL CYSTS OF ADULTS

Circumferential
Gingival cysts of adults are rare odontogenic cysts of
Schematic diagrams showing various types of dentigerous cysts developmental origin. The epithelial lining of these cysts
Figure 8
A B
CT images showing unilocular dentigerous cyst
308
Figure 9
Orthopantomograph (OPG) showing a dentigerous cyst associated with the impacted left lower third molar causing root
resorption of the adjacent second molar. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Figure 10 Figure 11
2–3 layered
epithelium
Connective
tissue capsule
Cystic lumen
Photomicrograph of dentigerous cyst.

Orthopantomograph (OPG) showing multilocular radiolucency
associated with the impacted mandibular third molar.
The radiograph showing displacement of the third molar and
are thin non-keratinized and are thought to arise from the the lesion extends to involve the entire ramus and apices
rests of dental lamina. Some authors suggest that these of the second molar. The lesion was histopathologically
cysts arise from the traumatic implantation of surface diagnosed as mural ameloblastoma. Courtesy: Department
epithelium. of Oral Medicine and Radiology, MCODS, Mangalore
Schroeder (1976), in a book titled Scientific Foundations
of Dentistry suggested that the gingival cyst arises from
maxilla. The swelling is soft and fluctuant with a smooth
the junction epithelium which is a derivative of the reduced
surface and usually have the same coloration as that of the
enamel epithelium.
gingiva. Some authors have reported bluish and red color
These occur as swellings on the facial aspect of the
swellings. The teeth in relation to these cysts are vital.
attached or interdental gingiva, most commonly between
5th and 6th decade of life and usually do not exceed 1 cm
in size.
The common sites of involvement are the mandibular The cyst reveals no radiographic findings. However, in large
canine and premolar region. Occasionally, these may be cysts superficial erosion of bone represented by a faint
seen in the lateral incisor, canine, premolar regions of the shadow of radiolucency may be appreciated.
309
Figure 12 Figure 14
Orthopantomograph (OPG) showing a multilocular

radiolucency in relation to the lower premolars, first and
second molars causing displacement of the second molar
in orthokeratinized odontogenic cyst. Courtesy: Department
of Oral Medicine and Radiology, MCODS, Mangalore
Surgical specimen of dentigerous cyst.
Courtesy: Dr NVS Sekhar Reddy
Figure 15
Figure 13
Gross specimen of orthokeratinized odontogenic cyst

showing keratin. Courtesy: Department of Oral Pathology,
MCODS, Mangalore
Photomicrograph of orthokeratinized odontogenic cyst.
Courtesy: Department of Oral pathology, MCODS,
Mangalore
or plaque formation (similar to those evident in lateral

periodontal cyst).
Hegde (2004) reported a rare finding (supposedly the
The thinner form of epithelium mimics the reduced enamel
first case in literature) calcifications within cystic lumen
epithelium. It is generally composed of one to three layers
of the gingival cyst.
of flat or cuboidal cells. The thicker variety may not show
any rete pegs. The nuclei are pyknotic and show perinuclear
Histologic findings
cytoplasmic vacuolation. The localized thickening of epi-
Histologically, these cysts have a variety of presentations. thelium or plaque may occasionally project into the cystic
The epithelium generally has three forms of presentation: cavity. The cells in the localized thickenings have a whorled
(i) extremely thin epithelium, (ii) thicker stratified squa- morphology. Rarely, these cells are swollen and clear and
mous epithelium and (iii) localized epithelial thickenings are referred to as water-clear cells.
310
Management Management
The cyst is removed surgically. Recurrences have not been Lateral periodontal cysts can be effectively managed with
reported. enucleation. A sound tooth can be retained. Recurrences
though uncommon, have been reported in literature.
Lateral Periodontal Cyst Botryoid Odontogenic Cyst

Lateral periodontal cyst is a non-keratinized developmental Botryoid odontogenic cyst (BOC) is considered as a variant
cyst occurring in the alveolar bone along the lateral aspect of the lateral periodontal cyst. Weathers and Waldron in
of a vital tooth. 1973 first used this term to describe a multilocular cystic
It is a relatively uncommon cyst that is slow growing, lesion of the jaw that on gross morphology resembled a
non-expansile developmental odontogenic cyst derived from bunch of grapes.
one or more rests of dental lamina, containing an embry- The exact nature of this cyst is still not understood.
onic lining of 1–3 squamous/cuboidal cell thickness and Some authors consider the BOC as a multilocular variant of
distinctive focal thickenings (plaques). These appear to lateral periodontal cyst. Others consider BOC and lateral
have no gender predilection. According to Altini and Shear periodontal cyst as two distinct unrelated entities. They
(1992), lateral periodontal cysts represent about 0.8% of consider BOC as a multicystic odontogenic lesion with the
all central cysts (intraosseous) of maxillary bones. These cysts histologic characteristics of lateral periodontal cysts.
are usually seen in adults between the 2nd and 8th decade Unlike the lateral periodontal cyst that usually has a uni-
of life. locular appearance, the botryoid odontogenic cyst is typi-
These are usually seen by chance in routine radio- cally polycystic (multilocular), but occasional unilocular
graphs. These are intraosseous forming beside vital tooth. cases have been reported.
These might even form near the crest of ridge. These cause However, histopathologically the BOC resembles lat-
no symptoms unless they erode through the bone to extend eral periodontal cyst. Another striking difference between
into the gingiva. Some patients may present with a swelling BOC and lateral periodontal cyst is its rate of recurrence.
on the buccal aspect of the gingiva. The most common site It is estimated that the recurrence rate of BOC is between
of involvement is the mandibular premolar area followed 15 and 33%, which is much higher than lateral periodon-
by the anterior part of the maxilla. tal cyst.
Many authors suggest the fact that the lateral periodon- High et al (1996) proposed the term ‘polymorphic odon-
tal cyst is more or less a histopathological diagnosis rather togenic cyst’. This term included GOC, mucoepidermoid
than a clinico-radiographic diagnosis. intraosseous carcinoma and botryoid odontogenic cyst.
Greer Jr and Johnson (1998) studied the clinicopathologic
Histologic features features of 10 botryoid odontogenic cysts. In their study
the radiographic size of the cysts varied between 5 and
The cyst is lined by a thin non-proliferating cuboidal to
45 mm radiographically.
stratified squamous non-keratinizing epithelium, ranging
It is believed that the size and multilocular pattern could
from 1 to 5 cell layers, and thus resembling the reduced
be the main factors responsible to the high recurrence rates
enamel epithelium.
of botryoid odontogenic cyst. The high recurrence rates
The cyst wall and the lining usually show no signs of
warrant the need for a more aggressive treatment. Some
inflammation. The lateral periodontal cyst presents two
authors suggest the systematic use of Carnoy solution along
important histologic features, namely, the presence of epi-
with enucleation and curettage.
thelial thickenings or ‘plaques’ and the presence of glycogen-
rich clear cells either in ‘plaques’ or in the superficial
layers of the lining epithelium.
CALCIFYING EPITHELIAL ODONTOGENIC
CYST (Gorlin Cyst)
The cyst may appear as a round, oval or teardrop-like well- It was thoroughly described by Gorlin and coworkers in
circumscribed interradicular radiolucent area, usually with 1962 and in 1963. Gold introduced the terms ‘keratinizing’
a sclerotic margin, lying usually between the apex and the and ‘calcifying odontogenic cyst’. It reportedly accounts for
cervical margin of the teeth. Resorption of adjacent teeth about 2% of all odontogenic pathologies affecting the max-
though uncommon, has been reported. Occasionally, loss illa and mandible. It may be found along with other odon-
of lamina dura and widening of the periodontal ligament togenic tumors. However, in 24% of the cases it is
space may be present. associated with odontomas.
311
The calcifying odontogenic cyst can either occur as an

Figure 16
intraosseous (central) or extraosseous (peripheral) lesion.
The peripheral form is very rare. The incisor and canine
regions are the most common sites of involvement. The
maxilla is more commonly affected. It can occur at any age.
However, most cases are seen in the 2nd decade of life.
Types
In 1981, Praetorius and coworkers attempted to classify
calcifying odontogenic cyst by categorizing it into two enti-
ties: cyst and neoplasm.
Cystic entity
It was classified as:
❍ Type 1: A simple monocystic type of typical Gorlin’s
cyst, with or without dentinoid calcified tissue.
❍ Type 2: Monocystic odontoma creative type, with all Intraoral photograph showing swelling in relation to
the characteristics of the previous type, except that the the attached gingiva of the right maxillary incisor and
hard tissue was complex or compound odontoma, and canine region. Courtesy: Department of Oral Medicine and
a presence of ameloblastic fibroma tissue in the cystic Radiology, MCODS, Mangalore
wall extending into the surrounding tissue.
❍ Type 3: Monocystic ameloblastomatous proliferating
type which was marked by ameloblastomatous prolif-
eration both in the walls and in the lumen, and hard Figure 17
dental tissue which consisted of dentinoid formation in
connection with islets of epithelia in the connective wall.
Neoplastic entity
It was described as an odontogenic tumor with ghost cells.
The epithelial elements consist of numerous ameloblasto-
matous proliferations of tissue in the connective tissue
of the stroma. Varying amounts of ghost cells are present
within the epithelial islets. The hard tissue is composed of
different amounts of dentinoid in direct contact with the
epithelium.
Clinical features
It produces slow growing painless non-tender swelling of
jaws (Figures 16 and 17). It occurs more in 2nd decade of
Intraoral photograph showing expansion of the labial and
life. It is commonly seen in the anterior part of the jaws. In
palatal cortical plates in the right maxillary incisor–canine
the maxilla, the canine region is the most commonly affected region. Courtesy: Department of Oral Medicine and
site. In the mandible, the cyst is rarely found posterior to the Radiology, MCODS, Mangalore
first molar. Occasionally the cyst may be seen crossing the
midline. On aspiration, it yields viscous granular yellow
fluid. According to Wood et al, 68% of cases occur in man-
smooth and well-defined or, irregular and poorly defined.
dibular molar area. At least 52% cases are associated with
It might contain small foci of calcified material. It is usu-
unerupted tooth, occasionally associated with pain.
ally present as a unilocular radiolucency. However, in
about 5–13% of the cases, it may present as multilocular
radiolucency. Intraosseous lesions may produce expansion
The radiographic features are quite variable. The central of the lingual cortical plate. Resorption of the roots of adja-
lesion may be radiolucent with a variable margin that is cent teeth is frequently seen (Figure 18).
312
Figure 18 Figure 19
Excised gross specimen of calcified odontogenic cyst (COC)

Maxillary occlusal radiograph showing a well-defined showing the cyst and the calcified mass. Courtesy:
unilocular radiolucency in the incisor–canine region Department of Oral Pathology, MCODS, Mangalore
containing radiopaque foci of calcification. Resorption of
the apex of the right central incisor can be appreciated.
MCODS, Mangalore odontogenic cyst, although controversy still exists regard-
ing its origin and the terminology.
Current literature review reveals the mention of only
23 cases of GOC. These appear to be odontogenic in origin
Differential diagnosis and present as a well-defined radiolucent swelling of the
jaws with a tendency to recur following conservative
Calcifying odontogenic tumor and ameloblastoma.
treatment. These occur over a wide age range with no gen-
der, race or ethnic predilection.
Management and prognosis The cyst is possibly derived from rests of dental lamina
The management of calcifying odontogenic cyst is primar- and comprises both secretory elements and stratified squa-
ily by enucleation and curettage (Figure 19). Some authors mous epithelium.
describe the possibility of malignant transformation. Only
eight cases till date have shown recurrences. Clinical and radiographic features
Glandular odontogenic cyst is commonly seen in the ante-
rior mandible. Occasionally maxillary involvement has been
GLANDULAR ODONTOGENIC CYST reported. The usual complaints are swelling, pain or dis-
comfort in the involved area (Figure 20). It is usually seen
as a slow-growing swelling. Pain is very unusual but has
It is also called sialo-odontogenic cyst, mucoepidermoid
been reported.
odontogenic cyst or polymorphous odontogenic cyst.
Reports in literature mention the occurrence of GOC in
Padayechee and Van Wyk were the first to describe a
a wide age range (14–80 years), however it is relatively
unique cyst of the jaws in 1987 which did not fit into the
common in the 4th and 6th decades of life.
standard classification of odontogenic cysts and tumors.
Radiographically it may be a unilocular or multilocular
Due to its unique histopathologic features, they suggested
well-circumscribed radiolucency usually displacing the roots
the term sialo-odontogenic cyst to denote a possible origin
of teeth (Figures 21 and 22).
or association with salivary gland tissue. Gardner et al (1988)
reported the first series of these cysts and used the name
glandular odontogenic cyst (GOC), stating that the presence
of mucous cells in the cyst lining did not imply an origin from The histopathologic features of this cyst have been described
salivary glands. as a combination of findings from a botryoid odontogenic
WHO recognized the name of this pathological entity cyst and a mucoepidermoid carcinoma, often causing a
as GOC in 1992. It was classified as a developmental diagnostic dilemma for pathologists.
313
❍ Little inflammation
Figure 20
❍ Occasional findings of hyperchromatic basal cells
within the cyst lining.

Glandular odontogenic cysts can be treated conservatively
by enucleation and curettage. It is believed that almost
55% of the cases show recurrence. The recurrence rate is
high because the thin cyst wall, tendency of epithelium to
separate from connective tissue or growth through cancel-
lous spaces of bone and presence of microcysts makes
complete removal of the cyst nearly impossible. Thus,
some authors suggest a local block excision. However, it is
wise to follow up the patient for several years to assess
any form of recurrence.
Intraoral photograph showing minimal obliteration of the

labial vestibule in relation to the maxillary anterior teeth. NON-ODONTOGENIC CYSTS
MCODS, Mangalore
Non-odontogenic cysts including developmental cysts
account for about 6% of cysts in the orofacial region.
Figure 21
Nasopalatine Duct Cyst
The nasopalatine duct cyst or incisive canal cyst was first
described by Meyer in 1914. Nasopalatine duct cyst is the
most common among the non-odontogenic cysts. It is
estimated that the cyst occurs in about 1% the population.
The etiopathogenesis of the cyst is still unclear. However,
it is thought that the cyst arises from a cystic degeneration
of the embryologic remnants of nasopalatine duct. Some
authors believe that trauma, bacterial infection of the naso-
palatine duct or mucous retention possibly predisposes to
the formation of the cyst.
Clinical features
Nasopalatine duct cyst is seen to affect individuals between
Maxillary occlusal radiograph showing well-defined
unilocular radiolucency extending from the mesial aspect of
the 4th and 6th decades of life. The cyst is seen in the
the maxillary right side lateral incisor to the mesial aspect of midline of the anterior maxilla approximating the incisive
the left side lateral incisor, causing displacement of teeth. foramen. It is seen slightly more frequently in men than in
Courtesy: Department of Oral Medicine and Radiology, women (3:1). The cysts often present as asymptomatic
MCODS, Mangalore swelling of the palate but can present with painful swell-
ing or discharge (mucoid or purulent in nature). The max-
illary central incisors may occasionally be displaced. The
Gardner proposed the diagnostic criteria for GOC which associated teeth are vital. A clear or straw-colored fluid is
included the presence of: obtained on aspiration.
❍ A cystic cavity lined by epithelium of varying thick-

Histologic features
ness with a flat interface between the epithelium and
underlying connective tissue The nasopalatine duct cyst may be lined by various types
❍ Variable numbers of mucous cells in the epithelium of epithelium such as stratified squamous epithelium, pseudo-
❍ Eosinophilic cuboidal cells in the superficial layer stratified columnar epithelium or cuboidal epithelium.
❍ Localized plaque-like thickening of the epithelium Abrams (1963), Gnanasekhar (1995) and Swanson (1991)
314
Figure 22
Orthopantomograph (OPG) showing well-defined unilocular radiolucency extending from the mesial aspect of the maxillary
right side lateral incisor to the mesial aspect of the left side lateral incisor. Courtesy: Department of Oral Medicine and
reported that about 9.8% of the nasopalatine duct cysts known by other names such as Klestadt’s cyst, nasoalveolar
are lined by respiratory epithelium. cyst, nasal vestibule cyst, nasal wing cyst and mucoid cyst
of the nose. Though the exact etiology for the cyst forma-
Radiographic features tion is unknown, Bruggeman (1920) suggested that the
cyst possibly originates from the remnant of embryonic
Radiographically, nasopalatine duct cyst is apparent as a nasolacrimal duct.
well-defined round, oval or inverted pear-shaped radiolu-
cency. Occasionally, the nasal spine is superimposed over Clinical features
the radiolucency imparting it a heart shape.
It is located in the midline in the anterior maxilla. The Nasolabial cyst is usually seen in the 4th and 5th decade
cyst is typically located in the interradicular region, apical of life. It is reportedly seen 3–4 times more commonly in
to the maxillary central incisor teeth. The cyst is well cor- women. Clinically patients present with a soft tissue swell-
ticated, unless infected. Adjacent teeth are usually displaced. ing that may cause obliteration of the nasolabial fold, and
However, the roots are rarely resorbed. elevate the ala of the nose. Intraorally, a swelling is seen
Large cysts can cause expansion of the palatal cortical in the labial vestibule. However, about 11% of the cases
plate and occasionally cause destruction of the floor of present with bilateral involvement. A nasolabial cyst is usu-
the maxillary antrum. Radiographically, when the diame- ally not painful, unless it is secondarily infected. The cyst is
ter of the incisive canal exceeds 6 mm, a nasopalatine cyst fluctuant on palpation. The cyst may rupture spontaneously
should be considered. On MRI, the cyst is identified as a or drain orally, nasally or sometimes drains extraorally
high-intensity, well-marginated lesion, indicating the pres- through a fistula. Occasionally, the swelling may encroach
ence of proteinaceous material. upon the lateral wall of the nasal vestibule causing nasal
obstruction. Large infected cysts can cause referral of pain
Management to the maxillary anterior teeth.
The cyst can be successfully managed with enucleation. Radiographic findings

It has low recurrence rates.
Although nasolabial is a soft tissue cyst, it can occasion-
ally cause erosion of the underlying maxillary bone which
may be appreciated on radiographs. Intraoral periapical
NASOLABIAL CYST radiographs and maxillary occlusal radiographs are gener-
ally sufficient to evaluate the extent of the cyst. Localiza-
Nasolabial cyst is classified as developmental, non-odon- tion technique may help in excluding cysts of odontogenic
togenic soft tissue cyst. It occurs in the nasolabial region. origin. Alternatively, the content of the cyst may be
It is estimated that the nasolabial cyst accounts for only aspirated and a radiopaque contrast agent can be injected
0.7% of the cysts of the jaws. The nasolabial cyst is also into the cyst followed by a posteroanterior projection or
315
occlusal radiograph. Posteroanterior view will reveal a to be derived from palatal salivary gland structures and
spherical or kidney-shaped (bean-shaped) cyst. The cyst generally regress within the first 3–4 months of life.
may cause depression of the underlying labial surface of Epstein pearls are cystic keratin-filled nodules found
the maxillary bone (secondary to pressure). The cyst may along the mid-palatine raphe and are thought to be derived
also cause distortion of the curve of the ‘bracket-shaped’ from entrapped epithelial remnants along the line of fusion.
line produced by the inferior margin of the anterior bony Epstein pearls are named after Alois Epstein (1849–1918),
aperture of the nose. Literature review reveals rare cases of a Czech pediatrician.
root resorption.
Histologic features
Histologic features The cyst is lined by parakeratinized stratified squamous
Almost all cysts are lined with pseudostratified columnar epithelium. Occasionally epithelial lined clefts are seen
epithelium. Sixty percent of the specimens show the pres- between the cyst and the surface epithelium of the oral
ence of goblet cells. mucosa. The basal cells are flat. The cystic lumen contains
concentric layers of keratin.
Management
Management
Nasolabial cysts have been managed with injection of
sclerotic substances, marsupialization and surgical removal. Mid-palatal raphe cysts disappear within the first few months
However, surgical excision is the treatment of choice. of life. Studies by Moreillon and Schroeder (1982) showed
Occasionally, perforation of the nasal mucosa is seen owing that with time the cystic epithelium differentiated, fused
to the close relation of the cyst to the nasal floor. Large with the oral epithelium and their contents were discharged.
defects have to be sutured.
CYSTS OF MAXILLARY ANTRUM AND

MID-PALATAL RAPHE CYST OF INFANTS SALIVARY GLANDS
It is also known as median palatine, median alveolar and The cysts associated with the maxillary sinus are either
median mandibular cysts. extrinsic or intrinsic in origin.
The terms ‘median palatine’, ‘median alveolar’ and These can be classified as:
‘median mandibular cysts’ are no more used in literature. It
1. Extrinsic cysts
is believed that the median alveolar and median palatine
2. Intrinsic cysts
cysts are the anterior and posterior extensions of the well-
a. Mucous retention cyst
known incisive canal cyst. The use of the term ‘median
b. Serous cysts
mandibular cyst’ is controversial as most cases in literature
c. The mucocele.
that describe this cyst are associated with non-vital man-
dibular anterior teeth (mimicking radicular cysts) or on
histopathological examination resemble keratocysts. Extrinsic Cysts
Two theories have been proposed to explain the origin
The extrinsic variety develops in structure adjacent to sinus
of the palatal cysts of the newborn: entrapment of the
and as they expand they may encroach upon the sinus air
epithelium along the mid-palatine raphe during fusion of
space. Extrinsic cyst may be odontogenic in origin or infre-
the palatal shelves in embryonic life or from remnants of the
quently non-odontogenic.
epithelium derived from the development of palatal minor
salivary glands.
The mid-palatal raphe cyst is a developmental cyst of Intrinsic Cysts
non-odontogenic origin. These cysts are usually present
This cyst is referred to in literature by a plethora of names
along the mid-palatal raphe at the junction of the hard and
like mucosal cyst, mucosal antral cyst, mucous cyst,
soft palate. Usually four to five cysts are seen together.
mucous retention cyst secreting or secretory cyst, muco-
They measure between 2 and 4 mm in diameter.
cele serous cyst, non-secreting or secretory cyst, mesothe-
Bohn’s nodules and Epstein pearls are common cysts that
lial cyst, lymphangiectatic cyst, interstitial cyst, pseudocyst
occur in an infant’s palate. Bohn’s nodules are smooth
and false cyst. Concurrent concept permits the usage of term
surfaced cysts that are found at the junction of the hard
‘mucosal cyst’. There are three types of mucosal cysts:
and soft palate, and occasionally scattered all over the
palate. These nodules are usually 1–3 mm in size, and filled a. The mucous (secreting or retention cyst)
with keratin. Bohn’s nodules are named after the German b. The serous (non-secreting cyst)
pediatrician Heinrich Bohn (1832–1888). These are thought c. The mucocele.
316
The mucocele frequently causes the expansion of the bony

Figure 23
antral wall whereas mucous and serous do not. If the
ostium is not occluded by intra-antral or intra-mucosal
inflammation, polyps or bony tumors, it provides an
avenue or release for expanding cystic pressure. Release
occurs either by rupture of the cyst resulting from abrupt
pressure changes accompanying sneezing or blowing of
the nose or by the cyst herniating though the ostium into
the nasal cavity, where it is subsequently ruptured. If the
ostium is blocked, cystic pressure progressively increases
and erodes the antral wall. The cysts associated with such
pressure atrophy are referred to as ‘mucocele’, an expanding
destructive cyst.
Benign Mucosal Cysts

CT axial section showing a large mucosal cyst in the
The intrinsic benign (non-destructive) cysts are most com- right maxillary sinus
mon in maxillary sinus and are assumed to be two patho-
genically different varieties:
Clinical and radiographic features
1. A mucous or secretory retention cysts
2. Serous, non-secreting cysts. The occurrence rate of this cyst is 1.47–13% of general
population. This cyst is found in all age groups; about 11%
of the cysts are diagnosed in children. Males are twice more
Mucous Retention Cysts affected compared to females. Symptoms accompanying
this cyst are general fatigue, dizziness, pain exacerbated
The mucous retention cysts result from the obstruction of by jarring, a sense of fullness or numbness in the ipsilat-
some ducts of the seromucinous glands in the lamina pro- eral cheek, toothache, frontal headache, nasal obstruction
pria of the sinus lining. The obstruction is secondary to and occasional copious post-nasal discharge (when the
local inflammation that may be either infectious or allergic cyst ruptures), optic neuritis, serous otitis media and some
in origin. The occluded glands continue to secrete, causing arthritis, specially in the knee.
their dilatation and resulting in accumulation of a mucoid Radiographically both varieties of cyst may be recog-
material forming cyst. The cyst is lined with either normal nized as a single curved, homogeneous, highly radiopaque
sinus (respiratory) epithelium or a flattened cuboidal or area (normal anatomic structures can usually be observed
squamous epithelium as a result of metaplasia induced by through the cyst) that is spherical, ovoid or dome shaped
intracyst pressure. The liquid that collects inside the cyst (Figure 23). Their outlines are usually smooth, uniform and
lumen may be sampled by intranasal needling as a diagnos- well-defined by radiolucent air space of the sinus. The cyst
tic aid. It is thick, tenacious, white translucent and sterile. can develop any where in the cavity. The base of the lesion
may be broad or narrow. The mucous cysts usually have a
broad base while serous cysts are more pedunculated. The
Serous Non-secretory Cysts
lesions are usually single but multiple cysts and bilateral
The serous cysts probably arise as a result of cystic degen- involvement of the sinuses have been reported. The size of
eration within an inflamed, thickened sinus lining or in the mucosal cyst varies from a few millimeters to more
large mucosal polyp. As the defect in the lamina propria of than 1,000 mm.
the affected mucosa becomes distended with edema fluid, The mucous cysts are usually small in size and hidden
they coalesce and form a cystic space that crowds and in intrasinus fluid (pus) and not recognized until it drains.
packs the surrounding connective tissue fibers forming the The condition of lining of the other part of the sinus also
cyst wall. This sequence of events produces the develop- helps to distinguish mucous variety which is associated
ment of a distinct epithelial lining, a fact surrounded by with thickened mucosa in majority of the cases. The serous
histological examination of these cysts. The fluid within cysts when involved, lining appears normal. The broad
the cyst is believed to be a transudate from the adjacent based mucous cyst will not change in position while the
osmotic pressure of the cyst fluid. It is similar in composi- serous cyst shows slight changes in contour in a radiograph
tion to plasma. The cause of cyst is uncertain. It seems that when the position of patient is altered in second projection.
inflammation accompanying sinusitis or reported allergy Transillumination does not usually provide diagnosti-
or a tooth extraction plays a role. cally useful information when compared to a radiograph.
317
It will help in distinguishing between the mucous and serous and bony sclerosis will be associated in antral polyps
cysts by demonstrating different translucency. The mucous and not in antral cysts. Cysts are solitary, polyps are fre-
cyst shows decreased translucency compared to serous quently multiple. Hyperplasia as a result of chronic dental
because the serous cyst has a high concentration of doubly infection is frequently seen in a radiograph of the antral
refractory cholesterol crystals. When aspirated, serous type floor, adjacent to teeth with pulpoperiapical problems or
will show a clear, amber fluid with high concentration of advanced periodontal diseases. Both the lesions may show
cholesterol crystals. In contrast, the mucous cysts will similar convex, homogeneous, faintly opaque shadows. The
show thick, whitish, opalescent mucous. differentiating feature of hyperplasia of dental infection is
the presence of a chronically infected tooth. Usually, dental
Investigations infection is not seen in association with benign mucosal
cyst or if present, is not considered as an etiological factor.
Periodic radiographic studies show benign mucosal cysts
remain stationary in size, completely disappear, decrease
or increase in size over an interval of a month. However, Extrinsic Cysts
majority of cysts regress spontaneously.
Surgical exploration and direct examination of the cyst Cysts like radicular, dentigerous, primodial and other
that have herniated into the mouth through the oro-antral odontogenic cysts may encroach on the sinus, but rather
fistula shows a round, distended, translucent mass with a easily be identified by their characteristic location in the
light bluish or yellowish color. The walls are thin and rup- jaws or their relationship to a tooth. Extrinsic cysts will
ture readily, yielding a watery fluid or a thicker sticky retain a thin curved radiopaque rim of bone which sepa-
mucoid fluid. rates the cyst from the antral shadow.
Microscopic studies show that the vast majority of those Cysts associated with the salivary glands are described
are non-secretory variety and that glandular elements are in Chapter 11 on Diseases of Salivary Glands.
not associated with these cysts. There are false cysts whose
lumina are not lined with epithelium but with the connec-
tive tissue. The outer surface of the cysts is lined with respi- INFLAMMATORY CYSTS
ratory epithelium which shows areas of metaplasia. The
lumina of these serous cysts are filled with amber fluid
Periapical Cyst (Radicular Cyst, Root End Cyst,
in which there are few inflammatory cells and suspended
Apical Periodontal Cyst)
cholesterol crystals. This layer of submucosa is sometimes
edematous and contains varying number of round cells. Its Periapical cyst is the most common inflammatory odonto-
luminal lining is composed of columnar epithelium with genic cyst. It is associated with non-vital teeth. The tooth
some mucous glands. As a result of pressure, the lining might be deeply carious, traumatized or improperly
epithelium may undergo metaplasia and appear as flat restored.
cuboidal epithelium.
Etiopathogenesis
Bacteria invade the tooth followed by the death and deg-
Benign mucosal cysts of the sinus must be differentiated radation of pulp. The first line of defense in the periapical
from other soft tissue lesions of the sinus such as polyps, area to seal the apex is the proliferation of epithelial rests
hyperplasia of dental infection, extrinsic cyst, sinusitis and of Malassez in the periodontal ligament. They proliferate to
malignant soft tissue tumors. This presents the primary conform a granulation tissue called periapical granuloma.
cern since the character of opacities produced by the tumor This consists of highly vascular tissue with immunocom-
fluid or thickened mucosa is not anyway different. A large petent cells like lymphocytes, macrophages, plasma cells.
cyst may fill sinus causing complete opacification and As the time elapses, the central area is deprived of nour-
obscure cystic characteristic feature. Other lesions like intra- ishment. The cells undergo liquefaction necrosis sur-
sinus fluid, calcified entities such as condensing osteitis, root rounded by epithelium to form a cyst.
tip and other foreign bodies, antroliths and odontomas gen-
erally have characteristic outline and much denser images
Clinical features
when compared to mucosal antral cyst, which presents as
a homogeneous, only slightly radiopaque appearance. More often (60%) these occur in the maxillary anterior
Antral polyps are much less common than benign region. These might cause painless bony expansion of either
mucosal cyst of the antrum. Antral polyps will not occur on buccal or palatal cortical plate (Figure 24).
the floor or inferolateral aspect of the sinus and are also The cortex might be intact where it is hard or fluctuant.
associated with thickened mucosa in contrast to normal When perforated, the bone thins out and exhibits crepitus.
appearing lining mucosa. Bone displacement, destruction However, occasionally the patient may complain of pain
318
Figure 24 Figure 25
Straw-colored aspirant from a periapical cyst.

Figure 26
Intraoral photograph showing a discolored right

maxillary central incisor and palatal swelling suggestive
of a periapical cyst. Courtesy: Department of Oral Medicine
when the cyst is secondarily infected. Sometimes, a

cyst may be seen at the opening of lateral accessory
root canals of a tooth, this may be referred to as ‘lateral
radicular cyst’.
In deciduous teeth Periapical cysts are rarely seen in Maxillary occlusal radiograph showing a well-defined
deciduous dentition. It is estimated that only 0.5–3.3% of radiolucency extending from the periapex of the maxillary
the radicular cysts occur in deciduous dentition. Most of right lateral incisor. The cyst is bound by a well-defined
the cysts are seen in relation to the mandibular molars and sclerotic border. Courtesy: Department of Oral Medicine and
generally discovered incidentally on radiographs. It has Radiology, MCODS, Mangalore
been seen that cysts in primary teeth are characteristically
present in the interradicular region and not near the apex
of the teeth. Many authors believe that this is due to the (Figure 26). In cysts that are secondarily infected and
short and resorbed roots as well as the presence of multi- those rapidly expanding the sclerotic border may be indis-
ple accessory canals in primary teeth. Hence, the term tinct. The radiolucency may sometimes appear hazy due to
‘periradicular cyst’ is more appropriate. Expansion of the pus accumulation. Few cysts might expand into the sinus
mandibular buccal cortical plate and displacement of the and elevate the floor. Root resorption is rarely seen.
underlying permanent tooth buds are usually seen associ-
ated with periradicular cysts. Histopathology
On aspiration, the contents of the cyst may vary from
brown (breakdown products of blood) to straw color (pres- Most of the radicular cysts are lined by stratified squamous
ence of cholesterol crystals) depending on the content of epithelium (Figure 27). In instances where the periapical
the cyst (Figure 25). cysts are in proximity to the maxillary sinus, the cyst
may exhibit pseudostratified ciliated columnar epithelium.
Rarely the periapical cysts are lined with ortho or parake-
ratinized epithelium.
It appears as well-defined periapical radiolucency associ- It is estimated that 10% of the cases reveal the presence
ated with apex of affected tooth. A diagnosis of periapical of Rushton’s hyaline bodies. These are generally found in
cyst is considered when the cyst measures at least 1.5 cm epithelial linings and rarely found in the fibrous capsule.
or more in diameter. The lesion is continuous with the lam- These are hairpin shaped, arch shaped or linear eosino-
ina dura of the tooth. The lesion will have sclerotic border philic structures that are amorphous and brittle. Presence
319
Figure 27 Figure 28
Orthopantomograph (OPG) showing residual cyst in the

edentulous site. Courtesy: Department of Oral Medicine
Photomicrograph of radicular cyst showing stratified

Residual Cyst
squamous epithelium with arcading pattern, cystic lumen and It is estimated that 10% of the cysts of odontogenic origin
connective tissue capsule. Courtesy: Department of Oral are residual cysts. Residual cysts are cysts that are left behind
Pathology, MCODS, Mangalore
following partial enucleation of a radicular cyst or a
retained radicular cyst after the tooth has been extracted.
Many authors believe that a residual cyst may also develop
of cholesterol clefts is another important feature of radicu- from partial removal of a dentigerous cyst. It may also form
lar cysts. The fibrous capsule of the cyst is made up of dense from a periapical granuloma after removal or exfoliation of
bundles of collagen fibers. Usually chronic inflammatory tooth. Subsequent cystic degeneration may take place in
cells are seen in the connective tissue approximating the the granuloma.
epithelium.
Simon (1980) described the presence of an epithelium
Clinical features
lined cavity that may open to the root canal in small peri-
apical lesions. Simon termed this ‘bay cyst’. Subsequently Residual cysts are mostly detected incidentally on radio-
Nair et al (1990) studied 256 periapical lesions. Their infer- graphs. The cyst is evident in edentulous site (Figures 28
ence was that 61% of the identified cysts fulfilled the cri- and 29). The patient may give a history of extraction of
teria of true periapical cysts and the rest were apical the tooth because the tooth was grossly carious or fractured
inflammatory lesions that contained a sac-like, epithelium- to involve the pulp.
lined cavity, continuous with the root canal. This epithe- It is almost always asymptomatic. Amber-colored aspirant
lium formed an attachment that seemed to seal off the is expressed on aspirating residual cysts.
infected root canal and its apical pouch from the periapical
region. It was suggested to term this a ‘periapical pocket Management
cyst’ rather than a ‘bay cyst’.
Residual cysts are surgically removed. However, large
Differential diagnosis residual cysts may be marsupialized.
The lesions that might resemble cyst radiographically are

periapical granuloma, periapical scar and surgical defect. Paradental Cyst
Craig is credited for describing the paradental cyst in
1976. The term ‘paradental cyst’ is reserved for those cysts
Root canal treatment or extraction of tooth along with the that are located in relation to the distal aspects of the roots
removal of the cyst. Depending upon the size and involve- of partially erupted third molars. Most patients have an
ment of other tissues, marsupialization or enucleation are associated chronic pericoronitis. Craig believed that the
done. Recurrence unlikely if removed completely. reduced enamel epithelium provided the cells of origin of
Literature review reveals descriptions of squamous cell the paradental cyst. However, other authors also believe
carcinoma originating from the epithelial lining of radicu- that the paradental cyst may originate from the cell rests
lar and residual cysts. of Malassez.
320
Figure 29 Clinical features

It is commonly seen in young children in the 6–11 year
age group. The cyst causes a buccal tilting of the crown of
the involved tooth. The associated tooth is vital. On clini-
cal examination, deep periodontal pockets may be evident
on the buccal aspect of the tooth. Occasionally, pain or
swelling may be present.
Periapical radiographs, occlusal radiograph and orthopan-
tomograph may be required to evaluate the presence of
buccal bifurcation cyst. Radiographically, the lamina dura
and the periodontal ligament space are unaffected. The cystic
radiolucency is always located on the buccal aspect of the
molar, which can be best appreciated on occlusal radiograph.
Occasionally, subperiosteal new bone may be laid down,
which may appear laminated.
Orthopantomograph (OPG) showing residual cyst in the

Management
edentulous site. Courtesy: Department of Oral Medicine The cyst can be managed by curettage and extraction of the
and Radiology, MCODS, Mangalore tooth. Some authors recommend enucleation.
PSEUDOCYSTS
Clinical features
It is also known as traumatic bone cyst, aneurysmal bone
Paradental cysts are mostly seen involving the mandibular
cyst, Stafne’s bone cyst.
third molars. However, maxillary third molar involvement
has also been reported in literature. Most of the cysts cover
the bifurcation of the roots and are usually attached to Traumatic Bone Cyst
the buccal root surface. Teeth are vital. Both sexes may
be affected. However, males are more commonly affected. The term ‘traumatic bone cyst (TBC)’ was used by Lucas in
These cysts are commonly seen in the 2nd and 3rd decades 1929. Though the term is popularly and widely used, lit-
of life. erature review reveals that other terms such as solitary
Radiographically, a well-defined radiolucency is seen bone cyst, hemorrhagic bone cyst, simple bone cyst and
in relation to the distal aspect of a partially erupted third unicameral bone cyst are used.
molar. Generally the distal part of the radiolucency is sep- The pathogenesis of the TBC is still not well understood.
arate and distinct from the distal follicular space of the However, trauma is the most frequently discussed etiologic
third molar. The width of the periodontal ligament space is factor in the formation of a TBC. According to Pommer,
unaffected. trauma leads to intraosseous hematoma formation. The
Histological features resemble that of radicular cyst. blood clot liquefies, unlike the normal healing process, and
The paradental cyst is managed effectively by surgical adjacent bone is destroyed by enzymatic activity. Blum
enucleation. The partially erupted third molar can be and Thoma suggested that a previous traumatic episode to
removed. the jaws contributed to the development of TBCs. Thoma
proposed that trauma initiates a subperiosteal hematoma
which causes a compromised blood supply to the area,
Mandibular Infected Buccal Cyst thereby leading to osteoclastic bone resorption. However,
even this theory is debatable as it is estimated that almost
(Mandibular Buccal Bifurcation Cyst)
50% of the patients do not give history of trauma. And even
The first description of the mandibular infected buccal if there is a history of trauma it does not coincide with the
cyst was given by Stoneman and Worth in 1983. The buccal time of the development of the cyst. Another explanation
bifurcation cyst is an uncommon lesion associated with the against trauma as the etiological agent is that, trauma usu-
permanent mandibular first or second molar in children. ally occurs on the anterior part of the mandible, whereas
Usually, it is identified just prior to eruption. the cyst occurs more frequently in the body of the mandible.
321
Clinical features Radiographically, it is characterized by ballooned out expan-

sion of the periosteum which is usually outlined by a wafer
The cyst is usually seen in the 2nd decade of life. It is
thin subperiosteum. This is, in turn, bounded by an area of
slightly more common in males. The cyst is generally
disintegrated cortex.
asymptomatic and hence incidentally discovered on routine
radiographic examination. Approximately 10–30% of the
Clinical features
patients complain of pain. Traumatic bone cysts frequently
affect the mandibular body (canine to molar region), fol- Aneurysmal bone cyst is commonly seen in the 2nd decade
lowed by the mandibular symphysis and rarely the maxil- of life. It is rarely seen after the 3rd decade of life. It is
lary anterior region. The ramus and condylar regions are believed that females are more commonly affected than
hardly involved. males.
Other features that may be noticed are tooth sensitivity, Literature review reveals that the mandible is more
paresthesia and delayed eruption of permanent teeth. commonly affected than the maxilla. The molar regions of
Aspiration may occasionally yield a straw-colored fluid or the maxilla and mandible are the most common sites of
bright blood. involvement. Extensive lesions in the mandible may involve
the angle and ascending ramus of the mandible. Rare sites
Radiographic features of involvement such as the coronoid process of the man-
dible, floor of the orbit and the zygomatic arch have been
Most TBCs are found incidentally on routine radiographic
reported in literature.
investigations. Radiographically, it is seen as a well-defined
Clinically the swelling is firm on palpation. Aneurysmal
radiolucent area with or without a sclerotic border. The
bone cysts tend to enlarge rapidly and cause thinning and
radiolucent area is commonly seen in the body of the man-
perforation of the overlying cortical plate. Some patients
dible and the symphyseal region and the anterior region of
complain of pain. Large lesions can cause displacement of
the maxilla. The radiolucent areas can occur unilaterally,
teeth and a progressive malocclusion. Associated teeth are
bilaterally or in multiple sites. The cyst may extend inter-
vital.
dentally, between the roots of teeth giving rise to a scalloped
Occasionally, patients may complain of difficulty in
margin. Extensive lesions may cause expansion (usually
mouth opening when the cyst involves the capsule of the
buccal cortical plate is expanded) and erosion of the corti-
temporomandibular joint.
cal plates resulting in pathological fracture. Occasionally
the mandibular canal can be displaced.
Histopathologic features Aneurysmal bone cyst may mimic other lesions such as
The tissue specimen for histopathological examination can ameloblastoma, giant cell tumor, hyperparathyroidism,
be taken during the surgical curettage. However the amount myxoma, TBC and OKC. However, on aspiration, blood
of tissue obtained is generally insufficient for histopatho- may be expressed from a vascular lesion or aneurysmal
logical analysis. Epithelial lining is typically absent. Other bone cyst. Clinically aneurysmal bone cyst may be differen-
findings include presence of fibrous connective tissue and tiated from a vascular lesion based on the absence of bruits
normal bone. The lesion may exhibit areas of vascularity, or thrill and lack of pulse pressure.
fibrin and erythrocytes. Giant cells may be occasionally
seen adjacent to the bone surface. Radiographic features
Radiographically, aneurysmal bone cyst resembles other
Management cystic lesions of the jaw. It may appear as a well-defined
Literature review reveals that some cases of TBC undergo unilocular radiolucent lesion or sometimes exhibit internal
spontaneous resolution. However, the most preferred and septa within the radiolucent lesion giving rise to a multi-
widely recommended treatment modality is surgical explo- locular appearance (honeycomb pattern).
ration followed by meticulous curettage of the bony walls. Dabska and Buraczewski (1969) and Wilner (1982)
It is believed that curettage and surgical exploration will described four stages in the radiographic appearance of
induce bleeding. This induced bleeding will form a clot aneurysmal bone cysts:
which will ultimately be replaced by healthy bone. Recur- 1. Initial lytic phase: a well-defined area of bone resorp-
rences are rare after surgical treatment. tion with no distinctive features is observed
2. Phase of active development: there is the typical sub-
periosteal, ‘blow-out’ expansile appearance
Aneurysmal Bone Cyst
3. Stabilization phase: there is a distinct peripheral bony
Jaffe and Lichtenstein in 1942, discovered a distinct clinico- shell with internal septa and trabeculations, resulting
pathological entity and termed it aneurysmal bone cyst. in the so-called ‘soap bubble’ appearance
322
4. Healing phase: Progressive ossification of the cyst

Figure 30
resulting in a dense bony mass of irregular structure.
CT scan may reveal fluid-fluid levels within the radiolu-
cent lesion. Fluid-fluid levels are produced due to layering
of solid blood components within the cyst.
Management
Malghem et al (1989) reported three cases of spontaneous
healing of aneurysmal bone cysts in about 7–9 months
duration.
Aneurysmal bone cysts can be managed by surgical
curettage or by excision. Some authors have reportedly used
calcitonin injections with unpredictable results.
Szendröi et al (1992) recommended direct injection of
calcitonin into the cyst as a useful non-invasive method
for the management of hypovascular aneurysmal bone
cysts. Photograph of the mandible showing the lingual defect
Adamsbaum et al (2003) studied the affect of Ethibloc which forms the site for lodgement of the submandibular
(Ethnor Laboratories) injection, which is an alcoholic solu- salivary gland. Courtesy: Dr Ravikiran Ongole
tion of zein (a vegetable protein dissolved in alcohol). It is
a fibrogenic and thrombogenic agent, on 17 patients in
the age group of 2–18 years. After 5 years of follow-up,
TBC by location which lies superior to inferior alveolar
14 out of 17 patients demonstrated complete healing
canal. Its anterior variant is found between incisors and
manifested by increased cortical and septal thickening.
premolars. Rarely, complication in the form of develop-
Inflammatory reaction following the injection such as
ment of neoplasm like mucoepidermoid carcinoma from
pain and fever is seen. They concluded that the percutane-
salivary gland has been reported rarely.
ous direct Ethibloc injection is a safe, efficient and non-
invasive treatment for aneurysmal bone cyst.
Other modalities of treatment include introduction of
demineralized bone and autogenous bone marrow that CYSTS OF SOFT TISSUES OF MOUTH,
promotes self-healing of a primary aneurysmal bone cyst. FACE AND NECK
It is estimated that 10–44% of the cases exhibit recur-
rence. Recurrences have been reported due to difficult Anterior Median Lingual Cyst
access and incomplete removal of the cyst. It is advisable
Fink in 1963 described a patient with cystic swelling pres-
to review the patients on a regular basis to evaluate for
ent in the anterior two-thirds of the tongue on the dorsal
recurrence.
surface. He termed this as a retention cyst of the tongue or
glossocele. Some authors use the term ‘intralingual cyst’
Stafne’s Bone Cyst originating from the foregut synonymously with anterior
median lingual cyst. Large cysts can interfere with feeding
Stafne’s bone cyst is also known as static bone cyst or and swallowing. Occasionally the cysts can impede closure
defect of mandible, lingual mandibular bone cavity and of the mouth.
latent bone cyst. Histologically these cysts may be lined by parakera-
Stafne in 1942 recognized this entity. It is the develop- tinized stratified squamous epithelium. The cystic capsule
mental inclusion of submandibular glandular tissue within may exhibit chronic inflammatory cells.
or, more commonly, adjacent to the lingual surface of the The cysts can be surgically excised. However, recur-
body of the mandible or indentation on the mandible rences have been reported.
(Figure 30). Name of the cyst is derived from its radio-
graphic appearance. It is a congenital defect.
NASOPHARYNGEAL CYSTS
It appears as an ovoid radiolucency located between the Nasopharyngeal cysts are rare entities which may either be
inferior canal and the inferior border of the mandible in congenital or acquired in nature. Most of the cysts are
the region of 2nd or 3rd molars. It is differentiated from asymptomatic. However, larger cysts can cause nasal
323
obstruction, closed posterior rhinolalia (nasal quality of These cysts rarely originate from Rathke’s pouch. These
voice) and conductive hearing loss. cysts exhibit a median base which is attached to the nasopha-
Piero Nicolai et al (1989) modified the classification of ryngeal vault. Histological picture of these cysts are very clas-
nasopharyngeal cysts originally proposed by Singh and sical. These cysts are lined by stratified squamous epithelium
Pahor (1977). that is related to the ectodermal origin of Rathke’s pouch.
Classification
Lateral Cysts
1. Midline cysts
These are usually branchiogenic in origin. The base of the
a. Congenital cysts
cyst is located between the posterior pillar and pharyngeal
i. Cysts of bursa pharyngea embryonalis
opening of the Eustachian tube.
ii. Cysts of Rathke’s pouch
These cysts have more fluid content and lesser cellular
b. Acquired cysts
debris compared to the midline cysts. Unlike the midline
i. Retention cysts of the median recess
cysts, the germinal centers are rarely present in the lateral
ii. Retention cysts of seromucinous glands
cysts. The inner lining of the cyst is made up of cylindrical
2. Lateral cysts
and ciliated epithelium.
a. Congenital cysts
Clinically these soft mucosal masses can be identified
i. Branchiogenic cysts
by posterior rhinoscopy or by using a nasal fiberscope.
b. Acquired cysts
Choanal polyp, sphenoid sinus mucocele and juvenile angio-
i. Retention cysts of seromucinous glands
fibroma can mimic nasopharyngeal cysts.
Clinical features Radiographic investigations
Midline cysts are usually seen in the 4th decade of life, Radiographs frequently reveal a soft tissue mass with sharply
whereas lateral cysts are usually seen in the 6th decade of defined margins high on the posterior pharyngeal wall.
life. Men are more commonly affected than women (3:1). These cysts lack bony involvement.
CT scan (axial and coronal sections) with contrast agent
helps in assessing the nature of the mass, spatial relation-
Midline Cysts ship with adjacent structures and bone involvement.
MRI shows a characteristic high signal intensity on
In the midline cysts, the retention variety is more common
T2-weighted and intermediate to high signal intensity on
than the congenital cysts.
T1-weighted images. The variation in signal intensity
It is believed that the retention cysts occur due to
on T1-weighted images may be related to differences in
the fusion of the median recess of the pharyngeal tonsil.
protein content or hemorrhage in the cyst.
Hemorrhagic contents along with shed epithelial debris
may be evident in the cyst. The cyst wall may show lym- Management
phoid follicles with pronounced germinal centers. The inner
epithelial lining is composed of cylindrical ciliated cells. It Temporary relief of symptoms can be achieved by aspira-
is believed that the presence of inflammatory stimulus may tion of the cystic contents or injection of sclerosing agents
produce squamous metaplasia. into the cystic lumen such as ethanolamine oleate solution.
The congenital midline cysts may originate from the pha- Surgical excision of the cyst is the treatment of choice.
ryngeal bursa (Tornwaldt’s bursa) or from Rathke’s pouch.
The clinical and histological features of the cysts origi-
nating from the pharyngeal bursa are similar to those THYROGLOSSAL DUCT CYSTS
of the retention cysts. However, to differentiate these cysts
a simple thumb rule can used—cysts deep to the pharyngo- The thyroglossal duct cysts are said to arise from the rem-
basilar fascia arise from pharyngeal bursa whereas cysts nants of the thyroglossal duct that failed to regress. These
originating superficial to the pharyngobasilar fascia are cysts can be present anywhere along the course (from the
more likely to be retention cysts. base of the tongue to suprasternal region) of the remnant
The pharyngeal bursa is a tubuliform invagination lying of the thyroglossal duct.
at the junction of the nasopharyngeal vault with posterior Cysts located near the foramen caecum are lined by
pharyngeal wall and extending between the heads of the stratified squamous epithelium, whereas cysts located near
longissimus muscles of the head just above the uppermost the thyroid gland are lined by cells similar to thyroidal
fibers of the superior constrictor muscle of the pharynx. It is acinar epithelium. It has been reported that almost half of
estimated that the pharyngeal bursa persists in about 3% the thyroglossal duct cysts contain normal thyroid tissue
of the normal adults. in their walls.
324
Clinical features Howie and Proops (1982) defined branchial cysts (or
lymphoepithelial cysts) as ‘lesions found behind the angle
Thyroglossal cysts are usually seen in the midline. However,
of the mandible in the anterior triangle of the neck at the
it is estimated that about 38% of the cysts are seen slightly
junction of the upper third and lower two-thirds of the
off the midline (located adjacent to the outer surface of the
sternocleidomastoid muscle. The cysts have a lining of strat-
thyroid cartilage, deep to the strap muscles). Almost all
ified squamous epithelium resting on a complete or incom-
cysts are present in the vicinity of the hyoid bone.
plete band of lymphoid tissue with part of the cyst wall
Based on the anatomic location the thyroglossal cysts
resembling a lymph node’.
can be categorized as suprahyoid, at the level of hyoid and
Golledge and Ellis (1994) defined these cysts according
infrahyoid.
to two parameters: position and histology. With regard to
About 20–25% are suprahyoid, 15–50% occurring at
position, the lesion must lie outside the midline of the
the level of the hyoid bone, where they may be anterior or
neck or within any position in the lateral aspect of the neck.
posterior to the hyoid bone, and 25–65% occurring in the
With regard to histology, the cyst lining is squamous or
infrahyoid part of the neck.
columnar and is surrounded by lymphoid tissue.
The differential diagnosis of thyroglossal duct cysts
includes dermoid cyst, branchial cleft cyst, lymphadenop- Pathophysiology
athy, and a cystic nodule arising from the thyroid gland.
Literature review reveals many theories to explain the origin
Ultrasonographic features of these cysts such as branchial remnants, cervical sinus
remnants, thymopharyngeal duct remnants and squamous
The cyst typically appears anechoic, well-circumscribed with
epithelium inclusions in a cervical lymph node.
increased through-transmission. However, the cyst may also
However, most authors agree that these cysts are con-
present as homogeneous or heterogeneous complex hypo-
genital branchial remnants. These branchial remnants may
echoic lesion. Some cysts may present as truly anechoic,
clinically present as a cyst, sinus or fistula. If a portion of
some as predominantly anechoic but containing internal
a cleft or pouch fails to involute completely, the entrapped
debris and some have a complex heterogeneous echo pat-
remnant will form an epithelial lined cyst called a bran-
tern, and few others have a uniformly homogeneous pseu-
chial cyst.
dosolid appearance. The coarse echo pattern is due to the
proteinaceous content of the cyst secreted by the cyst lining. Clinical features
Occasionally, such a uniform echogenic appearance may
lead to a false assumption of a solid lesion. However, when It is estimated that almost 17% of the cervical masses in
gentle but uniform pressure is applied over the cyst with the pediatric population are branchial cysts. They are typically
transducer, the contents tend to shift which is suggestive of present as a mass at the anterior border of the sternocleido-
a cystic lesion. It is estimated that about 45% of the cysts mastoid muscle, at the junction of its upper and middle
have thick walls due to inflammation and cellular debris. third. In more general terms, the cyst can occur at any
level from the hyoid bone to suprasternal notch.
Management It is seen to affect males and females equally. However
Sistrunk procedure involving resection of the cyst and some authors report that the branchial cyst is slightly
tract, and excision of the middle third of the hyoid bone is more common in males.
the standard procedure employed in the management of The branchial cleft cyst is an asymptomatic slow-
the thyroglossal duct cyst. However, incomplete resection growing, fluctuant mass that is usually seen in the 2nd
will cause recurrence. and 3rd decades of life. It is seen in close approximation
However, when a malignancy is evident, a near total or to the external ear, angle of the mandible and upper lateral
total thyroidectomy along with the Sistrunk procedure is aspect of the neck.
recommended. The adjoining lymph nodes need to be eval- These cysts generally occur unilaterally. However, in
uated because of the likelihood of a intrathyroidal foci of approximately 2% of the patients the cyst may occur
cancer. bilaterally (bilateral presentation may indicate a familial
tendency). Large branchial cysts can cause difficulty in
swallowing, speech and breathing. Infected cysts can turn
LYMPHOEPITHELIAL CYSTS (Branchial into abscesses and form sinuses that open into the skin or
Cleft Cysts) pharynx.
Diagnosis
The word ‘branchial’ is derived from the Greek word bragchia,
meaning gills. The term ‘branchial cyst’ was first used by The diagnosis of branchial cleft cyst is made by a thorough
Ascherson in 1832. He believed that these cysts arose from history, clinical signs, fine needle aspiration, ultrasonography
impaired obliteration of branchial clefts or pouches. and CT.
325
Straw-colored fluid is aspirated from the cyst. Micro- 4. Stage IV: Bilateral suprahyoid
scopically the aspirant may contain cholesterol crystals, 5. Stage V: Bilateral infrahyoid and suprahyoid.
squamous cells, lymphocytes and polymorphonuclear cells.
Rarely secondary infection or internal bleeding can result
Ultrasonographic imaging will show a circumscribed
in a sudden increase in the size of the lesion. Extremely
mass of homogeneous low echogenicity. CT is a useful tool
large sized cystic hygromas can impinge on the airway.
to assess the topographical plane of the cyst and its proxim-
Cystic hygromas are relatively more frequently seen
ity to the surrounding vital structures.
in Turner syndrome, Down syndrome, Noonan syndrome
and Klinefelter syndrome.
Lipoma, cystic hygroma, thyroglossal duct cysts, lipomas, Differential diagnosis
lymphomas, and dermoid cysts should be considered in Branchial cleft cyst, thyroglossal duct cyst and branchial
the differential diagnosis of branchial cleft cysts. cleft cyst can be considered in the differential diagnosis of
cystic hygroma.
Management
Infected cyst should be managed with appropriate anti- Radiographic features
biotics. Surgical excision of the cyst is the treatment of Ultrasonography, MRI and CT can be used to image cystic
choice. hygroma. CT and MRI demonstrate ring-like margin
enhancement with sharp demarcation of cystic areas. The
cystic areas tend to appear circumscribed and discrete.
CYSTIC HYGROMA MRI may show hyperintense areas on T2-weighted
images and hypointense areas in T1-weighted images.
Cystic hygroma is a developmental entity characterized by
progressive dilatation of the lymphatic vessels. It was first Histologic features
described by Wernher in 1843. It is estimated that 75% of Most of the cystic hygromas exhibit a multicystic appear-
the cystic hygromas affects the head and neck region. ance. It is estimated that only 10% have a unilocular mor-
Cystic lymphangioma or macrocystic lymphatic mal- phology. The lesion may vary in size from a few millimeters
formation, first described by Redenbacker (1828), is a term to several centimeters. The lumen contains cystic fluid
used as a synonym for cystic hygroma. which may appear clear or straw colored. The eosinophilic
It is believed that sequestered lymphatic rests that retain cystic fluid contains high levels of protein. Cystic hygro-
their embryonic growth potential penetrate adjacent struc- mas are made up of large irregular sinuses with a single
tures or dissect along fascial planes and eventually become layer of flattened epithelial lining and fibrous adventitial
canalized. These spaces retain their secretions and develop coats.
cystic components because of the lack of a venous outflow
tract. Management
Asymptomatic cases of cystic hygroma are best left
Clinical features
untreated with regular follow-up to evaluate for infection
Approximately 60% of the cystic hygromas are present at or secondary pressure effects on surrounding structures.
birth. By the age of 2 years 90% are apparent. The poste- Symptomatic cases can be managed with intralesional
rior triangle of the left side of the neck is most frequently injections of sclerosing agents (OK-432 [an inactive strain
affected site. On clinical examination, these are large, soft of group A Streptococcus pyogenes], bleomycin, pure etha-
to doughy in consistency, painless swellings that can be nol, and doxycycline) and surgical resection of the lesion.
compressed. The skin overlying the swelling can occasion- It is believed that the inactive bacteria used as a scle-
ally show a bluish coloration. Transillumination is positive rosing agent incites an inflammatory response leading to
in cystic hygromas. fibrosis of the cystic hygroma.
Though many authors strictly oppose aspiration of lym-
Staging of cystic hygroma phatic formations as it may lead to infection and hemor-
rhage, Burezq et al (2006) in their article discussing their
de Serres et al (1995) proposed a staging system based on
30 years experience in managing cystic hygromas report
the anatomical location:
interesting results obtained with serial aspirations. In their
1. Stage I: Unilateral infrahyoid 5-year follow-up of 14 patients who were subjected to
2. Stage II: Unilateral suprahyoid aspiration, no failures were reported. It is estimated that
3. Stage III: Unilateral and both infrahyoid and 15% of the cases show recurrence if incompletely surgically
suprahyoid excised.
326
DERMOID, EPIDERMOID AND TERATOID basal cell carcinoma arising from the wall of an epidermoid
CYSTS cyst, Ikeda et al described a case where basal cell carcinoma
originated from an epidermoid cyst and Lopez-Rios et al
reported a case of squamous cell carcinoma arising in the
Dermoid Cysts
wall of an epidermoid cyst.
Dermoid cysts may be located anywhere on the body sur-
face, however these are characteristically located in the
region of fusion of embryonic processes. It is estimated PARASITIC CYSTS
that dermoid and epidermoid cysts of the oral cavity
account for about 0.01% of all oral cysts. Literature review
Though parasitic cysts in the head and neck region are
reveals that these cysts account for about 1.6 to 6% of all
rare, the common cysts that may be seen are cysticercosis,
cysts affecting the head and neck region.
hydatid cysts and trichinosis.
Dermoid cysts are categorized by a cystic cavity lined
by epithelium which contains all skin appendages like the
hair follicles, sebaceous glands and sweat glands. Cysticercosis
Subtypes of dermoid cysts Human cysticercosis is a parasitic infection caused by Taenia
solium. Other forms of Taenia include saginata and Asian
Epidermoid cysts The epidermoid cyst cavity is lined by Taenia. It is believed that the Asian Taenia is an intermediate
epithelium which is devoid of any skin appendages. form between solium and saginata as it is morphologically
Teratoid cysts These cysts are lined by an epithelium similar to T. saginata and uses pigs as the intermediate host
which contains skin appendages as well as certain compo- like T. solium.
nents of the mesoderm such as muscle and bone. These Ingestion of fecally contaminated food, water, fruit or
cysts may contain respiratory, gastrointestinal, and con- vegetables containing the ova of T. solium causes human
nective tissues. cysticercosis. Human cysticercosis is endemic in various
parts of the world including Mexico, Africa, South-East
Etiology Asia, Eastern Europe, Central and South America and India.
Poorly cooked meat (pork) is ingested by humans who
The exact etiology of dermoid cysts is still unknown. are the only definitive hosts. The undercooked meat includes
It is believed that when epithelial tissue is traumatically eggs or proglottids of the pork tapeworm. In the stomach
implanted in utero or when a piece of epidermis is trau- the larval form (Cysticercus cellulose) is released following
matically implanted in the lines of fusion of embryonic digestion of the outer protective layer of the proglottids.
elements, the epithelium undergoes sequestration. These cysticerci are then disseminated by the arterial blood
to various tissues, preferentially to the CNS (60%), eye
Clinical features (70%) and skeletal muscle (5%).
Dermoid cysts can occur anywhere in the body. However,
these are commonly seen at the sites of fusion of embryonic Clinical features
elements. These are commonly seen on the hands and feet. Clinically the condition may range from an asymptomatic
Intraorally, midline of the floor of the mouth (sublin- nodule to life-threatening symptoms. Individuals may com-
gual dermoid cysts) is most commonly involved. However, plain of nausea, vomiting and severe epigastric pain. CNS
other rare sites of involvement are the tongue, lips, uvula involvement may cause irritability, convulsions and loss
and buccal mucosa. of consciousness.
On clinical examination, calcified nodules may be pal-
Ultrasonographic picture pated anywhere in the body. In the head and neck region
Ultrasonography produces an echogenic picture in der- the common sites for presence of calcified cysticerci include
moid and epidermoid cysts due to the presence of cellular muscles of mastication, muscles of facial expression and
material, cholesterol crystals, and keratin within the cyst. cervical muscles. Some of these calcified masses may be as
large as 10 mm.
Management
Diagnosis
Epidermoid, dermoid and teratoid cysts are best managed
by surgical excision. There is no definitive evidence for The diagnosis of cysticercosis should be done following
malignant transformation of epidermoid or dermoid cysts. thorough evaluation of the patient’s lifestyle, personal
However, individual case reports of such malignant changes hygiene history and nature of food consumption along
have been reported in literature. Dini et al reported of with the characteristic neurological and gastrointestinal
327
symptoms, presence of palpable firm calcified masses in manifest gradually unless they cause pressure effects in the
the soft tissues and specific laboratory and radiographic brain and eyes. The cysts grow slowly, and a cyst is rarely
imaging modalities. diagnosed during childhood or adolescence unless the
Serological tests such as enzyme-linked immunosorbent brain or the eyes are involved. Relatively large cysts (more
assay (ELISA) and enzyme-linked immunoelectrotransfer than 5 cm in diameter) tend to produce pressure effects
blot have the highest sensitivity and specificity in detect- on the adjoining structures. Abdominal tenderness, tender
ing isolated muscular cysticercosis. hepatomegaly, fever and chills are common symptoms seen
Plain radiography, CT, MRI and ultrasonography can be in hydatid disease.
used. Radiographs show well-defined, homogeneously radio-
paque elongated or ovoid mass which may mimic sialolith. Diagnosis
However cysticercosis presents as multiple calcifications.
Imaging modalities like ultrasonography, CT and MRI help
Management in diagnosing these cysts. These imaging modalities help
in assessing the dimension of the cyst and its relationship
Individuals should be educated to avoid the use of poorly to surrounding vital structures.
cooked pork. Fruits and vegetables (especially coriander In their study of hydatid cysts, Zeyrek et al (2008) and
used for garnishing and raw leafy vegetables used in sal- Köktürk et al (1999) reiterated the fact that the ‘air bubble
ads) should be thoroughly washed before consumption. sign’ in CT image was pathognomonic for perforated pul-
Hands should be thoroughly washed prior to handling monary hydatid cyst.
food substances. Apart from imaging modalities, serological tests such as
No treatment may be required once the larvae are no hydatid immunoelectrophoresis, ELISA, latex agglutination
more viable and have formed calcifications. Occasionally, and indirect hemagglutination (IHA) test can be employed.
based on the location of these calcifications some patients Casoni intradermal test is also less frequently used. In
may present with seizures, cerebral edema and vasculitis. this test Casoni antigen (sterile hydatid fluid) is injected
In such patients, anticonvulsants and corticosteroids intracutaneously. The presence of immediate or delayed
should be administered. wheal-and-flare reaction is read as a positive result. How-
Viable larvae can be effectively managed with antipar- ever, as the test has low sensitivity and the potential for a
asitic drugs such as praziquantel and albendazole. severe local allergic reaction, it is not routinely used.
Hydatid Cyst Radiographic features
Hydatid disease is caused by tapeworm (Echinococcus) Plain radiographs of the involved site may be taken.
infestation. Three species of Echinococcus are of relevance However, the findings may not be diagnostic and occasion-
to medicine; the most common Echinococcus granulosus ally no findings are seen. Radiographically, well-defined
(causes cystic echinococcosis), Echinococcus multilocularis radiolucency surrounded by a slender rim of radiopacity
(causes alveolar echinococcosis) and the rarest Echinococcus (calcification) may be evident.
vogeli (causes). Ultrasound may show the presence of daughter cysts
Hydatid disease is endemic to the cattle and sheep rear- and hydatid sand (the scoleces, daughter cysts and calcar-
ing regions of the world such as the Middle East, India, eous corpuscles of Echinococcus tapeworms in the fluid
Africa, South America, New Zealand, Australia, Turkey and within a primary hydatid cyst). CT and MRI can assess the
southern Europe. Exposure to food and water contami- extent and location of the cyst.
nated by the feces of an infected definitive host or poor Saint Martin and Chiesa (1984) described the presence
hygiene in areas of infestation can lead to echinococcosis. of ‘falling snowflakes’, an ultrasound sign, as characteris-
tic of hydatid sand.
Clinical features Khanna et al (2007) reported the presence of the ‘water
lily sign’ in the contrast-enhanced computed tomography
Hydatid disease primarily affects the liver (55–70%) and (CECT) of the chest of a patient. They suggest that the
the lung (18–35%). However, the less common sites that water lily sign (presence of a thin walled cystic cavity con-
are affected are the brain (common in children), heart, taining a freely floating endocyst) is pathognomonic for
kidney, ureter, spleen, uterus, pancreas, diaphragm and Echinococcus.
muscles.
Emamy and Asadian (1976), Saxena et al (1983) and
Bickers (1970) reported cases of hydatid cyst affecting the
parotid gland. Hydatid cysts are best managed surgically. However, the
The hydatid cyst grows slowly. It is usually diagnosed use of albendazole (1 month prior to surgery) and 2 weeks
in the 3rd and 4th decades of life. Symptoms usually of praziquantel (2 weeks prior to surgery) will sterilize the
328
Generally, many months after the initial intestinal phase,

Figure 31
encystment and repair takes place. This phase of repair is
termed convalescent phase. Patients are usually cachexic
and extremely dehydrated in this phase.
Miloro and Kinney (1994) reported trichinosis affecting
the lateral pterygoid muscle that led to degenerative changes
in the TMJ following meniscectomies.
Lopez-Lozano et al (1988) reported bilateral facial
paralysis secondary to trichinosis. Bruce (1975) and Cheung
(1997) reported oral squamous cell carcinoma associated
with trichinosis. It is proposed that trichinosis may act as a
co-carcinogen along with other chemical carcinogens.
Diagnosis
Hydatid cysts. Courtesy: Dr S Suguna Hemachander,
Mamata Medical College, Khammam, Andhra Pradesh The diagnosis of trichinosis involves blood picture, poly-
merase chain reaction (PCR), imaging techniques and
muscle biopsy.
Eosinophilia and leukocytosis may be seen in 60% of
cyst, decrease the chance of anaphylaxis, decrease the the patients. The erythrocyte sedimentation rate is elevated.
tension in the cyst wall (thus reducing the risk of spillage ELISA is 100% sensitive on 50th day of infection. PCR can
during surgery) and decreases the rate of recurrence. be used for parasite isolation.
During the surgery 0.5% of silver nitrate or hypertonic CT and MRI with contrast can be used to assess involve-
saline solution can be injected into the cystic lumen which ment of the CNS. The typical finding is 3–8 mm ring-like
helps in killing the daughter cysts (Figure 31) which will lesions. However, muscle biopsy is still the gold standard
reduce the opportunity for spread following spillage of the for diagnosing this condition. Approximately 1 g of muscle
cystic contents. tissue is obtained from the deltoid region for histopatho-
logical studies.
Trichinosis
Trichinosis is caused by the ingestion of undercooked meat
containing larvae of the nematode Trichinella spiralis. Untreated patients may die of pneumonia, encephalitis, or
These larvae enter the lymphatics and blood circulatory cardiac failure in about 4–8 weeks.
system and migrate to well-vascularized striated skeletal Antipyretics and analgesics along with albendazole
muscle. The parasite has a predilection for the most meta- (400 mg/kg 3 times a day for 14 days) or thiabendazole
bolically active muscle groups such as the tongue, diaphragm, (25 mg/kg twice a day for 5–7 days) will prevent systemic
masseter, intercostal and pectoral muscles. The average dissemination.
incubation period is between 1 and 30 days. The larvae are
estimated to have a 5–10 year life span. Treatment of cysts of the jaws
Clinical features The surgical treatment of cystic lesions of the jaws were
essentially established by Partsch of Germany.
The disease generally affects adults with no sex predilec- The treatment of benign cysts of the jaws is surgical,
tion. The disease process progresses through various phases and there are two main operative procedures:
such as intestinal phase, parenteral phase and convalescent
phase. The intestinal phase of the disease presents with 1. Marsupialization of the cyst cavity
diarrhea, anorexia, weakness and abdominal cramps. Some 2. Enucleation of the cyst, followed by:
individuals may present with macular rashes. High degree a. Primary closure of the wound, or
fever (104⬚F) is another characteristic feature. The intestinal b. Packing of the cystic cavity until healing by sec-
phase lasts for about 1 week. ondary intention has occurred.
In the parenteral phase the larvae migrate from the intes-
tine into the circulation. This process may continue for many Marsupialization of the cyst cavity (Partsch I procedure)
weeks to a few months. Patients may complain of severe It consists of removing the overlying cyst epithelium and
myalgia. At this stage, skeletal muscles, CNS, cardiac and bone, thus essentially deroofing the cyst thereby providing
respiratory system are involved. a wide communication between the cyst cavity and the
329
oral cavity. The result is a merging of the epithelium of the initially, followed by enucleation at a later date when
cyst with that of the oral mucosa. the cystic cavity has shrunk adequately.
Modifications of marsupialization
Enucleation with primary closure (Partsch II procedure)
❍ A modification of marsupialization is antrocystectomy, In 1910, Partsch advocated the enucleation of a cyst cap-
which is undertaken for large cysts encroaching upon sule followed by primary closure, and hence it is known as
the maxillary antrum. A wide opening is made between the Partsch II procedure. In this method, the cyst capsule is
the cyst cavity and the maxillary sinus. After healing, the completely detached from the bone and shelled out. The
cyst epithelium merges with the antral epithelium just cyst cavity is then totally free of epithelium.
as it does with the oral epithelium in marsupialization. This surgical procedure leaves the opening to the cyst
❍ Waldron’s technique is a two-staged technique that com- covered by a mucoperiosteal flap and the space filled with
bines the two standard procedures of marsupialization blood clot which eventually organizes to form normal bone.
330
CHAPTER
Tumors of Orofacial Region

Srikant N, Nandita Shenoy,
Ravikiran Ongole, Mala Kamboj
13
➧ Benign Odontogenic Tumors Traumatic Neuroma
Ameloblastoma Rhabdomyoma
Squamous Odontogenic Tumor Leiomyoma
Clear-Cell Odontogenic Tumor Hemangiomas and Vascular Malformations
Calcifying Epithelial Odontogenic Tumor Hemangioma
Adenomatoid Odontogenic Tumor Vascular Malformations
Keratocystic Odontogenic Tumor Capillary Malformations
Odontome Arteriovenous Malformations
Ameloblastic Fibroma, Ameloblastic Fibrodentinoma, Lymphatic Malformations
and Ameloblastic Fibro-odontoma Fibroma
Benign Cementoblastoma Giant Cell Fibroma
Odontogenic Myxomas Fibromatoses
Odontogenic Fibroma Myofibroblastoma
Desmoplastic Fibroma ➧ Malignant Odontogenic Tumors
➧ Benign Non-odontogenic Tumors ➧ Odontogenic Carcinomas
Squamous Papilloma Malignant Ameloblastoma (Metastasizing)
Verruca Vulgaris (Oral Warts) Ameloblastic Carcinoma
Verrucous Hyperplasia Primary Intraosseous Squamous Cell
Condyloma Acuminatum Carcinoma
Focal Epithelial Hyperplasia/Heck’s Disease Solid PIOC
Keratoacanthoma/Self-healing Carcinoma Clear Cell Odontogenic Carcinoma
Oral Melanoacanthoma Malignant Epithelial Odontogenic
Acquired Melanocytic Nevus Ghost Cell Tumor
Ossifying Fibroma ➧ Odontogenic Sarcomas
Juvenile Ossifying Fibroma Ameloblastic Fibrosarcoma
Peripheral Ossifying Fibroma Odontogenic Carcinosarcoma
Osteoma
➧ Epithelial Malignant Tumors
Chondroma
Epidermoid Carcinoma
Benign Chondroblastoma (Codman’s Tumor)
Verrucous Carcinoma
Benign Osteoblastoma
Basal Cell Carcinoma
Giant Cell Granuloma
Malignant Melanoma
Peripheral Giant Cell Granuloma
➧ Connective Tissue Malignant Tumors
Pyogenic Granuloma
Fibrous Tissue Origin
Fibrous Hyperplasia: Denture-related
Fibrosarcoma
Congenital Epulis of Newborn
Malignant Fibrous Histiocytoma
Lipoma
Synovial Sarcoma
Neurogenic Tumors
Cartilage Tissue Origin
Schwannoma (Neurilemmoma)
Chondrosarcoma
Neurofibroma
331
Adipose Tissue Origin Muscle Origin

Liposarcoma Leiomyosarcoma
Bone Tissue Origin Rhabdomyosarcoma
Osteosarcoma Nerve Origin
Ewing’s Sarcoma Neurofibrosarcoma
Vascular Origin ➧ Lymphoid Origin and Hematological
Kaposi’s Sarcoma Malignancies
Angiosarcoma
ODONTOGENIC TUMORS Theories of Tumor Development

These tumors have been postulated to arise from enamel
Odontogenic tumors are lesions of great interest and organ, remnants, epithelium of odontogenic cysts and oral
importance to oral physicians, pathologists and maxillofa- mucosal buddings.
cial surgeons alike, who for several decades have studied
and catalogued these lesions and developed modalities for
adequate treatment. Etiology
It is estimated that approximately 9% of tumors in the Various etiological and predisposing factors have been
oral cavity are odontogenic in nature, out of which 94.8% propounded for the development of tumors such as infec-
are benign and 5.2% are malignant, 58.5% of benign tion, irritation, trauma, dietary deficiency, viruses, hor-
odontogenic tumors are ameloblastoma. monal changes and genetic factors.
The age predilection for benign odontogenic tumors is
around 28 years and for the malignant odontogenic tumors
Classification
is more than 40 years. Most of the tumors have mandibular
predilection with the ratio of 3.2:1, with the maximum in With the first edition of the WHO classification on tumors,
case of ameloblastoma showing a rate of 12.8:1. about 30 years ago, the terminology and the diagnostic
There are different ways of defining the content of the framework became available, and this modern and logi-
term ‘tumor’ in its broadest sense and not restricted to cally constructed classification greatly intensified research
lesions that are definitively neoplastic. Benign tumor is into the subject, and markedly stimulated the urge to publish
defined as a growth made up of normal cells that have no new findings. In 2000, the International Agency for
signs of cancer. Research on Cancer (IARC) in Lyon, France started a new
These tumors have some characteristic properties such as book series, WHO Classification of Tumors. The new WHO
new uncoordinated growth that spreads by direct extension Blue Books encompass both histopathological and genetic
unlimited growth potential, do not metastasize, are encap- criteria for tumor classification (Table 1). Clinically it also
sulated and resemble tissue of origin histologically. can be classified based on site, age and gender predilec-
tion as given in Tables 2–4.
The common clinical features of benign tumors include:
slow growing and well-circumscribed swelling, bony expan-
BENIGN ODONTOGENIC TUMORS sion, displacement and abnormal mobility of teeth and
absence of lymphadenopathy.
These are defined as lesions derived from epithelial or
mesenchymal elements or both that are part of tooth form-
Diagnosis of benign tumors
ing apparatus. These comprise a group of lesions which
have in common the fact that they arise from the odonto- ❍ Complete history: Pain, loose teeth, occlusion, swell-
genic tissue. These develop from the epithelial part of the ings, dysesthesia, delayed tooth eruption
tooth germ, the ectomesenchymal part, or from both. Their ❍ Thorough physical examination: Inspection, palpa-
behavior varies from frankly neoplastic, including meta- tion, percussion, auscultation
static potential, to non-neoplastic hamartomatous. The ❍ Plain radiographs: Panaromic radiographs and dental
pathogenesis of these tumors are not clear but can be radiographs at contrasting angles
attributed to the following etiologies. ❍ Advanced imaging: CT for larger, aggressive lesions
332
Chapter 13 – Tumors of Orofacial Region
Table 1 Classification of odontogenic tumors Table 2 Tumors based on the age of predilection
1. Tumors of odontogenic epithelium without odontogenic 0–25 years More than 40 years
ectomesenchyme Adenomatoid odontogenic tumor Calcifying epithelial odontogenic
a. Ameloblastoma Ameloblastic fibroma tumor
b. Calcifying epithelial odontogenic tumor Ameloblastic fibro-odontoma Ameloblastoma
c. Squamous odontogenic tumor Odontoma Squamous odontogenic tumor
d. Clear cell odontogenic tumor Peripheral ossifying fibroma Odontogenic myxoma
2. Tumors of odontogenic epithelium with odontogenic Benign cementoblastoma Calcifying odontogenic fibroma
ectomesenchyme with or without dental hard tissue formation Ameloblastic odontoma
a. Ameloblastic fibroma
b. Ameloblastic fibro-odontoma
c. Ameloblastic fibro-dentinoma
d. Odontoameloblastoma
Table 3 Tumors based on the gender of predilection
e. Adenomatoid odontogenic tumor
f. Complex and compound odontoma Male Female
3. Tumors of odontogenic ectomesenchyme with or without included
Ameloblastoma Adenomatoid odontogenic
odontogenic epithelium
Ameloblastic fibroma tumor
a. Odontogenic fibroma
Ameloblastic fibro-odontoma Squamous odontogenic
b. Odontogenic myxoma
Ameloblastic odontoma tumor
c. Benign cementoblastoma
Calcifying epithelial odontogenic tumor Odontogenic myxoma
4. Advanced imaging Odontoma Calcifying odontogenic
CT for larger, aggressive lesions, ultrasonography for vascular lesions Benign cementoblastoma fibroma
5. Obtain tissue for histopathological evaluation

a. Fine-needle aspiration—to rule out vascular lesions and
inflammatory conditions
b. Excisional biopsy—small tumors and unilocular tumors Table 4 Tumors based on the site of predilection
c. Incisional biopsy—larger lesions prior to definitive therapy
Maxilla Mandible
Adenomatoid odontogenic tumor Ameloblastoma
❍ Obtain tissue Calcifying epithelial odontogenic Ameloblastic odontoma
– Fine-needle aspiration—rule out vascular lesions, tumor Odontoma
Ameloblastic fibro-odontoma Benign cementoblastoma
inflammatory conditions
Squamous odontogenic tumor Odontogenic myxoma
– Excisional biopsy—smaller cysts, unilocular tumors
Calcifying odontogenic fibroma
– Incisional biopsy—larger lesions prior to definitive
therapy.
Clinical features
Ameloblastoma
Ameloblastomas may be present over a wide age range but
Churchill in 1934, defined ameloblastoma as unicentric, are usually diagnosed in the 4th and 5th decades of life,
non-functional, intermittent growing anatomically benign although they can occur in children or the elderly. About
but clinically persistent. These are benign, locally aggres- 80% of tumors occur in the mandible, of which some 70%
sive polymorphic neoplasms that consist of proliferating arise in the molar region and ascending ramus, 20% in the
odontogenic epithelium. premolar region, and 10% in the incisor region. In the max-
illa, most of these also occur in the molar region but about
Classification 15% involves the antrum.
The tumor is slow growing and in the early stages may
Leon Barnes has classified ameloblastoma into four types on
be asymptomatic and discovered as an incidental finding.
the basis of behavioral pattern, anatomical location, radio-
As the tumor enlarges the patient may become aware of a
graphic appearances and histologic features as follows:
gradually increasing facial deformity and expansion of the
1. Unicystic jaw bone (Figure 1A, B). The enlargement is usually bony
2. Multicystic hard, non-tender, and ovoid or fusiform in outline but in
3. Desmoplastic advanced cases, egg-shell crackling may be elicited due to
4. Peripheral. thinning of the overlying bone. However, perforation of
333
bone and extension of the tumor into soft tissues are late MRI findings
features. In the maxilla, even large tumors may produce
Characteristic findings are: multilocularity, mixed solid and
little expansion as the lesion can extend into the sinus and
cystic components, irregularly thickened walls, papillary pro-
beyond. Teeth in the area of the tumor may become loos-
jections, and marked enhancement of the walls and septa.
ened, but pain is seldom a feature.
Radiographically, the ameloblastoma appears most com-
Histopathology
monly as a multiloculated radiolucency (Figure 2A, B). Roots
of teeth involved by the tumor show varying degrees of There are six histologic subtypes: follicular, plexiform,
resorption. As the tumor enlarges it may become associated acanthomatous, granular cells, basal cell and desmoplas-
with an unerupted tooth, particularly an impacted third tic. These can be found combined or isolated and not
molar, and the appearances may mimic those of a dentig- related to prognosis of the tumor. The two main patterns
erous cyst. Less frequently, ameloblastomas present as a are anastomosing epithelial strands and fields or discrete
single unilocular radiolucency indistinguishable from an epithelial islands. The former pattern is called the plexi-
odontogenic cyst. form (Figure 3) type, the others the follicular (Figure 4A).
Figure 1
A B
(A) Ameloblastoma involving mandible. (B) Intraoral view of ameloblastoma involving mandible.
Figure 2
A B
(A) Radiographic features of ameloblastoma (honeycomb appearance). (B) Ameloblastoma (soap bubble appearance).
334
Both may occur within the same lesion. The follicles con- central area is occupied by stellate reticulum-like cells.
sist of a central mass of loosely connected, angular cells Microcyst formation is commonly seen.
resembling the stellate reticulum of the normal enamel When extensive squamous metaplasia, often associated
organ, surrounded by a layer of cuboidal or columnar cells with keratin formation, occurs in the central portion of a
resembling ameloblasts. The nuclei of the latter are situ- follicular ameloblastoma, the term ‘acanthomaotus amelo-
ated away from the basal ends of the cells, and this is blastoma’ is used (Figure 4B).
described as reversed polarity. The follicles are separated
by varying amounts of fibrous connective tissue stroma. A Differential diagnosis
variety of changes can occur within the stellate area of the
Differential diagnosis of ameloblastoma are given in
follicles and these include cystic breakdown, squamous
Box 1.
metaplasia, and granular cell change. The tumor epithe-
lium in the plexiform type is arranged as a tangled net-
work of anastomosing strands and irregular masses, each Treatment
of which shows the same cell layers as for the follicular The therapeutic approach to the ameloblastoma is still a
pattern. Thus, each strand or mass is bounded by colum- controversy. There are problems to determine incidence,
nar or cuboidal cells resembling ameloblasts, while the management or recurrence rate. Not every ameloblastoma
has the same destructive potential or recurrence tendency.
For unilocular cystic lesions in young patients, curettage
Figure 3
or enucleation is effective treatment. Solid lesions show
high recurrence rates (50–90%) necessitating tumor exci-
sion or partial resection of the jaw bone. There seems
to be more consensus in the management of ameloblasto-
Ameloblast-like
mas in children, due to the psychological impact of an
columnar cells
aggressive resection, and its relationship with growth and
Stellate function.
reticulum-like Key points on ameloblastoma are given in Box 2.
cells
Interconnecting
strands
Box 1 Differential diagnosis of ameloblastoma
Dentigerous cyst
Keratocystic odontogenic tumor
Histopathological picture showing plexiform ameloblastoma.
Odontogenic myxoma
Courtesy: Department of Oral Pathology and Microbiology,
MCODS, Mangalore Lateral periodontal cyst
Figure 4
A B
Ameloblastic
follicles Ameloblastic
Cystic follicles
degeneration Squamous
Stellate metaplasia
reticulum-
like cells
Ameloblast- Stellate
like cells reticulum-
showing like cells
palisading
(A) Histopathological picture showing follicular ameloblastoma. (B) Acanthomatous ameloblastoma.

Courtesy: Department of Oral Pathology and Microbiology, MCODS, Mangalore
335
Box 2 Key points related to ameloblastoma Histopathology
1. A benign but locally invasive neoplasm

Based on the character and extent of tumor cell prolifera-
tion within the cyst wall, several histologic subtypes of
2. Molar region/ascending ramus of the mandible is the most
unicystic ameloblastoma are recognized, which include
common site
those of simple cystic nature, intraluminal proliferative
3. Typically multilocular soap bubble/honeycomb appearance on
nodules and infiltrative tumor islands in the cyst walls.
radiograph but may be unilocular
The lesion presents as a cyst lined by ameloblastomatous
4. Follicular and plexiform types are the two main histological patterns epithelium comprising a basal layer of columnar cells with
polarization of their nuclei away from the basal lamina,
covered by a loose, vacuolated layer of stellate epithelial
Literature review cells. The diagnosis is made based on histopathologic
Ameloblastoma is a tumor with a complex biological char- examination.
acter and presents a high recurrence rate even in patients
treated with radical therapy. It has been reported that the Treatment
aggressiveness of ameloblastoma may be related to the
This variant is believed to be less aggressive, tends to affect
histological subtype, in fact.
patients at a younger age and its response to enucleation
Reichart et al in a review of the literature, found recur-
or curettage is more favorable than the classic solid or
rence rates for plexiform, follicular, acanthomatous and
multicystic ameloblastomas.
unicystic ameloblastoma respectively of 16.7%, 29.5%,
4.5% and 13.7%. Proliferating cell nuclear antigen (PCNA)
is a cell cycle related antigen and has been used for the
Peripheral (Extraosseous) Ameloblastoma
evaluation of the proliferation ability of this tumor.
Ameloblastomas occasionally present in the gingival or
Determinants of biological behavior alveolar soft tissues without involving bone. Such lesions
may arise from the basal cell layer of the oral epithelium
Location Seventy-five percent in the mandible, but worse
or from extraosseous rests of the dental lamina. These are
prognosis in the maxilla due to spongier bone that facili-
much less invasive than intraosseous tumors and less dras-
tates dissemination of the tumor. There is an absence of the
tic surgery is required for their treatment. Histologically,
‘contention effect’ that provides the mandibular cortical
these may resemble intraosseous types or consist mainly
bone.
of basaloid cells.
Arquitectonic pattern This pattern of the lesion does have
a prognostic validity. Unicystic has a better prognosis than
multicystic or solid. Odontoameloblastoma (OA)
This is an extremely rare mixed odontogenic tumor
Unicystic Ameloblastoma appearing within the maxillary bone, with both epithelial
and mesenchymal components. It is also known as amelo-
Unicystic ameloblastoma, a variant of ameloblastoma first blastic odontoma, although the term ‘odontoameloblastoma’
described by Robinson and Martinez in 1977, refers to (OA) was included in the 1971 WHO classification and
those cystic lesions that show clinical and radiologic char- seems to be more appropriate due to the behavior of the
acteristics of an odontogenic cyst but on histologic exam- tumor like an ameloblastoma rather than as an odontoma.
ination show a typical ameloblastomatous epithelium lining Thoma et al described in 1944 the first case, ‘it is an ame-
part of the cyst cavity, with or without luminal and/or mural loblastoma in which focal differentiation into an odon-
tumor proliferation. This type of ameloblastoma typically toma appears to have taken place’.
presents in a younger age group than other variants of
ameloblastoma (2nd to 3rd decade) and occurs predomi-
Histopathology
nantly in the mandibular third molar region.
Radiographically, it appears as a well-defined unilocular The marked histological polymorphism of odontogenic
radiolucency, usually associated with an unerupted tooth, tumors make the final diagnosis difficult and in some
and is indistinguishable from a dentigerous cyst. The patho- cases it must be made based on clinical, radiologic and
genesis of the lesion is unknown. Although it may represent histopathologic features. Moreover, Wachter et al could not
ameloblastomatous change in a dentigerous or other type histologically differentiate between OA and ameloblastic
of odontogenic cyst, the possibility that it was a grossly fibro-odontoma, and therefore suggest that they are the
cystic ameloblastoma from the outset cannot be excluded. same entity.
336
Treatment Radiographic features

Commonly, its clinical presentation mimics an odontoma, These tumors show an irregular radiolucent area which
and therefore the definite diagnosis is based on the may or may not be clearly demarcated from the surround-
histologic analysis following a simple extirpation and ing normal bone. The radiolucency contains varying
curettage. amounts of radiopaque bodies due to calcification within the
tumor. Radiographic features which have been considered
characteristic of CEOT, coronal clustering and ‘driven snow’
Squamous Odontogenic Tumor patterns, are seen in only a small percentage of cases.
The squamous odontogenic tumor is rare. Radiographically,
it usually presents as a well-circumscribed radiolucency Histopathology
with a sclerotic border associated with the roots of teeth. The calcifying epithelial odontogenic tumor consists of
Histologically, the tumor consists of irregularly shaped sheets of polygonal cells with ample eosinophilic cytoplasm,
islands of well-differentiated squamous epithelium in a distinct cell borders, and very conspicuous intercellular
stroma of mature fibrous tissue. It is thought to be derived bridges. Nuclei are pleomorphic with prominent nucleoli;
from the rests of Malassez. cells with giant nuclei and multiple nuclei are also present.
The epithelial tumor islands as well as the surrounding
stroma frequently contain concentrically lamellated calci-
Clear-Cell Odontogenic Tumor (Carcinoma)
fications. Stromal deposits of bone and cementum may
This is a rare jaw tumor that some consider as a carcinoma occur (Figure 5).
because of reported metastases. The histogenesis is
unknown, although it is believed to be derived from odon- Treatment
togenic epithelium because of its primary occurrence in
the jaws. Clear-cell odontogenic tumor (carcinoma) has The treatment for CEOT has ranged from simple enucle-
been described mostly in women above the age of 60 years. ation or curettage to radical and extensive resection such
It may cause some pain. as hemimandibulectomy or hemimaxillectomy. The choice
Radiographically, the lesion is lucent and either uni- should be individualized for each lesion because the radio-
locular or multilocular. This rare lesion has an aggressive logical and histological features may differ from one
biologic behavior. Metastases to lung and regional lymph lesion to another.
nodes have been reported.
Microscopically, nests and cords of clear cells are seen. Prognosis
Some peripheral palisading may be present.
Differential diagnosis would include clear-cell variant The prognosis of the CEOT is good with infrequent recur-
of calcifying epithelial odontogenic tumor, central muco- rence. Malignant behavior is extremely rare. Although it has
epidermoid carcinoma, metastatic renal cell carcinoma,
and poorly fixed ameloblastoma.
Figure 5
Calcifying Epithelial Odontogenic Tumor

The calcifying epithelial odontogenic tumor (CEOT) is a Polyherdal
rare, benign epithelial neoplasm, also called ‘Pindborg epithelial cells
tumor’. It is an aggressive tumor of epithelial derivation
usually associated with an impacted tooth in the mandible
body/ramus. Calcifications
Clinical features
It occurs over a wide age range and is about twice as com-
mon in the mandible as in the maxilla. The chief sign is
cortical expansion and pain is not normally a complaint.
Most of the tumors arise in the molar or premolar area and
Histopathological picture showing calcifying epithelial
about half are associated with the crown of an unerupted
odontogenic tumor. Courtesy: Department of Oral Pathology
tooth. Although most tumors arise within bone, extraosse-
and Microbiology, MCODS, Mangalore
ous lesions have been reported.
337
not been established in the literature, 5 years should be the

Figure 6
absolute minimum follow-up necessary to assess the heal-
ing for this type of odontogenic tumor. A
Adenomatoid Odontogenic Tumor

History
Adenomatoid odontogenic tumor (AOT) is a relatively
uncommon distinct odontogenic neoplasm that was first
described by Steensland in 1905. However, a variety of
terms have been used to describe this tumor. Unal et al
produced a list containing all nomenclatures for AOT
reported in the literatures. Various names like adenoame-
loblastoma, ameloblastic adenomatoid tumor, adamanti-
noma, epithelioma adamantinum or teratomatous
odontoma have been used before to define the lesion cur-
rently called AOT. B
Definition
Philipsen and Birn (1971), defined it as an odontogenic
epithelial tumor with an inductive effect on the odontogenic
mesenchyme. The nature of the lesion is uncertain and it
may be hamartomatous rather than truly neoplastic.
(A) Intraoral photograph showing obliteration of the labial

Classification vestibule and the expansion of the labial and palatal
Based on the location it can be classified as: cortical palate on the left side of the anterior maxilla.
(B) Case of adenomatoid odontogenic tumor (AOT).
❍ Follicular Courtesy: Department of Oral Medicine and Radiology,
❍ Extra follicular MCODS, Mangalore
❍ Peripheral.
Clinical features
Histopathology
The adenomatoid odontogenic tumor usually presents
during the 2nd and 3rd decades of life. The majority of Lesion is well encapsulated and may be solid or partly
tumors arise in the anterior part of the maxilla (Figure 6A), cystic; in some cases the tumor is almost entirely cystic. It
especially in the canine areas, and there are usually few consists of sheets, strands, and whorled masses of epithe-
symptoms apart from a slowly enlarging swelling. Clinical lium which in places differentiate into columnar, amelo-
features generally focus on complaints regarding a missing blast-like cells. The columnar cells form duct or tubule-like
tooth. The lesion usually presents as asymptomatic swell- structures (hence adenomatoid) with the central spaces
ing which is slowly growing and often associated with an containing homogeneous eosinophilic material. These are
unerupted tooth. However, the rare peripheral variant occurs thought to represent abortive attempts at enamel organ
primarily in the gingival tissue of tooth-bearing areas. formation. There is very little supporting stroma. Small
Unerupted permanent canines are the teeth most often foci of calcification are scattered throughout the tumor
involved in AOT. and occasionally tubular dentin and enamel matrix may
be seen (Figure 7).
On radiographs it usually appears as a well-defined radio-
lucency but in some cases calcification within the tumor Radiologically, it should be differentiated from dentiger-
may produce faint radiopacities (Figure 6B). The lesion is ous cyst, which most frequently occurs as a pericoronal
often associated with an unerupted tooth and may simulate radiolucency in the jaws. Dentigerous cyst encloses only
a dentigerous cyst (simulating a ‘target’ like appearance). the coronal portion of the impacted tooth, whereas AOT
338
Figure 7 Figure 8
Duct-like space
Calcifications
Epithelial cells
(polyherdal to
spindle-shaped)
Dentinoid
Connective tissue
Histopathological picture of adenomatoid odontogenic tumor.

MCODS, Mangalore
Radiograph of calcifying odontogenic cyst in the anterior
maxilla. Courtesy: Department of Oral Medicine and
shows radiolucency usually surrounding both the coronal
and radicular aspects of the involved tooth.
Common neoplastic causes, such as ameloblastoma, permanent molar tooth. The lesion usually presents as a
CEOT, ameloblastic fibroma and ameloblastic fibro-odontoma slowly enlarging but otherwise symptomless swelling. The
are easily differentiated on histology. COC normally appears as a painless, slow-growing tumor,
affecting equally the maxilla and mandible, with predilec-
Treatment tion to the anterior segment (incisor–canine area). It gen-
All variants of AOT are well-encapsulated and show an erally affects young adults in the 3rd to 4th decade,
identical benign behavior. Conservative surgical enucleation without gender predilection.
or curettage is the treatment of choice with only rare Radiographically, the lesion appears as a well-defined
recurrence. unilocular or multilocular radiolucent area containing vary-
ing amounts of radiopaque, calcified material. It may be
associated with the crown of an unerupted tooth (Figure 8).
Literature review Histologically, the cyst is lined by epithelium which shows
The histogenesis of AOT is still uncertain, although recent a well-defined basal layer of columnar, ameloblast-like cells
findings strongly indicate that AOT is derived from a com- and overlying layers of more loosely arranged cells that
plex system of dental laminae or its remnants. It is often may resemble stellate reticulum. A characteristic feature is
considered as a hamartomatous lesion rather than a true the presence within the lining of masses of swollen and
neoplasm. keratinized epithelial cells which are usually referred to as
‘ghost’ cells since the original cell outlines can still be dis-
cerned. The ‘ghost’ epithelial cells may calcify. Breakdown
Calcifying Odontogenic Cyst of the epithelium may release keratinous debris into the
supporting connective tissue resulting in a prominent
The calcifying odontogenic cyst (COC) was first described foreign body, giant cell reaction.
as a distinct entity by Gorlin et al in 1962. This lesion is
uncommon and represents about 0.03% of the biopsy lesions
and less than 2% of all odontogenic cysts and tumors. Keratocystic Odontogenic Tumor
Keratocystic odontogenic tumor is more universally known
Clinical features
as odontogenic keratocyst, but has been renamed as there
The calcifying cystic odontogenic tumor occurs over is sufficient evidence that this lesion actually represents
a wide age range but is usually seen below 40 years of a cystic neoplasm. First described by Philipsen in 1956,
age. About 75% are intraosseous and either jaw may be the odontogenic keratocyst (OKC) was later designated by
involved. The majority, including those located in the gin- the WHO as a keratocystic odontogenic tumor (KCOT) and
gival or alveolar soft tissues, arise anteriorly to the first is defined as ‘a benign uni- or multicystic, intraosseous
339
tumor of odontogenic origin, with a characteristic lining ❍ Histopathology: Studies showed the basal layer of the
of parakeratinized stratified squamous epithelium and KCOT budding into connective tissue.
potential for aggressive, infiltrative behavior’. WHO rec-
ommends the term ‘keratocystic odontogenic tumor’ as it Histopathology
better reflects its neoplastic nature.
Histologically, these cysts are formed with a stratified
squamous epithelium that produces orthokeratin (10%),
Clinical features parakeratin (83%), or both types of keratin (7%). The epithe-
KCOTs comprise approximately 11% of all cysts of the lial lining appears corrugated when viewed under a micro-
jaws. These occur most commonly in the mandible, espe- scope. A well-polarized hyperchromatic basal layer is
cially in the posterior body and ramus regions (Figure 8). observed, and the cells remain basaloid almost to the sur-
They almost always occur within bone, although a small face. No rete ridges are present; therefore, the epithelium
number of cases of peripheral KCOT have been reported. often sloughs from the connective tissue. The epithelium is
Patients may present with swelling, pain and discharge or thin, and mitotic activity is frequent; therefore, OKCs grow
may be asymptomatic. Distinctive clinical features include a in a neoplastic fashion and not in response to internal pres-
potential for local destruction and a tendency for multiplic- sure. The lumen frequently is filled with a foul-smelling
ity, especially when the lesion is associated with nevoid cheese-like material that is not pus but rather collected
basal cell carcinoma syndrome (NBCCS) or Gorlin–Goltz degenerating keratin (Figure 9A–C).
syndrome. In the recent years, published reports have
influenced WHO to reclassify the lesion as a tumor. Radiographic features
Several factors form the basis of this decision.
Radiographically, KCOT presents predominantly as a uni-
❍ Behavior: As described earlier, the KCOT is locally locular radiolucency with well-developed sclerotic borders.
destructive and highly recurrent. These may also present as a multilocular radiolucency or
Figure 9
A Cystic space B
Stratified
squamous
parakera-
tinized
epithelium
Palisaded
basal cells
Corrugated
parakeratin
layer
Connective
tissue
C D
(A) Histopathological features of keratocystic odontogenic tumor. (B) and (C) Gross specimens of the resected tumor, showing
removal of the cystic lining and the resultant hollowing in the bone. Courtesy: Department of Oral Pathology and Microbiology,
MCODS, Mangalore. (D) Orthopantomograph showing well-defined scalloped radiolucent area extending across the midline.
340
unilocular radiolucency at varying ratio. The multilocular incisors and canines, followed by the antero- and postero-
appearance can be more of a unilocular with scalloped inferior regions.
borders lacking true compartment formation. Odontogenic Complex odontomas are more often found in the area
keratocysts of the maxilla are smaller in size compared to of the second and third lower molars. An increased preva-
the mandible. When they are large, they tend to expand lence of these tumors is observed in children and adoles-
bone. No difference in site distribution is seen between cents, with few differences in relation to patient sex. These
unilocular and multilocular cysts. These lesions can also lesions are normally diagnosed by routine radiological
present as a small and oval radiolucency between teeth studies in the 2nd and 3rd decades of life. As regards
simulating a lateral periodontal cyst. They can also appear their pathogenesis, odontomas have been associated with
as a radiolucency simulating a residual apical periodontal antecedents of trauma during primary dentition, as well as
cyst (Figure 9D). with inflammatory and infectious processes, hereditary
anomalies (Gardner’s syndrome, Hermann’s syndrome),
❍ On CT: Increased attenuation areas in cystic cavity. odontoblastic hyperactivity, or alterations of the genetic
❍ On MRI: Heterogeneous intermediate signal on T1 and components responsible for controlling dental development.
high signal on T2 weighted images.
Treatment
Odontomas manifest as a dense radiopaque lesion sur-
A review of the literature suggests that recurrence rate is rounded by a thin radiotransparent halo. Three develop-
relatively low with aggressive treatment, whereas more con- mental stages can be identified, based on the radiological
servative methods tend to result in more recurrences. features and degree of calcification of the lesion at the
time of diagnosis:
Recurrence
1. First stage is characterized by radiotransparency due
KCOTs have a high recurrence rate, reportedly between
to the absence of dental tissue calcification.
25% and 60% and when associated with NBCCS, the recur-
2. Second or intermediate stage presents partial cal-
rence rate is about 82%.
cification.
3. Third or classically radiopaque stage exhibits predom-
Odontome inant tissue calcification with a surrounding radio-
transparent halo.
History
Compound odontomas show an irregular radiopaque image
Paul Broca (1867), defined it as a tumor formed by the
with variations in contour and size, composed of multiple
overgrowth of transitory/complex dental tissues.
radiopacities corresponding to the so-called denticles. In the
1946 WHO classification complex type of lesion radiopacity is not specific; rather, a
1. Germinated composite odontome disorganized, irregular single or multiple mass is identified
2. Compound composite odontome (Figure 10).
3. Complex composite odontome
4. Dilated odontome Histology
5. Cystic odontome
Complex odontomas consist of a usually well-delineated
In 1950 Gorlin eliminated the term ‘composite odontome’. mass of dental hard tissues in a haphazard arrangement.
Recent WHO classification The bulk of the lesion consists of dentin recognizable by
1. Complex odontome: Malformation in which all the the presence of tubuli. Enamel plays a minor role, usually
dental tissues are represented, individual tissues being confined to small rims in cavities in the dentin mass. The
mainly well formed but occurring in a more or less stroma consists of mature fibrous connective tissue. Some-
disorderly pattern. times, odontomas may contain areas identical to the calci-
2. Compound odontome: Malformation in which all the fying odontogenic cyst, including ghost cells consisting of
dental tissues are represented, in a more orderly pattern; tiny teeth that may vary in number from only a few to
consists of tooth-like structures called the tooth-lets. numerous. These teeth do not resemble normal teeth, but
are usually cone shaped.
Clinical features
Prognosis
Odontomas are the most common maxillary tumors, and
according to different sources in the literature account Odontomas are benign tumors that sometimes produce
for 22–67% of all odontogenic maxillary neoplasms. As to no symptoms and constitute casual findings of routine
their locations, most are found in the areas of the upper radiological studies. However, they usually tend to cause
341
Figure 10
Orthopantomograph showing odontoma. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
signs and/or symptoms such as delayed eruption. If no strata in the palms of the hands and soles of the feet, is the
signs or symptoms appear then the lesions go undetected. only pathognomonic data which has been described in the
A very infrequent situation in relation to odontomas can literature regarding this condition.
be in the form of eruption.
Ameloblastic Fibroma, Ameloblastic

Basal Cell Nevus Syndrome or Gorlin–Goltz Fibrodentinoma and Ameloblastic
Syndrome Fibro-odontoma
Basal cell nevus syndrome (BCNS) was first described by The ameloblastic fibroma is a rare benign tumor in which
Gorlin and Goltz in 1960, is an inherited disorder, with a both the epithelial and mesenchymal elements are neoplas-
high penetration and variable phenotype expression. It tic. In ameloblastomas only the epithelium is neoplastic. It
has a low incidence, around 1 in 56,000 persons. Its etiol- is important to differentiate the lesion from ameloblastoma
ogy is unknown, although the latest genetic studies relate since, unlike the latter, it does not exhibit a locally invasive
this syndrome to a disturbance at gene level of chromo- growth pattern.
some 9 (9q22.3–q31), which is passed from one generation
to the next, although there are only sporadic cases due to Clinical features
spontaneous mutation.
The ameloblastic fibroma usually occurs in a younger
age group than ameloblastoma and is uncommon in above
Clinical features
21 years of age. It presents as a slowly enlarging painless
It is characterized by a series of associated manifestations, swelling and arises most frequently in the premolar or
the most common being maxillary keratocysts and cuta- molar region of the mandible.
neous basal cell carcinomas, and other less frequent ones
which can be present, such as cardiac disturbances such as Radiographic features
persistent ductus arteriosus, characteristic facies in the
It is a well-circumscribed lesion and does not require the
form of mandibular prognathia, frontal and parietal prom-
radical excision that may be necessary to affect cure with
inence, marked superciliary arches, wide bridged nose and
the ameloblastoma. Radiographically, the tumor appears
hypertelorism, skeletal (brachymetacarpalism of the fourth
as a well-defined, usually unilocular, radiolucency. It may
and fifth fingers, vertebrae problems, costal synostosis or
be associated with an unerupted tooth and mimic a den-
bifid ribs), skin (dermoid cysts, lipomas), neurological
tigerous cyst.
(congenital hydrocephalus, calcification of the cerebral
falx, learning difficulties), sight such as blindness, con-
Histopathology
genital cataracts, strabismus), hormonal (hypogonadism)
or associated with other malignant neoplasias. The appear- The tumor consists of proliferating strands and clumps of
ance of pitted depressions, due to the lack of corneous odontogenic epithelium lying in highly cellular fibroblastic
342
Figure 11 Box 4 Differential diagnosis of ameloblastic fibroma
❍ CEOT
❍ Odontoma
Highly cellular
mesenchymal ❍ COC
stroma
❍ AFO
Proliferating
odontogenic
Benign Cementoblastoma
epithelium
Cementum is a modified form of bone and, with the excep-
tion of the cementoblastoma, disorders of the jaws con-
taining cementum-like tissue are now classified as lesions
of bone. The cementoblastoma is still classified as an
Histopathological slide of ameloblastic fibroma. odontogenic tumor because of its unique association with
Courtesy: Department of Oral Pathology and the root of a tooth. Cementoblastoma is classified as an
Microbiology, MCODS, Mangalore odontogenic tumor because of its unique association with
the root of a tooth. This is one characteristic feature that
differentiates it from osteoblastoma of bone.
Box 3 Key points on ameloblastic fibroma and related lesions Clinical features
1. Ameloblastic fibroma and fibro-odontoma are benign neoplasms The cementoblastoma is a rare benign neoplasm most fre-
2. Ameloblastic fibro-odontoma is probably a hamartoma quently seen in patients below 25 years of age. It usually
arises in the molar or premolar area of the mandible and
3. Well-circumscribed and occur mainly in 1st and 2nd decades
is attached to the root of a tooth. Most cases involve the
4. Comprises odontogenic epithelium and richly cellular mesenchyme
mandibular first permanent molar. It presents as a slowly
5. Fibrodentinoma contains dentin enlarging swelling which sometimes gives rise to pain, but
6. Fibro-odontoma enamel and dentin the involved tooth is vital. Pain is main symptom, pain is
severe and not relieved by NSAIDs.
tissue resembling the dental papilla of the developing
tooth. The epithelial component shows a peripheral layer Radiographic features show a well-demarcated, mottled, or
of cuboidal or columnar cells which encloses a few stellate dense radiopaque mass with a radiolucent margin attached
reticulum-like cells (Figure 11). The appearances are simi- to the root of a tooth which usually shows resorption.
lar to ameloblastoma but the stellate cells are much less
abundant and cyst formation is unusual. In some lesions Histologic features
dentin may be present and such tumors may be designated
Tumor consists of a mass of calcified cementum-like tissue
ameloblastic fibrodentinoma. The dentin is usually poorly
containing scattered cells lying in lacunae. Around the
formed and includes entrapped cells. Tubular dentin is rare.
periphery and in other actively growing parts of the lesion,
extensive sheets of uncalcified matrix formed by plump,
Literature review
deeply staining cementoblasts may be seen.
Tumors previously classified as dentinomas are now thought
to be examples of the ameloblastic fibrodentinoma. The Prognosis
ameloblastic fibro-odontoma is a tumor which shows fur-
ther inductive changes leading to the formation of enamel. Lesions which are incompletely removed may recur.
Although the ameloblastic fibroma and fibrodentinoma
are benign neoplasms, it is probable that the ameloblastic
Odontogenic Myxoma
fibro-odontoma is a hamartoma. Histologically, it is difficult
to distinguish from a developing complex odontome, to These are uncommon, benign neoplasms arising from
which it is probably closely related. mesenchymal odontogenic tissue which are locally inva-
Key points on ameloblastic fibroma are given in Box 3. sive neoplasm consisting of rounded, angular cells lying
Differential diagnosis is given in Box 4. in abundant myxoid stroma.
343
Clinical features Desmoplastic Fibroma

Commonly seen in the 3rd decade of life with higher prev- This is a rare fibrous lesion of the jaws. It is benign, but
alence among women, and occurs mainly in the mandibu- aggressive, exhibiting a biologic behavior similar to fibro-
lar molar area. Presents as painless swelling or incidental matosis of soft tissue or low-grade fibrosarcoma. It is seen
finding. in young adults, especially in the mandible.
Radiographically, desmoplastic fibroma is lucent, with
Radiographic features margins that may be distinct or poorly defined.
Histologically, these lesions exhibit an interlacing or
Often multilocular with internal osseous trabeculae arranged fascicular growth pattern of benign fibroblasts and colla-
in ‘tennis racket’ pattern. gen. They neither contain epithelial rests nor make bone.
MRI: Prolongation of T1 and T2, reflecting rich myxoid Multinucleated giant cells are rarely present. Desmoplastic
stroma. Gradual contrast enhancement is typically seen on fibroma should not be confused with central low-grade
contrast-enhanced images. osteosarcoma which is more cellular and has cytologic
atypia.
Histopathology
Treatment
Odontogenic myxomas consist of rather monotonous cells
with multipolar or bipolar slender cytoplasmic extensions Ennucleation/excision or partial resection of the jaw bone.
that lie in a myxoid stroma. Nuclei vary from round to
fusiform in appearance. Binucleated cells and mitotic fig-
ures are present, but scarce. Occasionally, the lesion contains BENIGN NON-ODONTOGENIC TUMORS
odontogenic epithelial rests. The lesion spreads into the
jaw bone without any encapsulation, thereby engulfing
neighboring cancellous bone. ❍ Squamous Papilloma
❍ Verruca vulgaris
❍ Verrucous hyperplasia
Treatment ❍ Condyloma accuminata.
Excision or partial resection of the jaw bone with high
rate of local recurrence due to infiltrative growth pattern
The primary disease processes that give rise to swellings
enhancement is typically seen on contrast-enhanced
and tumors of the oral cavity include cysts, mucous
images.
extravasations and retention in the minor salivary glands,
foci of granulation tissue and inflammation, abscesses and
Odontogenic Fibroma connective-tissue proliferations that are well-defined or
encapsulated, as well as infiltrative sarcomas. Both epithe-
This jaw tumor is considered a neoplasm that is derived lial and connective-tissue disease processes can present as
from periodontal ligament or pulp-related fibroblasts. It is tumors. From a clinical perspective the three most important
a tumor of adults and appears as a well-defined radiolu- defining characteristics of any soft tissue swelling are
cency in either jaw. It is not, however, particularly aggres- location, coloration, and palpable nature.
sive, and it infrequently recurs after simple curettage.
Location Certain diseases tend to occur in specific sites
to the exclusion of others, as given in Table 5.
Histopathology
Coloration It is dependent upon the tissues present in
These lesions are more collagenous than myxomas but
the mass and the depth of the lesion. In general, yellow-
may range from myxofibrous to densely fibrous. Charac-
appearing lesions are comprised of lymphoid tissue or adi-
teristically seen in odontogenic fibromas are few to many
pose tissue, red swellings are vascular, blue swellings are
islands and strands of bland odontogenic epithelium.
mucinous or venous, and brown swellings contain mela-
Calcific deposits may also be found. A variant (granular
nin or blood pigments. Lesions with normal mucosal pink
cell odontogenic fibroma), in which granular cells are seen
coloration are generally composed of fibrous tissues or
in the connective tissue, has been described. The behavior
some other tissues lying deeper in the connective tissues
of this tumor does not appear to be different from odonto-
(Table 6).
genic fibroma. Abundant rest proliferation in follicular sacs
can occasionally simulate the appearance of odontogenic Palpable nature Based on the consistency they can be
fibroma or ameloblastic fibroma. grouped as shown in Table 7.
Clinicopathological correlation is important for the Human papillomavirus (HPV) and oral lesions given in
diagnosis of these lesions. Box 5.
344
Table 5 Soft tissue swellings according to site Table 7 Soft tissue swellings according to their consistency
Site Type of lesion Palpation characteristic Swelling

Lips and buccal Fibroma, mucocele, mesenchymal tumor, salivary Soft, fluctuant Mucocele, ranula
mucosa tumor Developmental cysts
Gingiva Parulis, pyogenic granuloma, peripheral fibroma, Sialocysts
peripheral giant cell granuloma, peripheral ossifying Gingival cysts
fibroma, gingival cyst, peripheral odontogenic tumors Parulis
Abscess
Palate Abscess, torus, salivary gland tumor
Soft non-fluctuant Lipoma
Dorsum of the Fibroma, granular cell tumor, pyogenic granuloma
Fibroma
tongue
Organized mucocele
Ventral aspect Mucocele, ranula, lymphoid aggregates,
Firm fixed Mesenchymal tumors
of the tongue lymphoepithelial cyst, osteocartilaginous choristoma
Granulomas
Salivary adenomas
Adnexal skin tumors
Firm movable Granular cell tumor
Table 6 Soft tissue swellings according to their color Seborrheic keratosis
Keratoacanthoma
Color Soft tissue mass
Fibromatosis
Blue-purple Hemangioma, varix, hematoma, peripheral
giant cell granuloma, mucocele, Kaposi sarcoma
Red Hemangioma, pyogenic granuloma, Kaposi sarcoma
Brown Nevus, hematoma, seborrheic keratosis, Kaposi
Box 5 Human papillomavirus and oral papillary lesions
sarcoma, melanoma
Black Melanoma Verruca vulgaris 2, 4
Yellow-orange Lymphoid aggregates, lymphoepithelial cyst, lipoma, Squamous papilloma 6, 11

granular cell tumor Condyloma acuminatum 6, 11
Focal epithelial hyperplasia 13, 32
Squamous cell carcinoma 16, 18, 31, 33, 35
Classification based on the tissue of origin is given in Proliferative verrucous leukoplakia 6, 11, 16
Table 8.
Papillary lesions of oral cavity

In the form of white lesion, usually solitary; may be
Papillary lesions are those that are tumefactive with a cau- multiple.
liflower surface. Some are pedunculated, others are sessile. Squamous papilloma affects the buccal mucosa, soft
Some are single, others are multiple or diffusely involve palate, uvula, tongue and gingiva.
broad areas of the oral mucosa. The vast majority of papil-
lomas are associated with or indeed caused by members of
Histopathology
the HPV family, yet there are a few papillary growths that
have not been associated with HPV. One lesion in particu- Epithelial hyperplasia with fibrovascular cores may be
lar, molluscum contagiosum, is caused by a member of the seen. The papillary projections may be sharp to blunt
poxvirus group. (Figure 12). Epithelium may be dysplastic in some lesions
from HIV-positive patients.
Squamous Papilloma
Diagnosis
A benign epithelial proliferation induced by HPV in most
cases; several subtypes have been identified, especially Lesions of the condyloma acuminatum, verruca vulgaris,
HPV-6 and 11. focal epithelial hyperplasia and verrucous carcinoma may
mimic lesions of squamous papilloma.
Clinical features
Treatment
Exophytic, papillary mass, measuring less than 1 cm, usually
pedunculated and soft in texture. Surgical excision is the preferred modality of management.
345
Table 8 Classification based on the tissue of origin

perioral skin lesions may be brownish. The oral mucosal
lesions are usually white to pink. It can be pedunculated or
Epithelial Papilloma broad based.
Keratoacanthoma Sites of predilection include the lips, palate, and attached
Nevus gingiva. The incidence of oral warts due to HPV has dra-
Connective tissue Fibroma matically increased in the potent antiretroviral therapy
Giant cell fibroma era. Diagnosis can be based on the clinical, microscopic
Peripheral ossifying fibroma and immunohistochemical demonstration of HPV com-
Lipoma mon antigen.
Vascular tissue Hemangioma
AV fistula
Histopathology
Carotid body tumor
Lymphatic origin Lymphangioma Histological changes can be in the form of surface hyper-
Neural tissue Neurilemmoma keratosis, granulosis and koilocytosis.
Neuroma AIDS-associated oral warts may appear dysplastic
Neurofibroma microscopically.
Neurofibromatosis
Muscular origin Leiomyoma Differential diagnosis
Rhabdomyoma
❍ Focal epithelial hyperplasia
Bone origin Osteoblastoma
❍ Keratoacanthoma
Osteoid osteoma
Benign osteoblastoma
❍ Papillary squamous carcinoma
Desmoplastic fibroma of bone ❍ Squamous papilloma
❍ Condyloma acuminatum.
Cartilaginous Chondroblastoma
AV: Arteriovenous.
Treatment
Treatment may involve surgery, laser surgery, or cryother-
apy. It should be noted that HPV survives in aerosol.
Figure 12 Topical 5-fluorouracil treatment has been used on external
lesions, but should be avoided in fair individuals as it can
cause hyperpigmentation. It should be noted, however, that
this is a specialized treatment and should only be used by
Hyperplastic those experienced with the use of this topical medication.
epithelium Lesions tend to recur after treatment.
Connective
tissue core Verrucous Hyperplasia
Lesion with an unknown etiology, even though tobacco
Blood vessels (smokeless) association has been reported, the role of HPV
is unclear and is even hypothesized to be a possible pre-
cursor to verrucous carcinoma.
Histopathological slide of papilloma. Courtesy: Department of Clinical features

Oral Pathology and Microbiology, MCODS, Mangalore
Presents clinically as exophytic, papillary, keratotic fronds
of epithelium or can be a part of the proliferative verru-
cous leukoplakia spectrum.
Verruca Vulgaris (Oral Warts)
Infection of mucosal epithelium by members of the human Histopathology
papillomavirus group—usually HPV-2, 4, 6, or 11.
Histologic features are in the form of papillary to verruci-
form surface projections in which keratin varies in thick-
Clinical features
ness. They can be broad, bosselated epithelial ridges with
Papular to nodular and exophytic appearance. The warts well-differentiated cellular features and can even mimic
may be cauliflower-like, spiked, or raised with a flat surface, an early verrucous carcinoma.
346
Differential diagnosis below the epithelium; marked pseudoepitheliomatous

hyperplasia.
Verrucous carcinoma, papillary squamous cell carcinoma
and proliferative verrucous leukoplakia can be considered
in the differential diagnosis. Diagnosis
Clinical appearance can be confusing, the symmetrical
Treatment nature, oval or round configuration, and keratotic core are
Excision or ablation (e.g. laser, electrocautery) has been indicative of KA.
used successfully in the management of verrucous
hyperplasia. Differential diagnosis
❍ Squamous cell carcinoma
Condyloma Acuminatum ❍ Molluscum contagiosum
Described in Chapter 22 on Sexually Transmitted Diseases ❍ Verruca vulgaris.
on page 630.
Treatment
Focal Epithelial Hyperplasia/Heck’s Disease Observation and careful follow-up local excision, cryo-
therapy and intralesional chemotherapy have also been
Described in Chapter 6 on Red and White Lesions on
tried. Wong et al in their study reported oral isotretinoin
page 165.
to be an effective treatment of multiple as well as solitary
keratoacanthomas. Canas et al reported that successful
Keratoacanthoma/Self-healing Carcinoma treatment was achieved with administration of oral
isotretinoin. Spieth et al reported that intralesional metho-
Keratoacanthoma (KA) is usually encountered on the facial trexate shows to be an effective, easy and inexpensive
skin and lips yet can also arise, albeit rarely, in the mouth. alternative.
Etiology
Unknown, may be related to several factors, as follows: Oral Melanoacanthoma (Melanoacanthosis)
❍ Viral—HPV subtypes 11, 13, 24, 33, 57 Oral melanoacanthoma is a benign acquired pigmentation
❍ Altered expression of cell cycle proteins including cyclin of the oral mucosa characterized by dendritic melanocytes
E, p53, PCNA dispersed throughout the epithelium. The lesion appears to
❍ Keratinocyte dedifferentiation reflected in deficient be a reactive process.
desmoglein production
❍ Immunosuppression
Clinical features
❍ Sun damage
❍ May represent a highly differentiated form of squamous Oral melanoacanthoma is seen in blacks, shows a female pre-
cell carcinoma. dilection, and occurs in the 3rd and 4th decades of life. The
buccal mucosa is the most common site of occurrence. The
Clinical features lesion is smooth, flat or slightly raised, and dark-brown to
black in color.
Clinically these are characterized by a tumefaction with
round, mounded borders that surround a central core of hard
keratinized material that may appear pale yellow or brown. Histopathologic features
The brown appearance is caused by extrinsic pigments that
Numerous benign dendritic melanocyte cells that are nor-
become incorporated with the excessive keratin. Intraoral
mally confined to the basal cell layer scattered throughout
KAs are generally non-pigmented. They can be solitary on
the lesional epithelium. Basal layer melanocytes are also
sun-exposed areas, including lip. They initially present as
present in increased numbers.
erythematous papule with rapid growth over 4–8 weeks;
extremely rare intraorally.
Histopathology
Because of the alarming growth rate of oral melanoacan-
Histopathology shows keratin plus normal, peripheral epi- thoma, incisional biopsy is usually indicated to rule out the
dermis and mature, premature keratinization; no invasion possibility of melanoma.
347
Acquired Melanocytic Nevus (Nevocellular Nevus, Treatment

Mole) No active intervention is required, unless in case of clinical
Nevus refers to congenital/developmental malformations. suspicion and cosmetic concern.
Acquired melanocytic nevus represents localized prolifer-
ation of cells from the neural crest, called ‘nevus cells’. They
are called as ‘first cousins’ of melanocytes and represent the Ossifying Fibroma
most common human ‘tumors with white adults reported
Described in Chapter 19 on Bone Diseases and Fibro-
to have an average to 40 cutaneous nevi per person but
osseous Lesions on page 571.
intraoral lesions are rare.
Clinical features Juvenile Ossifying Fibroma

Acquired melanocytic nevi begin to develop during child- Described in Chapter 19 on Bone Diseases and Fibro-
hood and most cutaneous lesions present before 35 years osseous Lesions on page 572.
of age with no sex predilection even though racial differ-
ences are seen.
Head and neck region is a common site of involvement. Peripheral Ossifying Fibroma
Junctional nevus is a sharply demarcated, brown or black
macule less than 6 mm in diameter. When nevus cells pro- Peripheral ossifying fibromas (POF) are reactionary lesions
liferate become slightly elevated soft papule it is called thought to originate from submucosal connective tissue and
as compound nevus. The degree of pigmentation becomes the periodontal ligament. The lesions are often confused
less; most lesions appear brown or tan. Nevus gradually with either the more vascular (red to pink) pyogenic gran-
loses its pigmentation, the surface may become somewhat uloma, or the fibroma that demonstrates more tissue col-
papillomatous, and hairs may be seen growing from the ored lesions. Peripheral odontogenic ossifying fibromas
center intradermal nevus remains less than 6 mm in diam- are classified most often in literature as reactionary lesions
eter. Ulceration is not a feature unless placed at a region rather than a benign tumor.
of recurrent trauma. Intraoral melanocytic nevi are dis-
tinctly uncommon, they may arise on the palate or gingiva, Etiology
although any oral mucosal site may be affected. More than
one in five intraoral nevi lack clinical pigmentation and It is thought that an exuberance of tissue is caused by an
females are more affected; the average age at diagnosis is initiating factor such as calculus, or a food particle that
35 years. becomes lodged in the sulcus, creating an initial irritation.
Histopathologic features Pathogenesis

The acquired melanocytic nevus is characterized by a Chronic irritation causes an abundance of fibrous connec-
benign proliferation of small, ovoid nevus cells, produce tive tissue containing immature bone. Often patients trau-
melanin. Nevus cells typically lack the dendritic processes matize the area again, and this promotes a cycle of more
like melanocytes tend to be organized into small, round exuberant tissue formation and varying surface ulceration.
aggregates (thèques). When lesional cells are found at There is a close resemblance to the pyogenic granuloma.
the junction between the epithelium and the connective The lighter surface color is due to hyperkeratosis produced
tissue, this stage is known as a junctional nevus. When through trauma.
the groups of cells begin to drop off into the underlying
dermis or lamina propria. Within the underlying connec-
Clinical features
tive tissue, the lesion then is called a compound nevus.
When found only within the underlying connective tissue Peripheral odontogenic ossifying fibromas occur more
this stage is called an intradermal nevus, the intraoral often in females and can be anywhere from 1 to 2 cm in
counterpart is called an intramucosal nevus. The superfi- size. They occur most often in young adults, originate
cial cells typically appear larger and epithelioid, with from the periodontal ligament, and occur exclusively on
abundant cytoplasm and the middle portion of the lesion the gingiva as a sessile or pedunculated mass. The lesions
have less cytoplasm, are seldom pigmented, and appear are normally found on the attached gingiva. The abundance
much like lymphocytes. Deeper nevus cells appear elon- of tissue is usually the same color as the surrounding tis-
gated and spindle-shaped, much like Schwann cells or sue, with a smooth surface. Sometimes the surface may be
fibroblasts. more textured, depending upon the existing oral forces
348
Figure 13 Treatment and prognosis

The treatment of choice is excision of the lesion with the
periodontal ligament, and no recurrence is expected in most
instances. Complete removal is indicated since peripheral
odontogenic ossifying fibromas can reoccur when they are
not adequately removed. It is advised that the lesion be
removed down to the periosteum and that the adjacent
teeth be scaled to remove any remaining irritants. This will
assist in lowering the rate of recurrence.
Osteoma
Benign, slowly growing lesion composed of well-differen-
tiated mature bone with a predominant lamellar structure.
It is usually an incidental finding or painless hard swelling.
Peripheral ossifying fibroma in the interdental regions of the
upper lateral and canine. Courtesy: Department of Oral Etiology
Medicine, KLE Institute of Dental Sciences, Bangalore
Sporadic form is idiopathic or may be a component of
Gardner’s syndrome.
Clinical features
Figure 14
Sporadic form with frontal and sphenoid sites predisposed
or may be multiple. Solitary lesions are rare in jaws.
Stratified
squamous
epithelium Radiographic findings
Well-circumscribed, dense, sclerotic. May be subperiosteal
or medullary (Figure 15).
Connective
tissue Histopathology
Area of Histology reveals dense areas of normal cortical and tra-
ossification beculae (Figure 16).
Diagnosis
❍ Radiographic features
Histopathological slide of peripheral ossifying fibroma. ❍ Microscopic features: Normal cortical and trabecular
Courtesy: Department of Oral Pathology and bone.
Microbiology, MCODS, Mangalore
❍ Tori, exostoses
❍ Ossifying fibroma
and location in the patient’s mouth. Additionally, the sur-
❍ Osteoblastoma
face epithelium may be more of a dark color depending
❍ Focal sclerosing osteitis.
upon the amount of trauma and inflammation that is pro-
duced with the lesion (Figure 13).
Treatment
Histopathologic features Local resection, if compromising.
Microscopically, the tissue is composed primarily of cellu-
Prognosis
lar fibroblasts with various types of calcification including
bone, cementum and dystrophic calcification. The surface Little recurrence potential when associated with Gardner’s
epithelium may exhibit varying degrees of ulceration syndrome, malignant conversion of intestinal polyps is
(Figure 14). assured.
349
Figure 15 Clinical features

Chondromas may be seen at any age and has no gender
preference. It is seen as a slowly growing, non-painful
swelling. The anterior maxilla, which is the site where
vestigial cartilage rests are found, is the most commonly
affected site. The posterior regions of the mandible (usu-
ally beyond the cuspids), condyle, coronoid process and
the body of the mandible are the most commonly affected
sites.
The soft tissue chondromas are usually seen affecting
the soft palate, tongue and buccal mucosa.
Enchondromas are associated with Maffucci’s syndrome
and Ollier’s disease.
Lower occlusal view showing osteoma. Courtesy: Department of Enchondromatosis or Ollier disease (WHO terminology)
Oral Medicine and Radiology, MCODS, Mangalore is defined by the presence of multiple enchondromas and
characterized by an asymmetric distribution of cartilage
lesions that can be extremely variable (in terms of size,
number, location, evolution of enchondromas, age of onset
Figure 16 and of diagnosis, requirement for surgery).
The condition in which multiple enchondromatosis is
associated with soft tissue hemangiomas is known as
Maffucci’s syndrome.
Compact bone Maffucci’s syndrome is a rare non-hereditary mesoder-
mal dysplasia characterized by the presence of multiple
hemangiomas of the soft tissue and multiple enchondro-
mas (usually of the phalanges and long bones). The syn-
Haversian system
drome can be associated with benign or malignant tumors
(parathyroid adenoma, pituitary adenoma, hemangioendo-
thelioma, adrenal tumor, ovarian tumor, chondrosarcoma
and breast cancer).
Histopathological picture of osteoma. Courtesy: Department
of Oral Pathology and Microbiology, MCODS, Mangalore Radiographically, chondromas exhibit ill-defined to occa-
sional well-defined radiolucent areas. Some lesions may
show the presence of radiopaque foci interspersed within
the radiolucent zones. Extensive lesions may cause destruc-
Chondroma tion of the cortical plates. Root resorption may be seen.
Chondromas are benign tumors of mesenchymal origin

composed of multiple nodules of mature hyaline cartilage. Histopathologic features
It is estimated that chondromas represent approximately Histologically chondromas are characterized by the pres-
2.38% of osteocartilaginous tumors. ence of lobulated masses partially subdivided in nodules of
Chondromas are well-recognized tumors in the bony skel- hyaline cartilage immersed in a fibrous stoma. Frequently
eton. However, these are relatively rare in the jaw bones. there are calcified foci and even ossification. The eosino-
Based on the location chondromas are classified as philic cytoplasm may exhibit vacuoles (Figure 17).
enchondromas (account for 80% of these tumors, located
in the medullary cavity of the long bones, diaphysis), juxta
Management
articular chondromas (related to extraskeletal cartilage),
extraskeletal chondroma or soft tissue chondroma (rare Long-standing chondromas can undergo a sarcomatous
cartilaginous tumor, involving hands, feet, tongue and change. Surgical resection is the treatment of choice. How-
buccal mucosa). ever, recurrences have been reported.
350
Figure 17 Histopathologic features

Polygonal chondroblasts are the main proliferating cells.
These have long or oval-shaped nuclei and a prominent
Connective cytoplasmic membrane with cytoplasmic granules consist-
tissue capsule
ing of hemosiderin pigment. Occasionally, multinucleated
cells can be found. Chondroid matrix can be found outside
the cytomembrane where small calcifications may occur.
The most important feature is the linear deposition of cal-
Chondrocytes cification surrounding individual chondroblasts, giving rise
in lacunae
to a chicken-wire pattern (Figure 17).
Hyaline Immunostaining occasionally can be helpful in confirm-
cartilage ing the diagnosis of chondroblastoma. S-100 protein is
strongly positive. Chondroblastomas are also positive for
vimentin. Reticulin stain reveals a honeycomb pattern.
Histopathological feature of chondroma. Courtesy:
Department of Oral Pathology and Microbiology, Management and prognosis
MCODS, Mangalore
Chondroblastoma generally exhibits local aggressive behav-
ior with infiltration and invasion to the soft and hard tissues
around.
Surgical resection is the main treatment modality. It is
Benign Chondroblastoma (Codman’s Tumor) estimated that, when located in the long bones, the curet-
tage of this tumor has a success rate of 90%. When located
Ernest Amory Codman, in 1931 first described this tumor in the temporomandibular region, the resection can be dif-
as a variant of the giant cell tumors. However, in the ficult because of the complex anatomical structure, which
1940s Jaffe and Lichtenstein renamed the Codman’s tumor results in a higher recurrence rate. It is estimated that the
as benign chondroblastoma in accordance to its chondro- recurrence rate is about 55%. Radiation should be avoided
blastic origin. because of the risk of developing a chondrosarcoma.
Chondroblastoma is a rare tumor of bones accounting
for about 1% of all bone tumors. It is rarely seen in the
maxillofacial region.
Benign Osteoblastoma
In the long bones, chondroblastoma is believed to arise
from the cartilage of the growth plates, and in the tem- Benign osteoblastoma is an osteoid and bone forming
poromandibular region, possibly from the cartilage of the benign tumor of bone. It is an uncommon lesion that
condylar process. accounts for 1% of all bone tumors and about 3% of all
benign bone tumors.
Clinical features
Clinical features
Chondroblastomas are usually seen in the 3rd decade of
life. Males are slightly more commonly affected than Benign osteoblastoma occurs in the 2nd and 3rd decades
females (1.86:1). of life, but the age range is 5–78 years. As to sex incidence,
In the maxillofacial and the temporal region, mandible, there is male predominance 2–3:1. Benign osteoblastoma
maxilla and the TMJ have been reported to be affected. can arise in any bone, but there is a predilection for the
Patients may complain of a local mass, auditory dyses- vertebra, long bones, skull and small bones of the hand
thesia, and limitation of mouth opening. and feet. Clinically, osteoblastoma is a well-circumscribed,
solitary lesion, which measures 2 cm or more and may be
as large as 12 cm. Usually, the lesion is not painful, or if
pain is present, it is not very responsive to salicylates.
On radiographs, chondroblastoma affecting the extremi-
ties appears as round to ovoid radiolucencies which are
well-defined, expansile in nature and usually exhibit a
sclerotic margin. Radiographically, the picture may vary in accordance with
Chondroblastomas affecting the condyle have been the size of the tumor and with the extent to which the tissue
reported to have caused resorptive defects and occasion- is calcified. Osteoblastomas are well-circumscribed, expan-
ally enlargement of the condyle. sive lesions with central radiopaque areas suggesting new
351
bone formation, but it is less likely to provoke an out- predominates 3:1 over that in maxilla and anterior to
standing bony sclerosis typical of osteoid osteoma. molar teeth with tendency for bony expansion. Most cases
are non-aggressive, slow growing, and asymptomatic, with
Histopathologic features no cortical breakthrough or root end resorption. Some cases
Histologically, benign osteoblastoma consists of a highly are recurrent and exhibit aggressive behavior with pain,
vascularized, fibrocellular stroma in which there are abun- perforation, and rapid enlargement.
dant newly formed trabeculae of immature bone and
osteoid. Proliferating osteoblasts are found lining the tra- Radiographic findings
beculae of immature bone and osteoid. The major problem Usually multilocular, occasionally unilocular, radiolucency
for pathologists is the correct differentiation between with well-defined margins but borders may be scalloped.
benign osteoblastoma and a number of lesions that may It can displace teeth; less commonly it resorbs tooth roots;
have similar characteristics. Benign osteoblastoma and they may even expand bone.
osteoid osteoma have almost identical histologic features.
Based on this histologic similarity, Dahlin and Jonson in MRI: Heterogeneous intermediate signal on T1 and
1954 suggested a name ‘giant osteoid osteoma’ for the T2 weighted image with contrast enhancement.
lesion that is denoted as the benign osteoblastoma in the
present. Differential diagnosis
Diagnosis ❍ Odontogenic lesions

❍ Ameloblastoma
The diagnostic evaluation is based on the histologic fea- ❍ Odontogenic myxoma
tures and the clinical behavior of the lesion. ❍ Odontogenic keratocyst
❍ Non-odontogenic lesions
Differential diagnosis ❍ Hemangioma
Osteoid osteoma, ossifying fibroma, condensing osteitis, ❍ Aneurysmal bone cyst
Paget’s disease, fibrous dysplasia, chondroblastoma, cemento- ❍ Traumatic bone cyst.
blasoma, aneurysmal bone cyst and giant cell granuloma.
Peripheral Giant Cell Granuloma
Peripheral giant cell granuloma (PGCG) is, for all practical
Surgical excision, malignant transformation (osteosarcoma)
purposes, a site-specific variant of pyogenic granuloma
within a benign osteoblastoma is very rare.
embedded with osteoclast-like multinucleated giant cells
and arising exclusively from the periodontal ligament
enclosing the root of a tooth. This unique origin, of course,
Giant Cell Granuloma means that such a lesion can only be found within or upon
The term ‘giant cell granuloma (GCG)’ has also been intro- the gingiva or alveolar ridge, no other site is acceptable.
duced to account for the lack of pre-existing trauma or It is also called variously giant cell reparative granu-
reparative tissue in some of these lesions. Central giant cell loma, osteoclastoma, giant cell epulis, and myeloid epulis,
granuloma (CGCG) is described as a benign lesion affecting this lesion was first reported as fungus flesh in 1848.
the mandible and maxilla that consists of a massive fibro-
histiocytic proliferation with numerous heavily hemosidrin- Clinical features
laden multinucleated giant cells. In 1953, Jaffe first described
The usual age for diagnosis is 4th to 6th decade, but there is
giant cell reparative granuloma (GCRG) as a benign lesion
no marked age predilection. More than 60% of cases occur
affecting the mandible and maxilla.
in females and this predilection is more pronounced in the
older age groups. Individual lesions are nodular and pedun-
Etiology culated, frequently with an ulcerated surface with a red,
Probably reactive or responsive in nature. Speculation brown or bluish hue. Generally larger than pyogenic gran-
suggests it may represent a developmental anomaly. uloma, the lesion may exceed 4 cm in size, but most lesions
remain less than 2 cm in diameter.
Clinical features
Most cases arise in those less than 30 years of age with
female predominance. It occurs exclusivity in mandible or Any alveolar region may be affected and radiographs may
maxilla; rarely in facial bones. Occurrence in mandible show either a saucerization of underlying bone, periodontitis
352
Poncet and Dor in 1897 first described pyogenic granu-

Figure 18
loma as granuloma pyogenicum. Over the years various
authors have suggested other names such as granuloma
Epithelium gravidarum, pregnancy tumor, Crocker and Hartzell’s dis-
ease, vascular epulis, benign vascular tumor, hemangio-
Inflammatory
matosis granuloma, epulis telangiectaticum granulomatosa,
cells and lobular capillary hemangioma.
Connective
tissue stroma Etiology
The etiology of the lesion is unknown, though it was orig-
Blood vessels inally believed to be a botryomycotic infection. It is theo-
Giant cells rized that pyogenic granuloma possibly originates as a
response of tissues to minor trauma and/or chronic irrita-
tion, thus opening a pathway for invasion of non-specific
Histopathological picture of peripheral giant cell microorganisms, although these microorganisms are sel-
granuloma. Courtesy: Department of Oral Pathology and dom demonstrated within the lesion.
Clinical features
of underlying tissues, or an isthmus of soft tissue connect- Pyogenic granuloma of the oral cavity occurs at any age
ing to an intraosseous central giant cell granuloma. and in all populations with no racial predilection. Lawoyin
et al (1997) reviewed 38 cases from Ibadan, Nigeria and
Histopathology reported an average range of 5–75 years with the mean
age of 33 years. Most studies demonstrate a definite female
The peripheral giant cell granuloma is comprised of an predilection with a female to male ratio of 2:1. This is
unencapsulated aggregation of rather primitive but uni- attributed to the vascular effect of female hormones that
form mesenchymal cells with oval, pale nuclei and with a occur in women during puberty, pregnancy, and meno-
moderate amount of eosinophilic cytoplasm. Mitotic activ- pause (Figure 18). The lesions tend to occur more often
ity is not unusual in the lesion and may even be pro- during the second and third trimester of pregnancy and
nounced in lesions developing in children and adolescents. such lesions are referred to as ‘pregnancy tumors’. Daley
Stromal cells may be spindled with a background of col- et al (1991) in their study indicated that the clinical diag-
lagenic fibers, or may be rounded with a less fibrotic back- nosis of ‘pregnancy tumor’ can be given when describing
ground. A thin band of routine fibrovascular tissue a pyogenic granuloma occurring in pregnancy, because it
separates the lesion from the overlying epithelium, often describes a distinct lesion not on the basis of histologic
with dilated veins and capillaries. When surface ulceration features but on etiology, biologic behavior, and treatment
is present, the ulcer bed consists of routine fibrinoid protocol.
necrotic debris over granulation tissue (Figure 18). Marks et al reported that pyogenic granulomas may
occur anywhere in the body surface and except the oral
Treatment cavity, these are common around the fingers and toes. In
the oral cavity, pyogenic granulomas show a striking pre-
Peripheral giant cell granuloma is treated by conservative dilection for the gingiva, with interdental papillae being the
surgical excision followed by curettage of any underlying most common site in 70% of the cases. These are more
bony defect and careful scaling and root planing of asso- common in the maxillary anterior area than any other
ciated teeth. A recurrence rate of 10% or more has been area in the mouth. Gingival irritation and inflammation
reported, hence, re-excision may be necessary. that result from poor oral hygiene, dental plaque, and cal-
culus or overhanging restorations may be precipitating
factors in many cases (Figure 19).
Pyogenic Granuloma
The typical clinical presentation of pyogenic granu-
Pyogenic granuloma or granuloma pyogenicum is a well- loma is a small, deep red to reddish-purple lesion occur-
known oral lesion. It is a localized granulation tissue ring on the gingiva, which is either sessile or pedunculated.
overgrowth in reaction to mild irritation. The name ‘pyo- The surface may be smooth, lobulated or occasionally
genic granuloma’ is a misnomer since the condition is not warty, which is commonly ulcerated and shows a tendency
associated with pus and does not represent a granuloma for hemorrhage either spontaneously or upon slight
histologically. trauma.
353
Figure 19 Figure 20
Stratified
squamous
epithelium
Vascular
spaces
Inflammatory
cells
Connective
tissue
Clinical photograph of pyogenic granuloma. Courtesy:

MCODS, Mangalore Histopathological picture of pyogenic granuloma.
Courtesy: Department of Oral Pathology and
Diagnosis
Although pyogenic granuloma can be diagnosed clinically 15% has been noted. After surgical excision of gingival
with considerable accuracy, radiographic, and histopatho- lesions, curettage of underlying tissue is recommended.
logical investigations aid in confirming the diagnosis and
planning the treatment. Marx et al (2003) reported that
radiographs are advised to rule out bony destruction sug- Fibrous Hyperplasia: Denture-related
gestive of malignancy or to identify a foreign body or sharp (Epulis Fissurata)
restorative margin that would need to be removed with the
It is more of a reactive lesion rather than a neoplasm.
lesion. Long-standing pyogenic granulomas, like other irri-
tation hyperplasias can show dystrophic calcifications. The
radiographic appearance of such calcifications varies from Etiology
barely perceptible, fine grains of radiopacities to larger, Trauma resulting from an ill-fitting denture. Exuberant
irregular radiopaque particles that rarely exceed 0.5 cm in fibrous tissue repair secondary to repeated inflammation
diameter. and trauma.
Clinical features
All clinically suspected pyogenic granulomas must be biop-
It is found on vestibular mucosa, usually at facial aspect of
sied to rule out more serious conditions as mentioned pre-
denture flange. Clinically appears as rounded folds of
viously. The pathology is distinct consisting of a matrix of
broadly based fibrous tissue surrounding the overextended
edematous connective tissue in which numerous thin walled
denture flange. Ulceration often noted at depth of tissue
vascular channels can be seen. These vessels sometimes
folds and is more common in anterior segment of the jaws.
are organized in lobular aggregates, and some pathologists
It may occur on hard palate as a polypoid or leaf-like mass
require this lobular arrangement for the diagnosis (lobular
(Figure 21).
capillary hemangioma). There is also moderately dense
mixed cellular infiltrate. The overlying stratified squamous
epithelium may be atrophic or hyperplastic, and is usually Diagnosis
degenerated or ulcerated in large areas; and the ulcer edge Location and presence of a chronically ill-fitting denture.
may have a primitive dysplastic appearance (Figure 20).
Treatment
Treatment
Excision of all tissue and relining or reconstruction of new
Treatment of pyogenic granulomas consists of conservative dentures after excision.
surgical excision, which is usually curative. Although these
are reactive hyperplasias, they have a relatively high rate
Prognosis
of recurrence after simple excision, especially in pregnant
patients. Pierson and Pierson reported a recurrence rate of No recurrence anticipated with properly fitting denture.
354
Figure 21 Differential diagnoses

Differential diagnoses include hemangioma, fibroma, gran-
uloma, embryonal rhabdomyosarcoma, malignant granular
cell myoblastoma, alveolar rabdomyosarcoma, chondro-
genic and osteogenic sarcoma and schwannoma.
Treatment
Surgery is the only possible treatment of these tumors.
Surgery should not be radical; it minimizes the danger of
damaging underlying alveolar bone and developing tooth
buds. Delay in operation may cause airway obstruction and
feeding difficulty. The tumor should be removed during
the immediate postnatal period.
Epulis fissurata of the lower ridge. Courtesy:

Department of Oral Medicine, Manipal College of Lipoma
Dental Sciences, Mangalore A lipoma is a benign neoplasm composed of fat cells, oral
and oropharyngeal lipomas are rather rare.
Etiology
Congenital Epulis of Newborn Its cause is unknown. Trauma and metaplasia of perivascular
connective tissue have been suggested as playing a role.
The Greek word epulis means ‘on the gum’ or ‘gum boil’
and has unfortunately been used for a variety of benign
tumors and tumor-like conditions having dissimilar struc- Clinical features
tures and histogenesis. Congenital epulis is a rare benign Asymptomatic, slow-growing; usually circumscribed, ses-
soft tissue tumor that was also named ‘gingival granular sile, or pedunculated. Usually soft and compressive with
cell tumor of the newborn’ by Eppley. Since its first doughy consistency. When it is located superficially, a yel-
description by Neumann in 1871, more than 170 cases lowish texture can be seen. In rare instances bilateral or
have been reported. multiple occurrence has been reported (Pisanty, 1976). The
most common sites include buccal mucosa, tongue, floor
Clinical features of mouth; it can be deep seated with no color alteration but
surface of larger lesions often is covered with telangiec-
It occurs in females eight times more frequently than in tatic vessels.
males and affects the maxilla three times more frequently
than the mandible. In 10% of cases, it may have multiple
locations. The size of this nodular or lobular tumor may Histologic examination
vary from a few millimeters to 90 mm of diameter. It has Histologic examination of a lipoma shows a well-delineated
a firm consistency, is covered by a smooth erythematous mass of lobules of fat cells with fibrous septa interspersed
mucosa and is attached by a stalk to the incisor, or some- between them (Figure 22). In rare instances one encounters
times, canine regions. This tumor is smooth-surfaced, a benign ‘infiltrative’ lipoma, which should not be confused
pedunculated, sometimes lobulated and arises from the with a liposarcoma (Bennhoff and Wood, 1978). The distinc-
alveolar crest. Its size varies from a few millimeters to tion between a benign lipoma and a low-grade liposar-
9 cm. It is almost always located on the gingiva and is coma may indeed be difficult in some cases. When fibrous
found predominantly on the maxilla. More frequent in tissue is a substantial part of a lipoma, the term ‘fibroli-
females. poma’ is applied. When vascularity is a prominent feature,
the term ‘angiolipoma’ is used.
Histopathology
These tumors have large cells with an eosinophilic granu-
lar cytoplasm, but the congenital epulis is covered with a ❍ Soft tissue tumor
normal gingival epithelium. The congenital epulis is more ❍ Minor salivary gland neoplasm
vascularized. ❍ Metastatic disease.
355
Figure 22 Figure 23
Epithelium Antoni A tissue
Connective Verocay bodies

tissue
capsule
Adipocytes
Histopathological picture of neurilemmoma.

Histopathological picture of lipoma. Courtesy: Department of Courtesy: Department of Oral Pathology and
Oral Pathology and Microbiology, MCODS, Mangalore Microbiology, MCODS, Mangalore
Treatment material between them, the so-called Verocay bodies

(Figure 23).
Surgical removal is the treatment of choice. Recurrences
have not been reported with the exception of infiltrating Treatment
and intramuscular lesions.
Management consists of conservative surgical removal.
Recurrence is rare.
Neurogenic Tumors
Although the distinction between a schwannoma (neuri-
Neurofibroma
lemmoma) and a neurofibroma may be debatable, most
authors adhere to the concept that there are indeed two A neurofibroma is, like a schwannoma, derived from
separate entities. sheath cells. It actually may represent in many instances a
hamartoma or a reactive process rather than a neoplasm.
Schwannoma (Neurilemmoma) Clinical features

A schwannoma is a benign neurogenic neoplasm com- A neurofibroma is asymptomatic and rarely occurs as a
posed of Schwann’s cells. In a review from the literature single lesion. Most often neurofibromas are part of von
152 cases of oral schwannomas were collected (Gallo et al, Recklinghausen’s disease. Quite often the tongue is involved;
1977). in some cases resulting in unilateral macroglossia. Moreover,
the possibility of hereditary neuropolyendocrine syndrome—
Clinical features consisting of mucosal neuromas, pheochromocytoma of
the adrenal glands, and medullary thyroid carcinoma—
The tumor may occur at all ages and does not show a pref- should be taken into account. In general, the mucosal neu-
erence for men or women. A schwannoma is a slowly grow- romas in that syndrome are already present at childhood,
ing, rather circumscribed, submucosally located tumor that being the first manifestation of the syndrome (Casino et al,
may be painful. No characteristic clinical features appear. 1981), Skin lesions with syndromic forms—café-au-lait
It may occur at any site in the oral cavity and rarely involves macules (characteristically six or more), uniform pigmen-
the oropharynx. tation with smooth contoured borders are seen.
Histopathologic features Radiographic findings (when intrabony lesions are

present)
Histologically, one sees an encapsulated tumor composed
of two cell types—the so-called Antoni type A and Antoni ‘Blunderbuss’ expansion of the inferior alveolar foramen
type B cells. The A cells have elongated nuclei and are with uniformly expanded alveolar canal in the body of the
often arranged in a palisade pattern, including hyalinized mandible.
356
Histopathologic features Histopathologic features

In contrast to the schwannoma, a neurofibroma is not A rhabdomyoma is a well-circumscribed tumor. Based on
encapsulated. Proliferating Schwann cells are haphazardly histopathologic characteristics and gross morphology, two
arranged, not showing the palisade arrangement of types are recognized: fetal and adult.
schwannomas. The adult type is composed of large, round or polygonal
cells with a slight granular cytoplasm. The cytoplasm may
Treatment contain lipoid material. Cross-striations may be found in
With a single neurofibroma, management consists of surgi- just a few cells. The fetal type almost exclusively occurs in
the first few years of life. Histologically, this type is char-
cal removal. With multiple or massive involvement, surgi-
acterized by immature skeletal muscle in varying stages of
cal removal may be impossible to carry out and is indicated
developmental and undifferentiated mesenchymal cells.
only when malignant changes are suspected. With von
Differentiating a rhabdomyoma from a rhabdomyosarcoma
Recklinghausen’s disease, there is a 5–15% risk of malignant
may be difficult.
degeneration. This seems especially true for deeply located
lesions (Maceri and Saxon, 1984).
Treatment
Traumatic Neuroma Management of a rhabdomyoma consists of surgical

removal.
A traumatic neuroma is a reactive hyperplasia caused by
injury of a nerve.
Leiomyoma
Clinical features A leiomyoma is a benign neoplasm composed of smooth
Traumatic neuromas may occur anywhere in the oral cavity. muscle cells. The source of a smooth muscle tumor in the
Occurrence in the oropharynx is exceptional. The lesion usu- oral cavity is believed to be the walls of blood vessels or
ally manifests itself as a small submucosal nodule that may undifferentiated mesenchymal cells. Occurrence of a leio-
be painful at palpation. No characteristic clinical features myoma in the oral cavity is rare. In two reviews of the
appear. literature (Natiella et al, 1982; Praal et al, 1982) approxi-
mately 80 oral leiomyomas were collected.
Histopathology
Clinical features
Histologically, masses of irregularly arranged nerve fibers
and Schwann cells are seen to spread diffusely throughout The majority of reported leiomyomas of the oral cavity and
the tissue, mimicking to some extent the picture of a neu- oropharynx were small, circumscribed, and asymptomatic
rofibroma. swellings, covered with an apparently intact mucosa. They
were either single or multiple.
Treatment
Management consists of conservative surgical removal, if Histopathologic features
possible, followed by coagulation of the adherent nerve. A leiomyoma is composed of whorls of smooth muscle cells.
Recurrence is rare. The diagnosis of leiomyoma can be difficult to make just
from light microscopic examination. The tumor should be
Rhabdomyoma differentiated from fibromatosis and schwannoma on the
one hand and leiomyosarcoma on the other hand.
A rhabdomyoma is a benign neoplasm of striated muscle.
It is an exceedingly uncommon tumor. Management
Clinical features Management consists of surgical removal with rare
recurrence.
The male-to-female ratio is more than 2:1. The mean age of
patients with a rhabdomyoma is about 40 years. Although
extracardiac rhabdomyomas show a preference for the
Hemangiomas and Vascular Malformations
head and neck, occurrence in the oral cavity and orophar-
ynx is rare. The floor of the mouth is the most common site. Congenital vascular anomalies have been and remain
Multifocal appearance is exceptional (Schlosnagle et al, poorly understood. Since 1982, hemangiomas and vascular
1983). The clinical presentation is a submucosal swelling malformations have been recognized as distinct entities that
without any specific signs or symptoms. exhibit unique characteristics and demand appropriately
357
tailored treatment plans. However, ‘hemangioma’ still con- Treatment

tinues to be used as a clinical and pathological description
The natural history of hemangiomas should influence the
of different types of vascular anomalies.
timing and type of intervention. Benign neglect is often
advocated. A useful approach to the management of hem-
History angiomas can be based on the following:
Before the 1980s, the terminology that was used to describe ❍ Stage of the lesion
vascular anomalies was confusing and ambiguous. The ❍ Type of lesion
descriptive terminology used in the past (port-wine stain, ❍ Management of residual deformity.
strawberry hemangioma, salmon patch) conjure up visual
approximation to the lesions but have no correlation with In general, life-threatening and sight-threatening heman-
the biological behavior or natural history of these lesions. giomas should be dealt with, regardless of the stage of the
Mulliken and Glowacki introduced a simple classification lesion. Active intervention should be considered in all dis-
in 1982 that was based on the clinical, histochemical, and figuring hemangiomas, but each case should be managed
cellular criteria to distinguish between the various vascu- on its merits after careful discussion and counseling, to pre-
lar anomalies. vent potential psychosocial trauma and cosmetic deformity.
Ethunandan and Mellor stated that prednisolone is the
first-line drug of choice for the treatment of life- or sight-
Hemangioma threatening hemangiomas.
Clinical features
Vascular Malformations
This is the most common ‘tumor’ in white infants, and the
head and neck region is the most commonly involved site These are errors of morphogenesis that are populated by
(60%). Most lesions are solitary (80%) and girls are more stable mature vascular endothelium.
affected than boys (3:1). Multiple cutaneous lesions (three
or more) are often associated with visceral involvement. Clinical features
Facial hemangiomas have a predilection for segmental
distribution and for regions of embryological fusion Both sexes are equally affected. Vascular malformations
(Figure 24A–C). Hemangiomas usually appear soon after are always present at birth (though some may not be
birth, typically proliferate during the 1st year of life and apparent until a later stage) and in contrast to hemangio-
then involute during the childhood years (up to 12 years). mas, these never proliferate or involute. Instead, these
expand slowly and relentlessly throughout life, in pace
with the growth of the patient. Trauma, puberty, and preg-
Classification nancy can cause accelerated growth. Unlike hemangio-
Superficial hemangiomas originate from the papillary mas, vascular malformations are associated with skeletal
dermis and present as bright red macular or papular abnormalities in up to 35% of cases. Capillary malforma-
masses. tions may be associated with Sturge–Weber and Klippel–
Deep hemangiomas originate from the reticular dermis Trenaunay syndromes.
or subcutaneous tissues and appear as bluish or relatively
colorless masses. Classification
Compound hemangiomas have superficial and deep com-
ponents and were previously called capillary cavernous These lesions are subclassified according to the predomi-
hemangiomas. The current classification of hemangiomas nant type of vessel and characteristics of flow (Box 2).
and vascular malformations is summarized in Table 9. Waner et al graded these lesions according to the degree of
ectasia of the vessels into grades I–IV which correlate well
with the clinical features and outcome of treatment. The
Histopathology
degree of ectasia increases with age and the clinical features
The histological features are dependent on the stage of the depend on the depth and size of the lesion. The lesions are
lesion. usually soft, compressible, and enlarge in size when venous
Proliferative phase: The lesion is highly cellular and con- pressure is increased.
tains plump proliferating endothelial cells and pericytes, with
a high mitotic activity and numerous mast cells. Vascular Histopathology
channels are not prominent (Figure 24D).
Venous malformations are characterized by an abnormal
Involutive phase: The endothelial cells are flattened, the collection of veins, which do not have any demonstrable
cell turnover is normal and there are few mast cells. mitotic activity in endothelial or pericyte cells. Capillary
358
Figure 24
A B
C D
Proliferating
capillaries
Epithelium
Extravasated
red blood
cells
(A, B, C) Clinical photograph of hemangioma. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
(D) Histopathological section of hemangioma. Courtesy: Department of Oral Pathology and Microbiology, MCODS, Mangalore
malformation with cobblestone appearance in an adult lack later and are more likely to expand slowly. In general,
a uniform smooth muscle layer. intervention should be planned as soon as the diagnosis is
made and timely intervention will not only prevent compli-
cations, but also allow the extent of resection to be consid-
Capillary Malformations erably reduced. The decision to intervene should be based
Capillary malformations never regress spontaneously. on the age, location, flow characteristics, morbidity of the
They often darken and thicken with age and the rate of treatment, and the risks of an untreated lesion.
progression is probably related to the degree of absence of
the autonomic nerves in addition to other factors such as
hormonal influences and trauma. Troilius and Wrangsjo Lymphatic Malformations
in their recent study have emphasized the psychological These lesions are the most difficult to eradicate. Microcystic
aspects of the birthmark and have suggested earlier lesions in particular are diffuse, do not respect tissues planes,
intervention to counteract the undesirable psychosocial and it is difficult to distinguish involved tissue from normal
effects. tissue.
Macrocystic lesions, on the other hand, are more local-
ized and respect tissue planes and are more easily excised.
Arteriovenous Malformations
Hancock advocated these options for treatment which
These lesions show slow but relentless growth which is the included laser ablation, excision, and sclerotherapy. Carbon
norm and high-grade lesions present earlier in life and dioxide laser should be reserved for superficial mucosal
expand more rapidly, whereas low-grade lesions present lesions. Because fluid-filled vesicles are almost always
359
Current classification of hemangioma and vascular Histopathology

malformations
Histologically, a fibroma is an unencapsulated, solid, nod-
1. Hemangiomas ular mass of dense and sometimes hyalinized fibrous con-
a. Superficial (capillary hemangioma) nective tissue that is often arranged in haphazard fascicles.
b. Deep (cavernous hemangioma) A mild chronic inflammatory infiltrate may be present. The
c. Compound (capillary cavernous hemangioma) surface epithelium may be hyperkeratotic, either hyper-
2. Vascular malformations
plastic or atrophic, and it may be ulcerated (Figure 25C).
a. Simple lesions (low-flow lesions)
i. Capillary malformation (capillary hemangioma, port-wine
stain)
ii. Venous malformation (cavernous hemangioma) The clinical differential diagnosis of a fibroma includes giant
iii. Lymphatic malformation (lymphangioma, cystic hygroma) cell fibroma, neurofibroma, peripheral giant cell granu-
b. High-flow lesions
loma, mucocele, and benign and malignant salivary gland
i. Arterial malformation
tumors.
c. Combined lesions
i. Arteriovenous malformations
ii. Lymphovenous malformations Treatment
iii. Other combinations Conservative excisional biopsy is curative, and its findings
are diagnostic. Recurrence is possible however, if the offend-
ing irritant persists.
connected to deeper cisterns, ablation should be continued

to a suitable depth to ensure destruction of most or all of Giant Cell Fibroma
these cisterns. Recurrences are not uncommon and can be A giant cell fibroma has a distinctive histologic appearance
treated in a similar manner. that sets it apart from a conventional fibroma.
Sclerotherapy is a promising, but as yet not fully evalu-
ated option. It can be used either as definitive treatment or Clinical features
for palliation when the morbidity of operation outweighs
the benefits. Ogita and Tsuto tried OK-432 (lyophilized It appears as an asymptomatic sessile or pedunculated nodule
Streptococcus pyogenes treated with benzyl penicillin) and that is smaller than 1 cm in diameter. Often, it has a bosselated
has been found to be successful in treating macrocystic or somewhat papillary surface. Most cases are diagnosed in
lesions, with the advantage of avoiding a scar. persons aged 10–30 years. No sex predilection has been
reported. The most common sites are the mandibular gin-
giva, followed by the maxillary gingiva, the tongue, and the
Fibroma palate.
The fibroma, also referred to as irritation fibroma, is by far Histopathology

the most common of the oral fibrous tumor-like growths.
While the terminology implies a benign neoplasm, most if Microscopically, a giant cell fibroma is an unencapsulated
not all fibromas represent reactive focal fibrous hyperplasia mass of fibrous connective tissue that contains numerous
due to trauma or local irritation. Although the term ‘focal characteristic large, plump, spindle-shaped and stellate
fibrous hyperplasia’ more accurately describes the clinical fibroblasts, some of which are multinucleated. These cells
appearance and pathogenesis of this entity, it is not com- are easily observed in the peripheral areas of the lesion,
monly used. while the more central areas contain typical fusiform
fibroblasts. The surface epithelium is often corrugated and
atrophic.
Clinical features
A fibroma may occur at any oral site, but it is seen most Differential diagnosis
often on the buccal mucosa along the plane of occlusion of The clinical differential diagnosis includes squamous papil-
the maxillary and mandibular teeth. It is a round-to-ovoid, loma, irritation fibroma, pyogenic granuloma, and periph-
asymptomatic, smooth-surfaced, and firm sessile or pedun- eral giant cell granuloma.
culated mass (Figure 25A, B). The diameter may vary from
1 mm to 2 cm. The surface may be hyperkeratotic or ulcer-
Treatment
ated due to repeated trauma. Fibromas are most often
observed in adults, but they may occur in individuals of Conservative excisional biopsy is curative, and its findings
any age and either sex. are diagnostic. Recurrence is rare.
360
Figure 25
A B
C D
Epithelium
(stretched)
Bundles of
collagen fibers
(A, B) Irritational fibroma on the buccal mucosa. Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore. (C) Histopathological picture of fibroma. Courtesy: Department of Oral Pathology and
Fibromatoses Histopathology
The fibromatoses represent a group of infiltrating fibrous Microscopically, fibromatosis is characterized by a poorly
proliferations with a biologic behavior and microscopic delineated, infiltrating cellular proliferation of mature
appearance intermediate between those of benign fibrous spindle cells arranged in streaming and interlacing fasci-
lesions and fibrosarcomas. In the head and neck region, cles. Collagen production is usually prominent. Infiltration
these are sometimes referred to as juvenile or aggressive of the adjacent structures is common at the periphery, but
juvenile fibromatoses. cellular atypia is not present.
Clinical features Differential diagnosis

Patients of any age may be affected, but three-quarters of The differential diagnosis of a soft tissue fibromatosis is
all cases are diagnosed when the patient is younger than myofibroma and rhabdomyosarcoma.
10 years. No significant sex predilection is apparent. The
most frequent site of occurrence is the soft tissues adjacent
Treatment
to the mandible. Intraoral presentations are rare, but they
most often involve the tongue or buccal mucosa. Lesions Treatment consists of wide excision. The reported recurrence
appear as firm, painless, poorly demarcated masses with a rate of 24% for oral fibromatosis is considerably lower
variable growth rate. They are locally aggressive and often than the 50–70% rate reported for fibromatoses of the entire
cause resorption of the underlying bone when present. head and neck region.
361
Table 9 Typical radiographic features Table 10 Malignant odontogenic tumors: WHO classification
(1992)
Typical radiographic feature Condition
Odontogenic carcinomas Odontogenic sarcomas
Tennis racket appearance Odontogenic myxoma
Malignant ameloblastoma Ameloblastic fibrosarcoma
Soap bubble appearance Ameloblastoma, aneurysmal bone
(ameloblastic sarcoma)
cyst, central hemangioma
Primary intraosseous Ameloblastic fibro-dentinosarcoma
Honeycomb appearance CEOC, Od. myxoma, CGCG, central
carcinoma Ameloblastic fibro-odontosarcoma
hemangioma
Malignant variants of Odontogenic carcinosarcoma
Driven snow appearance CEOC
other odontogenic
Sunburst appearance Osteoblastoma, osteosarcoma epithelial tumors
Sunray appearance Osteosarcoma, central hemangioma,
Malignant changes in
osteoblastoma
odontogenic cysts
Ground glass appearance FD, Paget’s, hyperparathyroidism,
ossifying fibroma
Pepper pot appearance Hyperparathyroidism
Eggshell appearance Ameloblastoma
MALIGNANT ODONTOGENIC TUMORS
Onion peel appearance Garre’s osteomyelitis
CEOC: Calcifying epithelial odontogenic cyst; CGCG: Central giant cell Odontogenesis or tooth formation depends on the various
granuloma; FD: Fibrous dysplasia. epithelial mesenchymal interactions. Thus, both epithelial
and mesenchymal tissues may form neoplastic lesions,
either purely epithelial or mesenchymal or showing a mix-
Myofibroblastoma ture of both. To be regarded as ‘odontogenic’, the tumor
Myofibroblasts are spindle-shaped cells with features of must arise in the gingiva or jaws. With their neoplastic
both fibroblasts and smooth muscle cells. Myofibroblasts potential, apart from forming benign odontogenic tumors,
have been identified in lesions other than myofibromas, but they can also form malignant odontogenic tumors.
when they are the predominant cell type in a tumor, the Malignant odontogenic tumors are very rare jaw enti-
terms myofibroma (if solitary) or myofibromatosis (if mul- ties comprising only about 4% of all odontogenic tumors.
ticentric) are applied. Gradual accumulation of single cases can only provide some
information about their histomorphology, clinical appear-
Clinical features ances and behavior. If they arise from epithelium they are
odontogenic carcinomas and if from mesenchyme they are
Tumors of myofibroblasts may occur in either sex and in odontogenic sarcomas or they can be of mixed type.
patients of all ages, with a mean age of 26.6 years. Solitary
myofibromas have a head and neck predilection, with the Classification
mandible being the most common site of occurrence. The
most common oral soft tissue sites are the tongue, lips, and Till date, there is no well-accepted classification of these
buccal mucosa. tumors most of which are pathologic curiosities. Nonetheless
their importance to the unfortunate individuals so afflicted
Radiographic features is not to be underestimated. In the recent years several
classifications have been suggested by several authors.
Intraosseous jaw lesions most often manifest as well-defined The most widely used being the WHO classification of
unilocular or multilocular radiolucencies. 1992 given by Pindborg (Table 10).
The classification has evolved with time as new articles
have been published over the years. The latest is the WHO
The clinical differential diagnosis for oral myofibroma classification of 2004 given by Reichert and Philipsen
includes irritation fibroma, fibromatosis, peripheral giant (Table 11).
cell fibroma, neurofibroma, leiomyoma, and benign and
malignant neoplasms of the minor salivary glands. Etiopathogenesis
The etiopathogenesis for odontogenic carcinomas is
Treatment
unknown; studies to find out more are difficult because of
Treatment for oral myofibromas is conservative excision. the rarity of these groups of lesions. Generally, these tumors
The recurrence rate is low, and spontaneous regression has arise de novo within the jaw bones, from epithelium con-
been reported. tained within cyst linings, or from malignant transformation
362
Table 11 Malignant odontogenic tumors: WHO classification ODONTOGENIC CARCINOMAS

(2004)
Terminology used to describe diverse odontogenic carcino-
Odontogenic carcinomas Odontogenic sarcomas
mas is varied and confusing. We will discuss the important
1. Metastasizing malignant 1. Ameloblastic fibrosarcoma lesions.
ameloblastoma (ameloblastic sarcoma)
2. Ameloblastic carcinoma 2. Ameloblastic fibro-
a. Primary intraosseous carcinoma dentinosarcoma Malignant Ameloblastoma (Metastasizing)
b. Secondary (dedifferentiated) a. Ameloblastic fibro-
intraosseous odontosarcoma It is defined as an ameloblastoma that metastasizes in
c. Secondary (dedifferentiated) spite of an innocuous histologic appearance. This implies
extraosseous that the primary tumor shows no feature different from
3. Primary intraosseous squamous cell the tumors that do not metastasize. Thus, this can only be
carcinoma (PIOSCC) a retrospective diagnosis, after the occurrence of metastatic
a. PIOSCC solid type deposits. Therefore, it is the clinical behavior and not the
b. PIOSCC derived from histology of the lesion that justifies the diagnosis of a
odontogenic cysts malignant ameloblastoma. WHO defines this as a neoplasm
c. PIOSCC derived from keratinizing in which the pattern of an ameloblastoma is combined with
cystic odontogenic tumors cytological features of malignancy, a definition not based
4. Clear-cell odontogenic carcinoma on behavior but histology.
5. Ghost cell odontogenic carcinoma Both the primary and metastatic foci are characterized
histologically by same benign tumor islands, which lack
any feature of malignancy. The metastatic foci show typi-
cal ameloblastic differentiation of the peripheral amelo-
of benign odontogenic tumors. Odontogenic carcinomas blasts with palisading and reverse polarization of nuclei.
arise from the epithelial components of the tooth germ. In The outer palisaded stratum of ameloblasts surrounds a
adults, the epithelial remnants of odontogenesis consist of zone of stellate reticulum which may show foci of squa-
the reduced enamel epithelium that surrounds the crowns mous metaplasia. No pleomorphism or mitotic figures are
of impacted teeth for as long as they remain trapped and seen. The average time from initial treatment of the pri-
unerupted within the jaws, the rests of Malassez which are mary tumor to the appearance of metastasis is 9 years.
located throughout the periodontal ligament and within These metastases generally only arises after many surgical
edentulous areas of the alveolar bone subsequent to tooth attempts at treatment of the original lesion and are often
loss and remnants of the dental lamina located in the gin- isolated pulmonary metastases that can sometimes be
giva. Odontogenic cysts of various types may also serve as treated surgically. Some also believe that metastases may
a tissue site for malignant transformation. be caused by aspiration or implantation at the time of
surgery. Metastatic deposits of this lesion are mostly seen
Diagnosis in lungs, lymph nodes, brain, viscera, skin and bone.
Diagnosis of these lesions may be complicated for two The treatment of choice of the primary tumor and recur-
main reasons: first, due to their low frequency, which causes rences is excision. Radiotherapy too has been tried but its
the information about their clinical and histopathological value is questionable. Overall, very few cases have been
features be scant, and secondly due to the similar histopatho- reported for a definitive treatment protocol to be evolved.
logical pictures with benign odontogenic tumors. The diag- Behaviorally, malignant ameloblastoma may progress to
nosis is based upon the unique histopathological features death.
of odontogenic carcinomas. In general, all tumors classified
as odontogenic carcinomas exhibit typical microscopic fea-
Ameloblastic Carcinoma
tures of malignancy with the exception of the malignant
(metastasizing) ameloblastoma and the clear cell odontogenic It is a malignant epithelial proliferation that is associated
carcinoma. Behaviorally, all the entities in this group have with an ameloblastoma (carcinoma ex ameloblastoma) or
the potential for either nodal or distant metastases. histologically it resembles an ameloblastoma (de novo
Odontogenic sarcomas are thought to arise from the ameloblastic carcinoma). It is an aggressive neoplasm that
ectomesenchymal tissue. They either arise de novo or from is locally invasive (extending out of bone to involve the
pre-existing ameloblastic fibroma. Odontogenic carcino- infratemporal fossa, parapharyngeal space, the masticator
mas are much more frequent than odontogenic sarcomas. space, or cervical soft tissue) and can spread to regional
A rare lesion having a mixed picture is termed as odonto- lymph nodes or distant sites, such as lungs and bones and
genic carcinosarcoma. myocardium.
363
The term was first used by Shafer in 1974 to denote carcinoma. These arise de novo or as dedifferentiated carci-
ameloblastoma in which there had been histological trans- nomas from a pre-existing benign peripheral ameloblastoma.
formation. Corio used the term for ameloblastoma that
shows cytological features of malignancy but is otherwise
Primary Intraosseous Squamous Cell Carcinoma
recognizable as an ameloblastoma and may later metasta-
size. Ameloblastic carcinoma may arise de novo or from a Primary intraosseous squamous cell carcinoma (PIOC) is a
pre-existent benign odontogenic lesion such as an amelo- squamous cell carcinoma that occurs within the jaw bone.
blastoma or an odontogenic cyst. It is called ‘primary’ because it is not a secondary deposit.
Metastasis from a distant primary to the jaw must be excluded.
Carcinoma ex ameloblastoma A carcinoma directly
The WHO defines primary intraosseous carcinoma as a
contiguous with an ameloblastoma is appropriately termed
squamous cell carcinoma arising within the jaw, having no
as a ‘carcinoma arising from an ameloblastoma’ (carcinoma
initial connection with the oral mucosa, and presumably
ex ameloblastoma, carcinoma in ameloblastoma). The car-
developing from residues of the odontogenic epithelium.
cinoma arises when an ameloblastoma undergoes ‘dedif-
These are typical invasive squamous cell carcinomas and
ferentiation’ (i.e. when a less-differentiated proliferative
range from well to poorly differentiated varieties. The WHO
clone arises from an ameloblastoma). This aggressive clone
extends the definition of PIOC to include those lesions having
overgrows the ameloblastoma and becomes the dominant
a distinctively odontogenic pattern with basal-type cells
component.
forming alveoli or arranged in a plexiform pattern with
De novo ameloblastic carcinoma If a carcinoma lacks a palisading of the peripheral cells.
component of conventional ameloblastoma, its unequivo-
cal categorization as an ameloblastic carcinoma is consid-
Solid PIOC
erably less secure. Its diagnosis is based on subjective
interpretation. Histologically, ameloblastic carcinoma is These occur mainly in the posterior mandible, are more
characterized by epithelial nests and strands similar to the often seen in males, may metastasize to regional lymph
plexiform or follicular patterns. The cells exhibit cytologi- nodes, and have a poor prognosis. It occurs commonly in
cal atypia and mitotic activity. Furthermore, areas of squa- elderly patients (mean age 50 years). These tend to occur
mous cell carcinoma may be seen. If the lesions are entirely in the mandibular body where they may compress the
composed of this component and lack resemblance to inferior alveolar nerve causing pain and/or paresthesia.
ameloblastoma, they should be diagnosed as primary Loosening of teeth and mandibular expansions are signs.
intraosseous squamous cell carcinoma. Radiographically, these may be well-circumscribed as well
as more diffuse with irregular moth-eaten borders. The
tumors metastasize regionally and distantly and show a
Clinical features
30–40% 5-year survival.
It occurs equally in both the sexes. It causes jaw swelling,
Cystic PIOC (PIOC arising in an odontogenic cyst, PIOC
frequently causing pain and rapid growth. Mandible is
ex odontogenic cyst) Cystic PIOC is a squamous cell car-
more frequently involved. Radiographically, an ill-defined
cinoma that demonstrates a cystic component with a
area of radiolucency is seen; sometimes showing the
lumen that contains fluid or keratin and a lining of strati-
presence of focal radiopacities. Root resorption, perforated
fied squamous epithelium that exhibits cytological atypia.
buccal and lingual cortical plates of jaw and extension
Primary intraosseous carcinomas may develop from a still
into the soft tissues illustrate the destructive potential.
recognizable precursor lesion such as the epithelial lining
Treatment could be aggressive surgery, but this may be
of an odontogenic cyst. The linings of residual apical peri-
followed by recurrences and extensive local spread. Radio-
odontal cyst, OKC and dentigerous cyst have shown malig-
therapy has not shown effective results, but chemotherapy
nant transformation. Moreover, reduced enamel epithelium
with paclitaxel and carboplatin and oral cyclophospha-
has been documented as tissue of origin in cases in which
mide has some effect. Death has been reported because of
an impacted tooth was presented within the tumor.
extensive local recurrence involving base of the skull and
Co-occurrence of squamous odontogenic tumor and ame-
cranial cavity. Prognosis is poor if the tumor has already
loblastomas has been reported. Most carcinomas arising
metastasized.
from the cyst linings are well or moderately differentiated.
Biomarker studies for proliferation and malignant geno-
type (aneuploidy) have shown that ameloblastic carcino- Carcinoma ex dentigerous cyst The most common
mas have a higher proliferating cell nuclear antigen (PCNA) odontogenic cyst to show carcinomatous changes is the
index and a greater degree of aneuploidy than histologi- dentigerous cyst. Few cases that lacked rigorous evidence
cally benign ameloblastoma. of dentigerous cyst formation, but showed thickening of
A rare variant is that which arises from gingival or alve- pericoronal soft tissue that was associated with an impacted
olar mucosa epithelium termed as peripheral ameloblastic tooth, were reported as carcinoma ex dentigerous cyst.
364
Intraepithelial neoplasia that involves gingival crevicular around the tumor cells. Squamous differentiation has also
epithelium can mimic carcinoma ex dentigerous cyst his- been reported.
tologically. Most carcinomas arising in dentigerous cysts Metastases are found in lymph nodes and lungs.
occur in the mandibular molar area; however, they Metastatic disease may occur more than 5 years after ini-
occasionally are associated with impacted canine teeth. tial diagnosis. Differential diagnoses include metastatic
Usually, the carcinoma is treated by surgical resection. renal cell carcinoma, ameloblastoma with clear cells, clear
Histologically, the lesion demonstrates membranous con- cell variant of CEOT and the clear cell variant of mucoepi-
nective tissue that is lined by stratified squamous epithe- dermoid carcinoma.
lium that exhibits evidence of intraepithelial neoplasia
that is associated with an invasive well-differentiated or
moderately differentiated squamous cell carcinoma. Malignant Epithelial Odontogenic Ghost Cell
Tumor
Carcinoma ex OKC Cases have been reported to arise
from cyst lining of OKC. In most, the radiographic findings It is also called odontogenic ghost cell carcinoma (OGCC),
are those of a benign OKC and the unexpected carcinoma is ghost cell odontogenic carcinoma, aggressive epithelial
recognized following incisional biopsy or enucleation. odontogenic ghost cell tumor, dentinogenic ghost cell
They have been usually treated by resection and postop- tumor. It is an ameloblastic carcinoma that shows evi-
erative radiation therapy. dence of ghost cell keratinization. It is a tumor that com-
bines the elements of a benign calcifying odontogenic cyst
Intraosseous mucoepidermoid carcinoma (central muco-
(COC) with a malignant epithelial component. The biolog-
epidermoid carcinoma, PIOC with mucous cells) It
ical behavior of the tumor is unpredictable, characterized
arises from odontogenic epithelium or an odontogenic
by indolent growth and others by local aggressive growth,
cyst. It represents a cystic primary intraosseous carcinoma
and distant metastasis. The tumor apparently arises most
that shows squamous differentiation and mucous cells;
often from malignant transformation of a pre-existing
lesional cells focally exhibit cytoplasmic mucicarmine
benign COC. OGCC has to show both the features of a
positively.
COC-ameloblastoma-like tissue together with ghost cell
keratinization as well as solid epithelial areas showing
Clear Cell Odontogenic Carcinoma cytonuclear atypia. Both components may be distinct from
each other or admixed.
It is also known as clear cell ameloblastic carcinoma, clear
Three distinct origins are thought: occurring de novo
cell ameloblastoma, clear cell odontogenic tumor. It is a
with benign COC and a malignant epithelial component his-
low-grade carcinoma that is composed of cells that show
tologically present in the same lesion, occurring after the
uniform nuclei and clear cytoplasm. It was initially desig-
recurrence of a benign COC, or arising from odontogenic
nated as clear cell odontogenic tumor, but this lesion
tumor. The biologic behavior of a lesion cannot be predicted
behaves aggressively and metastasizes too, so it is better
from the presence of ghost cells. The tissue that surrounds
termed as clear cell odontogenic carcinoma (CCOC).
the ghost cells gives the prognosis. Ghost cells are immu-
The anterior mandible is the site of predilection but other
noreactive for amelogenin, a protein that is unique to the
jaw areas may be involved also. About 70% of females are
enamel matrix.
affected with an age range of 17–89 years. The tumor has
It is seen more in males in an age range between 13 and
a varied radiographic appearance. Often, it presents as a
72 years. About 66% of the cases occur in maxilla. It is
unilocular expansile radiolucent lesion with an indistinct
more in Asians. Radiographically, it presents as an expans-
periphery; however, some cases are multiloculated and
ile multiloculated to poorly delineated radiolucent lesion.
well-circumscribed. Confinement of the lesion to the jaws,
Lesional tissue subjectively reveals features that warrant
presence of columnar cells resembling ameloblasts, and
a malignant interpretation, including cytologic atypia,
the occasional presence of dentinoid structures prove it to
increased mitotic activity, an infiltrative growth pattern
be odontogenic in nature.
(perineural or intravascular invasion) and necrosis.
Three histopathologic patterns have emerged. The more
common pattern is a biphasic tumor characterized by
oval and linear nests of clear cells intermixed with smaller
islands of polygonal cells with eosinophilic cytoplasm. ODONTOGENIC SARCOMAS
Occasionally, these two cell types coexist in a tumor nest
yielding a ‘glomeruloid appearance’. The nuclei are oval These tumors may arise de novo or from a pre-existent ame-
and monomorphic. If pleomorphism or hyperchromatism loblastic fibroma or ameloblastic fibro-odontoma. Whether
occur, it is generally within the nuclei of the polygonal an odontogenic sarcoma displays deposition of hard dental
cells. The stroma of CCOC is distinctive. Fibroblastic cel- tissues has not shown any prognostic significance. Odon-
lularity is high and the collagen is mature, wrapping tightly togenic sarcomas arising from pre-existing ameloblastic
365
fibroma occurs at a higher mean age (33 years) those that pertaining to molecular events in the carcinogenesis of
develop de novo (22.9 years). The main clinical problem of odontogenic epithelium and mesenchyme, thereby shedding
odontogenic sarcoma is relentless local growth. more light on the origin, pathogenesis, biologic behavior,
treatment and prognosis of malignant odontogenic tumors.
Ameloblastic Fibrosarcoma
It is also known as odontogenic sarcoma, ameloblastic sar- EPITHELIAL MALIGNANT TUMORS
coma. It is a malignant proliferation of connective tissue
cells that contains benign odontogenic epithelium that is Epidermoid Carcinoma
similar to ameloblastic fibroma. Typically, it presents in the Epidermoid carcinoma or squamous cell carcinoma is
mandible with an age range from 8 to 83 years. In the man- described in detail in Chapter 14 on Oral Cancer.
dible, posterior region is affected more. It demonstrates an
expansile radiolucency with indistinct margins, evidence of
extraosseous soft tissue extension, and sometimes, an asso- Verrucous Carcinoma
ciated impacted molar. Other radiographic appearances
include destructive/permeative, unilocular or multilocular. Verrucous carcinoma has also been referred to as snuff
Typically, the patients do not develop metastases; they dipper’s cancer and Ackerman’s tumor. Rock and Fisher in
form the group of a locally aggressive neoplasm. The sar- 1960 coined the term ‘oral florid papillomatosis’.
coma is most often treated by a wide surgical excision and Lauren Ackerman in 1947 first described this less severe
postoperative radiation therapy without elective neck dis- variant of squamous cell carcinoma associated with spit-
section. Ameloblastic fibrosarcoma has the histologic archi- tobacco chewing habit. He suggested that verrucous car-
tecture similar to an ameloblastic fibroma. cinoma needs to be considered as a distinct entity as it
Slender budding and branching epithelial cords of exhibits a characteristic morphologic appearance and spe-
bland cuboidal to columnar cells with uniform nuclei or cific clinical behavior. He noted that even extension lesions
epithelial islands that are indistinguishable from those are had excellent prognosis with proper treatment.
seen in follicular ameloblastomas are separate widely by This lesion has a predilection for mucous membranes of
hypercellular connective tissue that exhibits plump polyg- the head and neck and is most commonly found in the oral
onal to fusiform stromal cells that show mild to moderate cavity followed by the larynx. Since its earlier description,
cytologic atypia and numerous mitotic figures in a pale verrucous carcinoma has been also reported to occur in the
hypocollagenous myxoid extracellular matrix. esophagus, paranasal sinuses, nasal fossae, genital and anal
Ameloblastic fibrosarcoma can show focal evidence of mucosa, soles of feet, breast and axilla.
dentin formation or dentin and enamel formation, resulting Enriquez et al (1980), Pomatto et al (2001) and Nooshin
in the terms ameloblastic dentinosarcoma and ameloblastic Mohtasham et al (2008) reported verrucous carcinoma aris-
odontosacrcoma, respectively. The histologic differential ing from an odontogenic cyst.
diagnosis includes low grade spindled ameloblastic carci-
Predisposing and etiological factors
noma that demonstrates plump fibroblastic cells that are
associated with ameloblastomatous islands and sheets. The The exact etiology is still not understood. Chewing tobacco
distinction between the two is made by cytokeratin which is is the primary etiologic factor for these tumors. Most of these
found to be reactive by fibroblastic cells of spindled amelo- tumors originate from the site of the placement of tobacco.
blastic carcinoma. It has been suggested that opportunistic viruses such as
HPV-6 and 16, act in tandem with frank carcinogenesis to
promote development of verrucous carcinomas. Few other
Odontogenic Carcinosarcoma authors suggest that the lesions develop at sites of chronic
irritation and inflammation.
It is a tumor architecturally resembling an ameloblastic
fibroma in which both the epithelial and the connective
Clinical features
tissue components show cytologic evidence of malignancy.
For these extremely rare lesions that combine carcinoma- Verrucous carcinoma is usually seen between the 5th and
tous and sarcomatous elements and recognizable as odon- 7th decade of life and commonly in white men. It is esti-
togenic if the epithelial component resembles that of an mated that these tumors account for about 1–10% of all
ameloblastoma, the designations malignant ameloblastoma oral squamous cell carcinomas.
and fibrosarcoma or odontogenic carcinoma with sarco- In the initial stages, verrucous carcinoma appears as
matous proliferations have been used. white, translucent patches on an erythematous base. As they
The increase in the number of reported malignant odon- mature they begin to take on a cauliflower-like papilloma-
togenic tumors will help in the future to attempt studies tous growth with a superficial pebbly surface (Figure 26).
366
Figure 26 Figure 27
Parakeratin plug
Cleft lined by
parakeratin
Epithelium with broad
pushing borders
Subepithelial band of
chronic inflammatory
cell infiltrate
Histopathological picture of verrucous carcinoma.

MCODS, Mangalore

Clinical photograph of verrucous carcinoma. Courtesy:
Department of Oral Medicine and Radiology, Most of the patients suffering from verrucous carcinoma
MCODS, Mangalore have a good prognosis. The mainstay of treatment for ver-
rucous carcinoma is surgical excision. However, when the
tumor is not accessible for surgery, radiotherapy may be
The common intraoral sites that are affected are the used. Radiation therapy is planned for a 6-week period.
buccal mucosa, alveolar ridge, gingiva, floor of mouth, About 400–800 cGy is administered in fractions, 6–12 times.
tongue, tonsillar regions and the vermillion border of However, some authors report anaplastic transformation
the lip. (almost 30%) following radiotherapy.
Occasionally, local invasion into underlying soft tissues
and bone may be seen. The surface of the lesion may show Basal Cell Carcinoma
ulceration.
Rarely distant metastasis is seen. However, regional Basal cell carcinoma is also referred to as ‘rodent ulcer’. It
involvement of the lymph nodes may be observed. Nodes is the most common cutaneous malignancy, which typi-
may be enlarged and tender. cally affects the sun-exposed surfaces of the skin. It is a
The other forms of verrucous carcinoma are the Buschke– slow growing tumor. However, long-standing tumors can
Lowenstein tumor (anourologic type) and the epithelioma cause local destruction of tissues and metastasis is seldom
cuniculatum (palmoplantar type). encountered. It is estimated that less than 0.1% tumors
Buschke–Lowenstein tumor occurs as a large cauliflower- metastasize. The most common sites of metastasis are the
like tumor mass that is usually restricted to the glans lymph nodes, bones and lungs. It arises from the basal cells
penis, vagina, cervix and the perianal region; whereas epi- of the surface epidermis or external root sheath of the hair
thelioma cuniculatum is seen as ulcerated large exophytic follicle.
masses associated with sinus openings especially on the skin
overlying the first metatarsal head. Clinical features
These tumors can also occur on the toes and heel.
Basal cell carcinoma is usually seen in individuals over the
Occasionally, these tumors may bleed or produce foul
4th decade of life. Men are believed to be affected twice as
smelling discharge. Patients may complain of difficulty in
commonly as women as they are more frequently exposed
walking.
to sun. However, this may not be true all the time. It has
been noticed that fair complexioned individuals are rela-
Histologic features
tively more prone to develop basal cell carcinoma com-
Histologically, verrucous carcinoma shows hyperkeratosis, pared to dark complexioned individuals.
parakeratosis and acanthosis in the superficial portions. Basal cell carcinoma can have various clinical appear-
The surface is papillary with a thick parakeratinized cover- ances. Some of the relatively common varieties are nod-
ing that extends into deep cleft-like interpapillary spaces. uloulcerative type (most common variety), superficial
The rete ridges are broad and bulbous, that tend to ‘push’ spreading type, pigmented, morphea-form (sclerosing) and
into the underlying connective tissue (Figure 27). the cystic type.
367
The ulceronodular type of basal cell carcinoma, in the Management

initial stages, appears as a large non-tender papule which
Neville et al (2007) studied the efficacy of 5% imiquimod
slowly enlarges and exhibits a central depression. Over a
(commercially available in India as Imiquad 5% cream.
period of time the central depressed area reveals ulceration
Imiquimod is a keratolytic) in the treatment of nodular
associated with some bleeding and crusting. The patho-
basal cell carcinoma after initial treatment with curettage.
gnomonic feature of basal cell carcinoma is a waxy, trans-
In their study, 5% imiquimod was applied locally once a
lucent, or pearly appearing ulcer with a raised pale border.
day, 5 times per week for 6 weeks, following curettage.
Telangiectasias are common.
They concluded that imiquimod cream was an effective
The pigmented form resembles melanomas. They appear
treatment modality.
as bluish-black or brown colored macules. However, unlike
Gross et al (2007) studied the potential of 5%
melanocytic nevus these lesions are not necessarily uni-
5-fluorouracil cream for the treatment of small superficial
formly pigmented.
basal cell carcinoma. They used 5% 5-fluorouracil cream
The cystic variety of basal cell carcinoma is rare. This
twice daily for up to 12 weeks. The results showed that the
form of basal cell carcinoma appears as a bluish to gray col-
histologic cure rate was 90% and the mean time to clinical
ored, mucin-filled cyst-like lesions.
cure was 10.5 weeks.
The sclerosing type of basal cell carcinoma is uncommon.
Healsmith et al (1991) used intralesional interferon
The typical lesion mimics a scar. It appears as a white or
alpha-2b (IF-2b) for the treatment of basal cell carci-
yellow waxy sclerotic plaque. It is believed that the tumor
noma. The injected nine intralesional injections of IF-2b
cells initiate the proliferation of fibroblasts within the der-
(1.5 million units dissolved in 0.2–0.5 ml water), 3 times
mis and an increased collagen deposition (sclerosis).
per week for 3 weeks. At a 3-month follow-up, out of the
The superficial type is seen as an erythematous, well-
11 tumors they treated, six tumors had resolved both clin-
circumscribed patch or plaque. The lesion may be associated
ically and histologically, three tumors had reduced in size
with the formation of a white colored scale. These lesions
and one tumor grew larger.
may mimic lesions of psoriasis. Neville et al described the
Surgical excision for basal cell carcinoma is still the
presence of a fine elevated ‘thread-like’ border at the margins
most popular modality of treatment. It is recommended
of the lesions.
that an excision margin of 4 mm around the tumor be main-
It is estimated that the risk of developing a squamous
tained. Mohs micrographic surgery offers high cure rates for
cell carcinoma is increased slightly after a basal cell carci-
basal cell carcinoma (5-year cure rate of 99% for primary
noma, with a 6% risk at 3 years. Patients are at increased
tumors and up to 95% for recurrent basal cell carcinoma).
risk of developing malignant melanomas.
During this surgical technique, serial frozen sections are
examined histologically until all margins are clear.
Syndromic association
Barcelos et al (2008) described Bazex–Dupré–Christol syn-
drome, which is a rare genodermatosis that is character- Malignant Melanoma (Melanocarcinoma)
ized by follicular atrophoderma, multiple milia, congenital Malignant melanoma is a malignant neoplasm, with its
hypotrichosis, hypohidrosis and basal cell malformations origin in the neural crest cells. It was first described by
that include nevoid basal cell carcinomas. Weber in 1859. Lucke in 1869, recognized it as a distinct
The other commonly associated syndrome is Gorlin–Goltz clinical entity and named it as ‘melanotic sarcoma’.
syndrome, which is characterized by basal cell epithelioma, It is estimated that oral melanomas represent about
jaw cyst and bifid ribs. 1–2% of all oral malignancies. It accounts for about 3–5%
of all malignancies affecting the skin.
Apart from the oral mucosal involvement, melanomas
The basaloid appearance of the epithelial islands is the most can be seen affecting the nasal, vulval and anorectal mucosa.
striking histopathological features of basal cell carcinoma. It is believed that tobacco smoke may play a role in the
The cells exhibit increased nuclear cytoplasmic ratio. Cells development of melanoma in the palate. Other possible risk
show peripheral palisading (they are arranged perpendicu- factors include fair complexion, light hair, excessive expo-
lar to the basement membrane). sure to sunlight, exposure to formaldehyde, family history
The tumor has a characteristic invasive pattern with the of malignant melanoma and the history of dysplastic nevi.
formation of large islands. The cells within the core of the
epithelial islands have non-discrete cytoplasmic borders
Clinical features
and mimic syncytium. They do not have prominent nucle-
oli and lack intercellular bridges. The stroma shows vary- Melanoma is usually seen in the 4th to 7th decades of life.
ing amounts of collagen deposition with abundant mucin. It is seldom seen in individuals below 20 years of age.
368
❍ Color variation: Pigmentation is not uniform and may

Figure 28
display shades of tan, brown or black. White, reddish
or blue discoloration is of particular concern.
❍ Diameter: A diameter more than 6 mm is characteristic
of melanoma, although some may have smaller diam-
eters. Any growth in a nevus warrants an evaluation.
❍ Evolving: Changes in the lesion over time are charac-
teristic. This factor is critical for nodular or amelanotic
(non-pigmented) melanoma, which may not exhibit
the classic criteria listed above.
Types of melanoma
Based on the clinical and pathological features, melanomas
are broadly categorized into four types:
❍ Superficial spreading
❍ Nodular
❍ Lentigo maligna
Clinical photograph of malignant melanoma. Courtesy: ❍ Acral lentiginous.
MCODS, Mangalore Table 12 summarizes the important features of these types
of melanomas.
Self-assessment tool for diagnosis of melanoma

Males are usually more commonly affected than females Many authors believe that the ‘ugly duckling’ sign can be
(approximately 3:1.8). It has also been seen that melano- used as an effective self-examination tool. Individuals
mas are almost 5 times more common in whites than the are asked to compare the gross appearance of moles on
negroes. Melanomas are more common among Africans, their body. Usually all the moles will have similar topo-
Japanese and Hispanics. graphical appearance except for the ‘ugly ducking’ or the
The common primary sites of involvement are the hard malignant melanoma, which will appear different from
palate and maxillary gingiva. Other less commonly affected other moles.
sites are the buccal mucosa, lips, tongue, floor of the mouth Scope et al (2008) assessed the effectiveness of the ugly
and the mandible (Figure 28). duckling sign as a potential screening tool for malignant
Clinically the lesion has varied presentations, ranging melanoma. They found that all the melanomas were gen-
from a dark blue-black to brown and to an amelanotic erally apparent as ugly ducklings.
variety that may appear pink. Some authors believe that Differential diagnosis of melanoma includes oral mela-
there is a deficiency of tyrosine and an enzyme required notic macule, smoker’s melanosis, amalgam tattoo, mela-
for melanin production. Others believe that this enzyme noplakia, Addison’s disease and Peutz–Jeghers syndrome.
system is intact and can produce melanin but the quan-
tity is insufficient to be seen with histologic methods.
Histologic features
The latter explanation seems convincing because electron
microscopy does reveal the presence of melanosomes in Histologically a high concentration of melanocytes may be
all amelanotic melanomas reported till date. Amelanotic seen in the biopsy specimen. These atypical melanocytes
variants have a worse prognosis because of delayed recog- are larger, exhibit nuclear pleomorphism and hyperchro-
nition and subsequent treatment. matism in the epithelial and connective tissue junction
Melanomas may also be seen as a nodule which may (Figure 29).
exhibit ulceration. Based on the extent of penetration through the dermis
The indicative clinical signs for malignant melanoma to the subcutaneous fat, Clark suggested five levels of
are referred to as the ABCDE warning signs. microstaging:
❍ Asymmetry: The shape of the lesion is not the same on 1. Level I: Melanoma lies in the epidermis above an
both sides. intact basal lamina.
❍ Border irregularity: The edges are ragged, notched or 2. Level II: Melanoma cells that have compromised the
blurred. basal lamina, infiltrating the papillary dermis.
369
Table 12 Melanoma: characteristic features
Type of melanoma Characteristic features

Superficial spreading Most common type (comprises 70% of skin lesions)
Usually arises from a pigmented dysplastic nevus
Ulceration or varied colorations (blue, black, gray, pink) are seen
Common on the head, neck, and trunk of males and the lower extremities of females
Nodular melanomas Represent approximately 10–15% of cutaneous melanomas (almost 30% of these occur in the head and neck region)
Common on the trunk of males
Usually symmetrical and uniform
Dark brown or black in color
The radial growth phase may not be evident (due to its high risk)
Amelanotic melanomas represent approximately 5% of all nodular melanomas
Lentigo maligna Almost 10% of the cutaneous melanomas are lentigo maligna melanoma
melanoma It originates from a melanoma in situ called lentigo maligna or Hutchinson’s freckle
The Hutchinson’s freckle is generally in a radial growth phase for almost 12–15 years. At this time, the lesion appears as
a relatively flat brown, black and occasionally grayish-white macule
However, over a period of time this radial phase of growth turns into a phase of vertical growth indicating an invasion
into deeper planes of tissue. During this phase the flat macule exhibits nodular changes
At this stage the lesion is termed lentigo maligna melanoma
Acral lentiginous It is the most commonly occurring oral melanoma
melanoma In the initial stages it may appear as a macule, which slowly shows nodular appearance
It commonly affects the oral mucous membranes, palms and soles
5. Level V: Melanoma extending into the subcutaneous

Figure 29 fat.

Melanoma is best managed with surgical excision of the
lesion with wide margins of at least 2.5 cm. However,
widespread disseminated metastatic diseases can be man-
aged with chemotherapy and radiotherapy. Some authors
have reported good results with interferon.
It is believed that the prognosis of oral melanomas are
poorer than skin lesions. The prognosis of the condition
depends on the extent of invasion. When the invasion
limited to the epidermis, the condition is almost 100% cur-
able. However, when the lesions are less than 1 mm in
thickness, the 5-year survival rate is about 95%, but when
the lesions are more than 4 mm in thickness, the 5-year
survival rate falls to 45%. The 5-year survival rate for oral
melanomas is about 10%.
Histopathological picture of melanoma. Courtesy: CONNECTIVE TISSUE MALIGNANT TUMORS

Department of Oral Pathology and Microbiology,
MCODS, Mangalore
Fibrous Tissue Origin
Fibrosarcoma
3. Level III: Melanoma involving the papillary dermis, Fibrosarcoma is a malignant neoplasm of the fibroblas-
extending to the papillary-reticular dermis interface. tic origin. Fibrosarcoma may occur anywhere in the body.
4. Level IV: Melanoma extending into the reticular Extremities are the most commonly affected. However,
dermis. only about 10% involve the head and neck region.
370
They can present as a soft tissue mass or as a primary

Figure 30
(central and peripheral) or secondary bone tumor.
The primary fibrosarcoma produces variable amounts
of collagen. The secondary fibrosarcoma of bone arises from
a pre-existing lesion or as a complication of radiotherapy
involving bone or soft tissue. It is believed that the sec-
ondary fibrosarcoma is very aggressive. Long fascicle
Patients may complain of bone pain and swelling when
the tumor involves the bone. Larger tumors may lead to Short fascicle
destruction of the bone architecture and pathological
fracture. Fibrosarcomas involving the soft tissues are sel- Interlacing
dom painful. They are usually seen deep to the muscular fascicles
fascia.
They affect males and females equally and commonly
seen in the 2nd to 4th decades of life. However, reports of
fibrosarcoma involving the tongue, gingiva and jaw bones Histopathological picture of fibrosarcoma. Courtesy:
have been reported in young children. The common sites Department of Oral Pathology and Microbiology,
that are involved include the paranasal sinuses, lip, palate MCODS, Mangalore
and the periosteum of the maxilla or mandible. The man-
dibular premolar and molar region is the most common site.
Soft tissue involvement may produce a locally invasive
swelling that may show secondary changes such as hem- muscular immunomarkers help to differentiate fibrosar-
orrhage and ulceration. Occasionally, patients may report coma from other tumors.
of altered sensation if the tumor involves peripheral
nerves. Management
Radiographic features Radical surgical removal of the tumor is the treatment of

choice. Inaccessible tumors can be palliated with chemo-
Fibrosarcomas are evident as a solitary radiolucency with therapy and radiotherapy. It is estimated that the 5-year
ragged or ill-defined borders. Peripheral lesions may show survival rate is approximately 30%.
saucerization of the surface underlying bone. In many
individuals the normal bone surrounding the tumor may
show sclerosis. Malignant Fibrous Histiocytoma
The cortices show thinning or destruction in extensive
cases. Malignant fibrous histiocytoma (MFH) is a sarcoma with
The tumor can cause destruction of the sinus, nasal both fibroblastic and histiocytic features. MFH typically
fossa or the mandibular canal based on its location. arises in the soft tissues of the extremities and retroperito-
Displacement of teeth and alveolar bone with tooth neum, the head and neck region is rarely involved. These
floating in space appearance are common. Widening of the tumors account for about 10–20% of all sarcomas. In the
periodontal ligament space is seen. However, root resorp- present times, it is considered as one of the most common
tion is seldom evident. soft tissue sarcomas of adult life. These tumors typically
Some authors have reported the presence of sunray arise in the muscles and deep fascia. However, tumors
appearance or Codman’s triangle appearance when associ- involving bone have also been reported. They can be either
ated with disruption of the periosteum. benign or malignant.
Histopathologic features Clinical features

In low-grade malignancy, spindle cells are seen arranged It is commonly seen in adult males. Superficially located
in fascicles with low to moderate cellularity in a ‘herring- tumors are generally smaller in size compared to deep
bone’ pattern. Mild degree of nuclear pleomorphism is seen. seated tumors.
However, high-grade malignant lesion shows extensive The tumors can affect any part of the body. The com-
nuclear pleomorphism and increased cellularity. Atypical mon sites of involvement include the extremities, nose,
mitosis is present (Figure 30). paranasal sinuses, orbit, larynx, meninges, lungs, vagina,
Histologically, fibrosarcoma mimics malignant fibrous ovary and submandibular glands.
histiocytoma, liposarcoma or synovial sarcoma. However, The intraoral sites commonly affected are the buccal
positive immunostaining for vimentin and negativity for mucosa, vestibule, lips and tongue. The jaws bones also
371
have reportedly been involved. The painless nodular mass Radiographic features
may vary in size from a few millimeters to few centimeters
In the head and neck region, CT image may reveal a mul-
in size.
Fibrosarcoma can be considered in the differential tilocular tumor with smooth, well-defined margins and
diagnosis. Other conditions that can be included in the heterogeneous enhancement after injection of contrast
differential diagnosis are leiomyosarcoma, angiosarcoma medium. Occasionally, internal calcification may be
and neurofibrosarcoma. evident.
Histopathologically synovial sarcoma is divided into
The tumor is characterized by a heterogeneous population biphasic, monophasic (fibrous and epithelial) and poorly
of pleomorphic spindle cells arranged in a typical storiform differentiated (round cell) subtypes. A myxoid variant has
or ‘starry-night’ pattern interspersed among zones of ana- also recently been described.
plastic giant cells. The biphasic subtype is composed of spindle and epi-
Based on the histopathological differences, malignant thelial cell elements. The monophasic type is composed
fibrous histiocytomas have been classified as myxoid (good primarily of spindle cells and rarely epithelial cells.
prognosis), inflammatory (aggressive in nature), pleomor- The poorly differentiated subtype shares features of
phic and angiomatoid (very rare) varieties. The myxoid both the mono-and biphasic types along with poorly dif-
variety has a better prognosis whereas inflammatory type ferentiated areas characterized by high cellularity, pleo-
is more aggressive in its course. morphism, numerous mitoses and round cell morphology.
In some patients, necrosis may be evident.
Management Immunohistochemically, the epithelial-like cells are
Surgical management is the treatment of choice. Radiation positive for cytokeratins and epithelial membrane antigen,
therapy could be used as an adjunct to surgery. It is estimated and the spindle cells are positive for vimentin and fibro-
that the 5-survival rate is about 65%. nectin.
All the subtypes are characterized by a specific t(x;18)
(p11.2;q11.2) chromosomal translocation.
Synovial Sarcoma
Knox in 1936 proposed the term ‘synovial sarcoma’ as the
earliest of the cases reported, showed some histological Radical surgical excision with negative margins is the
resemblance to synovial tissue. However, in the present treatment of choice. Regional lymph nodes may have to be
times it is widely accepted that the tumor does not arise removed if clinical lymphadenopathy is evident. Occasion-
from the synovium. ally an adjunctive radiotherapy (65 Gy) is used. Chemo-
Synovial sarcoma arises from the pluripotent mesen- therapy has been tried to prevent distant metastasis.
chymal cells of the para-articular surfaces. It commonly Almost 60–90% of the patients may exhibit recurrence.
affects the extremities. It is estimated that about 10% of all The prognosis of the sarcoma varies on the size of the
soft tissue sarcomas are synovial sarcomas and around tumor and characteristics of the tumor. It is believed that
3–10% occur in the head and neck region. primary tumors larger than 4 cm in diameter have a poorer
prognosis. Calcifying-type synovial sarcoma has been
Clinical features reported to have a relatively better prognosis. The 5-year
survival rate ranges from 36 to 76%.
Synovial sarcomas usually affect men in the 2nd to 4th
decade of life. The common sites of involvement in the head
and neck are the hypopharyngeal and retropharyngeal Cartilage Tissue Origin
regions. Involvements of the parotid gland, tongue, buccal
Chondrosarcoma
mucosa, soft palate, floor of mouth, mandible and TMJ
have also been reported. Chondrosarcomas are malignant tumors of the bone with
Many of the patients may complain of a steadily growing cartilaginous differentiation. The tumor cells characteristi-
painful mass over a period of many months. Some indi- cally form cartilage. They constitute about 25% of all pri-
viduals may complain of difficulty in swallowing, dyspnea mary bone tumors. Phemister in 1930, reported sarcomas
and hoarseness of voice. of bone that contained abundant cartilage as chondrosar-
Almost 50% of the synovial sarcomas may metastasize comas. Lichtenstein and Jaffe defined chondrosarcomas as
to the lung. Other sites for metastases include the lymph arising from full-fledged cartilage and never containing
nodes and bone marrow. osteoid or bone stroma.
372
Chondrosarcomas can have varied behavioral patterns, Adipose Tissue Origin

from slowly growing tumor to a highly aggressive and
metastazing lesion. They may either occur as central or Liposarcoma
peripheral tumors. Liposarcoma is the malignancy arising from of the adipose
tissue. Virchow was the first to describe liposarcoma. After
Clinical features malignant fibrous histiocytoma, it is the second common
soft tissue sarcoma in adults.
Only 5–10% of chondrosarcomas occur in the head and
neck region. The larynx and the nasal cavity are the most
Clinical features
commonly affected. The less common sites being the max-
illa (anterior maxilla) and mandible (symphyseal region). Liposarcoma is commonly seen in males in the 4th to 6th
The tumor commonly affects males in the 3rd decade of decades of life and seldom seen in children. It usually affects
life. The tumor presents as a painless mass or swelling the retroperitoneum, inguinal region and lower extremi-
associated with loosening of the associated teeth mimick- ties. It is very rarely seen in the head and neck region (about
ing periodontal disease. 5–9% of cases). The oral cavity, larynx, hypopharynx,
scalp, orbit and soft tissues of the neck are the common
sites that are affected in the head and neck region. The
Histopathologic features intraoral sites include the buccal mucosa, tongue, gingiva
Tumors are further categorized by grade. Grade 1 repre- and floor of mouth.
sents the least aggressive in terms of histologic features, In the early stages, liposarcoma presents as an asymp-
and grade 3 represents the most aggressive. Most chon- tomatic slow-growing swelling. However, over a period of
drosarcomas are pathologically classified as conventional, time, as the tumor enlarges, it may become symptomatic
but other subgroups are clear cell, myxoid, mesenchymal, when it impinges on the adjacent vital structures.
and dedifferentiated.
Evans and coworkers proposed a histological grading Histopathologic features
system for chondrosarcoma.
Liposarcoma can easily be misdiagnosed clinically. On gross
❍ Grade I lesions: These resemble benign cartilage, hav- appearance, the tumor is well-encapsulated and circum-
ing a relatively uniform, lobular histologic appearance scribed. The WHO distinguishes the four variants proposed
and no metastasis. by Enzinger and Weiss based on developmental stage of
❍ Grade II lesions: Higher recurrence rate than Grade I the lipoblasts and overall degree of cellularity and pleo-
lesions. These exhibit occasional mitotic figures. The morphism.
rate of metastasis is approximately 10%. These four entities are: well-differentiated (subtypes—
❍ Grade III lesions: These are more cellular and pleo- lipoma-like, inflammatory, and sclerosing types), myxoid,
morphic in appearance, with a marked increase in the round-cell and pleomorphic. The dedifferentiated type is
number of mitotic figures. The rate of metastasis in the newer 5th type.
grade III lesions is more than 70%.
Radiographic features Wide surgical excision is the treatment of choice for lipo-
Radiographically, chondrosarcomas can present as ill-defined sarcoma. Lymph node dissection is not indicated unless
radiolucencies with few radiopaque foci (calcification/ metastasis is strongly suspected.
ossification of cartilaginous matrix). Occasionally, these Some authors feel that radiation therapy along with
may present as areas of dense radiopacification with ill- surgical excision may improve the condition of the patient.
defined margins. Involvement of the cortices can result in a The 5-year survival rate is approximately 60–70%.
sunburst pattern.
Symmetric widening of periodontal ligament space and
resorption of the roots of teeth may be evident. Bone Tissue Origin
Osteosarcoma (Osteogenic Sarcoma)
Management
Osteosarcomas are primary malignant bone tumors in
The treatment of choice is radical surgical excision with which mesenchymal cells produce osteoid. Osteosarcomas
negative margins. Distant metastasis is rare. However, dis- of the jaws represent less than 10% of all bone tumors
tant metastasis to the sternum, vertebrae and lungs has and less than 1% of all malignant tumors of the head and
been reported. neck.
373
mandible are the common sites affected. In the maxilla the

Figure 31
alveolar ridge, antrum and the palate are frequently
affected. The infraorbital rim and the zygoma have also
been reportedly affected (Figure 31).
The main symptoms of this lesion in jaw are swelling and
pain, paresthesia/anesthesia (of the lower lip and chin fol-
lowing involvement of the inferior alveolar nerve), loose
teeth and trismus. When the tumor extends to involve the
nasal cavity, maxillary sinus and orbit, clinical signs and
symptoms such as epistaxis, nasal obstruction, hemorrhage,
exophthalmos and blindness may be apparent.
The average time between presenting of symptoms and
diagnosis range from 3 to 5 months.
Amaral et al (2008) suggest that the differential diagnosis
for osteosarcomas of the jaw should include chondrosar-
coma, Ewing’s sarcoma, bone metastasis, fibrous dysplasia,
Clinical photograph of paraosteal osteosarcoma. Courtesy: osteomyelitis and even lesions that do not usually affect
Editor, JCDA. Illustration from non-squamous cell malignant the jaw bones as fibrosarcoma, leiomyosarcoma or rhab-
tumors of the oral cavity: an overview. Tom Daley and domyosarcoma.
Mark Darling. J Can Dent Assoc 2003;69(9):577–82
It occurs most often in the long bones, with predilec- The radiographic features of osteosarcoma of the jaws may
tion for the distal femoral metaphysis, proximal tibia, and vary from ill-defined radiolucent areas to mixed radiopaque-
humeral metaphysis. radiolucent lesions and dense sclerosis/radiopacification
Most of the osteosarcomas originate intramedullary (clas- (Figure 32A–C).
sic or conventional osteosarcoma). However, the other The earliest radiographic change consists of a symmet-
relatively rare types of osteosarcomas are the juxtacortical ric widening of the periodontal ligament space around a
(periosteal and parosteal [Figure 31]) and extraskeletal. tooth or several teeth as a result of tumor infiltration along
Many authors have described osteosarcoma of the jaws the ligament space. This radiographic feature is referred to
as a specific entity, with a clinical behavior different from as Garrington’s sign. Occasionally, lamina dura may be lost.
osteosarcoma of other skeletal bones. The other early radiographic feature was proposed by
Yagan et al (1985). They suggested that the irregular widen-
Predisposing factors ing of the mandibular canal, with areas of narrowing and
loss of fine parallel cortical margins of the walls of the
The exact pathogenesis for the tumor is still unknown. canal was an early sign of osteogenic sarcoma of the man-
However, various predisposing factors have been proposed dible. In some individuals, spiking resorption of the roots
such as trauma, virus, genetic mutations, pre-existing bone of teeth are seen.
cyst, Paget’s disease, osteogenesis imperfecta, osteochon- Other radiographic findings include ill-defined ‘moth-
droma fibrous dysplasia and previous history of radiation eaten’ destruction of bone (extensive sarcomas may cause
(radiation-induced sarcomas). pathological fracture), honeycomb-like appearance, granu-
It is suggested that the average latent period between lar appearance, sunray appearance, Codman’s triangle and
radiation treatment and development of sarcoma is 12.5 onion peel appearance.
years, following radiation dose of 45 Gy. It is believed that Bianchi and Boccardi (1999) described three specific CT
individuals who harbor the mutation in tumor suppressor appearances of osteosarcomas:
genes like p53 and retinoblastoma gene are more prone to
develop osteosarcomas. 1. Radiolucent with absence of bone formation within
the tumor
Clinical features 2. Mottled with small areas of amorphous ossification
3. ‘Lamellar’ ossification with bony plates radiating from
Osteosarcomas affecting the jaw bones are usually seen in a focus-like a sunburst.
the 3rd and 4th decades of life. However, children have
also been affected. Males are slightly more commonly Ng et al (2001) in a pioneering study described the ultra-
affected. sound features of osteosarcoma of the mandible. In the
The mandible and maxilla are equally affected. The mandible they recommend that the most accessible surface
symphysis, ramus and posterior parts of the body of the is the buccal cortex. Features such as bone thinning,
374
Figure 32
A B
(A–C) 3D-reconstructed images showing changes seen in osteosarcoma. Courtesy: Department of Oral Medicine and
erosion, expansion, and the sunray appearance of the buc-

Figure 33
cal cortex were all detected by ultrasound imaging.
Histologically, osteosarcomas are categorized as osteo- Osteoid
blastic, chondroblastic, fibroblastic and telangiectatic.
The characteristic features of osteosarcoma are the pres- Trabeculae of bone
ence of atypical osteoblasts. The osteoblasts are arranged Chondroid tissue
in a disorderly fashion (Figure 33). Irregular sheets of osteoid Pleomorphic,
may be seen. The telangiectatic type exhibits extensive hyperchromatic
blood-filled spaces. However, this type is generally seen in osteoblasts
young adults.
Management Histopathological picture of osteosarcoma. Courtesy:

The choice of treatment for osteosarcoma is radical sur- Department of Oral Pathology and Microbiology,
MCODS, Mangalore
gery along with adjuvant chemotherapy. As most of the
375
osteosarcoma metastasizes by hematogeneous route, there Histopathologic and laboratory studies

is a rationale for addition of adjuvant chemotherapy.
Histopathologically, Ewing’s sarcoma mimics eosino-
Literature reveals that the 8-year metastasis-free survival
philic granuloma, malignant lymphoma and metastatic
rate is 60–70%.
neuroblastoma.
The factors associated with poor prognosis include neu-
Microscopically, tightly packed round cells, compartmen-
ral sensory alteration as a presenting symptom, increasing
talized by fibrous bands with minimal stroma are seen. Indi-
age of patients and surgical margins less than 5 mm.
vidual cells have round to oval nuclei 10–15 m in diameter
with a distinct nuclear membrane. Mitotic figures are com-
monly seen. The cytoplasm is ill defined, scanty, and pale
Ewing’s Sarcoma (Round Cell Sarcoma) staining. The cytoplasm stains characteristically with peri-
Ewing’s sarcoma is uncommon, aggressive bone malignancy odic acid-Schiff (PAS), indicating the presence of glycogen.
occurring in childhood. It accounts for about 4–7% of all Immunostaining for CD99 (Mic-2) is highly sensitive
primary bone malignancies. for Ewing’s sarcoma. Immunostaining also shows abundant
James Ewing (1921) was the first to describe Ewing’s vimentin intermediate filaments. It is related to primitive
sarcoma. It is believed to originate from the immature neuroectodermal tumor sharing a common karyotypic trans-
reticulum cells or primitive mesenchymal cells of the bone location t(11;12)(11;22)(q24;q12).
marrow. Leukocytosis and elevated ESR may be evident.
Although Ewing’s sarcoma commonly affects bone, it Management and prognosis
may also arise from soft tissues. Ewing sarcomas arising
from soft tissues are referred to as extraskeletal Ewing’s Ewing’s sarcoma is best treated with a combination of sur-
sarcoma. Tefft et al (1969) first described this variant. gery, chemotherapy and radiotherapy. Surgery and radia-
The extraskeletal variety has been reported to arise in tion is used for local management. Systemic micrometastasis
various locations such as small intestine, vagina, kidney, is managed with chemotherapy. It is estimated that the
skin, larynx, esophagus, paravertebral region, or epidural 5-year survival rate is about 60%.
space. The prognosis of Ewing’s sarcoma may be poor when
the tumor metastasizes to other bones, lung, lymph node
Clinical features and liver.
Ewing’s sarcoma is usually seen in the first 2 decades of Vascular Origin

life and affects males twice as commonly as females.
It typically affects the pelvic girdle and the long bones of Kaposi’s Sarcoma
the lower extremities. Studies have shown that whites are
Kaposi’s sarcoma is a vascular tumor that was first described
predominantly affected.
by Moritz Kaposi, a Hungarian dermatologist in 1872. He
Only 2–7% of the tumors affect the maxillofacial
described a distinct variety of skin tumors in five male
region. The mandibular ramus region is relatively more
patients in their 6th and 7th decades of life. He termed these
commonly affected than the maxilla. About 90% of the
tumors as ‘idiopathic multiple pigmented sarcoma of the skin’.
mandibular lesions are primary tumors and about 10% are
There is a strong evidence to show that Kaposi’s sar-
metastatic.
coma is caused by human herpes virus 8 (HHV-8).
Swelling, pain, paresthesia, and loose teeth are frequent
Kaposi’s sarcoma has four distinct variants: classic or
symptoms. Occasionally, low-grade fever may be appar-
Mediterranean, endemic or African, epidemic or AIDS
ent.
associated, post-transplant or iatrogenic immunosuppres-
In the initial stages of the tumor, expansion of the cortical
sion associated Kaposi’s sarcoma. The histopathological
plates may be appreciable. However, as the tumor enlarges
and immunohistochemical features of all forms of Kaposi’s
and perforates through bone, as soft, tender mass may be
sarcoma are similar.
evident.
Clinical features
Classic Kaposi’s sarcoma usually occurs in adult males. The
Extensive ill-defined destruction of the bone may be the classic form commonly occurs in Jewish and Italian popu-
only finding in jaw lesions. A laminated periosteal reaction lations. Almost all individuals suffering from the classic
(onion skin/peel appearance) or sunray appearance is usu- form will tend to have an associated malignant lymphoma.
ally a feature of long bones. Occasionally, widening of the Intraoral findings are extremely rare. The palate may be
periodontal ligament space, loss of lamina dura, root resorp- involved. Though the classic variety may affect any part
tion and/or displacement and destruction of unerupted of the body, lower extremities are commonly affected than
tooth follicles are seen. the trunk, arms and hands. The skin of the extremities may
376
❍ Stage IV (disseminated visceral Kaposi’s sarcoma) has

Figure 34
widespread Kaposi’s sarcoma, usually progressing from
Stage II or Stage III, with involvement of multiple vis-
ceral organs.
The following modifications have also been proposed:
❍ Associated opportunistic infection(s) is evident.
❍ Patient is HIV-I seropositive.
❍ Cutaneous anergy or other evidence of severe immu-
nodeficiency is present.
Management
Individual solitary lesions are surgically excised. Electron
beam radiotherapy can be used effectively. Occasionally,
intralesional or systemic chemotherapeutic agents are
Clinical photograph of Kaposi’s sarcoma in a 28-year-old used. Vinblastine is the most commonly used antineoplas-
HIV-positive man who presented with multifocal flat to
tic agent.
nodular purple lesions of the facial skin and oral mucous
membranes. Facial (black arrow), palatal and tongue
(white arrow) lesions are illustrated. Courtesy: Editor, Angiosarcoma
JCDA. Illustration from non-squamous cell malignant
tumors of the oral cavity: an overview. Tom Daley and Angiosarcoma is an uncommon malignancy originating
Mark Darling. J Can Dent Assoc 2003;69(9):577–82 from the vascular endothelium of either the blood vessels
or lymphatic channels. It generally occurs in the skin and
subcutaneous or skeletal muscle and has been reported to
reveal blue to purple macules which turn into painless
occur in the spleen, bone, liver, and breast. Angiosarcomas
nodules over a period of time (Figure 34).
are usually seen in the 5th decade of life. However, indi-
Endemic Kaposi’s sarcoma is also referred to as African
viduals in the age range from 6 to 90 years have also been
Kaposi’s sarcoma. The African variety can present as
affected. More than half the angiosarcomas affect the head
benign nodular (similar to classic, occurs in young adults),
and neck region. The scalp and forehead are the most
infiltrative (locally invades adjacent soft tissues and bone),
commonly affected sites.
florid (widely disseminated with visceral involvement) and
Oral and salivary gland angiosarcomas are exceedingly
lymphadenopathic type (rapidly growing tumors of lymph
rare, comprising only about 2% of all angiosarcomas.
nodes, seen in young children).
Angiosarcomas may involve the lip, buccal mucosa, floor
This association of AIDS with Kaposi’s sarcoma was
of mouth, gingiva, tongue and the mandible.
first described in the early 1980s. It is considered to be the
The intraoral lesions may appear as simple bruise in the
most common form. It is estimated to appear in up to 40%
early stages. As the neoplasm advances, it tends to become
of AIDS patients and may account for up to 90% of all
nodular and ultimately ulcerate.
cancers found in the AIDS population.
The differential diagnosis for angiosarcoma includes
The iatrogenically induced variety is seen a few months
hemangioma, pyogenic granuloma, hemangiopericytoma,
and years following organ transplants in post-transplant
Kaposi’s sarcoma and hemangioendothelioma.
patients. This form of the disease results from the effects of
Histopathologically, the tumor is characterized by the
the immunosuppressive drugs. Cessation of the therapy can
presence of proliferation of endothelium-lined vascular
result in regression of the tumor.
channels forming a network of anastomosis with increased
Schwartz and coworkers in 1984, proposed the following
mitotic activity.
classification system for Kaposi’s sarcoma:
❍ Stage I represents localized nodular Kaposi’s sarcoma, Management
with more than 15 cutaneous lesions or involvement Angiosarcomas are best treated with surgical excision and
restricted to one bilateral anatomic site, and few, if radiation therapy.
any, gut nodules.
❍ Stage II includes both exophytic destructive lesions
and locally infiltrative cutaneous lesions as locally Muscle Origin
aggressive Kaposi’s sarcoma.
Leiomyosarcoma
❍ Stage III (generalized lymphadenopathic Kaposi’s sar-
coma) has widespread lymph node involvement, with or Leiomyosarcoma is a malignant mesenchymal neoplasm
without skin lesions, but with no visceral involvement. exhibiting smooth muscle differentiation. It accounts for
377
about 6–7% of all soft tissue sarcomas. However, it rarely

Figure 35
occurs in the oral soft tissues or jaw bones.
It typically affects the adults and occurs in the retro-
peritoneal region, gastrointestinal tract and uterus, reflect-
ing the affinity for affecting smooth muscles. These tumors
generally affect individuals over the 4th and 5th decades
of life.
Clinical features
Oral leiomyosarcomas may occur at any age. About 3–10%
of the leiomyosarcomas. Smooth muscle tumors in the oral
cavity may originate from the arterial tunica media, the
ductus lingualis, the circumvallate papillae, and pluripo-
tential mesenchymal cells, which are rich sources of
smooth muscle tissue.
A leiomyosarcoma in the oral cavity may be primary or
secondary (metastatic from another location). The common
sites of metastatic involvement are the mandible for bony
depositions and the gingivae and tongue for soft tissue Clinical photograph of rhabdomyosarcoma.
deposits.
It is estimated that 45% of the reported cases affecting
the oral cavity occur in the jaw bones.
Clinically they may appear as painless or occasionally Types of rhabdomyosarcomas
painful well-circumscribed mass, firmly adherent to the
Based on their anatomical location of occurrence, these
surrounding tissues. Some of these tumors may reveal
tumors are broadly categorized as orbital, parameningeal
ulcerations.
and non-orbital non-parameningeal forms. Parameningeal
tumors are said to have the worst prognosis.
Histopathologic features The International Classification of Rhabdomyosarcoma
Leiomyosarcoma is characterized by sheets of sweeping, in 1994, divides this tumor into four subgroups, namely,
alternating bundles and fascicles of densely packed spin- botryoid and spindle cell RMS, embryonal RMS, alveolar
dle cells with abundant fibrillar eosinophilic cytoplasm RMS, and undifferentiated sarcoma.
and indistinct cytoplasmic borders. The nucleus is usually
centrally located and blunt-ended, squared-off or cigar- Oral features
shaped. Occasional cells have perinuclear vacuoles. These tumors are generally seen in the first decade of life.
Masson’s trichrome staining and immunohistochemi- These are also occasionally seen in adolescents and young
cal evaluation for muscle antigens are helpful in differen- adults. Clinically a rapidly growing painless mass may be
tiating leiomyosarcoma from other sarcomas. Positive seen. Palate, tongue and alveolar ridge have been reported
reactions for desmin, vimentin, smooth-muscle actin and as the common sites affected (Figure 35). In the advanced
h-caldesmon have been demonstrated in this neoplasm. stages of the disease, pain, paresthesia, loosening of the
teeth, and trismus may be seen.
Management
Radical surgical excision is the treatment of choice along Management
with chemotherapy and radiotherapy. It is estimated that The primary tumors are best managed with surgical resec-
the 5-year survival rate is about 30%. tion, multidrug chemotherapy and radiation therapy. The
5-year survival rate is estimated to be about 60%.
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is considered as the most com- Nerve Tissue Origin
mon soft tissue sarcoma affecting children. It is a malignant Neurofibrosarcoma (Malignant Schwannoma,
neoplasm of skeletal muscle origin. Weber in 1854 is cred-
Neurogenic Sarcoma)
ited for giving the first description of rhabdomyosarcoma.
It is estimated that about 35% of RMS arises in the head Neurofibrosarcomas arise from the nerve sheath cells.
and neck. These account for about 10% of soft tissue sarcomas. It is
378
estimated that almost 50% of these tumors originate from those of fibrosarcoma but which are usually more irregular,
neurofibromatosis type 1. with wavy or comma-shaped nuclei.
Some less cellular myxoid areas may also be seen. Posi-
Clinical features tive immunostaining for S100 protein is often useful.
Neurofibrosarcomas are uncommon in the head and neck
Management
region. These are common in 4th to 6th decades of life.
The common sites affected are the lips, palate, gingiva, The tumor is managed with surgery and radiation ther-
buccal mucosa and mandible. Clinically the tumors appear apy. The tumors have a high recurrence rate as they tend
as rapidly growing mass of tissue occasionally associated to spread along the involved nerve. It is estimated that the
with pain and paresthesia. prognosis for patients with neurofibrosarcoma arising de
novo is about 50% for 5-year survival, whereas it is 15% for
Radiographic features neurofibrosarcoma arising in cases of neurofibromatosis.
Radiographs may exhibit an ill-defined radiolucency sug-
gestive of destruction of the bone. Widening of the infe- Lymphoid Origin and Hematological
rior alveolar canal may also be seen. Malignancies
Tumors of lymphoid origin, namely, Hodgkin’s and non-
Hodgkin’s lymphoma and hematological malignancies
The typical histopathological feature is the presence of fas- including leukemia and myeloma are described in Chapter 18
cicles of atypical spindle-shaped cells, which may resemble on Systemic Disorders and their Clinical Implications.
379
CHAPTER
Oral Cancer
14 SV Kumaraswamy, V Jeevan Prakash,
Praveen BN, Ravikiran Ongole
➧ Incidence of Cancer of Head and Neck Tumor Growth and Metastasis

➧ Etiology and Risk Factors Molecular Abnormalities
Genetic Susceptibility ➧ Clinical Signs of Cancer
Immune Status ➧ Clinical Examination of a Patient with
Environmental Factors Suspected Malignancy
➧ Tobacco ➧ TNM Staging
➧ Alcohol ➧ Nodal Metastasis
➧ Systemic Health ➧ Diagnosis of Oral Cancer
Ionizing Radiation ➧ Management of Oral Cancer
Role of Viruses Surgical Treatment
Role of Nutrition Radiation Therapy
Oral Hygiene and Dental factors Chemotherapy
➧ Molecular Basis of Cancer
Cellular Kinetics
Malignant tumors are popularly referred to as ‘cancer’. As oral carcinoma is one of the most prevalent cancers
However, the term ‘tumor’ is scientifically more appropriate. and is one of the 10 major causes of death, this chapter will
Tumor is defined as an autonomous new growth of tissue attempt to highlight the etiology, clinical features, diagnosis,
or an abnormal mass of tissue, the growth of which investigations and the treatment options.
exceeds and is uncoordinated with that of normal tissue
and persists in the same excessive manner even after the
cessation of stimuli which evoked the change. INCIDENCE OF CANCER OF HEAD
Hippocrates described various types of cancers. He AND NECK
referred to benign tumors as oncos (swelling in Greek) and
malignant tumors as carcinos (crab or crayfish in Greek). According to the World Health Report (2004), cancer
He compared malignant tumors to a crab since the sectional accounted for 7.1 million deaths in 2003 and it is estimated
morphology of the tumor along with its vascular supply that the overall number of new cases will rise by 50% in
mimicked the body of a crab with its claws and feet the next 20 years. Oropharyngeal cancer is more common
outstretched. in developing countries than developed countries. The
Aulus Cornelius Celsus, a Roman encyclopedist, trans- prevalence of oral cancer is particularly high among men,
lated carcinos into the Latin cancer, which also means crab. the eighth most common cancer worldwide. Incidence rates
Galen, the Roman physician used oncos to describe all for oral cancer vary in men from 1 to 10 cases per 100,000
tumors, which laid the foundation for the use of the present population in many countries. In South-Central Asia, can-
day term ‘oncology’. cer of the oral cavity ranks among the three most common
Oral malignancies may arise from the epithelium, mus- types of cancer. In India, the age standardized incidence rate
cle mass, odontogenic structures, specialized structures of oral cancer is 12.6 per 100,000 population. More than
like the tongue and the eye, from the salivary glands both 90% of all oropharyngeal cancers occur in patients over the
major and minor and also from the bone. However, it is age of 45. As with other head and neck tumors, male pre-
virtually impossible to describe all the forms of cancer in dominance is common, with a male to female ratio of 4:1,
this chapter. because of the greater use of tobacco by men.
380
Chapter 14 – Oral Cancer
It has been estimated that 43% of cancer deaths world- It is also a well-known fact that the host response dimin-
wide are due to tobacco, unhealthy diet, physical inactivity ishes with advancing age. HIV-positive immunocompro-
and infections. Tobacco use and excessive alcohol consump- mised individuals, may usually exhibit Kaposi’s sarcoma
tion have been estimated to account for about 90% of can- and non-Hodgkin’s lymphomas.
cers in the oral cavity; the oral cancer risk increases when
tobacco is used in combination with alcohol or areca nut.
About 96% of all oral cancers are carcinomas and the Environmental Factors
remaining 4% are sarcomas. Majority of oral carcinomas Exposure to actinic radiation There is enough evidence
are squamous cell carcinomas. It is estimated that 9 out of in literature to show that skin and lip cancers are more
every 10 oral malignancies is squamous cell carcinoma. It frequently seen in individuals whose occupation necessi-
is a disease of increasing age with 95% of the patients tates long working hours in the sun such as fishermen and
older than 40 years of age. farmers.
In India, it is estimated that there are 2–2.5 million can- It has been reported that fair skinned individuals, people
cer patients at any given point of time with about 0.7 mil- residing in high latitudes with clear atmosphere (UV light
lion new cases diagnosed every year and nearly half of can penetrate easily) such as Finland and Sweden, resi-
them die every year. Two-thirds of the new cancers is dents closer to the equator (long periods of sunshine) such
diagnosed at a very advanced and incurable stage. More as Greece are more susceptible to develop lip cancers.
than 60% of these affected patients are in the age group of The wavelengths of the light thought to be responsible for
35 and 65 years. Fifty percent of all male and 25% in the actinic damage are in the range of 2,900–3,200 A.
female are tobacco related cancers. Sunscreen lotions are effective in protecting the lip from
the damaging effects of UV light. Melanin pigment acts as
a protective agent against actinic radiation.
ETIOLOGY AND RISK FACTORS FOR ORAL Atmospheric pollution Air pollution arising from indus-
AND MAXILLOFACIAL CANCER trial wastes and automobile exhausts are particularly harm-
ful. Other sources include gases emitted from burning
The exact etiology for oral cancer is still questionable. How- firewood/coal for domestic purposes. Sulfur dioxide, carbon
ever, it is well known that a plethora of factors predispose monoxide, nitrogen gases have been implicated in causing
an individual to developing oral cancers. Historically, the pharyngeal, laryngeal and lower respiratory tract cancers.
widely known six “S” has been mentioned in literature,
namely, smoking, spirit, sharp teeth, sunlight, syphilis,
spicy food and sepsis. However, over a period of time TOBACCO
many predisposing factors have been proposed and sug-
gested such as genetic susceptibility, environmental factors, All the forms of tobacco (smoke and the smokeless/
systemic health of an individual and abusive habits such as chewable/inhaled) such as cigarettes, pipes, cigars, beedis,
the consumption of tobacco and alcohol. paan and snuff have been implicated in the development
of oral cancers. It is believed that tobacco use is respon-
Genetic Susceptibility sible for 90% of the oral cancers in males.
Family history of oral cancer is considered a risk factor.

Head and neck cancer patients show an increased suscep- Consumption of Tobacco in India
tibility to chromosome damage by mutagens. Some stud- It is estimated that 80–85% of tobacco is consumed for
ies suggest that lip cancers have shown some amount of smoking either as beedis or cigarettes (Figure 1). Almost
genetic predisposition. Mork et al (1999) reported a signifi- 13% chew tobacco in the form of paan (Figure 2, betel
cantly increased odds ratio for developing head and neck leaf, areca nut, tobacco, slaked lime and flavoring agents)
squamous cell carcinoma in female patients, aged less than or gutkha. Almost 15% are addicted to both habit of chew-
45 years, who had first-degree relatives with cancer. It is ing and smoking. Only about 1–3% use tobacco in the form
suggested that familial oral cancer may be attributed to both of snuff.
shared environmental factors within families and a common
oral cancer susceptibility gene with low penetrance.
Smokeless Form of Tobacco
People in the Indian subcontinent exhibit various forms
Immune Status
of tobacco chewing habits such as khaini, mishri, zarda,
It has been noticed that immunosuppressed individuals gutkha (Figure 3), mawa and nass. Paan is chewed (betel
tend to show an increased incidence of oral malignancies. quid) and the quid is usually placed in the buccal vestibule.
381
Figure 1 Figure 3
One of the popularly consumed packets of gutkha.

A typical road-side shop selling various tobacco products

ranging from cigarettes, beedis, paan to gutkha. Ironically
Figure 4
displaying the fact that tobacco causes cancer. Courtesy:
Department of Oral Medicine and Radiology, Manipal College
of Dental Sciences, Mangalore
Figure 2
Various constituents of paan. Courtesy: Department of Snuff used for inhalation. One of the risk factors for
Oral Medicine and Radiology, Manipal College of Dental causing nasopharyngeal and maxillary sinus cancers.
Sciences, Mangalore Courtesy: Department of Oral Medicine and Radiology,
Tobacco is also used in the form of a powder for inhala-

tion (Figure 4, snuff). Tobacco (Figure 5) contains nicotine
Smoke Form of Tobacco
(nitrosamine), nitrosodiethanolamine, nitrosoproline, polo-
nium and polycyclic aromatic hydrocarbon (tars). Areca nut In India, beedi (Figure 7), cigarette and chutta smoking is
(Figure 6 contains cholinergic muscarinic alkaloids such commonly seen. However, other popular forms of smoking
as arecholine and guavacoline) chewing is also widely include use of pipe, cigar and hookah. It is believed that
practiced in India. cigar and pipe smoking is more hazardous than cigarette
382
Figure 5 Figure 7
Processed tobacco leaf ready for use. Courtesy: Beedis made of tobacco wrapped in a tendu
Department of Oral Medicine and Radiology, (Diospyros melanoxylon) leaf, and secured with
Manipal College of Dental Sciences, Mangalore thread at one end. The tobacco content in beedis is
approximately 10–20%. Courtesy: Department of
Oral Medicine and Radiology, Manipal College of
Figure 6
Figure 8
Mature areca nut and the areca nut that is obtained

after it is dehusked, processed and dried. Courtesy:
smoking. Palatal cancers are more common in individuals Cigarettes with filters. Courtesy: Department of
who place the lit end of the beedis and cigarettes inside the Oral Medicine and Radiology, Manipal College of
mouth (reverse smoking). It has been proposed that smoking Dental Sciences, Mangalore
causes pooling of carcinogens in saliva thereby increasing
the incidence of cancers of the floor of the mouth, ventral
and lateral surface of tongue.
It is estimated that in the combustion mainstream of one aryl hydrocarbon hydroxylase that is principally produced
cigarette (Figure 8), there are approximately 500 mg (92%) in human leukocytes, is said to increase the carcinogenic
of gaseous content (mainly oxygen, nitrogen and carbon potential of benzopyrene.
dioxide and to a little extent carbon monoxide) and 8% of The carcinogenic properties of marijuana smoke are
particulate matter. Aromatic hydrocarbons in the form of similar to those of tobacco. Marijuana may interact with
‘tars’ may constitute less than 1 g to 35 mg. Nicotine content mutagen sensitivity and other risk factors to increase the
varies from 1 to 3 mg. Other constituents of tobacco smoke risk of head and neck cancer. Marijuana smoke has a four
include carbon monoxide, hydrogen cyanide and thiocya- times higher tar burden and 50% higher concentrations of
nate. Benzopyrene is considered the most potent carcino- benzpyrene and aromatic hydrocarbons than are present
gen. It preferentially binds to nucleoproteins. The enzyme in tobacco smoke.
383
ALCOHOL oxidative damage to DNA. Production of reactive oxygen

species and lipid peroxidation are increased in diabetic
Alcohol consumption is the most important risk factor for patients, especially in those with poor diabetic control and
development of oral cancer in non-smokers. It is also con- hypertriglyceridemia. Increased oxidative damage can be
sidered as the second independent major risk factor for the due to superoxide radical generation by monocytes through
development of oral cancer. It is estimated that an average nicotinamide adenine dinucleotide phosphate (NADPH)
consumption of over 30 ml of alcohol per day increases the oxidase. Superoxide can undergo either enzymatic or non-
risk of oral cancer linearly with the quantity of alcohol con- enzymatic dismutation to generate hydrogen peroxide. In
sumed. Though any form of alcohol when consumed in large the presence of transition metals, such as Fe and Cu,
quantities is dangerous, it is believed that dark colored drinks both these substances contribute to the generation of
are more hazardous (as they may contain higher beverage highly reactive hydroxyl radicals causing damage to cells.
congeners such as nitrosamines, hydrocarbons and other Hence, patients with poorly controlled diabetes are at
impurities which are known carcinogens). Carcinogens pres- great risk of developing oral cancer.
ent in the tar are insoluble in saliva but are highly soluble Syphilis In the older literature, syphilis was considered
in alcohol and easily absorbed in the oropharynx. an important predisposing factor for oral leukoplakia and
The International Agency for Research on Cancer pub- oral cancer. It was suggested that syphilitic infection
lished a monograph in 1982, which details the various meth- caused endarteritis and subsequently atrophy of the over-
ods in which alcohol predisposes to head and neck cancers. lying epithelium. This made the tongue more vulnerable
❍ Ethyl alcohol increases the permeability of oral mucosa. to the etiological factors. In modern times, tertiary syphilis
It also dehydrates the mucosa. It has a solvent action is rarely encountered in clinical practice due to effective
on the keratinocyte membrane thereby allowing the treatment.
passage of carcinogens into proliferating cells where Trieger et al (1958) reported a study that suggests
they may exert a mutagenic action. an increased prevalence of syphilis (approximately up to
❍ The immediate metabolite of ethanol is acetaldehyde, 60%) in patient groups with squamous cell carcinoma of
which readily damages cells. It is believed that those the tongue. It was seen that this association was stronger
who are rapid acetylators are at increased risk of devel- in males than females.
oping cancers. Michalek et al (1994) reported a study of 16,420 people
❍ Alcoholic liver disease is common in heavy drinkers with syphilis resident in the United States that showed a
and this minimizes the detoxification of carcinogens. significantly raised frequency of cancer of the tongue (and
❍ Alcohol, owing to its high calorific value, suppresses Kaposi’s sarcoma) in males. Dickenson et al (1995) screened
appetite in heavy drinkers. This predisposes to nutri- 63 patients of United Kingdom suffering from squamous
tional deficiency which in turn is a risk factor for can- cell carcinoma of the tongue. Five of these patients had
cers. Compared to non-users, alcohol users are serological evidence of past syphilis when detected by
3.6 times more likely to have oropharyngeal cancer, both specific and non-specific tests.
5.8 times for tobacco users, and 19 times for users of
both alcohol and tobacco.
Ionizing Radiation
Exposure to large amounts of ionizing radiation such as
SYSTEMIC HEALTH
in a nuclear mishap is a known risk for causing cancers.
Radiation exposure, known to cause DNA damage, may
Candidiasis Autoimmune polyendocrinopathy candidiasis– be a potential source of field cancerization of the upper
ectodermal dystrophy an autosomal recessive disease aerodigestive tract.
associated with a limited T lymphocyte defect, presumably Hashibe et al (2005) evaluated the possible risk of
supports the growth of Candida albicans and predisposes developing second primary cancers following radiotherapy
to chronic mucositis and oral cancer. for head and neck cancers in 30,221 oral squamous cell
Diabetes In diabetic patients, alterations occur in the oxi- carcinoma patients. Patients treated with radiation only or
dative equilibrium of free radicals. Elevated blood glucose radiation with surgery had elevated risks of developing a
levels can lead to excessive formation of free radicals. second primary tumor, whereas patients treated with surgery
Moreover, due to protein breakdown, the activity of anti- only did not appear to be at increased risk. They also sug-
oxidant scavengers and enzymes is reduced. Both the gested that the expected latent period between radiation
increase in free radicals and oxidative stress promote car- exposure and tumor occurrence, radiation became a risk
cinogenesis. factor after 10 years of follow-up for solid cancers of the oral
It has been suggested that poor diabetic control is asso- cavity, pharynx, esophagus, and lung, and after 1–5 years
ciated with an increased cancer risk due to enhanced of follow-up for second primary leukemia.
384
Role of Viruses trauma along with other carcinogens may aid in the
malignant transformation of epithelial cells.
The role of viruses remains unclear. Varying viral genomes Shah (2003) suggests that the microorganisms from
have been frequently found within cancer cells. Viruses dental plaque, by way of chemical carcinogenesis, may pro-
have been known to modify the DNA and the chromo- duce nitrosating enzymes which are toxic. It is also believed
somal structures and induce proliferative changes in the that individuals who do not maintain good oral hygiene
cells they infect. (inadequate brushing) may fail to dilute the carcinogens
Evidence of a viral carcinogenesis is perhaps strongest present in the oral cavity, especially derived from various
for infection with human papilloma viruses (HPV). D’Souza tobacco-related habits.
et al (2007) in a multicenter case-control study reported that
infection with HPV-16 increased the risk of cancer of the
oral cavity and particularly oropharynx.
The role of infection with Epstein–Barr virus (EBV) and MOLECULAR BASIS OF CANCER
herpes simplex viruses (HSV) remains uncertain. The role
of HSV, HSV-1 and HSV-2, as co-factors in association Though no one truly understands how these conditions
with tobacco, alcohol, or HPV-16 infection has also been either in their individual standing or in conjunction with
proposed in causing oral cancers. other entities leads to cancer, the greater understanding of
Human immunodeficiency virus (HIV), due its effect on the importance of genetics and the role of pro-oncogenes
immunosurveillance, acts as a cofactor along with other and tumor suppressor genes has given us a more detailed
viruses such as EBV and cytomegalovirus in predisposing insight into the evolution of a cancerous growth. Cellular
the affected individual to oral cancer. and molecular basis of malignancy though needs more
detailed discussion it could be best summarized under the
following sections.
Role of Nutrition
Various studies have shown that a diet with low vitamin A, Cellular Kinetics
vitamin C, vitamin E, iron, selenium, folate and other trace
element content is associated with an increased risk of oral, Generation time is the time required for a quiescent cell to
laryngeal, lung, gastric, ovarian, breast and cervical can- enter the cell cycle and give rise to two daughter cells.
cers. Certain dietary deficiencies may cause epithelial atro- Malignant cells usually have a shorter generation time than
phy which renders the epithelium vulnerable to the action non-malignant cells and a smaller percentage of cells in
of carcinogens. The relationship between sideropenic dys- G0 (resting phase), so a larger proliferation fraction exists.
phagia and oral cancer is well recognized (sideropenic Initial exponential tumor growth is followed by a plateau
dysphagia may be associated with epithelial atrophy in the phase when cell death equals the rate of formation of
upper alimentary tract). daughter cells. Compared to large tumors, small tumors
Garewal (1994) summarized the findings of 54 studies have a greater percentage of actively dividing cells and
that evaluated fruit and vegetable intake in the develop- thus show greater rates of proliferation.
ment of cancers in the upper aerodigestive tract; he found
that 52 of the studies demonstrated a protective effect of
the antioxidant content of fruits and vegetables. Tumor Growth and Metastasis
It has been shown that patients who ingest high levels As a tumor grows, nutrients are provided by direct diffu-
of vitamin C and fiber have half the risk of developing oral sion from the circulation. Local growth is facilitated by
cancer as those with minimal level of consumption. Block enzymes (e.g. collagenases) and cytokines that alter or
(1991) and Mirvish (1986) showed that a low intake of destroy adjacent tissues. As the ratio of surface area to vol-
vitamin C is associated with an increased risk of cancers of ume becomes smaller with increased tumor growth, tumor
the stomach, esophagus, oral cavity, larynx, and cervix. angiogenesis factors are produced, forming the indepen-
Gridley et al (1992) in a study involving 2,000 individu- dent vascular supply required for further tumor growth.
als proposed that the use of vitamin E supplements corre- Almost from inception, a tumor may shed cells into the
lated with a diminished risk for oral and pharyngeal cancers. circulation. From animal models, it is estimated that a
1-cm tumor sheds more than 1 million cells/24 h into the
venous circulation. Although most circulating tumor cells
Oral Hygiene and Dental Factors
die as a result of intravascular trauma, a tiny number
Though the exact etiological role of poor oral hygiene, (much less than 1 in 1 million) adhere to the vascular endo-
faulty restorations, ill-fitting dentures, sharp teeth in caus- thelium and penetrate into surrounding tissues, generating
ing oral cancers is not substantiated, these may be the independent tumors (metastases) at distant sites. Metastatic
possible contributing factors. It is believed that chronic tumors grow in much the same manner as primary tumors
385
and may subsequently give rise to other metastases. Another mechanism that results in defective function and
Experiments suggest that metastasis is not a random event transcription of tumor suppressor genes is aberrant methyl-
and that the primary tumor may regulate the growth of ation of the promoter region of these genes, which inhibits
metastatic tumors, for example, removal of the primary gene transcription. Greater degrees of aberrant methylation
tumor sometimes results in rapid growth of the metastases. and greater numbers of affected genes cause tumors to be
more malignant and are associated with shortened sur-
vival in lung, bladder, and prostate cancers. In vitro alter-
Molecular Abnormalities
ation of the aberrant methylation has caused reversion to a
Genetic mutations are largely responsible for the generation non-malignant, non-proliferative phenotype, suggesting
of malignant cells. These mutations alter the quantity or a potential therapeutic target.
function of protein products that regulate cell growth and Another important regulatory protein, p53, prevents
division and DNA repair. Two major categories of mutated replication of damaged DNA in normal cells and promotes
genes are oncogenes and tumor suppressor genes. cell death (apoptosis) in cells with abnormal DNA. Inactive
Oncogenes are abnormal forms of normal genes (proto- or altered p53 allows cells with abnormal DNA to survive
oncogenes) that regulate cell growth. Mutation of these and divide. Mutations are passed to daughter cells, confer-
genes may result in direct and continuous stimulation of the ring a high probability of neoplastic transformation. The
molecular biologic pathways (e.g. intracellular signal trans- p53 gene is defective in many human cancers.
duction pathways, transcription factors, secreted growth Gross chromosomal abnormalities can occur through
factors) that control cellular growth and division. deletion, translocation, or duplication. If these alterations
There are more than 100 known oncogenes that may con- activate or inactivate genes that result in a proliferative
tribute to human neoplastic transformation, for example, advantage over normal cells, then a tumor may develop.
the ras gene encodes the Ras protein, which regulates cell Chromosomal abnormalities occur in certain human can-
division. Mutations may result in the inappropriate activa- cers. In some congenital diseases (Bloom syndrome, Fanconi
tion of the Ras protein, leading to uncontrolled cell growth syndrome, Down’s syndrome), chromosomes break easily,
and division. In fact, the Ras protein is abnormal in about putting children at high risk of developing acute leukemia
25% of human cancers. Other oncogenes have been impli- and other cancers.
cated in specific cancers. These include various protein Most cancers are likely to involve several of the mecha-
kinases (bladder cancer, breast cancer), bcr-abl (chronic nisms described above that lead to neoplastic conversion.
myelocytic leukemia, B-cell acute lymphocytic leukemia), As with oncogenes, mutation of tumor suppressor genes in
C-myc (small cell lung cancer), N-myc (small cell lung germ cell lines may result in vertical transmission and a
cancer, neuroblastoma), and C-erb B-2 (breast cancer). higher incidence of cancer in an offspring. Telomeres are
Specific oncogenes may have important implications nucleoprotein complexes that cap the ends of chromosomes
for diagnosis, therapy, and prognosis (see individual dis- and maintain their integrity. Telomere shortening (with
cussions under the specific cancer type). Oncogenes typi- aging) results in replicative senescence, increased genetic
cally result from acquired somatic cell mutations secondary instability, and potential tumor formation. Telomerase is an
to point mutations (e.g. from chemical carcinogens), gene enzyme that carries out telomere synthesis and mainte-
amplification (e.g. an increase in the number of copies of nance, thus telomerase may potentially allow for cellular
a normal gene), or from insertion of viral genetic elements immortality. Telomerase activity may promote tumors
into host DNA. Occasionally, mutation of germ cell lines through multiple, complex mechanisms, especially by sub-
results in vertical transmission and a higher incidence of verting the normal DNA synthetic checkpoints.
cancer development in an offspring.
Tumor suppressor genes are inherent genes that play a
role in cell division and DNA repair and are critical for CLINICAL SIGNS OF CANCER
detecting inappropriate growth signals in cells. If these
genes, as a result of inherited or acquired mutations, become The hardest part of treating cancer is diagnosing it early,
unable to function, genetic mutations in other genes can but it is also the most easiest part of treating cancer
proceed unchecked, leading to neoplastic transformation. because if discovered early, the lesions are amenable to
As with most genes, two alleles are present that encode simple excision and patients have a good chance of a
for each tumor suppressor gene. A defective copy of one 5-year disease-free survival rate. It makes it easier to
gene may be inherited, leaving a person with only one remember if the clinical signs are discussed based on the
functional allele for the individual tumor suppressor gene. regional anatomy.
If an acquired mutation occurs in the other allele, the nor- The many signs and symptoms of oral cancer (Table 1)
mal protective mechanisms of the tumor suppressor gene are usually divided into early and late presentations. They
are lost, and dysfunction of other protein products or DNA can be so diverse that the differential diagnosis may not
damage may escape unregulated, leading to cancer. lead to oral malignancy.
386
The clinical presentation varies in most cases and there are period of time. The tumor may appear as small nodules and
no two cases that are similar in presentation and treatment. enlarges to from a wart-like growth which ultimately ulcer-
However, there could be a few similarities that are peculiar ates or may even begin as an ulceration that does not heal.
to the geographic area that could be grouped together and The lesion is often not painful and is noticed by the patient
explained as an entity, for example, carcinoma of the buc- only when there is a secondary infection of the ulcer. There
cal mucosa and the gingivobuccal sulcus that are peculiar to is induration and infiltration into the deeper tissues.
the Indian subcontinent and have been nicknamed as Extension into the muscle of mastication, buccinators,
‘Indian Cancers’. Cancers of the floor of the mouth are more alveolar mucosa and ultimately into the bone may occur
common in the western countries because of their habits. and if left unchecked which may cause perforation of the
The following section is just a brief explanation of the overlying skin (Figure 10). The induration of the skin is a
various clinical signs and symptoms based on their area of bad clinical sign and necessitates wider excision along
presentation. with the overlying skin.
Carcinoma of buccal mucosa

Figure 9
The lesions develop most frequently along or inferior to a
line opposite the plane of occlusion. It usually occurs at
the regions of the third molar area (Figure 9) as there is a
habit of keeping the quid (tobacco) in that area for a long
Table 1 Clinical signs of oral cancer
Early signs Late signs

• Persistent red and/or white patch • Indurated area
• Non-healing ulcer • Paresthesia, dysesthesia of
• Progressive swelling or the tongue or lips
enlargement • Airway obstruction
• Unusual surface changes • Chronic earache (chronic
• Sudden tooth mobility without serous otitis media)/otalgia
apparent cause • Trismus and dysphagia
• Unusual oral bleeding or epistaxis • Cervical lymphadenopathy
• Prolonged hoarseness of voice • Persistent pain or referred pain
• Altered vision Carcinoma of the left buccal mucosa extending into the
• Epiphora buccal vestibule. Courtesy: Dr Abhinandan
Figure 10
A B
Carcinoma causing perforation of the cheek. Courtesy: Dr Abhinandan
387
Some cases appear to be growing outward from the sur- a lymphatic cross-drainage exists, contralateral metastasis
face rather than invading the tissues is called exophytic or is often present.
verrucous growth. The most common site of metastasis is
the submandibular lymph nodes as they are the primary Carcinoma of labial mucosa
echelon nodes for these regions.
It is frequently seen in people who habitually keep a mixture
of tobacco and lime in the labial vestibule. The lower labial
Carcinoma of floor of mouth
mucosa (Figure 13) is more commonly involved than the
It is seen more commonly in men; reasons being cited that upper though the upper lip often gets involved in such cases
they tend to smoke and abuse tobacco a lot more than due to inadvertent contact exposure to the carcinogens.
women and the chemicals that mix with the saliva tend to The most common initial signs and symptoms are growth
pool in the floor of the mouth for a longer time. It is seen
most frequently in the anterior portion of floor. The typical
carcinoma of the floor of mouth is an indurated ulcer of Figure 12
varying size, on one side of the midline. It may take the
form of wart-like growth (Figure 11), which tend to spread
superficially rather than in depth. It may spread in four
primary directions, i.e. extend to the other side crossing
the midline, may extend upward involving the base of the
tongue or may come anteriorly invading the lingual mucosa
and ultimately invade the bone causing loosening of the
anterior teeth (Figure 12). Loosening of teeth is seen only
in advanced cases. However, patients seek an early consul-
tation because of the restriction in the movements of the
tongue often causing peculiar thickening or slurring or the
speech. There may be excessive salivation. In some cases,
there may be referred pain in the ears.
Carcinoma of floor of mouth may invade the deeper tis-
sues and may even extend into the submaxillary and sub-
Orthopantomograph (OPG) showing extensive destruction of
lingual glands. Metastasis from the floor of the mouth are
the alveolus in the anterior portion of the mandible with
found most commonly in the submandibular group of teeth floating in space appearance secondary to infiltration
lymph nodes and also sometimes in the facial nodes, since of the carcinoma of floor of mouth to the alveolus.
the primary lesion frequently occurs near the midline where Courtesy: Department of Oral Medicine and Radiology,
Figure 11
Figure 13
Wart-like growth in the anterior portion of the floor of

the mouth suggestive of carcinoma floor of mouth. Carcinoma of the lower lip showing signs of extensive
Courtesy: Department of Oral Medicine and Radiology, induration. Courtesy: Department of Oral Medicine and
Manipal College of Dental Sciences, Mangalore Radiology, Manipal College of Dental Sciences, Mangalore
388
Figure 14 Figure 16
Carcinoma affecting the right lateral margin of the tongue.

Ulcero-proliferative lesion on the lower lip and Manipal College of Dental Sciences, Mangalore
labial vestibule. Courtesy: Department of Oral Medicine and
Radiology, Manipal College of Dental Sciences, Mangalore
Figure 17
Figure 15
Extensive lesions affecting the lower lip, upper lip and

the right commissure. Courtesy: Department of Oral Medicine Carcinoma affecting the ventral surface of the tongue.
and Radiology, Manipal College of Dental Sciences, Mangalore Courtesy: Department of Oral Medicine and Radiology,
or swelling, soreness and ulceration of the lip which does

products. Squamous cell carcinoma is by far the most
not heal (Figure 14). Advanced lesion may be ulcerative-
common malignancy of the tongue, typically having three
infiltrative type which may restrict the movement of the
gross morphologic growth patterns: exophytic, ulcerative,
lower lip. Lymph node involvement may be unilateral or
and infiltrative. The infiltrative and ulcerative types are
bilateral as they drain primarily into the submandibular
observed most commonly on the tongue. Lateral margins
nodes. Occasionally, the carcinoma may spread to the cor-
(Figure 16) and ventral surface (Figure 17) of the tongue
ners of the mouth and extend extraorally (Figure 15).
are more frequently affected. The most common finding is
an indurated, ulcerated area of the tongue. The induration
Carcinoma of tongue
may extend deep into the tongue musculature and root of
The tongue is the most common intraoral site of cancer in the tongue. Malignancies of the tongue may grow to sig-
most countries. Of all potential etiologic factors, cancer of nificant size before they cause symptoms. Because of the
the tongue is correlated the closest with the use of tobacco relative laxity of the tissue planes separating the intrinsic
389
Figure 18 Figure 19
Carcinoma of the palate causing perforation.

Courtesy: Dr Abhinandan Carcinoma involving the right maxillary antrum and
the alveolus. Courtesy: Dr Abhinandan
tongue musculature, the cancer may spread easily and

others of such type. As it is seen over periods of prolonged
become symptomatic only when its size interferes with
exposure the mean age of occurrence is 60 years. Males
movement. Squamous cell carcinoma of the tongue may
are commonly affected more than females in the ratio of
arise in apparently normal epithelium, in areas of leuko-
2:1. It is the most common primary tumor of paranasal
plakia, or in an area of chronic glossitis. These lesions
sinuses comprising 80–90% of cancers in this site. There is
are usually larger than 2 cm at presentation, with the
facial pain, swelling, nasal obstruction and lymphadenop-
lateral border being the most common subsite of origin.
athy. Medial wall involvement leads to nasal obstruction,
At this point, the patient may develop speech and swal-
discharge, bleeding and pain. Epiphora will result if the
lowing dysfunction. Pain occurs when the tumor involves
lacrimal sac or nasolacrimal duct is obstructed. Involvement
the lingual nerve, and this pain may also be referred to
of the floor of the sinus leads to expansion of the alveolus,
the ear.
unexplained pain, numbness of teeth, loose teeth and swell-
Carcinomas of the tongue base are clinically silent until
ing of the palate or alveolar ridge (Figure 19) and malfitting
they deeply infiltrate the tongue musculature. These are
dentures. It may erode the floor and penetrate the oral
usually less differentiated. Because of the difficulties with
cavity.
direct visualization, these may extend into the oral tongue
Lateral wall involvement leads to facial and vestibular
or have clinical lymph metastases before the diagnosis is
swelling, pain and hyperesthesia of maxillary teeth. Roof
established.
involvement leads to diplopia, proptosis and pain over
the cheek and upper teeth. Posterior wall involvement
Carcinoma of palate
leads to painful trismus as the pterygoids get involved,
It is common in areas where reverse smoking is practiced obstruction of Eustachian tube causing stuffy ear, referred
or also among people who use pipe smoke. Palatal cancer pain and hyperesthesia over the distribution of second and
usually manifests as a poorly defined ulcerated painful third divisions of trigeminal nerve. It may involve the
lesion on one side of the midline (Figure 18). Most other infraorbital nerve and produce paresthesia of the cheek or
lesions are exophytic and with broad base and nodular erode blood vessels giving rise to epistaxis. Paresthesia of
surface. It frequently crosses the midline and may extend mandibular nerve can also occur if the tumor invades the
laterally to include tonsillar pillars or even the uvula. The cranium.
tumor of hard palate may invade the bone or occasionally
the nasal cavity whereas infiltrating lesions of the soft Primary intra-alveolar carcinoma
palate may extend into the nasopharynx.
These lesions are rarely seen and surface only when they
invade the overlying mucosa either on the facial or the
Carcinoma of maxillary sinus
lingual/palatal side as an exophytic growth. Most often
It is caused by sinusitis, snuff and smoke abuse, occupa- they present only as a non-healing socket or as loose teeth
tional hazards like in boot factories, metal workers and which on radiographs appears as ‘hanging teeth’ with no
390
bony support. There may be evidence of paresthesia as the

Table 2 TNM stage grouping
nerve gets involved.
Stage T stage N stage M stage
Stage 0 Tis N0 M0
CLINICAL EXAMINATION OF A PATIENT Stage I T1 N0 M0
WITH SUSPECTED MALIGNANCY Stage II T2 N0 M0
Stage III T3 N0 M0
Since most of the lesions are innocuous, they escape early T1 N1 M0
detection and present only when it is too late. Good illu- T3 N1 M0
mination, access and aseptic techniques go a long way in Stage IV A T4a N0 M0
aiding proper diagnosis. T4a N1 M0
Patients should often be examined thoroughly for any T1 N2 M0
suspicious red or white lesions that have no explainable T2 N2 M0
etiology. If any etiology is found such as sharp teeth mar- T3 N2 M0
T4a N2 M0
gins or ill-fitting dentures and bad restorations, they should
be removed and all habits must be stopped. The lesions Stage IV B Any T N3 M0
should be reviewed on 2-weekly intervals and must be T4b Any N M0
recorded by photographic evidence. Oral therapy of anti- Stage IV C Any T Any N M1
oxidants may sometimes make the lesion disappear but
nevertheless the risk remains and the patient must be pres-
ent for 6-month follow-up.
After a proper history taking, suspicious or sinister look- TNM Staging System for Oral Carcinoma
ing lesions must be examined for: (i) extent of the lesion/
Primary tumor (T)
swelling; (ii) margin characteristics of the ulcerations if
any; (iii) presence or absence of induration around or on TX: Primary tumor cannot be assessed
the lesion; (iv) involvement of the overlying skin/mucosa T0: No evidence of primary tumor
(v) trismus; (vi) radiating pain to the ear; (vii) foul smell; Tis: Carcinoma in situ
(viii) loss of function/mobility as in the case of tongue T1: Tumor 2 cm or less in greatest dimension
with slurred speech and excessive salivation; (ix) paresthe- T2: Tumor more than 2 cm but not more than 4 cm in
sia or anesthesia of the nerve in the vicinity of the lesion; greatest dimension
(x) discharge from the nose or persistent post nasal drip; T3: Tumor more than 4 cm in greatest dimension
and (xi) diplopia of the eye on the affected side. T4 (oral cavity): Tumor invades through cortical bone,
inferior alveolar nerve, floor of mouth, or skin of face, i.e.
chin or nose
T4a (oral cavity): Tumor invades adjacent structures
TNM STAGING (e.g. through cortical bone, into deep [extrinsic] muscle of
tongue, maxillary sinus, skin of face)
The tumor-node-metastasis (TNM) staging system was first T4b: Tumor invades masticator space, pterygoid plates, or
reported by Pierre Denoix in the 1940s. The International skull base and/or encases internal carotid artery.
Union Against Cancer (UICC) eventually adapted the sys-
tem and compiled the first edition of the TNM staging sys- Note: Superficial erosion of bone/tooth socket by gingi-
tem in 1968 for 23 body sites. It is important to realize that val primary is not sufficient to classify a tumor as T4.
the TNM staging system is simply an anatomic staging
system that describes the anatomic extent of the primary
tumor as well as the involvement of regional lymph nodes Regional lymph nodes (N)
and distant metastasis. Tumor size and the extent of spread
NX: Regional lymph nodes cannot be assessed
are considered to be the best indicators of the patient’s prog-
N0: No regional lymph node metastasis
nosis. Table 2 summarizes the most widely accepted staging
N1: Metastasis in a single ipsilateral lymph node, 3 cm or
protocol, the TNM classification of oral cancer. This system
less in greater dimension
has three basic clinical features:
N2: Metastasis in a single ipsilateral lymph node, more
1. The size (in centimeters) of the primary tumor than 3 cm but not more than 6 cm in greatest dimension;
2. The presence, number, size, and spread (unilateral or in multiple ipsilateral lymph nodes, not more than 6 cm in
bilateral) to the local lymph nodes greatest dimension; in bilateral or contralateral lymph
3. The presence or absence of distant metastasis. nodes, not more than 6 cm in greatest dimension
391
N2a: Metastasis in single ipsilateral lymph node more in that regard are more inclined to metastasize compared
than 3 cm but not more than 6 cm in greatest dimension to exophytic tumors. It has been well documented that
N2b: Metastasis in multiple ipsilateral lymph nodes, not for tongue and floor of mouth cancers, tumor thickness is
more than 6 cm in greatest dimension related to the risk of nodal metastases. Poorly differentiated
N2c: Metastasis in bilateral or contralateral lymph nodes, carcinomas have a higher risk of nodal metastases com-
not more than 6 cm in greatest dimension pared to well-differentiated lesions. In general, if the risk of
N3: Metastasis in a lymph node more than 6 cm in greater occult metastases exceeds 15–20%, then elective treatment
dimension. of regional lymph nodes is recommended due to its signifi-
cantly adverse impact on prognosis. Risk of nodal metasta-
Distant metastasis (M) ses from cutaneous malignancies is highly variable. Small
MX: Presence of distant metastasis cannot be assessed size (2 cm) squamous cell carcinomas of the skin of the
M0: No distant metastasis face and neck have a very low risk of nodal metastases.
M1: Distant metastasis. Similarly, small cancers of the sweat gland or adnexal origin
also have a low risk of metastases to regional lymph nodes.
The individual clinical parameters in the TNM classification Therefore, elective treatment of regional lymph nodes is
system are grouped to determine the appropriate disease generally not recommended except for patients with mas-
stage; stages are ranked numerically from 0 (which has the sive tumors. On the other hand, cutaneous melanomas have
best prognosis) to IV (the worst prognosis). In general, oral a predictably high risk of nodal metastases with increasing
staging classifications do not use histopathologic findings thickness and site of the primary tumor. Therefore, one
except to determine the definitive diagnosis. can justify elective treatment of regional lymph nodes for
thicker primary melanomas although increasing popular-
ity of sentinel node mapping techniques negates the con-
NODAL METASTASIS sideration of ‘elective lymph node dissections’.
Metastatic spread from primary carcinomas of salivary
Involvement of regional lymphatic by primary squamous origin is generally low (20%). Therefore, elective dissec-
cell carcinomas of the upper aerodigestive tract is depen- tion of cervical lymph nodes is recommended for high
dent on various factors related to the primary tumor. These stage (T3–T4) and high grade (poorly differentiated)
include the site, size, T-stage and location of the primary carcinomas.
tumor. In addition to this, histomorphologic features of the
primary tumor also influence the risk of nodal metastases.
The risk of nodal metastasis increases in relation to location Cervical Lymph Nodes
of the primary tumor, as one progresses from the anterior
Patients who present with tumors localized at the primary
to posterior aspect of the upper aerodigestive tract mean-
site without dissemination to regional lymph nodes enjoy
ing: the risk of the lips oral cavity oropharynx hypo-
an excellent prognosis. On the other hand, once dissemi-
pharynx. For tumors of the larynx and pharynx, the risk
nation to regional lymph nodes takes place, the probabil-
of nodal metastasis increases as it progresses from the
ity of 5-year survival, regardless of the treatment rendered,
center to the periphery, meaning the risk of regional lymph
reduces by nearly 50% of those patients with early staged
node metastasis from carcinoma of the true vocal cord is
patients. Clearly, therefore, the single most important
exceedingly small. The risk, however, increases as one
prognostic factor in the treatment of patients with squa-
progress for the vocal cords to the false vocal cords, aryepi-
mous cell carcinoma of the head and neck is the status of
glottic fold, pyriform sinus, and pharyngeal wall. Nearly
cervical lymph nodes. Thus, management of cervical
two-thirds of patients with primary carcinomas of the hypo-
lymph nodes becomes a vitally important component of
pharynx present with clinically palpable regional lymph
the overall treatment strategy for patients with cancers of
node metastasis.
the head and neck. The patients seeking surgical interven-
Certain primary sites have a significantly increased risk
tion for malignancies in the Indian scenario is only when
of nodal metastases compared to the other sites in the
it is in a very advanced stage, and all the levels of nodes
same region, for example, floor of mouth versus hard pal-
are involved and is almost inoperable.
ate in the oral cavity. In general, T-stage usually reflects
tumor burden or invasiveness and therefore the risk of
nodal metastases increase with increasing T-stage of the
Anatomy of Regional Lymphatics
primary tumor at any site. Similarly, tumor size (large ver-
sus small) increases the risk of dissemination to regional The lymphatic drainage from the scalp and skin of the
lymph nodes. head and neck region, the mucosa of the upper craniofa-
Certain histomorphologic features of the primary tumor cial regions, salivary glands, and the thyroid gland occurs
also increase the risk of nodal metastasis. Endophytic tumors to specific regional lymph node groups. Surprisingly, tumor
392
dissemination via regional lymphatic to these lymph node the digastric muscle and the inferior border of the body of
groups occurs in a predictable and sequential fashion too. the mandible. The lymph nodes adjacent to the subman-
Hence, specific regional lymph node groups should be dibular salivary gland and along the facial artery (facial
appropriately addressed in the treatment planning for any nodes) are included in this group.
given primary site. The plans should encompass all the Level II Upper jugular group: Lymph nodes around the
nodes both accessible to surgical ablation and those not upper portion of the internal jugular vein and the upper
accessible to surgery. part of the spinal accessory nerve, extending from the base
The anterior half of the scalp, the skin of the forehead of the skull up to the bifurcation of the carotid artery or
and the upper part of the face drain first to the preauricular, the hyoid bone (clinical landmark). The posterior limit for
periparotid and intraparotid lymph nodes are thus the first this level is the posterior border of the sternocleidomastoid
echelon lymph nodes. The initial drainage of the posterior muscle and the anterior border is the lateral limit of the
half of the scalp and the posterior aspect of the external ear sternohyoid muscle.
takes place to the post-auricular and suboccipital group of
lymph nodes. Cervical lymph nodes in the lateral aspect of Level III Mid-jugular group: Lymph nodes around the
the neck primarily drain the mucosa of the upper aerodi- middle third of the internal jugular vein from the inferior
gestive tract. These include the submental, prevascular facial, border of Level II up to the omohyoid muscle or the infe-
and submandibular group of lymph nodes located in the rior border of cricoid cartilage (clinical landmark). The
submental and submandibular triangles of the neck, deep anterior and posterior borders are the same as those for
jugular lymph nodes include the jugulo-digastric, jugulo- Level II.
omohyoid, and supraclavicular group of lymph nodes adja- Level IV Lower jugular group: Lymph nodes around the
cent to the internal jugular vein. Lymph nodes in the posterior lower third of the internal jugular vein form the inferior
triangle of the neck include the accessory nerve and the border of Level III up to the clavicle. The anterior and pos-
transverse cervical chain of lymph nodes in the floor of the terior borders are the same as those for Levels II and III.
posterior triangle of the neck. Parapharyngeal and retro-
pharyngeal lymph nodes are at risk of metastatic dissemi- Level V Posterior triangle group: Lymph nodes around
nation from tumors of the pharynx. the lower portion of the spinal accessory nerve and along
The central compartment of the neck includes the del- the transverse cervical vessels. It is bounded by the trian-
phian lymph node overlying the thyroid cartilage in the gle formed by the clavicle, posterior border of the sterno-
midline draining the larynx and perithyroid lymph nodes cleidomastoid muscle, and the anterior border of the
adjacent to the thyroid gland. Lymph nodes in the tracheo- trapezius muscle.
esophageal groove provide primary drainage to the thyroid Level VI Central compartment group: Lymph nodes in
gland as well as the hypopharynx, subglottic larynx, and the prelaryngeal, pretracheal (Delphian), paratracheal, and
cervical esophagus. Lymph nodes in the anterior superior trachoesophageal groove. The boundaries are: hyoid bone
mediastinum provide drainage to the thyroid gland and the to suprasternal notch and between the medial borders of
cervical esophagus, and serves as a secondary lymphatic the carotid sheaths.
basin for anatomic structures in the central compartment of
Level VII Superior mediastinal group: Lymph nodes in
the neck. Each anatomic subgroup of lymph nodes described
the anterior superior mediastinum and tracheoesophageal
above specifically serves as primary echelon lymph nodes
grooves, extending from the suprasternal notch to the
draining a specific site in the head and neck region. Thus,
innominate artery.
location of a palpable metastatic lymph node may often
indicate the potential source of a primary tumor.
Clinical features and diagnosis
The Head and Neck Service at Memorial Sloan Kettering
Cancer Center has described a leveling system dividing Lymph nodes in the head and neck region may be present
the lymph nodes in the lateral aspect of the neck into five for many reasons although the presence of a clinically
nodal groups of levels to establish a consistent and easily palpable, unilateral, hard, enlarged lymph node in the
reproducible, user-friendly method for description of adult should be considered metastatic until proven other-
regional cervical lymph nodes which establishes a common wise. The location of a palpable lymph node may point to
language between the clinician and the pathologist. The the potential site of a primary tumor. The important fea-
lymph nodes in the central compartment of the neck are tures to note during examination of the neck for cervical
assigned levels VI and VII. lymph nodes are the location, size, consistency, and num-
ber of palpable lymph nodes as well as signs of extracap-
Level I Submental group: The lymph nodes between the sular spread such as invasion of the overlying skin,
anterior bellies of the digastric muscles and above the hyoid fixation to deeper soft tissues, or paralysis of cranial
bone. Submandibular group: Lymph nodes in the triangu- nerves. Histologic diagnosis of metastatic carcinoma is usu-
lar area bounded by the anterior and posterior bellies of ally established by a needle aspiration biopsy and cytologic
393
examination of the smears. An enlarged metastatic cervi- Only 20–25% of patients with carcinoma of the parotid
cal lymph node may be the only physical finding present gland develop regional lymph node metastasis. The lymph
in some patients whose primary tumors are either micro- node groups at highest risk for early dissemination of
scopic or occult at the time of presentation. A systematic metastatic cancer from primary carcinoma of the parotid
search for a primary tumor should be undertaken in these gland are those in the preauricular, periparotid, and intra-
patients prior to embarking upon therapy for the meta- parotid regions as well as lymph nodes in the upper deep
static nodes. If a thorough head and neck examination, jugular chain and those in the upper spinal accessory chain
including fiberoptic nasolaryngoscopy, fails to show a pri- in the posterior triangle of the neck. Initial dissemination
mary tumor, then the diagnosis of metastatic carcinoma to of metastatic cancer from primary malignant tumors of
a cervical lymph node from an unknown primary (occult the submandibular salivary gland occurs at lymph nodes
primary) is established. in the supraomohyoid triangle. Thus, lymph nodes in the
submandibular triangle and upper and mid-jugular chain
of lymph nodes are at the risk of micrometastasis from
Patterns of Neck Metastasis
malignant tumors of the submandibular salivary gland in
Dissemination of metastatic cancer to regional lymph nodes the clinically negative neck.
from primary sites in the upper aerodigestive tract occurs Cutaneous malignant tumors of the scalp, such as squa-
in a predictable and sequential fashion. Thus, all regional mous carcinoma and melanoma, also spread to regional
lymph node groups are usually not at risk of nodal metasta- lymph nodes in a predictable fashion. A line joining the
ses initially from any primary site, in the absence of grossly helix of one ear to the helix of the opposite ear in a coronal
palpable metastatic lymph nodes. On the other hand, when plane separates the watershed areas of the scalp. Tumors
clinically palpable lymph nodes are present at the time of located anterior to this line metastasize to preauricular,
initial diagnosis, comprehensive clearance of all regional periparotid and anterior cervical lymph nodes (Levels I–
lymph node groups at risk is warranted. Understanding IV) and seldom metastasize to the posterior triangle of the
the sequential patterns of neck metastasis therefore greatly neck. On the other hand, primary tumors of the scalp poste-
facilitates surgical management of regional lymph nodes rior to this line metastasize to the suboccipital and postau-
in the clinically negative neck where the lymph nodes are ricular group of lymph nodes as well as those in the
at risk of harboring micrometastasis. posterior triangle of the neck and the deep jugular chain
For primary tumors in the oral cavity the regional lymph (Levels II–V).
nodes at highest risk for early dissemination by metastatic
cancer are limited to Levels I, II and III. This means the
Sentinel Node Mapping
regional lymph node groups are contained within the
supraomohyoid triangle of the neck. The lymph node The procedure of sentinel node mapping employs one or all
groups contained in the supraomohyoid triangle are: sub- of the following three techniques: (i) radioisotope scan imag-
mental, submandibular, prevascular facial, jugulodigas- ing; (ii) injection of blue dye; and (iii) use of a handheld
tric, upper deep jugular, superior spinal accessory chain of isotope tracer probe for localization. It has been shown that
lymph nodes, and mid-jugular lymph nodes. Skip metas- the combination of all three techniques increases the accu-
tasis to Levels I, II, or III is exceedingly rare. Therefore, if the racy and the yield of sentinel lymph node identification.
neck is clinically negative, Levels IV and V lymph nodes A preoperative technetium scan is obtained first. This
are generally not at risk of harboring micrometastasis requires injection of radioactive technetium (99mTc) labeled
from primary squamous carcinomas of the oral cavity. An sulfur colloid. Generally, 0.05 mCi of the isotope is injected
exception to this observation is primary squamous cell in four quadrants around the primary lesion and a gamma
carcinomas of the middle third of the lateral border of the camera is used for obtaining visual images at 3 minutes,
tongue where skip metastases to Level IV have been 15 minutes, and a delayed image at 1 hour. Usually, the
reported. first lymph node identified by the technetium scan is con-
Tumors on the lateral aspect of the oropharynx, hypo- sidered the sentinel lymph node. In some patients, more
pharynx and larynx, the first echelon lymph nodes are the than one sentinel lymph node is identified.
deep jugular lymph nodes at Levels II, III and IV on the Immediately prior to the surgical procedure, isosulfan
ipsilateral side and they are at highest risk of harboring blue dye 1% (Lymphazurin®) is injected similarly in four
micrometastasis in the clinically negative neck. Primary quadrants around the primary tumor (Figure 18). No more
tumors which involve both sides of the midline have a than 0.5 ml of the dye is injected into the subdermal plane
potential of microscopic dissemination of metastatic disease around the tumor. The operative procedure then is carried
to jugular lymph nodes on both sides of the neck. Similarly, out within 30 minutes of the injection. A handheld gamma
tumors of the medial wall of the pyriform sinus are probe is used before placing the incision to localize the
reported to have an increased risk of contralateral neck lymph node seen on the preoperative scan. The background
metastases. activity is averaged from measurements in four quadrants
394
of the neck and any node that has an in vivo 10-second ViziLite Plus is another popular screening tool for detec-
count more than three times that of the background is con- tion of oral cancers. As ViziLite Plus is passed over oral
sidered ‘hot’. Correlation is made with the preoperative scan tissue that has been treated with the rinse solution, normal
and this area is marked out with a marking pen on the skin. healthy tissue will absorb the light and appear dark,
An incision is placed directly overlying the localized sen- abnormal tissues will appear white.
tinel lymph node and by careful alternate blunt and sharp Venkatakrishna et al (2003) developed a HPLC-LIF
dissection, the ‘blue node’ is localized. The handheld probe technique to detect and record simultaneously spectra and
is again used to measure the count of highest radiotracer chromatograph of physiological samples. This system
activity. If the blue node corresponds to the area of highest enables the detection of multiple ‘markers’ in a single phys-
radiotracer activity, then the lymph node is excised and iological sample in a short time. Samples of saliva and
sent for pathologic analysis. The surgical area after exci- serum from normal and oral cancer subjects have been
sion of the lymph node is tested with the handheld probe studied with the set-up. Their study showed that body fluids
to show that the radiotracer activity is now reduced to that like saliva and serum of normal, premalignant and malig-
observed in the adjacent background area, confirming that nant subjects had substantially different protein profiles.
the true ‘sentinel lymph node’ has been excised and sent They suggest that by simultaneous recording of the
for pathologic analysis. If the residual radioactivity in the chromatographic peaks and corresponding fluorescence
basin is more than 10% of the ex vivo count of the hottest spectra, it is possible to carry out unambiguous discrimi-
node in the basin, further exploration to find more senti- nation between normal, premalignant and malignant
nel nodes is warranted. The radiotracer activity is measured cases even when markers are present in femto/subfemto
with a handheld probe from the lymph node ex vivo to mole quantities, which should assist in early diagnosis of
demonstrate that the lymph node itself has a count at least neoplasia.
10 times that of an adjacent non-sentinel node. This ensures
that the excised lymph node is indeed a true sentinel lymph
node. At present, opinions regarding the value of frozen Imaging Modalities
section for a sentinel node vary. Because of the special pro- Plain radiographic examination may include the use of
cessing required by sub-serial sectioning of the lymph node OPG, occlusal and intraoral periapical radiographs. Para-
to identify occult metastasis some investigators prefer not nasal sinus view is useful for assessing extent of bone
to send the lymph node for frozen section and wait for ‘rush involvement in the maxillary sinus region.
paraffin sections’ within 24 hours to get a more detailed
analysis of the sentinel lymph node. Further surgical man- Plain radiographs
agement of regional lymph nodes then depends on the
pathologic analysis of the excised sentinel lymph node. Intraoral periapical radiographs are useful to assess finer
details such as subtle bone invasion, bony trabecular
architecture (Figure 20) and destruction of the lamina
DIAGNOSIS OF ORAL CANCER dura. Occlusal radiographs (Figure 21) may help in evalu-
ating destruction of bone in the anterior portion of the
jaws which cannot be assessed adequately in an OPG. The
Various diagnostic tests can be employed to detect poten-
mandible is more commonly affected than the maxilla.
tially malignant and malignant lesions. In routine practice,
The typical radiographic features of carcinoma invading
vital staining, brush biopsy, exfoliative cytology, tissue
bone include: ill-defined destruction (moth-eaten appear-
biopsy and various imaging modalities (plain radiographs,
ance) of bone with ragged borders (Figure 22), cortical
CT, MRI, ultrasonography, etc.) can be used effectively.
plates may be thinned out leading to a pathological frac-
Newer diagnostic tools such as VELscope and ViziLite
ture (Figure 23), extensive lesions may cause destruction
Plus, Raman spectroscopy, high performance laser spec-
of floor of the nasal fossa, maxillary sinus (Figure 24), and
troscopy-laser induced fluorescence (HPLC-LIF) also play a
the cortical lining of the mandibular canal. Lamina dura
significant role in the early diagnosis of oral malignancies.
around teeth are lost. However, roots of teeth show no
The VELscope is based on the direct visualization of
signs of resorption as seen in benign tumors. This destruc-
tissue fluorescence and the changes that occur when abnor-
tion of bone around teeth gives rise to ‘floating in space’
mal cells are present. The VELscope handpiece emits a safe
appearance (Figure 25).
blue light into the oral cavity, causing tissue fluorescence
from the surface of the epithelium through to the basal
CT, MRI and PET
membrane—where pre-malignant changes typically start.
By utilizing special optical filters in the VELscope hand- Cross-sectional imaging modalities such as CT and MRI
piece the clinician is able to immediately view the different help in assessing the size and extent of a tumor in three
fluorescence signatures in the oral tissue to help differen- dimensions. MRI imaging is considered more sensitive in
tiate between normal and abnormal cellular activities. evaluating the possibility of intracranial invasion. On the
395
Figure 20 Figure 22
Orthopantomograph showing ill-defined destruction of

bone with ragged borders in the anterior portion
of the mandible. Courtesy: Department of Oral Medicine and
Intraoral periapical radiograph showing fine details of Radiology, Manipal College of Dental Sciences, Mangalore
loss of normal trabecular architecture in carcinoma involving
bone. Courtesy: Department of Oral Medicine and Radiology,
Figure 23
Figure 21
Pathological fracture. Courtesy: Department of Oral Medicine

and Radiology, Manipal College of Dental Sciences, Mangalore
Lower occlusal radiograph showing destruction of bone in the Metastatic nodes can be differentiated from normal
anterior portion of the mandible. Courtesy: Department of nodes by their size, presence of central necrosis, rim
Oral Medicine and Radiology, Manipal College of Dental enhancement and extranodal invasion. The size criterion
Sciences, Mangalore for nodes in the neck which are considered radiologically
positive for metastasis is as follows: nodes larger than
15 mm in the longitudinal diameter in the jugulodigastric
area and submandibular triangle, other nodes in the neck
other hand, the extent of bony involvement by the tumor are considered radiologically positive if larger than 10 mm.
mass is best assessed with CT. Extensive metastasis However, when retropharygeal nodes are larger than 8 mm
(involvement of carotid artery, cranium, parapharyngeal they are considered positive. When axial diameters are
space, etc.) may be evaluated using a CT scan with intra- considered, 11 mm and above is regarded positive for jug-
venous contrast or an MRI scan with gadolinium contrast. ulodigastric nodes and 10 mm and above for other nodes
Lymph nodes which are not amenable for clinical exam- in the neck.
ination (retropharyngeal and parapharyngeal nodes) or for The shape of the node can be denoted in terms of the
FNAC may be assessed using CT and MRI. LT ratio (longitudinal and transverse diameters). An LT
396
Ultrasound, specially using high resolution probes

Figure 24
(7.5–15 MHz) has always been considered a powerful tool
for assessment of cervical lymph nodes. Apart from locat-
ing lymph nodes, ultrasonography can evaluate their num-
ber, shape, dimensions, margins, and internal structure.
Vital staining
In 1964, Niebel and Chomet first reported the use of tolu-
idine blue (tolonium chloride) as a vital tissue stain to aid
in the early detection of oral precancerous and malignant
lesions. Though it is not cancer specific, it has been
reported to stain mitochondrial DNA, altered DNA in pre-
Orthopantomograph showing destruction of the alveolus malignant and malignant epithelial lesions and cells with
in the right molar region with involvement of the floor relatively increased amounts of DNA. Warnakulasuriya
of the maxillary sinus. Courtesy: Department of Oral
and Johnson (1996) reported that toulindine blue-positive
Medicine and Radiology, Manipal College of Dental
lesions with minimal or no identifiable dysplasia on initial
Sciences, Mangalore
biopsy were almost four times more likely to transform to
carcinoma than lesions found to be toluidine blue-negative.
Toluidine blue is also an useful adjunct to clinical exam-
ination and biopsy. Toluidine is a basic metachromatic dye
Figure 25
that stains for acidic tissue components and thus binds
more readily to DNA. In addition, it can help to determine
the most appropriate biopsy sites and to surgically delineate
margins. Meta-analysis of toluidine blue staining in oral
cancer screening found that its sensitivity ranged from
93.5 to 97.8%, and specificity from 73.3 to 92.9%.
TBlue630 is a patented, pharmaceutical-grade toluidine
blue-based metachromatic dye. It provides the deep blue
staining that allows identified lesions to be seen clearly
under normal light.
Orthopantomograph showing extensive destruction of

Exfoliative cytology
bone in the anterior portion of the mandible extending
to the molars on either side. Blue arrow indicates It is a known fact that dysplastic and cancerous cells tend
thinning of the lower cortical plate. White arrows to have fewer and weaker connections to each other and to
show ‘teeth floating in space’. Courtesy: Department their neighboring normal cells in the surrounding tissue.
of Oral Medicine and Radiology, Manipal College of
Dysplastic and cancerous cells therefore, tend to ‘slough off’
or exfoliate preferentially and can easily be collected from
the surface of the lesion. A sample of these cells applied
to a microscope slide will often contain abnormalities if
ratio of two nodes suggests metastasis (a rounded node is obtained from a dysplastic or cancerous lesion.
considered positive compared to a bean-shaped node). Sample can be obtained using firm strokes with a tongue
A newer imaging tool that is gaining favor among radi- blade, cement spatula or a newer technique using a brush
ologists is the positron emission tomography (PET). PET is (referred to as brush biopsy).
advantageous for the fact that it not only helps in local- The cytology is reported as:
izing a lesion but even demonstrates its metabolic activity.
❍ Class I (normal)—normal cells.
As malignant cells are known to divide rapidly, their
❍ Class II (atypical)—indicates the presence of minor
increased activity is easily picked up by the PET scan.
atypia but no malignant changes.
❍ Class III (indeterminate)—the cells display wider atypia
Distant metastasis imaging
that may be more suggestive of cancer, pre-cancer, or
Distant metastasis from the oropharynx can be assessed carcinoma in situ. Biopsy is recommended.
using chest radiography (traditional/CT), abdominal ultra- ❍ Class IV (suggestive of cancer)—a few cells with malig-
sonography (to evaluate liver metastasis), radionuclide nant characteristics or many cells with borderline
imaging and blood studies (tumor markers). characteristics. Biopsy is mandatory.
397
Brush biopsy Figure 26

This technique was introduced in 1999 as a substitute to
Normal epithelium
the conventional exfoliative cytology procedure. A com-
mercially available kit is used (oral CDX).
This biopsy method utilizes a brush to obtain a complete
transepithelial biopsy specimen with cellular representation Dysplastic epithelium
from each of the three layers of the lesion: the basal, interme-
diate, and superficial layers. Unlike previous cytologic instru-
ments, which collect only exfoliated superficial cells,
Islands of tumor
when used properly and rubbed against an area of suspect epithelium
tissue aggressively (to the point of minor bleeding) the
biopsy brush penetrates to the basement membrane, remov-
ing tissue from all three epithelial layers of the oral
mucosa. The oral brush biopsy does not require topical or
local anesthetic and causes minimal bleeding and pain. The Keratin pearl
brush biopsy instrument has two cutting surfaces, the flat
end of the brush and the circular border of the brush. Either
surface may be used to obtain the specimen. In a recent
study, paired, same site samples of tongue tissue were Histopathological features of squamous cell carcinoma.
obtained from patients, first by brush biopsy and then by Courtesy: Department of Oral Pathology,
surgical punch biopsy. The study demonstrated that the MCODS, Mangalore
brush biopsy technique, unlike cytology, sampled the full
thickness of oral epithelium.
Results of brush cytology specimen are classified into 2. Moderately differentiated squamous cell carcinoma
one of the four categories: 3. Poorly differentiated squamous cell carcinoma.
1. Inadequate: incomplete trans-epithelial specimen
2. Negative: no epithelial abnormality Well-differentiated squamous cell carcinoma
3. Atypical: abnormal epithelial changes of uncertain It consists of sheets and nest of cells with characteristic
diagnostic significance appearance of squamous origin. These cells are generally
4. Positive: definitive cellular evidence of epithelial dys- large and show distinct cell membrane. These resemble the
plasia or carcinoma. cells of squamous epithelium, both structurally and func-
tionally. The intercellular bridges are prominent (Figure 26).
Histopathologic features The nuclei are distinctly dark staining. Increased num-
ber of mitotic figures but relatively few when compared to
All suspicious lesions should be subjected to an excisional moderately differentiated carcinoma. It also shows hyper-
or incisional biopsy and the specimen should be subjected chromatism prominent and multiple nucleoli and increased
to histopathological evaluation. nucleocytoplasmic ratio. Most prominent features are the
In general, all carcinomas exhibit the following features: presence of individual cell keratinization and formation of
epithelial dysplasia, keratinization (varies with degree of numerous keratin pearls of varying size.
differentiation), local invasion by break in the basement Each keratin pearl consists of a central area of keratin
membrane and invasion and proliferation into the under- surrounded by whorls of prickle cells. Pleomorphism of
lying connective tissue (clinically manifested as fixation cells, keratinization, and keratin pearls deep to epithelial
and induration of the lesion), metastasis by blood or lym- surface and loss of intercellular bridges or cohesiveness may
phatic channels histopathologically seen as invasion of be seen.
tumor cells into the capillaries and lymphatic ducts per-
meation and perineural invasion—some of the tumor cells Moderately differentiated/less well-differentiated
may have atypical invasion pattern along the nerve squamous cell carcinoma
sheath.
The histopathological features are graded into three The tumor cells are less differentiated and have less resem-
types depending upon the degree of differentiation of the blance to squamous epithelium. The characteristic shape of
neoplastic proliferating cells. They are: an epithelial cell may not be evident. The cell to cell con-
tacts and relation and arrangement are altered.
1. Well-differentiated or highly differentiated squamous The greater number of mitotic figures shows that the
cell carcinoma growth rate is more rapid. These may be of varied size and
398
shape. Keratin pearls may not be present. Numerous epi- may not be microscopically involved) and therapeutic
thelial islands of prickle cells with peripheral basal cells (clinically palpable nodes and presumed to be microscopi-
may be seen. cally involved).
Commando surgery A combined resection wherein the pri-
Poorly differentiated squamous cell carcinoma
mary tumor, affected lymphatics and involved adjacent
This is the tumor with proliferation of anaplastic cells, structures are removed is referred to as a commando surgery.
highly invasive with poor prognosis. The tumor cells bear
Microscopically frozen–section oriented histologic surgery
little resemblance to their cells of origin and often will
(MOHS) technique In this technique, surgically resected
present diagnostic difficulties because of the primitive and
specimens are immediately frozen and histologically ana-
uncharacteristic histologic appearance. These cells show
lyzed to assess for clear margins. This immediate assessment
lack of cohesiveness and are extremely vagarious. The
will aid in removing all tumor cells.
mitotic figures are extremely high.
Radiation therapy Radiotherapy plays an important role
Management in the management of head and neck cancer. The majority
of new cases of invasive head and neck cancer will need
There are three recognized treatment modalities for manag-
radiotherapy as a primary treatment. This radiation, in
ing head and neck cancers: surgery, radiotherapy and che-
effect, disrupts the electron orbital structure of tissue atoms,
motherapy. As a thumb rule, Stage I and Stage II cancers
which subsequently damages individual cells and tissue.
can be managed either by surgery or radiotherapy. However,
Normal tissue function depends primarily on cell integrity
Stage III and Stage IV cancers are managed using a com-
and viability, as well as on the ability of the cells to replace
bination of radiation therapy and surgery.
and maintain their structure and organization. Cells are
Based on the nature of the tumor, the treatment modal-
most vulnerable to injury when they are in the process of
ity is chosen. Factors that influence the choice of treat-
dividing and multiplying.
ment include: site and location of the primary (tongue,
The ionizing radiation exerts its effect on cells by dis-
floor of mouth/anteriorly or posteriorly), size of the tumor
placing the electrons from the molecules and atoms with
(based on T stage), proximity to the bone, status of nodal
which they collide, causing ionization and inducing a cas-
involvement, histological typing of the tumor (cell type
cade of events that may alter the cells transiently or per-
and degree of differentiation), ability to achieve adequate
manently. The most important target of ionizing radiation
surgical margins and preserve functions, physical and
is DNA. The radiation may damage the DNA directly (Direct
mental status of patient and history of any treatment.
Target Theory), or indirectly by inducing the formation of
Surgical management Surgical treatment aims at com- free radicals, particularly those that form from the radioly-
plete removal of the primary as well as the metastatic nodes. sis of water (Indirect Target Theory). Other cell molecules
It is never used as a palliative mode of treatment. Criteria that may also be directly or indirectly damaged include
for choosing surgical management are: tumors which are the lipids in cell membrane and proteins that function as
non-sensitive to radiation, tumors involving bone, recur- critical enzymes.
rent tumors in sites that have been previously irradiated, In addition to anti-tumor effects, ionizing irradiation
to reduce bulk of tumor prior to radiation therapy (debulk- causes damage in normal tissues located in the field of
ing), where side effects of surgery are expected to be less radiation. This becomes particularly evident in the head
significant than those associated with radiation and when and neck region, a complex area composed of several dis-
nodes are to be removed. similar structures that respond differently to radiation:
Surgical management aims at clearance of both the mucosal linings, skin coverings, subcutaneous connective
primary lesion and the involved regional lymphatics. tissue, salivary gland tissue, teeth, and bone/cartilage.
The extent of resection of the primary lesion depends Acute changes produced by radiotherapy are observed in the
upon the size and the adjacent structures that may have oral mucosa (erythema, pseudomembrane-covered ulcer-
been infiltrated. ation), salivary glands (hyposalivation, changed salivary
composition), taste buds (decreased acuity), and skin (ery-
Neck dissection (cervical lymphadenectomy) In this pro-
thema, desquamation).
cedure, all lymph nodes are removed beginning from the
lower border of the mandible superiorly to the clavicular Radiation dose The radiation dose needed for the treat-
region inferiorly, and from the trapezius muscle posteri- ment of cancer is based on location and type of malig-
orly to the midline anteriorly. The sternocleidomastoid nancy, and whether or not radiotherapy will be used solely
muscle, omohyoid muscle, jugular vein and submandibular or in combination with other modalities. Most patients with
salivary gland are resected. Based on the clinical involve- head and neck carcinomas, treated with a curative intent,
ment of lymph nodes, neck dissections can be categorized receive a dose between 50 and 70 Gy. This dose is usually
as prophylactic (clinically non-palpable nodes, may or given over a 5- to 7-week period, once a day, 5 days a week,
399
2 Gy per fraction. The total dose for preoperative radio- dose to the opposite side. When a large tumor or midline
therapy or radiotherapy for malignant lymphomas is usu- lesion is present, a parallel-opposed field set-up or three-
ally lower. field set-up may be needed, which produces relatively uni-
form exposure for midline disease.
Fractionated radiation Fractionated radiation is used
because there is a difference in the responses of tumor tis- Brachytherapy Brachytherapy involves inserting radioac-
sue and normal tissue. In general, normal tissue repairs tive wires/seeds directly into a tumor, such as in the tongue.
sublethal DNA damage, especially in the low-dose range, One of the advantages of this type of therapy is there is
better than tumor tissue. The sparing effect of fractionated less radiation exposure to other parts of the body. In inter-
radiation is the largest for late-responding tissues, whereas stitial implant treatment, implants deliver the radiation in
early-responding tissues respond more like tumor tissue. the immediate vicinity of the implanted isotope and also
Next to DNA-repair advantages, fractionated irradiation deliver it a relatively low dose rate, thus sparing the normal
allows for the re-population of tissue between fractions tissues. Interstitial sources can be placed directly or prefer-
(especially during the weekend, when the tumor and normal ably, after loaded into tubes or needles. The sources most
tissues are not radiated), thereby reducing early effects. commonly used include 192Ir,137Cs,125I, and 198Au.
This, however, also applies for rapidly proliferating malig- Another variant of these radioactive implants are appli-
nant tissue. Another advantage is that fractionated irradia- cators which are radioactive substances that are impreg-
tion allows for re-oxygenation of radio-resistant hypoxic nated into prosthesis which may be placed on the surface
tumors between fractions, leading to a higher percentage of the cancerous tissue.
of radiosensitive oxygenated cells.
Newer modalities of radiation therapy Intensity modu-
Ideal candidates for radiotherapy Small lesions less than lated radiation therapy (IMRT) is most applicable in the
3 cm and superficial without necrotic areas can be readily treatment of head and neck cancers (especially nasopha-
managed with radiation therapy. The choice of treatment ryngeal lesions). IMRT employs a computer directed radia-
also depends on functional and cosmetic concern. If tion source that targets the cancer more accurately than
regional excision can be accomplished without much mor- conventional radiation therapy. This is accomplished with
bidity, surgery should be the treatment of choice. On the computer algorithms to design treatment plans whose goals
contrary, if the lesion involves large areas like tongue, are to maximize dose to tumor targets while limiting dose
floor of the mouth or buccal mucosa, and if excised it to normal structures. Thus, the major advantage of IMRT
would result in morbidity, thus local irradiation would be over conventional head and neck radiation is the potential
the treatment of choice. But if the lesion is invasive involv- to significantly limit dose deposition to normal structures,
ing the bone, radiation sterilization of the tumor is not such as the parotid glands, thereby reducing the degree
possible without extensive normal tissue damage, there- of xerostomia. A potential disadvantage of IMRT is an
fore surgery or a combination of irradiation and surgery increased volume of normal tissue exposure. This is because
may be required. Lesions that are extremely radiosensitive multiple small fields of radiation, called beamlets, rotate
such as lymphomas, radiation therapy should be the treat- around the patient.
ment of choice. If the location and clinical behavior of a Additional ability to modify dose and fields and radia-
lesion indicates significant lymphatic permeation, then tion has been introduced with image-guided radiotherapy
radiotherapy alone or in combination with surgery is the (IGRT) in which integrated CT, guides the changes in radi-
treatment of choice as a wider volume. ation throughout the course of treatment as treatment causes
a change in volume of tumor during treatment.
Radiation technique Radiation therapy may be adminis-
tered to a localized lesion by using brachytherapy (applica- Radiation therapy combined with chemotherapy The
tors or implants) or to a region of the head and neck by rationale for administering drugs prior to radiation ther-
using teletherapy (external beam radiation). apy is to control already disseminated tumor cells, poten-
tiate the killing effects of ionizing radiation, be more
External beam radiation therapy The source of radiation
effective against the remaining tumor growth fraction once
can either be low energy (orthovoltage: 50–300 kVp) or
radiation has reduced tumor bulk and elimination of sub-
high energy (cobalt-60 or linear accelerators of 4 million
clinical metastasis.
electron volts). Low energy beams are used for small sized
intraoral tumors, lip, and skin cancers. The high energy Effects of radiation therapy Following radiation therapy
radiation provides variable penetration due to its ability to various mucocutaneous changes are seen in the patient.
vary the energy of the photons. It spares bone and skin. Some of these changes include: hyperpigmentation of the
For teletherapy, three principal field arrangements are skin surface in the field of radiation, transient loss of hair,
planned: wedged-pair fields, parallel-opposed fields and mucositis, loss of taste, salivary dysfunction, radiation car-
three-field technique. The wedged-pair field allows a ies, fungal infections, pain, post-radiation osteoradione-
therapeutic dose to unilateral disease while sparing a high crosis, and difficulty in speech and mastication.
400
Mucocutaneous changes In about 2 weeks following erythematous candidiasis. In such patients, topical appli-
radiation therapy the skin in the radiation field turns ery- cation of anticandidal agents may not be effective as the
thematous and may show hyperpigmentation along with salivation is compromised.
mucositis. Radiation dose of about 180–220 cGy per day is
Osteoradionecrosis Radiation therapy causes endarteritis
sufficient to produce mucositis. It is characterized by intense
obliterans, which in turn results in hypovascularity, hypo-
erythema of the mucosa, pain and occasionally associated
cellularity and finally hypoxic tissue. Owing to its inher-
with sloughing.
ent poor vascularity, the mandible is more commonly
Topical anesthetics in the form of viscous xylocaine,
affected than the maxilla.
topical analgesics (benzydamine hydrochloride) are effective
Trauma (external, iatrogenic), periodontal diseases, ill-
in managing radiation mucositis.
fitting dentures, peridontally weak or pulpally affected
Loss of taste sensation When the taste buds are in the teeth in the line of radiation are predisposing factors for
radiation field there is partial or complete loss of cells in osteoradionecrosis.
taste buds. Patients may report of a significant change in Patients complain of pain, foul taste, paresthesia/
the perception of all types of tastes. In most patients, the anesthesia and intraoral or extraoral discharging sinuses.
cells of the taste buds regenerate in 4 months duration. Lymphadenopathy may be seen. As the condition worsens,
Patients may be advised to incorporate a change in diet pathological fracture occurs. Radiographically, it is difficult
to facilitate better taste perception. Zinc sulfate capsules to differentiate between the ill-defined patchy destruction
(220 mg twice daily with food, elemental zinc 100 mg) are of bone that is seen in cancer of the bone, osteoradione-
beneficial. crosis and suppurative osteomyelitis.
As a preventive measure, if extractions are planned, it
Salivary function Radiation induces fatty degeneration, is desirable to allow as much healing time as possible.
fibrosis, acinar atrophy and cellular necrosis within the Seven to 14 days and up to 21 days have been suggested
salivary glands. Comparatively, serous acini are more radio- as healing times prior to radiotherapy.
sensitive than mucous acini. Patients present with reduced Patients should be instructed to avoid mucosal irritants,
amount of salivation and the saliva is typically thick and discontinue the use of dental appliances if they contact the
ropy. Radiation-induced xerostomia may occasionally area of the lesion, maintain nutritional status, stop smoking
reverse in about 12 months. However, irreversible changes and alcohol consumption.
are seen at a total dose of 6,000 cGy for 5 weeks. Patients Osteoradionecrosis is best managed with topical antibiotic
are advised to frequently sip water and chew on sugarless (tetracycline) or antiseptic (chlorhexidine) rinses. Hyper-
gum to stimulate salivation. If the xerostomia is very severe, baric oxygen (HBO) therapy increases the oxygenation of
salivary substitutes such as carboxymethylcellulose can tissue, increases angiogenesis, and promotes osteoblast and
be prescribed. Salivary stimulants such as pilocarpine fibroblast function.
hydrochloride (5 mg 4 times a day) and bethanechol HBO therapy (Figure 27A, B) is usually carried out for a
(25–200 mg/day) have also proven to be beneficial. period of 20–30 dives at 100% oxygen and 2–2.5 atmo-
Radiation caries Radiation caries occurs secondary to spheres of pressure.
the alteration in the chemical composition of saliva or Sequestrated bone may be managed with limited resec-
reduction in salivation following radiotherapy and not tion or may require mandibulectomy. If surgery is required,
due to the direct action of radiation on the tooth. The most postsurgical HBO therapy of 10 dives is recommended.
common sites of involvement are the cervical areas fol- Chemotherapy It is provided as an induction therapy
lowed by cuspal/incisal tips of teeth. Three distinct types before local therapies. It is also an adjunctive modality for
are seen: dark pigmentation of crown, circumferential other cancers. The objective of induction chemotherapy is
caries/amputation caries (generally involves cervical third) to promote initial tumor reduction and to provide early
and widespread superficial caries especially involving the treatment of micrometastases. The goal of chemotherapy
smooth surfaces of the crown. is to eradicate the rapidly growing cells of the tumor or
Patients should be encouraged to maintain good oral modify their growth. Chemotherapeutic agents affect the
hygiene. Three percent hydrogen peroxide rinses and topi- rapidly dividing cells of the target tumor and the lining
cal fluoride application will help in preventing further dam- epithelium, the oral ecology and the vascular, inflamma-
age to the tooth structure. tory and healing responses of the oral cavity. These altera-
Effects on developing teeth Tooth eruption may be tions may result in mucositis and ulceration of the oral
delayed. The teeth may be malformed with stunted roots. mucosa. Chemotherapeutic agents also target the hemo-
Occasionally, the tooth may not be formed. poietic cells of the bone marrow, resulting in anemia,
thrombocytopenia and leukopenia. Unfortunately, chemo-
Candidiasis Owing to the poor oral hygiene and nutritional therapy is often toxic to other cells that rapidly divide
status, patients may present with pseudomembranous or normally. These include bone marrow, hair, and the
401
and their precursors; rapid nucleic acid synthesis occurs

Figure 27
during cell division.
A Drugs used in oral cancer chemotherapy Various drugs are
seen in cancer chemotherapy of the head and neck such as
cetuximab (epidermal growth factor receptor inhibitor),
docetaxel, cisplatin, 5-fluorouracil, leucovorin, methotrex-
ate, and bleomycin.
We shall describe four drugs in the following text namely:
methotrexate, bleomycin, cisplatin and 5-fluorouracil.
Methotrexate Methotrexate is an antimetabolic and an
inhibitor of folic acid metabolism. The rationales for intra-
B arterial route are that, prolonged contact of the tumor cell
population with the antimetabolite will result in sequential
death of cells as the cells enter the most fragile metabolic
phase of cellular division. It can be administered either
intra-arterially or per orally. Systemic methotrexate is
given in intermittent weekly or semiweekly IV injections
of 40–60 mg/m2 of body surface area.
Methotrexate is known to cause bone marrow suppres-
sion, mucositis, anorexia, nausea, emesis, renal toxicity and
hepatic dysfunction.
Bleomycin It is part of a group of antibiotics that was
isolated in Japan from Streptomysis verticillus. Bleomycin
acts by interfering with the DNA function of the cell. The
ideal dose of belomycin is 0.25–0.50 unit/kg given weekly
or twice weekly. However, care should be taken not to
(A) Patient being shifted into the HBO chamber. exceed a dose of 400 units and this dose can cause pulmo-
(B) Doctor monitoring the patient’s condition undergoing nary toxicity and death. Some patients may complain of
hyperbaric oxygen (HBO) therapy. Courtesy: Sechrist skin rashes and erythema. The greatest advantage with
Industries, Inc., La Palma Ave, Anaheim, CA 928 bleomycin is its lack of causing myelosuppression.
Cisplatin Cisplatin is a relatively new drug which acts by
altering DNA structure. It is given IV (80–120 mg/m2, IV
mucosa of the entire gastrointestinal tract, including the
for 3–4 weeks). Dehydrated patients may suffer from renal
oral cavity.
impairment. Hence, it is important to maintain adequate
The chemotherapy cycle consists of cytotoxic drug
hydration before the drug is administered.
administration followed by a rest period for healthy tissue
recovery prior to repeated drug administration. An impor- 5-Fluorouracil This drug is usually administered along
tant clinical implication of this format is that peripheral with cisplatin. Its acts by interfering with the cellular
white blood cell counts change dramatically during the metabolism. The standard dosage is 1,000 mg/m2/day.
course of this cycle. Majority of the cytotoxic drugs have Stomatitis is a common side effect. Bone marrow suppres-
more profound effect on rapidly multiplying cells, because sion is seen when weekly or daily bolus injections are
the most important target of action are the nucleic acids administered.
402
SECTION Teeth and
VI Periodontium
15 Dental Caries, Pulp and Periapical Lesions 405

16 Gingival and Periodontal Diseases 440
17 Regressive Alterations of Teeth 458
403
Chapter-15.indd 403 10/3/2012 2:04:18 PM


CHAPTER
Dental Caries, Pulp and
Periapical Lesions
Vivekananda Pai, Ravikiran Ongole
15
➧ Definition and Etiology ➧ Radiographic Evaluation of Carious Lesions
➧ Contributory Factors in Dental Caries ➧ Fascial Space Infection
➧ Classification of Dental Caries ➧ Ludwig’s Angina
➧ Microbiology of Dental Caries ➧ Osteomyelitis
➧ Clinical Diagnostic Methods
DEFINITION AND ETIOLOGY According to Miller, the carious process occurs in two
distinct steps: in the initial stages there is decalcification
Dental caries is a microbial disease of the calcified tissues of enamel and destruction of dentin, and in the second
of the teeth, characterized by demineralization of the inor- stage there is dissolution of the softened residue of the
ganic portion and destruction of the organic substance of enamel and dentin.
the tooth. The acidic attack which causes destruction and dissolu-
The World Health Organization (WHO) defines caries as tion of the residue is carried by the proteolytic action of the
a localized post-eruptive, pathological process of external bacteria. This two-step process is supported by the pres-
origin involving softening of the hard tooth tissue and ence of carbohydrates, microorganisms and dental plaque.
proceeding to the formation of a cavity.
Dental caries is derived from the Latin word caries which Role of carbohydrates
means decay or rotten.
According to Miller’s observations, teeth decalcify when
Etiology incubated in saliva and bread/sugar mixture and show no
change when incubated with fat. His simple experiment dem-
Dental caries is considered as the most prevalent disease in onstrated the cariogenic effects of carbohydrates.
humans, second only to the common cold. Dental decay is However, the cariogenic potential of dietary carbohy-
complex and multifactorial. Various theories have been pro- drates varies on their physical form, chemical composition
posed to explain the etiology of dental caries. and frequency of intake. It is a well known fact that carbo-
The acidogenic theory, proteolytic theory and proteolysis- hydrates which have a slow clearance rate from the oral
chelation theory have been the most discussed. cavity are more cariogenic than those which have a higher
clearance rate. The risk of caries incidence increases greatly if
Miller’s Chemicoparasitic Theory/Acidogenic the dietary sugar is sticky in nature, and when there is
increased frequency of ingestion of the sugars.
Theory
Polysaccharides are less easily fermented by plaque bac-
Willoughby Miller in 1882 suggested that dental decay is a teria than monosaccharides and disaccharides. Glucose,
chemoparasitic process. He believed that caries was caused sucrose and fructose which are highly fermentable have a
by a variety of microorganism and the acids in the oral greater role to play in the causation of dental caries. These
cavity were synthesized by the action of microorganisms. sugars are readily broken down by the bacteria to produce
He recognized four important factors in his study of the acids that in turn cause the dissolution of the hydroxyapa-
carious process which are: role of microorganisms, role of tite crystals of the enamel and dentin.
carbohydrate substrate over the tooth surface, role of acids Sucrose is readily fermented by the cariogenic bacteria
and the role of dental plaque. (mainly Streptococcus mutans) to produce acids, which can
405
Section VI – Teeth and Periodontium
demineralize the tooth. S. mutans use sucrose to synthesize It is believed that enamel lamellae and enamel rods which
an extracellular insoluble polysaccharide with the help of the are composed of organic material form the pathways for the
enzyme ‘dextran’, which helps in adhering the plaque firmly advancing microorganisms.
on to the tooth surface. It has been established that enamel contains 0.56% of
The pH of plaque falls to 4.5–5 in about 1–3 minutes of organic matter of which 0.18% is keratin and 0.17% is a
sugar intake. It is estimated that pH returns to neutral levels soluble protein.
in approximately 30 minutes. Gottlieb in 1944 suggested that the initial lesion of the
The physical nature of the diet intake also has a bearing carious process is due to the proteolytic enzymes attacking
on the incidence of carious lesions. Coarse fibrous foods the lamellae, rod sheaths, tufts and walls of tubules.
aid in greater clearance rate from the oral cavity thereby He believed that the yellow pigmentation that is charac-
minimizing the carious incidence. However, sticky refined teristic of caries was due to pigment produced by the pro-
food and sweetened soft drinks predispose to debris accu- teolytic organisms. He also proposed that the Staphylococcus
mulation and the increased likelihood of carious lesions. plays a vital role in initiating proteolytic activity.
Pincus in 1949 proposed that the proteolytic break-
Role of microorganisms down of the dental cuticle is the first step in the carious
process. He proposed that the Nasmyth’s membrane and
Lactobacillus acidophilus along with a combination of other the enamel proteins are mucoproteins, which are acted
bacteria such as S. mutans and Actinomyces species are upon by the sulfatase enzyme of the bacilli yielding sulfuric
closely associated with the causation of dental caries. It is acid. The sulfuric acid thus produced combines with the
believed that a few types of bacteria may be intimately calcium of the hydroxyapatite crystal and destroys the
involved in the initiation of the carious process whereas some inorganic component of the enamel.
may be involved in the progression of the carious process.
Role of acids Proteolysis–Chelation Theory

Miller in his observations reported that many of the micro- Some of the minor flaws of the acidogenic and the proteolytic
organisms in the oral cavity were capable of producing theories were addressed in the proteolysis–chelation the-
acids and these acids were usually present in deeper carious ory. This theory was put forward by Schatz and coworkers
lesions. In many of the lesions studied, lactic acid was in 1955.
identified in carbohydrate saliva combined mixtures. A Chelation is a process in which there is complexing of
specific microorganism, Leptothrix buccalis, was isolated the metal ions to form complex substance through coordi-
from the dentinal tubules, suggesting that the microorgan- nated covalent bond which results in poorly dissociated
isms and the acids may have a synergistic action to dis- and/or weakly ionized compound. Chelation is indepen-
solve the organic portion of the tooth. dent of the pH of the medium.
The proteolysis–chelation theory considers dental car-
Role of dental plaque ies to be a bacterial destruction of organic components of
Plaque as described by GV Black is a ‘gelatinous plaque of enamel and the breakdown products of these organic com-
the caries fungus in a thin, transparent film that usually ponents to have chelating properties and thereby dissolve
escapes observation, and which is revealed only by careful the minerals in the enamel even at the neutral/alkaline pH.
search’. It generally consists of salivary components such Mucopolysaccharides, lipids and citrates may also act as
as mucin and desquamated epithelial cells and of microorgan- secondary chelators.
isms. However, long-standing plaque may accumulate to a Schatz and coworkers observed that there is a simulta-
perceptible degree in 24–48 hours. It is estimated that car- neous microbial degradation of organic component by
iogenic plaque contains 2 ⫻ 108 bacteria/mg weight. proteolysis and the dissolution of inorganic part by the
The pH of plaque also varies in caries free and individuals process of chelation.
with high caries activity. The pH is about 7.1 in caries free
individuals and approximately 5.5 in individuals with high
caries activity. Sucrose Chelation Theory
This theory proposes that if there is a very high concentra-
tion of sucrose in the mouth of a caries-active individual,
Proteolytic Theory
there can be formation of complex substances like calcium
The initial work on the proteolytic theory was done by saccharate and calcium complexing intermediaries by the
Heider and Bodecker in 1878 and Abbott in 1879. Studies action of phosphorelating enzymes. These complexes cause
showed that the organic portion of the tooth plays an release of the calcium and phosphorus ions from the
important role in the development of dental caries. enamel and thereby resulting in tooth decay.
406
Chapter 15 – Dental Caries, Pulp and Periapical Lesions
Autoimmune Theory Figure 1

According to this theory few odontoblasts, within the pulp
of few certain teeth are damaged by the autoimmune mech- Host factor
anisms, leading to a compromised defense mechanism and
the loss of integrity of enamel or dentin. These targeted sites
are potential sites for the development of carious lesions.
Enzymatic Theory
Dental
Diet Time
This theory is based on the fact that plaque bacteria produces caries
enzymes. Enzymes involved may include phosphatase (dis-
solves apatite), chondroitin sulfatase (dissolves dentinal chon-
droitin sulfate), hyaluronidase (dissolves dentinal hyaluronic
acid) and proteases (dissolve dentinal collagen). All these
enzymes are produced by plaque bacteria.
Microorganisms
Acid–enzyme Theory
Diagram depicting the contributing factors for
This theory is a combination of the acidogenic and the enzyme dental caries
theories. It is believed that the acids produced have a
major role in enamel dissolution and the enzymes proba-
bly play a greater role in dentinal dissolution.
2. Microflora
3. Substrate or diet (physical nature and chemical nature)
Levine’s Ionic See-saw Theory 4. Time.
This theory was proposed by Levine in 1977. According
to this theory, there is a chemical relationship between
enamel plaque and the factors which determine the move- Host Factor
ment of minerals from saliva/plaque to enamel and vice Tooth Factor
versa, which was termed as the ionic see-saw mechanism.
Levine proposed that the demineralization and remin- Morphology and position in arch
eralization of enamel is a continuous process. If in a given Compared to the smooth surfaces of teeth, deep pits and
interval of time, more ions leave the enamel than enter it, fissures are more prone to carious attack because of
then there is a net demineralization, which heralds the food lodgment and bacterial stagnation. Owing to their,
beginning of the carious process. It was proved that the ions complex occlusal morphology consisting of numerous
are constantly exchanged between enamel and plaque. pits and fissures, the permanent mandibular first molars
This theory emphasizes the importance of pH of plaque, followed by the maxillary first molars and mandibular
calcium and phosphate ion concentration at the interphase and maxillary second molars are more prone to carious
and fluoride ion concentration. attack.
Apart from the morphology of the tooth, the position of
the tooth in the arch has a heavy bearing on the incidence
CONTRIBUTORY FACTORS IN DENTAL of carious lesions.
CARIES Irregularities in the arch form, crowding and overlap-
ping of the teeth also favor the development of caries as these
The four factors contributing to the caries process are regions provide an excellent environment for plaque accu-
(Figure 1): mulation.
Partially impacted third molars are more prone to caries
1. Host factor and so are the buccally or lingually placed teeth.
a. Tooth factor
i. Morphology and position in the arch
Chemical nature
ii. Chemical nature
b. Saliva The chemical components of enamel such as dicalcium phos-
i. Composition, pH and antibacterial activity phate dihydrate and fluorapatite make the enamel resistant
ii. Quantity and viscosity of flow to carious attack to a certain extent.
407
The presence of mineral ions such as Ca, F, Zn and Fe in Spak et al, 1994) or caries susceptibility, it is still a mys-
higher concentrations, decreases the enamel solubility. tery as to how much saliva is adequate enough.
Higher the solubility of the enamel surface greater is the The pH of saliva at which it ceases to be saturated with
possibility of caries development. Hence, they can be seen calcium and phosphorus is referred to as the ‘critical pH’.
in case of enamel hypoplasia. Normally, the critical pH is 5.5. Below this value, the inor-
The mineral content of enamel tends to increase with ganic content tends to demineralize. The normal pH of
advancing age. Such teeth have an increased resistance to resting saliva is 6–7.
carious attack.
Buffering capacity The buffer capacity of both unstimu-
lated and stimulated saliva involves three major buffer sys-
tems: the bicarbonate (HCO–3), the phosphate, and the
Saliva protein buffer systems. These systems have different pH
Composition ranges of maximal buffer capacity. The bicarbonate and
phosphate systems have pH values of 6.1–6.3 and 6.8–7.0,
Hay et al (1982) and Lagerlof (1983) in their reports reiter- respectively.
ated the fact that human salivary secretions are supersatu- Since most of the salivary buffering capacity operative
rated on calcium and phosphate. during food intake and mastication is due to the bicarbonate
The concentrations of inorganic calcium and phospho- system, sufficient saliva flow provides the oral cavity with
rus show considerable variation within resting and stimu- the neutralizing components.
lated saliva. Caries prone individuals have low calcium The phosphate and protein buffer systems make a minor
and phosphorus levels. Salivary proteins such as statherin, contribution to the total salivary buffer capacity, relative
acidic proline-rich proteins (PRPs), cystatins, and histatins to the bicarbonate system. The phosphate system is, in
help in the maintenance of the homeostasis of the super- principle, analogs to the bicarbonate system but without
saturated state of saliva. According to Hay and Moreno the important phase-buffering capacity, and it is relatively
(1989), statherin is present in stimulated saliva in concen- independent of the salivary secretion rate.
trations sufficient to inhibit the precipitation of calcium Lagerlof and Oliveby in 1994 showed that a low flow
and phosphate salts. Studies by Gibbons and Hay (1988) rate combined with a low or moderate buffer effect indi-
have shown that statherin may contribute to the early cated poor salivary resistance against microbial attack.
colonization of the tooth surfaces by certain bacteria, such It is a well-established fact that the buffer capacity of
as Actinomyces viscosus. the saliva and the caries experience are inversely related.
The acidic PRPs account for 25–30% of all proteins in The buffer effect of saliva is influenced by the hormonal
saliva, and they have high affinity for hydroxyapatite in vitro and metabolic changes, as well as by altered general health.
(Hay and Moreno, 1989). The acidic PRPs bind free calcium, It is generally accepted that the buffer effect is greater in
adsorb to hydroxyapatite surfaces, inhibit enamel crystal men than in women (Heintze et al, 1983). In women, the
growth, and regulate hydroxyapatite crystal structure (Hay buffer effect decreases gradually, independent of flow rate,
and Moreno, 1989). toward late pregnancy and promptly recovers after deliv-
The amount and quality of acidic PRPs and agglutinins ery. Introduction of either hormone replacement therapy
are found to be different in caries-free and caries-active in menopausal women (Laine and Leimola-Virtanen, 1996)
individuals as shown by the studies of Rosan et al (1982) or low-dose oral contraceptives (Laine et al, 1991) can
and Stenudd (1999). slightly increase the buffer capacity.
The role of cystatins in the caries process is still unclear. Carbonic anhydrases (CAs) participate in the mainte-
However, they may play a minor role in the regulation of nance of pH homeostasis in various tissues and biological
calcium homeostasis in saliva. Phosphorylated and non- fluids of the human body by catalyzing the reversible
phosphorylated cystatins bind to hydroxyapatite. hydration of carbon dioxide. Recent research suggests that
salivary CA VI plays a role in protecting the teeth from
Salivary flow rate, pH and buffer capacity caries (Kivela et al, 1999a, b). CA VI has been reported to
bind to the enamel pellicle and retain its enzymatic activity
Saliva has the most important function of caries prevention
on the tooth surface.
by way of its flushing and neutralizing effects, commonly
It is also believed that the urea and saline in saliva
referred to as ‘salivary clearance’ or ‘oral clearance capac-
become hydrolyzed to produce ammonia and the later can
ity’. As a thumb rule, the higher the flow rate, the faster the
cause rise in the salivary pH. This rise in pH can counter
clearance and the higher the buffer capacity. Reduced sal-
the attacks on the tooth surface during the progression of
ivary flow rate and the concomitant reduction of oral
caries.
defense systems may cause severe caries and mucosal
inflammation. Though, patients with impaired saliva flow Antibacterial activity The primary oral innate defense
rate often show high caries incidence (Papas et al, 1993; factors are peroxidase systems, lysozyme, lactoferrin, and
408
histatins. In vitro studies have shown that these proteins Increased viscosity of saliva may hinder its natural
are known to limit bacterial or fungal growth, interfere cleansing action thereby promoting the deposition of plaque
with bacterial glucose uptake or glucose metabolism and on the tooth surface. Likewise when the salivary viscosity is
promote aggregation and, thus eliminate bacteria. low, the amount of minerals and bicarbonates are inade-
Hanstrom et al (1983) and Tenovuo and Larjava (1984) quate thereby limiting its anticaries activity.
reported that the salivary peroxidase and myeloperoxidase
systems eliminate H2O2, which is highly toxic for mam-
malian cells. Microflora
The immunoglobulins, IgG, IgM, IgA, and secretory
The main etiological agent in occlusal and pit and fissure
IgA (sIgA), form the basis of the specific salivary defense
caries is the S. mutans. Deep dentinal caries is commonly
against oral microbial flora, including Streptococcus
associated with lactobacilli, certain gram-positive anaer-
mutans. The most abundantly available immunoglobulin in
obes and filaments such as Eubacterium and Actinomyces.
saliva is dimeric slgA, which is produced by plasma cells
Root caries or cemental caries is predominantly associ-
located in the salivary glands. Two IgA subclasses are
ated with Actinomyces viscosus. However other species of
present in saliva; IgAl forms the major component of
Actinomyces such as A. naeslundii and A. nocardia have
immunoglobulins, although the relative amount of IgA2 is
also been isolated.
higher in saliva than in other secretions (Tappuni and
Challacombe, 1994).
In human beings, IgG, mainly of maternal origin, is the
only detectable immunoglobulin in the saliva of neonates. Substrate and Dietary Factors
Salivary IgA is absent at birth but is generally detectable The role of diet in the causation of dental caries has been
by the age of 1 week. extensively studied.
The IgG concentration decreases to non-detectable lev- A variety of dietary factors have been implicated in the
els after some months but appears again after tooth erup- causation of dental caries.
tion (Brandtzaeg, 1989). Low concentrations of IgG can be
detected in stimulated parotid saliva (Brandtzaeg, 1989),
Physical nature of diet
but most of the IgG detected in whole saliva enters the
mouth from the gingival crevicular fluid, thus originating It is believed that coarse and fibrous food helps in cleansing
from sera. the debris from the tooth surface thereby minimizing the
In most children above 3 years of age, salivary IgAs incidence of carious lesions. However, refined and sticky
against S. mutans can be detected, and their amount starchy food aid in the formation of dental caries.
increases with the length of exposure (Smith and Taubman,
1992). Salivary Igs can bind to the salivary pellicle, and
Chemical nature of diet
they are found also in dental plaque (Newman et al, 1979;
Fine et al, 1984). In the oral cavity, immunoglobulins It is a well-known fact that food with high refined carbohy-
act by neutralizing various microbial virulence factors, drate content has the greatest potential to give rise to carious
limiting microbial adherence, and agglutinating the bacte- lesions.
ria, as well as by preventing the penetration of foreign The type of carbohydrate (monosaccharide, disaccharide
antigens into the mucosa. IgGs are also capable of opso- or polysaccharide), frequency of intake and the time for which
nizing bacteria for phagocytes, which are reported to the ingested food remains stagnant in the oral cavity or on
remain active in dental plaque and saliva (Scully, 1980; the tooth surface determine the incidence and severity
Newman, 1990). of the carious lesions.
Animal studies have shown that sugar in the solid and
sticky form is more harmful to the tooth than the same amount
Quantity and viscosity of flow
of sugar in a liquid form.
The consistency or viscosity of the saliva and the amount It is believed that vitamin B deficient individuals have
of saliva produced has a significant influence on the inci- lower incidence of dental caries. Vitamin B is essential for
dence of dental caries. the growth of oral acidogenic flora and also serves as a
The average person produces at least 500 ml of saliva over component of coenzymes involved in glycolysis.
a period of 24 hours. The unstimulated flow rate is 0.3 ml/ Vitamin D plays an important role in the normal devel-
min, whereas the flow rate during sleep is 0.1 ml/min and opment of teeth. Various studies have shown that the teeth
during eating or chewing, it increases to 4.0 to 5.0 ml/min. are hypoplastic and usually have higher incidence of den-
Any reduction in this quantity of saliva as seen in diseases tal caries in vitamin D deficiency.
such as Sjögren’s syndrome, diabetes, etc. predisposes to Teeth may be poorly calcified in individuals exposed to
dental caries. low doses of calcium during intrauterine life and infancy.
409
Such poorly calcified teeth may be susceptible to carious CLASSIFICATION OF DENTAL CARIES
attack. Higher levels of selenium is known to predispose to
the carious lesions affecting permanent teeth. 1. Dental caries based on location
Fluoride content in the diet has no significant role because a. Pit and fissure caries
of its metabolic unavailability. Therefore, the fluoride content b. Smooth surface caries
in cooking salt and its effect on reducing the incidence of c. Root surface caries
carious lesions is still questionable. However, fluoridated 2. Dental caries based on severity
water minimizes the caries incidence. a. According to morphology of teeth
Phosphates, molybdenum and vanadium in the diet b. According to severity and progress
helps in minimizing the incidence of carious lesions. c. According to age pattern
d. According to rapidity of progress.
Role of heredity
Literature review reveals various studies to assess According to Morphology of Teeth
the genetic modifications in dental enamel, genetic modi-
fication of immune response, genetic regulation of i. Pit and fissure caries (also called type-1 caries): Caries
salivary function and inherited alterations in sugar metab- occurring on anatomical pits and fissures of all the
olism. teeth. The specific areas or surfaces involved include
Bachrach and Young (1927) compared the caries inci- occlusal surfaces of molars (Figure 2) and premolars,
dence of monozygotic twins with same-sex dizygotic (93 buccal and lingual surfaces of molars (Figure 3) and
pairs) and different-sex dizygotic (78 pairs) twins. Their lingual surfaces of maxillary incisors.
results showed that the monozygotic twins had a more The lesions are smaller in the beginning but become
similar caries incidence than dizygotic twins and that dif- wider as they spread toward the dentin due to orienta-
ferent-sex dizygotic twins had the greatest variance. The tion of enamel rods.
authors concluded that heredity plays a subsidiary part in In these places there can be entrapment of food
the incidence of caries. It is believed that heredity affects leading to fermentation of carbohydrates with lack of
the dental decay only in as much as it controls the shape neutralization of acid produced by salivary buffers
of a tooth and its pits and fissures and its position in the which leads to destruction of enamel and dentin. The
dental arch. enamel bordering the pit and fissure may appear
Senpuku et al (1998) and Acton et al (1999) have cor- opaque and bluish-white as it becomes undermined.
related specific HLA-DR types with binding S. mutans Clinically these lesions appear brown or black,
antigens and S. mutans colonization. with little softening and opaqueness of the surface.
Acton concluded that ‘genes within MHC modulate the When the lesion is examined by a fine explorer tip, a
level of oral cariogenic organisms’. ‘catch point’ is often felt, where the explorer teeth
Mariani et al (1994) in their study of celiac disease, catches the area.
enamel defects and HLA typing observed that HLA-DR3 When the lesion reaches the dentinoenamel junc-
was associated with increased enamel defects and tion (DEJ), they spread laterally to cause undermining
HLA-DR5, 7 were associated with a reduced frequency of of the enamel.
enamel defects. Studies have shown that the genes in the ii. Smooth surface caries (also known as type-2 caries):
HLA complex are associated with altered enamel develop- These carious lesions occur on the smooth surfaces of
ment and increased susceptibility to dental caries. the teeth (e.g. proximal surfaces or gingival areas
of the buccal and lingual aspect of tooth).
Proximal caries usually begins just below the con-
Role of immunity
tact point, and appears in the early stage as a well
Salivary IgA and immunoglobulins secreted in the gingi- demarcated faint white opacity of the enamel without
val crevicular fluid such as IgG, IgM and IgA along with apparent loss of continuity of enamel.
neutrophil leukocytes and macrophages play an important The white spot lesion becomes pigmented yellow
role in the prevention of dental caries. It is believed that or brown and it often extends buccally and lingually.
the immune response exerted by the gingival crevicular The surrounding enamel becomes bluish white as
immune system is more potent compared to the salivary the lesion continues to progress (Figure 4). The sur-
immune mechanism. face of the affected enamel becomes rough and later
Salivary IgA prevents S. mutans from adhering to the on, there is formation of a cavity (Figure 5).
tooth surface. The IgG antibodies acting as opsonins, facil- iii. Root caries: Caries occurring at the cementoenamel
itate phagocytosis and the death of S. mutans by the action junction or cementum. This occurs predominantly in the
of macrophages and neutrophil leukocytes. older age when there is gingival recession.
410
Figure 2 Figure 4
Pit and fissure carious lesion on the occlusal surface of

The early stages of a proximal carious lesion.
a mandibular molar. Courtesy: Department of
The surface of the enamel reveals a bluish-black
hue with no discontinuity of the enamel surface.
MCODS, Mangalore
Figure 3
Figure 5
A carious lesion on the buccal surface of the mandibular

molar. Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
iv. Linear enamel caries: Caries occurring on the labial

surfaces of anterior teeth. This is also known as Proximal surface carious lesion. Courtesy: KLE Society’s
‘odontoclasia’. The caries occurs at neonatal zone Institute of Dental Sciences, Bangalore
because of trauma at birth or metabolic disturbances.
• Incipient lesion can undergo remineralization

According to Severity and Progress
thereby reversing the process.
i. Incipient caries: Initial carious lesion limited to the ii. Rampant caries: This is an acute fulminating type of
teeth is called incipient caries and is characterized carious process, which is characterized by simultaneous
by a virtually intact surface but a porous subsurface involvement of multiple number of teeth (may be all
(subsurface demineralization). teeth) in multiple surfaces (Figure 6A, B).
411
Figure 6
A B
Extensive destruction of teeth in child suffering from rampant caries. Courtesy: Department of Oral Medicine and
• Rapid coronal destruction occurs within a short

Figure 7
span of time, causing early involvement of the
pulp.
• The common age of occurrence is 4–8 years for
the deciduous teeth and 11–19 years for the per-
manent teeth.
• It can even occur in persons who maintain a good
level of oral hygiene regularly.
• Moreover, this type of caries attacks those surfaces
of teeth, which are otherwise considered immune
to the disease.
iii. Arrested caries
• Any carious lesion usually an incipient one may
become arrested, if there is a change in oral envi-
ronment. Arrested caries, clinically appears as a
dark brown pigmentation with smooth surface,
referred to as ‘eburnation’, which is a Latin word Recurrent carious lesion beneath faulty restorations.
that means arrested caries.
MCODS, Mangalore
• It can be on occlusal as well interproximal surfaces.
• It can occur in both enamel and dentin.
iv. Recurrent caries: It occurs at the interface of tooth and
restorative material because of many factors such as
defective cavity preparation, microleakage and com- by the nursing bottle, for a considerably longer duration
bination of these (Figure 7). of time, preferably during sleep.
v. Radiation caries: One of the complications of radio- 1. It has been variously attributed to prolonged use of
therapy of oral cancer lesions is xerostomia, which • A nursing bottle containing milk or milk formula,
leads to an early development of widespread caries. fruit juice or sweetened water
• Breast feeding
• Sugar or honey-sweetened pacifiers.
According to Age Pattern
2. Invariably there is a prolonged habitual use of one of
Nursing bottle caries: A type of acute carious lesion, which the above after 1 year of age, usually as an aid for sleep-
occurs among those children who take milk or fruit juice ing at night or naptime.
412
3. Clinically presents as widespread destruction of decidu-

Figure 8
ous teeth, most commonly the four maxillary incisors,
followed by the first molars and then the cuspids if the
habit is prolonged.
4. The lower teeth are not usually affected as they remain
under the cover of the tongue, so the absence of caries in
the mandibular incisors distinguishes this disease from
ordinary rampant caries.
5. Both the nursing bottle and rampant cause early pulp
involvement.
Because they spread at a very rapid pace and as a result,

the pulp hardly gets any time to protect itself by forming
secondary dentin.
According to Rapidity
i. Acute dental caries
• It is a form of caries which runs a rapid clinical
course and results in early pulp involvement by
carious process.
• It occurs most frequently in children and young Deep occlusal caries with brownish pigmentation.
adults, presumably because the dentinal tubules Courtesy: Department of Oral Medicine and
are large and open and show no sclerosis. Radiology, MCODS, Mangalore
• The process is usually so rapid that there is little
time for the deposition of secondary dentin. present in saliva and in dental plaque in individuals with
ii. Chronic dental caries rampant caries due to xerostomia as well as in children
• Slowly progressing in nature and tends to involve nourished with bottle milk. S. mutans and lactobacilli have
the pulp much later than the acute caries. been found to increase in significant numbers in the plaque
• Most common in adults. as well as dentin of teeth restored with amalgams having
• These caries lesions exhibit a large cavity with marginal defects wider than 40 ␮m.
brownish pigmentation (Figure 8). Fitzergerald et al (1994) were of the view that in asso-
• Pain is not a common feature of chronic caries ciation with these three major microorganisms, others also
because of the protection afforded by secondary played a role in secondary caries. They found S. mutans,
dentin. S. sanguis and S. salivarius in 35%, 24% and 14% of
• The slow progression of the lesion allows suffi- growth positive samples respectively. Other isolates like
cient time for both sclerosis of the dentinal tubules S. gordonii, S. milleri, S. oralis and S. mitis were also rec-
and deposition of secondary dentin in response to ognized. Certain organisms, which occurred very frequently
the adverse irritation. were Propionibacterium, Bifidobacterium, Eubacterium and
Peptococcus. Actinomyces were found in 46% of the sam-
ples. A. viscosus and A. naeslundii were most prevalent
MICROBIOLOGY OF DENTAL CARIES followed by A. israelii and A. odontolyticus.
S. mutans can adhere to the tooth surface by glucan which
The predominant group of microorganism is streptococci. is produced by utilization of dietary sucrose. These organ-
Among these strains S. mutans is responsible. These are isms ferment mannitol and lactose with the production of
gram-positive organisms which are round or ovoid. These acid. These can take up dietary sucrose and breakdown
may appear rod shaped, non-sporulating and non-motile. into glucose and fructose by means of the enzyme, inver-
These can be cultured on blood agar with formation of tase. Finally glucose and fructose is broken down to lactic
refractory colonies measuring 0.5–1.5 mm at 37⬚C. These acid. These have the ability to store glucose and fructose
are pathogenic to human beings. from degradation for the synthesis of acids in the absence of
The three common organisms seen to be associated with dietary sucrose.
secondary caries are S. mutans, lactobacilli and Actinomyces Clinical features of dental caries, pulp and periapical
viscosus. Fontanna et al (1996) observed a definite relation- lesions are given in Table 1.
ship of S. mutans and secondary caries. S. mutans is also Sequelae of pulpitis is depicted in Flowchart 1.
413
Table 1 Clinical features of dental caries, pulp and periapical lesions
Extent of Signs and Clinical findings Clinical Radiographic diagnosis Final diagnosis
carious lesion symptoms diagnosis
Caries involving Asymptomatic Chalky white Enamel May not be Enamel
enamel patient rarely discoloration of the tooth caries evident on a caries
notices blackish or radiograph until
discoloration of blackish discoloration at least 40%
the tooth of the tooth usually demineralization
seen in the pits and occurs
fissures and proximal Ill-defined radiolucency
aspects of teeth involving the enamel cap
Caries involving Sensitivity on Blackish discoloration Dentinal Ill-defined Dentinal
dentin consuming hot, of the tooth caries radiolucency caries
cold or sweet food Catch experienced or involving the
Sensitivity subsides on using an reversible dentin
on removal of explorer pulpitis (Figure 9)
stimulus Frank cavitation
Food lodgment
patient may notice
blackish
discoloration of
the tooth
Caries involving Dull aching pain, Frank cavitation Chronic Radiolucency Chronic
pulp which has involved tooth irreversible involving the pulp irreversible
periods of non-tender on pulpitis No periapical pulpitis
exacerbation percussion changes (Figure 11)
Food lodgment Grossly decayed Widening of the Chronic apical
tooth/root stump periodontal ligament periodontitis
(Figure 10) space at the periapex
(Figure 12)
Discontinuity of the lamina Chronic periapical
d ura at the periapex or abscess
ill-defined periapical
radiolucency (Figure 13)
Well-defined radiopacity at Focal sclerosing
the periapex (Figure 14) osteomyelitis
or condensing
osteitis seen
in young
individuals with
good immune
response or
when the
virulence of the
microorganisms
is low
Well-defined radiolucency Periapical
measuring ⬍1.5 cm in granuloma
diameter at the periapex (Figure 16)
of the tooth (Figure 15)
Well-defined radiolucency Periapical cyst
measuring ⬎1.5 cm in
diameter at the periapex
of the tooth bounded by
a sclerotic border
(Contd.)
414
Caries involving Pain Frank cavitation Acute apical Radiolucency involving pulp Acute apical
pulp Food lodgment Involved tooth periodontitis No periapical changes periodontitis
Patient usually tender on percussion
avoids chewing Calculus build up on Radiolucency involving pulp Acute
on the affected the occlusal surfaces with widening of the exacerbation
side of the teeth on the periodontal ligament space of chronic
Patient may affected side is indicative at the periapex apical
complain of of unilateral chewing Acute Caries involving pulp periodontitis
fever and Intraoral or extraoral periapical Generally no periapical Acute periapical
malaise swelling tender on abscess changes or may be abscess
palpation (Figure 17A, B) associated with widening
Vestibular tenderness of the periodontal ligament
and/or obliteration space at the periapex
Caries involving Dull aching pain Frank cavitation Chronic Radiolucency involving Chronic
pulp or asymptomatic Involved tooth non- periapical pulp with discontinuity of periapical
Food lodgment tender on percussion abscess lamina dura at the abscess
Deep carious Intraoral or extraoral periapex and or diffuse
lesion, grossly non-tender swelling radiolucency at the
decayed tooth, may or may not be periapex (Figure 20)
root stump present (Figure 18)
Patient may Sinus opening on the
complain of attached gingiva or
halitosis, bad vestibule associated
taste or a salty with the affected tooth
taste (usually (Figure 19) or in close
indicative of pus proximity (occasionally
discharge) sinus openings may be
seen distant from the
affected site as the sinus
tracts may follow the
path of least resistance
(through the bone)
Vestibule may be obliterated
When the involved tooth Acute Phoenix
is tender along with exacerbation abscess
the above findings of a chronic
periapical
abscess
(Phoenix
abscess)
Caries with Pain Diffuse extraoral swelling, Cellulitis Ill-defined radiolucency Cellulitis of the
pulpal Diffuse extra- tender, stretched involving pulp infraorbital,
involvement oral swelling and shiny overlying Usually very minimal buccal,
(sudden onset) skin, local raise in periapical changes such submandibular
Fever, malaise temperature (Figure 21) as widening of the space, etc.
Based on the fascial Limited mouth opening periodontal space or ill-
space involved, (extent and clinical defined radiolucency at
patient may features of the fascial the periapex may evident
complain of spaces are described in at acute nature of the
difficulty in eating, the section on fascial condition is the reason
swallowing, limited space infections) for the very minimal bone
mouth opening Involved tooth is carious changes
and occasionally and tender on percussion
difficulty in Presence of intraoral
breathing swelling and vestibular
obliteration
(Contd.)
415
Table 1 Continued
Caries with Patient may Tooth involved shows a Periapical Well-defined radiolucency Periapical cyst
pulpal complain of dull deep carious lesion, cyst measuring ⬎1.5 cm in
involvement aching pain or may tender or non-tender diameter at the periapex
be asymptomatic on percussion of the tooth surrounded
Diffuse expansion of by a sclerotic border
the cortical plates. The (Figures 23 and 24).
affected site may reveal Absence of the sclerotic
a well-defined bony border may be indicative
hard swelling (usually of an infected cyst
mildly tender or (Figure 25)
non-tender on
palpation) (Figure 22)
Occasionally ‘egg shell
crackling’ of the
cortical plates may be
appreciated on
palpation
Clinical Diagnostic Methods

Flowchart 1
Diagnostic methods for caries in common use include
visual inspection, tactile examination with a probe, bite- Enamel caries
wing radiography, intraoral periapical radiographs and cer-
tain diagnostic aids such as fiberoptic transillumination,
Dentinal caries or reversible pulpitis
endoscopic techniques, light scattering, laser fluorescence,
ultraviolet illumination, dye penetration, iodide penetra-
tion and electrical resistance. Chronic irreversible pulpitis
Early diagnosis enables small lesions to be identified so
that remineralization of lesions by preventive measures can
be attempted. Acute Apical periodontitis Chronic
In the clinical examination for dental caries every assess-
able surface of each tooth must be examined for localized
changes in color, texture, and translucency. Periapical abscess Infected Periapical
abscess
Dental caries is most prevalent in the faulty pits and
fissures of the occlusal surfaces, where developmental
lobes of the posterior teeth fail to coalesce, partially or Cellulitis Acute
suppurative Dentoalveolar
completely. osteomyelitis abscess
Followed by
Visual, Tactile Methods of Caries Detection chronic phase Periapical
granuloma
Earlier sharp explorers were used to detect incipient fis-
sure caries. However, in recent times it is strongly discour- Ludwig’s angina/fascial
aged as it was found to fracture enamel and serve as a space infections Periapical
source for transferring pathogenic bacteria among various cyst
teeth. It was also noted that the use of an explorer did
Cavernous sinus thrombosis
not increase diagnostic validity compared to visual
examination.
Visual examination of the tooth surface should be carried Death
in a dry, well-illuminated field.
Chalkiness or apparent softening or cavitation of the tooth
Sequelae of pulpitis
structure is diagnostic of the carious process. Occasionally
416
Figure 9 Figure 10
Radiograph showing proximal caries

involving dentin suggestive of dentinal caries.
MCODS, Mangalore
a brown-gray discoloration is seen. However, one should

be careful to distinguish between superficial staining and
a carious lesion. Grossly decayed teeth and root stumps.
Occlusal enamel can be evaluated for loss of translu- Courtesy: Department of Oral Medicine and
cency and change in color, which are characteristics of Radiology, MCODS, Mangalore
caries.
Proximal surface caries is usually diagnosed radiographi-
cally. Clinically when the carious process has invaded the
proximal surface enamel and dentin, a white chalky appear- in gingival areas that are less accessible for cleaning.
ance or a shadow under the marginal ridge may be seen. Care- The earliest clinical evidence of incipient caries on these
ful probing with an explorer on the proximal surface may surfaces is a white spot that is visually different from
detect cavitation (defined as a break in the surface contour of the adjacent translucent enamel and will partially or
enamel). totally disappear on wetting. Drying again will cause it
Proximal caries can also be detected by tooth separa- to reappear. This disappearing and reappearing phenome-
tion or through transillumination. Brown spots on intact non distinguishes the smooth incipient caries form
hard proximal surface of enamel adjacent to and usually the white spots resulting from non-hereditary enamel
gingival to the contact area are often seen in older patients hypocalcificaton.
whose caries activity is low. Such a spot is no longer cari- In patients with attachment loss, care must be taken to
ous and, in fact, are more resistant to caries as a result of inspect for root surface caries. Carious lesion occurs on the
fluorhydroxyapatite formation. Transillumination is accom- cemental surfaces of the teeth. Active root caries is detected
plished by placing the mirror or light source on the lingual by the presence of softening and cavitation.
side of the anterior teeth and directing the light through Advanced smooth surface caries will exhibit discolor-
the teeth. ation and demineralization and will feel soft to penetration
Use of a dental floss in the interdental regions along the by the explorer. The discoloration may range from white to
proximal surfaces of teeth may also be used to detect initial dark brown, with rapidly progressive caries usually being
proximal carious lesions. A frayed floss is usually diagnostic light in color. With slowly progressive caries in a patient
of proximal caries. with low caries activity, darkening occurs over time because
Another form of smooth surface caries often occurs of extrinsic staining and remineralization of decalcified
on the facial and lingual surfaces of the teeth, particularly structure occasionally may harden the lesion. Such an
417
Figure 11 Figure 13
Caries
involving
pulp
No periapical changes
Illustration showing loss of lamina dura at the
Illustration showing the radiographic features
periapex and diffuse radiolucency at the periapex
in chronic irreversible pulpitis. Coronal radiolucency
suggestive of chronic periapical abscess
involving pulp with no periapical changes
Figure 14
Figure 12
Widening of periodontal
ligament space at the periapex
Illustration showing the radiographic features of

chronic apical periodontitis. Coronal radiolucency Radiograph depicting well-defined radiopacity at
involving pulp with widening of the periodontal ligament the periapex suggestive of condensing osteitis.
space at the periapex Courtesy: Department of Oral Medicine and
arrested lesion may at times be rough, although cleanable

Scoring system for visual changes associated
and a restoration is not indicated except for esthetics. The
with carious lesions (ICDAS)
dentin in an arrested remineralized lesion is termed ebur-
nated or sclerotic. Score 0: Sound tooth surface
Following a visual clinical examination the carious lesion Score 1: First visual change in enamel
can be scored using the International Caries Detection and Score 2: Distinct visual change in enamel
Assessment System (ICDAS). Score 3: Microcavitation
418
either with white light or with filtered fluorescence excited

Figure 15
by a blue curing light have shown an increased sensitivity
to detect initial occlusal lesions in comparison to clinical
visual examination.
Fiberoptic transillumination Friedman and Marcus (1970)

described the use of fiberoptic transillumination for the
detection of approximal caries. The working principle is
that the decayed tooth material scatters light more strongly,
and thus has a lower index of light transmission than a
sound tooth structure.
Use of fiberoptic transillumination for the diagnosis of
occlusal dentinal decay was studied by Verdonschot et al
(1992) as part of a comparison of various systems available
Well-defined periapical for the diagnosis of the dentinal decay of occlusal surfaces
radiolucency less than 1.5 cm in vivo. They found a very low sensitivity (0.13) and a
high specificity (0.99).
Illustration showing the radiographic features of
periapical granuloma. Well-defined radiolucency Light-scattering In a study by Angmar-Mansson and
measuring less than 1.5 cm at the periapex Ten Bosch (1987) to assess the scattering of light by cari-
ous lesions, incipient caries lesions looked whiter than the
surrounding sound enamel because of the strong scatter-
ing of light within the lesion. Presumably, this is primarily
Figure 16 due to the fact that the remaining small mineral particles
in the lesion are embedded in water rather than in min-
eral-rich sound enamel.
Ogaard and Ten Bosch (1993) were of the opinion that
this technique is useful only for caries lesions on smooth
surfaces.
Laser autofluorescence Visible light within the blue-green

region has been used as the light source for the development
of a sensitive method for the detection of smooth surface and
fissure caries at an early stage (Bjelkhagen et al, 1982).
The tooth is illuminated with a broad beam of blue-
green light of 488 nm wavelength from an argon ion laser.
The fluorescence in the enamel occurring in the yellow
region (about 540 nm) is observed through a yellow high-
pass filter (520 nm) to exclude the tooth-scattered blue
laser light.
Demineralized areas appear dark in this situation. Natural
lesions (diameter ⬍1 mm) with a lesion depth as small as
5–10 ␮m can be detected and measured using laser auto-
fluorescence.
The histopathological picture of a periapical
granuloma. Courtesy: Department of Oral Ultraviolet illumination Ultraviolet (UV) light has been
Pathology, MCODS, Mangalore used to increase the optical contrast between the carious
region and the surrounding sound tissue. The natural fluo-
rescence of tooth enamel, as seen under UV illumination,
is decreased in areas of less mineral content, such as in caries
Score 4: Underlying dark shadow from dentin with or
lesions, artificial demineralization, or developmental defects
without cavitation
(Alfano and Yao, 1981).
Score 5: Distinct cavity with visible dentin
The caries lesion appears as a dark spot against a fluores-
Score 6: Extensive distinct cavity with visible dentin.
cent background. Alfano and Yao showed that the UV illu-
Endoscopic methods Initial studies by Longbottom and Pitts mination method was more sensitive than simple visual/
(1992) on the potential benefits of endoscopic examination, tactile methods.
419
Figure 17
A B
(A) Intraoral photograph showing a well-defined swelling on the palatal surface of decayed maxillary molar suggestive of
an acute periapical abscess. (B) Tender extraoral swelling suggestive of an acute periapical abscess. Courtesy: Department of
Figure 18 Figure 19
Swelling associated with maxillary molar suggestive of

a periapical abscess. Courtesy: Department of A sinus opening in the vestibule associated with
Oral Medicine and Radiology, MCODS, Mangalore the discolored central incisor. Courtesy: Department of
Oral Medicine and Radiology, KLE Society’s Institute of
Foreman (1988) stated that, in UV light, there is generally

an inverse correlation between the intensity of fluorescence
and the degree of mineralization in mineralized dental tissues, Shern and coworkers (1990) compared sodium fluores-
just as there is with the laser fluorescence method. cein with potassium iodide to disclose the porosity of an
artificial incipient lesion. The extent of porous (active)
Penetration of dyes Various dyes, fluorescent or non- white spot lesions was disclosed only by fluorescein.
fluorescent, have been used for staining the porous caries Van de Rijke et al (1991) presented an optical quantifica-
lesion to enhance the contrast between the carious region tion of approximal in vitro caries using a fluorescent dye,
and the surrounding sound enamel. Fluorol.
420
Figure 20 Figure 22
Radiograph reveals diffuse periapical

radiolucency associated with the mandibular
molar suggestive of a periapical abscess.
Radiology, MCODS, Mangalore Extraoral photograph showing an extraoral swelling over
the right side of the mandible which was bony hard on
palpation. Courtesy: Department of Oral Medicine and
Figure 21
Figure 23
Well-defined periapical
radiolucency more than 1.5 cm
with sclerotic border
Extraoral photograph showing diffuse extraoral swelling Illustration showing the well-defined periapical
involving the left submandibular, buccal and infraorbital radiolucency measuring more than 1.5 cm in
fascial spaces. Courtesy: Dr Francisco Lopez Sanchez, diameter suggestive of a periapical cyst
University of Michoacan, Morelia, Mexico
was registered by a reflectance colorimeter, and a four

O’Brien et al (1989) used a blue tracer dye to increase the
times increase between the lesion and the surrounding
color contrast in the detection of approximal incipient car-
sound area was shown with the dye.
ies lesions by fiberoptic transillumination. Artificial incipi-
ent caries lesions were photographed by transillumination Iodide penetration The iodide penetration method for
with or without the blue dye added. The color difference measuring enamel porosity of the incipient caries lesion on
421
Figure 24 Figure 25
Maxillary occlusal radiograph showing a well-defined

periapical radioluceny associated with a fractured
maxillary central incisor suggestive of a periapical
cyst. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore Intraoral periapical radiograph showing a periapical
cyst with no definitive sclerotic border suggestive of
a secondary infection (infected periapical cyst).
smooth surfaces was developed by Brudevold and coworkers
and was later improved by Zero et al.
Potassium iodide was applied for a specified period of time
to a well-defined area of the enamel surface, and thereaf-
ter the excess was removed. Permeability was estimated by body of the lesion could be differentiated acoustically
measurement of the quantity of iodide that could be eluted from intact enamel on the basis of amplitude changes of the
from the test area. enamel surface echo and the amelodentinal junction echo.
Electrical resistance The electrical resistance method is They also found that there was a correlation between the
based on the assumption that electrical conductivity is a mineral content of the body of the lesion and the relative
function of porosity. Enamel demineralization results in echo amplitude changes, which was explained by changes
increased porosity, and saliva fills the pores and forms con- in specific acoustic impedance. However, the authors con-
ductive pathways for electrical current. cluded that the method is not sensitive enough to detect
Thus, the greater the amount of demineralization, the changes of shallow caries lesions in vivo.
higher the electrical conductivity through the affected enamel Vitality tests The tooth is said to be vital when it is capa-
is expected to be. ble of responding to stimuli.
An instrument (Vanguard electronic caries detector) has To check the vitality there are three basic stimuli in the
been designed to measure the electrical conductivity of form of thermal, electrical or mechanical:
a tooth to evaluate the presence of lesions in the occlusal
surface by measuring the electrical conductivity from the ❍ Thermal heat/cold application
probe tip in the fissure through the dental pulp to a hand- ❍ Electric pulp testing: Direct electric stimulation of sen-
held earth. The electrical conductivity is expressed numer- sory nerves in the pulp
ically on a scale from 0 to 9. In an in vitro study of ❍ Mechanical stimulation: Blowing air to the exposed
occlusal pit-and-fissure caries in molars, Flaitz et al (1986) dentin and test cavity preparation.
compared electrical conductivity data of extracted teeth
with the histologically measured lesion depth. The results
Thermal Test
showed a linear relation between scale value and depth.
Cold test
Ultrasonic imaging Ng et al (1988) introduced ultra-
sonic imaging for detecting early caries in smooth sur- Adjacent or contralateral unaffected teeth should be tested
faces. They showed that artificial enamel lesions with less for baseline comparisons because the duration of pain may
than 57% of the sound enamel mineral content in the differ among individuals.
422
A cotton applicator tip sprayed with a freezing agent or

Figure 26
carbon dioxide snow, icesticks, and ethyl chloride spray
applied directly to the tooth. The cold stimulus is applied
immediately to the middle one-third of the facial surface of
the crown of the tooth/exposed metal surface of crowns
and kept in contact for 5 seconds or until a point the
patient begins to feel pain.
Heat test
The tooth in question is isolated. A 3-inch gutta percha
stick is warmed over a flame until it becomes soft and just
begins to glisten.
The heated stick is then applied to the middle one-third
of the facial surface of the crown. Normally a response is Use of electric pulp tester. Courtesy: Department of
appreciated in about 2 seconds. Oral Medicine and Radiology, MCODS, Mangalore
Interpretation of the results

1. No response—a non-vital pulp.
However, a false negative response can be encoun- False positive response (when a patient reports sensation
tered when the tooth exhibits excessive calcification, in a tooth with a necrotic pulp) can be encountered under
immature apex, recent trauma and a premedicated the following conditions:
patient.
❍ Patient anxiety
2. Momentary mild-to-moderate response—normal pulp.
❍ Saliva conducting the stimulus to the gingiva
3. Exaggerate response that subsides quickly—reversible
❍ Metallic restoration conducting the stimulus to the adja-
pulpitis.
cent teeth
4. Painful response that lasts for several minutes after the
❍ Liquefaction necrosis conducting the stimulus to the
stimulus is removed—irreversible pulpitis.
attachment apparatus.
False negative response (although the pulp is vital, patient
Electric Pulp Tester
does not indicate that any sensation is felt in the tooth) can
Evaluation of responding nerve endings can be done with be encountered under the following conditions:
an electrical pulp tester (Figure 26). A small electric current
❍ Premedication with drugs or alcohol
delivered to the tooth causes a tingling sensation is sug-
❍ Immature teeth
gestive of vital pulp.
❍ Trauma
The teeth to be tested must be isolated and dried with
❍ Poor contact with the tooth
2 ⫻ 2 inch gauze and the area must be kept dry with a
❍ Inadequate media
saliva ejector. The patient must be made aware of the pro-
❍ Partial necrosis with vital pulp remaining in the apical
cedure. The electrode should be coated with a viscous con-
portion of the tooth
ductor. The electrode should then be applied to the dry
❍ Individuals with atrophied pulps or with high pain
enamel of the tooth being tested on the middle one-third
thresholds.
of the facial surface of the crown.
The digital reading that indicates the current flow should
Limitations
always start with zero. The current flow should be
increased slowly to allow the patient to respond for tingling There is no reasonable assurance; however, these nerves
sensation. If a positive response is not obtained the elec- are located in an intact pulp. Necrotic and degenerating
trode should be applied to several different locations on the pulp tissue often leaves an excellent electrolyte in the pulp
lingual and facial surfaces of the tooth to ensure that space. This electrolyte can easily conduct the electrical cur-
the negative response is not due to the result of improper rent to nerves further down into the pulp space, simulat-
electrode placement. Each tooth should be tested at least ing normal pulp response. This is more complicated in a
two or three times and an average result should be recorded. multi-rooted tooth, where the status of the pulp varies in
Enamel thickness influences the results. Thinner enamel each root.
in the anteriors yields faster response than thicker enamel Electrometric pulp tester should not be the primary
of the posteriors. Electrodes should not be applied on instrument of choice when assessing pulpal health. A posi-
restorations. tive cold test provides a more accurate response that is easier
423
to interpret. However, neither of these tests ensures vitality Dual Wavelength Spectrophotometry
of the pulp if the results are positive.
The above mentioned pulp vitality assessment methods Dual wavelength spectrophotometry (DWLS) is a method
rely on stimulation of A-delta nerve fibers and give no independent of a pulsatile circulation. The presence of
direct indication of blood flow within the pulp. These test- arterioles rather than arteries in the pulp and its rigid
ing methods have the potential to produce an unpleasant encapsulation by surrounding dentin and enamel make it
and occasionally painful sensation and inaccurate results difficult to detect a pulse in the pulp space. This method
(false positive or negative can be obtained in many measures oxygenation changes in the capillary bed rather
instances). In addition, each is a subjective test that than in the supply vessels and hence does not depend on
depends on the patient’s perceived response to a stimulus a pulsatile blood flow.
as well as the dentist’s interpretation of that response. It detects the presence or absence of oxygenated
Many studies have shown that blood circulation and not blood at 760 and 850 nm. The blood volume or concentra-
innervation is the most accurate determinant in assessing tion channel (760 ⫹ 850 nm) is arranged to respond lin-
pulp vitality as it provides an objective differentiation between early to the increase in light absorption. The oxygenation
necrotic and vital pulp tissue. channel (760 ⫺ 850 nm) senses the oxygenated blood
The newer techniques that have been tried effectively to because of the greater absorption at 850 nm as compared
evaluate the vitality of the pulp include pulse oximetry, dual to 760 nm.
wavelength spectrophotometry and laser Doppler flowm- In vivo and in vitro studies were conducted to differen-
etry. tiate between pulp chambers that were empty, filled with
oxygenated blood or fixed pulp tissue. DWLS was able to
differentiate with reproducible readings between a pulp
Pulse Oximetry chamber of a vital and non-vital tooth in vivo.
The pulse oximeter is a non-invasive oxygen saturation moni- In young children, in cases of avulsed and replanted
toring device widely used in medical practice for recording teeth with open apices, the blood supply is regained within
blood oxygen saturation levels during the administration the first 20 days after replantation but nerve supply lags
of intravenous anesthesia. behind.
The pulp oximeter is based on the modification of the Repeated spectrophotometric readings taken at the start
principle of Beer’s law, which relates the absorption of of the replantation and continuing up to 40 days later
light, by a solute to its concentration and optical properties at revealed an increase in blood oxygenation levels indicat-
a given light wavelength. It also depends on the absorbance ing a healing process and that the pulp of the avulsed
characteristics of hemoglobin in the red and infrared range. tooth was recovering. Hence, endodontic treatment need
In the red region, oxyhemoglobin absorbs less light than not be undertaken.
deoxyhemoglobin and vice versa in the infrared region. A major advantage is that it uses visible light that is
The system consists of a probe containing a diode that filtered and guided to the tooth by fiberoptics. Thus, unlike
emits light in two wavelengths: laser light, added eye protection is unnecessary for the
patient and the operator.
1. Red light of approximately 660 nm
2. Infra-red light of approximately 850 nm.
Laser Doppler Flowmetry
A silicon photo detector diode is placed on the opposing sur-
faces of the tooth, which is connected to a microprocessor. Laser Doppler flowmetry is a non-invasive, electro-optical
The probe is placed on the labial surface of the tooth technique, which allows the semi-quantitative recording of
crown and the sensor on the palatal surface. Ideal place- pulpal blood flow. The laser Doppler technique measures
ment of the probe is in the middle third of the crown. If placed blood flow in the very small blood vessels of the micro-
in the gingival third, disturbances from gingival circulation vasculature.
or any gingival trauma or bleeding will interfere with the This technique depends on the Doppler principle
readings. Incisally, less of pulp tissue is present for adequate whereby light from a laser diode incident on the tissue is
detection of the pulse. scattered by moving RBCs and as a consequence, the fre-
A number of clinical studies have proved that the pulse quency broadened. The frequency broadened light, together
oximetry is an effective and objective method of evaluat- with laser light scattered from static tissue is photo detected
ing dental pulp vitality. Though the surrounding insula- and the resulting photocurrent processed to provide a
tion of the enamel and dentin are hindrances to the blood flow measurement.
detection of a pulse in the pulp, it has proved to be a suc- The Doppler shifted laser light, back-scattered out of
cessful method in 70% of the clinical trials. It is also useful the tooth is detected by a photocell on the tooth surface.
in cases of impact injury where the blood supply remains The output is proportionate to the number and velocity
intact but the nerve supply is damaged. of the blood cells.
424
Radiographic Evaluation of Carious Lesions breakdown of the occlusal surface. Radiographically, an

extensive ill-defined radiolucent area is seen.
It has been proved time and again over the years that more
dental caries are detected by intraoral radiographs than by Proximal caries
clinical examination alone. Radiographic detection of dental
caries is based on the fundamental principle that as the The proximal carious lesion is clinically apparent as a
caries process proceeds, the mineral content of enamel and chalky white discoloration. It is estimated that it takes
dentin decreases with a resultant decrease in the attenua- about 4 years for an initial proximal carious lesion to be
tion of the X-ray beam as it passes through the teeth. This seen clinically. In the initial stages a discrete radiolucent
is recorded as a radiolucent image on the image receptor. ‘wedge-shaped defect’ or notch may be visible usually
Detection of the carious lesion may be influenced by a confined to the enamel cap.
number of factors such as the extent of demineralization Moderate carious lesions usually involve more than the
of the carious lesion (minimum of 40% demineralization outer half of the enamel to extend into the DEJ. Radio-
of the tooth structure is required to be evident on a radiographically, proximal caries can present either as a trian-
graph), location of the carious lesion, exposure parame- gle with its broad base at the surface of the tooth or a
ters, type of image receptor, image processing, display diffuse radiolucent image or a combination of the above.
system, viewing conditions, and ultimately, the training In advanced lesions the carious process involved the DEJ.
and experience of the human observer. Radiographically, the entire thickness of enamel is involved.
Cervical burnout, abrasion, attrition, proximal wear and The carious lesion may either present as a triangular pro-
restorative materials that appear radiolucent may mimic cess or appear ill-defined and diffuse or may appear as a com-
dental caries. bination of the above.
Radiographs are taken to evaluate the extent of the carious In a severe form of the proximal carious process, a radio-
lesions and to assess periapical changes secondary to the car- lucent area involving more than half the dentin and
ies attack. Intraoral periapical radiographs and bitewing approaching the pulp chamber is characteristic.
radiographs are widely used for assessing carious lesions.
White and McMullin (1986) concluded that both ultra- Facial, buccal and lingual caries
speed film and xeroradiography (XR) were somewhat pref- Smooth surface carious lesions will usually begin along the
erable to Ektaspeed film. Wenzel et al (1991) compared pits and fissures. Almost all carious lesions occurring on the
conventional film radiographs, digitized film radiographs, facial and lingual surface of teeth have a very sharply
and radiovisuography (RVG) for the detection of occlusal defined outline. In the initial stages these lesions are round
caries in non-cavitated extracted teeth. Histologic sections and as the carious process advances they appear as large
served as the validation criterion. The two digital methods elliptical defects.
with contrast enhancement tended to perform better than, Occasionally, these carious lesions may be difficult to
although not significantly different from, the other three detect as they may be superimposed over the DEJ mimick-
methods. ing occlusal caries.
The authors concluded that radiography can much Generally, these lesions appear as well-defined circular
improve diagnosis compared to clinical inspection. radiolucent areas surrounded by dense area of non-
They also concluded that digital techniques, no matter carious tooth structure.
which, improve the sensitivity without substantial loss of
specificity. Root caries
Occlusal caries Root surface carious lesions are those that involve the cemen-
tum and dentin. These are typically seen in teeth which
Incipient carious lesions occurring on the occlusal surface
exhibit gingival recession or interdental bone loss. Older
of teeth usually begins in the pits and fissures. The carious
individuals are more likely to show the presence of root
lesion starts at the side of the fissure than at the base and
surface carious lesions. Radiographically, a saucer like or
it tends to penetrate perpendicular toward the DEJ.
a notched radiolucency is seen at the cervical margin of
Radiographs can help to detect occlusal lesions only
the tooth that usually extends apically along the root surface.
when the carious process reaches the dentin.
A moderate carious lesion will generally show defini-
Recurrent caries
tive radiographic findings such as a broad-based thin radio-
lucent zone in the dentin with little or no changes apparent Recurrent caries or secondary caries occurs adjacent to a
in the enamel. restoration. It may result from poor marginal adaptation
As the carious process advances into a severe form, the of a restoration, which allows marginal leakage; inade-
underlying dentin is extensively involved and hence fails quate extension of a restoration, incomplete excavation
to support the overlying enamel structure resulting in the of the primary carious lesion and fractured restoration.
425
Radiographs show radiolucent areas adjacent to a restora- Concepts of Fascia and Fascial Spaces
tion. Restorative materials such as composite, silicate and
acrylic can resemble recurrent caries. These radiolucent Fasciae are extensive, broad sheets of dense connective tis-
restorative materials can be differentiated from recurrent sue. Fasciae envelop anatomic structures such as the mus-
caries by their well-defined and smooth outlines (Figure 27). cles, glands and organs and thereby separate structures that
must move over each other during movement. These fas-
Rampant caries ciae also serve as pathways for the course of vascular and
neural structures.
Rampant caries usually occurs in children. There will be Shapiro defined fascial spaces as potential spaces
extensive smooth surface caries involving many teeth. between layers of fascia. These spaces are normally filled
with loose connective tissue and vital structures. The use
Radiation caries of the term ‘space’ is a misnomer as tissues show no evi-
Radiation caries can be considered as a type of rampant dence of voids. The pus destroys the loose connective tissue
caries seen in patients who receive radiation therapy for and separates the anatomical boundaries of the compartment
head and neck tumors. It occurs secondary to xerostomia. as it increases in volume, thus creating an abscess cavity
Caries begins at the cervical region and may aggressively bounded by muscles, tissues and bone.
encircle the tooth causing entire crown to be lost with
only root fragments remaining in the jaws. Radiograph Superficial fascia
shows dark radiolucent shadows appearing at the cervical
It is a layer of dense connective tissue that courses deep to
margins of teeth.
the subcutaneous tissue throughout the entire body. Below
the mouth the muscles of facial expression lie deep to the
superficial fascia, whereas in the upper face the muscles of
FASCIAL SPACE INFECTION facial expression are positioned superficial to this layer. The
superficial cervical fascia is a sheet of fibrous connective
Cellulitis or fascial space infection is characterized by a tissue that encircles the head and neck and is attached to
diffuse inflammation of soft tissues. Fascial space infections the fascia of the thorax, shoulders and axilla. It contains
in the head and neck region usually occur as an extension the platysma muscle.
of periapical, periodontal and pericoronal infections.
Deep fascia
The deep cervical fascia is divided into the superficial or
Figure 27 investing layer, the middle or visceral layer and the deep
or prevertebral layer. The superficial layer of the deep cer-
vical fascia completely encircles the neck with its attach-
ments being superiorly: the external occipital protuberance,
the mastoid process and the zygoma, and anteriorly the
mandible and the hyoid bone and inferiorly the scapula,
the clavicle and the manubrium of the sternum. It envelops
the trapezius and sternocleidomastoid muscles. The middle
layer encircles the viscera of the neck including the pharynx,
esophagus, larynx, trachea and thyroid gland. It also encloses
the strap muscles anteriorly. The deep layer envelops the
paraspinous muscles and vertebral bodies. More importantly,
anterior to the vertebral bodies it divides into a preverte-
bral layer and a more anterior alar layer. This creates three
potential spaces, namely, the prevertebral space, the danger
space and the retropharyngeal space. The carotid sheath is
formed from contributions of all three layers of the deep cer-
vical fascia and runs from the base of the skull to the level
of the clavicle.
The deep spaces of the neck can be divided into those
Radiograph showing recurrent carious lesion below which involve the entire length of the neck (including the
the margins of the radiopaque restorative material. retropharyngeal space, the danger space and the preverte-
Courtesy: Department of Oral Medicine and bral space), those that are limited to above the hyoid bone
(the submaxillary, the sublingual and the parapharyngeal
426
spaces) and those limited to below the hyoid bone. Refer to The parapharyngeal space can be compared to an
line diagrams (Figures 28 to 32). inverted cone with its base lying superiorly at the base of
The retropharyngeal space is the potential space that the skull and its apex inferiorly at the hyoid bone. It is
exists between the posterior aspect of the visceral layer divided into a prestyloid and a poststyloid component. Its
and the alar division of the deep layer. It extends from the medial and lateral borders are, respectively, the lateral pha-
base of the skull to the level of the first or second thoracic ryngeal wall and the superficial layer of the deep cervical
vertebrae. It contains two lateral chains of lymph nodes fascia as it overlies the mandible, the parotid gland and the
separated by a midline raphe. The danger space lies between internal pterygoid.
the alar and prevertebral layers of the deep cervical fascia. The submandibular space is divided by the mylohyoid
It extends from the base of the skull to the posterior medi- muscle into the sublingual space above and the submaxillary
astinum at the level of the diaphragm and is limited later- space below. These two spaces communicate freely around
ally by its fusion with the prevertebral layer and the the posterior edge of the mylohyoid muscle. The entire
vertebral transverse process. The prevertebral space lies space is bounded by the mandible anteriorly and laterally.
between the vertebral bodies and the prevertebral layer of The hyoid bone limits its inferior aspect and the intrinsic
the deep cervical fascia. It extends from the base of the muscles of the base of tongue from its posterior border.
skull to the level of the coccyx. The sublingual space contains the sublingual gland, the
Figure 28 Figure 30
Buccinator
Tongue Temporalis muscle
muscle
Superficial temporal space
Buccal space
Infratemporal space
Sublingual space
Lateral pterygoid muscle
Submandibular
Pterygomandibular space
space
Masseteric space
Illustration showing submandibular and buccal spaces
Masseter muscle
Figure 29 Illustration showing infratemporal and

superficial temporal spaces
Parotid gland Parapharyngeal space
Superior Figure 31
Parotid constrictor
muscle Lateral pharyngeal space
space
Prevertebral space
Masseter Retropharyngeal space
muscle Peritonsillar
space Superior constrictor muscle
Pterygoid muscle
Submasseteric
space Pterygomandibular Mandible
space
Buccal space Masseter muscle
Buccinator
muscle Buccinator muscle
Mylohyoid muscle
Illustration showing pterygomandibular and Illustration showing retropharyngeal and

submasseteric spaces lateral pharyngeal spaces
427
❍ By lymphatics to the regional lymph nodes and even-

Figure 32
tually into the blood stream
Buccopharyngeal ❍ By the blood stream.
fascia
General factors
Alar fascia ❍ Host’s resistance or immunocompetence of the host
❍ Virulence of microorganisms.
Prevertebral fascia
Local factors Intact anatomical barriers:
❍ Alveolar bone
❍ Periosteum
Retropharyngeal ❍ Adjacent muscles and fascia.
space
LUDWIG’S ANGINA
Prevertebral space
The necrotizing fasciitis, Ludwig’s angina, was first

described by German surgeon Karl Wilhelm Friedrich von
Ludwig in 1836.
It is a potentially life-threatening, rapidly expanding,
diffuse inflammation of the submandibular and sublingual
spaces that occurs most often in young adults with dental
Prevertebral fasciae and prevertebral space
infections.
hypoglossal nerve and Wharton’s duct. The submandibular Clinical features

space contains the submandibular gland. Ludwig’s angina begins as a mild infection and can rapidly
The anterior visceral space lies in the anterior aspect of progress to brawny bilateral induration of the upper neck.
the neck, is enclosed by the visceral layer and completely Anxiety, cyanosis and sitting posture are late signs of impend-
surrounds the trachea, esophagus and thyroid gland. It ing airway obstruction, and indicate the need for an imme-
extends from the thyroid cartilage to the level of the fourth diate artificial airway. The most serious complication of
thoracic vertebrae in the superior mediastinum. Ludwig’s angina is asphyxia caused by expanding edema of
Pain and swelling are commonly present and may help soft tissues of the neck.
identify the space involved. Dysphagia is more common The symptoms include severe neck pain and swelling,
when the parapharyngeal and retropharyngeal spaces are fever, malaise and dysphagia. Stridor suggests an impending
involved. Trismus is seen with involvement of the sub- airway crisis. Patient may report of severe toothache, trismus
maxillary space or anterior aspect of the parapharyngeal and foul breath. The mortality rate can go up to 20–50%
space. Other symptoms may include respiratory distress if if the infection spreads to the mediastinum, carotid sheath,
the abscess partially compromises the airway or dental skull base and meninges.
complaints if this is the source of infection. Other findings Complications such as descending necrotizing mediastini-
may include oropharyngeal abnormalities such as swelling tis usually occurs through the retropharyngeal space (71%)
of the lateral or posterior pharyngeal walls, in parapha- and the carotid sheath (21%).
ryngeal or retropharyngeal abscess.
Nearly 40% of deep space infections are caused by mixed Predisposing factors
flora. Other changes include the emergence of gram-negative
organism, primarily Klebsiella pneumoniae, as important These include deep dental caries, severe periodontal infec-
pathogens as well as an increase in the prevalence of anaer- tion, a compromised immune system and recent trauma.
obic infections. Notwithstanding, streptococcal species, pri- Ludwig’s angina in children can occur de novo, without any
marily alpha Streptococcus and Staphylococcus aureus, are apparent precipitating cause.
still the most commonly isolated organisms.
Mode of spread
Spread of orofascial infection Infections in the head
Ludwig’s angina is a rapidly progressive, potentially ful-
and neck region usually spread by hydrostatic pressure,
minant cellulitis involving the sublingual, submental and
along the path of least resistance.
submandibular spaces. It typically originates from an
Routes of spread and factors affecting spread of oral infected or recently extracted tooth, most commonly the
infections (Table 2) lower second and third molars. The submandibular space is
❍ By direct continuity through tissues involved by penetration of the thin inner cortex of the
428
Table 2 Head and neck fascial spaces
Space Boundaries Contents
Submental space Lateral: Lower border of mandible and anterior bellies of digastric Submental lymph nodes and anterior
muscles jugular veins
Superior: Mylohyoid muscle
Inferior: Suprahyoid portion of the investing layer of deep cervical
fascia
Submandibular space Anteromedially: The floor is formed by mylohyoid muscle, which Superficial lobe of submandibular salivary
is covered by loose areolar tissue and fat gland and submandibular lymph nodes,
Posteromedially: The floor is formed by hypoglossus muscle facial artery and vein
Superolaterally: Medial surface of mandible below the mylohyoid
ridge
Anterosuperiorly: Anterior belly of digastric
Posterosuperiorly: Posterior belly of digastric, stylohyoid and the
stylopharyngeus muscles
Laterally: Platysma and skin
Sublingual space Superiorly: Mucosa of floor of the mouth Geniohyoid and genioglossus muscles, the
Inferiorly: Mylohyoid muscle hyoglossus muscle complex
Laterally: Medial side of the mandible, above the mylohyoid It also contains submandibular salivary
muscles gland, sublingual salivary gland, lingual
Medially: Hyoglossus, genioglossus and geniohyoid muscles nerve and hypoglossal nerve
Posteriorly: Hyoid bone laterally and inferiorly by mylohyoid
muscle and lingual side of mandible
Canine space Inferiorly: Caninus muscle
Anteriorly: Orbicularis oris
Posteriorly: Buccinator muscle
Medially: Anterolateral surface of maxilla
Buccal space Buccal space is the potential space between buccinator and Buccal pad of fat, Stensen’s duct, facial
masseter muscle artery
Anteromedially: Buccinator muscle
Posteromedially: Masseter overlying the anterior border of ramus
of mandible
Laterally: By forward extension of deep fascia from the capsule of
parotid gland and by platysma muscle
Inferiorly: Limited by the attachment of the deep fascia to the
mandible and by depressor anguli oris
Superiorly: The zygomatic process of the maxilla and the
zygomatous major and minor muscles
Infratemporal fossa space Bounded laterally, by ramus of mandible, temporalis muscle and Medial and lateral pterygoid muscles,
(retrozygomatic space) its tendon maxillary artery, mandibular nerve and
Medially: Medial pterygoid plate, lateral pterygoid muscle, medial middle meningeal artery
pterygoid muscle, lower part of temporal fossa of the skull and
lateral wall of pharynx
Superiorly by infratemporal surface of greater wing of sphenoid
and by zygomatic arch
Inferiorly: Lateral pterygoid muscle, which forms the floor of the
fossa, and its lower head is said to mark the border between
pterygomandibular and infratemporal spaces
Anteriorly: Infratemporal surface of maxilla
Posteriorly: Parotid gland
Temporal space Spaces in relation to temporalis muscle:
(masticatory space) 1. Superficial temporal space—lies between temporal fascia and
temporalis muscle
2. Deep temporal space—lies deep to temporalis muscle and skull
Communicates with infratemporal and pterygopalatine fossa
(Contd.)
429
Table 2 Continued
Space Boundaries Contents
Parotid space The space is formed by splitting of the superficial layer of deep Parotid gland and parotid lymph nodes,
cervical fascia surrounding the parotid gland, and lies posterior facial nerve, retromandibular vein, and
to the masticator space external carotid artery
Inferiorly: Stylomandibular ligament, which separates parotid
space from the mandibular space
Submasseteric space Anterior: Anterior border of masseter muscle and buccinator Masseter, lateral and medial pterygoids,
(masticatory space) Posterior: Parotid gland and posterior part of masseter part of mandible and branches of
Inferior: Attachment of the masseter to the lower border of mandible mandibular division of trigeminal nerve
Medial: Lateral surface of the ramus of mandible
Lateral: Medial surface of the masseter muscle
Pterygomandibular space Lateral: Medial surface of ramus of mandible Lingual nerve, mandibular nerve, inferior
Medial: Lateral surface of medial pterygoid muscle alveolar nerve or mandibular artery,
Posterior: Parotid gland mylohyoid nerve
Anterior: Pterygomandibular raphe
Superior: Lateral pterygoid muscle
Parapharyngeal space
Lateral pharyngeal and
retropharyngeal spaces
These spaces form a ring
around pharynx and
communicate with
submandibular space and
retromandibular space
Lateral pharyngeal space Superiorly: Base of skull Anterior compartment: Lymph nodes,
Inferiorly: Hyoid bone (submandibular gland and posterior belly ascending pharyngeal, facial artery,
of digastric) loose areolar connective tissue
Anteriorly: Bounded by pterygomandibular raphe Posterior compartment: Carotid sheath
Laterally: Bounded by the ramus of mandible, insertion of medial (internal jugular vein, internal carotid
pterygoid muscle and deep lobe of parotid gland artery and vagus nerve), glossopharyngeal
Medially: Bounded by pharyngeal wall and pharyngeal constrictors nerve, spinal accessory nerve, hypoglossal
Posteriorly: Bounded by styloid muscle and upper part of carotid nerve and cervical sympathetic trunk
sheath, prevertebral fascia
Retropharyngeal space Laterally: Carotid sheath
The retropharyngeal space is continuous with retroesophageal
space into the posterior mediastinum to the level of the sixth
thoracic vertebra
There are no middle attachments in the retropharyngeal space,
thereby permitting unimpeded inferior extension of
inflammatory and neoplastic processes into mediastinum
Spread of infection through the carotid sheath into the
mediastinum is referred to as ‘spread of infection via the
Lincoln’s highway’
mandible by periapical dental abscesses. Spread to the sub- isolates vary and are often mixed. Alpha-hemolytic strepto-
lingual space is around the posterior margin of mylohyoid cocci, staphylococci and Bacteroides are commonly
muscle. It has, however, been reported as a result of man- reported. Other anaerobes such as peptostreptococci, pepto-
dibular fracture, submandibular sialadenitis, peritonsillar cocci, Fusobacterium nucleatum, Veillonella species and
abscess, epiglottitis and oral malignancies. spirochetes are also seen. A foul breath/odor usually indi-
cates the presence of an anaerobe. Gram-negative organ-
Microbiology
isms such as Neisseria catarrhalis, Escherichia coli,
Causative bacteria include many gram-negative and anaero- Pseudomonas aeruginosa and Hemophilus influenzae have
bic organisms, streptococci and staphylococci. The bacterial also been reported.
430
Anatomical considerations Endotracheal intubation is associated with high rate of

failure with acute deterioration in respiratory status result-
The submandibular space is composed of two spaces sepa-
ing in emergency ‘slash’ tracheostomy.
rated anteriorly by the mylohyoid muscle: the sublingual
Although distorted anatomy, edema, and secretions may
space, which is superior, and the submaxillary space,
contribute to difficulty with fiberoptic intubation, in skilled
which is inferior. The spread of infection is halted anteri-
and experienced hands, flexible fiberoptic nasal intuba-
orly by the mandible and inferiorly by the mylohyoid
tion is the preferred method of airway management and
muscle. The infectious process expands superiorly and
has a high rate of success. Application of topical anesthesia
posteriorly, elevating the floor of the mouth and the
enables the patient to tolerate the procedure with greater
tongue.
comfort.
The hyoid bone limits the process inferiorly, and swell-
When fiberoptic bronchoscopy is not feasible, not
ing spreads to the anterior aspect of the neck, causing
available, or has failed, cricothyrotomy and tracheostomy
distortion and a ‘bull neck’ appearance. This then evolves
are the options. Tracheostomy may be difficult or impos-
to an infectious compartment syndrome of the subman-
sible in advanced cases of neck infection because of the
dibular and sublingual spaces.
position needed for the procedure and anatomical distor-
tion of the anterior neck. The choice of airway maneuvers
Investigations must be individualized, depending on the judgment and
Conventional radiographs such as intraoral periapical experience of the physician in charge.
radiographs or orthopantomograph (OPG) may help to Recommended initial antibiotics are high-dose penicillin G,
identify the offending tooth. However, a contrast- sometimes used in combination with an antistaphylococcal
enhanced computed tomography (CECT) scan is helpful in drug or metronidazole. In penicillin-allergic patients,
assessing the extent of spaces involved. clindamycin hydrochloride is a good choice.
Alternative choices include cefoxitin sodium or combi-
Treatment nation drugs such as ticarcillin–clavulanate, piperacillin–
tazobactam or amoxicillin–clavulanate (Augmentin).
The treatment plan for each patient should be individual- Intravenous dexamethasone sodium phosphate (Decad-
ized and based on a number of factors. The stage of the ron), given for 48 hours, has been beneficial in reducing
disease and co-morbid conditions at the time of presenta- edema, which helps maintain airway integrity and enhances
tion and the resources available. antibiotic penetration.
Treatment includes assessment and protection of the Dexamethasone reduces edema and cellulitis, provides
airway, use of intravenous antibiotics, surgical evaluation the initial chemical decompression protecting the airway
and, if necessary, operative decompression. Intravenous and allows improved antibiotic penetration into the area.
dexamethasone and nebulized adrenaline have been used Surgical drainage may be indicated if no clinical
to reduce upper airway edema in such cases to defer or improvement is seen within 24 hours.
avoid airway instrumentation altogether. Distorted airway
anatomy, tissue immobility, and limited access to the
mouth make orotracheal intubations by direct laryngos-
copy difficult. In advanced cases, induction of general OSTEOMYELITIS
anesthesia is dangerous because this may precipitate com-
plete airway closure and make mask ventilation and intu- By strict definition, osteomyelitis is the inflammation of
bation impossible. Securing of the airway in the awake medullary portion of bone. However, clinical evidence has
state is therefore the safest option. shown that it is seldom limited to the endosteum and it usu-
Blind nasal intubation is to be avoided as, besides hav- ally affects the cortical plates and the periosteum.
ing a high failure rate, it could cause catastrophic bleed- Considering the extent of the bone involvement, osteomy-
ing, laryngospasm, airway edema, rupture of pus into the elitis may best be described as an inflammatory condition
oral cavity and aspiration. Complete airway obstruction of bone that usually begins as an infection of the medul-
could be precipitated, potentially necessitating an emer- lary cavity which rapidly involves the haversian system and
gency cricothyrotomy. Classically, tracheostomy was con- quickly extends to the periosteum of the area.
sidered as the standard of care for establishment of a Osteomyelitis can be broadly categorized as exogenous
definitive airway. osteomyelitis (47%), osteomyelitis secondary to vascular
Elective awake tracheostomy has been suggested for all insufficiency (34%) and hematogeneous osteomyelitis (19%).
patients with deep neck infections to avoid the dangers The implantation of pins, plates, screws, dental implant
of emergency tracheostomy in a severely compromised and artificial joint can also seed infection as a nidus for
airway. pathogens, and therefore create postoperative osteomyelitis.
431
Predisposing factors either of the two major events: extension of a periapical

abscess, periodontal abscess or post-traumatic/post-surgical
Conditions affecting host resistance Systemic, meta-
complication.
bolically compromised individuals (factors such as age of
Naidu et al (2008) reported a case of osteomyelitis of
patient, malnutrition, immunosuppression and congenital
the mandibular symphysis caused by bite of a brown
or acquired pathophysiology disrupting microvascular per-
recluse spider.
fusion of the calcified tissue structure and investing soft
Other predisposing conditions include bony pathologies
tissue envelope).
such as Paget’s disease and osteopetrosis, compound frac-
Examples include diabetes, AIDS, use of steroids,
tures, history of local irradiation, host-debilitating condi-
agranulocytosis, leukemia, severe anemia and cancer che-
tions such as diabetes mellitus and long-term systemic
motherapy.
steroid therapy.
Conditions affecting jaw vascularity Conditions that The mandible is more prone osteomyelitis compared to
affect the vascularity of the jaw bones include radiation, other craniofacial bones due to its morphological charac-
bone malignancy, osteoporosis, osteopetrosis, Paget’s dis- teristics such as a relatively thin cortical plate and poor
ease of bone, fibrous dysplasia and bone necrosis (mercury, vascular supply to its medullary portion. The regions in
bismuth, arsenic poisoning, long-term chemotherapeutic the mandible that are at higher risk of perforation include
agents like bisphosphonates). the lingual aspect of molar teeth and in the labial aspect
of anterior teeth (Thadepalli and Mandal, 1988).
Microbiology of osteomyelitis
Microorganisms specific for different age groups Pathogenesis
(Osteomyelitis of other bones) With the initiation of the infection, the intramedullary
Newborns (younger than 4 months): S. aureus, Enterobacter pressure increases substantially, compromising the vascu-
species, and group A and B Streptococcus species. larity, thus heralding the beginning of a stage of bony
Children (aged 4 months to 4 years): S. aureus, group A necrosis.
Streptococcus species, Hemophilus influenzae, and Entero- The purulent material traverses networks of Haversian
bacter species. and perforating canals, eventually accumulating under the
Children, adolescents (aged 4 years to adult): S. aureus periosteum and lifting it from the bony cortex.
(80%), group A Streptococcus species, H. influenzae, and As the pus accumulates, periosteal perforation can
Enterobacter species. occur ultimately forming abscesses and often fistulous
Adult: S. aureus and occasionally Enterobacter or tracts within mucosal and cutaneous tissues. In chronic
Streptococcus species. cases granulation tissue, dead bone (sequestrum) separated
from surrounding healthy tissue, and eventually, a reac-
Osteomyelitis of jaw bones The microorganisms respon-
tive sleeve of new periosteal tissue (involucrum) formation
sible for osteomyelitis affecting the jaw bones reflect the
may also be seen.
polymicrobial nature of odontogenic infections in general
and agents associated with suppurative infection and peri-
apical abscesses in specific.
Fusobacterium nucleatum, Prevotella intermedia, Pepto- Classification of Osteomyelitis
streptococcus, Actinomyces and Streptococcus species (alpha Waldvogel classification system for
hemolytic) are the predominant isolates from osteomyelitis osteomyelitis
affecting the jaws.
Hosts who have serious underlying illnesses may exhibit ❍ Hematogeneous osteomyelitis
the presence of facultative gram-negative bacilli and S. aureus. ❍ Osteomyelitis secondary to contiguous focus of infection
Mycobacterium tuberculosis, Treponema pallidum and – No generalized vascular disease
Actinomyeces spp. produce specific forms of osteomyelitis. – Generalized vascular disease
Tubercular osteomyelitis of the spine is referred to as ❍ Chronic osteomyelitis (necrotic bone).
Pott’s disease. Burkholderia cepacia complex have been
implicated in vertebral osteomyelitis in intravenous drug Cierny–Mader staging system for osteomyelitis
abusers. Systemic mycotic (fungal) infections may also
cause osteomyelitis. The two most common pathogens Anatomic type
involved in such infections are Blastomyces dermatitidis Stage 1: Medullary osteomyelitis—involved medullar bone
and Coccidioides immitis. without cortical involvement; usually hemato-
geneous
Osteomyelitis of jaw bones Osteomyelitis of the mandible Stage 2: Superficial osteomyelitis—less than 2 cm bony
occurs primarily from odontogenic infection caused by defect without cancellous bone
432
Stage 3: Localized osteomyelitis—less than 2 cm bony affected bone is warm, erythematous and tender on palpation.
defect on radiograph, defect does not appear to Patients complain of fever, malaise and anorexia.
involve both the cortices Teeth in the area of involvement are loose and tender on
Stage 4: Diffuse osteomyelitis—defect more than 2 cm, percussion. Pus discharge may be present around the sul-
pathologic fracture, infection, nonunion. cus of the tooth (Figure 33A, B). Patients may complain of
a fetid odor. Paresthesia and anesthesia of lip is seen. The
Physiologic class
WBC count is elevated. If the acute phase is not controlled
❍ A host: Healthy within 10–14 days after onset then subacute suppurative
❍ B host osteomyelitis is established.
– Bs: Systemic compromise
– Bl: Local compromise
– Bls: Local and systemic compromise
❍ C host: Treatment worse than the disease. Generally, no radiographic findings are associated with
acute osteomyelitis. A minimum of 40% demineralization
Factors affecting immune surveillance, metabolism and
of bone is required to be evident. Radiographic evidence of
local vascularity are:
acute osteomyelitis may be detected at least 4–14 days
❍ Systemic factors (Bs): Malnutrition, renal or hepatic fail- after onset of acute osteomyelitis. The full extent of bone
ure, diabetes mellitus, chronic hypoxia, immune dis- dissolution may be evident 3 weeks after the initial attack.
ease, extremes of age, immunosuppression or immune The radiographic features always lag behind the actual prog-
deficiency. ress of the condition. Bony trabeculae appear indistinct and
❍ Local factors (Bl): Chronic lymphedema, venous stasis, fuzzy. The initial radiolucent areas appear as ‘moth-eaten’
major vessel compromise, arteritis, extensive scarring, regions with ill-defined margins.
radiation fibrosis, small-vessel disease, neuropathy,
tobacco abuse. Histopathologic features
Histologic sections show medullary spaces filled with inflam-
Classification based on the clinical course and
matory exudate, chiefly composed of neutrophils. Other
radiographic features
cells that are seen include lymphocytes and plasma cells.
Suppurative osteomyelitis Osteoblasts bordering the bony trabeculae are generally
❍ Acute suppurative osteomyelitis destroyed, leading to loss of trabecular viability and slow
❍ Chronic suppurative osteomyelitis resorption of the bone.
❍ Primary (no preceding acute phase)
❍ Secondary (follows acute phase) Management
❍ Infantile osteomyelitis.
High dose intravenous antibiotic therapy, identification and
Non-suppurative osteomyelitis correction of host compromise factors is required. Careful
❍ Focal sclerosing osteomyelitis (condensing osteitis) enquiry and investigation may reveal diabetes, autoimmune
❍ Diffuse sclerosing osteomyelitis disease, alcohol-starvation syndrome, intravenous drug
❍ Proliferative periostitis (periostitis ossificans) abuse, severe anemia, HIV, steroid, chemotherapy. Factors
❍ Osteoradionecrosis. that delay recovery should be identified and corrected. Initial
management is often aided by hospitalization. Once the
Clinical features infection is controlled treatment is continued on an outpa-
tient basis using home intravenous therapy with percuta-
Osteomyelitis may be acute, subacute or chronic, and
neous indwelling catheter and antibiotic pumps.
presents a different clinical course depending on its
Specimens should be obtained for Gram staining, aerobic
nature.
and anaerobic cultures and antibiotic sensitivity testing.
Conventional radiographs and bone scans should be
obtained to determine the extent of the disease and caus-
Acute Suppurative Osteomyelitis
ative factors such as fractures and presence of sequestra.
In maxilla, the disease usually remains fairly well local- Once the acute stage has resolved with antibiotics,
ized to the area of infection. In mandible, bone involvement other procedures like sequestrectomy, debridement, direct
tends to be more diffuse and widespread. It may occur at placement of antibiotics into the wound by means of
any age. Infants affected are seriously ill and may not indwelling catheters or antibiotic impregnated beads,
survive. hyperbaric oxygen therapy, decortication, resection of
In adults, there is severe pain and elevation of tempera- infected bone and immediate or late bone graft recon-
ture with regional lymphadenopathy. The skin overlying the struction.
433
Figure 33
A B
Intraoral sinus opening and diffusely enlarged gingiva associated with acute osteomyelitis.
Antibiotic therapy Antibiotics of value in the treatment Penicillin V, 2 g, in combination with metronidazole,
of osteomyelitis are penicillins, extended spectrum penicil- 0.5 g q8h PO, for 2–4 weeks after last sequestrum removed
lin, semisynthetic penicillins, clindamycin, cephalosporins and patient without symptoms.
and metronidazole. Or
Many organisms responsible for osteomyelitis are now
penicillin resistant, including prevotella, porphyromonas Clindamycin, 600–900 mg q6h IV, then clindamycin,
and fusobacterium. Therefore, a drug resistant to these 300–400 mg q6h PO.
organisms such as metronidazole should be added to pen- Or
icillin. Some of the newer antibiotics providing effective Cefoxitin (mefoxin), 1 g q8h IV or 2 g q4h IM or IV, until
coverage for the more refractory organisms include metro- no symptoms, then switch to cephalexin, 500 mg q6h PO,
nidazole, clindamycin, ticarcillin and clavulanic acid, for 2–4 weeks for penicillin allergic patients clindamycin
a variety of cephalosporins, carbapenems, vancomycin in (as above) cefoxitin (as above), if allergy not of anaphy-
combination with other antibiotics and fluoroquinolones lactoid type.
such as ciprofloxacin.
Antibiotic regimen for osteomyelitis of jaw
Chronic Suppurative Osteomyelitis
Regimen 1: For hospitalized/medically compromised patient
or when intravenous therapy is indicated. This may develop after the acute phase of the disease has
Aqueous penicillin, 2 million units IV q4h, in combina- regressed, or sometimes can occur without a preceding acute
tion with metronidazole 500 mg q6h. When improved for phase.
48–72 hours switch to penicillin V, 500 mg per oral q4h, in Clinical features are similar to that of the acute type, except
combination with metronidazole 500 mg PO q6h, for an that all signs and symptoms are milder. Pain is less severe.
additional 4–6 weeks. Temperature is mildly elevated. Teeth may tend to become
Or loose and occasionally tender on palpation. Acute exacer-
bation may occur periodically.
Ampicilin/sulbactam, 1.5–3 g IV q6h when improved
Intraoral or extraoral draining fistulas may be seen
for 48–72 hours, switch to amoxicillin/clavulanate (aug-
(Figure 34). The cortical plates may be expanded and in
mentin), 875/125 mg per oral b.i.d., for an additional
severe cases pathological fracture may be evident. The bone
4–6 weeks.
is thickened and assumes a ‘wooden’ character on palpation
Regimen 2: For outpatient treatment. (Figure 35). Leukocytosis is slightly more than the normal.
434
Figure 34 Figure 35
Extraoral swelling in chronic suppurative osteomyelitis.

Extraoral draining sinus in chronic suppurative MCODS, Mangalore
osteomyelitis. Courtesy: Department of
Figure 36
A B
Radiographs showing ill-defined radiolucent areas with moth-eaten appearance characteristic of chronic suppurative osteomyelitis.
Histologic features extending to involve the lower border of the mandible in

severe cases (Figure 36A, B). Evidence of sequestrum/
The typical histological picture shows intertrabecular areas involucrum may be found. Extensive destruction of the
of bone filled with chronically or subacutely inflamed bone may cause pathologic fracture.
fibrous connective tissue. Presence of scattered sequestra
and pockets of abscess are common.
Management
After intraoral debridement, saucerization or decortication,
Radiographs will reveal a poorly defined radiolucency small pediatric nasogastric feeding tubes, French catheters,
(moth-eaten appearance) within the body of the mandible or polyethylene irrigation tubes 3–4 mm in diameter and
435
6–10 inches long are placed into the bone bed through necessary to resolve low-grade persistent chronic osteo-
separate skin incisions along the lateral bony surface through myelitis. Using an extraoral approach bone is debrided
holes drilled into bone. These drains are held to the skin with until bleeding surfaces are encountered distally and prox-
sutures or tape. Alternatively, two tubes may exit from one imally. Single or multiple blocks of autologous corticocan-
stab incision or a single tube may be used for instillation and cellous bone grafts are placed for immediate reconstruction.
suction.
The tubes are flushed with saline solution and the irriga-
tion solution is introduced through one tube while the other Infantile Osteomyelitis
tube is connected to low pressure suction. Various irrigating
Infantile osteomyelitis usually occurs few weeks after
solutions can be used often containing antibiotics, wetting
birth and generally involves the maxilla. This uncommon
agents and proteolytic enzymes.
condition involves risks with ocular, intracranial spread
Antibiotics in high concentration also may be placed in
and facial deformities.
direct contact with the bone manually or with an implantable
It is believed to occur by hematogeneous route or from
pump.
perinatal trauma.
Tobramycin or gentamicin is contained in acrylic resin
Generalized symptoms include fever, irritability, mal-
bone cement beads. Impregnated chains of beads are useful,
aise, anorexia, dehydration and even convulsions and
especially in chronically infected bone associated with frac-
vomiting.
tures and in chronic sclerosing osteomyelitis refractory to
Facial cellulitis is seen centered about the orbit associated
systemic antibiotics. The beads and drain are left in place
with inner and outer canthal swelling, palpebral edema, clo-
10–14 days and then removed through a small incision.
sure of the eye and proptosis. Purulent discharge from the
Hyperbaric oxygen (HBO) therapy has been used to pro-
nose and the medial canthus may be evident.
mote healing in refractory chronic osteomyelitis.
In the acute stage, surgery should be limited to removal
of severely loose teeth and bone fragments and incision, Chronic Focal Sclerosing Osteomyelitis
drainage of fluctuant areas. Deeply located or extensive (Condensing Osteomyelitis or Osteitis)
abscesses may require treatment with the patient under
general anesthesia. Condensing osteitis is a focal sclerosing form of osteomy-
elitis that occurs in very mild infection (microorganisms
Sequestrectomy Sequestra can be cortical, cancellous or of low virulence) or when the host immunity is at its
cortical-cancellous and generally are not seen until at peak.
least 2 weeks after the onset of infection. These can persist The bone reacts unusually to infection by laying down
for several months. In the chronic state, the involucrum or bone rather than getting destructed. In individuals with
shell of bone produced by the periosteum may be perfo- high tissue resistance, the infection acts as a stimulant
rated by tracts (cloacae) through which the pus escapes to thereby resulting in proliferation of the tissue.
epithelial surfaces. Sequestra are avascular, so are poorly
penetrated by antibiotics.
Clinical and radiographic features
Once the sequestra are formed, they can be removed
with a minimum of surgical trauma. Condensing osteitis is generally seen in young adults. The
mandibular first molars are most commonly affected. The
Saucerization Saucerization is the unroofing of the bone
tooth in question presents with a deep carious lesion. Patients
to expose the medullary cavity for thorough debridement.
may either present with mild pain or may occasionally be
Saucerization is useful in chronic osteomyelitis because it
asymptomatic.
permits removal of formed and forming sequestra. The pro-
Radiographic evaluation will reveal a deep carious
cedure can be done after resolution of the acute phase. This
lesion approximating pulp with a well-defined radiopacity
decompresses the bone to allow ready extrusion of pus, debris
at the periapex of one or more roots. The root outline is
and avascular fragments. The patient is more comfortable. usually sharply defined and visible. The boundaries of the
Decortication This refers to removal of chronically periapical radiopacity may appear well circumscribed or
infected cortex of bone. Once the disease is in its subacute or occasionally blend with the surrounding bone.
chronic stage, use of decortication promotes resolution based
on the premise that the affected bone is avascular and har- Management
bors microorganisms. This can be used as initial treatment
As condensing osteitis is an extension of pulpal infection
of primary and secondary chronic osteomyelitis, or when
beyond the periapex of the tooth, endodontic treatment or
initial regimens have failed.
extraction is the only treatment option.
Resection and reconstruction Resection of osteomyelitic Eliasson et al (1984) studied the effectiveness of end-
area with immediate or delayed reconstruction may be odontic treatment for periapical condensing osteitis on
436
49 roots of 36 teeth. These patients were followed up by Radiographic features

means of radiographs and patient files after endodontic
Radiographs reveal diffuse sclerotic patchy ‘cotton-wool’
treatment for a mean observation period of 4.3 years.
appearance of bone. Extensive involvement of the man-
Their study showed that there was total regression of
dible bilaterally and occasionally the maxilla and mandi-
periapical condensing osteitis on 36 of the 49 roots. No
ble may be affected. The borders of the patchy sclerosis are
condensing osteitis showed progress. They concluded that
diffuse and cannot be differentiated from the surrounding
there was rebuilding of bone structures to normal appear-
normal bone (Figure 37).
ance after endodontic treatment of periapical condensing
osteitis.
Following extraction of the affected tooth the residual Management
dense bone may be remodeled. However, on occasions, Owing to the extensive bone involvement surgical man-
this residual sclerotic bone may remain unaltered for agement is not indicated. In some patients the resolution
many years and referred to as a ‘bone scar’. of the causative periodontal disease results in improvement
of this condition. However, antibiotics have been employed
Chronic Diffuse Sclerosing Osteomyelitis to combat periods of acute exacerbations.
The diffuse form of sclerosing osteomyelitis is also a pro-

liferative response to a generalized form of mild infection. Periostitis Ossificans
However, a distinct difference between the diffuse form and (Chronic Osteomyelitis with Proliferative Periostitis)
the focal form is that the former occurs due to an exten-
sion of a diffuse periodontal disease. Wood et al (1988) in their two part series of articles titled
‘Periostitis ossificans versus Garrè’s osteomyelitis. Part I.
What did Garrè really say?’ and ‘Periostitis ossificans ver-
Clinical features
sus Garrè’s osteomyelitis. Part II. Radiologic analysis of
The diffuse sclerosing form may occur at any age. However, 93 cases in the jaws’, described their opinions and obser-
it is usually seen in edentulous areas of the mandible in elderly vations on the use of the terms Garrè’s osteomyelitis and
individuals. The disease progresses in an insidious manner periostitis ossificans.
and seldom presents clinical signs and symptoms.
The chronic phase of the condition may exhibit periods 1. The name ‘Garrè’ is correctly written as Garrè and not
of acute exacerbation that manifests as vague pain, bad Garré as mentioned in many textbooks and articles in
taste, minimal suppuration and fistulous tracts that may scientific literature.
open intraorally. 2. Garrè did not describe the typical radiologic, histo-
This may occur at any age, but is most common in older logic and bacteriologic features if acute osteomyelitis
persons, especially in edentulous areas. The disease is often since these investigative aids were not available to
of an insidious nature so usually presents no clinical features. him (X-rays were discovered by Röntgen only 2 years
In case of acute exacerbation there can be mild suppuration, after Garrè published his article in 1893.
many times with fistulous opening. In such cases, there can 3. Chronic sclerosing osteomyelitis was never really out-
be vague pain and bad taste in the mouth. lined by Garrè. He described recalcitrant osteomyeli-
Montonen et al (2006) conducted an immunohisto- tis, which was essentially untreatable at that time.
pathological study of diffuse sclerosing osteomyelitis in the 4. The term Garrè’s osteomyelitis should not be used.
clinically acute and subacute phases and compared it with
healthy bone. They found that the receptor activator of nuclear Gorman is given the credit for using the term periostitis ossi-
factor kappa B ligand (RANKL) was found in the lesions of ficans in 1951 to describe a productive inflammatory con-
diffuse sclerosing osteomyelitis. They also observed that dition of the mandibular periosteum. Lovemann (1941)
the periods of acute exacerbations were characterized by was probably the first to recognize the condition.
RANKL and induction of cathepsin K in mononuclear pre- Tong et al (2006) reported a case of osteomyelitis with
cursor cells, which subsequently seemed to differentiate proliferative periostitis. The authors believe that the term
into osteoclasts or foreign body giant cells. The proportion chronic osteomyelitis with proliferative periostitis is the
of bone to soft tissue increased with the duration of most accurate description of periostitis ossificans.
disease. They concluded that RANKL-driven osteoclasto- Nortjé et al (1988) conducted a radiological analysis
genesis and acidic cysteine endoproteinase cathepsin K of 93 cases of periostitis ossificans occurring in the jaws.
seemed to play important roles in diffuse sclerosing osteo- In their study, the age at initial consultation ranged from
myelitis as osteoclast-mediated bone resorption may rep- 2 to 69 years with a mean of 13.3 years. Males were more
resent the primary disease process later followed by new commonly affected than females (1.27:1). The causes
bone formation. for periostitis ossificans were due to periapical lesions
437
Figure 37
Orthopantomograph showing diffuse sclerosis of the mandible giving rise to cotton-wool appearance.
secondary to dental caries, untreated fractures and pri-

Figure 38
mary tuberculosis of the mandible. Two patients in their
study had lateral inflammatory odontogenic cysts.
The mandibular left first molar was most commonly
involved. The other sites involved were mandibular second
molar, mandibular premolar, mandibular primary second
molar, angle of the mandible, mandibular third molar and
maxillary premolar.
The periosteal reaction was visible at the buccal, lingual
and inferior aspects of the mandibular body. The authors
also reported of periosteal reaction occurring in the max-
illa and sigmoid notch. In their study, the number of the
periosteal laminations ranged from 1 to 12. The size of most
of the sequestra ranged from 1 to 2 mm. Larger sequestra
were seen in patients with infected fractures. The size of
the sequestra was age dependent with smaller sequestra
present in younger patients (Figure 38).
In about 63% of their patients, follicles of unerupted teeth
adjacent to the site of periostitis showed destruction. Other
changes that were noted included supraeruption of teeth,
bodily movement of unerupted tooth bud and widening of
the periodontal ligament space of the teeth inciting the
infection and occasionally of the adjacent vital teeth.
The periosteal reaction can either occur as long, narrow
periosteal reaction (common in adults) or fusiform, short and
wide periosteal reaction (seen in children).
When X-ray beams interact with successive layers of Illustration showing formation of periosteal new bone
periosteal new bone on the buccal cortical plate, they get
attenuated and the resultant image will show a patchy or
granular radiopacity in the body of the mandible on lat-
Radiographic investigations
eral projections (Figure 39A, B).
Periostitis occurring in the maxilla has been referred to Nortjé et al (1988) recommended the use of lateral oblique
as ‘halo-shadow’, which results from periosteal stripping and panoramic radiographs (lateral radiographs) to demon-
and subsequent bone formation in the floor of the maxil- strate the periosteal reaction. If the periosteal reaction is not
lary sinus related to a first molar tooth. evident on lateral radiographs, occlusal radiographs have to
438
Figure 39
Periosteal
new bone
Patchy
X-rays
radiopacity
Illustration showing the appearance of granular radiopacity in the body of the mandible on lateral projections
Figure 40 been noted. Although the incidence appears to be low

(0.2–1.5%) the occurrence of neoplastic disease consider-
ably complicates the resolution of inflammatory process.
There can be surgically induced discontinuity defects,
necessitating jaw reconstruction.
Anachorectic pulpitis
Bacterial infection of an intact pulp is referred to as
anachorectic pulpitis. It is suggested that the bacteria in
the circulation may tend to affect inflamed pulp. The
tooth in question is not necessarily carious.
Focal reversible pulpitis or pulp hyperemia

It is a reversible, transient pulpitis caused due to irrita-
tion of the pulpal terminal of the dentinal tubules upon
application of thermal stimulus (especially cold sti-
mulus). However, the pain subsides on removal of the
irritant.
Chronic hyperplastic pulpitis

Pulp polyp. Courtesy: Department of Oral Medicine and It is an excessive exuberant proliferation of a long-
Radiology. KLE Society’s Institute of Dental Sciences,
standing inflamed dental pulp. Usually affects teeth
Bangalore
with large carious lesions. Commonly seen in children
and young adults and often associated with the pri-
mary or permanent first molars.
be employed. The authors suggest that the posteroanterior Clinically it appears as red to pink mass of tissue pro-
view of the mandible is occasionally the only radiograph that truding from the pulp into the large open cavity of the
will adequately show the periosteal reaction. carious tooth (Figure 40).
Complications of osteomyelitis
There can be progressive, diffuse sclerosis of the medullary
With chronic osteomyelitis, neoplastic conversion of and cortical portions of the maxillofacial skeleton, especially
inflammatory metaplasia to squamous cell carcinoma has mandible, over time.
439
CHAPTER
Gingival and Periodontal
16 Diseases
Praveen BN, Jeeth Rai, Francisco Lopez
Sanchez
➧ Classification System in Periodontal Disease Gingival Lesions Associated with Mononucleosis

➧ Gingival Diseases Acute Inflammatory Gingival Enlargement
Plaque Microbiology Gingival Changes Associated with Tooth Eruption
Organisms in Various Periodontal Diseases ➧ Periodontal Diseases
➧ Host–Microbial Interaction Local Contributing Factors
Defense Mechanism Iatrogenic Factors
Plaque-induced Gingivitis ➧ Syndromes
Non-plaque-induced Gingivitis Hormonal Factors
Gingival Diseases Associated with Medications ➧ Sex Hormones
Gingival Diseases Associated with Systemic Diseases ➧ Stress and Psychosomatic Factors
Gingival Diseases Associated with Malnutrition
➧ Nutritional Factors
Gingival Diseases Associated with Heredity
Effects of Vitamin Deficiency
Gingival Diseases Associated with Ulcerative Lesions
➧ Radiographic Evaluation of Periodontal
Gingival Lesions Manifested in Childhood Diseases
Gingival Lesions Associated with Chicken Pox
Diseases
Periodontal diseases range from simple and early inflam- I. Gingival diseases
mation of marginal gingiva to advanced gingivitis and A. Dental plaque-induced gingival diseases: These diseases
subsequently periodontitis. A good classification system is may occur on a periodontium with no attachment loss
crucial in identifying and differentiating the various kinds or on one with attachment loss that is stable and not
of periodontal diseases. progressing.
Periodontal disease classification is useful to help 1. Gingivitis associated with dental plaque only
establish diagnosis, determine prognosis and facilitate a. Without local contributing factors
treatment planning. b. With local contributing factors
Different classification systems of periodontal diseases 2. Gingival diseases modified by systemic factors
have been used for years and also replaced by newer clas- a. Associated with endocrine system
sification systems as the knowledge of understanding i. Puberty-associated gingivitis
microbiology, etiology, pathogenesis and host response ii. Menstrual cycle-associated gingivitis
have improved vastly over the past few decades. iii. Pregnancy-associated gingivitis and pyogenic
Currently, the most accepted classification is the ‘1999 granuloma
World Workshop Classification System’. iv. Diabetes mellitus-associated gingivitis
b. Associated with blood dyscrasias
i. Leukemia-associated gingivitis
CLASSIFICATION SYSTEM IN PERIODONTAL ii. Others
DISEASE 3. Gingival diseases modified by medications
a. Drug-influenced gingival diseases
The classification is based on the 1999 International Work- b. Drug-influenced enlargements
shop for Periodontal Disease. c. Drug-influenced gingivitis
440
Chapter 16 – Gingival and Periodontal Diseases
d. Oral contraceptive-associated gingivitis II. Chronic periodontitis

e. Others A. Localized (less than 30% of sites involved)
4. Gingival diseases modified by malnutrition B. Generalized (more than 30% of sites involved)
a. Ascorbic acid deficiency gingivitis C. Slight (1–2 mm clinical attachment loss)
b. Others D. Moderate (3–4 mm clinical attachment loss)
B. Non-plaque-induced gingival lesions E. Severe (more than 5 mm clinical attachment loss)
1. Gingival diseases of specific bacterial origin
III. Aggressive periodontitis
a. Neisseria gonorrhoeae
A. Localized—slight, moderate or severe
b. Treponema pallidum
B. Generalized
c. Streptococcal species
d. Others IV. Periodontitis as a manifestation of systemic diseases
2. Gingival diseases of viral origin A. Associated with hematological disorders
a. Herpes virus infections 1. Acquired neutropenia
i. Primary herpetic gingivostomatitis 2. Leukemias
ii. Recurrent oral herpes 3. Others
iii. Varicella zoster B. Associated with genetic disorders
b. Others 1. Familial and cyclic neutropenia
3. Gingival diseases of fungal origin 2. Down’s syndrome
a. Candida species infections—generalized gingival 3. Leukocyte adhesion deficiency syndromes
candidiasis 4. Papillon–Lefevre syndrome
b. Linear gingival erythema (LGE) 5. Chediak–Higashi syndrome
c. Histoplasmosis 6. Histocytosis syndrome
d. Others 7. Glycogen storage disease
4. Gingival lesions of genetic origin 8. Infantile genetic agranulocytosis
a. Hereditary gingival fibromatosis 9. Cohen syndrome
b. Others 10. Ehlers–Danlos syndrome (types IV and VIII)
5. Gingival manifestations of systemic conditions 11. Hypophosphatasia
a. Mucocutaneous lesions 12. Others
i. Lichen planus V. Necrotizing periodontal diseases
• Pemphigoid A. Necrotizing ulcerative gingivitis (NUG)
• Pemphigus vulgaris B. Necrotizing ulcerative periodontitis (NUP)
• Erythema multiforme
• Lupus erythematosus VI. Abscess of the periodontium
• Drug-induced A. Gingival abscess
• Others B. Periodontal abscess
b. Allergic reactions C. Pericoronal abscess
i. Dental restorative materials VII. Periodontitis associated with endodontic lesions
• Mercury A. Combined periodontic–endodontic lesions
• Nickel
• Acrylic VIII. Developmental or acquired deformities and conditions
• Others A. Localized tooth-related factors that modify or
ii. Reactions attributed to predispose to plaque-induced gingival diseases/
• Toothpastes or dentifrices periodontitis
• Mouthrinses or mouthwashes i. Tooth anatomic factors
• Chewing gum additives ii. Dental restorations/appliances
• Foods and additives iii. Root fractures
iii. Others iv. Cervical root resorption and cemental rear
6. Traumatic lesions (factitious, iatrogenic or acci- B. Mucogingival deformities and conditions around
dental) teeth
a. Chemical injury i. Gingival/soft tissue recession, facial or lin-
b. Physical injury gual surfaces, interproximal (papillary)
c. Thermal injury ii. Lack of keratinized gingiva
7. Foreign body reactions iii. Decreased vestibular depth
8. Not otherwise specified iv. Aberrant frenum/muscle position
441
v. Gingival excess Necrotizing forms of periodontitis

• Pseudopocket
Necrotizing ulcerative gingivitis (NUG) and NUP are now
• Inconsistent gingival display
collectively referred to as ‘necrotizing periodontal disease’.
• Excessive gingival display
NUG and NUP are different stages of same infection and
• Gingival enlargement
should not be classified as separate disease entities.
vi. Abnormal color
C. Mucogingival deformities and conditions on eden-
tulous ridges
i. Vertical and/or horizontal ridge deficiency GINGIVAL DISEASES
ii. Lack of gingival/keratinized tissue
iii. Gingival/soft tissue enlargement Increasing evidence indicates that gingivitis is not a single
iv. Aberrant frenum/muscle position disease, but an assortment of diseases that are the end result
v. Decreased vestibular depth of a variety of different processes. Inflammation of gin-
vi. Abnormal color giva by bacteria is most common, but pathological changes
D. Occlusal trauma in the gingiva can also result from systemic conditions
i. Primary occlusal trauma. (e.g. puberty), drugs (e.g. amlodipine) and neoplasms (e.g.
leukemia). Hence, any disease that primarily affects gingival
The current classification has many changes seen which is tissues should be primarily classified as a gingival disease.
drastically different from the previous classifications. The The gingival diseases associated with children, adoles-
most noticeable change is that periodontitis is now classi- cents and young adults have several common characteristics.
fied based on the rate of progression of the disease and not Universal features include clinical signs of inflammation,
on the age of onset. signs and symptoms that are confined to the gingiva,
reversibility of the disease on removal of the etiology, and
Adult periodontitis–chronic periodontitis the presence of microbial plaque to initiate or exacerbate
the severity of the lesion.
The term ‘adult periodontitis’ is discarded since this
form of periodontitis is seen in wide range of ages and
found both in primary and permanent dentitions. Hence, Plaque Microbiology
the term ‘chronic periodontitis’ was chosen because it does
Oral cavity can be regarded as a single microbial ecosys-
not reflect the age of individual rather reflects the rate tem or macroenvironment because the colonization of oral
of progression of periodontal disease, which is slow in cavity starts at the time of birth. Within hours of birth the
nature. sterile oral cavity gets colonized by facultative and aero-
bic bacteria, which will be followed by anaerobic bacteria
Early onset forms of periodontitis–aggressive by 2nd day. By 2 years of age there will be around 400
periodontitis different kinds of bacteria. After tooth eruption, a more
complex oral flora is established. However, all these spe-
The term ‘early onset periodontitis’ has been discarded as
cies are seen to be living in harmony with the host. But,
this form of periodontitis is seen in various ages and in
however, there is an imbalance in this relationship between
older individuals too. Thus, the term ‘aggressive periodon-
the host and microorganisms, disease prevails which is
titis’ was chosen. Progressive periodontal disease can be
controlled by various other factors.
either localized or generalized. The term ‘localized aggres-
Oral environment is dominated with saliva, which is very
sive periodontitis’ replaces localized juvenile or localized
complex in composition. Although saliva is not a good
early onset periodontitis. The term ‘generalized aggressive
medium for supporting the growth of bacteria, it is likely that
periodontitis’ replaces generalized juvenile or generalized
organisms shed from intraoral reservoirs find transient
early onset periodontitis. The term ‘pre-pubertal periodon- residence in saliva. Oral cavity provides two types of surfaces
titis’ has been discarded and is currently described as for colonization—soft and hard tissue (teeth)—main difference
localized or generalized periodontitis or periodontitis as being soft tissue desquamation; hence the colonies are shed
manifestations of systemic disease. frequently whereas the hard surface provides a solid medium
for the bacteria to adhere and develop complex layers.
Systemic diseases and forms of periodontitis
Terminology
The present classification system highlights the role of cer-
tain systemic conditions like smoking and diabetes that Dental plaque: Dental plaque is clinically defined as a
can modify periodontitis and that certain systemic condi- structural resilient yellow-grayish substance that adheres
tions that can cause destruction of periodontium such as tenaciously to the intraoral hard surfaces, including
neutropenia and leukemias. removable and fixed restoration.
442
Biofilm: Biofilm is the term that describes the relatively e. C. rectus

indescribable microbial community associated with tooth f. Veillonella
surface or any other hard non-shedding material. 5. Generalized aggressive
Materia alba: Materia alba refers to soft accumulation of a. F. nucleatum
bacteria and tissues cells that lack the organized structure b. Lactobacillus
of dental plaque and is easily displaced with a water spray. c. Eubacterium
d. A. naeslundii
Formation of plaque e. A. actinomycetemcomitans
It is a complex procedure initially involving the formation B. forsythus
of pellicle (glycoprotein, phosphoproteins) around the tooth P. gingivalis
surface. Then the early bacterial colonization takes place Campylobacter
with facultative aerobic gram-positive organisms. Some of 6. NUG/NUP
the early colonizers are Actinomyces and Streptococcus a. P. intermedia
sanguis which adhere to the pellicle through adhesins. b. Fusobacterium
Later plaque maturation takes place with co-aggregation c. F. nucleatum
of secondary colonizers including Fusobacterium nuclea- d. P. gingivalis
tum, Prevotella intermedia, Porphyromonas gingivalis,
7. Periodontal abscess
Capnocytophaga, etc. Thus in maturation of plaque there
a. F. nucleatum
is a transition from the early aerobic gram-positive facul-
b. P. micros
tative species to an anaerobic gram-negative species.
c. P. intermedia
d. P. gingivalis
Organisms in Various Periodontal Diseases e. B. forsythus.
1. Periodontal health
a. Streptococcus HOST–MICROBIAL INTERACTION
b. Actinomyces
c. Veillonella
Health is not a static condition, it is a dynamic state in which
d. Fusobacterium
the living and functioning individuals remain in balance
2. Gingivitis with a constantly changing environment. These changes
a. Streptococcus in environment also cause changes in tissue activity so that
b. Actinomyces normal function can continue, a process known as homeo-
c. Peptostreptococcus stasis. If the environmental changes over-ride this homeo-
d. Eubacterium stasis, the normal function cannot continue and this change
e. Capnocytophaga is termed as disease.
f. Fusobacterium
g. Veillonella
Defense Mechanism
3. Chronic periodontitis
a. Streptococcus These protect the body from attack from microorganisms
b. Peptostreptococcus and can be classified as:
c. Eubacterium ❍ Non-specific mechanism
d. Actinomyces ❍ Specific mechanism.
e. Lactobacillus
f. P. gingivalis Non-specific mechanisms
g. Campylobacter rectus
h. F. nucleatum ❍ Bacterial balances
i. Selenomonas ❍ Surface integrity
j. Actinobacillus actinomycetemcomitans ❍ Surface fluid and enzymes
k. Eikenella corrodens ❍ Inflammatory reaction
❍ Neutrophil and macrophage activity.
4. Localized aggressive
a. A. actinomycetemcomitans
Specific mechanisms
b. Eubacterium
c. A. naeslundii ❍ T-cell response (cell-mediated immune system)
d. F. nucleatum ❍ B-cell response (antibody-mediated immune system).
443
Plaque bacteria produce a number of factors (virulence fac- Plaque-induced Gingivitis

tor), which causes disease directly, or individually by stim-
ulating the immune and inflammatory system. It is now It is the inflammation of gingiva which results from the bac-
known that individuals prone to periodontal disease have teria located at the gingival margin. The association of plaque
an aberrant immune inflammatory response to plaque which with gingival inflammation made it a frequently postulated
is genetically determined. cause of gingivitis. The initial histologic changes from health
to plaque-induced gingivitis may not be evident clinically
❍ Direct effects of bacteria (Page and Shroeder, 1976; Bimstein et al, 1985) but as
A. Evasion of host defense gingivitis progresses, clinical signs becomes more obvious.
i. Direct damage to polymorphonuclear leuko- Plaque-induced gingivitis begins at the gingival margin
cytes (PMNs) and macrophages and can spread into the deeper gingival component.
ii. Reduced PMN chemotaxis Clinical signs of gingival inflammation involve the change
iii. Degradation of immunoglobulins in color, contour, size, shape, consistency and surface tex-
iii. Modulation of cytokine function ture are associated with a stable periodontium which exhib-
iv. Degradation of fibrin its no loss of periodontal attachment or alveolar bone. The
v. Altered lymphocyte function classic clinical indicators of signs of inflammation are bleed-
B. Damage to crevicular epithelium ing on probing and color change from pink/coral pink to
i. Production of volatile sulfur compounds reddish pink or erythematous gingiva. In children, gingivi-
C. Degradation of periodontal tissues by bacterial tis is not intense as that found in young adults with similar
enzymes quantity of accumulation of dental plaque (Mattson and
i. Proteolytic enzymes Goldberg, 1985).
ii. Hydrolytic enzymes In the initial and established stages of gingivitis, dental
❍ Indirect effects of bacteria plaque is predominantly comprised of gram-positive aerobic
i. Inflammation microorganisms including Streptococcus mitis, S. sanguis,
ii. Production of reactive oxygen species Actinomyces viscosus, A. naeslundii and Eubacterium spp.
iii. Immunity As age advances, development and severity of gingivitis is
iv. Production of cytokines and prostaglandins mainly dependent on the quality of the plaque rather than
v. Production of matrix metalloproteinases (MMPs). the quantity and the other contributing factors like the host
Host modulation: Periodontal disease is multifactorial in immune response, environmental, genetic and the behav-
nature as a result of interaction between plaque microor- ioral factors. The common clinical signs and symptoms of
ganism and host responses. Host modulation refers to the gingivitis include redness, edema, bleeding on probing,
alteration/modification of host response to the microbial tenderness and enlargement (Loe et al, 1965; Suzuki, 1988)
stimulus by the use of number of medications. The two (Figure 1).
major categories are NSAIDs and inhibition of MMPs. Radiographically no changes will be seen as the inflam-
mation is confined only to the gingival sulcus. Histologic
NSAIDs
Prostaglandin E2 (PGE2), a product of cyclooxygenase path- Figure 1
way has been shown to increase the amount of alveolar bone
destruction by amplifying local inflammation. NSAIDs block
COX pathway thus decreasing PGE2 synthesis. A number of
NSAIDs have been used including ibuprofen, flurbiprofen,
indomethacin, ketoprofen and naproxen.
Matrix metalloproteinases
Matrix metalloproteinases are a family of proteolytic
enzymes secreted by a number of cells including leuko-
cytes, epithelial connective tissue cells whose primary func-
tion is degradation of extra-cellular matrix components.
Tetracycline and other drugs of the same group show to
inhibit the MMPs production from the host, primarily the
neutrophil MMPs. In doing so they increase host resistance
to connective tissue destruction forms of tetracycline are Edematous gingival margin associated with gingivitis.
submicrobial dose of tetracycline/doxycycline and chemi-
MCODS, Mangalore
cally modified tetracycline.
444
changes include proliferation of basal junctional epithe- changes in the gingiva have been observed (Muhlemann,
lium leading to apical and lateral cell migration, vascular 1948). However, the number of women who exhibit overt
dilatation and vasculitis of blood vessels adjacent to gingival changes fluctuating in conjunction with the men-
the junctional epithelium, progressive destruction of col- strual cycle is small (Marriotti, 1994). The most common
lagen fibers, cytopathologic alteration of resident fibro- gingival changes involve minor signs of inflammation dur-
blasts and progressive inflammatory cell infiltrate (Page ing ovulation. More specifically, gingival exudates have
and Shroeder, 1976). been shown to increase at least 20% during ovulation in
more than three-fourths of women tested (Hugoson, 1971).
Since these changes in crevicular fluid flow are not observ-
Non-plaque-induced Gingivitis able unless measured electronically, most young women
Gingival diseases associated with endogenous with gingival inflammation-induced by menstrual cycle
sex steroid hormones will present with a very mild form of the disease.
Since the 19th century, evidence suggests that the tissues of

Pregnancy-associated gingival disease
the periodontium are modulated by androgens, estrogens and
progesterone. Much of this evidence has come from observing Some of the most remarkable endocrine and oral alterations
the changes in gingival tissues during distinct endocrino- accompany pregnancy as a result of the rise in plasma
logic events (puberty, menstrual cycle, pregnancy, etc.). hormone levels over several months. During human gesta-
The principal explanation for sex steroid hormone- tion, pregnancy-associated gingivitis is characterized by
induced changes in the gingiva has pointed to changes of an increase in the prevalence and severity of gingivitis
microbiota in dental plaque, immune function, vascular during the second and third trimesters (Loe and Silness,
properties and cellular function in the gingiva. Sex steroid 1963; Hugoson, 1971). In spite of the same plaque scores,
hormones will affect the host by influencing cellular func- gingival inflammation is significantly higher during preg-
tion (in the blood vessels, the epithelium and the connective nancy than postpartum. In addition, gingival probing depths
tissue) and immune function, and together with hormone- are higher and bleeding on probing or toothbrushing is
selected bacterial populations occupying the gingival sulcus, increased (Miyazaki et al, 1991), and gingival crevicular
induce specific observable changes in gingival tissues fluid flow is elevated in pregnant women.
(Mariotti, 1994). Clinical features of pregnancy-associated gingivitis is
similar to that of plaque-induced gingivitis, except for the
Puberty-associated gingivitis propensity to develop frank signs of gingival inflammation
in the presence relatively little local irritation by plaque
Puberty is not a single episode but a complex process of during pregnancy.
endocrinological events that produce changes in the phys-
ical appearance and behavior of adolescents. Average age
of menarche is 12–13 years. The early onset of puberty, par- Pregnancy tumor
ticularly for adolescent girls, increases the time of exposure Pregnancy-associated pyogenic granuloma or ‘pregnancy
of periodontal tissues to steroid hormones and the possibility tumor’ was described over a century ago (Coles, 1874). The
of gingival disease. pregnancy-associated pyogenic granuloma ‘is not a tumor
The incidence and severity of gingivitis in adolescents but an exaggerated inflammatory response during pregnancy
are influenced by a variety of factors, including plaque lev- to an irritation resulting in a solitary polypoid capillary hem-
els, dental caries, mouth breathing, crowding of teeth and angioma which can easily bleed on mild provocation’ (Sillis
tooth eruption (Stamm, 1986). A number of studies have et al, 1996). These granulomas present clinically as a pain-
demonstrated an increase in gingival inflammation in cir- less, protuberant, mushroom-like exophytic mass attached
cumpubertal individuals of both sexes without a concomi- by a sessile or pedunculated base arising from the gingival
tant increase in plaque levels (Sutcliffe, 1972; Hefti et al, margin or more commonly from an interproximal papilla.
1981). Although puberty-associated gingivitis has many It is more common in the maxilla and may develop as early
of the clinical features of plaque-induced gingivitis, this as first trimester ultimately regressing or completely disap-
disease will develop frank signs of gingival inflammation pearing following parturition (Ziskin and Nesse, 1946).
in the presence of relatively small amounts of local irritants
(plaque) during the circumpubertal period.
Gingival Diseases Associated with Medications
Menstrual cycle-associated gingivitis
The use of chemical for the benefit of human beings has
Following menarche, there is a periodicity of sex steroid led to an astonishing array of drugs for the alleviation of
hormone secretion over a 25–30 days period—the menstrual human afflictions as well as the creation of new maladies
cycle—during which clinically significant inflammatory that affect the gingiva.
445
Drug-induced gingival enlargement Phenytoin

The disfiguring overgrowth of gingiva is a significant out- The first reported case of phenytoin-induced gingival
come principally associated with antiepileptic drugs such as enlargement was reported by Kimball (1939). Phenytoin is
phenytoin sodium, immunosuppressors such as cyclospo- indicated in treatment of epileptic seizures. Phenytoin
rine and calcium channel blockers such as nifedipine, amlo- induces gingival enlargements in approximately 50% of
dipine, verapamil, diltiazem and sodium valproate (Hassel patients (Angelopoulos and Goaz, 1972) (Figure 2A, B). One
and Hefti, 1991; Seymour et al, 1996). prominent theory of the etiology of phenytoin-associated
Most common clinical features seen in drug-induced gingival enlargements suggests that the growth of geneti-
gingival enlargement are as follows: cally distinct populations of gingival fibroblasts results
in the accumulation of connective tissues because of
❍ Individual variations in the pattern of gingival
reduced catabolism of the collagen molecule (Hassel and
enlargement, i.e. genetic predisposition (Hassel and
Hefti, 1991).
Hefti, 1991; Seymour et al, 1996).
❍ A tendency to occur more in the anterior gingiva.
Calcium channel blockers (Figure 3A, B)
❍ A higher prevalence rate in younger age groups.
❍ Onset within 3 months of usage of the medication, Calcium channel blockers are a class of drugs that exert
usually involves the interdental papilla. their effects principally at voltage-gated Ca channels
❍ Usually manifests as bead-like enlargement of the located in the plasma membrane, and are commonly pre-
papilla and marginal gingiva, and in severe cases scribed as antihypertensive, antiarrhythmic and antiangi-
gingival enlargement covers major portion of the nal agents. In 1984, calcium channel blockers were first
teeth and thereby interfere in occlusion, which is a linked to gingival enlargements (Ramon et al, 1984) and
rare entity. the prevalence of gingival lesions associated with these
❍ Although the condition can be found in a periodon- drugs has been estimated to be approximately 20%
tium with or without bone loss, there is no association (Barclay et al, 1992). The mechanism of gingival enlarge-
with attachment loss or tooth mortality. ment is still under investigation, but these drugs may
directly influence the gingival connective tissues by stim-
Furthermore, all of these drugs produce clinical lesions
ulating an increase of gingival fibroblasts as well as an
and histological characteristics that are indistinguishable
increase in the production of connective tissue matrix
from one another (Hassel and Hefti, 1991; Seymour et al,
(Fu et al, 1998).
1996). Finally, the influence of plaque on the induction of
gingival enlargement by drugs in humans has not been
Cyclosporine
fully elucidated (Hassel and Hefti, 1991); however, it does
appear that severity of the lesion is affected by the oral Cyclosporine is a powerful immunoregulating drug used
hygiene of the patient. primarily in the prevention of organ transplant rejection
Figure 2
A B
Enlargement of the labial and palatal aspects of the gingiva in patients using phenytoin sodium.
Gingival enlargement leads to the formation of pseudopockets. Courtesy: Dr Francisco, Mexico
446
Figure 3
A B
Enlargement of the labial and palatal aspects of the gingiva in a patient on nifedipine. Courtesy: Dr Francisco, Mexico
(Seymour and Jacobs, 1992). The clinical features of tissues by oral contraceptives were completed when dosage
cyclosporine-induced gingival enlargement were first levels were much higher than today. A recent clinical study
described in 1983 (Rateitschak-Pluss et al, 1983). The drug in young women found that oral contraception had no
appears to affect 25–30% of the patients taking this medi- effect on gingival tissues (Marrioti et al, 2000). It appears
cation (Hassel and Hefti, 1991; Seymour et al, 1987). that current oral contraceptives are probably not as harm-
Hypotheses explaining why cyclosporine affects the gin- ful to the periodontium as the early formulations.
giva are diverse; but a leading theory suggests that the
principal metabolite of cyclosporine, hydroxycyclosporine
(M-17), in conjunction with the parent compound stimu- Gingival Diseases Associated with Systemic
lates fibroblast proliferation (Mariotti et al, 1988). The Diseases
increase in cell number coupled with a reduction in the Leukemia-associated gingivitis
breakdown of gingival connective tissues (Hassel and Hefti,
1991) has been postulated to be the cause of excessive Leukemia is a progressive, malignant hematological disor-
extracellular matrix accumulation in cyclosporine-associ- der characterized by an abnormal proliferation and devel-
ated gingival enlargements (Figure 4A, B). opment of leukocytes and precursors of leukocytes in the
blood and bone marrow. Leukemia is classified according
to its duration (acute or chronic) and the type of cell
Oral contraceptive-associated gingivitis
involved (myeloid or lymphoid) and the number of cells in
Oral contraceptive agents are one of the most widely used the blood (leukemic or aleukemic). There are noticeable cor-
classes of drugs across the globe. The earlier onset of men- relations of leukemias with age. For example, acute lym-
arche, changing social mores and increased emphasis on phoblastic leukemia constitutes 80% of all childhood
family planning has increased the use of oral contracep- leukemias, whereas acute myelogenous leukemia usually
tives in younger age groups. Case reports have described affects the adults. Oral manifestations have primarily been
gingival enlargement induced by oral contraceptives in oth- described in acute leukemias; they consist of cervical ade-
erwise healthy women with no history of gingival over- nopathy, petechiae and mucosal ulcers as well as gingival
growth (Lynn, 1967; Kaufman, 1969; Sperber, 1969); in all inflammation and enlargement (Lynch and Ship, 1967).
cases, the increased gingival mass was reversed when oral Signs of inflammation in the gingiva include swollen,
contraceptive was discontinued or the dosage was reduced. glazed and spongy tissues which are red to deep purple in
Clinical studies have demonstrated that women using hor- appearance (Dreizen et al, 1984). Gingival bleeding is a
monal contraceptive drugs have a higher incidence of gin- common sign in patients with leukemia and is the initial
gival inflammation than women who do not use these oral sign or symptom in 17.7% and 4.4% of patients with
agents (Lindhe and Bjorn, 1967; El-Ahisry et al, 1970; acute and chronic leukemias (Lynch and Ship, 1967).
Pankhurst et al, 1981) and that long-term use of oral contra- Gingival enlargement has also been reported initially
ceptives may affect periodontal attachment levels (Knight beginning at the interdental papilla and followed by the
and Wade, 1974). All studies recording changes to gingival marginal and attached gingiva. Although local irritants
447
Figure 4
A B
Cyclosporine-induced gingival enlargement. Courtesy: Dr Francisco, Mexico
can predispose to and exacerbate the gingival response in (Lamster et al, 1998). In addition, LGE lesions have been
leukemia, they are not prerequisites for lesions to form in shown to have reduced proportions of T cells and macro-
the oral cavity (Dreizen et al, 1984). phages and an increased number of immunoglobulin G
plasma cells and polymorphonuclear leukocytes (Gomez
Linear gingival erythema (LGE) et al, 1995). These host cell responses and unusual microbiota
Infection with the human immunodeficiency virus (HIV) may be responsible for the refractory nature of this lesion
produces an irreversible and progressive immunosuppres- to the conventional periodontal treatment of gingivitis.
sion that renders the person infected susceptible to a variety
of oral diseases. In humans, HIV depletes CD4 lymphocytes Gingival Diseases Associated with Malnutrition
(T helper cells), which leads to the development of a variety
of fungal, viral and bacterial oral infections (Connor and Although some nutritional deficiencies can significantly
Ho, 1992). exacerbate the response of the gingiva to plaque bacteria,
Oral manifestations of HIV infection have been used to the precise role of nutrition in the initiation or progression
stage HIV disease (Justice et al, 1989; Royce et al, 1991) to of periodontal diseases remains to be elucidated. Studies
identify prophylactic treatment of other serious infections of the periodontal status of individuals in developed and
(Force, 1993), and to indicate disease prognosis (Dodd et al, developing countries have failed to show any relationship
1991; Katz et al, 1992). between periodontal disease and general nutrition (Russell,
In the gingiva, manifestations of HIV infection were for- 1962; Waerhaug, 1967; Wertheimer et al, 1967).
merly known as HIV-associated gingivitis but are now known Severe vitamin C deficiency or scurvy was the earliest
as LGE. This condition is distinguished by a 2–3 mm mar- nutritional deficiency to be examined in the oral cavity (Lind,
ginal band of intense erythema in the free gingiva (Winkler 1953). Even though scurvy is unusual in areas with an ade-
et al, 1988), which may extend into the attached gingiva as quate food supply, certain populations on restricted diets
a focal diffuse erythema and/or extend beyond the muco- (e.g. infants in families of low socioeconomic class) are at
gingival line into the alveolar mucosa (Winkler et al, 1988). risk of developing this condition (Oeffinger, 1993). In scurvy
The characteristics of LGE may be localized to one or two the gingiva is typically red, swollen, ulcerated and suscep-
teeth but more commonly involvement of marginal gingiva tible to hemorrhage (Van Steenberghe, 1997). Although
is generalized. there is no dispute about the necessity of dietary ascorbic
The etiology of this gingival lesion is not well understood; acid for periodontal health, in the absence of frank scurvy,
however, research has begun to investigate the relation- the effect of declining ascorbic acid levels on the gingiva
ship of periodontal pathogens and the local host response in can be difficult to detect clinically (Woolfe et al, 1980) and
regard to how HIV infection affects the gingiva. The anaer- when it is detected usually has characteristics that are
obic microflora from the subgingival sites of HIV-infected similar to plaque-induced gingivitis.
patients with gingivitis seems to be essentially the same as
in non-infected patients (Moore et al, 1993). Despite the
Gingival Diseases Associated with Heredity
similarities in anaerobic microflora, organisms not gener-
ally associated with gingivitis in HIV-negative patients, Benign, non-inflammatory fibrotic enlargement of the max-
such as Candida species, have been identified in LGE illary and/or mandibular gingiva associated with a familial
448
aggregation has been designated by terms such as gingi- but when attachment loss occurs this condition should be
vostomatitis elephantiasis, familial elephantiasis, juvenile considered as an NUP.
hyaline fibromatosis, idiopathic gingival fibromatosis and The cause of NUG may be bacterial. The four zones of the
hereditary gingival fibromatosis. Although there were almost NUG lesion include the bacterial zone (the superficial area
100 published reports of hereditary-associated gingival consisting of various bacteria and some spirochetes); the
overgrowths in the 20th century, information about the neutrophil rich zone (follows the bacterial zone and con-
natural history of this rare disease is extremely limited and sists leukocytes and bacteria including spirochetes); the
its etiology is unknown. necrotic zone (consisting of disintegrated cells and
Hereditary gingival fibromatosis appears to be a slowly connective tissue elements with many large and interme-
progressive gingival enlargement which develops upon diate spirochetes); and the spirochetal infiltration zone
eruption of the permanent dentition. However, gingival (the deepest zone that is infiltrated with no other bacteria
enlargement can also occur in the primary dentition but with intermediate and large spirobacteria but with
(Emerson, 1965; Jorgenson and Cocker, 1974; Lai et al, intermediate and large spirochetes) (Listgarten, 1965).
1995; Miyake et al, 1995). The disease can be localized or The cultivable flora of NUG that predominates includes
generalized and may ultimately cover the occlusal surfaces Prevotella intermedia and Fusobacterium species, while
of teeth. The enlarged gingiva is non-hemorrhagic and microscopically, Treponema and Selenomonas species are
firm, but there can be an overlay of gingival inflammation observed (Loesche et al, 1982; Rowland et al, 1993).
which can augment the enlargement. The histologic features Additional factors include smoking (AAP, 1996), psycho-
of hereditary gingival fibromatosis include dense fibrotic logical stress (Moulton et al, 1952; Cohen-Cole et al, 1983),
connective tissue as well as epithelial hyperplasia with malnutrition (Grupe and Wilder, 1956; Goldhaber and
elongated and increased rete pegs (Johnson et al, 1986; Giddon, 1964; Johnson and Engel, 1986) and immune
Clark, 1987). suppression (Moulton et al, 1952; Rowland et al, 1993) can
Hereditary gingival fibromatosis can be inherited as a predispose and individual to NUG.
simple mendelian trait, in some chromosomal disorders and Although NUG can affect any age group, it is consid-
as a malformation syndrome (Witkop, 1971; Jones et al, ered to be a disease of young adults in developed countries
1977; Takagi et al, 1991; Goldblatt and Singer, 1992; Hallet (Melnick et al, 1988). In developing countries, NUG is a dis-
et al, 1995). Although the specific genes for this disease have ease found in children from families with low socioeconomic
not been identified, genetic analysis supports the presence status (Melnick et al, 1988). The onset of NUG in children
of two different gene loci on chromosome 2p (Shashi et al, is associated with inappropriate nutrition intake, especially
1999). Research into the cellular responses of this disease low protein consumption (Sheiham, 1966; Taiwo, 1995). In
suggests an accumulation of specific populations of gingi- addition, viral infections such as measles can induce NUG
val fibroblasts resulting in an abnormal accumulation of in malnourished children (Enwonwu, 1972; Osuji, 1990).
connective tissues (Huang et al, 1997; Tipton et al, 1997). Even though NUG has occurred in epidemic patterns, this
disease is not considered communicable (Rosebury, 1942).
Gingival Diseases Associated with Ulcerative
Lesions Gingival Lesions Manifested in Childhood
Necrotizing ulcerative gingivitis has been known for cen- Diseases
turies by numerous names including ‘trench mouth’ and
Acute herpetic gingivostomatitis
Vincent’s infection. Acute NUG is a term used to describe
the clinical onset of the disease and should not be used as The herpes simplex virus produces some of the most com-
a diagnostic classification, since some forms of NUG may mon acute infections in humans. Of the two herpes simplex
be recurrent or possibly chronic. virus serotypes, type 1 is responsible for most oropharyn-
Onset is usually sudden with intense gingival pain, which geal infections, including acute herpetic gingivostomati-
prompts the patient to seek professional care. Clinical signs tis. This disease is observed in young adolescents and
include papillary necrosis, giving a ‘punched-out’ appear- adults but has its highest incidence in infants and children
ance of the gingival papilla and gingival bleeding that younger than 6 years of age (Scott et al, 1941). There are
requires little or no provocation (Grupe, Johnson and no predilection for either sex with the primary infection.
Engel, 1986). Although these symptoms and signs must be Following the primary infection, the virus moves through
present for a diagnosis of NUG, other features may occur nerves to neuronal ganglia where it remains dormant until
such as fever, malaise, lymphadenopathy, metallic taste reactivated by various stimuli including trauma, exposure
and fetor ex ore (malodor) (Schluger, 1943; Wilson, 1952; to sunlight or ultraviolet lamps, fever, stress, fatigue, men-
Murayama et al, 1994). Systemic reactions of acute NUG struation, pregnancy, upper respiratory tract infection,
are usually more severe in children. Significant destruc- allergy or gastrointestinal disturbances (Stevens, 1975;
tion of the gingival connective tissue is possible with NUG Shafer et al, 1974).
449
Although most cases of primary herpetic infection are may be expressed. The lesion is generally self-limiting, ulti-
symptomatic (Gibson et al, 1990; McDonald et al, 1994), mately rupturing if permitted to progress. The gingival abscess
the primary infection in some cases may manifest as acute should not be confused with the periodontal abscess, which
herpetic gingivostomatitis, which is characterized by sev- affects the supporting periodontal structures.
eral oral and systemic manifestations. The symptomatic
infection is characterized by fever, malaise, headache, irri-
tability, dysphagia and lymphadenopathy. In the oral cav- Gingival Changes Associated with Tooth Eruption
ity, lesions can affect the lips, tongue and pharynx. As the crown penetrates the oral mucosa, the marginal gin-
Initially, gingival inflammation is characterized by a dif- giva and sulcus form. During the physiologic process of
fuse, erythematous, shiny appearance precedes the appear- eruption, the gingival margin becomes edematous and
ance of vesicles (White, 1998). The vesicles vary in size and erythematous. It is not uncommon for erupting primary or
are usually discrete, spherical sacs which rupture to form permanent teeth to be associated with a form of dentiger-
small, ragged and painful ulcers that are covered by a gray ous cyst (also called eruption cyst/eruption hematoma). The
membrane and surrounded by an erythematous, elevated eruption cyst usually appears as a site of translucent, fluc-
halo (White, 1998). The ulcers persist for 7–10 days and heal tuant, circumscribed swelling over the erupting tooth. When
spontaneously, leaving no scars (White, 1998). the cystic cavity contains blood, the swelling appears as a
The diagnosis for this infection is usually determined by purple or deep blue fluctuant, circumscribed swelling termed
the patient’s history and clinical signs and symptoms, and as eruption hematoma. Primary canines and molars appear
confirmed laboratory culture of the herpes simplex virus. to be more frequently involved than primary incisor teeth.
Lesions of recurrent aphthous stomatitis have often been
confused with acute herpetic gingivostomatitis, but can be
distinguished clinically by the absence of diffuse erythema
of gingiva, acute toxic systemic symptoms and herpes PERIODONTAL DISEASES
simplex virus culture.
Local Contributing Factors
Gingival Lesions Associated with Chicken Pox It is well known that the primary cause of gingival inflam-
mation and periodontal destruction is bacterial plaque.
Varicella, which primarily affects individuals below the age of
These factors that tend to accelerate the disease locally, are
15 years (Preblud, 1986), produces the skin lesions character-
termed as local contributing factors in the progression of
ized by vesicles and pustules that break and crust over. In the
periodontal disease.
oral cavity, small ulcers may develop in any area of the mouth;
The local factors are:
however, lesions are found most often on the palate, gin-
giva and buccal mucosa (Badger, 1980). The ulcers that appear ❍ Calculus
during the course of the skin rash are usually not painful. ❍ Iatrogenic factors
– Overhanging restoration
– Margins of restoration
Gingival Lesions Associated with Mononucleosis
– Contours
Mononucleosis is produced by the Epstein–Barr virus and – Restorative materials
is primarily a disease of children and young adults. The clin- – Occlusion
ical symptoms are most prominent in young adults, and – Restorative procedures
common signs and symptoms include fatigue, malaise, – Pontic
headache, fever, sore throat, enlarged tonsils and lymph- – Improper removable partial dentures
adenopathy. Alterations in the oral cavity include gingival ❍ Malocclusion
bleeding, petechiae of the soft palate, ulceration of the ❍ Food impaction
gingiva and buccal mucosa and pericoronitis. ❍ Orthodontic therapy
❍ Anatomic contributing factors
– Proximal contact relationship
Acute Inflammatory Gingival Enlargement
– Cervical enamel projections and enamel pearls
A gingival abscess is an acute, painful, rapidly expanding – Bifurcation ridges
lesion localized to the gingiva. Most gingival abscesses are – Developmental grooves
detected on the marginal gingiva or the papilla. Gingival – Root anatomy—morphology and length
abscesses usually arise from an insult such as trauma caused – Root fusion, cemental tears
by food which forces bacteria into the tissue. Within hours, – Proximity to adjacent teeth
a bright red gingival swelling will convert to a lesion that ❍ Endodontic lesions
is pointed and fluctuant mass from which purulent exudates ❍ Caries
450
❍ Habits
Figure 5
– Toothbrushing trauma
– Mouth breathing, tongue thrusting and other habits
– Factitial injuries
❍ Smoking
❍ Trauma from occlusion
❍ Trauma
– Physical
– Chemical
– Thermal
❍ Radiation therapy
❍ Mucogingival problems
❍ Cysts and tumors.
Calculus
Calculus is mineralized dental plaque that forms on surface
of teeth and dental prosthesis. It is one of the most important
and commonly occurring local contributing factor as it is Thick band of subgingival calculus firmly adherent to the
cervical margin of the tooth. Courtesy: Department of
invariably covered with bacterial plaque on its surface.
Types
❍ Margins of restoration: A subgingival margin is asso-
Supragingival calculus It is white or whitish yellow in
ciated with more plaque accumulation and more peri-
color hard with clay-like consistency and is easily detached
odontal destruction than supragingival margins, margins
from the tooth. It is most commonly found on lingual surface
placed at the level of gingiva.
of mandibular teeth and buccal surface of maxillary teeth.
❍ Contours: Overcontoured restorations tend to accumu-
Subgingival calculus It is located below the crest of late more plaque, and the natural self-cleansing mech-
marginal gingiva and appears dark brown or black in anism fails.
color and flint-like consistency. It is firmly attached to the ❍ Open contours: These are associated with increased
tooth (Figure 5). food impaction and papillary inflammation.
❍ Restorative materials: Silicate cements and self-curing
Composition acrylic resins accumulate more plaque.
❍ Restorative procedures: Rubber dam, clamps, matrix
Calculus consists of organic and inorganic constituents.
organic contents are mainly desquamated epithelial cells, band wedges, gingival retraction chords are shown to
leukocytes and microorganisms. Inorganic constituents are cause injury to the periodontium.
❍ Pontics: When pontics are in contact with gingival tis-
mainly calcium, calcium phosphates and calcium carbonates,
magnesium phosphates and other metals. sue, they tend to accumulate more and oral hygiene
techniques are more difficult.
Significance of calculus ❍ Improper removable partial denture: It causes an
increase in mobility of abutment teeth and also favors
Calculus is a local factor in periodontal disease because it accumulation of plaque leading to gingival inflamma-
always has a layer of plaque on its surface. Because calculus tion and periodontal pocket.
is firmly attached to the tooth through an organic pellicle, ❍ Malocclusion: Plaque control is more difficult in individ-
it resists removal through routine oral hygiene techniques. uals with malocclusion. Malocclusion is usually associated
Calculus plays an important role in the progression of with recession if the tooth is buccally placed and plaque
periodontal disease by keeping plaque in close contact control is difficult because of lack of attached gingiva.
with the periodontal tissues in gingiva. ❍ Food impaction: It is forceful wedging of food into the
periodontium by occlusal forces. This harbors more
microorganisms in interproximal area leading to perio-
Iatrogenic Factors
dontal destruction.
❍ Overhanging restorations: These contribute to peri- ❍ Orthodontic therapy: Orthodontic appliances are asso-
odontal disease by acting as a local plaque retentive ciated with food debris and plaque accumulation and
area causing accumulation of plaque and changing changing the ecosystem. These also cause increase in
the ecology that favors the growth of microorganisms. forces on the periodontium.
451
❍ Carious tooth: Endodontically treated tooth and root

Figure 6
stumps act as plaque retentive areas leading to peri-
odontal diseases. In some instances patients with
pulpal/periapical diseases avoid chewing food from the
affected side leading to more deposition of calculus.
❍ Anatomic contributing factors: Proximal contact
relationship—open proximal contact may cause food
impaction, which in turn causes periodontal disease.
❍ Cervical enamel projection and enamel pearls: Cervical
enamel projections are narrow wedge-shaped extrusions
of enamel extending from cementoenamel junction
toward the furcation area. Enamel pearls are bead-like
projections commonly found in furcation area. The
clinical significance of cervical enamel projection and
enamel pearls is that they act as a local plaque reten-
tive area and also periodontal attachment does not take Cervical abrasion and localized gingival recession.
place in them, hence leading to periodontal diseases. Courtesy: Department of Oral Medicine and Radiology,
❍ Bifurcation ridges: It is a convex excrescence of cemen- MCODS, Mangalore
tum that runs longitudinally between the mesial and dis-
tal roots of mandibular molars. Thus, it makes removal
of plaque and calculus more difficult. Habits
❍ Palatogingival groove: It is a developmental groove ❍ Toothbrush trauma: This can abrade the epithelium
that begins at the cingulum and extends apically in leading to gingival recession (Figure 6). It also causes
the anterior teeth. Most commonly it is seen in maxil- abrasion of the tooth surface.
lary lateral incisor. These grooves act as a plaque ❍ Mouth breathing, tongue thrusting and other habits:
retentive area. Mouth breathing causes dehydration of gingival tissue
❍ Cemental tears: It is a piece of detached cementum with increasing their susceptibility to inflammation. Tongue
some amount of dentin attached to the alveolar bone thrusting causes anterior open bite and spacing between
through periodontal ligament. This is often induced by the teeth causing difficulty in maintaining good oral
some form of acute trauma leads to rapid bone destruc- hygiene.
tion with vertical bone loss. ❍ Factitial injury: It is self-inflicted injury, which include
❍ Root fusion: Progression of periodontal disease is faster picking of gingiva with finger nail; tooth picks, pins
if roots are fused. and other materials. This causes a direct damage to the
❍ Root proximity: There is a thin interseptal bone when
periodontium.
the roots are in close proximity, which has an increased
❍ Radiation therapy: It causes soft tissue ischemia, fibro-
rest for periodontal destruction.
sis and osteoradionecrosis in bone. Periodontal tissue
❍ Extraction of impacted third molars: This results in
destruction is more in patients who underwent radia-
periodontal problems distal to second molar with pock-
tion therapy.
ets of recession and bone loss.
❍ Mucogingival problems: Mucogingival area is defined as
Smoking a generic term used to describe the mucogingival junc-
tion and its relationship to the gingiva, alveolar mucosa,
It is one of the etiological factors in ANUG. It is classified frenum, muscle attachments, vestibular fornices and the
as an environmental factor with local and systemic effects. floor of the mouth. A mucogingival deformity may be
The local effects are peripheral vasoconstriction and local- defined as a significant departure from the normal shape
ized ischemia. The individual harbors more pathogenic and of the gingival and alveolar mucosa.
virulent subgingival microorganisms. The systemic effects
are decreased immunity with non-specific (decreased poly- Some of the common mucogingival problems are decrease in
morphoneutrophils, chemotaxis, phagocytosis) and specific the width of attached gingiva, gingival recession crossing
(decreased IgG, IgA). There is also activation of proinflam- the mucogingival junction, high frenal attachments and shal-
matory cytokines including IL-1. TNF- IL-6 all lead to low vestibule.
periodontal diseases. Mucogingival problems are commonly associated with
Since immunity and inflammation are reduced in smok- plaque accumulation, as the patient cannot perform the
ers there are lesser clinical signs of gingival inflammation routine oral hygiene techniques.
with increased local deposits and periodontal destruction; ❍ Traumatic injuries: These can be physical, chemical and
it is one of the etiological factor in NUG. thermal in nature and can cause injury to gingival and
452
supporting periodontal tissues. These directly affect

Figure 7
the gingiva and make routine plaque control proce-
dures difficult.
❍ Cysts and tumors: Some of the cysts and tumors because
of their expansile growth cause destruction of the
periodontium.
Systemic factors
It is well known that bacterial plaque is a main etiological
factor responsible for gingival inflammation and periodon-
tal destruction. The bacterial plaque causes a marked host
response that varies from one individual to other, hence sus-
ceptibility of individual to periodontitis depends on various
factors, including systemic and genetic factors.
Extensive destruction of supporting bone and missing teeth in
Genetic factors Papillon–Lefevre syndrome. Courtesy: Department of Oral
Periodontal disease is multifactorial with plaque being
major factor, but some of these factors fail to explain the
variation of disease in different individuals with same Chediak–Higashi syndrome
amount of local factor. This is attributed to genetic suscep-
tibility. Genetic pleomorphism in IL-1 has been shown to It is an autosomal recessive disease that affects the produc-
be associated with chronic periodontitis. Studies have tion of organelles in many cells including melanocytes,
shown a link between susceptibility of aggressive peri- platelets and leukocytes. Neutrophils are characterized by
odontitis and the human leukocyte antigen of chromo- abnormal giant lysosomes containing enzymes and with
some 6, which is responsible for production of IgG2, which impaired ability to release them.
in turn is responsible for periodontal destruction. The It is associated with severe gingivitis, periodontal disease
genetic polymorphism in the genes for the Fc- receptor and loss of dentition at an early age.
on the phagocytic cell is identified in localized aggres-
sive periodontitis. Polymorphism in the gene promoter Lazy leukocyte syndrome
region of chromosome 6, results in increased production It is a rare disease characterized by defect in PMN chemo-
of TNF-, which has been shown to be associated with taxis and an abnormal inflammatory response.
periodontitis.
Leukocyte adhesion deficiency (LAD)
It is a rare inherited genetic disorder resulting from inabil-
SYNDROMES ity to produce or express CD18, an integrin useful in leu-
kocyte adhesion.
Down’s syndrome Both primary and secondary dentitions are affected in
LAD which presents as acute inflammation and rapid
It is a congenital disease characterized by mental defi-
destruction of bone.
ciency, growth retardation and severe periodontal disease.
Incidence is about 1:800–1,000. Both deciduous and per-
Ehlers–Danlos syndrome
manent dentitions are affected. The destruction increases
with age. The rapid periodontal destruction is commonly It is an inherited condition affecting connective tissue with
attributed to immunologic defects. reduction of collagen fiber production. The oral mucosa,
gingiva and periodontium are affected.
Papillon–Lefevre syndrome
Hypophosphatasia
It is an inherited autosomal recessive disease character-
ized by diffuse palmoplantar hyperkeratosis, severe destruc- It is an autosomal recessive condition where there is a defi-
tion of periodontium and calcification of dura. Incidence ciency of the enzyme alkaline phosphatase and character-
is 1–4:10,00,000. Deciduous teeth are usually lost by ized by abnormal mineralization of bone and dental tissues.
5–6 years and permanent teeth a few years later (Figure 7). There is premature exfoliation of deciduous teeth, loss
Defects in PMNs adherence and chemotaxis has been of alveolar bone, absence of gingival inflammation and
reported. absence of cementum. The permanent teeth are not affected.
453
Mucopolysaccharidoses (MPS) Figure 8

It is a group of inherited disorders characterized by dis-
turbances in mucopolysaccharide metabolism and their
increased storage in various tissues.
These include Hurler’s syndrome (MPS I) and Hunter’s
syndrome (MPS II).
The teeth in both these conditions are small and widely
spaced and exhibit delayed eruption. Gingival enlarge-
ment may be commonly seen.
Hormonal Factors
Diabetes: It is a complex metabolic disorder characterized
by glucose intolerance.
It is associated with: Extensive periodontal abscess in a diabetic patient.
❍ Microvascular diseases (retinopathy, neuropathy and
nephropathy)
❍ Macrovascular diseases (cardiovascular, cerebrovascular)
❍ Increased susceptibility to infections
❍ Delayed wound healing
❍ Periodontitis. Figure 9
Oral effects
Patients with diabetes have diminished salivary flow,
burning mouth and candidiasis.
Diabetes also has profound effect on periodontium result-
ing in gingivitis, gingival enlargement, periodontal abscess
formation, periodontitis and loss of teeth (Figures 8 and 9).
Pathogenesis
❍ Alteration of bacterial flora
❍ Altered neutrophil function
❍ Altered collagen metabolism.
SEX HORMONES
The female sex hormones affect periodontal tissues. Estrogen Extensive destruction of interdental bone and loss of teeth
promotes keratinization and increased mucopolysaccha- in a diabetic patient. Courtesy: Department of Oral Medicine
ride content, the gingival connective tissue. Progesterone and Radiology, MCODS, Mangalore
increases the permeability and gingival blood vessels.
Changes seen are:
❍ Puberty: There is an increased prevalence and severity of Prevotella intermedia during pregnancy. In some
of gingivitis. cases a soft, pedunculated granuloma is present in the
❍ Menstrual cycle: Gingival crevicular fluid (GCF) flow interdental papilla, which is a slow-growing lesion
increases at the time of ovulation. referred to as pregnancy tumor.
❍ Pregnancy: The changes in gingiva usually starts ❍ Oral contraceptives: These are associated with increased
around the 3rd month of gestation and the severity of GCF flow and a greater prevalence of gingival inflam-
inflammation gradually increases during pregnancy. mation.
Gingiva becomes bright red, swollen, sensitive and ❍ Menopausal gingivostomatitis: In this the gingiva
bleeds spontaneously. There is an increase in GCF pro- appears dry and shiny. It varies in color from pale to
duction and tooth mobility. There is an increased level bright red and bleeds easily.
454
❍ Hyperparathyroidism: It causes a disease known as Scurvy is associated with enlarged, hemorrhagic, bluish,
osteitis fibrosa cystica or von Recklinghausen’s bone red gingiva. Gingivitis is not caused by vitamin C deficiency
disease. but the severity of gingivitis increases with vitamin C defi-
ciency. Gingival healing is also impaired.
Oral changes include increased tooth mobility. Radio-
Vitamin C deficiency also results in osteoporosis of alve-
graphically, it shows widening of periodontal ligament
olar bone, hemorrhage in the periodontal ligament and
space with absence of lamina dura, closely meshed tra-
increased tooth mobility.
baculae and cystic space known as Brown’s tumor.
Vitamin D It is necessary for the calcium–phosphorus bal-
ance. Deficiency of vitamin D causes rickets in children and
STRESS AND PSYCHOSOMATIC FACTORS osteomalacia in adults. Vitamin D deficiency in animals
causes osteoporosis of alveolar bone, uncalcified osteoid,
Stress and other psychosomatic factors have direct anti- reduction in width of periodontal ligament, severe osteo-
inflammatory and anti-immune effects or can have behavior- clastic resorption of alveolar bone.
mediated effects on the body’s defenses. Stress is also one
Vitamin E There is no relationship between vitamin E
of the etiological factor in acute NUG.
deficiency and periodontal disease in humans. In animals
Stress increases cortisol hormone which suppresses
vitamin E accelerates gingival wound healing.
immune system by suppressing neutrophil activity and
decreasing IgG and IgA production, thus allowing the Protein deficiency In experimental animals the oral
periodontal pathogens to cause more destruction. changes of protein deficiency include degeneration of CT
Psychosomatic disorders can result in periodontal tissue of periodontium, osteoporosis, delayed wound healing and
destruction through development of certain habits like nail abnormal deposition of cementum.
biting, using foreign objects, self-inflicted gingival inju-
ries can be seen in patients with mental disabilities. Hematological disorders
Leukemia: Its characterized by diffuse replacement of bone
marrow with proliferating leukemic cells, abnormal num-
ber or form of WBCs in blood and widespread metastasis.
NUTRITIONAL FACTORS
It can be lymphocyte or myelocytic, acute, subacute or
chronic.
There are no nutritional factors that causes gingivitis or
periodontitis, but they produce changes in the oral cavity Oral manifestations There is a widespread infiltrate of
including the gingiva and alveolar bone that can acceler- leukemic cells in oral cavity, particularly gingiva, which
ate the process of gingivitis and periodontitis. appears bluish red with enlargement and spontaneous bleed-
ing. Oral ulceration and infections are commonly noticed
in these patients; microscopically the gingiva exhibits a
Effects of Vitamin Deficiency dense infiltrate of immature leukocytes.
Vitamin A In experimental animals, vitamin A cause Leukocyte disorders These are usually associated with
hyperkeratosis and hyperplasia of gingiva with a increased severe periodontal destruction as PMNs are the first line of
rate of pocket formation. In humans studies are inconclusive. defense against periodontal pathogens.
Vitamin B complex Thiamin deficiency (beriberi) causes
Neutropenia It is a leukocyte disorder in which there is
hypersensitivity of oral mucosa, minute vesicles and ero-
a low count of neutrophils in blood (1,500 cells/l).
sion of oral mucosa.
Neutropenia can be genetic, familial, idiopathic or secondary
Riboflavin deficiency (riboflavinosis) causes glossitis,
to some kind of infections. There are many kinds of neu-
angular cheilitis and seborrheic dermatitis.
tropenias all of which affect the periodontal health.
Niacin deficiency (pellagra) causes glossitis, stomatitis,
black tongue and severe gingival inflammation with Agranulocytosis It is a leukocyte disorder in which there
necrosis. is a low count of circulating granulocyte in blood.
Folic acid deficiency causes necrosis of gingival, peri- Ulcerations with the absence of inflammatory reaction of
odontal ligament and alveolar bone without inflammation. the oral cavity is one of the characteristic sign of agranu-
locytosis.
Vitamin C Ascorbic acid deficiency results in scurvy,
Gingival hemorrhage, necrosis and fetid odor are also
where there is defective formation of collagen fibers and
present.
impaired osteoblastic activity. There is also an increased
capillary permeability, susceptibility to traumatic hemor- Cyclic neutropenia It is an inherited condition where
rhages, hyperreactivity of contractile elements and slug- there is cyclic depression of neutrophils in peripheral blood
gish blood flow. at intervals varying from 15 to 55 days and manifested
455
with pyrexia, oral ulceration and skin infections. Oral fea-

Summary of the clinical features of gingivitis and periodontitis
tures include oral ulcerations, severe gingivitis, periodon-
tal destruction and alveolar bone loss. Gingivitis
Presence of local factors like calculus and/or at least one of the
Chronic benign neutropenia of childhood There is mod- following clinical features:
erate neutropenia with absolute lymphocytosis and mono- • Erythematous gingiva
cytosis. Oral findings include bright red hyperplastic • Soft and edematous gingiva
edematous gingiva which bleeds easily and permanent • Blunting of the interdental papilla
destruction with generalized bone loss. • Loss of gingival stippling
• Gingiva bleeds spontaneously on palpation or probing
Familial neutropenia It is an inherited condition which
Periodontitis
can occur in benign and severe forms. There is moderate
Gingivitis plus at least one of the following clinical features:
to severe form of neutropenia in these conditions. Oral
• Gingival recession
findings are similar to that of chronic benign neutropenia • Periodontal pocket
of childhood. • Mobility
• Pathologic migration
Chronic idiopathic neutropenia This occurs mainly in
• Pus discharge from the gingival sulcus
females with persistent neutropenia from birth. Oral find-
• Furcation involvement
ings are similar to that of chronic benign neutropenia of
childhood.
Lazy leukocyte syndrome There is a defect in leuko-
RADIOGRAPHIC EVALUATION OF
cytic chemotaxis and mobility associated with severe
gingivitis.
PERIODONTAL DISEASES
Chronic granulomatous disease It is an inherited condi- Thumb rules

tion characterized by inability of neutrophils to generate
❍ Radiographs will not show soft tissue changes (e.g.
hydrogen peroxide due to absence of the enzyme NADPH
destruction of the periodontal ligament and pocket
oxidase. Oral condition includes severe and diffuse gingi-
depth). However, contrasting agents such as use of peri-
vitis with ulcerations.
odontal probe, gutta percha points may help delineate
Anemia It is defined as the reduction of concentration of soft structures.
hemoglobin in blood below at normal level. ❍ Intraoral periapical (IOPA), bitewing radiographs and
Oral findings include recurrent aphthous ulcers with orthopantomograph (OPG) reveal only two-dimensional
angular cheilitis, smooth and bald tongue, gingiva becomes images of three-dimensional structures. Hence, bone
pale in color. loss in different planes may not be evident in a single
Fanconi’s anemia which is an inherited form of anemia radiographic projection.
is associated with early tooth loss. ❍ The disease process is always ahead of the radiographic
presentation. It is estimated that for radiographic visual-
Acatalasia It is an inherited disease with the absence of ization a minimum of 40% demineralization is required.
acatase enzyme in RBCs and WBCs. Catalase is responsible ❍ Intraoral periapical radiographs have the best resolu-
for conversion of hydrogen peroxide (H2O2) to O2 and H2O; tion when compared to CT and OPG. Early and minimal
thus preventing oxidation of hemoglobin in RBCs. bone changes and bone marrow morphology are hence
Thrombocytopenic purpura It is characterized by low best appreciated in an intraoral periapical radiograph.
platelet count, spontaneous bleeding into skin and mucous ❍ Infrabony defects are impossible to be detected on intra-
membrane. Oral finding includes petechia in oral cavity. oral radiographs as one or both cortical plates super-
Gingiva is inflamed, soft and friable. Spontaneous bleeding impose over the defect.
in commonly seen.
Need for taking radiographs
Agammaglobulinemia It is an immune deficiency result-
❍ Evaluate the nature and extent of the causes for peri-
ing from a low antibody production from B cells. It is
commonly associated with severe periodontal destruction odontal destruction (subgingival calculus, overhanging
and tooth loss. margins of proximal restorations and poorly contoured
crowns).
Multiple myeloma It is a multifactorial neoplasm of ❍ Assess the width of the periodontal ligament space.
plasma cells with infiltration into maxilla and mandible. ❍ Evaluate crestal bone loss.
Oral lesions include gingival ulceration with bleeding ❍ Assessment of the pattern and extent of alveolar bone
and alveolar bone destruction. destruction (horizontal, angular, furcation involvement).
456
❍ Assessment of the anatomic morphology of the roots Interpretation of radiographs

and the crown root length.
❍ Radiographs have to be always mounted and viewed
❍ Assessment of the location of various anatomical
using a viewer box. Wet films should not be
structures.
interpreted.
❍ Evaluation of periapical pathologies, endo-perio lesions,
❍ Compare the normal anatomical findings with those
bone pathology and systemic conditions resulting in
that are diseased.
periodontal destruction.
❍ Normally the crestal bone is pointed in the anterior
Technique and exposure parameters regions and flat topped in the posterior teeth and par-
allel to the line joining the cementoenamel junctions
❍ Paralleling technique to be followed wherever feasible. of adjacent teeth.
❍ Use wire grids along with the film (will help in accu- ❍ The crest of the alveolar bone lies about 1.5 cm below
rate assessment of bone loss). the cementoenamel junction.
❍ 70 kVp, 8 mA machines are most ideal. ❍ Presence of a well-defined radiopaque cortical bor-
❍ Low contrast with long gray-scale are most suited for der in the alveolar crest is indicative of healthy peri-
evaluation of periodontal destruction. Lighter radio- odontium.
graphs tend to depict the cortical margins. ❍ Widening of periodontal ligament space at the cervical
portion of the tooth may indicate initial periodontitis.
Choice of radiographs ❍ In healthy periodontium, the lamina dura forms a
❍ Orthopantomograph can be used as a scout radiograph definitive, sharp angle with the alveolar crest.
to evaluate generalized destruction of periodontium. It ❍ Interproximal crater (type of angular defect) is evident
can also be used for assessing cortical plate width and as a trough-like depression that forms in the crest of
density for implant placement and relationship of the interdental bone.
teeth/implant to adjacent anatomical structures.
❍ Intraoral periapical radiographs can be used for assess- Radiographic features of periodontitis: Early to advanced stage
ing height and pattern of interdental bone, involve-
• Localized erosion of interdental alveolar bone crest
ment of furcation area, widening of the periodontal
• Blunting of the alveolar crest
ligament space, root resorption, periapical lesions and • Loss of the sharp angle between the lamina dura and the alveolar
endo-perio lesions. crest
❍ Bitewing radiographs can be used to assess crowns • Loss of the cortical surface of the crest
and interdental regions of a few upper and lower teeth in • Widening of the periodontal ligament space along the lateral margins
one radiograph. These radiographs are used for assessing of the tooth
crestal bone loss and factors that cause periodontal • Interdental horizontal/vertical bone loss
destruction such as subgingival calculus deposits, ill- • Vertical bone loss may appear either as an interproximal crater (seen
fitting crowns and poor restorative margins. as a trough-like depression in the interdental bone below the level of
❍ Full mouth IOPA radiographs are excellent means to the crestal edges) or as infrabony defect (vertical radiolucency within
bone extending apically along the root from the alveolar crest)
assess the periodontal status in a patient. The full mouth
• Bone surrounding the periodontal lesion may exhibit sclerosis owing
series consists of 16 IOPA radiographs (excluding four
to the inflammation.
radiographs for the third molars) and four bitewings.
457
CHAPTER
Regressive Alterations
17 of Teeth
Ravikiran Ongole, Sumati Nagappa
Baddannavar, Praveen BN
➧ Classification of Regressive Alterations Occupations Associated with Exposure to Acidic Fumes

Affecting Teeth Abfraction
➧ Tooth Surface Loss ➧ Classification of Tooth Wear
➧ Attrition Classification of Severity of Tooth Wear
Classification Based on Clinical Presentation
➧ Abrasion
➧ Resorption of Teeth
➧ Erosion (Corrosion)
Regressive Alterations of Dentin
Causes for Corrosion
Regressive Alterations of Pulp
Erosion Associated with Common Food Substances
Regressive Alterations of Cementum
Erosion Associated with Medications
Hypercementosis
Erosion Related to Deleterious Habits
Cementicles
Occupation-related Dental Erosion
With increasing oral health awareness and the advances in – Secondary to other pathology
healthcare delivery system the incidence of carious lesions – Resorption of roots of deciduous teeth
and periodontal diseases have drastically reduced over the ❍ Changes in dentin
ages. As such many individuals tend to retain healthy teeth – Secondary dentin
even in their old age. However, these teeth are subject to – Reparative dentin
functional wear and tear in addition to the natural age- – Dead tracts
related regressive alterations affecting the tooth and its ❍ Changes in pulp
supporting structures. – Reticular atrophy of pulp
Early recognition of the signs and symptoms of these – Pulp calcifications
regressive changes and understanding the basic patho- ❍ Changes in cementum
physiology behind such alterations will help the physician – Cementicles
plan an effective treatment plan. – Hypercementosis.
CLASSIFICATION OF REGRESSIVE TOOTH SURFACE LOSS

ALTERATIONS AFFECTING TEETH
Eccles in 1982 described ‘tooth surface loss’ or ‘tooth wear’
❍ Tooth wear as pathological loss of tooth tissue by a disease process
– Attrition rather than dental caries. Tooth wear was earlier referred
– Abrasion to as wasting diseases affecting teeth.
– Erosion (corrosion) Mair in 1992 described the term ‘non-carious cervical
– Abfraction lesions’ or ‘cervical wear’ to refer to loss of tooth substance
❍ Resorption of teeth at the cementoenamel junction. These non-carious cervi-
Internal cal lesions include cervical erosion, abrasion and abfraction.
– Internal inflammatory Clinical studies by Kitchin (1941) have shown that cervical
– Internal replacement wear lesions are often situated on the vestibular surfaces
External of teeth, seldom on lingual surfaces and rarely on proxi-
– Idiopathic mal surfaces. These are also more pronounced on incisors,
458
Chapter 17 – Regressive Alterations of Teeth
canines, and premolars and more prevalent in the maxilla Wear of the proximal surface causes decrease of arch
than in the mandible. length. Lambrechts et al (1989) reported that the rate of
The term tooth wear or tooth surface loss will include attrition is about 29 ␮m/year for molars and 15 ␮m/year
attrition apart from the cervical wear lesions. for premolars.
Yip et al (2004) studied the differential wear of teeth
and restorative materials. They found that the lowest wear
ATTRITION rates for restorations and the opposing dentition occur with
metal alloys, machined ceramics, and microfilled and micro-
Pindborg defined attrition as the loss of enamel, dentin or fine hybrid resin composites.
restoration by tooth-to-tooth contact. Tooth-to-tooth fric-
tion causes the form of wear called ‘attrition’. Two types of Radiographic findings
attrition have been described. They are physiologic and
Radiographically the morphology of the crowns is lost and
pathologic.
the occlusal and incisal edges of teeth are bereft of the
Physiologic attrition is referred to as the gradual and
radiopaque enamel cap. The curved cuspal edges are reduced
regular loss of tooth structure as a result of normal mastica-
to single flat plane. The incisocervical length of the crown
tion. However, pathologic attrition is confined to local areas
is shortened. Owing to the secondary dentin formation,
or specific groups of teeth caused by abnormal friction.
the size of the pulp chambers are reduced. In severe cases,
The physiologic causes for attrition include mastication
the pulp chamber and the pulp canals may be obliterated.
and deglutition. Pathologic causes that cause attrition are
Occasionally, hypercementosis may be seen associated with
abnormal occlusion, bruxism and habits such as tobacco
attrited teeth.
and betel chewing and defective tooth structure such as in
dentinogenesis imperfecta.
Clinical significance
Attrition involves occlusal/proximal surfaces of teeth.
It occurs more frequently in males than in females due to Attrition of teeth leads to esthetic and functional distur-
greater masticatory forces. It appears as a small polished bances. Dentinal hypersensitivity is usually the most com-
facet on cusp tips and causes flattening of incisal edges in mon complaint. Severe attrition leads to pulpal exposure
case of anterior teeth (Figure 1). The wear will lead to (Figure 2), non-vital teeth and periapical pathology. The
exposure of the dentin causing hypersensitivity. The worn razor sharp cuspal or incisal edges of teeth may cause
surfaces of opposing teeth occlude together very accurately. traumatic ulcers.
Figure 1 Figure 2
Severe attrition of the mandibular incisors causing exposure

Attrition of the mandibular posterior teeth in a patient with of the pulp. Photograph also showing attrition of incisal
the habit of tobacco chewing. Courtesy: Department of edges of maxillary central incisors. Courtesy: Department
Oral Medicine and Radiology, MCODS, Mangalore of Oral Medicine and Radiology, MCODS, Mangalore
459
It is often believed that generalized attrition will also

Figure 3
result in a reduction in occlusal face height (vertical dimen-
sion of occlusion). However, it has been often noticed that
in spite of the extensive loss of tooth surface the resting
facial height appears to remain unaltered primarily because
of dentoalveolar compensation. Bruxism-associated attri-
tion may be associated with temporomandibular dysfunction.
Management
Patients should be educated regarding the consequences of
attrition. Sharp edges of teeth can be smoothened. Desen-
sitizing toothpastes will help patients presenting with den-
tinal hypersensitivity. A soft bite guard will help break the
habit of bruxism and prevent further loss of tooth structure.
Teeth with pulpal exposure warrant endodontic treatment.
Finally, crowns may be fabricated for restoring esthetics.
Cervical abrasion with respect to the upper molar along
with gingival recession. Courtesy: Department of Oral
ABRASION Medicine and Radiology, MCODS, Mangalore
Pathologic wearing away of tooth substance through some

abnormal mechanical process especially in the presence of
abrasive materials is known as abrasion. It usually occurs Figure 4
on exposed root surfaces of teeth. Different foreign bodies
produce different patterns of abrasion. Most common type
of abrasion is toothbrush and dentifrices (toothpaste and/or
tooth powder) abrasion. Many of the residents in the vil-
lages of the Indian subcontinent use charcoal, brick and ash
to cleanse teeth. These indigenous dentifrices abrade teeth
further.
Clinical features
It is seen frequently on exposed root surfaces and in cervi-
cal regions of labial and buccal surfaces due to overzeal-
ous toothbrushing in horizontal manner. Maxillary teeth
are involved more than mandibular teeth, and left side is
affected commonly in case of right-handed persons and
vice versa. It appears as a V-shaped or a wedge-shaped Highly polished surface of the cervically abraded
ditch on the root side of the cementoenamel junction in teeth. Courtesy: Department of Oral Medicine and
Radiology, KLEDC, Bangalore
teeth with some gingival recession (Figure 3). The exposed
dentin appears highly polished (Figure 4). Improper use of
dental floss and tooth picks may also produce such lesions
Grippo et al (2004) described a term, ‘masticatory abra-
on the exposed proximal root surfaces.
sion’ to refer to tooth wear on the occlusal or incisal surfaces
In habitual pipe smokers, notching of teeth can be seen
due to friction from the food bolus. They reported that the
that conforms to the shape of the pipe stem. It can also be
masticatory abrasion can also occur on the facial and lingual
seen as occupation-related oral finding in carpenters and
aspects of teeth as coarse food is forced against these sur-
tailors who hold objects against the teeth during work.
faces by the tongue, lips and cheeks during mastication.
Exposure of dentinal tubules and the consequent irritation
of odontoblastic processes stimulates the formation of sec-
ondary dentin.
When tooth wear is accelerated by chewing an abrasive Radiographically, abrasive lesions caused by toothbrush
substance between opposing teeth, the process is termed are seen as half-moon shaped, well-defined radiolucent
demastication and it exhibits the feature of both attrition areas in the cervical regions of the teeth. These defects are
and abrasion. usually seen involving the maxillary premolar teeth.
460
However, abrasive lesions caused by dental floss are

Figure 5
usually slender half-moon-shaped radiolucent areas in the
proximal regions of the neck of the teeth. Owing to the
normal flossing pattern, the distal surface of teeth exhibit
relatively deeper radiolucent areas compared to the mesial
surfaces of teeth. Pulp chambers may be obliterated.
Radiographically, these radiolucent areas mimic cervical
carious lesions, root surface caries and cervical burn out.
Management
Patients should be educated regarding the correct brush-
ing and flossing technique. They should be advised to
discontinue any deleterious habits associated with their
occupations. Use of abrasive dentifrices should be strongly
discouraged.
Pulpal exposure is rarely a complication of cervical abra-
sion as the formation of secondary dentin protects the pulp
from being involved. Dentinal hypersensitivity when pres- Erosive lesions on the buccal surface of the maxillary teeth.
ent can be managed with the use of desensitizing toothpastes Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
and mouthrinses.
EROSION (Corrosion) Typically, when the occlusal surfaces of posterior teeth

with restorations are eroded, the margins of the restorations
Eccles in 1982 described erosion as the loss of dental hard appear higher than the level of the eroded enamel.
tissues by chemical action not involving bacteria. In simple
terms, erosion is the chemical or electrochemical dissolution
of teeth. Causes for Corrosion (Flowchart 1)
Grippo et al (2004) quoting the description of erosion by 1. Endogenous/intrinsic causes
The American Society for Testing and Materials Committee
on Standards proposed that the term erosion should be Frequent episodes of vomiting as seen in pregnancy, acid
replaced by the term corrosion. The American Society for reflux or regurgitation, anorexia nervosa and bulimia cause
Testing and Materials Committee on Standards defines ero- corrosion of teeth. Bodecker (1945) showed that the gingi-
sion as ‘the progressive loss of a material from a solid sur- val crevicular fluid is acidic and may cause corrosion when
face due to mechanical interaction between that surface and in contact with the cervical regions of the teeth.
a fluid, a multicomponent fluid, impinging solid or liquid Typically, the enamel is translucent and thin. The com-
particles’. In order to explain this in simple terms Grippo mon sites for erosion are the palatal surfaces of anterior
gave an example of river water flowing forcefully against teeth and occlusal surfaces of posterior teeth. Occasionally
the bridge supports leading to its erosion. other sites that may be affected include the areas of pooling
It is a common fact that no such powerful gush of oral of the contents of the gastric reflux.
fluids exists in the oral cavity. Thus, the term erosion may The erosion occurs from the action of hydrochloric acid
be replaced by corrosion to describe chemical dissolution and the proteolytic enzyme pepsin that is contained in
of teeth. gastric juice.
Erosion of the lingual or palatal surface of teeth due to
regurgitation of the acidic contents of the stomach, aggra-
Clinical features
vated by the circular movement of the tongue is termed
Erosive lesions are usually smooth surface lesions evident perimolysis (Figure 6).
on the buccal and labial surface of teeth. Occasionally, Another term used with regards to regurgitation of the
proximal surfaces may be involved. acidic contents is stress reflux syndrome. This syndrome
Clinically, erosions appear as wide, polished and smooth typically affects young working adults. The acidic reflux
areas on the enamel approximating the cervical margin of usually occurs during the working hours at day. The regur-
the tooth (Figure 5). The erosive areas are almost always gitated acidic contents are held in the mouth before being
shallow and exhibit ‘scooped-out’ architecture. The com- swallowed again. This syndrome produces erosion of the
mon teeth to be affected by erosion are the anterior teeth. buccal surfaces of mandibular posterior teeth.
461
Flowchart 1
Erosion
Intrinsic causes Extrinsic causes
Frequent vomiting Reflux Carbonated drinks (pH of 2–3)

• Anorexia nervosa • Gastroesophageal • Citrus food
• Pregnancy reflux disease • Occupational hazards (wine tasters,
• Bulimia • Stress reflux syndrome workers in acidic environments)
Intrinsic and extrinsic causes for tooth erosion
Figure 6 Evans and Briggs (1994) reported a pregnant woman who

had tooth surface loss on the palatal surfaces of maxillary
incisors due to prolonged pregnancy-induced vomiting.
2. Exogenous/extrinsic causes
Acids present in the food consumed such as fruits, carbon-
ated soft drinks, etc. cause corrosion of teeth. Other causes
for corrosion include occupations where an individual is
exposed to acidic fumes, habitual sucking of lemon, tama-
rind, etc. Chewable forms of certain medications such as
vitamin C tablets can aggravate the corrosion of teeth.
pH of the erosive agent though an important factor for
the erosive potential, other factors such as frequency and
method of contact of the erosive agent and the proximity
of toothbrushing after exposure to the erosive substance
have a substantial bearing on the tooth erosion.
Erosive lesions on the palatal surface of the maxillary
anterior teeth. Courtesy: Department of Oral Medicine Erosion Associated with Common Food
Substances
Many of the commonly used food substances have an
Pregnancy and dental erosion Laine (2002) described acidic pH. Most of these commodities have a pH less than
the effect of pregnancy on periodontal and dental health. the critical pH of 5.5 that causes demineralization of teeth.
He reported that the tooth environment is significantly It is believed that brushing teeth immediately after con-
altered in pregnancy. He stated that a number of salivary suming acidic food may further weaken the tooth and
cariogenic microorganisms may increase in pregnancy, make it vulnerable to erosive potential of dietary acids.
along with decrease in salivary pH and buffer effect. Table 1 summarizes the acidic content of some com-
These changes in salivary composition in late preg- monly consumed food substances.
nancy and during lactation may temporarily predispose the
individual to dental caries and erosion.
Erosion Associated with Medications
Rockenbach et al (2006) studied the salivary flow rate,
pH, and concentrations of calcium, phosphate and sIgA in Pierro et al (2005) evaluated the free sugar concentration
Brazilian pregnant and non-pregnant women. In their study, and pH of pediatric medicines. The median of pH values of
there was no difference in salivary flow rates and concen- the syrups varied from 2.6 to 6.1. In their study, most of
trations of total calcium and phosphate between pregnant the pediatric syrup medicines showed high concentration
and non-pregnant women. However, pregnant women had of free sugars and pH below the critical value (5.5), which
lower pH (6.7) than non-pregnant women (7.5). can increase their cariogenic and erosive potentials.
462
Table 1 Acidic content of common food substances

contrasting results could be explained with the different
patterns of betel quid chewing habit and the amount of
Food substance pH lime and other constituents that have been used in the
Apples 2.9–3.5 betel quid.
Grapes 2.9–3.4
Chronic alcoholics
Lime 1.8–2.4
Harris et al (1997) studied the effects of alcohol on oral
Oranges 2.8–4.0
and dental health in 107 patients. They found a high inci-
Pineapples 3.3–4.1 dence of tooth wear and trauma to the dentition.
Strawberries 3.0–4.2 Robb and Smith (1990) studied the prevalence of path-
Coffee 2.4–3.3 ological tooth wear in 37 chronic alcoholics. These patients
Black tea 4.2
had more tooth wear compared to age and sex matched
controls. The erosion was more commonly seen in males
Wines 2.3–3.3
and those who consumed alcohol regularly than those
7Up® 3.5 who had alcohol in episodic binges. Almost 40% of the
Pepsi ®
2.7 patients exhibited erosion on the palatal surfaces of the
Coke ®
2.7
upper anterior teeth.
Mandel (2005) suggested that the acidity of wine can
Vinegar 2.4–3.4
contribute significantly to dental erosion.
Ketchup 3.7
Fruit jam 3.0–4.0
Occupation-related Dental Erosion
Source: Adapted from Clark et al (1990) and Gandara et al (1999).
Wine tasters
Wines primarily contain tartaric acid and to a lesser
Hellwig and Lussi (2006) described the effects of oral extent malic acid, lactic acid and citric acid. Minute
hygiene products and acidic medicines. Acidic or EDTA- amounts of succinic acid, citramalic acid, galacturonic
containing oral hygiene products and acidic medicines acid and mucic acid may be found in some wines. Carbonic
have the potential to soften dental hard tissues. Chewable acid is usually added in champagne to produce the spar-
acetyl salicylic acid tablets and chewable hydrochloric kling. The pH of wine is approximately 3.0–4.0. It is a
acid tablets for treatment of stomach disorders can cause common fact that teeth that are softened after drinking
erosion of teeth. Chewable forms of vitamin C supplements wine are highly vulnerable to attrition or abrasion. It is
have also been known to cause erosion. also believed that wine alters the inherent salivary protec-
tion mechanism.
In studies involving wine tasters by Ferguson et al
Erosion Related to Deleterious Habits (1996), Chaudhry et al (1997), Gray et al (1998), the erosion
of teeth was primarily confined to the maxillary incisors.
Areca nut chewing habit
Most of the wine tasters evaluated about 20–30 wines per
Apart from the mechanical wear of tooth surface, chewing day and most of these tasters had been in the profession
betel quid may have the potential to cause erosion of teeth. for about 10–23 years.
It is believed that the betel quid can cause a change in sali- Chikte et al (2005) reported that wine tasters kept the
vary pH and its buffering capacity. wine to be tasted in the mouth for 10–30 seconds. In their
Rooban et al (2006) studied the effect of habitual areca study, wine tasters exhibited almost three times higher risk
nut chewing on resting whole mouth salivary flow rate for dental erosion compared to non-taster controls.
and pH. The mean pH of saliva in non-chewers in their study Mok et al (2001) in their in vitro study stated that the
was 6.77; whereas the salivary pH was 6.27 in individuals erosive potential of wine increased with increasing tem-
who chewed betel quid along with lime. It is believed that perature of the wine.
in habitual betel quid chewers, lime probably reacts with Various in vitro studies have shown that red wine, white
bicarbonate buffering system by the loss of bicarbonate, wine and champagne have the potential to cause erosion of
turning saliva more acidic. Lime (calcium oxide in aqueous teeth. White wine and champagne are said to cause rela-
form—calcium hydroxide) could cause a free radical injury tively more erosion than red wine.
or the high alkaline content of lime may react with the Piekarz et al (2008) showed that dentin is more suscep-
salivary buffering system and alter the pH. tible to wine erosion than enamel. This finding supports
However, Reddy et al (1980) observed no difference in the hypothesis that erosion progresses much more rapidly
salivary pH between the chewers and non-chewers. These once the dentin is involved.
463
Professional swimmers Parafunctional habits, malocclusion, excessive mastica-

tory load and use of dental appliances are some known
Centerwall et al (1986) studied the extent of enamel erosion
causes for abfraction.
among professional swimmers at a gas-chlorinated swim-
Abfraction commonly occurs in the cervical region of
ming pool. In their study, the pool water sample had a pH
teeth, where flexure may lead to the fracture of the slender
of 2.7. Although the recommended pH for swimming pools
layer of enamel rods, as well as microfracture of cementum
is 7.2–8.0. They used the term ‘swimmer’s erosion’ for the
and dentin.
acid erosion of dental enamel caused by inadequately main-
Morphologically they appear as crescent-shaped lesions
tained gas-chlorinated swimming pools.
along the cervical margin of the tooth. It often affects a
single tooth with adjacent unaffected teeth. It commonly
Occupations Associated with Exposure affects the mandibular dentition because of the lingual ori-
to Acidic Fumes entation and increased susceptibility to concentration of
tensile stresses in the cervical region.
Workers of battery and galvanizing factories, galvanizing,
pickling, plating and chemical manufacturers are constantly
exposed to sulfuric acid and hydrochloric acid and to some
CLASSIFICATION OF TOOTH WEAR
amount of phosphoric, nitric and hydrofluoric acids. Inhala-
tion of acidic fumes leads to the erosion of the incisal and
labial surfaces of anterior teeth. Bell et al (2002) in their study to assess tooth wear in chil-
Elsbury et al (1951) reported erosion of teeth in tin fac- dren with Down syndrome, classified the severity of tooth
tory workers who are exposed to 11 mg/m3 tartaric acid dust wear as follows:
for 30 hours a week.
Ten Bruggen Cate (1968) reported dental erosion in indi- Classification of Severity of Tooth Wear
viduals involved with the preparation of sanitary cleansers.
Individual studies have also shown dental erosion in ❍ Group A—No tooth wear or mild tooth wear
workers of silicone producing units. – Small degree of physiological wear present on 1–2
teeth
Management – Tooth wear that requires no clinical intervention
❍ Group B—Moderate level of tooth wear
Piekarz et al (2008) studied the role of Tooth Mousse (GC – Higher than physiological level of wear on two or
Corporation) in preventing erosion of teeth in wine tasters. more teeth that have not affected function or esthetics
Tooth Mousse contains an anticariogenic remineralizing – Tooth wear that requires clinical intervention
agent CPP-ACP (a casein phosphopeptide that stabilizes ❍ Group C—Moderate-severe tooth wear
amorphous calcium phosphate nanocomplex) and has supe- – Pathological wear on four or more teeth, defined as
rior remineralizing properties compared to fluoride. teeth with approximately two-thirds to one-half of
Studies have shown that Tooth Mousse reduces tooth the clinical crown remaining
wear from attrition in both acidic and neutral environ- – Tooth wear that requires immediate and aggressive
ments. It has also been shown that Tooth Mousse can min- clinical intervention
imize erosion of enamel caused by citric acid. Studies have ❍ Group D—Severe tooth wear
shown that Tooth Mousse can reduce enamel, dentin and – Pathological wear on four or more teeth, defined as
cemental erosion. teeth with one or more of the following features:
Dentinal hypersensitivity caused by erosion can be man- • Less than one-third of the clinical crown remaining
aged with desensitizing agents. Larger erosive lesions can • Wear to the gum-line
be restored. • Severely scooped dentin
– Tooth wear of a severity rarely seen in this age group
– Tooth wear that requires immediate and aggressive
ABFRACTION clinical intervention.
Abfraction may be described as the microstructural loss of

Classification Based on Clinical Presentation
tooth substance in areas of stress concentration caused by
tooth flexure. Theoretically it is believed that occlusal ❍ Group A—no wear
forces create stresses in the cervical area of the enamel ❍ Group B—well-defined facet (indicating attrition)
and dentin thereby predisposing the tooth to abrasion and ❍ Group C—ill-defined facet (indicating multifactorial
erosion. etiology)
464
Table 2 Tooth wear index criteria
Score Surfaces of tooth involved Criteria

0 Buccal, lingual, incisal No loss of enamel surface characteristics
1 Buccal, lingual, occlusal, incisal Loss of enamel surface characteristics
2 Buccal, lingual, occlusal Loss of enamel, visible dentin for ⬍1/3 of the surface
3 Buccal, lingual, occlusal Loss of enamel, visible dentin for ⬎1/3 of the surface
incisal Loss of enamel just exposing dentin
4 Buccal, lingual, occlusal Complete loss of enamel, or pulp exposure, or secondary dentin
incisal Pulp exposure or exposure of secondary dentin
9 Buccal, lingual, occlusal, incisal Excluded from analysis missing tooth, partially erupted, orthodontic band,
composite restoration, any crowns, tooth fracture, and fissure sealant
Source: Proposed by Smith and Knight, modified by Millward et al.
❍ Group D—well-defined areas of dentin or pitting of ❍ Score between 3 and 8: Oral hygiene and dietary assess-
dentin (indicating erosion). ment, and advice, routine maintenance and observa-
Tooth wear index criteria are given in Table 2. tion, Repeat at 2-year intervals.
❍ Score between 9 and 13: Oral hygiene and dietary
Bartlett et al (2008) proposed a new scoring system for
erosive tooth wear which can be used both for scientific assessment, and advice, identify the main etiological
and clinical purposes. They termed this scoring system as factor(s) for tissue loss and develop strategies to elim-
Basic Erosive Wear Examination (BEWE). inate respective impacts. Consider fluoridation measures
In this system, all teeth in every sextant are assessed or other strategies to increase the resistance of tooth
and the most severely affected tooth in every sextant is surfaces. Ideally, avoid the placement of restorations
scored and the cumulative score will guide the dental prac- and monitor erosive wear with study casts, photographs
titioner in choosing the best treatment modality. or silicone impressions. Repeat at 6- to 12-month
intervals.
❍ Score 14 and over: Oral hygiene and dietary assessment,
Criteria for grading erosive wear
and advice, identify the main etiological factor(s) for
❍ Score 0—No erosive tooth wear tissue loss and develop strategies to eliminate respec-
❍ Score 1—Initial loss of surface texture tive impacts. Consider fluoridation measures or other
❍ Score 2—Distinct defect, hard tissue loss ⬍50% of the strategies to increase the resistance of tooth surfaces.
surface area Ideally, avoid restorations and monitor tooth wear
❍ Score 3—Hard tissue loss ⱖ50% of the surface area. with study casts, photographs or silicone impressions.
The buccal/facial, occlusal, and lingual/palatal surfaces Especially in cases of severe progression consider spe-
are examined. Once all the sextants have been assessed, cial care that may involve restorations. Repeat at 6- to
the sum of the scores is calculated. 12-month intervals.
BEWE scores
RESORPTION OF TEETH
❍ Sextant (17–14)—record the highest score
It is defined as a condition or pathologic process resulting
in the loss of dentin, cementum and/or bone.
❍ Sextant (33–43)—record the highest score Types of resorption
1. Internal resorption
The sum of the highest scores for each sextant is taken as
a. Internal replacement
the indicator for treatment planning.
b. Internal inflammatory
i. Transient
Management strategies
ii. Progressive
❍ Score ⱕ2: Routine maintenance and observation, repeat 2. External resorption
at 3-year intervals. a. External surface resorption
465
Figure 7 Figure 8
Intraoral periapical radiograph showing external resorption External root resorption of the second molar secondary to the
of the root apex in a long-standing periapical abscess. dentigerous cyst associated with the impacted third molar.
Courtesy: Department of Oral Medicine and Radiology, Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore MCODS, Mangalore
b. External inflammatory
Figure 9
c. Ankylosis
d. Replacement resorption
3. Combined internal and external
4. Transient apical breakdown.
Internal resorption can occur due to various causes like
chronic pulp inflammation, trauma, pulpotomy, restor-
ative procedures, herpes zoster, hereditary, cracked tooth
syndrome, invaginated cingulum and orthodontic tooth
movement.
External resorption can be caused due to replantation
of tooth, orthodontic forces, eruption of neighboring teeth,
root fracture, trauma, necrotic pulp, chronic periapical
inflammation or infections (Figure 7), root planing, citric
acid treatment and other pathological conditions like cysts
(Figure 8), ameloblastoma (Figure 9), giant cell tumor,
fibrous osseous lesions, hereditary conditions, bleaching
(especially causes cervical external resorption) and orthog-
nathic surgery.
Diagnosis is often misinterpreted. External resorption
can be described as internal or vice versa. Often the diag-
nosis can be simplified by a careful assessment of the his- External root resorption of the mandibular first molar
tory, particularly if it is trauma. secondary to ameloblastoma involving the body and
A pink appearance of the crown is indicative of a highly ramus of the mandible. Courtesy: Department of
vascular tissue which has removed sufficient enamel and Oral Medicine and Radiology, MCODS, Mangalore
dentin to allow it to be visible through the overlying tooth
substance. Although earlier descriptions of a pink tooth as
being internal is incorrect as it may be either internal or Clinical assessment of the mobility may be of use in
external in origin. In the absence of history of trauma, it is differential diagnosis. A tooth displaying no mobility or held
more likely that the pink spot will indicate an external in infraocclusion indicates ankylosis or replacement resorp-
origin. tion. Percussion test will also produce a resonant sound.
466
The key diagnostic tool is a good radiograph. The margins

Flowchart 2
of internal resorptive defect are smooth and well defined;
in contrast to that of external are rough and has ‘moth-eaten’ Traumatic injury
appearance. Most internal resorptive defects are symmet-
rical and external resorptive defects are usually asymmet-
rical. The anatomic configuration of the root canal is altered Intrapulpal hemorrhage
and increased in size with internal resorption. In external
resorption, the canal is unaltered, and its outline can be fol-
lowed through the resorptive defect. Radiographs exposed Organizes to granulation tissue
at different horizontal beam angles can aid in distinguish-
ing internal from external resorption. If the defect is inter-
Compresses the dentinal wall
nal, the relationship of the canal to the defect will remain
the same, regardless of the angles. If the defect is external,
the relationship of the defect to canal will shift as the Predentin formation stops and
horizontal angle of the beam is altered. Key cells involved odontoclasts differentiate
in resorption are monocytes, macrophages, osteoclast, odon-
toclasts, dentinoclasts and cementoblasts.
Internal resorption
Systemic regulatory factors
Parathyroid hormone increases osteoclastic activity by Events in resorption of teeth
activation of osteoclasts to increase the production of car-
bonic anhydrase and promotion of fusion of marrow cells structure has no relationship with the normal bone forms
to produce multinucleated giant cells. of tooth, hence termed as metaplastic tissue. Radiographi-
1,25-Dihydroxy vitamin D increases the activity of osteo- cally, it appears as an enlargement of the pulp space. The
clasts without increase in number and calcitonin inhibits pulp space thus enlarged is less radiodense giving the
resorption by suppressing the osteoclastic cytoplasmic motil- appearance of partial canal obliteration.
ity and producing cell retraction.
Neuronal regulatory factors Events in resorption of teeth (Flowchart 2)
Neuropeptides released by the terminal nerve endings such Treatment consists of pulp tissue removal and canal cleaning
as substance P (SP), calcitonin gene related peptide (GRP), and shaping, followed by scaling the canal system.
neurokinin A, vasoactive intestinal polypeptide (VIP), etc.
increase vasodilatation, thus an increase in macrophages External resorption
and an increased osteoclastic activity, remove mechanism External resorption is the one which occurs primarily from
of resorpion. the periodontal space affecting the root surface. Four cat-
egories of external resorption have been described based
Internal resorption
on the clinical and histological manifestations:
It is also called chronic perforating hyperplasia of pulp,
❍ External surface resorption
internal granuloma, odontoclastoma and pink tooth of
❍ External inflammatory resorption
Mummery. Resorption of internal type occurs from pulp
– Cervical
space/root canal space and is asymptomatic. It is usually
– Apical
discovered during routine radiographic evaluation. A pink
❍ Ankylosis
colored discoloration is visible if extensive internal resorp-
❍ External replacement resorption.
tion occurs in the coronal portion of the tooth, as described
by Mummery. Most cases are found in the anterior region. External surface resorption is a transient phenomenon in
Etiological factors are mainly trauma and caries. which the tooth undergoes spontaneous destruction and
Internal resorption can be of two types: internal repair. It is found in all the teeth and considered to be a
replacement (metaplastic) and internal inflammatory. normal physiologic response. It is a self-limiting process
Internal replacement type occurs as a result of low- and does not require any treatment.
grade irritation of the pulp such as chronic irreversible External inflammatory root resorption is described as a
pulpitis or pulp necrosis. This can occur because of trauma bowl-shaped defect which penetrates the dentin. This
or application of extreme heat to the tooth. There is con- occurs following irritation or injury of the periodontium due
comitant resorption of hard tissue with frequent deposition to trauma, periodontal infection or orthodontic treatment.
of hard tissue resembling bone or cementum. The resultant Based on location it can be cervical and apical. Cervical
467
resorption occurs following injury to the epithelial cervical

Figure 10
attachment apparatus. It can occur because of physical
insult like trauma, surgical procedures, orthodontic treat-
ment, bruxism and periodontal root planing. Chemical
injury as a result of 30% hydrogen peroxide can cause
cervical resorption.
Apical resorption occurs because of traumatic injury
(luxative and intrusive movements), periradicular peri-
odontitis and orthodontic procedures.
Ankylosis is primarily associated with luxation injury,
especially avulsion. It is the union of tooth and bone with
no intervening connective tissue following extensive
resorption. Ankylosis can be further categorized as tran-
sient or progressive in nature.
External replacement resorption is caused due to luxa-
tive injury. This is a continuous process by which the tooth
is gradually resorbed and replaced by bone. It differs from
Dead tracts and reparative dentin. Courtesy: Department of
ankylosis in which it exhibits the presence of intervening
inflamed connective tissue.
Regressive Alterations of Dentin in transmitted light and white in reflected light (Figure 10).
The causes for dead tracts include caries, attrition, abrasion
Secondary dentin Physiologic dentin deposition pro- and erosion. These are seen more in narrow pulpal horn
ceeds throughout life. It is a protective response and can areas, because of crowding of odontoblasts. More commonly
be differentiated from primary dentin by the sharp bend- these are seen in older individuals.
ing of tubules producing a line of demarcation. It has
incremental patterns of deposition. The tubular structures
are less regular than those of primary dentin. The deposi- Regressive Alterations of Pulp
tion occurs in greater quantities in the floor and roof of Reticular atrophy of pulp change is seen in older individu-
pulp chamber than on walls; so, there is reduction of pulp als. Clinically asymptomatic, responds normally to vitality
chamber and pulp horns as the tooth ages. tests. Histopathologically, large vacuolated spaces in pulp
Reparative dentin It is also known as irregular or tertiary are seen with decreased cellular elements. Degeneration and
dentin. It is a protective response to noxious stimuli such disappearance of odontoblast is also seen.
as caries operative procedures, restorative materials, abra- Pulp calcifications Pulp calcifications (pulp stones, den-
sion, erosion or trauma. It is deposited at the rate of 1.5 ␮m ticles) are nodular calcified masses appearing in either or
per day, produced by replacement odontoblasts. Reparative both the coronal and radicular portion of pulp (Figure 11).
dentin has less tubules and less mineralized. There is a def- These are usually asymptomatic until they impinge on
inite demarcation between secondary and reparative den- nerves or blood vessels. Pulp stones are classified as true
tin which is called ‘calcitraumatic line’. denticles or false denticles according to microscopic
appearance. True denticles are rare and resemble dentin
Dentinal sclerosis It is a regressive alteration in tooth
structurally with dental tubules and odontoblastic pro-
surface that is characterized by calcification of the den-
cesses and are usually seen close to the apical foramen.
tinal tubules. It is also called transparent dentin. The causes
False denticles do not resemble dentin. These do not exhibit
for dentinal sclerosis includes aging caries and tooth wear.
dentinal tubules but have concentric layers of calcified
Exact mechanism of deposition of calcium salts is not
tissue. Flowchart 3 describes the formation of pulp stone.
understood. Most likely source is dentinal fluid within the
Pulp stones can be either free, attached or embedded
tubules. Initially, only sporadic hydroxyapatite crystals
according to the mode of attachment. Free dentricles are
are deposited which gradually fill the tubules. This makes
not attached to walls of pulp space; attached stones are
it to appear transparent in transmitted light and dark in
attached to wall of pulp cavity, and embedded dentricles
reflected light. It decreases the permeability of dentin and
are attached and become an integral part of dentin. Various
slows the carious process. It decreases the conductivity of
causes of formation of pulp stones are aging, systemic
odontoclastic process.
causes like cholelithiasis, cardiac, renal lithiasis, arterioscle-
Dead tracts When the odontoblasts disintegrate completely, rosis, gout, acromegaly, and diseases such as dentin dys-
their empty tubules are filled with air, so they appear black plasia type II, pulpal dysplasia, tumoral calcinosis, calcinosis
468
universalis and Ehlers–Danlos syndrome. It may cause of the periodontal ligament fibers that anchor the tooth to
problem during endodontic therapy. Radiographically, it the surrounding structures. It is avascular.
appears as radiopaque masses in pulp space (Figure 12). The inorganic component of cementum consists of
hydroxyapatite and fluoride. It is interesting to note that
the fluoride content in cementum is the highest compared
Regressive Alterations of Cementum to all mineralized tissues.
Cementum is the mineralized dental tissue that overlies the Cellular cementum (presence of cementocytes) is gener-
anatomic roots of teeth. It provides the site for attachment ally seen at the apical one-third of the root; whereas the
rest of the root surface is covered by acellular cementum
(lack of cementocytes).
Figure 11
Figure 12
Radiograph revealing well-defined radiopacities in the pulp

chamber of first and second molars suggestive of pulp stones.
Histologic appearance of a free pulp stone. Courtesy: Courtesy: Department of Oral Medicine and Radiology,
Department of Oral Pathology, MCODS, Mangalore MCODS, Mangalore
Flowchart 3
Process of formation of pulp stone
Local metastatic dysfunction Trauma
Hyalinization of injured wall Vascular damage

(thrombosis)
Fibrosis
Mineralization
(nidus formation)
Grows with time
Pulp stone
Process of formation of pulp stone
469
Figure 13 Figure 14
Bulbous roots evident in extracted teeth specimen. Courtesy:

Intraoral periapical radiograph showing thickening of the
Department of Oral Pathology, MCODS, Mangalore
roots. Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
The thickness of cementum overlying the root varies

based on the location. The thickness of the cementum at
Figure 15
the cementoenamel junction is the thinnest and it measures
20–50 ␮m at the apex. The thickness of cementum is roughly
150–200 ␮m.
Hypercementosis
Hypercementosis is a non-neoplastic deposition of excessive
cementum which is continuous with the normal radio-
graphic cementum. It is asymptomatic and seen in adult-
hood. The incidence of hypercementosis increases with
age. It may appear in one tooth or involving multiple teeth
(Figure 13). Hypercementosis is frequently seen in relation
to the premolars.
The cause for hypercementosis can be either due to
local factors like abnormal occlusion, inflammation, non-
functional tooth, tooth repair, root fracture, and cemental Radiograph revealing bulbous roots with intact lamina dura.
tear or systemic factors like Paget’s disease, hyperpituita- Courtesy: Department of Oral Medicine and Radiology,
rism (acromegaly and gigantism), arthritis, calcinosis, MCODS, Mangalore
rheumatic fever, thyroid goiter, idiopathic.
Radiographically, thickening or blunting of root is seen.
Enlarged root is surrounded by the radiolucent periodontal
Cementicles
ligament space and intact radiopaque lamina dura (Figures 14
and 15). Small foci of non-neoplastic calcified tissue, which lie in
Histological section reveals deposition of cellular or periodontal ligament along the lateral and apical root
secondary cementum in concentric layers over the existing areas are called cementicles. Calcification of epithelial cell
thin layer of acellular or primary cementum. This cellular rest of Malassez, aging and cemental tears undergoing
cementum has also been referred to as osteocementum remodeling have all been implicated in the formation of
owing to its histological resemblance to bone. cementicles. These present as circular laminated structures.
Treatment is not required but it may pose problem during When attached to root, cementum shows roughened globular
extraction. outline on the root surface.
470
SECTION System Review
VII
18 Systemic Disorders and their Clinical Implications 473

19 Bone Diseases and Fibro-osseous Lesions 568
20 Autoimmune Disorders 590
21 Granulomatous Diseases 605
22 Sexually Transmitted Diseases 625
23 Nutritional and Metabolic Disorders 633
471
Chapter-18.indd 471 10/3/2012 2:27:07 PM


Systemic Disorders and CHAPTER
their Clinical Implications
Arshiya Ara, Ceena Denny, Gajendra
Veeraraghavan, Seema Patil, Vishwananth R,
Sarat Gummadapu, Veena KM, Ranganath
18
Ratehalli, Sandeep Deshpande
Cardiovascular Disorders ➧ Neoplastic Disorders

➧ Symptoms Suggestive of Cardiovascular Leukemia
Disease Lymphomas
Chest Pain Multiple Myeloma
Breathlessness or Dyspnea ➧ Bleeding Disorders
➧ Common Cardiovascular Disorders and Classification of Bleeding Disorders
their Dental Considerations ➧ Vascular Disorders (Vessel Wall)
Hypertension Hereditary Hemorrhagic Telangiectasia
➧ Ischemic or Coronary Heart Disease Ehlers–Danlos Syndrome
Angina Pectoris Marfan’s Syndrome
➧ Myocardial Infarction Scurvy
➧ Congenital Heart Disease ➧ Platelet Disorders
Cyanotic ➧ Thrombocytopathic Disorders
Acyanotic ➧ Thrombocytopenic Disorders
➧ Rheumatic Fever Idiopathic Thrombocytopenic Purpura
➧ Infective (Bacterial) Endocarditis Thrombotic Thrombocytopenic Purpura
Clinical Conditions that Warrant Use of Antibiotic ➧ Disorders of Coagulation
Prophylaxis
➧ Inherited Coagulation Disorders
Treatment Needing Antimicrobial Prophylaxis in
Hemophilia A
Patients at Risk of Infective Endocarditis
Hemophilia B and C
➧ Heart Failure Von Willebrand’s Disease
Classifications of Heart Failure ➧ Acquired Coagulation Disorders
Oral Disease Aspects Liver Disease
Hematological Disorders Vitamin K Deficiency
➧ Red Blood Cell Disorders Disseminated Intravascular Coagulation
➧ Polycythemia Respiratory Disorders
➧ Anemia ➧ Classification of Respiratory Tract Disorders
Iron Deficiency Anemia ➧ Common Symptoms of Respiratory Diseases
Paterson–Kelly Syndrome (Plummer–Vinson Syndrome) Cough
Anemia due to Vitamin B12 and Folic Acid Deficiency Sputum
Aplastic Anemia Hemoptysis
Glucose-6-phosphate Dehydrogenase Deficiency Chest Pain
Sickle Cell Anemia Breathlessness or Dyspnea
➧ Thalassemia Wheezing
➧ White Blood Cell Disorders ➧ Upper Respiratory Tract Infections
➧ Qualitative Disorders Viral Infections
Chediak–Higashi Syndrome Allergic Rhinitis
➧ Non-neoplastic Disorders Pharyngitis and Tonsillitis
Leukopenia ➧ Lower Respiratory Tract Infections
Reactive Leukocytosis Asthma
473
Section VII – System Review
Chronic Obstructive Pulmonary Disease or ➧ General Overview of Disorders of Endocrine

Chronic Obstructive Airway Disease System
Cystic Fibrosis Categories of Disturbances of Endocrine Function
➧ Granulomatous Diseases Pathophysiology of Hypothalamic–Pituitary Axis
➧ Malignant Disorders ➧ Growth Hormone
Bronchogenic Carcinoma Growth Hormone Deficiency
➧ Other Respiratory Diseases Growth Hormone in Children—Dwarfism
Legionnaire’s Disease (Legionellosis) Adult GH Deficiency
Obstructive Sleep Apnea Syndrome Growth Hormone Excess
Gigantism
Renal Disorders Growth Hormone Excess in Adults
➧ Renal Diseases ➧ Disorders of Posterior Pituitary
Chronic Renal Failure (Neurohypophysis)
Uremic stomatitis Diabetes Insipidus
Gastrointestinal Disorders ➧ Thyroid Gland
➧ Gastroesophageal Reflux Disease Pathophysiology of Hypothalamic–Pituitary–Thyroid
➧ Inflammatory Bowel Disease Axis
➧ Disorders Associated with Thyroid Gland
➧ Ulcerative Colitis
Hypothyroidism
➧ Crohn’s Disease
Hyperthyroidism
➧ Hiatal Hernia
➧ Parathyroid Glands
➧ Peptic Ulcer Disease Hyperparathyroidism
➧ Eating Disorders Hypoparathyroidism
Anorexia Nervosa Tetany
Bulimia Nervosa ➧ Hypothalamus–Pituitary–Adrenal Axis
➧ Liver Diseases Cushing’s Syndrome
Jaundice Endocrinopathic Pigmentation
Hepatitis Adrenal Insufficiency
Neuromuscular Disorders Aldosteronism
Disorders of Adrenal Medulla
➧ Bell’s Palsy
Sex Hormones
➧ Epilepsy
➧ Pregnancy
➧ Parkinsonism Multiple Endocrine Neoplasia Type III
➧ Multiple Sclerosis (Multiple Mucosal Neuroma Syndrome)
➧ Muscular Dystrophy ➧ Saliva and Monitoring of Hormone Levels
➧ Oromandibular Dystonia
Mental Health and its Relevance to Dentistry
➧ Myasthenia Gravis
➧ Concepts of Mental Illness and its Classification
Endocrinal Disorders ➧ Diagnostic Criteria and Screening Questions
General Overview of Functional Mechanisms of
➧ Interesting Interface between ‘Dentistry’ and
Endocrine System
‘Psychiatry’
Hormones
➧ Management of Psychiatric Disorders
CARDIOVASCULAR DISORDERS Cardiovascular history taking

Clinicians must assess the status of cardiovascular system
Cardiovascular disease is quite common, though more fre- within the context of the patient’s overall health. These asso-
quent and severe in later life, can also affect young indi- ciated conditions can heighten cardiovascular risk during
viduals. It is one of the leading causes of death in the world. dental care. Consequently, an initial medical history should
A thorough knowledge of cardiovascular diseases is nec- be obtained from all patients and it must be reviewed with
essary because of its implications in dentistry and also the the patient at each appointment to identify serious cardiac
initial measures taken by the dentists in case of certain conditions. The clinicians should also note history of cere-
emergency conditions can be lifesaving. brovascular disease, renal dysfunction, chronic pulmonary
474
Chapter 18 – Systemic Disorders and their Clinical Implications
disease, diabetes mellitus, anemia, dyslipidemia, periph- – The functional capacity can be expressed in terms
eral vascular disease, orthostatic intolerance and anemia. of metabolic equivalent (MET) values. Functional
Further an accurate record of current medication taken by capacity can be classified as:
the patient, use of tobacco products, alcohol and over-the- ❍ 1 MET or more
counter and recreational drugs should be documented. – Do light work around the house
– Walk a block on level ground at 3.2–4.8 km/
hour
SYMPTOMS SUGGESTIVE OF ❍ 4 METs or more
CARDIOVASCULAR DISEASE – Climb a flight of stairs or walk up a hill
– Walk on level ground at 6.4 km/hour
Chest Pain – Run a short distance
– Do heavy work around the houses (scrubbing
It is the common presentation of cardiac diseases, though can floors, lifting or moving heavy furniture)
be a manifestation of disease of the lungs, musculoskeletal ❍ 10 MET or more
system or gastrointestinal system. Pain is usually felt behind – Participate in strenuous sports (swimming,
the sternum, radiates across the chest and down the arms, singles tennis, football, basketball or skiing)
it may also radiate to the back or to the mandible.
The functional capacity can be graded as:
❍ Excellent (if 10 MET)
Breathlessness or Dyspnea
❍ Good (7–10 MET)
It is a major symptom of many cardiac disorders, particu- ❍ Moderate (4–7 MET)
larly left heart failure. Dyspnea may vary in severity from ❍ Poor ( 4 MET).
an uncomfortable awareness of breathing to a frightening Patients are at an increased cardiac risk when unable to
sensation of fighting for breath. meet a 4 MET demand during normal daily activity.
There are three forms of dyspnea:
❍ Orthopnea: Lying flat causes a steep rise in left atrial Physical examination
pressure in patients with heart failure resulting in The general appearance of the patient can provide valu-
pulmonary congestion and severe dyspnea. A semi- able information related to his/her physical and emotional
recumbent position helps such patients. state. Pallor, cyanosis, peripheral edema, dyspnea, tremors,
❍ Paroxysmal nocturnal dyspnea: Frank pulmonary Cheyne–Stokes respiration, obesity and anxiety are clues
edema on lying flat awakens the patient from sleep that suggest the presence of cardiovascular disease.
with distressing dyspnea. Symptoms are corrected by
standing upright. Cyanosis It is defined as the blue color of the skin and
❍ Exertional dyspnea: Exercise causes a sharp increase mucous membranes due to the presence of excessive amount
in left atrial pressure resulting in dyspnea. of deoxygenated hemoglobin in these tissues. It is seen in
heart failure, cyanotic congenital cardiac disease and chronic
Fatigue Exertional fatigue is an important symptom of cardiac disorders.
heart failure and is more severe toward the end of the day.
Finger clubbing It is the obliteration of the angle between
Palpitation Awareness of the heartbeat is common during nail base and adjacent skin of the finger. Clubbing is char-
exertion or heightened emotions. However, it may be indic- acterized by the thickening of the nail bed secondary to
ative of an abnormal cardiac rhythm. hypervascularity and opening of the anastomotic channels
Dizziness and syncope Cardiovascular disorders pro- in the nail bed. It is seen in congenital cardiac disease and
duce dizziness and syncope by transient hypotension infective endocarditis.
resulting in abrupt cerebral hypoperfusion. Recovery is usu-
Distended neck veins Systemic fluid retention and inabil-
ally rapid.
ity of heart to pump blood into the lungs may be seen as
Lower limb pain or discomfort A tight or cramping pain distention of the veins in the neck.
in lower limb muscles on exercise may be present.
Swelling of legs due to peripheral edema Bilateral
Edema of legs Bilateral swelling of the legs due to edema swelling of both the legs may be due to chronic heart failure.
is a common feature of chronic heart failure. Edema is detectable by fingertip pressure over the tibial bone
The history should also determine the patient’s functional for about 30 seconds, a pit due to physical displacement of
capacity. excessive tissue fluid is observed.
❍ Functional capacity Blood pressure Determination of the blood pressure pro-
– Functional capacity refers to an individual’s capacity vides a useful clue that will confirm or rule out significant
to perform a spectrum of common daily tasks. cardiovascular disease. It should be recorded on all new
475
patients at the time of initial appointment and at all sub- ❍ Central nervous system (CNS) disorders (head injury,
sequent appointments on all patients with a history of hyper- infection, hemorrhage and brain tumors)
tension, cardiovascular disease, diabetes mellitus, thyroid ❍ Autonomic hyperactivity
disorders, adrenal disease, renal dysfunction and significant ❍ Sleep apnea
use of tobacco, alcohol or coffee. The auscultatory method ❍ Drug-induced (cyclo-oxygenase [COX-1 and COX-2]
of measurement of BP is recommended. In patients older inhibitors, sympathomimetics, steroid hormones, cyclo-
than 50 years, elevated systolic pressure may predict the sporine and tacrolimus).
potential for cardiovascular morbidity and mortality.
Lifestyle risk factors increasing the chances of a person
Pulse rate and rhythm The pulse pressure closely cor- becoming hypertensive are:
relates with systolic pressure and is a reliable cofactor that
❍ Obesity
will provide us with information on cardiovascular disease.
❍ High dietary salt intake
Rate below 60 or above 100 in adults and if associated with
❍ Excess alcohol
symptoms such as sweating, weakness, dyspnea and chest
❍ Smoking
pain should be considered as a risk factor in association
❍ Physical inactivity
with non-cardiac procedures. Further abnormalities in the
❍ Stress/anxiety.
normal rhythm of the pulse should provoke a search for
any underlying cardiac diseases.
Signs and symptoms
A patient with hypertension is usually asymptomatic for
COMMON CARDIOVASCULAR DISORDERS quite a few years. The early symptoms include occipital
AND THEIR DENTAL CONSIDERATIONS headache, vision changes, ringing ears, dizziness, weakness
and nose bleeding. Odontalgia due to hyperemia or conges-
Hypertension tion of dental pulp has also been reported.
Untreated hypertension reduces the life span by 10–20
Hypertension is a disorder characterized by an abnormal years. Even mild hypertension that has not been treated
elevation of arterial pressure, which if sustained and for 7–10 years increases the risk of complications. Sus-
untreated, is associated with a significant increase in mor- tained hypertension results in arterial damage (atheroscle-
bidity and mortality. The systolic or diastolic pressure or rosis) and the onset of these vascular changes in the kidney,
both are elevated in hypertension. cardiovascular system, cerebrovascular system and eyes can
It may be asymptomatic for long periods but ultimately cause complications such as renal failure, coronary insuf-
leads to damage with resultant symptoms in several organs ficiency, myocardial infarction, congestive cardiac failure,
including kidney, heart, brain and eyes. cerebrovascular accident (stroke) and blindness in patients.
It is generally accepted that a sustained systolic blood Malignant hypertension develops in 1% of hypertensives.
pressure of 140 mmHg or more and a sustained diastolic The chief complication is severe ischemic damage to kidney
blood pressure of 90 mmHg or more is abnormal. and renal failure. In the absence of treatment, it can be
fatal within 1 year of diagnosis.
Etiology
Blood pressure is measured by the use of a sphygmo-
Majority of the patients with hypertension have no cause manometer, an instrument that indirectly records the dia-
for their disease. These patients are diagnosed to have stolic and systolic pressure.
primary, idiopathic or essential hypertension. Essential Food, exercise, alcohol and smoking should be avoided
hypertension is seen in elderly, obese and individuals who for 30 minutes before measurement of BP and also the
are tensed and fearful. Genetic factors also play a role. patient should be at rest for at least 5 minutes.
However, a few patients have underlying systemic diseases Faulty BP readings involve using improper size cuffs or
that produce hypertension, which is known as a secondary applying the cuffs too loosely or too tightly.
hypertension.
Classification of blood pressure
Systemic diseases causing secondary hypertension
❍ Renal disease (renal parenchymal disease, renal artery Classification Systolic BP Diastolic BP
(mmHg) (mmHg)
stenosis)
❍ Adrenal abnormalities (primary aldosteronism, Cushing’s Normal 120 80
syndrome, pheochromocytoma) Pre-hypertension 120–139 80–90
❍ Coarctation of aorta Stage 1 hypertension 140–159 90–99
❍ Hyperthyroidism
Stage 2 hypertension 160 100
❍ Pregnancy (eclampsia)
476
Malignant hypertension It is a highly elevated blood Therapeutic goals and pharmacologic strategies for hyper-
pressure associated with organ damage (eyes, brain, lungs tension
and kidneys are affected). The systolic and diastolic blood
pressures are usually more than 240 mmHg and 120 mmHg Therapeutic goal Pharmacologic strategies
respectively. Reduce volume overload Diuretics
Block beta-1 adrenergic Beta-1 adrenergic receptor antagonists
White coat hypertension It is a phenomenon in which receptors ACE inhibitors
patients exhibit elevated blood pressure in a clinical setting Dilate blood vessels Angiotensin II receptor antagonists
but not when recorded by themselves at home or when Reduce sympathetic outflow Calcium channel blocking agents
ambulatory. White coat hypertension is believed to be sec- from the central nervous Alpha-1 adrenergic receptor antagonists
ondary to anxiety, some individuals may experience during system Alpha-2 adrenergic receptor antagonists
their visit to a hospital.
General management Oral side effects of antihypertensive medicines
❍ Acute emotions should be avoided. Drugs Side effects

❍ In patients with secondary hypertension, manage the Diuretics Dry mouth, lichenoid reactions
primary cause.
Beta blockers Dry mouth, lichenoid reactions, taste
❍ Patient should practice the following for a lifetime:
change
– Weight loss—maintaining ideal bodyweight lowers
ACE inhibitors Loss of taste, dry mouth, ulcerations,
systolic BP reading by 5–20 points.
angioedema
– Reduction in salt intake less than 2.4 g/day
Calcium channel blockers Gingival enlargement, dry mouth, altered
– Restricting alcohol and caffeine
taste
– Stop smoking
– Exercise (lowers BP by 5–9 mmHg) Alpha blockers Dry mouth
– Intake of fruits, vegetables and low fat dairy Direct-acting vasodilators Facial flushing possible, increased risk of
products. gingival bleeding and infection
Central-acting agents Dry mouth, taste changes, parotid pain
Specific management Angiotensin II antagonists Dry mouth, angioedema, sinusitis, taste

loss
Antihypertensive therapy
❍ Direct-acting vasodilators: Nitroglycerin and minoxidil
directly relax vascular smooth muscle. Methyldopa and
clonidine act in the CNS to decrease sympathetic ner-
vous system output. Patients with controlled hypertension can receive dental
❍ Angiotensin II receptor blockers: Losartan and care in short appointments.
telmisartan prevent angiotensin II from binding on Anxiety and pain should be avoided since endogenous
smooth muscle sites in arteries, promoting vasodila- epinephrine release in response to pain or fear may induce
tation. dysrhythmias.
❍ Diuretics: Frusemide and hydrochlorothiazide reduce Preoperative reassurance and sedation with 10 mg
blood volume and decrease vascular resistance. temazepam or 5 mg diazepam may be helpful.
❍ Beta blockers: Propranolol and sotolol reduce heart Raising patients from supine position may cause postural
rate, and force of contraction. Selective beta blockers hypertension and loss of consciousness if patient is using
are preferred. Non-selective beta blockers are contra- thiazides, calcium channel blockers.
indicated in patients with asthma, because their beta Aspirating syringe should be used to give local anes-
agonist action is blocked. thesia to avoid intravenous entry of epinephrine.
❍ ACE inhibitors: Captopril, ralopril retard renin–angio- Epinephrine in local anesthesia is not contraindicated
tensin system leading to vasodialatation. unless systolic pressure is over 200 mmHg or diastolic pres-
❍ Calcium channel blockers: Amlodipine, nifedipine, sure is over 115 mmHg.
decrease the calcium influx in smooth and cardiac mus- Epinephrine containing local anesthesia should not be
cles, reduce total peripheral resistance and decrease given in large doses in patients taking non-selective beta
force of contraction. blockers since interaction may induce hypertension and
❍ Alpha blocking agent: Prazosin, terazosin prevent nor- cardiovascular complications.
epinephrine from binding to receptors in arterioles Gingival retraction cords containing epinephrine should
leading to vasodialatation. be avoided.
477
Administration of two to three cartridges of local anes- It may be defined as a temporary inability of the coronary
thesia with epinephrine 1:100,000 will not cause cardio- arteries to supply the myocardium with the sufficient
vascular changes. amount of circulated blood. Atherosclerotic narrowing of
Epinephrine should be used with caution in patients tak- the coronary arteries is an important cause of this imbal-
ing tricyclic antidepressants and diuretics since acute hyper- ance in oxygen supply. It is rarely caused by spasm of the
tensive changes and dysrhythmias, respectively may occur. blood vessels. Initially, the atherosclerosis does not lead to
any symptoms. However, the obstruction becomes large
over a period of decades to cause pain. The pain of angina
ISCHEMIC OR CORONARY HEART DISEASE is described as a sense of choking, tightness, heaviness, or
compression of the chest, sometimes radiating to the left
It is one of the most common medical problem in general arm or jaws. The common precipitating causes include
population. Coronary heart disease is an inflammatory physical exertion and emotions.
disease affecting the large and medium sized arteries of Levine’s sign is characteristic of ischemic chest pain.
heart resulting in inadequate or decreased coronary blood It is seen in angina pectoris and myocardial infarction.
flow. Atherosclerosis and hypertension are the major con- This sign described by Dr Sam Levine who observed many
tributory factors. of his patients suffering from chest pain hold their fist
Atherosclerosis basically refers to the formation of fibro- over the chest.
fatty lesions in the walls of arteries. These may increase in
Variants of angina
size to cause stenosis of the vessels reducing the blood flow
to the heart on exercise. This is known as angina. In the Stable angina Pain only on exertion and relieved by rest
later stages, the atherosclerotic plaque ruptures and exposes within 10 minutes. Stable angina is caused by fixed ath-
the arterial blood to the plaque contents and stimulates eromatous plaque in one or more coronary arteries.
the formation of hemostatic plug. This occlusive thrombus
Unstable angina Angina at rest, angina appearing more
may cause myocardial infarction.
frequently, appearing at lower level of exertion, requiring
It is related to a variety of risk factors. Knowledge about
larger doses for relief or relief taking longer. It is caused
the risk factors will help the dentist to assess the risk of
by dynamic obstruction of coronary artery due to plaque
cardiac problems in patients with no coronary problems.
rupture with superimposed thrombosis and spasms.
Risk factors Prinzmetal’s angina Angina at rest and occurs commonly
at night caused by coronary artery spasm.
Non-modifiable factors
1. Family history: Presence in patient’s parents, increases Management
the risk of occurrence in patients at an early age.
2. Gender: Men between 25 and 65 years of age are more Pain is relieved by nitroglycerin. Nitroglycerin lowers
prone and women at post-menopausal age. peripheral vascular resistance and lowers oxygen demand
of the heart.
Modifiable factors Drugs such as aspirin are used to inhibit platelet function.
1. Total cholesterol of more than 240 mg/dl is associated Beta adrenergic blockers prevent the effects of cardiac
with ischemic heart disease. sympathetic stimulation and reduce myocardial oxygen
Elevated LDL, decreased HDL, increased total to HDL demand by decreasing the heart rate, ventricular systolic
cholesterol and hypertriglyceridemia also contribute. pressure and peripheral arterial pressure.
Statins and fibrates correct lipid abnormalities. Calcium channel blockers prevent calcium from entering
2. Hypertension: Systolic pressure more than 140 mmHg muscle cells (arterial or cardiac muscles) thus preventing
or diastolic pressure more than 90 mmHg increases contraction leading to vasodilatation.
the chances of ischemic heart disease. When angina does not respond to drugs, coronary angio-
3. Smoking: Women who smoke more than 19 cigarettes plasty, percutaneous coronary arterioventral procedures or
per day are likely to have ischemic heart disease. bypass grafts are indicated.
4. Other risk factors are diabetes mellitus, obesity, lack of
exercise, lack of fruits and vegetables in the diet, alco- Dental considerations
hol use, stress, elevated levels of C-reactive proteins. Preoperative nitroglycerin can be given prophylactically
before dental therapy.
Effective local anesthesia is a must. Long-acting local
Angina Pectoris
anesthesia with bupivacaine can be used with vasocon-
It is the most common clinical presentation of ischemic strictor to prolong the effect of local anesthesia. Aspirating
heart disease and is infrequent before the age of 40 years. syringe is a must.
478
Increased heart rate and blood pressure during long 2. Patients within 6 months of an MI (recent MI) are at
appointments indicate need to conclude dental care. the risk of further complications, hence, elective den-
If a person develops angina during dental treatment, the tal care should be deferred. However, the first 6 weeks
procedure should be terminated and the patient should be is more critical, and with the physician’s consent,
seated in semi-inclined position and the patient should be simple emergency dental treatment under LA may be
given nitroglycerin 0.3–0.6 mg sublingually. If pain persists done during the first 6 months.
even after 3 minutes, additional doses (up to 3 mg) every 3. Anxious patients may be given preoperative glyceryl
5 minutes should be given and medical help should be nitrate.
sought. If pain persists, 300 mg of chewable aspirin is given. 4. Effective local anesthesia is important.
Moreover, morphine sulfate (IV) relieves pain and anxiety. 5. Aspirating syringes must be used.
Nausea, bradycardia, hypertension indicating myocar- 6. Use of epinephrine impregnated gingival retraction
dial infarction may occur. cords should be avoided.
Tricyclic antidepressants are contraindicated. Conscious
sedation and general anesthesia should be deferred for Management of myocardial infarction
3 months in patients with recent onset angina or unstable Same as that of angina.
angina. Additionally, patients are on platelet inhibitors. Care
should be taken to prevent significant bleeding.
Local hemostasis should be promoted (procoagulant
MYOCARDIAL INFARCTION
materials such as collagen or topical thrombin should be
used). Local pressure should be applied for a longer dura-
It is a severe form of coronary artery disease. An anginal
tion. Injecting vasoconstrictor containing local anesthesia
pain lasting longer than 30 minutes is considered to be a
directly into surgical site and using sutures is not required.
myocardial infarction. The pain may be accompanied by
nausea and vomiting, tachycardia, grossly irregular pulse,
pallor and difficulty in breathing, sweating, and restlessness.
CONGENITAL HEART DISEASE
About 10% have painless infarctions.
Diagnosis It is a common heart disease among children, present in

1% of live births. Congenital defects involving heart or
The diagnosis of acute myocardial infarction is based on the adjacent vessels may be associated with other congenital
presence of two of the following three criteria: (1) signs and anomalies. These congenital anomalies can be cyanotic or
symptoms compatible with myocardial ischemia, (2) typical acyanotic in nature.
ECG changes—ST segment elevation at the end of the PR
segment, (3) measurement of creatinine kinase (CK) and
myocardial-bound CK (CK-MB). Cyanotic
Elevations in the serum troponin T and troponin 1 levels, Transposition of great vessels, tetralogy of Fallot, Eisen-
which are sensitive markers for myocardial injury, have menger’s syndrome. Cyanotic defects are lethal.
also been used to test for acute myocardial infarction.
Acyanotic
Therapeutic goals and pharmacologic strategies for coronary
heart disease Atrial and ventricular septal defects, patent ductus arterio-
Therapeutic goals Pharmacological strategies
sus, coarctation of aorta, pulmonary stenosis, mitral valve
prolapse, aortic stenosis.
Inhibit progression of Lipid-lowering agents
atherosclerosis HMG-Co-A reductase inhibitors
Causes
Improve circulation in Nitrated calcium channel blockers
coronary arteries ❍ Idiopathic
Reduce workload Beta-1 adrenergic receptor antagonists ❍ Acquired (congenital rubella, maternal drug misuse).
Prevent thrombus formation Antithrombotic agents
Prevent coagulation Anticoagulants
Clinical features
❍ Most striking feature in some congenital heart disease
is cyanosis (more than 5 g reduced hemoglobin per
Dental aspects
deciliter of blood)
1. Physician’s consent is necessary before treating the ❍ Causes severely impaired development and often gross
patient. clubbing of fingers and toes
479
❍ Eventually polycythemia develops ❍ All rheumatic heart lesions are at the risk of infective
❍ Cyanosis due to right to left shunt leading to chronic endocarditis and hence precautions and treatment sim-
hypoxia ilar to infective endocarditis should be carried out
❍ Shunt from left to right leads to pulmonary hyperten- ❍ Local anesthesia can be given
sion, direction changes later leading to cyanosis ❍ General anesthesia should be avoided.
❍ Patients with congestive heart disease liable to infec-
tive endocarditis and others are pulmonary edema,
polycythemia, bleeding tendency, growth retardation, INFECTIVE (BACTERIAL) ENDOCARDITIS
fatigue and brain abscess.
Infective endocarditis (IE) is a rare, potentially lethal infec-
Dental considerations tion, predominantly affecting heart valves. The endocardium
❍ Antimicrobial prophylaxis can also be affected. It results from bacteremia originating
❍ Bleeding tendencies due to platelet dysfunction and from any site and representing almost any species. It is
excessive fibrinolytic activity caused mainly by bacteria as well as fungi.
❍ Dental bacteria may cause cerebral abscess Bacterial endocarditis can be defined as an infection that
❍ Aspiration during local anesthetic procedure is a must affects the endocardium in valvular, mural and septal defects,
due to epinephrine in local anesthesia as well as in arteriovenous and arterioarterial shortcircuits.
❍ Gingival retraction cord containing epinephrine should It usually occurs in older individuals, peak prevalence
be avoided. is 6th or 7th decade. It is less frequent in the young, except
in intravenous drug users. It occurs predominantly in males.
Oral manifestations associated with congenital Most patients have pre-existing cardiac disease. The mitral
heart diseases valve is more frequently affected followed by aortic valve
and tricuspid valve. Sterile vegetations, i.e. comprising of
❍ Delayed eruption of both the dentitions platelets and fibrins accumulate over the damaged valves
❍ Greater frequency of positional anomalies and these get readily infected during bacteremias resulting
❍ Enamel hypoplasia in infective endocarditis.
❍ Teeth have bluish white skimmed milk appearance and
gross vasodilatation in pulp Main groups affected by Approximate percentage of all
❍ Greater incidence of caries and periodontal activity. infective endocarditis cases of infective endocarditis
No obvious cardiac valve disease 40%
Chronic rheumatic heart disease 30%
RHEUMATIC FEVER Congenital heart disease 10%
Prosthetic cardiac valves 10%
Rheumatic fever is the most common cause of cardiac
Intravenous drug abuse 10%
valve disorders. The mitral valve is affected more fre-
quently. It results from an altered immunologic response to
Oral microorganisms could play an important role in the
a group A beta hemolytic streptococcal pharyngitis, lead-
pathogenesis of infective endocarditis. Some oral micro-
ing to formation of Aschoffs nodules in the myocardium
organisms pass into the blood stream and colonize areas of
which develops 1–3 weeks after the streptococcal infection.
valvular endothelium on a previous sterile incipient vege-
Though, only 3% of cases of beta hemolytic pharyngitis
tation. Staphylococcus aureus usually produces an acute
resulting in rheumatic disease, there is no way to forecast
infection, whereas Streptococcus viridans, enterococci, cer-
which individuals will have rheumatic heart disease (RHD).
tain gram-positive and gram-negative bacteria and fungi
Common manifestations include new onset murmur,
may produce subacute infection. Dental treatment precedes
carditis, polyarthritis, chorea, erythema marginatum, fever
infective endocarditis only in 5–10% of cases though a
and subcutaneous nodules (modified Jones criteria). The
number of oral manipulations can cause bacteremia.
beta streptococcal infection is confirmed by increased
It is estimated that the prevalence of bacteremia follow-
serum antistreptolysin O (ASO) antibody titer or positive
ing scaling and root planing is about (0–25%), with single
throat culture. RHD resulting in valvular injury is con-
tooth extraction (25–50%) and almost 50–80% with mul-
firmed by echocardiography for any patient with a history
tiple extractions.
of rheumatic fever, it is imperative to determine whether
The prevalence of bacteremia is about 25–50% with
the infection resulted in RHD.
periodontal surgery.
It has been reported that some cases of infective endo-
carditis have been associated with oral infections in the
❍ Consult with the physician absence of dental manipulations and hence a synergistic
480
effect between the presence of periodontal or periapical Patients undergoing chemotherapy are prone to develop
infections and dental manipulations could favor the devel- infections as the chemotherapeutic agents suppress the
opment of infective endocarditis. inherent immune system. Invasive dental procedures such
Moreover, a number of studies have reported that as extraction of teeth, subgingival scaling and periodontal
brushing teeth, chewing gum or eating may provoke bac- surgeries that might cause significant bleeding warrant
teremia and that the prevalence of IE increases when these antibiotic prophylaxis in these patients.
acts are performed by patients with intraoral infections. Patients inflicted with HIV/AIDS generally do not
Because of the high morbidity and mortality associated require antibiotic cover; nevertheless it is always wise
with IE, antibiotic prophylaxis has been advised. Though, to perform dental procedures such as extraction of teeth
antibiotic therapy has not proven to prevent endocarditis and periodontal surgeries under antibiotic cover as it
and it also carries with a risk of adverse reactions (ana- might minimize the risk of the patient acquiring a super-
phylaxis), it is still advised prophylactically for certain infection.
treatment procedures. Patients with uncontrolled insulin-dependent diabetes
mellitus (IDDM) are vulnerable to infections. Invasive den-
tal procedures that involve significant amount of bleeding
Clinical Conditions that Warrant Use of
may require antibiotic cover.
Antibiotic Prophylaxis
Cardiac conditions
Treatment Needing Antimicrobial Prophylaxis in
❍ Previous history of bacterial endocarditis Patients at Risk of Infective Endocarditis
❍ Prosthetic cardiac valves
❍ Complex cyanotic congenital heart diseases ❍ Extractions
❍ Acquired valvular dysfunction ❍ Subgingival procedures (probing, cord placement or
❍ Surgically constructed systemic pulmonary shunts scaling)
❍ Hypertrophic cardiomyopathy ❍ Oral/periodontal/implant surgery or raising mucogin-
❍ Mitral valve prolapse with regurgitation. gival flaps
❍ Endodontics beyond root apex
Neurosurgical shunts ❍ Sialography
❍ Intra-ligamental local anesthesia
Shunts are placed in patients with hydrocephaly to help
❍ Rubber dam, matrix or wedge placement.
in the drainage of cerebrospinal fluid. The ventriculoatrial
shunt allows drainage of the CSF from the lateral ventricles
to the venous circulation. The ventriculo-peritoneal shunt Procedures for which antimicrobial prophylaxis is
helps drain the CSF into the abdominal cavity. not required
❍ Dental radiography
Indwelling catheters and stents
❍ Endodontics not beyond apex
Antibiotic prophylaxis is indicated only in instances where ❍ Exfoliation of primary teeth
the catheters are on the right side of the heart. Only the first ❍ Impression taking
2 weeks are critical and indicated for antibiotic prophylaxis ❍ Non-surgical procedures that do not induce bleeding
when stents are placed in cardiac patients. The risks of devel- ❍ Abscess incision and drainage
oping a superinfection are very minimal after the first few ❍ Suture removal
weeks as an epithelial layer develops over these stents. ❍ Orthodontic band removal.
Patients with renal diseases undergoing Prophylactic antibiotic regimen

hemodialysis
Patients receiving peritoneal dialysis do not require antibi- Patient category Oral medications
otic prophylaxis. However, patients who have an arteriove- Adults, not allergic to penicillin 2.0 g amoxicillin 1 hour before
nous shunt (made up of autogenous tissue or a silastic tube) procedure
implanted for dialysis require antibiotic coverage during Adults, penicillin allergic 600 mg clindamycin 1 hour before
dental procedures, as these shunts are vulnerable to infection. procedure or 2.0 g cephalexin
1 hour before procedure or 500 mg
Patients with compromised immune status azithromycin or clarithromycin 1 hour
before procedure
These patients are more prone to develop an overwhelm-
ing septicemia from a relatively harmless transient bacte- Children, not allergic to 50 mg/kg amoxicillin 1 hour before
penicillin procedure
remia because of their compromised immune status.
481
Stage D: Patients with end stage disease requiring special-

Patient category Non-oral medications
ized treatment such as mechanical circulatory sup-
Adults, not allergic to 2.0 g ampicillin IM or IV within 30 minutes port, continuous ionotropic infusion or cardiac
penicillin before procedure transplantation.
Adults, penicillin allergic 600 mg clindamycin IV within 30 minutes
2. The New York Heart Association
before procedure or 1.0 g cefazolin IM or
Class one: Asymptomatic patients
IV within 30 minutes before procedure
Class two: Patient symptomatic with moderate exertion
Children, not allergic to 50 mg/kg ampicillin IM or IV within
Class three: Patient symptomatic with mild exertion
penicillin 30 minutes before procedure
Class four: Patient symptomatic at rest.
Chlorhexidine hydrochloride and povidone–iodine mouth- Clinical manifestations

rinses may reduce the incidence and magnitude of bacte- Left heart failure manifests as tachycardia, fatigue on
remia before dental treatment. exertion, dyspnea on mild exercise, intolerance to cold,
Moreover, patients should be encouraged to maintain paroxysmal nocturnal dyspnea and nocturnal cough.
meticulous oral hygiene. Right heart failure is characterized by fatigue, jugular
It has been suggested by some authors that in patients venous distention, fullness in the abdomen, with an enlarged
with poor oral health, antibiotic coverage should be given liver and tenderness in the upper right quadrant. In advance
for all dental procedures. cases ascites and pitting edema of lower extremities is seen.
Management
HEART FAILURE Reduction in physical activities, stress reduction, fluid restric-
tion, dietary sodium restriction, weight loss, subcutaneous
Heart failure is a clinical syndrome defined as chronic heparin administration, supplemental portal oxygen, med-
inadequate contraction of the heart muscle, resulting in ications prescribed include diuretics, cardiac glycosides,
insufficient cardiac output. It is basically an inability of the vasodilators, ACE inhibitors and sympathomimetic drugs.
heart to pump blood at a rate required by the body tissues.
It occurs frequently in the elderly population. It is a mani- Dental considerations
festation of one or more underlying conditions including:
❍ Physician’s consent is a must.
❍ Systemic or pulmonary hypertension ❍ Patient should avoid heavy meals just before their den-
❍ Myocardial infarction tal appointment since digitalis may cause nausea and
❍ Cardiomyopathy vomiting if the doses are high.
❍ Congenital heart disease ❍ The dentist should avoid stimulating gag reflex. Rubber
❍ Rheumatic fever dam should be used cautiously.
❍ Cardiac dysrhythmias ❍ Patient should not be in supine position because dys-
❍ Pericardial disease and other infections. pnea is worsened.
❍ Dental treatment may precipitate dysrhythmias and
Heart failure reflects impairment of either the left or right angina in uncontrolled patients.
ventricle. Left ventricular failure commonly develops in ❍ Anxiety must be minimized and patient’s pain control
patients with coronary artery disease, hypertension and should be effective.
congenital heart disease. Right ventricular failure is mostly ❍ Patients should be treated in late morning, because
caused by left ventricular failure which increases pulmo- endogenous epinephrine peaks during early morning
nary venous pressure and leads to pulmonary arterial and cardiac complications may arise.
hypertension. Heart failure leads to hypoperfused tissues ❍ Aspirating syringe should be used since epinephrine
and congested organs. can theoretically increase hypertension.
❍ Bupivacaine is cardiotoxic.
❍ Epinephrine in local anesthesia should be avoided in
Classifications of Heart Failure
patients taking beta blockers, since hypertension may
1. American Heart Association be induced.
Stage A: Patients at risk of failure without any structural ❍ For patients in whom significant bleeding is expected,
disorder monitor protrombin time.
Stage B: Patients with a structural disorder of heart, with- ❍ During the course of treatment, vital signs should be
out symptoms of heart failure monitored.
Stage C: Patients with present or past symptoms of heart ❍ Medications such as diuretics may cause orthostatic
failure and structural disorder hypotension.
482
❍ NSAIDs other than aspirin should be avoided in patients that supports the association between periodontal infec-
taking ACE inhibitors, since there is a risk of renal damage. tions, atherosclerosis and vascular disease. They further
recommend that irrespective of whether there is an asso-
Special considerations ciation between periodontal diseases and cardiovascular
Appointment timing Most sudden cardiac arrests may lesions or not, periodontal treatment must be recommended
come during peak endogenous epinephrine levels (08:00– on the basis of the value of its benefits for the oral health of
11:00 hours) and so appointments are best for late morning patients, recognizing that patients are not healthy without
or early afternoon. good oral health.
Preventive strategies Dental management plans for Lichenoid stomatitis Cardiovascular drugs such as diuret-
patients with cardiovascular diseases should include appro- ics, beta-1 blockers, ACE inhibitors may cause development
priate preventive strategies. of lichenoid lesions. Lichenoid lesions are indistinguishable
from oral lichen planus. The diagnosis is confirmed when
Toothbrush The use of electromechanical brushes have condition resolves after the offending drug is discontinued.
been shown to be more effective than conventional brushes
in reducing plaque and gingivitis and hence should be rec- Xerostomia Cardiovascular drugs with xerostomic side
ommended in all patients with cardiovascular disease. effects include diuretics, beta-1 blockers and centrally act-
ing sympathetic agonists. Xerostomia is usually caused due
Topical rinses and fluorides The use of topical agents,
to the drug’s parasympatholytic or antimuscarinic effects.
such as chlorhexidine gluconate is useful to combat gingi-
Xerostomia can lead to complications such as high inci-
vitis and other periodontal pathosis that result from plaque
dence in dental caries. Moreover, it can contribute to dif-
accumulation. For patients with xerostomia and a high
ficulties in mastication, swallowing, speech. The dryness
incidence of dental caries, preventive modalities such as
of the oral mucosa if persistent can lead to atrophy and
dietary analysis and counseling, and prophylaxis combined
susceptibility to candidiasis and other superinfections.
with home fluoride use should be implemented. A topical
fluoride, 1.1% sodium fluoride in the form of a brush-on Gingival hyperplasia Calcium channel blockers such as
gel, may be preferred to a topical solution. nifedipine can cause gingival enlargement primarily affect-
Sialagogues Xerostomic patients, in whom the salivary ing the labial or facial interdental papilla and it is firm and
glands respond to stimulation benefit from dietary measures painless. However, it is usually associated with erythema
such as carrots, celery, or from chewing xylitol containing and edema resulting in pain, bleeding, and difficulty in
gums. Drugs such as pilocarpine hydrochloride 2.5–5 mg in mastication.
2–6 increments per day and cevimeline hydrochloride can be Patients on antithrombotic agents
used to treat drug-induced xerostomia. However, in patients
with no residual salivary gland function, salivary substitutes, An increasing number of cardiovascular patients are pre-
oral moisturizer, and artificial saliva provide some relief. scribed antithrombotic, anticoagulant, and thrombolytic
agents and hence special precautions need to be taken in
Local hemostatic agents They are used for surface dress-
such patients.
ing for wounds and over extraction sockets. They arrest
Aspirin is one of the commonly prescribed antithrombotic
bleeding by creating a mechanical matrix which facilitates
agents. It irreversibly acetylates cyclo-oxygenase resulting
blood clotting when the absorbable hemostatic agent is
in inhibition of thromboxane A2 mediated platelet aggrega-
applied directly to the hemorrhagic site. Examples include
tion. However, it is relatively a weak antithrombotic agent,
absorbable gelatin sponge, microfibrillar collagen hemo-
since the action of other mediators of platelet aggregation
stat and oxidized regenerated cellulose.
are not inhibited, hence the effect of aspirin therapy on
Adverse drug events Dentists must have an awareness intra- and post-operative surgical and periodontal proce-
of the potential for adverse drug events since patients with dures are minimal.
cardiovascular diseases are frequently treated with multi- If the patient is also taking oral anticoagulant, signifi-
ple drugs. Hence, a rational approach should be developed cant synergism may mandate modifications of drug regi-
toward the use of pharmacotherapeutic agents in the man- men. Measurement of patients bleeding time can be helpful
agement of odontogenic problems. to determine the degree of antithrombotic effect and its
impact on invasive dental procedures.
Oral Disease Aspects
Considerations for patients on anticoagulant agents
Periodontal disease
For any patient taking anticoagulant the dentist and phy-
Demmer and Desvarieux (2006) in their article titled sician must assess the risk for altered bleeding after an inva-
‘Periodontal Infections and Cardiovascular Disease: The sive dental procedure. Assessment includes magnitude of
Heart of the Matter’ suggest that there is still some evidence invasive procedure, patient’s bleeding history, patient’s
483
bleeding profile which includes prothrombin time (PT), paramount of epinephrine in LA. However, vasoconstrictors
tial thromboplastin time (PTT), and international normal- in LA may be contraindicated in patients with severe CVS
ized ratio (INR). disorders such as unstable angina, recent MI or bypass
Oral anticoagulants are commonly prescribed for arte- surgeries, uncontrolled dysrhythmias, severe hypertension
rial or venous thromboembolism. This therapy is usually and severe congestive heart failure.
monitored by prothrombin time which is converted to INR. Intraligamentary injection of LA with vasoconstrictor
For most clinical indications, a moderate–intensity antico- is generally contraindicated in patients with significant
agulant effect with a target INR of 2.0–3.0 and PT of 1.5– CVS disease.
2.5 is appropriate.
Warfarin is the commonly prescribed oral anticoagulant. Pacemakers
Most patients can undergo minor oral surgical procedures It is a small implanted electronic device that stimulates the
such as extraction and alveolectomies without alteration of heart to beat and pace the heart rate when it is too slow
their warfarin regimen. However, to prevent serious bleeding (bradycardia). Pacemakers have pulse generators and elec-
the preoperative assessment of the patient’s level of anti- trode leads and these are powered by lithium batteries.
coagulation is necessary. Warfarin has plasma half-life of Modern pacemakers are bipolar, implanted transvenously
36–42 hours, hence any change in dosage requires at least in the subclavian or cephalic vein and typically located in
2 days to be reflected in INR value. Once an acceptable range right ventricle or on the chest wall, either with the pectoral
is achieved local anesthesia (LA) can be administered with muscle or underneath the skin or in abdominal wall.
caution to minimize hematoma formation and using metic-
ulous local measures such as minimal trauma, application Dental apparatus/instruments with no known
of gelfoam and placement of sutures to ensure hemostasis. effect on cardiac pacemakers
❍ Electric toothbrushes
Heparin therapy
❍ Electronic apex locators
For a patient on heparin therapy requiring an oral surgical ❍ Piezoelectric ultrasonic scalers.
procedure, the drug should be discontinued approximately
4 hours before the procedure. Surgery is performed using Dental apparatus/instruments with likely
local anesthesia, atraumatic surgical technique, application effects on cardiac pacemakers
of local hemostatic agents and careful suturing. Postop- ❍ Diathermy units
eratively heparin therapy may be re-instituted the same ❍ Electronic dental analgesia units
day if there is no active bleeding. ❍ Electrosurgical units
❍ Ferromagnetic seaters
Local anesthesia ❍ Lithotripsy units
The use of local anesthetic agents with vasoconstrictors in ❍ MRI (magnetic resonance imaging) units
patients with cardiovascular disease is controversial. The two ❍ TENS (transcutaneous electric nerve stimulation) units
most commonly used vasoconstrictors are epinephrine and ❍ Ultrasonic instrument baths.
levonordefrin. Patients receiving LA without vasoconstrictor
Interference with pacemakers/defibrillators
have impaired pain control compared to those receiving LA
with epinephrine. Hence, patients with cardiovascular dis- High frequency external electromagnetic radiation can
ease may be at greater risk of experiencing massive endog- interfere with pacemaker sensing function.
enous epinephrine release secondary to poor LA than they Pacemakers can be disrupted by ionizing radiation,
are from the small amount of vasoconstrictor used in LA. ultrasonic and electromagnetic interference from a wide
Some studies have found that infiltration anesthesia range of sources.
containing 3.6 ml of lignocaine with 1–80,000 epinephrine Digital phones, TV transmitters may pose interference,
can be given safely in CVS patients who have an exercise but the risk is small and only used in close proximity to
capacity of more than 4 MET. Most studies have shown no the pacemaker.
significant changes in BP or heart rate in patients with mild Electrical appliances like remote controls, electric blan-
to moderate cardiovascular system (CVS) disease after den- kets, heating pads, shavers, sewing machines and microwave
tal injection of 1.8–5.4 ml of 2% of lignocaine with 1–100,000 ovens are safe.
epinephrine. Hence, it is recommended that patients with
mild to moderate CVS disease receive the smallest amount
of LA needed to provide anesthesia with an aspirating HEMATOLOGICAL DISORDERS
syringe. Moreover, use of conscious sedation to decrease
stress to minimize endogenous release of epinephrine may Hematological diseases include abnormalities involving
help in ensuring hemodynamic stability than avoiding small RBCs, WBCs and platelets. As platelet abnormalities lead
484
to defective coagulation mechanism. They will be discussed POLYCYTHEMIA

in the section pertaining to bleeding and clotting disorders.
RBCs and WBCs perform many of the vital functions of Polycythemia is an abnormal increase in the erythrocyte
the body. Their qualitative or quantitative defects can lead count. Polycythemia is of three types: (1) primary prolif-
to derailment of internal homeostasis. The oral cavity is erative polycythemia (polycythemia rubra vera), (2) sec-
equally affected and demonstrates a plethora of manifes- ondary polycythemia, and (3) apparent polycythemia.
tations. These are sometimes specific for a particular disease Polycythemia rubra vera (PRV) is an idiopathic myelo-
and many of the times, non-specific. The oral manifesta- proliferative disorder seen after 5th decade of life. PRV is
tions of the hematological diseases have to be identified, not only characterized by proliferation of erythrocytes, but
underlying systemic cause treated and consideration should also granulocytes and platelets. While secondary polycy-
be given for a patient, while undertaking a particular dental themia results from increased erythropoietin concentration,
procedure. apparent polycythemia results from decrease in plasma
Hematological diseases can be broadly classified as fluids rather than any increase in RBC concentration. RBC
qualitative and quantitative defects. levels may reach up to 6–12 million cells/mm3 leading to
increased blood viscosity and thrombosis. Cyanosis of the
Qualitative disorders face and extremities (due to deoxygenated blood), and
hemorrhagic tendency may be seen in the later stages of
Cells affected Disorder the disease.
WBCs Chediak–Higashi syndrome
Oral manifestations
Enzyme deficiencies
e.g. G-6-P dehydrogenase deficiency, pyruvate Polycythemia rubra vera will exhibit oral manifestations
kinase deficiency such as petechiae, purpura, spontaneous bleeding of the
Hemoglobinopathies gingiva owing to hemorrhagic tendency and purple red dis-
RBCs
e.g. Sickle cell anemia, thalassemia coloration on tongue, mucosa owing to cyanosis. Leukemic
Abnormal shape manifestations may also be seen if PRV progresses to acute
e.g. Hereditary spherocytosis, hereditary myeloid leukemia, due to the proliferation of leukocytes.
elliptocytosis Secondary polycythemia also exhibits petechiae, ecchy-
mosis and purple red discoloration of tongue and mucosa.
Apparent polycythemia, which does not affect the quantity
Quantitative defects of RBC, does not exhibit any appreciable oral changes.
Cells affected Disorder
Increase in number
e.g. Leukocytosis, leukemia, lymphoma
Bleeding tendency during dental treatment procedures war-
rants adequate hemostasis measures. Cytotoxic chemotherapy
WBCs Decrease in number
should be administered prior to dental treatment procedures
e.g. Granulocytopenia, agranulocytosis,
cyclic neutropenia
if hemoglobin levels are not controlled.
Increase in number Treatment

e.g. Polycythemia
Decrease in number The type of polycythemia should be diagnosed first and
RBCs treated accordingly. Phlebotomy and chemotherapeutic
e.g. Anemia (sickle cell anemia, thalassemia and
anemia due to deficiency of vitamin B12, folic acid agents like busulfan and chlorambucil are the common
and iron) treatment options.
ANEMIA
RED BLOOD CELL DISORDERS
Anemia is defined as a reduction in the oxygen carrying
Red blood cell precursors, the pluripotential stem cells, capacity of the blood. It is not a disease but rather a symp-
give rise to erythroid progenitor cells on stimulation by tom complex due to either reduction in number of circu-
various growth factors. These progenitor cells are further lating RBCs or due to an abnormality in the hemoglobin
stimulated by erythropoietin, a hormone produced by kid- contained within RBCs. RBCs with a normal range of
ney, leading to their differentiation and maturation into 5–6 million cells/mm3 may dwindle due to loss of blood or
erythrocytes. due to decreased production or increased destruction.
485
Anemias can be classified in many ways. They can be ❍ Genitourinary causes like menstruation, malignancies,
classified based on their etiology or size of the RBC with etc.
hemoglobin concentration in two. ❍ Trauma/surgery.
Blood loss from various sources is the most common cause
Classification based on etiology of iron deficiency. Menstruation is the most likely cause in
❍ Blood loss anemia: Iron deficiency anemia women between the age of 15 and 45 years. In adult and
❍ Anemia due to decreased production: Aplastic anemia, postmenopausal women, chronic GI tract losses should be
anemia due to folic acid and vitamin B12 deficiency the first consideration. In addition to clinical manifestations
❍ Anemia due to increased destruction (hemolytic anemia): common to all anemias such as fatiguability, pallor and dys-
Thalassemia, sickle cell anemia, glucose-6-phosphate pnea, iron deficiency anemia patients exhibit spoon-shaped,
dehydrogenase (G6PD) deficiency. cracked and split nails (koilonychia).
Oral manifestations
Classification based on size and hemoglobin
concentration Iron deficiency can contribute to impaired cellular immu-
nity, deficient bactericidal activity of polymorphonuclear
❍ Microcytic hypochromic anemia: Iron deficiency anemia, leukocytes, inadequate antibody response, and epithelial
thalassemia. abnormality that may cause high prevalence of oral can-
❍ Macrocytic normochromic anemia: Anemia due to folic didiasis, angular cheilitis, and atrophic glossitis in patients.
acid and vitamin B12 deficiency. Characteristic oral manifestations of tissue iron depletion
❍ Normocytic normochromic anemia: Sickle cell anemia. are glossitis and glossodynia, angular cheilitis, papillary
Patients suffering with any type of anemia will have atrophy of tongue (Figure 1), paresthesia, pallor and dys-
depleted oxygen carrying capacity manifesting as pallor, phagia due to esophageal strictures.
weakness and difficulty in breathing (dyspnea). Hemo-
globin levels below 13.5 g/dl in adult males and less than Dental considerations
11.5 g/dl in adult females is considered anemic. Anemias Increased bleeding tendency might be a problem while
due to increased destruction (hemolytic anemia) are further treating anemic patients with hemoglobin levels less than
classified based on the etiology as following: 10 g/dl. This tendency is exhibited due to altered rheologic
❍ Extracorpuscular factors causing hemolysis, e.g. auto- interactions between cells when hemoglobin level falls
immunity, infections, liver disease and Rh factor below a critical level. General anesthesia is also contrain-
incompatibility. dicated in severe anemia, as oxygen carrying capacity is
❍ Intracorpuscular factors causing hemolysis, e.g. here- severely impaired. Therfore, patients presenting with typi-
ditary spherocytosis, G6PD deficiency, sickle cell anemia, cal features of anemia should be thoroughly investigated
thalassemia and paroxysmal nocturnal hemoglobinuria. and referred to a physician for further investigations and
treatment.
Iron Deficiency Anemia Diagnosis
Iron is an essential element in which it participates in Following are the list of investigations for iron deficiency
hemoglobin synthesis, electron transport for cellular respi- anemia:
ration, DNA synthesis and other vital enzymatic reactions. ❍ Blood count: Decreased RBC and hemoglobin
Iron deficiency anemia is one of the most common diag- ❍ Peripheral smear: Microcytic hypochromic anemia
noses in the world, affecting both pediatric and adult pop- ❍ Serum iron: Decreased
ulation with a variety of etiologies. ❍ Serum iron binding capacity: Increased
❍ Mean corpuscular volume: Decreased.
Causes
Hypochromic microcytic anemia alone is also seen in
Inadequate ingestion/increased requirements Infants, other conditions like thalassemia and sideroblastic anemia.
children and in pregnancy. Other mentioned investigations should be done to effec-
Decreased absorption or utilization Partial gastrectomy, tively rule out these latter two conditions.
malabsorption syndrome.
Treatment
Blood loss
Once the diagnosis of iron deficiency has been made, the
❍ Gastrointestinal (GI) tract causes like peptic ulcer cause of iron deficiency should be sought and treated. The
❍ Respiratory causes like infections, malignancies, etc. best treatment of iron deficiency is an iron salt by mouth
486
all these is the pernicious anemia, which is due to atrophy

Figure 1
of gastric mucosa resulting in a lack of intrinsic factor
secretion. The intrinsic factor binds to vitamin B12 and is
necessary for its absorption. Autoimmunity was consid-
ered as an etiological factor for destruction of gastric pari-
etal cells or the intrinsic factor. Pernicious anemia is
commonly seen in adult life with patients being suscepti-
ble to gastric carcinoma, rheumatoid arthritis and neuro-
muscular abnormalities.
Folic acid deficiency causes severe anemia but does not
cause neurological abnormalities seen in pernicious ane-
mia. It is prevalent in alcoholics, drug users and in patients
whose diet is devoid of leafy vegetables. Anticancer drugs
such as methotrexate are known to cause folate deficiency
by interfering with DNA synthesis. Anemia due to defi-
ciency of vitamin B12 and folic acid is also called megalo-
blastic anemia because of the presence of large RBCs with
small immature nuclei.
Bald tongue and angular cheilitis in a patient with iron
deficiency anemia. Courtesy: Dr Sarat Gummadapu
Oral manifestations
Patients with pernicious anemia exhibit glossitis, sore or
(ferrous sulfate 200 mg twice daily). Oral iron may need burning tongue, angular cheilitis, papillary atrophy, recur-
to be given for 3 months or more to replenish marrow rent oral ulcerations and taste abnormalities. The glossitis
iron stores. can either be atrophic or Moeller’s, where a pattern of red
lines may be seen without depapillation. Atrophic tongue
occurs not only in anemias such as vitamin B12 deficiency,
Paterson–Kelly Syndrome (Plummer–Vinson folic acid and iron deficiency, but also due to diabetes,
Syndrome) xerostomia and candidiasis. Several hypotheses regarding
the pathogenic role of nutrient deficiencies in atrophic
Paterson–Kelly syndrome presents as a classic triad of
glossitis have been proposed. Because cells of the tongue
dysphagia, iron deficiency anemia and esophageal webs.
papilla have a high rate of turnover, deficiencies in micro-
Dysphagia is due to muscular degeneration in the esophagus
nutrients needed for cell proliferation or cell membrane
leading to esophageal strictures or webs. The patients are
stability may lead to depapillation. Nutrient deficiencies
commonly females in the age range of 4th to 7th decade
may also contribute to oral pathology by altering the pat-
and complain of ‘spasm in the throat’ or ‘food sticking in
tern of microbial flora. Burning mouth sensation can be also
the throat’.
encountered due to neuropathy or candidosis, which may
Symptoms resulting from anemia (weakness, pallor,
be precipitated by anemia. Neuropathy can be explained by
fatigue, tachycardia) may dominate the clinical picture.
the fact that defective myelin synthesis is seen in patients
Additional features are glossitis, angular cheilitis and koil-
with vitamin B12 deficiency.
onychia. The most important etiologic factor is iron defi-
Oral manifestations of folic acid deficiency include
ciency. Paterson–Kelly syndrome can be treated effectively
angular cheilitis, aphthous stomatitis and pharyngitis.
with iron supplementation. Since this syndrome is associ-
ated with an increased risk of squamous cell carcinoma of Diagnosis
the pharynx and esophagus, patients should be monitored
closely. Diagnosis of pernicious anemia requires the demonstration
that megaloblastic hemopoiesis is present, vitamin B12 defi-
ciency is present and intrinsic factor is missing. Diagnostic
Anemia due to Vitamin B12 and Folic Acid tests performed and would be indicative of either vitamin
Deficiency B12 deficiency or folic acid deficiency are:
Vitamin B12 (cyanocobalamin) and folic acid are needed ❍ Blood count: Decreased RBCs and hemoglobin
for the maturation of RBCs in bone marrow. A deficiency ❍ Peripheral smear: Macrocytic normochromic anemia
in the daily intake or absorption of these can result in ane- ❍ Mean corpuscular volume: Increased
mia. Vitamin B12 deficiency can occur due to pernicious ❍ Serum B12 assay: Decreased (in vitamin B12 deficiency)
anemia, gastrectomy, tropical sprue, alcoholism, sclero- ❍ Serum folic acid assay: Decreased (in folic acid
derma and drugs such as colchicine. The most common of deficiency)
487
❍ Schilling’s test: Positive (in vitamin B12 deficiency). Oral manifestations

❍ Antibodies to parietal cells or intrinsic factor: Present
Oral manifestations like gingival bleeding, pallor, pete-
(in pernicious anemia).
chiae, ecchymosis; neutropenic ulcers and delayed healing
Treatment are observed in patients with aplastic anemia (effects of ane-
mia, thrombocytopenia and neutropenia). Petechial hem-
Since oral changes would appear to precede many of the orrhages were the most common intraoral finding in
systemic indicators of vitamin B12 deficiency, it is hoped patients with aplastic anemia. These petechiae might be due
that the recognition of these will lead to early diagnosis to minor trauma from normal deglutition and mastication
and institution of therapy. Regardless of the etiology of rather than due to thrombocytopenia. Rapidly progressing
vitamin B12 deficiency, high dose oral supplementation severe periodontitis, which is a feature of several quantita-
(1,000–2,000 mcg daily for 2 weeks), followed by 1,000 mcg tive and qualitative neutrophil defects like cyclic neutro-
daily for maintenance is currently recommended. Histori- penia, agranulocytosis and leukocyte adhesion deficiency
cally pernicious anemia was treated with intramuscular (LAD), is also evident in aplastic anemia. Bacterial sepsis
vitamin B12 supplementation. However, several studies have and fungal infections represent the most frequent cause of
demonstrated that high doses of oral vitamin B12 are just death in this condition.
as effective as, and are better tolerated than intramuscular
cyanocobalamin in patients with vitamin B12 malabsorption. Dental considerations
Management for folic acid deficiency consists of adminis-
tration of oral folic acid (5 mg/day), which is given for Thorough oral examination should be carried out in a
a period of 4 months. The differentiation of vitamin B12 patient with aplastic anemia as bleeding tendency and infec-
deficiency and folic acid deficiency is crucial as folic acid tions pose a serious problem. Local hemostatic measures
supplements may correct the anemia but will not stop the such as pressure pack application and systemic antifibri-
neurological manifestations. nolytic agents such as aminocaproic acid and tranexamic
acid should be considered. Tranexamic acid is given in
a dosage of 20 mg/kg body weight 4 times a day starting
Aplastic Anemia 24 hours before oral procedures and continuing for 3–4 days
Aplastic anemia is a rare, potentially life-threatening fail- afterwards. Chlorhexidine mouthrinses will reduce the micro-
ure of hemopoiesis characterized by pancytopenia and bone bial load in the oral cavity. Antibiotic prophylaxis, typically
marrow aplasia. Although most cases are acquired (drugs, with amoxicillin or clindamycin, and platelet transfusion
viruses, chemicals, toxins and radiation), some are inher- of thrombocytopenic patients, when necessary, should be
ited. The pathophysiology of acquired aplastic anemia is adequate to prevent serious sequelae for all dental proce-
immune mediated in most cases; autoreactive lympho- dures. However, intramuscular injections and nerve block
cytes mediate the destruction of hemopoietic stem cells. anesthesias are to be avoided because of risk of thrombo-
Fanconi’s anemia is an inherited aplastic anemia that cytopenia and bleeding tendency.
manifests in early childhood. The term ‘anemia’ in aplastic
anemia is a misnomer, since all the three cellular elements Management
of the bone marrow are often involved (pancytopenia), i.e. Cause should be identified and removed. Patients with
granulocytes, erythrocytes and platelets. Clinical manifesta- asymptomatic cytopenias probably need no treatment.
tions are proportional to the peripheral blood cytopenias Treatment for patients with severe cytopenias includes bone
and include dyspnea, fatigue and pallor (effects of anemia), marrow transplantation, immunosuppressive therapy and
petechiae and easy bruising (effects of thrombocytopenia) high dose cyclophosphamide without transplantation of
and susceptibility to infections (effects of neutropenia). bone marrow.
Diagnosis
Glucose-6-phosphate Dehydrogenase Deficiency
A complete blood count, leukocyte differential, reticulo-
cyte count, and a bone marrow aspirate and biopsy can Glucose-6-phosphate dehydrogenase is an enzyme in the
establish the diagnosis. Peripheral blood flow cytometry can RBC needed for the glucose metabolism. Deficiency of this
be done to rule out paroxysmal nocturnal hemoglobinuria. enzyme leads to accumulation of oxidants in the RBCs.
Patients younger than 40 years should be screened for These substances, which produce methemoglobin and dena-
Fanconi’s anemia by the use of clastogenic agents, diepoxy- tured hemoglobin, precipitate to form Heinz bodies. The
butane or mitomycin, which test for increased chromo- Heinz bodies cause alteration of cell membrane by attach-
somal breakage seen with this disorder. A family history of ment, thus leading to hemolysis of cell. Beans, drugs and
cytopenias should raise suspicion of an inherited disorder infection commonly trigger the hemolysis and lead to acute
even when no physical abnormalities are present. intravascular hemolysis. Clinical features such as dyspnea,
488
palpitations, weakness and jaundice may be seen. Oral mani- which is possibly stimulated by parvovirus, leading to
festations common to other anemias may be encountered. rapid onset of anemia. Stroke, which is the most dreaded
complication of sickle cell disease, is also a result of
Dental considerations vasoocclusive event in the brain.
Avoidance of triggering drugs such as dapsone and sul- 3. Hemolysis in patients with sickle cell anemia leading
fasalazine. Blood transfusion can be considered prior to to jaundice.
dental treatment if anemia is very severe.
Oral manifestations
Treatment Manifestations of oral cavity reflect the systemic features:
Hemolytic episodes are self-limited many of the times and ❍ Delayed eruption secondary to their general under-
most patients with drug- or infection-induced hemolysis development.
recover fully following treatment. Oxidant drugs should ❍ Pallor.
be avoided. ❍ Susceptibility to osteomyelitis due to compromised
blood supply: This is a result of intravascular sickling
leading to ischemic infarction and necrosis of bone that
Sickle Cell Anemia creates a favorable environment for bacterial growth.
Sickle cell anemia and thalassemia are described as hemo- ❍ Uncommon manifestations include anesthesia of man-
globinopathies due to the defective globin portion of the dibular nerve and asymptomatic pulpal necrosis.
hemoglobin molecule. While sickle cell anemia is due to a Anesthesia and pulpal necrosis are caused by restric-
qualitative defect in the globin chains (valine replaces glu- tion of the microvascular blood supply.
tamic acid), thalassemia is due to a quantitative defect. The ❍ Dental radiographs demonstrate step ladder trabecular
minor alteration in the globin chain renders the hemoglo- pattern due to hyperplasia and widening of marrow
bin susceptible to low oxygen tension, low temperature or spaces (as body tries to compensate for anemia).
decreased pH. In the presence of latter factors, hemoglobin ❍ Lateral skull radiographs demonstrate thickening of
forms a sickle-shaped crystal within the erythrocyte and diploe with ‘hair-on-end’ appearance due to perpen-
leads to stasis, vascular occlusion and ultimately hemolysis dicular arrangement of trabeculae (Figure 2).
of the red cells in end-capillary circulation. Sickle cell dis- ❍ Areas of radiopacity demonstrated on the radiograph
orders, which are inherited as autosomal recessive, are of are suggestive of previous infarction and calcification.
two types: sickle cell trait and sickle cell anemia. While in
Diagnosis
sickle cell trait (heterozygous), only one of the beta chains
is abnormal, both the beta chains are abnormal in sickle ❍ Peripheral smear: Normocytic normochromic cells
cell anemia (homozygous). These patients with sickle cell ❍ Complete blood count: Decreased hemoglobin
trait are relatively asymptomatic unless they are subjected ❍ Hemoglobin electrophoresis: Demonstrates abnormal
to extremely low oxygen tension (15 mmHg), such as in hemoglobin.
an unpressurized aircraft.
Treatment
Clinical manifestations
Patients with sickle cell anemia need regular monitoring
Manifestations of sickle cell anemia are divided into three of their hematological state. Sickle cell anemia can only be
categories. symptomatically treated, as there is no cure. Prompt and ade-
quate relief of pain is of prime importance. Vigorous hydra-
1. Systemic manifestations including impaired growth and
tion, analgesics and appropriate antibiotics best achieve
development and increased susceptibility to infections:
management of the painful vaso-occlusive crisis, if there is
Significant delay in sexual maturity, weight and height
any evidence of infection. Dehydration is common due to the
achievement is observed. Increased susceptibility to
patient’s inability to produce concentrated urine. Blood trans-
infection might be due to impaired splenic function or
fusion is advised in case of severe anemia and bone mar-
because, macrophages are involved in phagocytosis of
row transplantation was found to be successful in a patient
the defective RBCs and may not be available for kill-
with homozygous sickle cell disease. Folic acid supplements
ing bacteria.
were also found to be beneficial as patients exhibit folic
2. Vaso-occlusive events and organ damage: The stasis of
acid deficiency due to the rapidly proliferating bone marrow.
sickled red cells lead to vascular occlusion and infarc-
tion of the end organ. This vaso-occlusive phenomenon
leads to multiple crises. These crises are painful crises
in chest and abdomen, splenic sequestration crises due The main principles are to prevent trauma, infection,
to infarction and fibrosis of spleen, and aplastic crisis, hypoxia, acidosis or dehydration as these can precipitate
489
Figure 2 Oral manifestations

❍ Developmental and growth abnormalities leading to
malocclusion, spacing and occlusal abnormalities
❍ Pain and swelling of parotid glands due to deposition
of iron
❍ Deciduous and permanent teeth may be discolored due
to the deposition of iron
❍ Some cases might also show teeth with short roots,
thin lamina dura and prominent premaxilla (Chip-
munk facies)
❍ Radiographic changes include generalized rarefaction
of alveolar bone, chicken-wire appearance of enlarged
marrow spaces and coarse trabeculation. In the skull,
proliferation of marrow may completely erode the cor-
tex, leaving only periosteum, and produce a ‘hair-on-
end’ radiographic appearance.
Lateral skull radiograph reveals ‘hair-on-end’ appearance in Diagnosis
sickle cell anemia. Courtesy: Dr Sarat Gummadapu
❍ Peripheral smear: Normocytic normochromic cells
❍ Complete blood count: Decreased hemoglobin and
decreased RBC
sickle cell crises. Hemoglobin concentration of at least 7 g/dl ❍ Hemoglobin electrophoreses: Demonstrates abnormal
must be ensured for adequate oxygen delivery during gen- hemoglobin.
eral anesthesia for dental and other surgeries.
Treatment
Treatment of thalassemia major includes multiple blood
THALASSEMIA transfusions and the primary complication of the treatment
is iron overloading leading to deposition in liver, spleen,
Thalassemias are a group of congenital hemoglobinopa- pancreas and thyroid. Another complication is chronic active
thies characterized by a reduced rate of production of one hepatitis due to viral infection. Folic acid is also advised to
or more of globin chains (alpha or beta) in the hemoglobin offset the increased demand associated with chronic hyper-
molecule. Alpha chain is reduced or deficient in alpha activity of the bone marrow. Splenectomy was indicated
thalassemia and beta chain is reduced or deficient in beta previously, to decrease the sequestration of RBC.
thalassemia. Beta chains are commonly involved, when
compared to alpha chains. If affected individuals are Dental considerations
heterozygous for beta chains, it is called as beta thalasse-
mia trait or beta thalassemia minor. If affected individuals A patient who has had a splenectomy is at risk of massive
are homozygous for beta chains, then it is called as beta infection following bacteremia. It has been suggested that
thalassemia major or Cooley’s anemia. In thalassemia, these patients receive prophylactic antibiotics like oral
imbalance in globin chain production leads to hemolysis penicillin or erythromycin prior to dental treatment. Hepa-
thus resulting in microcytic, hypochromic type of anemia. titis risk to the patients due to transfusion should also be
The hemoglobin levels can even reach up to 2–3 g/dl. considered and universal precautions taken. Poor healing
While, beta thalassemias are most frequently found in is also a complication of the dental treatment. Surgery for
Mediterranean and black populations, alpha thalassemias facial deformities has been used successfully.
are found more frequently in South East Asia, Middle East
and Mediterranean populations.
WHITE BLOOD CELL DISORDERS
White blood cells (WBCs) or leukocytes originate from
Systemic manifestations are mildly similar to sickle cell pluripotent hematopoietic stem cells in either bone mar-
anemia, which are indicative of severe anemia. Growth and row or lymphoid tissue. These cells are responsible for the
development of the child is retarded with ashen gray skin protection of the body against foreign invaders such as
color due to pallor and jaundice. Patients may also present fungi, bacteria, viruses and parasites. Leukocytes are of
with cardiomegaly, splenomegaly and hepatomegaly. three types: granulocytes, monocytes and lymphocytes.
490
While, the granulocytes and the monocytes protect the Oral manifestations
body against invading organisms by phagocytosis, the
❍ Gingival and periodontal disease
lymphocytes function by boosting the immunity.
❍ Increased caries incidence
White blood cell disorders can also be classified as
❍ Oral ulcerations
follows:
❍ Early loss of teeth.
Classification
Qualitative disorders NON-NEOPLASTIC DISORDERS
❍ Chediak–Higashi syndrome
Non-neoplastic disorders The number of circulating WBCs is expressed as the number
❍ Leukocytosis
of cells found in a cubic millimeter of blood, which nor-
❍ Leukopenia
mally ranges from 4,500 to 11,000/mm3 in adults. The dif-
ferential WBC count is an estimation of the percentage of
Neoplastic disorders each cell type per cubic millimeter of blood. A normal dif-
❍ Leukemia ferential count is as follows: neutrophils, 50–60%; eosino-
❍ Lymphoma phils, 3%; basophils, less than 1%; lymphocytes, 20–30%;
❍ Multiple myeloma. and monocytes, 3–7%. The term leukocytosis is defined as
WBCs released by the bone marrow, circulate in peripheral an increase in the number of circulating WBCs to more than
blood forming two pools of cells, a marginal one and a 11,000 cells/mm3, and leukopenia as a reduction in the
circulating one. Cells in the marginal pool adhere to vessel number of circulating WBCs (usually to 4,500 cells/mm3).
walls and are readily available. When infection threatens
the body, the storage and marginal pools can be called on Leukopenia
to help fight the invading organisms. Growth promoting
substances called colony-stimulating factors (CSFs) are A decrease in the peripheral WBC count may occur because
responsible for the growth of committed granulocyte- of decreased numbers of any of the specific types of leu-
monocyte stem cells. In response to infection, there will be kocytes, but most often it involves the neutrophils (neu-
local release of CSFs leading to increased production of tropenia). A reduction in the number of granulocytes in
granulocytes and monocytes in the bone marrow. blood is known as neutropenia or sometimes, when severe
as agranulocytosis. Affected persons are extremely sus-
ceptible to infections, which may be severe enough to
cause death. Patients may also exhibit fever, weakness and
QUALITATIVE DISORDERS
marked fatigability.
Since leukocytes play a central role in host defense, it is Causes for neutropenia
not surprising that defects in leukocyte function, both
genetic and acquired, lead to increased vulnerability to Inadequate or ineffective granulopoiesis
infections. These qualitative defects can be: ❍ Aplastic anemia
❍ Defects in adhesion: Leukocyte adhesion deficiency ❍ Leukemia
(LAD type I) ❍ Due to cancer chemotherapeutic agents.
❍ Defects in microbicidal activity: Chronic granuloma- Accelerated removal or destruction
tous disease
❍ Defects in phagolysosome function: Chediak–Higashi ❍ Idiopathic
syndrome. ❍ Drugs such as aminopyrine
❍ Splenomegaly
❍ Overwhelming bacterial or fungal infections.
Chediak–Higashi Syndrome
Oral manifestations
It is a rare autosomal recessive disorder characterized by
abnormal granules in the granulocytes. This abnormality Ulcerations and necrotizing lesions may be seen on the
results in neutrophils with decreased chemotactic and bac- gingiva, floor of the mouth, buccal mucosa, pharynx or
tericidal ability. Patients develop severe neutropenia as a other sites within the oral cavity (agranulocytic angina).
result of ineffective granulopoiesis and most die in child- Ulcers lack the surrounding inflammation and are charac-
hood from infections or advanced lymphoproliferative terized by necrosis and foul smell. All of these lesions often
syndrome. Patients exhibit neuropathy, hypopigmentation, show massive growth of microorganisms, with a relatively
recurrent bacterial infections and hepatosplenomegaly. poor leukocyte response (minimal swelling and pus).
491
Treatment ❍ Eosinophilic leukocytosis

– Allergic diseases
Cause must be identified and all drugs should be discon-
– Parasitic infestations
tinued. Infections must be prevented or treated, if present.
– Drug reactions
Current treatment efforts also include administration of
– Certain malignancies
recombinant hematopoietic growth factors, such as granu-
❍ Basophilic leukocytosis
locyte CSF, which stimulate neutrophil production by the
– Malignancies
bone marrow.
❍ Monocytosis
– Chronic bacterial infections such as tuberculosis,
systemic lupus erythematosus, etc.
Oral infections in patients with severe neutropenia should ❍ Lymphocytosis
be considered potentially life-threatening because they can – Infections due to hepatitis A, cytomegalovirus and
lead to bacteremia and septicemia. Ulcers can be treated with Epstein–Barr virus.
topical anesthetics and antiseptic mouthrinses. In severe
pulpal and periodontal infections, broad-spectrum antibi- Oral manifestations are seen as a consequence of underly-
otics can be advised until culture reports are available. ing disease process rather than the elevated leukocyte levels.
For example, in infectious mononucleosis, there is lym-
Cyclic neutropenia phocytosis and patients complain of sore throat, petechiae
and fever. Patients exhibiting these clinical signs must be
It is a rare disorder that occurs secondary to a periodic evaluated and investigated as these clinical findings can
failure of the stem cells in the bone marrow. It is character- be confused with leukemia.
ized by transient severe neutropenia that occurs approxi-
mately every 21 days. Neutrophil count is at its bare
minimum for a period of 3–7 days during which the clinical
manifestations pertaining to neutropenia are observed. NEOPLASTIC DISORDERS
The most common signs are fever, stomatitis, pharyngi-
tis and skin abscesses. Severity of infection is directly pro- Leukemia
portional to the severity of neutropenia. Treatment and
dental considerations are similar to that of neutropenia. Leukemia is a heterogeneous group of hematological dis-
orders that arise from a hematopoietic stem cell character-
ized by a disordered differentiation and proliferation of
Reactive Leukocytosis neoplastic cells. This neoplastic proliferation in marrow
results in diminished production of normal erythrocytes,
An increase in the number of WBCs is a common reaction granulocytes and platelets in leukemia. These neoplastic
in the variety of inflammatory states caused by microbial cells ultimately infiltrate various organs like spleen, CNS,
and non-microbial stimuli. Leukocytosis is relatively non- lymph nodes, skin and gingiva. Leukemia is categorized
specific and can be classified on the basis of particular according to its clinical behavior: acute or chronic; and
white cell series affected. In response to increased demand, histogenic origin: lymphocytic or myelocytic. The classifi-
increased number of immature neutrophils called ‘bands’ cation is called as FAB (French, American, and British) and
enters the circulation, a process called a ‘left shift’. This is is widely accepted. The etiology is unknown but genetic
called as leukemoid reaction, which is often secondary to factors, irradiation, viruses, chemicals such as benzene and
viral infections. This reaction can be distinguished from some drugs have been implicated. All types of leukemia
acute leukemia, as there is an orderly maturation and are more common in males compared to females.
proliferation of all normal myeloid elements in the bone
marrow.
Acute Leukemia
Causes of leukocytosis
About 50% of all leukemias are in the acute form. Acute
Physiologic
leukemias are divided into two major groups: acute lym-
❍ Exercise phocytic leukemia (ALL) and acute monocytic leukemia
❍ Pregnancy (AML). While, ALL is more commonly seen in children,
❍ Stress AML is frequently seen in the adults. ALL is frequently
derived from B-lymphocytes or their precursors. The acute
Pathologic
leukemias have increased number of immature cells called
❍ Neutrophilic leukocytosis blasts in the peripheral circulation. As blasts accumulate
– Acute bacterial infections in the marrow, they suppress normal hematopoietic stem
– Burns cells.
492
Clinical features in effect, the chronic leukemias have a slower onset of

symptoms, a better prognosis, and more mature WBCs
The major manifestations of acute leukemia result from
than do acute leukemias. The two major types of chronic
the paucity of normal RBCs, WBCs and platelets. They
leukemia are chronic granulocytic or myelocytic leukemia
have the following characteristics:
(CML) and chronic lymphocytic leukemia (CLL).
❍ Abrupt stormy onset.
❍ Symptoms related to depression of normal marrow
function: Fatigue, owing mainly to anemia, fever, reflect- Chronic Myelocytic Leukemia
ing infections due to granulocytopenia and bleeding, Chronic myelocytic leukemia accounts for about 14% of
secondary to thrombocytopenia. all leukemias, is almost exclusively a disease of adults
❍ Generalized lymphadenopathy, splenomegaly and with the peak of presentation being at 40–60 years and is
hepatomegaly due to dissemination of leukemic cells. characterized by the presence of Philadelphia chromosome,
❍ CNS manifestations such as palsies, vomiting and an acquired genetic defect resulting from translocation of
headache due to meningeal spread. These features are genetic material from chromosome 22 to chromosome 9.
more common in children and in ALL. CML has two phases: chronic and blastic. While malignant
❍ Localized tumors or chloromas, which turn green on cells with normal function are present in chronic phase,
exposure to sunlight, due to local infiltration of leuke- cells with further malignant transformation are found in
mic cells. blastic phase. Patient often survives for years before the
disease enters blast phase.
Laboratory diagnosis
Laboratory findings show WBC count sharply elevated, Clinical features
sometimes reaching 100,000 cells/mm3. Platelet count is
usually depressed below 100,000 cells/mm3 and numerous Some of the patients are asymptomatic and some exhibit
blast cells can be identified. Bone marrow aspirate reflects the following:
the peripheral circulatory changes. ❍ Shortness of breath due to anemia
❍ Abdominal discomfort due to splenomegaly
Treatment ❍ Weight loss and fever
Therapeutically, the aim is to reduce the population of leu- ❍ Bleeding tendency in the later stages.
kemic clone enough to allow reconstitution with the prog-
eny of remaining normal stem cells. Chemotherapy satisfies Laboratory diagnosis
the above requirement and is conveniently divided into
three phases. The purpose of the first phase (induction) is ❍ Complete blood count: Increased WBC count.
to hit hard and induce a state of remission by killing tumor ❍ Bone marrow aspirate: Demonstrates increased cellu-
cells with cytotoxic agents. The purpose of the second phase larity and Philadelphia chromosome.
(consolidation) is to consolidate the killing of remaining
leukemic cells. The purpose of the third phase (remission) Treatment
is to provide maintenance treatment to prevent any remain-
ing leukemic cell mass from expanding. Patients are cured Control of chronic phase is often successful when com-
when no leukemic cells remain. Chemotherapeutic agents pared to blastic phase. Imatinib, a tyrosine kinase inhibitor
commonly used are vincristine, cytarabine, daunorubicin is the first line of treatment in the chronic phase. As the
and methotrexate. disease enters blastic phase, other treatment options have
Long-term survival occurs when the leukemic cell mass to be explored.
is greatly reduced and kept from increasing over a long Allogenic hemopoietic stem cell transplantation can cure
period. If a patient relapses, remission again is difficult to approximately 70% of chronic phase CML patients but with
induce and bone marrow transplantation is then advised. risk of complications and death due to graft-versus-host
There are some regions in the body where the leukemic disease (GVHD) and opportunistic infections.
cells migrate and are not acted upon by the chemothera-
peutic agents. These regions are called sanctuaries (CNS in
ALL). In these patients, radiation along with intrathecal Chronic Lymphoid Leukemia
methotrexate can be tried. Chronic lymphoid leukemia is one of the most common
leukemia, occurring predominantly in later life and increas-
ing in frequency with advancing years. It is almost invari-
Chronic Leukemia
ably B-lymphocytic in origin. In many patients it is a chance
Onset of chronic leukemia is insidious with the course finding with no symptoms, while others present with fea-
(untreated) of the disease running up to 2–6 years. So tures of bone marrow failure or immunosuppression.
493

❍ Recurrent infection because of (functional) leukopenia
and immune failure (reduced immunoglobulins)
❍ Anemia due to hemolysis or marrow infiltration
❍ Painless lymphadenopathy
❍ Splenic discomfort.
Laboratory diagnosis
❍ Complete blood count: Increased WBC count, platelets
and normal or reduced RBC
❍ Blood film: Lymphocytes increased
❍ Bone marrow aspirate: Reflects peripheral blood, infil-
trated with lymphocytes.
Treatment
Supportive treatment involves treatment of hemolytic ane- Gingival enlargement and bleeding in patient
mia with steroids, infection with antibiotics and infiltrations suffering from acute myeloid leukemia.
with chemotherapy. Specific treatment involves administra- Courtesy: Dr Sarat Gummadapu
tion of chlorambucil to reduce blood count and to decrease
lymphadenopathy and splenomegaly and thus successfully
palliating the disease. Fludarabine alone or in combination (result of radiotherapy for cranial involvement), and lichen-
with cyclophosphamide had a much greater impact on the oid lesions, desquamative gingivitis due to graft-versus-host
bone marrow and can induce complete remission. reaction. The oral mucosal cells due to their high mitotic
index are frequently compromised, due to their susceptibil-
Oral manifestations of leukemia ity to chemotherapeutic agents, predisposing the patients to
mucositis and xerostomia. The dental anomalies encoun-
Leukemic patients are prone to the development of variety tered are microdontia, dental agenesis and arrested root
of oral conditions associated with the malignancy and treat- development.
ment. The oral manifestations of acute leukemias reflect
the systemic complications. The manifestations are: Radiographic findings Most children with leukemia had
detectable radiographic changes of the jaws. These changes
Signs and symptoms related to depression of marrow include loss of lamina dura, displacement of teeth, loss of
function Pallor, owing to anemia; petechiae, ecchymosis the crypt outline around unerupted teeth, widened peri-
and gingival bleeding owing to thrombocytopenia (spon- odontal ligament and loss of cancellous bone trabecula-
taneous if platelet level falls below 20,000 cells/mm3); and tion. The latter leads to an appearance of generalized
infections, owing to neutropenia. The latter can manifest rarefaction. There are other reports that mention general-
as ulcerations and can be bacterial, viral or fungal. ized bone loss, increased mobility and protrusion of teeth in
Neutrophils are known to play a crucial role in protective leukemic patients. Periodontal destruction, which is severe,
host response to colonization. In patients with leukemia, may have been due to infiltration of leukemic cells into alve-
neutropenia is compounded by the chemotherapy thus olar bone.
increasing the risk of different opportunistic fungal infec- The patients of chronic leukemia also exhibit oral hem-
tions. Among the opportunistic infections present in the orrhage, petechiae, ulcerations and gingival swellings.
oral cavity, the most frequent is candidiasis. Mucormycosis
also occurs commonly in pulmonary and rhinocerebral Dental considerations
sites and can become widely disseminated to other organ
systems. Viral infections are also common in leukemia, Dental treatment should only be carried out after consul-
with herpes simplex being the most common. tation with the physician, as various aspects of management
and the probable life expectation may affect it. Preoperative
Signs and symptoms related to infiltration of leukemic
precautions should include screening for hepatitis B and
cells Gingival and salivary gland enlargement (Figure 3),
HIV. Preventive oral healthcare is essential and where
neural and developing tooth crypt involvement, and pain-
indicated, conservative dental treatment may be possible.
less cervical lymphadenopathy.
Management of chronic dental infections in patients with
Signs and symptoms related to treatment of leukemia hematologic malignancies ideally should be based on data
Oral ulcerations and mucositis due to chemotherapy and/or that correlates examination findings with outcomes of
radiation, craniofacial and dental anomalies in children treatment. One result of compromised hematologic status
494
among dental patients with hematologic malignancy, idio- features of HD include Pel-Ebstein fever, a cyclic spiking
pathic or drug-induced blood dyscrasias and sickle cell of high fever, and generalized severe pruritis. Pruritis is a
anemia is that certain clinically relevant laboratory values well-known cutaneous, paraneoplastic manifestation of HD.
have been proposed as important in management protocols. As the disease progresses, signs and symptoms arise from
When oral surgical procedures are anticipated, a platelet pressure and obstruction caused by enlarging nodes. Diag-
count of at least 50 109/l and absolute neutrophil count of nosis is made from nodal biopsy or bone marrow aspirate.
at least 0.5 109/l are sought by the provider to comfortably
assure effective hemostasis and reduce the risk of postop- Treatment
erative bacterial infection. But, in smokers, dental extrac- The outlook after aggressive radiotherapy and chemother-
tions should not be used as a means of controlling chronic apy for patients with this disease, including those with
asymptomatic periodontal and pulpal diseases, as these disseminated disease, is generally very good. With current
patients are prone to fungal infections. Oral ulcerations in modalities of therapy, the histologic picture has very little
leukemic patients should be managed by topical antibacte- impact on the prognosis; instead clinical stage appears to
rials along with analgesic and anesthetic rinses. Removing be an important prognostic indication. However, long-term
irritants, applying local pressure and hemostatic agents survivors of combined chemotherapy–radiotherapy proto-
such as absorbable gelatin or collagen sponges should be cols are at much higher risk of developing acute leukemia.
used to manage bleeding tendency. If the patient does not Currently, combination chemotherapy of doxorubicin, bleo-
respond, then platelet transfusions might be warranted. mycin, vincristine and dacarbazine is used for most patients.
Lymphomas Non-Hodgkin’s Lymphoma

The lymphomas represent seventh most common malignancy Non-Hodgkin’s lymphoma (NHL) is a lymphoproliferative
worldwide. These encompass a group of entities that vary disorder of unknown cause that can occur in all races and
widely in terms of their clinical presentation and behavior. age groups. Unlike HD, which often begins with a single
These originate in lymph nodes or in extranodal tissue in any focus of tumor, NHL is usually multifocal when first detected.
part of the body, and from any type of lymphocyte. Three Ninety percent are of B-cell derivation. NHL has been
types of lymphomas can be considered: Hodgkin’s, non- reported in association with AIDS and is classified based
Hodgkin’s and Burkitt’s. Multiple myeloma also needs a on patterns of distribution (diffuse or nodular), cell type
special mention, though it is not a lymphoma, as it is a (lymphocytic, histiocytic and mixed) and degree of differ-
malignancy of plasma cells, which arise from B-lymphocytes. entiation of cells (well, moderate and poor).
Hodgkin’s Disease
The most common presentation of NHL is a painless per-
Hodgkin’s disease (HD) is a disorder of unknown etiology sistent enlargement of lymph nodes (Figure 4). Unlike HD,
involving primarily the lymphoid tissue. It arises almost in extranodal lesions can occur in GI tract, Waldeyer’s ring,
a single node or chain of nodes and spreads characteristi- spleen, skin and bone marrow. Signs and symptoms include
cally to the anatomically contiguous nodes. There are two fever of unknown cause, weight loss, malaise, sweating, and
peaks of incidence, one in early adulthood and one around abdominal or chest pain. The clinical differences between
fifth decade of life, and males have increased incidence when HD and NHL are outlined below.
compared to females. Four subtypes are recognized based
on histologic features (Rye system): lymphocyte predomi- Clinical differences between HD and NHL
nance, nodular sclerosis, mixed cellularity and lympho-
cyte depletion. The lymphocyte predominance has the best HD NHL
prognosis and lymphocyte depletion, the worst. HD is also Extranodal involvement uncommon Extranodal involvement seen
staged into four stages based on the clinical features (Ann More often localized to a single axial More frequent involvement of
Arbor classification). Prognosis becomes worse as the stage group of nodes multiple peripheral nodes
increases. Histologically, HD shows multinucleated Reed– Orderly spread by contiguity Non-contiguous spread
Sternberg cells.
Pel–Ebstein fever Non-specific fever
Treatment
Hodgkin’s disease usually presents as a painless enlarge-
ment of the lymph nodes (rubbery). Extranodal involve- Non-Hodgkin’s lymphoma is radiosensitive and the treat-
ment is rare. The signs and symptoms include fever, weight ment may include a combination of radiotherapy and che-
loss, sweating, pruritis and fatigue. Characteristic clinical motherapy. High dose chemotherapy with autologous stem
495
should arise if lymphadenopathy appears without signs of

Figure 4
infection or more than one lymph node chain is involved,
or a lymph node of 1 cm or more in diameter persists for
more than 1 month. A lymphoma may form in the oral
cavity or oropharynx but this is rare except in NHL. HD may
exhibit inflammatory gingival overgrowth, root resorption
and bone loss, which are thought to be paraneoplastic. The
most common site for non-Hodgkin’s extranodal lympho-
mas is the GI tract. In the head and neck region it occurs
within soft tissues or bone; the salivary gland, cheek,
paranasal sinuses and gingiva are the most common sites.
Maxilla is more commonly involved when compared with
the mandible. The radiographic methods used for diagno-
sis of malignant lymphoma include MRI, CT scanning and
ultrasound. Ceysens et al noted that the CT appearance of
extranodal NHL might often mimic other malignant and
inflammatory lesions. Further, 67Ga scintigraphy and bone
scintigraphy may also be useful for evaluating malignant
Enlargement of the accessory group of lymph nodes on lymphoma. NHL is more commonly seen in HIV patients
the right side of the face in a patient suffering from presenting as a slow-growing, painless, bluish, soft masses
non-Hodgkin’s lymphoma. Courtesy: Dr Sarat Gummadapu on the palate. Numb chin syndrome, which is the mental
neuropathy and facial and oral numbness restricted to the
distribution of the mental nerve, is also found to be fre-
cell transplantation is the treatment of choice for relapsed quently associated with malignant lymphoma. This might
disease. Commonly used drug protocols include cyclophos- be either due to direct invasion of tumor cells into the
phamide, doxorubicin, vincristine and prednisone (CHOP nerve or the compression of the nerve by metastatic man-
and CVP). dibular tumors, or the involvement of the trigeminal nerve
root by metastatic meningeal tumors.
More commonly, dental complications result from radio-
Burkitt’s Lymphoma therapy or chemotherapy administered in children with HD
during tooth development. These include agenesis, hypo-
Burkitt’s lymphoma (BL) is a B-cell neoplasm endemic in
plasia and blunted or thin roots. Patients with lymphoma
some parts of Africa and sporadic in other areas, including
sometimes complain of burning mouth symptoms that may
the United States. The tumor is the human cancer most
be related to drug toxicity, xerostomia, candidiasis or ane-
closely linked with a virus (Epstein–Barr virus). The tumor
mia. Patients who have received more than 3,000 rad (cGy)
is considered as the fastest growing human tumor as it
are susceptible to xerostomia and would benefit from sali-
doubles in 1–3 days.
vary substitutes or pilocarpine. Radiation also can damage
taste buds, cause trismus of masticatory muscles, and stunt
Clinical features
craniomandibular growth and development.
Both the endemic and non-African cases mainly affect
children or young adults. In both the forms, the disease
Multiple Myeloma
rarely arises in lymph nodes. In African patients, involve-
ment of maxilla or mandible is the common mode of pre- Multiple myeloma (MM) is a relatively uncommon malig-
sentation, whereas abdominal tumors are more common nant neoplasm of the plasma cells (which arise from the
in North America. B-lymphocytes) that often appears to have a multicentric
origin within bones. The cause of the condition is unknown,
Treatment although sometimes a plasmacytoma may evolve into
multiple myeloma. This disease makes up about 1% of all
Majority of patients can be cured with aggressive chemo-
malignancies and 10–15% of hematologic malignancies.
therapy. BL was found to be responsive to cyclophospha-
The abnormal plasma cells in the bone marrow proliferate
mide, methotrexate, vincristine and cytarabine.
from a single malignant precursor (monoclonal) and pro-
duce immunoglobulins that are not normal or functional.
Oral manifestations and dental considerations
This monoclonal nature of plasma cells can be differenti-
Painlessly enlarged cervical lymph nodes are the initial ated from polyclonal nature of plasma cells seen in chronic
complaint in many of the cases. Suspicion of lymphoma inflammation.
496
Clinical and radiographic features BLEEDING DISORDERS

Multiple myeloma is typically a disease of older adults
(4th to 5th decade), with men being affected slightly more The integrity of circulation is maintained by blood flowing
often than women. The manifestations commonly seen are through intact vessels lined by endothelial cells. Efficient
anemia, thrombocytopenia and leukopenia due to suppres- mechanisms have evolved to maintain the circulation as
sion of other cells originating in the bone marrow. This may a transport system, which both prevent blood loss from
lead to pallor, fatigue, infections and bleeding tendency. Bone a damaged vessel by securing hemostasis, and also pre-
pain, due to motion or pressure against bony tumor masses vent the cessation of flow due to thrombosis. Hemostasis
and pathological fractures. Renal failure and amyloidosis depends upon interactions between vessel wall, platelets
due to deposition of abnormal proteins in kidney and var- and clotting factors. There are two phases of hemostasis:
ious parts of the body. Bence Jones proteins, which are the primary and secondary. In the initial primary phase, the
light chains of immunoglobulins, are excreted in the urine. damaged vessel constricts and platelets aggregate at the
site of damage to form a plug to arrest hemorrhage within
Hypercalcemia, due to destruction of bone: Radiographi- a few minutes. This is followed by activation of the coagu-
cally, multiple well-defined ‘punched-out’ radiolucencies lation system with secondary deposition of a fibrin mesh to
or ragged radiolucent lesions may be seen. secure the platelet plug. These two phases are interlinked;
damaged endothelium and the subendothelial matrix acti-
Oral manifestations vates platelets, which then provide the optimal surface for
Macroglossia, gingival and soft tissue enlargement due to the binding of the clotting factors and generation of insol-
deposition of amyloid. uble fibrin. Fibrinolysis is the major means of disposing of
Pallor, petechiae, ecchymosis and increased bleeding fibrin after its hemostatic function has been fulfilled, and
tendency. The elevated serum monoclonal antibody can act it can be considered the rate limiting step in clotting. The
directly as a thrombin inhibitor or inhibit the interaction protagonists of the hemostasis are as follows:
of von Willebrand factor (vWF) with platelets or interfere
with fibrin polymerization and lead to excessive bleeding Vessel wall
tendency. After tissue injury, serotonin and other vasoactive sub-
Jaw swelling, jaw pain, tooth mobility and paresthesia due stances are released, which mediate the immediate reflex
to tumor growth: Multiple myeloma can have three differ- vasoconstriction. This alone might be sufficient to arrest
ent radiographic manifestations in the jaw bone: (i) nor- bleeding from small vessels.
mal findings, when there is non-detectable or mild bone
resorption, (ii) multiple punched-out radiolucencies from Platelets
the focal proliferation of plasma cells inside the bone mar- If the defect in the blood vessel is very small—and many
row and (iii) generalized bone rarefaction and osteoporotic small holes develop in the vasculature each day—it is often
changes resulting from diffuse or total replacement of the sealed by a platelet plug rather than by a blood clot.
bone marrow by malignant cells. Platelets are minute round or oval disks, which circulate in
the blood. When these circulating platelets are exposed to
Treatment damaged vascular surfaces (in the presence of normal
Chemotherapy constitutes the main modality with radiation vWF and endothelial cells), they are activated to produce
therapy as palliative modality of treatment for painful bone physical and chemical changes. These changes produce an
lesions. Alkylating agent, such as melphalan or cyclophos- environment that causes the platelet to aggregate and
phamide, is often used in conjunction with prednisolone, release ADP and platelet factors, which cause further platelet
and approximately 60% of patients respond initially to aggregation and promotes clotting mechanism.
this regimen. Complete remission is never attained and all
patients will relapse without further treatment. The prog- Clotting mechanism
nosis is variable, but the survival of treated patients aver- Coagulation involves a series of enzymatic reactions lead-
ages 3 years. ing to conversion of soluble plasma fibrinogen to fibrin clot.
This process involves multiple proteins, many of which are
synthesized by liver (fibrinogen, prothrombin, factors V,
Dental treatment can be complicated by anemia, infections, VII, IX, X, XI and XII) and many are vitamin K dependent
hemorrhagic tendency, renal failure and corticosteroid (factors II, VII, IX and X). The scheme of reaction is a bio-
therapy. Bleeding may result from several causes, including amplification in which, the precursor is altered to an active
thrombocytopenia, abnormal platelet function, abnormal form, which, in turn, activates the next precursor in the
coagulation or hyperviscosity. sequence. Beginning with an undetectable biochemical
497
reaction, the coagulation mechanism results in the forma- 40 mmHg. A piece of filter paper is used to dry the borders
tion of insoluble fibrin. The clotting of blood also requires of the tiny cuts every 30 seconds until no more oozing is
calcium and phospholipids. present. Normal ivy bleeding time is between 1 and 6 min-
The mechanism initially proceeds by two separate path- utes and is prolonged if it is more than 15 minutes. Increase
ways (intrinsic and extrinsic) that converge by activating in bleeding time could be caused by platelet defects—
a third (common) pathway. The extrinsic pathway is initi- quantitative or qualitative—or by blood vessel defects. But
ated by release of tissue thromboplastin and does not its use as a predictive screening test for oral surgical pro-
require contact activation. Tissue thromboplastin binds to cedures has been discouraged as it is a poor indicator of
factor VII in the presence of calcium, and this complex is clinically significant bleeding at sites other than skin.
capable of activating factor X to Xa. The intrinsic pathway
is initiated when factor XII is activated by surface contact Prothrombin time (PT) The normal range of PT is 11–13
(e.g. with collagen or subendothelium), and it involves the seconds. The screening test examines the efficiency of
interaction of factors XII and XI. The activated factor XI extrinsic and common coagulation pathways. Increase in
with the help of divalent cation (calcium) and phospholipids PT can be due to vitamin K deficiency, fat malabsorbtion,
converts factor X to Xa. The factor Xa heralds the initia- coumarin therapy, liver disease or disseminated intravas-
tion of common pathway. Factor Xa converts prothrombin cular coagulation (DIC). Because different laboratories use
to thrombin, which further converts fibrinogen, a soluble different types of reactives derived from different tissues
plasma protein, to insoluble fibrin. Finally, fibrin polymer- (lung and brain) and different species (rabbit, bovine and
izes to form a gel, stabilizing the platelet plug. The clot thus human), it is necessary to standardize the PT. This has been
formed has to be broken down after or else it may lead to done using the international normalized ratio (INR). The
thrombosis. This critical function is carried out by TPA normal coagulation profile is reported as an INR of 1.0.
(tissue plasminogen activator), which converts plasmino- Activated partial thromboplastin time (aPTT) The nor-
gen to plasmin. The plasmin thus formed degrades fibrino- mal range is 22–36 seconds. The aPTT tests the coagula-
gen and fibrin into fibrin degradation products (FDPs). tion factors involved in the intrinsic coagulation pathway
This phase is the fibrinolytic phase, which forms the rate (factors VIII, IX, XI and XII). It is therefore elevated in con-
limiting step of hemostasis. ditions like hemophilia and von Willebrand’s disease, which
are a result of deficiency of one of these factors. It is also
Laboratory investigations elevated in heparin anticoagulant therapy.
A number of procedures that are performed in dentistry Tourniquet test The tourniquet test for capillary fragility,
may cause bleeding. Under normal circumstances, these which assesses the Rumpel–Leede phenomenon, is useful
procedures can be performed with little risk to the patient; in identifying disorders of vessel wall integrity or platelet
however, the patient whose ability to control bleeding has disorders. After inducing stasis by inflating the sphygmo-
been altered by drugs or disease may be in grave danger manometer cuff around the arm for 5 minutes, petechial
unless the problem is identified before undertaking any hemorrhages are visible on the volar aspect of the arm.
dental procedure. An alert clinician will get suspicion
based on the history offered by the patient, which under-
scores the importance of meticulous history taking. The Classification of Bleeding Disorders
most commonly used laboratory screening tests for bleed- Bleeding disorders can be broadly classified as:
ing disorders are bleeding time, platelet count, prothrombin
time and activated partial thromboplastin time. In addition 1. Vascular disorders (vessel wall)
to this, capillary fragility test can also be conducted. 2. Platelet disorders
3. Disorders of coagulation.
Platelet count This is usually obtained as a part of com-
plete blood count. Normal platelet count is 150,000–
450,000 cells/mm3. Decrease in the number of platelets is
VASCULAR DISORDERS (Vessel Wall)
called as thrombocytopenia. If the platelet count falls
below 50,000 cells/mm3, then hemorrhage may result due
to trauma or minor surgery. Spontaneous clinical hemor- Infections, chemicals, collagen disorders or certain types
rhage is usually not observed with platelet counts above of allergy can alter the structure and function of the vas-
10,000–20,000. cular wall to the point at which the patient may have a
clinical bleeding problem. The vascular phase in the hemo-
Bleeding time (BT) This is a functional test of primary stasis begins immediately following injury and involves
hemostasis. The ivy template method is performed using vasoconstriction of arteries and veins in the area of injury.
a special device that produces two small wounds keeping Retraction of arteries that have been cut, and build up of
the cuff of a sphygmomanometer constantly inflated at extravascular pressure by blood loss from cut vessels aids
498
in collapsing adjacent capillaries and veins leading to ces- gene on chromosome 15. It is characterized by skeletal
sation of bleeding. The various vascular disorders that may disproportion (arm span more than height), arachnodac-
lead to excessive bleeding tendency are: tyly (long, thin spider-like fingers), generalized hyper-
mobility of joints and CVS anomalies like mitral valve
Vessel wall disorders prolapse, aortic incompetence, etc. The oral manifestations
1. Congenital are: high arched palate, bifid uvula, malocclusion and
a. Hereditary hemorrhagic telangiectasia multiple odontogenic cysts of the maxilla and mandible.
b. Connective tissue disorders TMJ dysarthrosis is also reported.
• Ehlers–Danlos syndrome
• Osteogenesis imperfecta
• Marfan’s syndrome Scurvy
• Cushing’s syndrome
Ascorbic acid (vitamin C) is the most active reducing
2. Acquired disorders
agent in the aqueous phase of living tissues and is involved
a. Severe infections
b. Allergic manifestations
in the hydroxylation of proline in protocollagen to
c. Drugs hydroxylproline in mature collagen. It has been suggested
• Steroids that high dose of vitamin C improves immune function
d. Others (including resistance to common cold) and cholesterol
• Scurvy turnover, but such effects remain unproven in controlled
• Senile purpura trials. The scurvy, i.e. deficiency of vitamin C, leads to
defective formation of collagen. This impairs healing of
wounds, and causes capillary hemorrhage and reduced
Hereditary Hemorrhagic Telangiectasia platelet adhesiveness (normal platelets are rich in ascorbic
acid). Hemorrhage can occur in the muscles, joints, nail
It is a rare disorder with autosomal dominant inheritance.
beds and gingival tissues. Gingival involvement may
Dilatation of capillaries and small arterioles produce char-
include swelling, bleeding, secondary infection and loos-
acteristic small red spots that blanch on pressure in the
ening of teeth. For treatment, a dose of 250 mg vitamin C,
skin and mucous membranes, particularly the nose and
8-hourly by mouth should saturate the tissues quickly. The
GI tract. Recurrent epistaxis and chronic GI tract bleeding
general deficiencies of the patient’s former diet also need
are the major problems and may cause chronic deficiency
to be corrected and other vitamin supplements given if
anemia.
necessary.
Perioral and intraoral angiomatous nodules or telangi-
ectasia are common with progressive disease, involving
Investigations for vascular disorders
areas of the lips, tongue and palate that may bleed with
manipulation during dental procedures. Diascopy test is ❍ Bleeding time: Elevated.
positive unlike petechiae or ecchymoses. Mucocutaneous ❍ Tourniquet test: Positive.
lesions may bleed profusely with minor trauma or occa-
sionally spontaneously. Iron deficiency anemia has to be
treated with iron supplements, and persistently bleeding PLATELET DISORDERS
lesions may be treated with cryotherapy, laser ablation,
electrocoagulation or resection.
Platelet numbers or function may be impaired by many
diseases or drugs. Decrease in the number of platelets is
Ehlers–Danlos Syndrome called as thrombocytopenia, which can occur either due to
impaired production or excessive destruction. Defect in the
Ehlers–Danlos disease is a congenital disorder of collagen
function of platelets is called as thrombocytopathy, which
synthesis in which there is joint hyperextensibility, skin
can occur due to drugs, liver disease or can be inherited. For
extensibility and tissue fragility such that capillaries are
convenience, thrombocytopenic disorders and thrombocy-
poorly supported by subcutaneous collagen leading to ecchy-
topathic disorders are again divided into congenital and
mosis. Oral findings include bleeding after toothbrushing,
acquired.
hypermobility of TMJ, fragility of oral mucosa and gingiva,
stunted teeth and pulp stones on dental radiograph.
THROMBOCYTOPATHIC DISORDERS
Marfan’s Syndrome
The connective tissue disorder is inherited as an autosomal Disorders of the platelet function are usually associated
dominant trait and is caused by mutations in the fibrillin with excessive bruising and gingival bleeding and, in
499
some of the acquired forms, with thrombosis. The platelet

Congenital Acquired
count is normal or increases and the bleeding time is pro-
longed. Platelet function is abnormal due to a defect either May–Hegglin anomaly Impaired production
in the adhesion, aggregation or release of active sub- • Bone marrow failure
• Leukemia
stances. Inherited platelet disorders like Glanzmann’s
• Aplastic anemia (drugs, chemicals)
thrombasthenia and Bernard-Soulier syndrome are defi-
Excessive destruction
cient in platelet membrane glycoprotein resulting in fail- • Immune
ure of platelet aggregation. Bernard-Soulier syndrome is • Idiopathic thrombocytopenic purpura
also characterized by large platelets resulting in epistaxis, Dilutional
menorrhagia and prolonged bleeding from tooth extrac- • Massive transfusion
tions. Storage pool disease is also an inherited thrombocy- Others
topathic disorder, which shows lack of storage pool of • DIC
platelet dense bodies, causing poor platelet function. • Thrombotic thrombocytopenic purpura
Medications can also reduce absolute number of platelets
or interfere with their function, resulting in post surgical
hemorrhage. Drug related platelet disorders are reversible Idiopathic Thrombocytopenic Purpura
within 7–10 days of discontinuation of drug. Aspirin acti-
vates an enzyme called prostaglandin synthetase, resulting Idiopathic thrombocytopenic purpura (ITP) is due to
in inactivation of cyclo-oxygenase and decreasing biosyn- immune destruction of platelets. The antibody-coated
thesis of prostaglandin and thromboxane that are needed platelets are removed following binding to receptors on
to regulate interactions between platelets and endothe- macrophages. The condition is seen in both children and
lium. A single 100 mg dose of aspirin provides rapid com- adults. In children, it is usually acute but self-limiting and
plete inhibition of platelet cyclo-oxygenase activity and may follow a viral infection or immunization. In adults, it
thromboxane production. Most NSAIDs have similar but is usually less acute than in children. It is characteristically
less significant antiplatelet effects compared with aspirin. seen in women and may be associated with other autoim-
mune disorders such as SLE, thyroid disease and autoim-
Congenital Acquired mune hemolytic anemia and also after infections with
viruses such as HIV. Platelet autoantibodies are detected in
Storage pool disease Drug-induced (e.g. Aspirin)
about 60–70% of the patients, and are presumed to be
Bernard–Soulier syndrome Renal and liver disease present, although not detectable, in the remaining patients.
Glanzmann’s thrombasthenia Myeloproliferative disorder Easy bruising, purpura, epistaxis and menorrhagia are
common. In severe ITP, oral hematomas and hemorrhagic
bullae may be present.
Children do not usually require treatment. If treatment
THROMBOCYTOPENIC DISORDERS is necessary on clinical grounds, high-dose prednisolone is
effective, given for a very short course. Intravenous immu-
These are caused by reduced platelet production in the noglobulin (IV IgG) should be reserved for very serious
bone marrow or excessive peripheral destruction of plate- bleeding or for urgent surgery. Adults with ITP having
lets. The underlying cause may be revealed by history and platelet counts more than 30,000 cells/mm3 require no
examination but a bone marrow examination will show treatment unless a surgical procedure is being carried out.
whether the platelets are reduced, normal or increased and Otherwise, first-line therapy consists of oral corticosteroids,
will provide essential information on morphology as well. 1 mg/kg body weight but IV IgG is useful where a rapid
In patients with thrombocytopenia due to failure of pro- rise in platelet count is desired, especially before surgery.
duction, no specific treatment may be necessary but the Second-line therapy involves splenectomy, to which the
underlying condition should be treated if possible. If the majority of patients respond.
platelet count is very low or the risk of bleeding is very
high, then platelet concentrate administration is indicated.
Thrombotic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura (ITP), which is due
to increased destruction of platelets and thrombotic throm- Thrombotic thrombocytopenic purpura (TTP) is a rare, but
bocytopenic purpura are the commonly acquired throm- very serious condition, in which platelet destruction leads
bocytopenic disorders. May–Hegglin anomaly is a rare to profound thrombocytopenia. It is a characteristic symp-
hereditary condition characterized by the triad of throm- tom complex of fever, florid purpura, fluctuating cerebral
bocytopenia, giant platelets and inclusion bodies in leuko- dysfunction and hemolytic anemia often accompanied by
cytes. Clinical features and the pathogenesis of bleeding of renal failure. The underlying cause is not fully understood
these are poorly defined. but TTP seems to be due to endothelial damage associated
500
with the presence in the circulation of very high molecular patient requiring deep subgingival scaling and root planing
weight multimers of vWF. TTP is associated with pregnancy, in areas of inflammation may require medical management
metastatic malignancy, drugs such as mitomycin C and before a comprehensive initial therapy appointment.
infections. Microvascular infarcts occur in gingival and
other mucosal tissues in about 60% of the cases. Treatment Restorative and prosthodontic therapy Restoring cari-
includes plasma exchange, using cryoprecipitate depleted ous teeth and preparing and cementing fixed prosthesis
FFP (cryo-poor supernatant). Most patients are also treated usually produce minimal bleeding. The risk of gingival
with prednisone, 1 mg/kg daily. bleeding can be reduced by the use of a thin rubber dam
and selection of an atraumatic, stable clamp or hemostatic
gingival retraction cord. Wedges and matrices can be used
Oral manifestations and dental considerations with gentle handling. Removable appliances can be con-
Oral manifestations Oral manifestations of vessel wall structed conventionally. Attention should be paid to removal
disorders and platelet disorders are similar in which they of rough areas in trays and final prostheses that might
exhibit small pinpoint hemorrhages, called petechiae and produce traumatic ulceration and bleeding.
larger patches called ecchymosis. Platelet disorders with
Endodontic therapy Endodontic treatment of infected
severely altered hemostasis can lead to spontaneous gingi-
teeth is especially important in the coagulopathy patient
val bleeding. Continuous oral bleeding over long periods
because it may avert the need for extraction. Generally,
of time fosters deposits of hemosiderin and other blood
there are no contraindications to root canal therapy, pro-
degradation products on the tooth surfaces, turning them
vided the instrumentation does not extend beyond the apex.
brown. Patients with disorders of coagulation mechanism
Pulpotomies, pulpectomies and root canal therapies can be
will exhibit bleeding due to trivial injuries, severe hemo-
generally performed without bleeding complications. The
philiacs experience bleeding into the joints (hemarthrosis)
minimal bleeding during pulp extirpation can be con-
and soft tissue hematomas. Oral bleeding occurs from sites
trolled intrapulpally with epinephrine or formocresol.
that are susceptible to trauma like labial frenum, tongue
and buccal mucosa. Though hemarthrosis is a common com- Orthodontic therapy There is no contraindication to
plication in hemophiliac’s weight-bearing joints, it rarely orthodontic treatment. Properly managed fixed appliances
occurs in the TMJ. are preferred over removable and functional ones that
reposition the mandible because they are less likely to pro-
Dental considerations voke bleeding both intraorally from tissue irritation and
within the TMJ. Archwires should be secured with elastic
Basic management principles for patients with severe
bands rather than free wires with sharp edges that cut the
defects: When severe bleeding is expected to result from
mucosa. Careful adaptation and cementation of bands and
dental treatment, the goal of management is to restore the
placement and removal of archwires are encouraged.
hemostatic system preoperatively to within the requisite
parameters and to support coagulation with adjunctive med- Pediatric dental therapy Pediatric dental patient occa-
ications or local measures. For reversible coagulopathies, sionally presents with prolonged oozing from exfoliating
removal of the causative agent or treatment of the primary primary teeth. Administration of factor concentrates and
illness or defect may allow the patient to return to normal extraction of the deciduous tooth with curettage may be
for the dental treatment period. For irreversible coagulop- necessary for the patient’s comfort and hemorrhage con-
athies, however the missing or defective element may need trol. Pulpotomies can be performed without excessive
to be replaced from an exogenous source. Because of the pulpal bleeding. Stainless steel crowns should be prepared
risks and expense of blood products, dental treatment to allow minimal removal of enamel at gingival areas.
plans should be arranged to maximize the benefit from the
transfusion by completing all procedures likely to induce Pain control/local anesthesia The administration of local
bleeding at one treatment visit, when possible. anesthetics in bleeding disorder patients requires certain
precautions to prevent hematoma formation. Although
Preventive and periodontal therapy Preventive dental patients with mild-to-moderate disease are at low risk, those
care and the maintenance of a healthy periodontium are with severe disease, especially severe hemophilia A, have
especially important for the patient with severe bleeding been shown to have 11% rate of hematoma formation fol-
disorder. Gingivitis can predispose the patient to sponta- lowing inferior alveolar nerve blocks. Hematoma spread to
neous gingival bleeding. Instructions in thorough brushing the lateral pharyngeal, retropharyngeal and submandibular/
and flossing, diet and nutrition, fluoride use, and semi- sublingual spaces may cause respiratory obstruction and
annual examination with gentle probing and prophylaxis death. Hence, block anesthesia and some infiltration anes-
are emphasized as with normal patients. Calculus can be thesia in the patient with a severe coagulopathy (including
removed atraumatically in stages with an ultrasonic scaler many mild-to-moderate hemophiliacs with factor levels
or hand instruments to reduce hyperemia. The periodontal below 20%) should be avoided until the hemostatic defect is
501
corrected. For acute treatment of a forming hematoma, INHERITED COAGULATION DISORDERS

application of pressure and ice are useful adjuncts to sys-
temic correction of the hemostatic defect. Use of periodontal The inherited disorders can result from deficiency of fac-
ligament injections in mild-to-moderate hemophiliacs may tors essential in the clotting cascade or deficiency of vWF.
be effective for restorative procedures. Intrapulpal anesthe- Of the inherited coagulopathies, von Willebrand’s disease
sia is safe and effective following access for pulp extirpation. is the most common. It results from the deficiency of von
An alternative to local anesthesia is nitrous oxide/oxygen Willebrand’s factor (vWF). Hemophilia A, caused by coag-
sedation which raises pain threshold and reduces anxiety. ulation factor VIII deficiency, is the next most common
Intramuscular injections should be avoided unless the coag- coagulation disorder, followed by hemophilia B, a factor IX
ulation defect has been corrected. Postoperative pain is deficiency. Clinical bleeding can vary from mild to severe,
controlled with acetaminophen and narcotics. Aspirin and depending on the specific clotting factor affected and the
other NSAIDs are contraindicated. When general anesthesia level of factor deficiency.
is considered, oral intubation is preferred over nasal intuba-
tion, which may induce a difficult-to-control nasal bleeding.
Oral surgery Surgical procedures impose the highest risk Hemophilia A
of bleeding in the coagulopathy patient. Though primary Hemophilia A is due to defective factor VIII (antihemophilic
importance is placed on systemic preoperative manage- factor, AHF). This is a glycoprotein of several components,
ment such as plasma product infusion, local measures are including factor VIII C (procoagulant that participates in
also extremely important in assisting clot formation and clotting cascade), VIII K:Ag (vWF, which binds to platelets
maintenance. Surgical technique should be atraumatic, and is the carrier for factor VIII C) and VIII R:Rco (Ristocetin
with care taken to remove osseous fragments and granula- cofactor, which supports platelet aggregation). In hemo-
tion tissue, reappose buccal and lingual plates, and smooth philia A only factor VIII C is reduced.
bony margins. Although having no direct effect on hemo- Inherited as an X-linked recessive trait, hemophilia
stasis, primary closure protects the blood clot, makes the affects males. Females are carriers as the normal X chro-
postoperative period more comfortable, and decreases the mosome suppresses the effect of abnormal X (females have
risk of postoperative bleeding. Placement of absorbable XX chromosomal disposition). Hemophilia is denoted as
hemostatic agents such as bovine collagen in the apical severe if factor VIII is less than 1%, moderate if factor VIII
third of extraction sites promotes stable clots. Topical is 1–5% and mild if factor VIII level ranges between 5%
buffered thrombin solution also has potent hemostatic and 25%. Hemophilia typically becomes apparent in child-
properties. It directly converts fibrinogen to fibrin and can hood when bleeding into muscles or joints (hemarthroses)
be applied to the bleeding site on an oxidized cellulose follows injuries. Hemarthroses can cause joint damage and
product, such as oxycel or surgical. Antibiotics should be cripple the patient, but bleeding after dental extractions is
considered when absorbable hemostatic agents are used or sometimes the first or only sign of mild disease. Bleeding
signs of active infection exist because postoperative infec- into the cranium, bladder and other sites can cause severe or
tion can lead to resumed bleeding. fatal complications. Hemorrhage in hemophiliacs is dan-
The antifibrinolytic epsilon-aminocaproic acid (EACA) is gerous either because of loss of blood, or because there may
an extremely useful adjunct to prevent postsurgical bleed- be damage to joints, muscles and nerves or pressure on
ing without the need for subsequent transfusions and to vital organs if hemorrhage is internal. This compression of
treat gingival oozing following deep scaling and root plan- the pharynx and larynx following hematoma formation in
ing. EACA prevents premature destruction of the fibrin clot the neck can be fatal.
by virtue of its ability to inhibit plasminogen activation. The
usual adult dosage is 50 mg/kg body weight every 6 hours for
Investigations
7–10 days. A 25% oral solution used topically and systemi-
cally is preferred over the tablet form or intravenous route. ❍ Bleeding time: Normal
❍ Prothrombin time: Normal
❍ Platelet count: Normal
❍ Activated partial thromboplastin time: Elevated
DISORDERS OF COAGULATION
❍ Factor VIII C: Deficient
❍ Factor VIII R: Ag—Normal
Coagulation disorders may be inherited or acquired. The
❍ Factor VIII R: Rco—Normal.
inherited disorders are uncommon and usually involve
deficiency of one factor only. The acquired disorders occur
Management
more frequently and almost always involve several coagu-
lation factors. The coagulation disorders can be classified Bleeding is treated by administration of factor VIII concen-
as follows. trate by IV infusion. Factor VIII concentrate is freeze-dried
502
and may be stored in domestic refrigerators at 40C. This Investigations

allows it to be administered by the patient immediately
❍ Bleeding time: Elevated
after bleeding has started, reducing the likelihood of chronic
❍ Platelet count: Normal
damage to joints and the need for in-patient care. The
❍ Activated partial thromboplastin time: Elevated
majority of severely affected patients are given prophy-
❍ Factor VIII C: Decreased
laxis 3 times per week from early childhood in an attempt
❍ vWF: Decreased.
to prevent permanent joint damage. Synthetic vasopressin
(desmopressin)—IV or SC or intranasal—produces a rise in
Management
factor VIII C proportional to the initial level of factor VIII.
It avoids the complications associated with blood products Treatment depends on the severity of the condition and may
and is useful for treating bleeding episodes in mild hemo- be similar to that of mild hemophilia, including the use of
philia and as prophylaxis before minor surgery. It is inef- desmopressin where possible. Intermediate purity factor VIII
fective in severe hemophilia. or vWF concentrates should be used to treat bleeding or to
cover surgery in patients who require replacement therapy.
Cryoprecipitate should be avoided because of greater risk
Hemophilia B and C of transfusion transmitted infections. Aspirin and other
Christmas disease (factor IX deficiency) is clinically identical NSAIDs should also be avoided.
to hemophilia A and inherited in the same way, but it is about
one-tenth as common as hemophilia A. Factor IX replacement
is needed before surgery and desmopressin is not used. ACQUIRED COAGULATION DISORDERS
Concentrates used to treat factors VIII and IX deficiencies
are specific for each state, and therefore a correct diagnosis Liver Disease
must be made to ensure effective replacement therapy.
Factor XI deficiency (PTA deficiency) is one of the more Liver disease may result in a number of defects in hemo-
common among other congenital coagulation defects and stasis. The defects are:
is sometimes known as hemophilia C. It is transmitted as ❍ Vitamin K deficiency: Occurs due to intrahepatic or
an autosomal dominant trait. Bleeding symptoms do occur extrahepatic cholestasis
but are usually mild. In the event of major surgery or ❍ Reduced synthesis: Reduced synthesis of coagulation
trauma, hemorrhage can be controlled with infusions of factors may be due to severe hepatocellular damage
fresh frozen plasma. ❍ Thrombocytopenia: Results from hypersplenism due
to splenomegaly
❍ Functional abnormalities: Functional abnormalities of
Von Willebrand’s Disease
platelets and fibrinogen are found in many patients
Von Willebrand’s disease is due to the inherited deficiency with liver failure
in vWF, which is a component of factor VIII. It affects females ❍ Disseminated intravascular coagulation (DIC): May occur
as well as males and is usually inherited as an autosomal in acute liver failure.
dominant condition, but a severe form of the disease may
be inherited as a sex-linked recessive trait like the hemo-
philia. vWF is responsible for platelet adhesion to dam- Vitamin K Deficiency
aged endothelium and also acts as a carrier for factor VIII, Vitamin K is necessary for carboxylation of coagulation fac-
protecting it from proteolytic degradation. Thus both bleed- tors II, VII, IX, X, without which these factors cannot bind
ing time and activated partial thromboplastin time are calcium. Deficiency of vitamin K may be due to inade-
elevated in von Willebrand’s disease. quate stores, malabsorption and oral anticoagulants (vita-
min K antagonists). PT and aPTT are prolonged and there
Clinical features may be bruising, hematuria and gastrointestinal or cerebral
Von Willebrand’s disease causes bleeding that has features bleeding. Deficiency may be treated by phytomenadione
similar to that caused by platelet dysfunction. But if severe, (vitamin K) 10 mg IV. Newborn babies have low levels of
it can resemble hemophilia. Three types of von Willebrand’s vitamin K, and this may cause minor bleeding in the first
disease have been identified. Type I and type II patients week of life (classic hemorrhagic disease of the newborn).
usually have mild clinical features. Bleeding follows minor
trauma or surgery, and epistaxis and menorrhagia often
Disseminated Intravascular Coagulation
occur. Hemarthroses are rare. Type III patients have more
severe bleeding but rarely experience the joint and muscle There is a widespread generation of fibrin within blood
bleeds seen in hemophilia A. vessels, owing to activation of coagulation by release of
503
procoagulant material, and by diffuse endothelial damage This section provides an overview of the more common
or generalized platelet aggregation. There is a consumption respiratory disorders and explores the relationship between
of platelets and coagulation factors and secondary activa- these conditions and oral health. A dentist with adequate
tion of fibrinolysis leading to production of fibrin degrada- knowledge and skills to collect information pertaining to
tion products (FDPs), which may contribute to coagulation those conditions which are likely to place these patients at
defect by inhibiting fibrin polymerization. The consequences a higher risk of developing complications by receiving inva-
of these changes are a mixture of initial thrombosis fol- sive dental treatment, can suitable modify dental treatment
lowed by a bleeding tendency due to consumption of coag- to meet the needs of these patients.
ulation factors and fibrinolytic activation. DIC may be
caused by malignant disease, septicemia, hemolytic trans-
fusion reactions, trauma, burns, surgery and liver disease. Classification of Respiratory Tract Disorders
The clinical presentation of DIC varies from no bleeding at Respiratory tract disorders can be grouped as:
all to profound hemostatic failure with widespread hemor-
rhage. Bleeding may occur from oral cavity, nose and I. Upper respiratory tract infections
venepuncture sites. The underlying condition is treated 1. Viral respiratory infections
and this may be all that is necessary in patients who are 2. Allergic rhinitis
not bleeding. Transfusion of platelet concentrates, cryo- 3. Pharyngitis and tonsillitis
precipitate and red cell concentrates is indicated in patients 4. Sinusitis
who are bleeding. II. Lower respiratory tract infections
1. Pneumonia
Anticoagulant therapy 2. Asthma
3. Chronic obstructive pulmonary disease (COPD) or
Anticoagulation with warfarin is frequently used as a pre- chronic obstructive airway disease (COAD)
ventive measure for embolic phenomenon related to sev- 4. Cystic fibrosis
eral conditions. Some of the most common ones are atrial 5. Pulmonary embolism
fibrillation, dilated cardiomyopathy, systolic congestive heart III. Granulomatous disorders
failure, valvular heart disease and deep vein thrombosis. 1. Tuberculosis
Warfarin inhibits production of vitamin K-dependent fac- 2. Sarcoidosis
tors II, VII, IX and X. The injudicious use of coumadin on IV. Malignant disorders
occasion can lead to spontaneous bleeding that can be 1. Bronchogenic carcinoma (lung cancer)
severe or even fatal. 2. Nasopharyngeal T and NK cell lymphomas
V. Other respiratory disorders
1. Legionnaire’s disease (legionellosis)
RESPIRATORY DISORDERS 2. Lung abscess
3. Bronchiectasis
The lungs, with their combined surface area of more than 4. Obstructive sleep apnea syndrome (OSAS)
500 m2, are directly open to the external environment. 5. Occupational lung disease.
Thus, structural functional, or microbiological changes
within the lungs can be closely related to epidemiological,
Common Symptoms of Respiratory Diseases
environmental, occupational, personal, and social factors.
Primary respiratory diseases are responsible for a major Cough, production of sputum, hemoptysis, chest pain,
burden of morbidity and ultimately deaths and lungs are breathlessness and wheeze are common symptoms associ-
often affected in mouth system diseases. ated with respiratory diseases.
Respiratory infections are commonly encountered among
dental patients. The anatomic proximity of respiratory Cough
tract with the oral cavity lead to much interplay between
oral and respiratory infections and there is a growing body Cough is defined as an explosive expiration that provides
of literature pointing to a direct association between oral normal protective mechanism for clearing of tracheo-
pathogens and respiratory diseases. Recent studies have bronchial tree of secretions and foreign materials.
reported an oral bacteria as a causative pathogen in respi-
ratory diseases and conditions associated with significant Origin of the cough Common causes Nature/characteristics
morbidity and mortality and furthermore some respiratory
Pharynx Post-nasal drip Usually persistent
illnesses may have an effect on orofacial morphology and
Larynx Laryngitis, tumor Harsh, barking
even on dentition.
504
Trachea Tracheitis Painful mediastinal tumors), and pain associated with the chest
Bronchi Bronchitis Dry or productive
wall (rib fractures, direct invasion of chest by tumor, spinal
nerve root involvement).
Asthma Dry or productive, worse
at night
Breathlessness or dyspnea
Bronchiectasis Productive. Changes in
posture induce sputum It is the unpleasant subjective awareness of the sensation
production of breathing. The common respiratory causes for breath-
lessness include: acute severe asthma, chronic asthma,
chronic obstructive pulmonary disease, pneumonia, and
Sputum
pneumothorax. Occasionally other respiratory conditions
Sputum is matter (mucus, phlegm) that is expectorated may also predispose to dyspnea such as laryngeal edema,
from the respiratory tract (usually lower respiratory tract), inhalation of foreign object, extreme cases of pleural effu-
that is mixed with saliva, which can then be spat from the sion, bronchial carcinoma and acute respiratory distress
mouth. It is usually associated with diseases of the lower syndrome.
respiratory tract.
Wheezing
Type Appearance Cause
It is a continuous, coarse, whistling sound produced in the
Serous Clear, watery, may be Acute pulmonary edema respiratory passages during breathing. The primary causes
frothy and pink for wheezes to occur are either narrowed or obstructed respi-
Mucoid Clear, gray, white and Chronic bronchitis, asthma, chronic ratory passages or increased velocity of airflow within the
occasionally black obstructive pulmonary disease respiratory passages.
Purulent Yellow, green, brown All types of bacterial infection Wheezes may occur at various stages in the inspira-
Rusty Rust colored sputum Pneumococcal pneumonia tory and expiratory respiratory cycle. Diseases involv-
or golden yellow ing the brochioles may exhibit a wheeze that occurs in
the expiratory phase of respiration. Tumors, hypersensi-
tivity pneumonitis and foreign body obstructions may
Hemoptysis
produce a monotonal wheeze throughout the inspiratory
Hemoptysis is the expectoration of blood or of blood- phase.
stained sputum from the bronchi, larynx, trachea, or Partial collapse of the lungs may lead to production of
lungs. wheeze that occurs at the termination of both the expiratory
and inspiratory phases. Such a wheeze usually signifies the
Causes for hemoptysis periodic opening of deflated alveoli.
Stridor is a term used for a harsh, high-pitched, vibrat-
Common causes Uncommon causes Others causes
ing sound that is heard in respiratory tract obstruction.
Pulmonary infarction Mitral stenosis Foreign body inhalation Obstructions in the upper airway passages (trachea, lar-
Bronchial carcinoma Aspergilloma Chest trauma ynx, epiglottis) may produce stridor that occurs in the
Tuberculosis Bronchial adenoma Iatrogenic inspiratory phase, whereas lower airway obstructions will
Lung abscess Metastatic Bronchoscopy
produce a stridor in the expiratory phase of respiration.
pulmonary Transbronchial biopsy Clinical causes for wheeze
Acute bronchitis Malignant disease
❍ Infections (croup [laryngotracheobronchitis], whoop-
Chronic bronchitis Laryngeal tumors
ing cough, laryngitis)
Chronic obstructive ❍ Laryngo-, tracheo- or bronchomalacia
pulmonary disease ❍ Laryngeal or tracheal tumors, mediastinal masses
❍ Tracheal stenosis
Chest pain ❍ Emotional laryngeal stenosis
❍ Foreign body aspiration
Chest pain is commonly associated with anxiety, cardiac ❍ Asthma
diseases, respiratory diseases, and disorders of the muscu-
loskeletal and gastrointestinal system.
Chronic obstructive pulmonary disease
Chest pain associated with the respiratory system can
manifest as pleural pain (pneumonia, pulmonary infarction, ❍ Bronchorrheal states (chronic bronchitis, cystic fibrosis)
tuberculosis, malignant disease), retrosternal pain (tracheitis, ❍ Bronchiectasis
505
❍ Interstitial fibrosis, hypersensitivity pneumonitis infection of middle ear) may be present. Maxillary sinusitis
❍ Pulmonary edema may result in facial pain.
❍ Forced expiration in normal subjects
❍ Medications (many asthmatics wheeze following intake Oral manifestations and dental considerations
of aspirin).
The soft palate may reveal the presence of well-defined
Respiratory diseases and their dental significance are listed minute erythematous macular lesions. Use of antihista-
below. mines for the management of upper respiratory tract infec-
tions may result in xerostomia.
UPPER RESPIRATORY TRACT INFECTIONS Diagnosis

Laboratory tests are not really necessary for the diagnosis
Viral Infections of upper respiratory tract infections. However, the virus
may be isolated by culture or rapid diagnostic assay. A
Main causes for upper respiratory tract infections (RTI)
complete hemogram may reveal increased numbers of
}}
Rhinoviruses mononuclear cells, lymphocytes, and monocytes.
Respiratory syncytial viruses
Parainfluenza virus Common cold Management
Coxsackie viruses
Echoviruses Most of the infections are self-limited. The treatment is
Adenoviruses aimed at managing symptoms. Patient should be adequately
Influenza viruses hydrated. Antihistaminics are used to manage the rhinor-
Epstein–Barr viruses Tonsillitis and pharyngitis rhea. Analgesics and antipyretics are used to treat fever and
Beta-hemolytic streptococci myalgias.
Majority of the upper respiratory tract infections are

caused by Rhinoviruses (Picornaviridae family). Other Allergic Rhinitis
viruses that are commonly implicated include the respira-
tory syncytial viruses (Paramyxoviridae family), influenza Rhinitis is the recurrent inflammation of the nasal mem-
viruses (Orthomyxoviridae family) and coxsackie viruses branes and is characterized by a symptom complex that
(Picornaviridae family). consists of a combination of any of the following: sneezing,
As rhinoviruses are the major causative factors for upper nasal congestion, nasal itching, and rhinorrhea. The eyes,
respiratory tract infection, only they will be discussed. ears, sinuses, and throat may also be affected. When the
Rhinoviruses spread via aerosols of respiratory droplets conjunctiva is also affected the term allergic rhinoconjunc-
and contaminated surfaces and direct person to person tivitis is used. It is estimated that almost 20% of the popu-
contact. Once the virus enters the upper respiratory tract it lation suffer from allergic rhinitis. Seasonal, perennial,
binds to intercellular adhesion molecule-1 (ICAM-1) recep- sporadic/vasomotor and occupational allergic rhinitis are
tors on the respiratory epithelial cells and begins to replicate some of the types of allergic rhinitis.
within 15 minutes of entering the host’s respiratory tract. Environmental triggers such as pollen, dust mites, ani-
The infected cells release chemokines and cytokines. These mal dander may incite a type I allergic hypersensitivity
substances activate the inflammatory mediators of the reaction.
host. Following an incubation period of about 10 hours the In susceptible individuals, exposure to certain foreign
first symptoms begin to appear. proteins leads to allergic sensitization, which is character-
Rhinoviruses rarely cause lower respiratory tract disease ized by the production of specific IgE directed against
probably because they grow poorly at 37C. these proteins. This specific IgE coats the surface of mast
cells, which are present in the nasal mucosa. When the
specific protein (pollen/dust mite, spore) is inhaled, it can
Clinical features
bind to the IgE on the mast cells, leading to immediate and
The common clinical features seen in rhinovirus infections delayed release of a number of mediators such as hista-
include non-specific signs and symptoms such as fever, mine, chymase, kinins and heparin, which in turn produce
sneezing, malaise, myalgia, headache, cough, rhinorrhea the symptoms of rhinorrhea.
with discharge (serous or purulent), nasal congestion and Rhinits is present in two phases: the early or immediate
oro-nasal-pharyngeal irritation. phase and the late phase. The early phase is seen within a
As a consequence sinusitis and earache (obstruction few minutes of exposure to the allergen and characterized
of the pharyngotympanic tube by edema or by bacterial by nasal congestion, sneezing, itching, redness, tearing, and
506
post nasal drip. The late phase usually occurs about 6 hours Posture and allergic rhinitis
after the early phase and patients present with reduction
Hasegawa (1994) studied the effects of supine and lateral
in the frequency of sneezing and itching. However, conges-
recumbent positions in patients with allergic rhinitis. Their
tion and increased mucus production is more common. This
investigations showed that posture induces complete nasal
phase may last for a few days.
obstruction in the supine or lateral recumbent positions in
Fatigue, malaise, drowsiness or sleepiness are com-
some patients with allergic rhinitis.
mon systemic effects of rhinitis. It is believed that allergic
rhinitis may often occur along with asthma or atopic
Diagnostic tests
dermatitis.
Blood tests will reveal an elevated serum IgE and eosinophil
Orofacial features and dental considerations count. Radioallergosorbent test (RAST) may be employed
to test sensitivity to specific allergens. Alternatively a skin
The readily visible facial features in allergic rhinitis are prick test is performed. A drop of a known allergen extract
‘allergic shiners’, transverse nasal crease and Dennie–Morgan is placed on the forearm and a lancet is used to make a
lines. ‘Allergic shiners’ is the term used to describe the small prick on the skin through the drop. If the patient is
dark circles around the eyes due to vasodilation or nasal allergic to the test substance a small lump is seen in 15–20
congestion. minutes time.
Rhinorrhea prompts individuals to frequently rub the
nose in an upward direction with the palm. This is classi- Dental considerations
cally referred to as ‘allergic salute’. Repeated rubbing of
the nose causes the formation of a transverse crease at the Use of first generation antihistamines may result in dry
tip of the nose. Dennie–Morgan lines are prominent infra- mouth. Long-term use of steroid based inhalers may cause
orbital creases. These are also seen in atopic dermatitis. oral candidiasis.
The typical intraoral findings include tonsillar hypertro-
phy and streaks of lymphoid tissue on the posterior part of
Pharyngitis and Tonsillitis
the oropharynx which gives rise to a cobble-stone appear-
ance. Other symptoms include itching sensation in the pal- Inflammation of the pharynx is referred to as pharyngitis.
ate and tongue protrusion. A study by Elad et al (2006) It is usually caused by the direct infection of the pharynx,
showed the patients with allergic rhinitis had lower sali- primarily by viruses or bacteria. Group A beta-hemolytic
vary flow rates compared to healthy individuals. Streptococcus (GABHS) pharyngitis accounts for 15–30% of
cases in children and 5–15% of cases in adults.
Gingival inflammation Pharyngitis may also be caused due to post nasal drip
secondary to rhinitis, gastroesophageal reflux, thyroiditis,
Matsson and Möller (1990) studied the degree of gingival allergies, a foreign body, chronic mouth breathing and
inflammation in children with rhinoconjunctivitis due to smoking. It is estimated that approximately 1.1% of the
birch pollinosis. Their study showed that during the pollen visits to the primary care center are for acute pharyngitis.
season, children with allergic rhinoconjunctivitis exhibit It usually occurs in late winter and early spring. Trans-
an enhanced degree of gingival inflammatory reaction. mission of typical viral and GABHS pharyngitis occurs
mostly by hand contact with nasal discharge, rather than
Dental and skeletal abnormalities by oral contact. The first symptoms are seen after an incu-
Trask et al (1987) analyzed the effects of perennial allergic bation period of 24–72 hours.
rhinitis on dental and skeletal development. The allergic
Viral pharyngitis
subjects were characterized by deeper palatal vault height,
retroclined mandibular incisors, increased total anterior The viral form of pharyngitis is characterized by the pres-
facial height and lower facial height, a larger gonial angle, ence of rhinorrhea, cough, conjunctivitis, coryza, malaise or
and greater SN, palatal, and occlusal planes to mandibular fatigue, hoarseness, and low-grade fever.
plane angles. Also, the allergic subjects had smaller SNB The common viral causes for pharyngitis include
and SN-pogonion angles and an increased overjet com- Ebstein–Barr infection (infectious mononucleosis), coxsackie
pared to normal controls. virus infection (herpangina). Patients present with fever,
Martínez Esteinou and Omaña Vidal (1988) showed that malaise and fatigue, exudative tonsillopharyngitis, gener-
the patients with history of allergic rhinitis and nasopha- alized lymphadenopathy and hepatosplenomegaly. Almost
ryngeal obstruction (may result in mouth breathing) of 90% of the patients present with a classic maculopapular rash.
allergic origin exhibited a high palatal vault, retroinclin- Coxsackie virus infection is characterized by the presence
able maxillary incisors and increased total anterior facial of tonsillopharyngitis and discrete minute ulcers (2–3 mm)
height. on the anterior tonsillar pillars, uvula and soft palate.
507
Bacterial pharyngitis streptococcal infections especially in the course of acute

rheumatic fever.
Patients with bacterial pharyngitis generally do not have
rhinorrhea, cough, or conjunctivitis. GABHS (mainly Strep-
Management
tococcus pyogenes) is the most common bacterial cause of
pharyngitis. Other less common bacterial causes include Viral pharyngitis runs a short course and usually treated
Corynbacterium diphtheriae, Neisseria gonorrheae, Chlamydia symptomatically. Patients may be advised to use antiseptic
and Mycoplasma pneumoniae. and analgesic mouthrinses. Acute streptococcal pharyngitis
Pharyngotonsillitis caused by GABHS is characterized is treated with oral penicillin V or erythromycin or IM injec-
by pharyngeal erythema and swelling, tonsillar exudate, tions of benzathine penicillin G or oral cephalosporins.
edematous uvula, palatine petechiae, and anterior cervical
lymphadenopathy. The infections last for about one week Dental considerations
when left untreated.
GABHS may be persistent on toothbrushes and removable
Scarlet fever is associated with GABHS pharyngitis and
orthodontic appliances, thus patients with GABHS infec-
usually presents as a punctate, erythematous, blanchable,
tions should be instructed to thoroughly clean their tooth-
sandpaper-like exanthem. The rash is found in the neck,
brushes and removable orthodontic and acrylic appliances
groin, and axillae, and is accentuated in body folds and
daily and also to change to a new toothbrush after the acute
creases (Pastia’s lines). The pharynx and tonsils are ery-
stage of any oropharyngeal infection.
thematous and covered with exudates. The tongue may be
Amoxicillin and ampicillin should be avoided in treat-
bright red with a white coating (strawberry tongue).
ment of GABHS as they tend to cause rashes, especially if
The complications of GABHS infection are rheumatic
the sore throat in glandular fever is misdiagnosed as a
fever, peritonsillar abscess and poststreptococcal glomeru-
streptococcal sore throat.
lonephritis. Peritonsillar abscess occurs in fewer than 1%
of patients treated with antibiotics. Patients with periton-
sillar abscess typically have a toxic appearance and may
present with a ‘hot potato voice’, fluctuant peritonsillar
LOWER RESPIRATORY TRACT INFECTIONS
mass, and asymmetric deviation of the uvula.
Of the various lower respiratory tract infections, some of
those which have significant dental considerations will be
Clinical scoring system for validation of discussed below.
Streptococcal infection
Symptoms and points
Asthma
❍ Fever (subjective or measured in office): 1 point
❍ Absence of cough: 1 point Asthma is a dynamic condition characterized by chronic
❍ Tender anterior cervical adenopathy: 1 point airway inflammation and bronchial hyperactivity resulting
❍ Tonsillar swelling or exudates: 1 point. in symptoms of paroxysmal wheeze, cough, chest tightness
and dyspnea.
Age
Predisposing/risk factors
❍ Younger than 15 years: 1
❍ 15–45 years: 0 Asthma originates from the interaction of multiple genetic
❍ Older than 45 years: 1. and environmental factors. Asthma is known to run in
families. Childhood asthma usually occurs in atopic indi-
Scoring viduals who express elaborate amounts of IgE when exposed
❍ 0 or 1 points: streptococcal infection ruled out (2%) to common allergens/antigens.
❍ 1 to 3 points: order rapid test and treat accordingly Environmental factors are: house dust mites, automobile
❍ 4 to 5 points: probable streptococcal infection (52%), exhausts, industrial gaseous wastes, climatic changes,
consider empiric antibiotics. tobacco smoke, airborne irritants (including acrylic and
aerosolized dental materials) and pollen. Other factors that
predispose to asthma are infections (viral and bacterial
Diagnosis
infections), drugs (-blockers, salicylates/NSAIDs cause
Along with the clinical findings, blood smear examination, bronchoconstriction) and anxiety.
estimation of liver enzymes, culture for group A Strep- Extrinsic asthma is precipitated by allergens in house
tococcus and detection of antigen will aid in the diagnosis. dust, feathers, animal fur, molds, milk, eggs, fish, fruits,
Antistreptolysin ‘O’ titers raise about 150 U within 2 weeks nuts and NSAIDs; whereas intrinsic is related to mast cell
of acute infection. They are useful for documenting recent instability and hyperresponsive airways.
508
Pathophysiology inhaled steroids has also been linked to increased levels of

gingivitis.
It generally is believed that both genetic and environmental
Mandel et al (1969) and Wotman et al (1973) have
factors, as well as allergens, are important in the initiation
shown that asthmatic children exhibit more calculus than
and continuation of the airway inflammation.
do healthy children. It was found that the children suffering
Airway inflammation in asthma has been characterized
from asthma have increased levels of calcium and phos-
as acute, subacute or chronic. The acute state of inflamma-
phorus in submaxillary and parotid saliva.
tion is caused by the release of chemical mediators from
For many years the association between asthma and
activated resident cells, such as local airway mast cells
dentofacial morphology has been evaluated and discussed.
undergoing histamine degranulation.
It is suggested that the reason for the abnormalities is due
Subacute inflammation is marked by early cellular infil-
to the impaired nasorespiratory function. Various dentofa-
trates, especially eosinophils that release mediators with
cial abnormalities seen in asthmatics are increased upper
direct toxic effects on the respiratory epithelium.
anterior and total anterior facial height, higher palatal vaults,
The airway inflammation is described as chronic when
greater overjets, higher prevalence of posterior crossbites,
lymphocytes and eosinophils mediate a persistent, ongo-
long and tapered facial form, increased lower facial height
ing inflammation, thus resulting in a continuous cycle of
and narrow maxillary arch.
damage and repair. Long-standing chronic inflammation
can lead to irreversible airway obstruction in some patients.
Bronchial smooth muscle contraction contributes mark- Management
edly to the airway obstruction seen in asthma, while vaso-
Patients should be educated to avoid all known allergens.
dilation, diapedesis and vascular permeability account for
For patients with mild, intermittent asthma, the occasional
the edematous changes. These changes are attributed to cell-
use of an inhaled short-acting 2-agonist (albuterol, ter-
derived mediators. Mucus hypersecretion is also observed
butalin) is indicated. Bronchospasm can be managed with
and can result in the development of mucus plugs and
aerosolized bronchodilator containing methylxanthines
associated dyspnea.
such as theophylline and oxtriphylline. Steroids such as
betamethasone and triamcinolone and mast cell inhibitors
Clinical symptoms and signs (cromolyn sodium) are also used effectively.
Cough, shortness of breath, chest tightness, wheezing,
tachypnea and tachycardia, are usually noticed. Typically Dental considerations
these symptoms show ready reversal when bronchodilators
are used. In acute asthmatic attacks patients are extremely For dental considerations see Table 1.
distressed, flaring of the nares, sweating, central cyanosis,
use of accessory respiratory muscle, pulsus paradoxus and
silent chest may be seen. Chronic Obstructive Pulmonary Disease or
Chronic Obstructive Airway Disease
Orofacial features
Chronic obstructive pulmonary disease (COPD) is caused
Asthmatic patients are known to have an increased inci- by chronic bronchitis and emphysema. Chronic bronchitis
dence of carious lesions, reduced salivary flow, an increased is defined as the excessive production of mucus and persis-
prevalence of oral mucosal changes, increased levels of tent cough with sputum production for more than 3 months
gingivitis and related orofacial abnormalities. in a year over 2–3 consecutive years.
It is believed that the prolonged use of 2-agonists, Emphysema is dilatation of air spaces distal to the ter-
which is associated with diminished salivary production minal bronchitis with destruction of alveoli and reduction
and secretion results in increased caries development. This in the alveolar surface area available for respiratory
reduction in salivary flow (26% drop in whole saliva) is exchange.
accompanied by concomitant increase in lactobacilli and
Streptococcus mutans in the oral cavity. Anti-asthmatic
Etiopathogenesis
medications containing fermentable carbohydrate and
sugar may also add to the bacterial build-up. The main etiological agent is tobacco smoke. Other etio-
The use of corticosteroids via nebulizers leads to dys- logical agents include industrial dust, automobile exhaust,
phonia, dryness of mouth and oropharyngeal candidiasis. smoke emitted from traditional cooking using firewood.
Linder et al (1995) reported tongue hypertrophy associated The contents of smoke produce a severe inflammatory
with inhaled corticosteroid therapy in premature infants. response. The respiratory epithelium is damaged and the
Since most of the asthmatics are habitual mouth breath- enzymes necessary for respiratory metabolism are impaired.
ers, gingival enlargement is routinely encountered. Use of The oxygen carrying capacity of RBCs is diminished.
509
Table 1 Dental considerations for asthma
Do’s Don’ts
Fluoride supplements for those taking 2-agonist Barbiturates and narcotics should be avoided because they can cause
bronchospasm and reduce respiratory functions
Instruct patient to rinse mouth with water after using inhalers. Oral Avoid antihistamines (promethazine, diphenhydramine) as they can
hygiene measures to reduce gingivitis and periodontitis cause a drying effect and increase risk of forming tenacious mucus in
acute attack
Schedule patient’s appointments for late morning or later in the day, to Dental materials such as methyl methacrylate that precipitate attack are
minimize the risk of an asthmatic attack best avoided. Ensure that acrylic appliances are cured prior to insertion
Antifungal medications should be prescribed in patients on inhalational Drug interactions with theophylline are common (macrolide antibiotics
corticosteroids increase the level of theophylline, phenobarbitals may reduce the level,
tetracycline have been associated with more accentuated side effects
when given together with theophylline)
Steroid prophylaxis should be advised in patients who are on long-term Avoid aspirin and other non-steroidal anti-inflammatory agents as they
systemic corticosteroids might cause allergic reactions
Use stress-reducing techniques, Conscious sedation should be Oxygen and bronchodilators should be available in case of an
performed without bronchoconstrictions (hydroxyzine) exacerbation of asthma
Nitrous oxide can be used in mild cases In severe asthmatics, nitrous oxide may irritate the airways
Local anesthetics containing epinephrine may be used. However, In some asthmatic patients there may be an interactions between
preservatives such as sodium metabisulfite should be avoided as they epinephrine and 2-agonists that may producing increased blood
may exacerbate an asthmatic attack pressure and arrhythmias
Judicious use of rubber dams prevents reduced breathing capability
Care should be taken in the positioning of suction tips as they may elicit
a cough reflex
During an acute asthmatic attack, discontinue the dental procedure,
remove all intraoral devices, place the patient in a comfortable position,
make sure the airway is opened, and administer a 2-agonist and oxygen
and if there is no improvement, administer epinephrine subcutaneously
(1:1,000 concentration, 0.01 mg/kg of body weight, up to a maximum
of 0.3 mg) and avail medical assistance
Clinical features Patients should ideally be seated upright for the treat-
ment. Rubber dam should be avoided as it can further
Early morning productive cough, dyspnea and orthopnea
compromise breathing. Drugs to be avoided include IV bar-
are common clinical features. Dyspnea leads to reduced
biturates, diazepam and midazolam as they cause respiratory
oxygen saturation, carbon dioxide build-up and respira-
depression.
tory failure or cor pulmonale (right sided heart failure).
General anesthesia is best avoided. Wherever possible
Some patients with emphysema try their best to main-
all treatment should be performed under local anesthesia
tain normal levels of blood gases by hyperventilating. These
without epinephrine. Bilateral mandibular nerve blocks
patients are referred to as ‘pink panters or pink puffers’.
should be avoided.
However, patients suffering from chronic bronchitis cannot
hyperventilate and become hypoxic and hypercapneic (high
levels of carbon dioxide in blood). Such a state of hypox- Management
emia results in central cyanosis in association with elevated Patients should be advised to abstain from exposure to
jugular venous pressure giving rise to a blue bloated appear- smoke. Expectorants and humid environment will hasten
ance. These patients are referred to as ‘blue bloaters’. the recovery process. Bronchodilators (methylxanthines—
theophylline, aminophyline), sympathomimetic agents
(isoproterenol, ephedrine, salbutamol) and corticosteroids
Patients suffering from emphysema may present with xero- are effectively used. Acute infective phases can be managed
stomia. Occasionally, tobacco related mucosal lesions may with antibiotics. Digitalis and diuretics are used for cor
be seen. pulmonale.
510
Cystic Fibrosis MALIGNANT DISORDERS

Cystic fibrosis (CF) is an autosomal recessive genetic dis-
order that affects the functioning of nearly all of the Bronchogenic Carcinoma
body’s exocrine glands. The pulmonary manifestations are It is estimated that almost 95% of all primary lung tumors
characterized by repeated endobronchial infections, an are bronchogenic carcinomas. These carcinomas may arise
exaggerated inflammatory response, airways obstruction, from the bronchial epithelium or the mucous glands.
and bronchiectasis. It is estimated that the incidence of CF Cigarette smoking is considered as the single most impor-
is approximately 1 in 3,500 live births. tant etiological factor in the production of lung cancer.
Other predisposing factors include: occupation related expo-
Pathogenesis of cystic fibrosis sure to asbestos, chromium and cadmium, and exposure to
radiation.
Felix (2009) describes the current concept in the evolution
of cystic fibrosis. The CF gene defect leads to an absent
or malfunctioning cystic fibrosis transmembrane conduc- Clinical features
tance regulator (CFTR) protein, which results in abnormal The earliest clinical signs and symptoms include: cough,
chloride conductance on the apical membrane of the epi- breathlessness, pleural pain and hemoptysis. Local infil-
thelial cell. In the lung, this results in airway surface liquid tration of the tumor leads to pleural effusion, brachial neu-
depletion and, since airway surface liquid is essential to ritis and recurrent laryngeal nerve palsy (produces bovine
support ciliary stability and functioning, ciliary collapse cough). When the superior vena cava is obstructed facial
and decreased mucociliary transport. The consequence of cyanosis and edema is seen. Esophageal obstruction may
this is a vicious circle of phlegm retention, infection, and cause dysphagia.
inflammation. Distant metastasis is characterized by symptoms of las-
situde, anorexia and weight loss.
Clinical features specific to respiratory system A wide spectrum of clinical features is appreciated based
on the site of metastasis. Common metastatic sites include
Patients present with a chronic or recurrent cough, which bones, liver, adrenal glands, lymph nodes, brain and spinal
can be dry at the beginning and can produce mucoid (early) cord. Palpable lymphadenopathy, particularly in the supra-
and purulent (later) sputum. Prolonged symptoms of bron- clavicular fossa, suggests metastasis.
chiolitis occur in infants. Paroxysmal cough followed by Hepatomegaly is seen when there is hepatic metastasis;
vomiting may occur. Recurrent wheezing, recurrent pneu- cerebral metastasis leads to epilepsy, hemiplegia and dis-
monia, atypical asthma, pneumothorax, hemoptysis, and turbed vision. Bone metastasis results in pain, swelling and
digital clubbing are all complications and may be the initial in severe cases pathological fracture of the bone affected.
manifestation. Dyspnea on exertion, history of chest pain,
recurrent sinusitis, nasal polyps, and hemoptysis may occur.
These patients may have a sweat sodium concentration Dental considerations
in excess of 70 mmol/l. Spirometry can be used to assess Metastasis to the jaw bones is relatively rare. On rare occa-
the lung function. sions, the metastatic lesion may appear as a soft tissue swell-
ing or pigmented lesion of the oral mucosa. Abraham et al
Dental considerations (2003) described facial pain as the presenting symptom of
lung carcinoma. They reported 31 cases with a symptom of
Swelling of submandibular gland or parotid gland may be
unilateral facial pain, although there was no metastatic
seen. Tooth eruption may be delayed. Enamel hypoplasia is
lesion in the mandible. The most frequent locations of pain
a common feature. Pancreatin, which is used in the treatment
were the ear, the jaw and the temporal region, 61% occurring
of CF can cause oral ulcerations. Extensive use of antibiot-
on the right and 39% on the left side. The pain resolved or
ics and steroids can predispose to oral candidiasis. The low
partially remitted in most patients following treatment of
fat, high carbohydrate diet will lead to carious teeth.
the carcinoma. They suggested that this pattern of referred
pain from the chest to the face presumably involves either
direct tumor invasion or compression of the vagus nerve by
GRANULOMATOUS DISEASES malignant lymph nodes. The vagus nerve, which contains
motor, visceral and somatic afferent and parasympathetic
Clinical features and oral manifestations of tuberculosis fibers, innervates both thoracic and cranial structures.
and sarcoidosis have been described in Chapter 21 on General anesthesia is best avoided as the respiratory
Granulomatous Diseases. function is compromised.
511
OTHER RESPIRATORY DISEASES inspiratory collapse of the walls of the pharynx, which
determines the complete closure (apnea) or partial closure
Legionnaire’s Disease (Legionellosis) (hypopnea) of the airway. Apneas or hypoapneas are of
varying duration and have distinctive effects on cardiore-
Bacteria of the Legionella species are small gram-negative spiratory homeostasis. Its repetition during sleep, some-
rods that belong to family Legionellaceae. These intracellu- times several hundred times in one night, and day after
lar pathogens develop in the alveolar macrophages, and less day for years, ends up producing significant alterations in
frequently in other macrophages, monocytes and leukocytes. the central nervous system, in myocardial and cerebral cir-
Legionella commonly occurs in natural and artificial culation and in pulmonary and systemic circulation.
water reservoirs, less often in soil and organic matter.
Its proliferation is favored by the water temperature of Clinical manifestations
25–42C, the presence of algae or protozoa, and calcium Snoring is the most common symptom. It occurs due to the
or magnesium salt-containing sediments. narrowing of the pharynx and vibration of the soft parts
The source of infection may be air-conditioning sys- of the upper airway (the pharyngeal walls, the veil of the
tems, showers, fountains, dental unit waterlines and other palate and the uvula).
sources producing water mist. The main daytime manifestations are xerostomia, gas-
Infection occurs by the inhalation of bacteria-laden troesophageal reflux, impotence, irritability, depression,
water droplet aerosol or dust. Incubation time is 2–10 days. decreased libido, non-restorative sleep, concentration dif-
The Legionellaceae comprises more than 45 species but ficulties and headaches. While nocturnal manifestations
Legionella pneumophila is isolated from 90% of culture include diaphoresis, xerostomia, salivation, altered sleep-
proven clinical cases of Legionnaire’s disease. patterns, awareness of apnea and sensation of suffocation
Infection with the Legionella rod causes legionellosis. or panting.
Three clinical types of legionellosis can be distinguished: These patients are usually associated with neuropsychi-
1. Sporadic or epidemic infection in the form of legionel- atric and cardiorespiratory disorders (arterial hypertension,
losis pneumonia, described as Legionnaire’s disease cardiac insufficiency, bradycardia and nocturnal arrhyth-
2. Pontiac fever—a flu-like form having a mild course mias, dilated myocardiopathy, pulmonary hypertension and
3. An extra-lung form in immunosuppressed patients, ischemia).
often taking a severe clinical course, with the septic
syndrome, coagulation disorders, acute cardiovascular Orofacial features and management
deficiency and nephritis. The most common mouth and facial characteristics found
include retrognathic jaw, a narrow palate, a wide neck,
Diagnosis a deviated nasal septum and relative macroglossia.
Orthognathic surgery and uvuloplastopharyngoplasty
Legionellosis diagnostics is based on serological studies of
may be useful. The treatment of choice for OSAS is with
the blood serum to indicate the level of antibodies, on the
continuous positive upper airway pressure (CPAP). This can
patient’s urinalysis to determine the presence of a specific
be achieved using a nasal mask at night. A specific level of
antigen with the immunoenzymatic ELISA and radioim-
pressure is applied in the upper airway, preventing its col-
munological (RIA) tests and on the bacteriological exami-
lapse providing a mechanical widening of the upper airway.
nation of the bronchial tree secretion, bronchoalveolar
Medroxyprogesterone acetate, almitrine, protriptyline
washings, lung biopsy material and sputum.
and theophylline are some of the pharmaceuticals tested so
far. However, these are not very effective. Intraoral appliance
Dental considerations was first used in the 1980s. The mandibular advancement
High- and low-speed handpieces, ultrasonic instruments and devices (MAD) in their two versions (fixed advancement
air–water syringes produce air-water aerosols, which may and adjustable advancement) are really efficient for man-
be source of infection. Both the dental team and the patient aging obstructive problems of the upper airway. The MADs
are exposed to the infected aerosols via inhalation. By inhal- carry out an anterior and inferior movement of the jaw
ing them and choking, the air–water aerosol with the drop- generating anatomical variations in the upper airway facil-
lets of 0.2–5.0 m in diameter, can contain Legionella. itating an increase in the pharyngeal area.
Obstructive Sleep Apnea Syndrome

RENAL DISORDERS
Obstructive sleep apnea syndrome (OSAS) is a disorder that
derives from the intermittent and repetitive occlusion of ‘Homeostasis’, a term introduced by WE Cannon, which
the upper airway during sleep. This occlusion is due to the means the maintenance of constant internal environment.
512
The internal environment in the body is the extracellular Renal failure is defined as the stage of renal function in
fluid in which the cells live. This fluid is present outside the which the kidney is no longer able to maintain the integrity
cell and it constantly moves throughout the body. It includes of the internal environment of the organism. Chugh (1999)
the blood which circulates in the vascular system and fluid defined renal failure as the deterioration of renal function
present in between the cells which is called interstitial resulting in decline in glomerular filtration rate and rise in
fluid. This fluid should be maintained relatively constant urea and non-nitrogenous substances in the blood.
in composition for the normal functioning of the cells, since
changes in the extracellular fluid are reflected in changes
Acute Renal Failure
in fluid within the cells and in the cell function.
Homeostasis is essential to maintain the normal physi- Any condition that interferes with kidney function can cause
ological activities in the body. So, whenever there is threat acute renal failure. In this condition, the kidney stops work-
to alter the physiologic activities, various systems of the ing entirely or almost entirely. In chronic renal failure large
body try to regulate and adjust the functions in such a way numbers of nephrons are destroyed progressively until the
that the condition is brought back to normal. kidney simply cannot perform normal function.
The human kidney which is an important excretory organ, Vascular or glomerular lesion cause hypertension but not
situated on the posterior wall of the abdomen, one on each side renal failure is referred to a hypertensive kidney disease.
of the vertebral column, plays a significant role in the main- In nephrotic syndrome, glomeruli have become more
tenance of body homeostasis through its various functions. permeable than normal so that large amounts of protein
are lost into the urine. Specific tubular abnormalities are
Functions of Kidney characterized by abnormal reabsorption or lack of reabsorp-
tion of certain substances by the tubules.
1. It excretes waste products, especially the nitrogenous and Many diseases which affect the kidney are associated
sulfur containing end products of protein metabolism. with alterations in the mouth or influence the course of
2. It helps to maintain the normal hydrogen ion concen- the common dental disease or affect the mode of treatment
tration of body fluids and electrolytes. or can cause complications. Severe renal disease especially
3. It eliminates drugs and various toxic substances from chronic renal failure can play havoc with the skeletal
the body. structure including the maxilla and mandible.
4. Regulations of arterial pressure by renin–angiotensin
system, renin secreted by juxta glomerular apparatus
converts angiotensin I to angiotensin II which brings Chronic Renal Failure
about elevation of blood pressure by vasoconstriction. Physicians earlier used to use the diagnostic term ‘chronic
5. It helps in the regulation of erythropoiesis through the Bright’s disease’ for common end point of diffuse, severe
formation of erythropoietin secreted by glomerular cells. renal parenchymal disease. However, now the term ‘chronic
6. It plays an important role in vitamin D metabolism, renal failure’ or ‘end-stage renal disease’ (ESRD) is preferred.
as it brings about hydroxylation of inactive form of Davidson (1994) stated that chronic renal failure is irre-
vitamin D and converts to active metabolite which versible deterioration in renal function. Scott (1996) defined
helps in calcium absorption by intestine. ESRD as a chronic, progressive disease that is characterized
Nephrons that are about 1 million per kidney are the func- by the destruction of nephrons, the kidney’s functional unit.
tional unit of the kidney that helps to filter wastes from Chugh (1999) defined chronic renal failure as slow insid-
blood, modulate excretion of salts and water from the body ious irreversible deterioration of renal functions resulting
and allow the kidney to perform its excretory, metabolic in the development of clinical syndrome of uremia mani-
and endocrine functions. Since, the nephrons are incapable fested by excretory, metabolic, neurological, hematological
of regeneration, renal function is maintained until approxi- and endocrinal abnormalities.
mately half of the nephrons are destroyed. When the kid-
ney’s compensatory mechanisms are overwhelmed, signs Etiology
and symptoms of renal failure begins to manifest. Chronic renal failure may be caused by any condition which
destroys the normal structure and function of the kidney.
RENAL DISEASES Harrison (1983) stated that glomerulonephritis, tubuloin-
terstitial disease, diabetic nephropathy and nephro-sclerosis
are among the most common cause of chronic renal failure.
1. Acute renal failure
Guyton (1991) has given the causes for chronic renal
2. Chronic renal failure
failure as:
3. Hypertensive kidney disease
4. Nephrotic syndrome ❍ Chronic glomerulonephritis
5. Specific tubular abnormalities. ❍ Traumatic loss of kidney tissue
513
❍ Congenital absence of kidney tissue b. Hiccups

❍ Congenital polycystic disease (in which large cysts c. Nausea
develop in the kidney and destroy surrounding neph- d. Vomiting
rons by compressions) e. Uremic gastroenteritis
❍ Urinary tract obstruction resulting from renal stones 2. Neuromuscular
❍ Pyelonephritis and disease of the renal vasculature. a. Drowsiness
b. Lack of concentration
Pathogenesis c. Insomnia
The various by-products of protein and amino acid metab- d. Loss of memory
olism in a patient with uremia exert toxic effect on every e. Mild behavioral changes
organ systems in the body. As the kidney fails, the neph- f. Errors in judgment
ron population falls. If this progresses the glomerular fil- g. Neuromuscular irritability
tration rate falls, and the blood urea nitrogen (BUN) rises, h. Seizures and coma
which results in mild azotemia (abnormal retention of nitro- i. Peripheral neuropathy
gen products in blood), impaired ability to concentrate urine, 3. Hematologic
nocturia and mild anemia. If this continues, frank renal a. Normochromic–normocytic anemia
disease follows with its associated polyuria. The anemia may b. Bleeding
become severe and hypocalcemia, hyperphosphatemia and 4. Immunologic
metabolic acidosis may occur. When azotemia is associ- a. Infection
ated with adverse clinical signs and symptoms, it is called 5. Endocrine–metabolic
uremia. Advanced uremia is associated with derangement a. Loss of libido
of function in many major organ systems. b. Decreased thyroid function
Mirahamadi et al stated that the prolongation of life in c. Amenorrhea
patients with end-stage uremia by the use of maintenance 6. Cardiovascular
hemodialysis has resulted in the occurrence of a number a. Hypertension.
of clinical syndromes that may be related to the persis- Gastrointestinal The GI tract is extensively affected by
tence of certain biochemical abnormalities associated with chronic renal failure. Anorexia, hiccups, nausea and vom-
impaired renal function. iting are common and early manifestations of uremia.
According to Harrison, urea represents some 80% or more ‘Uremic fetor’, a uriniferous odor to the breath, derives
of the total nitrogen excreted into urine in patients with from the breakdown of urea in saliva to ammonia and was
chronic renal failure maintained on diets containing 40 g associated with unpleasant taste sensation. Mucosal ulcer-
or more of protein per day. Creatinine may cause adverse ations leading to blood loss can occur at any level of GI
effects in uremic subjects following conversion to more tract in very late stage of chronic renal failure—so-called
toxic metabolites such as sarcosine and methyl guanidine. ‘uremic gastroenteritis’.
Davidson has said that disturbances in water, electrolyte
and acid–base balance undoubtedly contribute to the clin- Neuromuscular Subtle disturbances of CNS function
ical picture in patients with chronic renal failure, but the including inability to concentrate, drowsiness and insomnia
exact pathogenesis of the clinical syndrome of uremia is are among the earliest symptoms of uremia. Mild behav-
unknown. Almost any substance present in abnormal con- ioral changes, loss of memory and errors in judgment soon
centration in the plasma has been suspected of being a follows and are associated with signs of neuromuscular
‘uremic toxin’. It is most likely that the syndrome is caused irritability, including hiccups, cramps and fasciculation
by accumulation in body fluids of a number of substances, and twitching of large muscle groups. Asterixis, myoclo-
among which phosphate, parathyroid hormone, urea cre- nus and chorea are common in terminal uremia, as are
atinine, guanidine, phenols and indoles must be included. stupor, seizures and coma. Peripheral neuropathy may also
Scott stated that uremic syndrome primarily results from be a common complication of advanced chronic renal
the retention and accumulation of excretory products and failure.
the diminished endocrine and metabolic functions of the Cohen stated that neuromuscular signs and symptoms
kidney. He has given the following laboratory findings in were secondary to hypertensive encephalopathy, electrolyte
progressive renal disease. disturbances and hypocalcemia. He observed that along with
neurological hyperirritability, peripheral neuropathy can
Systemic manifestations of renal failure occur as a result of disturbance of the conduction mecha-
nism rather than a loss of nerve fiber. He noticed that the
Systemic manifestations: predominant patient complaint was ‘burning feet’ or par-
1. Gastrointestinal esthesia that may progress to muscle weakness, atrophy
a. Anorexia and finally paralysis.
514
Hematologic Patients with chronic renal dysfunction will up to 90% of renal patients will show oral symptoms. With
often have hematologic problems, most commonly anemia renal insufficiency and uremia, patients may complain of
and bleeding. a bad odor and metallic taste in the mouth. This is due to
Normochromic–normocytic anemia occurs regularly in high urea content in saliva and its subsequent breakdown
chronic renal failure and contributes to fatigability and list- to ammonia. Patients also complain of dry mouth and often
lessness in these patients. Erythropoiesis may be depressed are prone to retrograde parotitis. These complications are
due to the effects of retained toxins on bone marrow and believed to result from a combination of direct gland
diminished biosynthesis of erythropoietin by the diseased involvement, chemical inflammation, dehydration and
kidney. Hemolysis occurs because of hypertension, reten- mouth breathing.
tion of waste products, altered body fluid pH and electro- Frerichs (1851) was the first to describe the oral mani-
lytes composition which create an unsuitable environment festation of uremia. He stated that the condition was not
for erythrocytes. Abnormal hemostasis, i.e. bleeding into found in acute renal failure but developed slowly when
GI tract, pericardial sac and intracranial vault, in the form chronic uremia has been present for months or years.
of subdural hematoma or intracerebral hemorrhage, has Lancereaux (1887) was the first to mention that uremic
been attributed to intrinsic coagulation defect, a deficiency stomatitis was a complication of uremia.
of platelet factor III or the anticoagulants used in conjunc- Jones and Mason (1990) mentioned the oral lesions
tion with dialysis. which can occur in renal failure as follows:
Immunologic Enhanced susceptibility to infection occurs 1. Erythematopultaceous stomatitis
in chronic renal failure due to defect in leukocyte formation 2. Ulcerative stomatitis
and function. Mucosal barrier to infection may be defec- 3. Hemorrhage
tive. Anti-inflammatory steroids and immunosuppressive 4 Hyperkeratosis and generalized pallor
drugs add further risk of infection. 5. Poor taste perception
Cohen has quoted reduced immune capacity in uremic 6. Infection
patients due to uremic intoxication and protein caloric mal- 7. Peripheral giant cell lesion.
nutrition. He stated that uremic plasma suppresses lym-
Several mechanisms have been postulated for the patho-
phocyte responses such as granulocyte dysfunction and
genesis of each form of uremic stomatitis. The oral lesion
suppressed cell-mediated immunity. He suggested, together
may be a reaction to toxins in the tissues or to the action
these impairments place uremic patient at a high risk of
of ammonia or to irritating ammonium compounds formed
infection.
by the action of bacteria on urea. The manifestation of
Endocrine-metabolic Number of hormonal abnormali- uremia in the mouth and GI tract may also be caused by
ties including loss of libido in both the sexes, are due to the hemorrhagic diathesis common in uremia. Local hem-
the associated hyperprolactinemia. Thyroid function was orrhage may cause a decrease in vitality and viability of
diminished. Amenorrhea was common in female. the affected tissues allowing bacterial infection. These
Cardiovascular Hypertension develops in 80–90% of infections result in ulceration and psuedomembranous
patients during the course of chronic renal failure. The hyper- formation.
tension contributes to the development of cardiomyopthy, Wysocki et al presented a case of primary hyperoxaluria
atherosclerosis and progression of the renal failure itself. with oxalosis (accumulation of related deposits in various
Pericarditis was very common in untreated end stage renal extra-renal sites) involving oral tissues, including bone,
failure. Vascular calcification may develop and be suffi- gingiva, dental pulp, periodontal ligament and dentin in a
ciently severe to cause inadequate perfusion of the limbs. 27-year-old male patient.
Goldstein (1990) quoted Baries classification of uremic
Dermatologic Severe pruritus was one of the important stomatitis as follows:
manifestations of the clinical syndrome of hyperparathyroid-
ism in patients with advanced uremia. The skin of the uremic Type I Erythematopultaceous, initially manifests as a red
patients may be dry and there may be ecchymosis resulting thickening of the buccal mucosa, which later includes a
from bleeding tendencies. Pruritus has been attributed to cal- gray, thick, pasty, gluey exudate and pseudomembrane
cium and phosphate deposition in the skin and high circulat- which covers the gingiva, fauces and the oral mucosa. When
ing PTH levels. In advanced uremia a white, uremic ‘frost’ the psuedomembrane is removed with tongue blade, a
may evaporate on the skin surface with a fishy scale odor. swollen, dry, red but not ulcerated mucosa is found. Asso-
The patient frequently complains of brittle nails. ciated manifestations include fetor oris, dry burning sen-
sation, excessive saliva and perversion of taste.
Oral manifestations
Type II Ulcerative form which is similar to type I but
Several changes occur in the oral cavity that is associated includes loss of integrity of the mucosa with frank ulcer-
with chronic renal failure and uremia. It was estimated that ation. The ulcers can be superficial or deep and frequently
515
involve the gingiva. Purpura and anemia may be seen on to balance the systems, thus creating the skeletal lesions.
the mucosa. Excessive salivation is again noted. This increase in parathyroid activity is secondary to altered
Parotid and submandibular gland swelling may be seen serum calcium and phosphate levels and is therefore known
in patients with chronic renal failure without accompany- as secondary hyperparathyroidism.
ing uremic stomatitis. The skeletal changes occurring secondary to chronic
Ziccardi stated that restricted fluid intake results in renal failure consist of bone remodeling, osteomalacia,
xerostomia, the most common oral manifestation in CRF ostitis fibrosa cystica and osteosclerosis.
(circulating recombinant form) patients. Kelly (1980) quoted Lucas (1883) who stated that abnor-
McCreary et al reported lesion mimicking oral hairy malities of the skeletal system can occur in children with
leukoplakia in a case of uremic stomatitis in a 48-year-old renal disease and he used the term ‘renal rickets’ to describe
male with an extensive white lesion involving dorsal, lat- the condition.
eral and ventral surfaces of the tongue and buccal, labial Parsons (1927) described first radiographic interpreta-
and retromolar mucous membrane and suggested that oral tion of secondary hyperparathyroidism. He noted osteopo-
manifestation of uremia should be considered in the dif- rosis, bending and fracture of long bones, cortical thinning
ferential diagnosis of oral hairy leukoplakia. and subperiosteal resorption.
Wysocki et al reported a case of 31-year-old male suf- The skeletal changes of primary and secondary forms of
fering from chronic renal failure with oxalosis in which hyperparathyroidism are indistinguishable from each other.
they found tooth resorption, mobility, dental pain and But ‘brown’ tumors which appear as well-circumscribed uni-
eventual tooth loss as significant clinical problems. locular or multilocular radiolucencies are rare in secondary
Sowell stated that uremia present during development of hyperparathyroidism.
the dentition results in teeth with enamel hypoplasia and Kelly et al (1980) reported that decreased density of bone,
brownish discoloration. Altered maxillary and mandibular thinning and absence of cortices and fractures and defor-
growth and resulting malocclusion may occur because of mities of the weight bearing skeleton, periarticular, soft
impaired skeletal growth. tissue and vascular calcification are common in secondary
Woodhead et al (1982) in a review article of Clark hyperparathyroidism. They suggested that alterations of
(1987) found that enamel hypoplasia was the most com- the trabecular pattern (Figure 5) have led to qualitative
mon finding in chronic renal failure. They observed the terms such as ‘chalky’, ‘ground glass’, ‘granular’ and ‘salt
anatomical location of enamel hypoplasia on the teeth and pepper’ to describe the appearance of the bone.
correlated well with the age of onset of advanced renal
failure. Other less frequently observed changes included
Dental manifestations
discoloration and hypocalcification of the enamel.
Scott stated that some patient may have severe erosion Radiographic alterations of the jaw bones in chronic renal
of the dentition due to frequent regurgitation, resulting from disease are not uncommon and often represent one of the
the nausea associated with hemodialysis treatments. Other
dental findings include tooth mobility and drifting without
appreciable pathological periodontal defects and abnormal Figure 5
bone healing following dental extraction.
Radiographic manifestations
Liu and Chu (1943) coined the term ‘renal osteodystrophy’,
which denotes osseous changes that occur in patients with
ESRD.
Skeletal changes are caused by the disorders in calcium
and phosphorus metabolism, abnormal vitamin D metabo-
lism, and increased parathyroid activity. In chronic renal
failure, intestinal absorption of calcium is reduced because
the kidneys are unable to convert vitamin D into its active
form 1,25-dihydroxycholecalciferol which is required for
absorption of calcium from the digestive tract. Impaired
absorption of calcium because of defective kidney func-
tion and corresponding retention of phosphate causes a
decrease in the serum calcium level. This is associated with
Intraoral periapical radiograph showing altered
a compensatory hyperactivity of the parathyroid glands.
trabecular pattern. Courtesy: Dr Veena Hegde
The parathyroid hormone then extracts calcium from bone
516
earliest detectable signs. Subperiosteal bone resorption may

Figure 6
be seen as a partial or complete loss of the lamina dura,
thinning of the mental foramen, inferior alveolar canal
resorption and destruction of condylar process of the man-
dible and maxillary sinus floor. In addition, the trabecular
pattern of the affected bone assumes a ‘ground glass’ or ‘salt
and pepper’ appearance. Infrequently, radiolucent brown
tumors may be seen.
Fordham and Franklin (1963) reported the first case
associated with ESRD in a 2-year-old boy with a brown
tumor of maxilla which is clinically, chemically, radio-
logically and according to parathyroid findings consistent
with secondary parathyroid hyperplasia. They observed
these changes mostly in the lower molar area superior to
the mandibular canal.
Spolnik et al found 73% of their dialysis patients’ dental
radiographs were abnormal. He found altered bone density
as the most common abnormality and suggested its diag-
nostic value in the evaluation of bone disease. Photograph showing a patient undergoing
Maxwell et al examined 30 male chronic hemodialysis hemodialysis. Courtesy: Dr Veena Hegde
patients using panoramic and periapical radiographs and
found 22 patients with abnormal jaw radiographs. They
between the patient’s plasma and dialysate (the electrolyte
reported pulpal narrowing and calcification in half of dial-
composition of which mimics that of extracellular fluid)
ysis patients. They concluded that dental radiography was
across a semi-permeable membrane that allows uremic tox-
as good a screening technique for evaluating renal osteo-
ins to diffuse out of the plasma while retaining the formed
dystrophy as hand radiography and in many cases, was
elements and protein composition of blood. The usual dial-
superior to axial skeleton radiography.
ysis system consists of a dialyzer, dialysate production unit,
Scott et al stated that the manifestations of metabolic
roller blood pump, heparin rate, and pressure of dialysate
renal osteodystrophy and compensatory hyperparathyroid-
and to detect blood leaks and arterial and venous pressures.
ism of the mandible and maxilla include bone demineral-
The typical patient undergoes hemodialysis 3 times per
ization, decreased trabeculation, ‘ground glass’ appearance,
week, with each treatment lasting approximately 4 hours.
loss of lamina dura, radiolucent giant cell lesions and met-
During treatment anticoagulants are administered.
astatic soft tissue calcifications. He further suggested that
It was estimated that 60% of hemodialysis patients
with such bone loss, it was not uncommon for patients to
need dental treatment to prevent the complications caused
have spontaneous fractures of the jaws from trauma as
by infection or dietary difficulties. Comprehensive oral
well as to be at risk of fracture during oral and periodontal
evaluation and dental treatment are part of the basic
surgical procedure.
healthcare of the chronic renal failure patients. As the
technology and medicine advance, the number of patients
Dental considerations with chronic renal disease who require dental care is also
increasing.
Dental treatment for patients with chronic renal disease
The following considerations are important when eval-
and transplants is recognized as an essential health service.
uating and treating the dental patient with renal disease:
Dental management is complicated because of the oral dis-
ease and the medical problems seen in this population. These 1. Timing Disagreement exists over the ideal time for
patients survive under narrowly controlled conditions of dental treatment of hemodialysis patients. Carl and Wood
intake and activity as well as under great physiologic and indicated that the dental treatment should be performed
psychologic stress with medications, diet, invasive treat- first before hemodialysis, when the patient is free of anti-
ments, limited lifestyle and dependency on others. Dental coagulant. Any problems arising from medications given
treatment, even minor dental procedures can present major by the dentists will be removed later by dialysis.
problems and should be considered particularly stressful Sowell, Manton, Ziccardi, and Scott all recommend that
when superimposed on such situation. dental treatment should be timed so that the effects of
Hemodialysis (Figure 6) is the most prevalent therapy dialysis are present, but the effects of heparin have worn
for chronic renal disease. Hemodialysis refers to the removal off. This enables clotting to be well established before the
of nitrogenous and toxic products of metabolism from the next dialysis session, when heparin will again be adminis-
blood by means of a dialysis system. Exchanges occur tered. They suggested treatment midway between dialysis
517
sessions ideally about 12 hours post dialysis is the ideal atrophy. Supplemental steroids should be considered before
time. Because at this time, the toxins are low in the blood, stressed dental procedures.
the effect of the heparin is minimal, and the patient is best
5. Medications and drug therapy for dental purposes
able to respond to the stress of treatment.
The practitioner prescribing or administering medications
2. Asepsis Scott stated that because patients undergoing to patients with compromised renal function must be famil-
dialysis are exposed to a large number of transfusions and iar with the metabolism and excretion of each of the
blood exchanges, as well as renal failure related immuno- agents involved and the functional status of the individual
suppression, they are at greater risk of infections such as patient’s kidneys. To provide safe yet effective drug therapy,
hepatitis B and C and HIV infection. He suggested that modification of the usual dosage regimen may be necessary.
these patients should be encouraged to undergo periodic For drug administration for dental purposes, two dosage
testing for hepatitis infectivity and HIV antibody. categories are sufficient: (i) patients with mild to moderate
The presence of these infections do not contraindicate renal failure or renal insufficiency and (ii) patients with
dental treatment, but rather indicate the need for special severe renal failure or who are functionally anephric. One
care and meticulous techniques. of the commonly used drug in dentistry is penicillin, which
exists as either a sodium or potassium salt. Potassium salts
3. Hematologic and physiologic status Clinical and lab- should be avoided due to the potential of hyperkalemia.
oratory evaluation for anemia, bleeding disorders and leu- Other alternative drugs such as clindamycin or erythromy-
kocyte disorder may be indicated, depending on the status cin, which are metabolized by the liver, can be considered.
of renal disease or therapeutic modality and the type of NSAIDs should be avoided because of their nephrotoxic
dental treatment advised. Evaluation and stabilization of effect. However, NSAIDs can be used once these patients
fluid and electrolyte balance should be accomplished before progress to ESRD and no longer have any residual renal
dental treatment. function.
The arteriovenous site should never be jeopardized.
6. Local anesthesia The kidney is the main excretory
That arm should never be used for injection of medication,
organ for all the local anesthetics commonly used in den-
either intramuscularly or intravenously, nor should the
tistry and their metabolites. The use of cocaine is contrain-
access site be used as an injection site. It is also essential
dicated since it is excreted entirely unchanged in the urine.
to avoid any factor that may occlude the arteriovenous
Goldstein recommended that slow administration of LA
shunt such as a blood pressure cuff, constricting clothing
not more than 25% of the maximum total recommended
or cramped arm position. If the device is in the leg, the
dosage for the normal patient, is a practical and safe guide-
patient should be allowed to stand or walk occasionally
line for local anesthetic injections for dental purposes in
during long procedure.
the medically controlled patient with absent renal function.
Dental procedures should be delayed for at least 2 weeks
after placement or revision of a vascular access and should
be avoided during periods of infection or thrombosis of Uremic Stomatitis
the access.
Uremic stomatitis has become relatively rare, seen mostly
4. Immunosuppression and corticosteroid medication in cases of undiagnosed and untreated chronic renal failure.
Many renal transplant patients are on a regimen of anti- Painful plaques and crusts are distributed predominantly
inflammatory and immunosuppressive medications. Because on the buccal mucosa, the floor or dorsum of the tongue,
infections are the major cause of morbidity and death in and the floor of the mouth. The incidence has decreased
the renal transplant patient and a majority of septicemic because of readily available dialysis centers throughout the
infection have been attributed to oral diseases such as peri- country. The most commonly accepted mechanism behind
odontitis, pulpal infection and oral ulceration along with the development of uremic stomatitis is irritation and chem-
dental treatment which provide a convenient portal of ical injury of mucosa by ammonia or ammonium com-
entry for microorganism into the circulatory system. pounds formed by the hydrolysis of urea in saliva by urease.
Scott stated that the patients with chronic renal failure This occurs when the intraoral concentration of urea exceeds
who receive hemodialysis have an increased susceptibility 30 mmol/l. Hemorrhagic diathesis from inhibited platelet
to the development of infective endocarditis. They suggested aggregation may also play a role due to local hemorrhage,
antibiotic prophylaxis should be given prior to dental care resulting in decreased vitality and viability of the affected
to prevent infective endocarditis, vascular access infection tissues, thus allowing bacterial infection.
and bacteremia in both renal transplant and patient under- There are two predominant types of uremic stomatitis.
going hemodialysis. In Type I, there is a generalized or localized erythema of
Topical antifungal agents are recommended for control the oral mucosa and a thick gray pseudomembranous exu-
of localized oral infections. date which does not leave a bleeding or ulcerated base
Long-term systemic steroid administration suppresses when removed. Additional findings may include pain, burn-
the patient’s ability to respond to stress by causing adrenal ing, xerostomia, halitosis, gingival bleeding, dysgeusia, or
518
candidal infection. Type II leaves ulceration if the pseudo- Patients may complain of dentinal hypersensitivity and
membranous film is removed. This type may indicate a foul taste (dysgeusia). In chronic cases pulpal exposure may
more severe form of stomatitis, secondary infection, ane- be seen. Exposure of the oral mucosa to the acidic reflux
mia or underlying systemic hematologic disturbances caused may result in atrophy and erythema.
by renal failure.
Histologically, both types of uremic stomatitis show Complications
evidence of an intense inflammatory process with heavy
polymorphonuclear leukocytic infiltration and necrosis of It is estimated that approximately 50% of patients with
the oral mucosa. Bacterial colonization is most commonly reflux develop esophagitis. Based on the severity, esophagi-
associated with Fusobacterium, Spirochaeta or Candida. tis is divided into four grades:
1. Grade I—erythema
2. Grade II—linear non-confluent erosions
GASTROINTESTINAL DISORDERS 3. Grade III—circular confluent erosions
4. Grade IV—Barrett’s esophagus.
GASTROESOPHAGEAL REFLUX DISEASE Barrett’s esophagus is characterized by replacement of
normal squamous epithelium with columnar epithelium.
Gastric esophageal reflux is defined as the passage of gas- It has been linked to the development of adenocarcinoma
tric contents into esophagus. It is believed that Helicobacter of the esophagus.
pylori, a spiral shaped bacterium located in the mucous Long-standing esophagitis results in significant amount
layer of the stomach, may inhibit or exacerbate acid reflux. of blood loss and subsequently resulting in iron deficiency
The symptoms that develop when the esophageal mucosa is anemia. Herbst triad, consists of finger clubbing, hypopro-
exposed to the gastric reflux are referred to as gastro- teinemia and iron deficiency anemia associated with gas-
esophageal reflux disease (GERD). It is a common, chronic troesophageal reflux.
gastrointestinal disorder that affects approximately 30%
of the population.
Investigations
Predisposing factors and pathophysiology Gastroesophageal reflux disease is best diagnosed based on
Genetic factors, smoking, lower esophageal sphincter abnor- the symptoms of water brash, heartburn and regurgitation.
malities, hiatal hernia, defective esophageal peristaltic activ- Only 30% of the patients have positive esophageal findings
ity (common in esophagitis) and defective gastric emptying, on endoscopy. Barium esophagographs can be used to diag-
increased abdominal pressure (pregnancy and obesity), and nose hiatal hernia and esophageal strictures.
dietary behavior (diet consisting of fat, coffee, alcohol and
chocolate relax the lower sphincter and incite symptoms). Management
Patients should be encouraged to consume small frequent
Clinical features meals along with antacids instead of large meals. They
Gastroesophageal reflux disease is more common in adults should be educated not to go to bed immediately after a
in their third decade of life. The common symptoms are heavy meal. It has been suggested that the raising of the
heartburn (mimics anginal pain, burning sensation spread- head of the bed by about 4 inches will minimize regurgita-
ing upward from the epigastrium to the neck) and regurgi- tion of the acid contents.
tation. Water brash is also a very common complaint in H2 blockers (cimetidine 400 mg, q.i.d. for 4 weeks;
patients. It is the presence of a sudden burst of salivation ranitidine 150 mg b.i.d. up to 8 weeks), proton pump inhib-
in the mouth due to reflex salivary gland stimulation in itors (omeprazole 20 mg once daily for 4 weeks; lansopra-
response to presence of acid in the gullet. Other clinical zole 30 mg once a day for 4 weeks).
findings are chest pain, cough, laryngitis and laryngeal To reduce dysgeusia associated with acidic reflux, about
polyps. In severe recurrent reflux episodes, aspiration and half teaspoon of baking soda (sodium bicarbonate) can be
pulmonary fibrosis may occur. added to 250 ml of water and this solution can be used as
mouthrinse. Fluoride application will aid in remineraliza-
Oral considerations tion of teeth.
Bargen and Austin (1937) first described the association
between GERD and erosion of teeth. In the initial stages
enamel may exhibit subtle changes. As the condition pro- INFLAMMATORY BOWEL DISEASE
gresses, complete loss of tooth structure may be seen. The
teeth typically affected are the palatal aspect of the upper Inflammatory bowel disease (IBD) encompasses a group of
anteriors and premolars. diseases with poorly defined etiology that affect the digestive
519
tract which includes Crohn’s disease (CD) and ulcerative Oral manifestations and dental considerations
colitis (UC). A significant number of individuals develop
Aphthous ulcers may result from nutritional deficiencies
extra-intestinal manifestations (hepatic manifestations
of iron, folic acid and vitamin B12 due to poor absorption
and primary sclerosing cholangitis).
in the gut and/or blood loss directly related to the ulcer-
Nucleotide oligomerization domain (NOD2) and human
ative colitis. Anti-inflammatory medications such as the
leukocyte antigen (HLA) genes are the most extensively
5-aminosalicylates, which often represent the mainstay of
studied genetic regions (IBD1 and IBD3, respectively) in IBD.
therapy for IBD patients and which are excreted in saliva,
Mutations of the NOD2 gene are associated with CD and
are known to cause aphthous ulcers. Pyoderma gangreno-
several HLA genes are associated with UC and CD.
sum may occur in the form of deep ulcers that sometimes
ulcerate through the tonsillar pillar. Pyostomatitis vegetans,
a purulent inflammation of the mouth, may also occur.
ULCERATIVE COLITIS These oral lesions are characterized by deep-tissue vege-
tating or proliferative lesions that undergo ulceration and
Ulcerative colitis is an IBD affecting part or whole of the then suppuration. These lesions disappear with a total colec-
large intestine frequently lower colon and rectum. It was tomy. Ulcerative colitis patients also can develop hairy leu-
first described by a physician, Sir Samuel Wilks from koplakia, a lesion more commonly associated with AIDS.
London in 1859. The precise etiology of ulcerative colitis Drugs to be avoided include NSAIDs, antibiotics,
is not well understood. A current hypothesis suggests including amoxicillin–clavulanate and clindamycin which
that primary dysregulation of the mucosal immune system could aggravate diarrhea. Surgical treatment is contrain-
leads to an excessive immunologic response to normal dicated until the disease is under control because of the
microflora. risks associated with anemia (such as delayed healing, an
increased risk of infection, the side effects of narcotic
Clinical features analgesics, and depression of respiration). Long-term use
of steroids can also result in adrenal suppression. Patients
Ulcerative colitis is most common between the 2nd–4th undergoing surgery require increased doses of steroids
and 5th–8th decades of life. Males and females are equally before and after the procedure because their own adrenal
affected. The characteristic symptoms and signs include response to stress is blunted.
bloody diarrhea, rectal urgency and difficulty to pass stools.
The frequency of bowel movements and the amount of
blood present reflect the activity of the disease. The diar-
rhea is severe, possibly five to eight bowel movements in
CROHN’S DISEASE
24 hours. Patients usually complain of pain that is in both
abdominal quadrants and that is crampy in nature and exac- Crohn’s disease was first observed by the German surgeon
erbated prior to bowel movement. Along with the change Wilhelm Fabry in 1623, and was later described by and
in the pattern of bowel movements, the patient may have named after physician Dr Burril B Crohn from New York.
nocturnal diarrhea. It is an idiopathic, relapsing chronic inflammatory disease
The extraintestinal manifestations include osteoporosis, of the GI tract categorized as IBD and is an important cause
arthritis, primary sclerosing cholangitis, uveitis, pyoderma of morbidity in children and adolescents.
gangrenosum, deep venous thrombosis, pulmonary embo- The prevailing hypothesis regarding the pathogenesis of
lism. The most serious complication of ulcerative colitis is CD is an interaction between environmental factors and an
carcinoma of the colon. altered immune response in genetically predisposed children,
leading to chronic inflammation of the GI tract. In CD there
is a disordered regulation of mucosal and systemic immune
response resulting in the perpetuation of the inflammatory
Differential diagnosis of ulcerative colitis
cascade. A dysregulated Th1 response (T helper 1) seems to
Disease Clinical characteristics be crucial in the conversion of physiologic to pathologic
Crohn’s colitis Perianal lesions common; bleeding less
inflammation. The immunological profile in CD is predom-
common than in ulcerative colitis inantly a cell-mediated response. Active mucosal inflam-
mation of the small and large intestine results in diarrhea,
Infectious colitis Sudden onset; pathogens present in stool;
pain may be a predominant feature
protein-losing enteropathy, bleeding, abdominal pain and
stricture formation. Proinflammatory cytokines and eico-
Ischemic colitis Affects older age groups; vascular disease
sanoids increase vascular permeability and cause electrolyte
often present; sudden onset, often painful
secretion, and augment smooth muscle contraction. Many
Pseudomembranous Recent antibiotic use; clostridium toxin
cytokines promote the recruitment and activity of collagen
colitis detectable in stool
forming cells leading to fibrous tissue proliferation and
520
thereby resulting in bowel wall thickening and stricture years before the intestinal symptoms. Hepatobiliary com-
formation. The causal role of TNF-
in the etiopathogene- plications such as chronic hepatitis, sclerosing cholangitis,
sis of CD has gained importance. cholelithiasis and elevated aminotransferase may precede
the active disease. The renal manifestations of IBD include
Clinical presentation nephrolithiasis, hydronephrosis and enterovesical fistula.
Other extraintestinal manifestations are thromboembolic
It can occur in all age groups and follows the same bimodal
manifestations, vasculitis, pancreatitis, interstitial pneu-
pattern of age distribution as IBD. Early onset IBD (EOIBD),
monitis and pericarditis, others include weight loss, growth
i.e. presentation before the age of 5 years, is a unique sub-
failure, bone disease (due to malnutrition). Delay in sexual
group constituting 4% of pediatric IBD. In this subset, it is
maturation seen in some children with CD may have a
difficult to differentiate between ulcerative colitis (UC) and
significant effect on self-esteem and socialization.
CD. In UC there seems to be a preponderance of CD in
boys, whereas in India it was more prevalent in girls. CD Oral manifestations
depends upon the site of involvement of the GI tract. It
affects the ileocecal region, typically with ulceration, fis- Aphthous ulcerations are the most common oral manifes-
suring and fibrosis of the wall, but can affect any part of tation of CD. The oral manifestations may occur along with
the GI tract. Abdominal pain and systemic symptoms are intestinal disease or precede it and may be the primary
generally more severe in CD than in UC. A dyspeptic type presentation. Cobble stone appearance of the oral mucosa,
of epigastric pain is seen in children with gastroduodenal oral epithelial tags and folds, gingivitis, persistent lip swell-
involvement. Diarrhea occurs in two-thirds of affected ing, lichenoid mucosal reactions, granulomatous inflam-
children. In children with predominantly ileal involve- mation of minor salivary gland ducts, candidiasis, and
ment, constipation may be a rare presentation. Gross blood angular cheilitis. Some patients may have asymptomatic
in the stools is unusual with isolated small bowel disease intestinal disease. Melkersson–Rosenthal syndrome (facial
and more common when the colon is involved. Fever swelling, facial palsy and fissured tongue) and cheilitis gran-
occurs in approximately 50%. Fatigue, anorexia, weight ulomatosa may also be incomplete manifestation of CD.
loss and diminution in growth velocity are other presenting High prevalence of caries and periodontitis in CD has also
symptoms in children. Perirectal involvement fistulae, fis- been reported.
sure and skin tags are important clues to the diagnosis. CD Differentiating CD from ulcerative colitis can be chal-
is subcategorized as predominantly inflammatory, fistuliz- lenging, particularly early in the course of the disease,
ing or stricturing disease based on the clinical phenotype. but it is an important step because appropriate treatments
Crohn’s disease could represent a persistent infection and potential complications vary for these two conditions
with a fastidious organism or an abnormal and prolonged (Table 2).
response to a common pathogen. Various organisms have
been linked with CD but none of them are evident in the
Table 2 Comparison of ulcerative colitis and Crohn’s disease
etiopathogenesis. In genetically susceptible individuals the
microvascular injury caused by these infections could result Feature Ulcerative colitis Crohn’s disease
in a granulomatous vasculitis of the mesenteric vessels, lead- Sites mainly Ileum Colo-rectum
ing to microvascular thrombosis, multifocal gastrointesti- affected
nal infarction, and finally gross pathologic sequelae such
Abdominal pain Variable Common
as ulcerations, fistulas, fibrosis and strictures.
Depth of Mucosal Transmural
inflammation
Complications
Distribution Diffuse, contiguous Segmental, non-contiguous
Hemorrhage, obstruction, perforation, abscess and fistula spread; always spread (’skip lesions‘); less
formation are well known in CD. Perianal disease may involves rectum; common rectal involvement;
present with abscess formation, perirectal and perianal fis- spares proximal occurs in entire
tulization and can precede the intestinal manifestation by gastrointestinal tract gastrointestinal tract
years. Perforation with internal fistula is another serious Iron deficiency Common Common
complication. Carcinoma of the colon is a long-term com- Other Vitamin B12 –
plication of IBD. The two well-accepted risk factors for complications deficiency
cancer are duration and severity of the disease. Diarrhea Severe Less severe
Extraintestinal manifestations include skin manifesta-
Fistula and Rare Common
tions like erythema nodosum and pyoderma gangreno- sinus tracts
sum. Ocular manifestations such as episcleritis and anterior
Colonic Present High
uveitis are less common. Arthritis is the most common
carcinoma risk
extraintestinal manifestation in children and may occur
521
Investigations to evaluate the entire small bowel and was found to be

superior in detecting CD lesions in patients with mild clin-
The small bowel has been defined for many years as the
ical suspicion and negative traditional imaging studies.
‘black box’ of the gastrointestinal system due to its inac-
Other possible applications of VCE have been suggested
cessibility to endoscopic exploration. The conventional
in a pediatric setting, in the evaluation of post-surgical
radiological methods, i.e. small bowel enteroclysis (SBE)
recurrence and in the surveillance of mucosal healing after
and small bowel follow-through (SBFT), have long been
biologic therapy. The major limitations of the use of VCE
the only imaging methods providing information on the
in CD are an incomplete evaluation of the small bowel,
morphological features of the small bowel valuable in the
occurring in 20–35% of patients, and by the possibility
diagnosis and management of Crohn’s disease. Typical small
of capsule retention, occurring in about 7% of patients
bowel changes which can be observed by means of these
with CD.
techniques include irregular thickening and distortion of
the valvulae conniventes, loops adhesions (mass-like effect) Double balloon endoscopy Double balloon enteroscopy
or separated loops because of wall thickening and mesen- (also called push and pull enteroscopy) was described for
teric inflammatory infiltration. Transverse and longitudinal the first time by Yamamoto et al in 2001. The principle of
distribution of ulcerations can separate islands of thickened the double balloon technique allows not only a complete
internal wall, resulting in the typical cobble stone appear- endoscopic evaluation of the small bowel but also makes
ance. Strictures are often separated by healthy bowel tracts it possible to take biopsies and to carry out therapeutic
(skip lesions); impaired small bowel peristalsis is commonly interventions along the whole small bowel in patients sus-
observed within rigid stenotic tracts. Extrinsic compression pected of CD and negative upper and lower endoscopy.
may be observed, due to mesenteric lymph node enlarge-
ment. Barium meal series is useful to identify small bowel Management
involvement and lesions such as strictures, fistulas and
ulcerations may be identified. Apart from a total proctocolectomy for UC, there is no
cure for IBD. Medications, however, aid in the induction
Bowel ultrasonography The main ultrasonography find- and maintenance of remission, and target various points
ings in CD are represented by thickening and stiffness of along the disordered immune pathway implicated in IBD.
the gut wall, modifications or lack of its echo stratifica- Aminosalicylates remain the standard induction and main-
tion, reduction of peristalsis, mesenteric fibro-fatty prolif- tenance therapies for UC but have a more equivocal role in
eration, lymph node enlargement; in case of complications, CD. Antibiotics are also commonly used in CD, especially
narrowing of the intestinal lumen, abscesses and fistula with colonic and perianal disease. Budesonide is effective
are usually easily detectable. The use of power Doppler as a first-line agent for ileal and/or right colonic CD
methods and of oral (polyethylene glycole, PEG) and/or although maintenance benefits remain to be proven. Con-
intravenous (Levovist) contrast media, have been sug- ventional steroids induce remission for both CD and UC
gested to improve the diagnostic accuracy of ultrasonog- but are reserved for patients with moderate-severe disease
raphy, particularly in discriminating inflammatory from or for those who have failed more first-line therapy. New
fibrotic strictures and in better defining the presence of therapies include anti-TNF
antibodies; recombinant cyto-
internal fistulas. kines and growth factors. Immunomodulators such as 6-MP
Computed tomography Computed tomography (CT) has and azathioprine, as well as methotrexate, are effective
been utilized for the detection of extra-enteric complica- steroid-sparing and maintenance therapies. Cyclosporine
tions of CD, mainly intra-abdominal abscesses, but is also or tacrolimus can be effective for severe or refractory UC.
suitable in the evaluation of strictures, prestenotic dilata- Anti-TNF agents have been effective for patients with
tions and fistulas. Non-enhanced CT scan is also used in moderate-severe UC and CD, independent of concomitant
the diagnosis of post-surgical complications (intra-perito- medications.
neal abscesses, anastomotic dehiscence, extra-abdominal
abscesses and fistulas, incisional hernias, ascites, volvolus,
bowel adhesions, etc). The main findings at CT scan observed HIATAL HERNIA
in CD patients, are small bowel wall stratification and/or
thickening (‘target’ or ‘double halo’ appearance), with or
Hiatal hernia is the herniation of part of the stomach into
without contrast enhancement, edema of the mesenteric fat,
the thoracic cavity through the esophageal hiatus in the
engorged ileal vasa recta (‘comb sign’), submucosal fibro-
diaphragm. Hiatus hernias affect anywhere from 1 to 20%
fatty infiltration and mesenteric adenopathy.
of the population. It is estimated that about 9% present
Video capsule endoscopy Video capsule endoscopy with symptoms. Hiatal hernia is usually seen in the elderly
(VCE) is a potentially safe and painless endoscopic method although people of any age can be affected.
522
Etiology PEPTIC ULCER DISEASE

It is believed that insufficient dietary fiber and the use of the
unnatural sitting position for defecation (need for strain- Peptic ulcer disease of the GI tract is characterized by muco-
ing at stool) may cause increased intra-abdominal pres- sal damage secondary to pepsin and gastric acid secretion.
sure thereby pushing the stomach through the esophageal It usually occurs in the stomach and proximal duodenum;
hiatus in the diaphragm. Other contributing factors caus- less commonly, it occurs in the lower esophagus, the distal
ing increased pressure within the abdomen include: lifting duodenum, or the jejunum, as in unopposed hypersecre-
heavy weights or frequent bending over, frequent or hard tory states such as Zollinger–Ellison syndrome, in hiatal
coughing, hard sneezing, violent vomiting and obesity hernias (Cameron ulcers), or in ectopic gastric mucosa (e.g.
(extra weight pushes down on the abdomen increasing the in Meckel’s diverticulum. It is believed to affect almost 10%
pressure). Other etiological factors are hereditary, smoking, of the adults (usually between 25 and 65 years) at least
stress and drug abuse (cocaine). once in their lifetimes.
Etiology
Clinical features
H. pylori infection and the use of non-steroidal anti-inflam-
The sign and symptoms include heartburn, belching, chest
matory drugs are the most common causes of peptic ulcer
pain and nausea, which tend to become worse on leaning
disease. Other medications that have been known to cause
forward, lifting heavy objects or lying down. In severe cases,
ulcers include steroids, bisphosphonates, potassium chloride
dysphagia and painful swallowing may occur. Infants may
and chemotherapeutic agents (e.g. intravenous fluorouracil).
regurgitate blood stained food and present with difficulty
A variety of other infections and co-morbidities are asso-
in breathing and swallowing. Occasionally pain may radi-
ciated with a greater risk of peptic ulcer disease (e.g. cyto-
ate to the jaw and along the arm mimicking anginal pain.
megalovirus, tuberculosis, Crohn’s disease, hepatic cirrhosis,
Other clinical features are hiccups, dry cough and increase
chronic renal failure, sarcoidosis, myeloproliferative dis-
in the contractile force of the heart.
order). Critical illness, surgery or hypovolemia leading to
There are two major types of hiatus hernia: the sliding
splanchnic hypoperfusion may result in gastroduodenal
hiatus hernia (95%), where the gastroesophageal junction
erosions or ulcers; these may be silent or manifest with
moves above the diaphragm together with some of the
bleeding or perforation. Smoking increases the risk of ulcer
stomach. The second rare type is rolling (or paraesopha-
recurrence and slows healing.
geal) hiatus hernia, when a part of the stomach herniates
through the esophageal hiatus beside, and without move- Clinical features
ment of the gastroesophageal junction. Occasionally, a third
type is described, which is a combination of the first and Typical symptoms of peptic ulcer disease include episodic
second. gnawing or burning epigastric pain; pain occurring 2–5 hours
after meals or on an empty stomach; and nocturnal pain is
Investigations and management relieved by food intake, antacids or antisecretory agents.
A history of episodic or epigastric pain, relief of pain after
Endoscopy is more frequently used than barium studies in food intake, and night time awakening because of pain with
the diagnosis and assessment of hiatal hernia. Manometry relief following food intake are the most specific findings
and esophageal pH testing may provide useful information. for peptic ulcer.
Treatment is indicated if symptoms interfere with day to Less common features include: indigestion, vomiting
day routine of the patient. Weight reduction, diet modifica- (coffee ground vomitus), loss of appetite, intolerance of fatty
tion, raising the head of the bed and reduction of alcohol, foods, heartburn and a positive family history.
caffeine and nicotine intake are all recommended. The serious or alarming symptoms that necessitate further
Routinely, antacids to H2-receptor antagonists and pro- investigations and immediate treatment include: anemia,
ton pump inhibitors are sufficient. Surgery should be con- hematemesis, melena (black tarry stools); vomiting sug-
sidered in severe and resistant cases of hiatal hernia. gests obstruction; anorexia or weight loss suggests cancer;
persisting upper abdominal pain radiating to the back sug-
Dental considerations gests penetration; and severe, spreading upper abdominal
Since many of the medications used for treating hiatal pain suggests perforation.
hernia can cause xerostomia, cervical caries is common. If
Diagnosis
reflux into the oral cavity is present, oral manifestations are
the same as those of GERD. Patients older than 55 years and those with alarming symp-
Patients can be advised to frequently sip water. Salivary toms should be referred for esophago-gastro-duodenoscopy
substitutes can be recommended. NSAIDs should be avoided. (EGD). It is more sensitive and specific for peptic ulcer
523
disease than upper gastrointestinal barium studies and Anorexia has two subtypes: restricting type and binge-
allows biopsy of gastric lesions. eating/purging type.
Patients younger than 55 years with no alarm symp-
toms should be tested for H. pylori infection and advised Diagnostic criteria for anorexia nervosa
to discontinue the use of NSAIDs, smoking, alcohol and
illicit drug use. Presence of H. pylori can be confirmed with 1. Refusal to maintain body weight at or above a mini-
a serum enzyme-linked immunosorbent assay (ELISA), urea mally normal weight for age and height (e.g. weight
breath test, stool antigen test, or endoscopic biopsy. loss leading to body weight less than 85% of that
expected; or failure to make expected weight gain
Dental considerations during period of growth, leading to body weight less
than 85% of that expected).
Non-steroidal anti-inflammatory drugs and steroids should 2. Intense fear of gaining weight or becoming over-
be strictly avoided. Most patients present with xerostomia weight, even though patient is underweight.
secondary to the anticholinergic drugs. Anemia related 3. Disturbance in the way in which one’s body weight or
symptoms and dental considerations have to kept in mind. shape is experienced, undue influence of body weight
or shape on self-evaluation, or denial of the serious-
Management ness of the current low body weight.
Treatment of peptic ulcer disease should include eradication 4. Amenorrhea in postmenarchal females (i.e. the absence
of H. pylori in patients with this infection. The recom- of at least three consecutive menstrual cycles. A woman
mended duration of therapy for eradication is 10–14 days. is considered to have amenorrhea if her periods occur
Regimen: Omeprazole 20 mg 2 times daily or lansopra- only following hormone administration.)
zole 30 mg 2 times daily plus amoxicillin 1 g 2 times daily
or metronidazole 500 mg 2 times daily (if allergic to peni-
cillin) plus clarithromycin 500 mg two times daily. Bulimia Nervosa
Surgery is indicated in patients who are intolerant of Bulimia also has two subtypes: purging and non-purging.
medications or do not comply with medication regimes, and Bulimia is characterized by uncontrollable binge-eating
those at high risk of complications (e.g. transplant recipi- episodes, often followed by purging behaviors such as vom-
ents, patients dependent on steroids or NSAIDs, those with iting or the use of laxatives. Patients with binge-eating/
giant gastric or duodenal ulcer, those with ulcers that fail purging-type anorexia also might binge and purge. Patients
to heal with adequate treatment). who have bulimia may be of normal weight, or they may be
under- or overweight, whereas patients with binge-eating/
purging-type anorexia are underweight.
EATING DISORDERS
Eating disorders are associated with various medical and Diagnostic criteria for bulimia nervosa
psychologic consequences, including death, osteoporosis, 1. Recurrent episodes of binge eating. An episode of binge
growth delay and developmental delay. Anorexia nervosa eating is characterized by both of the following:
and bulimia nervosa are two of the most devastating eat- a. In a discrete period of time (e.g. within any
ing disorders. Eating disorders are most commonly seen 2-hour period), eating an amount of food that is
among adolescents and young adults of developed nations. larger than what most people would eat during a
They are many times more common in females than in similar period of time and under similar circum-
males. stances.
These disorders are seen in individuals who participate b. A sense of lack of control over eating during the
in activities that promote thinness, such as modeling, movies episode.
and athletics, and certain personality traits such as low self- 2. Recurrent inappropriate compensatory behavior to
esteem, difficulty expressing negative emotions, difficulty prevent weight gain, such as self-induced vomiting;
resolving conflict, and being a perfectionist. It is also seen misuse of laxatives, diuretics, enemas, or other medi-
that up to one-third of women with type 1 diabetes may cations; fasting; or exercising excessively.
have eating disorders. 3. The binge eating and inappropriate compensatory
behaviors both occur, on average, at least twice a
week for 3 months.
Anorexia Nervosa
4. Self-evaluation is unduly influenced by body shape
Patients with anorexia use caloric restriction or excessive and weight.
exercise to control emotional need or pain, and they are 5. The disturbance does not occur exclusively during epi-
terrified of becoming overweight. sodes of anorexia nervosa.
524
Screening for eating disorders Immunological function Acts as a ‘sieve’ for antigens
Morgan et al (1999) suggested the use of the SCOFF ques- Functions of bile juice Acts as a detergent
tionnaire for assessing eating disorders. Emulsifies fat
Absorption of fat-soluble vitamin
SCOFF questions Excretion of hemoglobin by-products
Neutralizes any stomach acid
❍ Do you make yourself Sick (induce vomiting) because
Bactericidal potential
you feel uncomfortably full?
❍ Do you worry that you have lost Control over how
much you eat? Clinical presentation of a patient with suspected
❍ Have you recently lost more than One stone (14 lb liver disease
[6.4 kg]) in a 3-month period?
As a dentist it is very important to look for certain clues in
❍ Do you think you are too Fat, even though others say
the case history that can take us toward a diagnosis of
you are too thin?
hepatobiliary abnormality in such patients.
❍ Would you say that Food dominates your life?
Ask your patient for alcohol abuse, intravenous drug
❍ One point for every yes answer; a score 2 indicates
abuse, rule out familial history of liver diseases, accidental
a likely case of anorexia nervosa or bulimia nervosa
environmental toxin exposure. Enquire if the patient is on
(sensitivity: 100%; specificity: 87.5%).
any hepatotoxic medications, recent travel to another coun-
try or place and whether he/she has exaggerated reactions to
Oral manifestations
certain medications or chemicals. These clues will not only
Frequent vomiting leads to erosion of the enamel on the help in diagnosing any hepatobiliary abnormality but also in
lingual surfaces of the maxillary teeth. Palatal ulcerations deciding an appropriate treatment plan for your patients.
may be noticed. Parotid enlargement may develop as a The symptoms associated with liver disease include: right
sequela of starvation. hypochondrial pain, abdominal distention, ankle swelling,
hematemesis and melena. They can also have pruritus,
amenorrhea, breast swellings, confusion and drowsiness.
LIVER DISEASES Some important clinical signs are nail changes in the form
of clubbing of nails, leukonychia and also smooth nails.
The liver has many essential functions, and liver disease Dupuytren’s contracture, palmar erythema and flapping
presents a number of concerns for the delivery of medical tremors (asterixis), spider nevi, gynecomastia and loss of
and dental care. A number of conditions that cause liver body hair may be appreciated. Patient may present with
dysfunction may be related to lifestyle and a variety of ascites, hepatomegaly or spleenomegaly.
diseases. This section of the chapter will deal with two
common liver conditions, namely, hepatitis and jaundice, Investigations
that have important dental implications.
❍ Aspartate aminotransferase (AST), alanine transaminase
Functions of liver (ALT), alkaline phosphatase (ALP)
❍ Gamma-glucosyl transferase
Functions of the liver can be categorized as follows: ❍ 5-Nucleotidase
❍ Serum albumin
Nutrient metabolism Carbohydrate ❍ Serum bilirubin (direct and indirect)
Protein
❍ Prothrombin time
Lipids
❍ Viral markers.
Protein synthesis Albumin
Coagulation factors Imaging
Complement factors
Hepatoglobin ❍ Ultrasonography
Ceruloplasmin ❍ CT
Storage Iron ❍ MRI.
Copper
Vitamin A
Vitamin B12 Jaundice
Vitamin D Jaundice or icterus is a yellowish discoloration of tissue
Excretion Bile salts resulting from the deposition of bilirubin. Tissue deposition
Bilirubin of bilirubin occurs only in the presence of serum hyper-
Metabolism of drugs bilirubinemia and is a sign of either liver disease or less
525
often, a hemolytic disorder. The degree of serum bilirubin abdominal discomfort, diarrhea, nausea, and/or jaundice.
elevation can be estimated by physical examination. Diagnosis is made based upon signs and symptoms in com-
Slight increases in serum bilirubin are best detected by bination with serologic tests for IgM anti-HAV (positive with
examining the sclerae, which have a particular affinity for recent infection) and IgG anti-HAV. The risk of acquiring
bilirubin due to their high elastin content. The presence of HAV infection for healthcare workers is quite low. One
scleral icterus indicates a serum bilirubin of at least effective method of prevention of HAV infection is the
51 mol/l (3.0 mg/dl). The ability to detect scleral icterus is administration of HAV immune globulin either before the
made more difficult if the examining room has fluorescent exposure or within 2 weeks following exposure. Immune
lighting. If the examiner suspects scleral icterus, a second globulin provides passive immunity toward HAV.
place to examine is underneath the tongue. As serum bili- There are currently only two vaccines available that
rubin levels rise, the skin will eventually become yellow in provide active immunization. Those considered to be at
light-skinned patients and even green if the process is increased risk of HAV infection are people traveling inter-
long-standing; the green color is produced by oxidation of nationally, drug users, people with chronic liver disease,
bilirubin to biliverdin. and those with occupational risks should be vaccinated.
Educating healthcare providers and the public about vac-
cine availability and efficacy can be effective in preventing
Hepatitis
HAV outbreaks. Recovery from hepatitis depends on virus
Findlay and colleagues in 1939 postulated that the etiology replication, the host response and the appearance of anti-
of this particular disease was probably a virus carried in bodies. Greater morbidity and mortality is associated with
the blood of some apparently by inadvertent inoculation very young and older patients. Although most patients
with vaccines contaminated with human serum containing recover from viral hepatitis, sequelae include persistent
the infectious agent. infection (or carrier state), dual infection (HDV [a defective
The term ‘serum hepatitis’ first came into use in the virus] with HBV), chronic active hepatitis, fulminant hepa-
early 1940s as a synonym for post-inoculation hepatitis. The titis, cirrhosis, hepatocellular carcinoma and death. Dual
present diseases now labeled hepatitis A and hepatitis B infections increase the risk for fulminant hepatitis, the
were first described as a single entity, separate from other latter having a mortality rate of about 80%.
causes of jaundice, called ‘sporadic and epidemic catarrhal
jaundice’ was thought to be infectious, but unknown, caus-
Hepatitis B
ative agent (Cockayne, 1912). MacCallum in 1947 suggested
that to avoid confusion in terminology, the two postulated Hepatitis B virus is a 42 nm DNA virus of the Hepadnaviridae
viruses be termed virus A, which produces infectious hep- family. The DNA genome is circular and comprised of two
atitis after a short incubation period and virus B, which strands, with one strand being partially incomplete. It is a
produces serum hepatitis after a long incubation period. highly infectious virus that produces three distinct parti-
Hepatitis has a number of potential causes, both infec- cles during replication:
tious and non-infectious. Alcohol, prescription medications,
❍ The Dane particle or complete virus (HBV), composed
and drug abuse are predominant non-infectious causes,
of an outer shell and an inner core
while viruses and bacteria are important infectious etio-
❍ 22 nm non-infectious spherical particles
logic factors. Viral and drug-induced hepatitis are examples
❍ Non-infectious filamentous units.
of primary hepatitis. Secondary hepatitis may occur as a
sequela of other disease entities such as mononucleosis, The outer shell of the Dane particle carries the hepatitis B
syphilis and tuberculosis. surface antigen (HBsAg), which is anchored in a lipid bilayer
derived from the host cell. Internally is the inner core that
is composed of a protein known as the hepatitis B core
Hepatitis A
antigen (HBcAg), and the hepatitis B early antigen
Hepatitis A is caused by the hepatitis A virus (HAV), an (HBeAg), an antigenic component derived from cleavage
enterovirus of the Picornaviridae family. HAV is a single- of the core antigen. Hepatitis B virus infection remains a
stranded RNA virus surrounded by a protein capsid. Trans- serious healthcare problem. There are 350 million hepatitis
mission is primarily by the oral-fecal route. Various methods B virus carriers worldwide, with the highest carrier rates
of spread are through contact of an infected person, trav- (8–20% of population) in South-East Asia, China and sub-
eling to an endemic region, and ingestion of contaminated Saharan Africa. Globally, 1.25 million persons die per year
food or water. Hepatitis A is typically a self-limiting disas a result of hepatitis B. In 2000, 6,646 cases of type B
ease and usually causes mild illness characterized by sud- hepatitis were reported to the Centers for Disease Control
den onset of non-specific symptoms. In children of age and Prevention (CDC) in the United States. There are over
6 years or younger, it is usually asymptomatic. In adults, 1.5 million carriers in the United States, and an estimated
infection may present with symptoms such as fever, fatigue, 6,000 deaths occur annually due to cirrhosis and primary
526
hepatocellular carcinoma associated with HBV infection. individuals infected with HBV, which may then progress to
The main causative factor for the prevalence of HBV is its severe fulminant infection. Transmission of HDV can occur
replication pattern. The steps of HBV replication provide via infected blood or blood products and is primarily seen
therapeutic targets for antiviral therapy. Nucleoside analogs in intravenous drug users and hemophiliacs. HDV may
interfere with viral DNA synthesis by blocking further also be transmitted through sexual activity. Serologic test-
synthesis when incorporated into viral DNA and or coming for HDV and anti-HDV is used to detect infection.
petitively inhibiting viral polymerase. Effective prevention of HBV will protect against HDV
Current protocols for treatment of HBV infection use infection. The screening of the blood supply for HBV has
interferon and nucleoside analogs such as lamivudine. altered the epidemiology of HDV. There is currently no
Other nucleosides with activity against hepatitis B cur- treatment for HDV infection.
rently being used are famciclovir and adefovir.
Hepatitis E and G
Hepatitis C
Hepatitis E virus (HEV) is a non-enveloped, single stranded
Hepatitis C virus, previously known as one of the non-A RNA virus that is highly unstable due to the lack of a lipid
non-B hepatitis viruses, is a small, positive-sense, single- membrane. Transmission, similar to HAV, is by the oral-
stranded RNA virus of the Flaviviridae family. Six major fecal route. The primary method of spread is through con-
genotypes of HCV and 40 related subtypes have been taminated drinking water and occurs mostly in places with
identified. The virus has a core protein and two envelope inadequate sanitary precautions. Symptoms of disease com-
glycoproteins. The prevalence of HCV infection worldwide monly include malaise, nausea, abdominal pain, and/or
is between 0.3% and 1.5%, with an estimated 300 million fever. The infection is usually self-limiting, although there
carriers worldwide. The genetic diversity of HCV, and its have been cases of fulminant hepatitis and chronic disease.
ability to mutate, allows the virus to avoid neutralization In pregnant patients infected with HEV, there is a higher risk
and establish a chronic infection in about 85–90% of of fulminant hepatitis. There is no HEV vaccine available,
infected persons. Eighty-five percent of patients with HCV and treatment is palliative.
will develop chronic hepatitis. Chronic HCV infection is
the major cause of cirrhosis and hepatocellular carcinoma.
Hepatitis G Virus (HGV)
Acute HCV infection usually presents with mild flu-like
symptoms, while chronic disease is variable in presenta- Hepatitis G is caused by two isolated viruses that appear
tion. Patients may experience non-specific symptoms such to be almost identical. They are single-stranded, positive-
as fatigue, nausea, and/or abdominal pain. Majority of sense RNA viruses similar to HCV. HGV is transmitted
HCV-infected patients develop chronic active hepatitis through blood and blood products, sexual activity and
that is characterized by persistent and intermittent vire- perinatal contact. Due to similar transmission routes, HGV
mia, fluctuating elevations of serum alanine aminotrans- may be found in association with other hepatitis viruses,
ferase (ALT) levels and slow but progressive liver damage. especially HCV. Individuals at risk are organ transplant
The first decade is usually marked by inflammatory cell and blood transfusion recipients, IV drug users, dialysis
infiltration of the portal tracts and focal liver cell necrosis. patients, and healthcare workers with exposure to blood
Mild fibrosis ensues that is followed by more severe fibro- and chronic HCV patients. Diagnosis is based on detection
sis, and bridging between portal tracts and hepatic veins. of HGV RNA in serum 1–4 weeks after infection. Serum
By the second decade after infection, fibrosis progresses to anti-HGV indicates past infection. Although the rate of
cirrhosis in at least 20% of patients with chronic HCV remission is low, little evidence exists that HGV causes
infection. Progression is more likely if patients consume significant liver damage, even with persistent viremia.
excessive amounts of alcohol.
Future treatment of hepatitis C may include agents that
Autoimmune Hepatitis
specifically target the virus rather than current non-specific
forms of antiviral therapy. Research is focused on target- Autoimmune hepatitis is a chronic inflammatory disease.
ing the helicase and polymerase crucial to HCV replication. Although the etiology is not clear, it is thought that environ-
Other therapeutic targets are the viral proteases and the mental and viral factors may cause alterations in cellular
59 and 39 strands of HCV RNA. markers on hepatocytes leading to an autoimmune response
in genetically susceptible individuals. The etiology appears
to be related to antigen specific and generalized suppres-
Hepatitis D
sor defects that perpetuate the autoimmune response. The
Hepatitis D virus (HDV, delta agent) is a defective RNA main finding in autoimmune hepatitis is IgG hypergam-
virus that uses the HBV surface antigen as a viral enve- maglobulinemia, which may be due to chronic infections
lope. HDV can occur as a coinfection or superinfection in or alteration in immune response. Autoimmune hepatitis
527
may lead to liver cirrhosis. Treatment protocols for autoim- blood usually by the 4th week of infection and is followed
mune hepatitis patients usually include steroid therapy. within a week by the hepatitis B early antigen (HBeAg).
If remission is not obtained by steroid therapy alone, aza- Two to four weeks after the appearance of surface antigen,
thioprine or its metabolite (6-mercaptopurine) may be added antibodies against the core antigen (anti-HBcAg) appear.
to the protocol. Subsequently, antibodies against the hepatitis B early anti-
gen (anti-HBeAg) appear by about the 16th week, and finally
the appearance of antibodies against the surface antigen
Fulminant Hepatitis
(anti-HBsAg) appear by about 28th week. Clearance of the
Fulminant hepatitis is sudden, severe liver dysfunction virus is marked by the disappearance of HBeAg, appearance
which may lead to hepatocellular necrosis as well as hepatic of anti-HBeAg and the eventual disappearance of HBsAg.
encephalopathy. Signs of fulminant hepatitis include jaun- Failure to produce anti-HBsAg results in a chronic carrier
dice, hepatomegaly and right upper quadrant tenderness state. Carriers persistently display HBsAg in their serum
during the inflammatory stage. As necrosis occurs and for more than 6 months.
progresses, the liver becomes atrophic and less readily pal- The presence of the HBeAg in a carrier’s serum indicates
pable. With the onset of severe liver failure, liver enzymes, virus replication in the liver and an infectious state. Anti-
bilirubin, prothrombin time and partial thromboplastin time body against HCV (anti-HCV) can be detected in 50–70%
will be elevated, while hemoglobin and hematocrit will of patients at the onset of symptoms and in 90% within
decrease. The prognosis for fulminant hepatitis is poor, and 3 months after onset of infection. Anti-HCV is commonly
the treatment of choice is liver transplantation. detected using an enzyme immunoassay (EIA) that contains
HCV antigens from the core and non-structural genes.
Clinical course of hepatitis B and C A supplemental recombinant immunoblot assay (RIBA) and
the qualitative reverse transcriptase polymerase chain reac-
Hepatitis B and C virus infections can occur at any age,
tion (RT-PCR) for HCV RNA can be performed for confir-
but are more common after puberty. The incubation period
mation. The RIBA is often positive after the 3rd week of
for HBV is 45–180 days and for HCV it is 14–180 days.
infection, whereas HCV RNA can be detected in blood as
Hepatitis B and C viruses produce symptoms in 10% and
soon as 1 week after initial exposure.
25–30% of patients, respectively. Symptoms initially are
Elevation of the serum bilirubin often corresponds with
flu-like and include fatigue, fever, loss of appetite, diarrhea,
the peak of the icteric phase. During recovery, the trans-
headache, malaise, myalgia, nausea, vomiting and weak-
aminase level begins to fall, however, elevations in the
ness. During acute HBV infection, about 50% of symptom-
bilirubin level regress more slowly. Failure of the PT to
atic persons become icteric within about 10 days of the
return to normal is a significant prognostic sign of exten-
onset of symptoms. A minority become icteric with HCV
sive hepatic cellular destruction, indicative of a fulminant
infection. Icterus is the stage of infection in which patients
clinical course or a chronic rather than an acute infection.
demonstrate jaundice in the skin, conjunctiva of the eye,
oral mucosa, and urine as a result of serum bilirubin levels
Oral manifestations
rising three- to four-fold above normal levels (0.2–1.2 mg/dl).
About 10% of patients infected with HBV also demonstrate The oral cavity may show evidence of liver dysfunction with
serum sickness-like manifestations, including angioedema, the presence of hemorrhagic changes, petechiae, hematoma,
arthralgia, and a rash. As the disease progresses, abdomi- jaundiced mucosal tissues, gingival bleeding, and/or icteric
nal pain increases and hepatomegaly and splenomegaly mucosal changes. HCV has been linked to the onset. Sjogren’s
develop. Two to eight weeks are required for recovery from syndrome and chronic hepatitis have been associated with
symptoms, with hepatomegaly and abnormal liver function lichen planus in some studies. Glossitis may be seen with
persisting for weeks to months. The course of the disease alcoholic hepatitis, especially if combined with nutritional
varies with alcohol use and the viral strain and load. The deficiencies. Ecchymosis and reduced healing after surgery
acute infection rarely requires medical treatment other than may also be identified. In some cases, parotid gland enlarge-
rest and the avoidance of hepatotoxic drugs. Patients who ment is evident. These oral changes often appear in com-
fail to produce an adequate immune response can develop bination with general signs and symptoms of liver disease
fulminant hepatitis or a chronic infection. such as fatigue, malaise, confusion, weight loss, nausea, vom-
iting, hepatomegaly, hemorrhagic changes, spider angiomas,
Investigations and diagnosis edema, ascites, dark urine, and pale/clay-colored stool.
The diagnosis of acute HBV infection is made by recogni-
tion of the clinical features, specific serologic tests for viral
antigens and antibodies, and elevated liver enzymes. The Comprehensive and current medical and dental histories
hepatitis B surface antigen (HBsAg) is the first detectable should be elicited. Consultation with and/or referral to treat-
specific marker. Hepatitis B surface antigen appears in the ing physician prior to dental treatment is also recommended.
528
Prior to dental treatment appropriate laboratory investiga- neurapraxia, but ultimately Wallerian degeneration ensues.
tions should be carried pout. These include: Herpes zoster virus shows more aggressive biological behav-
ior than herpes simplex virus type 1 because it spreads
❍ Complete blood count with differential erythrocyte
transversely through the nerve by way of satellite cells.
count, leukocyte count, hemoglobin, hematocrit, plate-
It is commonly seen in pregnancy, diabetes, influenza,
let count, bleeding time
cold or any other respiratory illness. It is immunologically
❍ Prothrombin time, partial thromboplastin time, inter-
mediated; probably caused by herpes virus, mainly herpes
national normalized ratio
simplex virus type 1 and herpes zoster virus.
❍ Liver function tests.
Medications that are metabolized in the liver and/or impair Clinical features
hemostasis should be judiciously used or avoided. Medica- Bell’s palsy is generally considered as a condition based
tions that have to be judiciously used include: analgesics on exclusion. Patients report of no history of trauma, local
(acetaminophen, non-steroidal anti-inflammatory agents, infection, tumor, or CNS disease. They present with periph-
opioids); anesthetics; antibiotics (ampicillin, tetracycline), eral dysfunction of the facial nerve, involving all distal
medication with antiplatelet activity (aspirin) and sedatives branches. The onset is usually abrupt and maximal facial
(long-acting benzodiazepines, barbiturates). weakness is observed at 24–72 hours. Generally, the uni-
Soft tissue trauma should be as minimal as possible lateral facial weakness is complete. However, in about
during dental treatment. Universal infection control mea- one-third of the patients only partial weakness is noticed.
sures should be practiced such as professional immunization Numbness or pain around the ear on the affected side and
against HBV, disinfection of dental units, proper handling reduction in taste on the affected side; altered taste on the
of instruments, sterilization of instruments, good hand anterior two-thirds of tongue are other important clinical
washing technique and personnel protective gear. findings. Drooping of the corner of the mouth on the affected
side with drooling of saliva and loss of facial creases are
common findings. About one-third of the patients report
NEUROMUSCULAR DISORDERS of hyperacusis (hypersensitivity to sounds) and inability to
close eyelid on the affected side.
BELL’S PALSY
Bell’s palsy is a lower motor neuron disease of the facial Some of the relatively common conditions that have to be
nerve (cranial nerve VII) characterized by acute unilateral ruled out for before diagnosing Bell’s palsy are Ramsay Hunt
peripheral facial weakness involving muscles innervated syndrome, tumors affecting facial nerve, diabetes mellitus
by the facial nerve. Bilateral weakness is very rare and and sarcoidosis and Lyme neuroborreliosis.
occurs in less than 1% of patients.
Bell’s palsy is named after the 19th century Scottish Investigations
anatomist and surgeon Sir Charles Bell (1774–1842), who Electromyography can confirm the presence and severity
first described the condition along with the anatomy and of nerve damage. Radiographic evaluation of the cerebel-
function of the facial nerve. lopontine angle, internal acoustic canal and mastoid region
The annual incidence of Bell’s palsy is about 20 per can be undertaken to eliminate presence of tumors.
100,000 persons, with equal numbers of men and women
affected and the incidence increases with age. There is no Management
predilection for either side of the face. It occurs more com-
Spontaneous improvement is generally seen within 6 months
monly in patients with diabetes and in pregnant women.
in most cases. However, about 15% show incomplete recov-
Patients who have had one episode of Bell’s palsy have an
ery and exhibit residual nerve dysfunction including par-
8% risk of recurrence. About 10% of those with Bell’s
tial palsy and motor synkinesis (involuntary movement
palsy have a family history of the condition.
accompanying a voluntary one).
It is believed that the treatment is more likely effective
Etiopathogenesis
before 72 hours and less effective after 7 days. Older
Etiology Common condition affecting 15-to 45-year-old patients are less likely to recover completely. The combi-
age group, both sexes. The cause is unknown, but site of nation of acyclovir 400 mg 5 times/day and prednisolone
damage is probably the portion of facial nerve lying within (40–60 mg daily for a week) is believed to be more effec-
the facial canal (stylomastoid). tive than steroid alone.
Inflammation of facial nerve in the canal with demye- Acyclovir (400 mg five times per day for 10 days) helps
lination and edema further hazards with blood supply. in limiting the damage to nerve from any associated her-
Inflammation of the nerve initially results in a reversible petic infection.
529
Supportive measures include: protecting the cornea be seen in sensory seizures. Partial seizures usually last
with an eyepad or surgical placement of gold weights in the from 30 seconds to 2 minutes. In 30% of patients with
upper lid. Artificial tear substitutes should be used. Bell’s partial seizures, the partial seizure evolves into a second-
palsy and blepharospasm can be treated with injections of ary generalized seizure. In such cases, the excessive elec-
botulinum toxin. trical activity that starts in a limited area spreads to
Facial exercises can be beneficial in patients with Bell’s involve both sides of the brain.
palsy. These should be performed while standing in front
Generalized seizures There are two basic forms of gen-
of a mirror and include trying to raise the eyebrows, open-
eralized seizures, namely, tonic-clonic (grand mal) seizures
ing and closing the eyes, blowing, and whistling.
and absence (petit mal) seizures.
Grand mal seizures are usually seen in childhood or
at teenage. These seizures begin after a brief period of aura
EPILEPSY (warning signs like irritability, headache, nausea). The sei-
zures begin with abrupt loss of consciousness. The tonic
The word ‘epilepsy’ is derived from the Greek word ‘epilam- phase is characterized by rigidity of the arm, leg, chest
banein’ meaning to take or to seize. Epilepsy can be defined and back muscles. The patient may fall, pupils become
as a chronic neurological disorder characterized by fre- dilated and the spine becomes extended (opisthotonos).
quently recurrent seizures. A seizure manifests as an epi- Following this phase there is a clonic phase characterized
sodic disturbance of movement, feeling or consciousness by repetitive jerking and twitching movements of the limbs,
caused by sudden synchronous, inappropriate and excessive tongue and lips. Tongue biting, frothy salivation and
electrical discharges that interfere with the normal func- vomiting may occur. In this phase, bowel and bladder con-
tioning of the brain. trol may also be lost. The seizures usually last for about
Epilepsy usually affects young children (genetic predis- 2–3 minutes. Following this is the post ictal phase in
position to epilepsy associated with chromosome 12 anom- which the patient is tired and goes to sleep for as along as
alies) and elderly individuals who have a history of stroke, 12 hours.
tumors and Alzheimer’s disease.
Status epilepticus When tonic-clonic seizures last more
Clinical types of epilepsy than 5 minutes or recur in a series of three or more seizures
without return to consciousness between attacks, a serious
❍ Primary or idiopathic epilepsy (70%): Cause of seizures neurological emergency called convulsive status epilepticus
is unknown. has developed.
❍ Secondary or acquired epilepsy: Cause of seizure is
known such as head trauma (loss of consciousness Absence (petit mal) seizures Absence (petit mal) seizures
30 minutes), metastatic brain tumors, stroke, infec- are seen in childhood. These are called absence seizures as
tions, hypertension and diabetes as well as electrolyte these are not preceded by aura or warning signs. These
imbalances, dehydration and lack of oxygen. High doses seizures last for about 30 seconds and may only present as
and withdrawal from chronic use of drugs such as rapid eye blinking or stare, and brief loss of consciousness.
heroin, cocaine, barbiturates, amphetamines and alco- However, these recur very frequently almost up to 100 attacks
hol can also lead to seizures. in a day.
Classification of seizures and clinical features Diagnosis and dental considerations
Broadly, seizures can be classified as partial or general- Apart from a good clinical history, diagnostic tools such as
ized. A seizure is termed ‘partial’ when the electrical electroencephalography (EEG) and MRI help in diagnosing
discharge causing it occurs in a specific area of the brain the type of epilepsy. The number of decayed and missing
or ‘generalized’ when the discharge affects the entire brain teeth, the degree of abrasion and periodontal indexes are
cortex. significantly higher in patients with epilepsy. Patient may
present with minor oral injuries such as tongue biting, soft
Partial seizures Partial seizures are further subdivided tissue lacerations and fractured teeth; but also frequently
into simple and complex. In simple seizures consciousness lead to tooth injuries.
is not impaired, whereas in complex seizures there is It is believed that enzyme-inducing antiepileptic drugs
impaired consciousness. There are several types of simple such as phenytoin, phenobarbital, carbamazepine alter the
partial seizures: motor seizures, sensory seizures, auto- metabolism and clearance of vitamin D and have been asso-
nomic seizures and psychic seizures. ciated with osteopenia and osteomalacia. Such patients have
Motor seizures may cause patients to clench and grind an increased risk of fracture. Hence, these patients should
teeth and facial muscles/muscles of mastication may be supplemented with 1,000 mg of calcium and 400 IU of
become stiff. Unpleasant odors and taste disturbances may vitamin D per day.
530
It is estimated that about 50% of the patients using phe- becomes unclear and softer. Face becomes expressionless
nytoin exhibit gingival hyperplasia within 12–24 months of with an increased blink reflex. Patients generally tend to
initiation of treatment. hypersalivate and drool.
Xerostomia and stomatitis have been reported rarely as
side effects of carbamazepine. Valproic acid can cause Dental considerations
direct bone marrow suppression, which can impair wound Most of the orofacial findings in parkinsonism are secondary
healing and increase postoperative bleeding and infections. to the medications (levodopa, bromocriptine, catechol O
Decreased platelet count is the most common and best methyl transferase [COMT] inhibitors) used for treating the
recognized hematologic effect of valproic acid. condition.
When restoration and replacement of teeth are considered Local anesthesia is best used without epinephrine.
it is best to use a fixed rather than removable prosthesis Because the epinephrine can interact with COMT inhibi-
and inclusion of additional abutments should be planned tors and cause hypertension and dysrhythmias. Xerostomia
for fixed partial dentures. may be seen when anti-muscarinic drugs are used. Levodopa
Rash is a common side effect of antiepileptic drugs causes taste disturbances and turns the saliva red. The other
(almost 5–7%). Although most drug-associated rash is
side effects of these drugs are that they produce dyskinesia
benign, serious rashes, including Stevens–Johnson syn-
such as lip and tongue tremors (fly catchers tongue).
drome and toxic epidermal necrolysis do occur.
Metronidazole, antifungal agents such as fluconazole
and antibiotics such as erythromycin may interfere with
the metabolism of certain antiepileptic drugs and render MULTIPLE SCLEROSIS
them ineffective for seizure control.
Multiple sclerosis (MS) is a chronic inflammatory disease of
the CNS. The etiology is still not clear. It is thought that
PARKINSONISM genetic predisposition together with immunity and envi-
ronmental factors could trigger the disease.
Parkinson’s disease is a serious brain disorder characterized
Clinical features
by degeneration of the basal ganglia. Patients present with
bradykinesia (slow movements), increased muscular tone Multiple sclerosis is usually seen between the 2nd and 4th
(rigidity), tremors and loss of postural reflexes. decades of life. It is slightly more common in women than
in men. MS exhibits a wide variety of symptoms as a result
Etiology of the demyelination of the CNS, and a later slow down or
The exact etiology is still unknown. Though previously no blocked transmission at the level of axons. Ectopic impulses
strong genetic basis was considered, it is now believed may also appear, causing paroxysms and convulsions.
that genetic factors may have a role to play. Parkinsonism Multiple sclerosis is categorized into four subtypes:
may be caused by head injuries, drug abuse (phenothi- (i) relapsing–remitting (RR), (ii) progressive-relapsing (PR),
azine, valproate, prochlorperazines and methyl phenyl tet- (iii) secondary-progressive (SP) and (iv) primary-progressive
rahydropyridine). Environmental exposure to toxins such (PP). Almost 85% of the patients belong to the relapsing-
as carbon monoxide, heavy metals and herbicides/pesti- remitting subtype.
cides may have a crucial role in the etiology. Clinically, MS usually appears with a number of symp-
This led to the suggestion that many cases of apparently toms of neurological anomalies which settle in a matter of
idiopathic toxins such as pesticides, herbicides, manga- minutes or hours, and which frequently progress in later
nese, heavy metals and carbon monoxide. days. The most common features are: visual and oculomotor
anomalies, optical neuritis, anterior internuclear ophthalmic
Clinical features paralysis, palsies, paresthesia, lack of motor coordination
and tonic spasms. Other less common features are genito-
In the initial stages of the condition patients may complain urinary dysfunction, increased frequency and urge of urina-
of fatigue, aching limbs, depression and mental sluggish- tion and urinary incontinence, lack of bladder control and
ness. It has also been seen that these patients tend to have episodic retention of urine. In the later stages, paroxysmal
a tiny handwriting (micrographia). The unique feature in ataxia and dysarthria or shaking of upper limbs is fre-
parkinsonism is a unilateral presentation of the signs/symp- quently seen which indicate brain damage.
toms initially which turns into a bilateral involvement as
the condition worsens.
Other clinical features are tremors of the hands and feet
at rest, tongue tremors, gait becomes slower, muscular Patients may be encouraged to sit upright on the dental chair
tone and rigidity increases (log wheel rigidity) and speech to avoid respiratory embarrassment. Hypothetically, nitrous
531
oxide causes demyelination. Hence, it is best avoided. in the 5th decade of life and women are more prone to
Facial myokymia or facial hemispasm may be seen. Patients develop OMD.
report of perioral paresthesia. CCD is an involuntary, sustained, contraction of the
periorbital, facial, oromandibular, pharyngeal, laryngeal
or cervical muscles. OMD involves the masticatory mus-
MUSCULAR DYSTROPHY cles, lower facial, and tongue muscles and may result in
trismus, bruxism, involuntary jaw opening or closure, and
involuntary tongue movement.
Muscular dystrophy (MD) is a life-threatening recessive
These involuntary movements may produce inappropri-
neuromuscular disease affecting the short arm of the
ate deviation of the mandible, subluxation, intraoral soft
X chromosome in the p21-2 position. Of the various forms
tissue trauma and bone resorption. Dysphagia, dysphonia
of MD, severe generalized familial muscular dystrophy
and difficulty with mastication also often occur with OMD.
and mild restricted muscular dystrophy have significant
Brueghel’s syndrome is an association of OMD with bleph-
orofacial findings.
arospasm.
Severe generalized familial muscular dystrophy is pop-
ularly referred to as Duchenne muscular dystrophy (DMD).
It is commonly seen in young male children. In the early
stages of the disease, muscle enlargement may be seen MYASTHENIA GRAVIS
which becomes atrophic as the disease worsens. The early
recognizable symptom is the inability to walk and run. Myasthenia gravis is described in Chapter 20 on Autoimmune
Muscles associated with the lower extremities, pelvis and Disorders.
shoulder girdle rapidly atrophy. Similarly, muscles of the
face, larynx and pharynx are affected.
Symons et al (2002) carried out a study to assess the ENDOCRINAL DISORDERS
dental characteristics of patients with DMD. Their study
showed that patients with DMD had high plaque and cal-
The specialty of endocrinology encompasses the study of
culus accumulation, leading to gingival inflammation, due
glands and the hormones they produce. The term ‘endocrine’
to decreased muscle function. However, the caries experi-
was coined by Starling to denote the action of hormones
ence was low. Disturbances in tooth form, number and
secreted internally in contrast to exocrine glands which
eruption of the second premolars were observed in 39% of
secrete into a lumen. The term ‘hormone’ is a Greek word
patients. Anterior and posterior open bites were common,
meaning ‘to set in motion’ describing the dynamic actions
associated with lip incompetence, mouth breathing, mac-
of hormones as they elicit cellular responses and regulate
roglossia and tongue thrusting. Maxillary and mandibular
physiologic processes through feedback mechanisms.
arch breadths were significantly larger, on average, in the
The classic endocrine glands—pituitary, thyroid, parathy-
DMD group than in controls. Rather than a normal para-
roid, pancreatic islets, adrenals and gonads—communicate
bolic arch form, the dental arches in DMD patients tended
broadly with other organs through the nervous system,
to be hyperbolic, with the posterior teeth being displaced
hormones, cytokines and growth factors. In addition to its
buccally, consistent with an imbalance between the lin-
synaptic functions, the brain produces a vast array of pep-
gual and facial musculature.
tide hormones, spawning the discipline of neuroendocrinol-
An elevated serum creatinine phosphokinase level is
ogy. The immune and endocrine systems are also intimately
typical for this condition. The disease has no known treat-
intertwined. The adrenal glucocorticoid, cortisol, is a pow-
ment as of date and usually the affected individuals do not
erful immunosuppressant. Cytokines and interleukins have
live beyond the 2nd decade of life.
profound effects on the functions of the endocrine organs.
Common endocrine diseases, such as autoimmune thyroid
disease and type I diabetes mellitus are caused by dysregu-
OROMANDIBULAR DYSTONIA lation of immune surveillance and tolerance.
Endocrinal disease can present with either overt disease
Dystonia is an involuntary, repetitive, sustained (tonic) or subtle findings to the oral health clinician. Patients with
or spasmodic (rapid or clonic) muscle contraction. Few of hormonal dysfunctions caused by endocrinal diseases may
the dystonias that are of interest to a dental surgeon are be found to have significant abnormalities on head and neck
cranial-cervical dystonia (CCD) and oromandibular dysto- examination. Large pituitary adenomas frequently present
nia (OMD). Multiple etiological factors are proposed for with changes in vision and loss of visual field integrity,
OMD, namely, genetic predisposition, injury to the CNS, and the oral health clinician may be the first medical pro-
peripheral trauma, medications, metabolic or toxic states fessional to note these diseases. For example, in active
and neurodegenerative disease. It usually affects individuals Graves’ disease (one of the conditions causing excessive
532
function of the thyroid), defects in the motor function of Table 3 Hormones released by the anterior pituitary gland
cranial nerves III, IV and VI may be observed. In acromeg-
aly and in Cushing’s disease, pituitary adenomas secrete Cell types of the anterior Hormones released
active hormones, causing gradual changes in facial fea- pituitary gland
tures and orofacial structures. Thyrotrophs Thyroid stimulating hormone (TSH)
In addition to the changes an oral health clinician can Corticotrophs Adrenocorticotropic hormone (ACTH)
observe and document on physical examination, patients
Gonadotrophs Gonadotropins, luteinizing hormone (LH)
with disease caused by endocrinal dysfunction who often
and follicle-stimulating hormone (FSH)
have alterations in the blood levels of several circulating
Somatotrophs Growth hormone (GH)
hormones. Notably, abnormal hormone levels may also be
the result of previous medical or surgical therapy for under- Lactotrophs Prolactin
lying disease. Normally, blood levels of hormones secreted
by the endocrinal organs are exquisitely regulated. Disrup-
tion of their function can produce life-threatening condi- regulation of digestion, use and storage of nutrients, elec-
tions, adversely affecting the safety of patients during all trolyte and water metabolism and reproductive functions.
but the most minor clinical interventions.
Prompt recognition of endocrinal dysfunction can pre-
vent complications of dental treatment and can provide a Hormones
safe setting for clinical and therapeutic interventions in
Hormones function as chemical messengers moving through
these patients. Even after definitive medical or surgical treat-
the blood to distant target sites of action (pituitary hor-
ment of endocrinal diseases, patients may require lifelong
mones like growth hormone, thyroid stimulating hormone
replacement of several hormones. Replacement therapy
[TSH] and adrenocorticotropic hormone [ACTH]); act more
may be indicated after partial resection or partial destruc-
locally as paracrine or autocrine messengers that incite more
tion of endocrinal tissue. As a result of the destruction of
local effects (like histamine, bradykinin and eicosanoids).
specialized cells, the endocrinal organs may no longer be
Most are present in body fluids at all times in greater or
capable of secreting selected hormones. Replacement hor-
lesser amounts as needed.
mone therapy may interfere with standard preoperative
instructions before dental surgery or treatment and may
Categories of hormones according to structure
require the oral healthcare clinician, in consultation with
the patient’s physician, to alter the medical regimen or the ❍ Amines and amino acid derivatives—Dopamine, cate-
preoperative instructions to ensure a safe outcome. cholamines and thyroid hormone
A general understanding of the pathophysiology, rela- ❍ Peptides, polypeptides—Gonadotropin releasing hor-
tionship and the regulation of endocrine function will mone (GnRH), thyrotropin-releasing hormone (TRH)
help the clinician recognize new presentations of endo- somatostatin and vasopressin
crine disease. Understanding endocrine function and the ❍ Proteins and glycoproteins—TSH, luteinizing hormone
regulatory role will allow the clinician to avoid complica- (LH), follicle stimulating hormone (FSH)
tions during the treatment of these patients with complex ❍ Steroids—Cortisol and estrogen
disease. ❍ Vitamin derivatives—Retinoids and vitamin D.
General Overview of Functional Mechanisms of Mechanisms of action of hormones

Endocrine System Hormones interact with high-affinity receptors which are
linked to one or more effector system in the cell. Some recep-
The hypothalamic–pituitary–endocrine axes control the
tors are located on the cell membrane called membrane
major endocrine glands. Peptidergic neurons in the hypo-
receptors which bind with proteins and catecholamines.
thalamus release small peptides into the anterior pituitary.
These act through second messenger mechanisms (Table 4).
Specialized pituitary secretory cells (Table 3) respond to the
Others are located in the nucleus called nuclear receptors
small peptides and release larger peptides into the systemic
which bind to hormones that can diffuse into the cell like
circulation. The specific endocrine glands are the target
steroids, thyroxine and vitamin D (Table 5). They modulate
of specific specialized pituitary peptides. A negative feed-
the synthesis of enzymes, transport proteins or structural
back by the hormone product of the target organ has been
proteins.
demonstrated upon the secretory pituitary cell and upon
hypothalamic neuronal secretions.
Feedback regulatory system
The basic functions of the endocrine system include
growth and development, sex differentiation, metabolism Positive feedback—estrogen mediated stimulation of the
and adaptation to an ever-changing environment such as midcycle LH surge. Though chronic low levels of estrogen
533
❍ Secreted in a complicated cyclic manner

Table 4 Hormone receptor interactions—second messengers
like cyclic AMP
– Female sex hormones
❍ Regulated by feedback mechanisms that monitor sub-
• Glucagon stances such as glucose (insulin) and water (ADH) in
• Insulin
the body.
• Epinephrine
• Parathyroid hormone
• Thyroid-stimulating hormone (TSH)
• Adrenocorticotropic hormone (ACTH)
General Overview of Disorders of
• Follicle-stimulating hormone (FSH) Endocrine System
• Luteinizing hormone (LH)
Endocrinology interfaces with numerous physiologic sys-
• Antidiuretic hormone (ADH)
• Secretin
tems and hence has no standard endocrine history and
examination is complete more so as most of the glands are
inaccessible. The examination mostly focuses on hor-
Table 5 Hormone receptor interactions—intracellular interactions
monal excess or deficiency as well as direct examination of
palpable glands like thyroid and gonads. It is important to
• Estrogens evaluate patients in the context of their presenting symp-
• Testosterone toms, review of symptoms, family and social history and
• Progesterone detecting subtle symptoms and signs suggestive of under-
• Adrenal cortical hormones
lying endocrine disease. Clinical judgment based on
• Thyroid hormones
knowledge of disease prevalence and pathophysiology is
required to decide when to embark on investigative evalu-
are inhibitory, gradually rising estrogen levels stimulate ation of these disorders. A biochemical testing for hor-
LH secretion. monal abnormality with quantitative assessment of their
Negative feedback—each of the hypothalamic-pituitary hor- levels and dynamics, imaging tests like CT, MRI, thyroid
mone axes is governed by negative feedback maintaining scan and ultrasonography are used for diagnosis of endo-
hormonal levels in a narrow range like thyroid hormone crine disorders.
on TRH–TSH axis.
Categories of Disturbances of Endocrine
Types of hormone actions
Function
Action at a distant site Released into the bloodstream, the
hormones circulate as free, unbound molecules—peptide Endocrine diseases can be divided into three major types
and protein hormones like TRH and LH usually circulate of conditions: hypofunction, hyperfunction and hormone
unbound in the blood or circulate as hormones attached to resistance.
transport carriers.
Steroid hormones and thyroid hormone are carried by Hypofunction
specific carrier proteins synthesized in the liver.
An organ is in the state of hypofunction if there is under-
Paracrine actions Hormones acting locally on cells other production of hormone.
than those that produced the hormone, e.g. the action of sex
steroids on the ovary; somatostatin secreted from -cells Causes
inhibit insulin secretion from -cells of the pancreatic islets.
❍ Congenital defects
Autocrine actions Hormones exerting action on the cells ❍ Glandular destruction due to infarction, hemorrhage,
from which they were produced, e.g. the release of insulin infection, inflammation, autoimmune responses, neo-
from pancreatic -cells can inhibit its release from the same plastic growth or surgeries
cells, IGF-I (insulin-like growth factor I) acts on many cells ❍ Decline in function with aging
that produce it like chondrocytes, breast epithelium and ❍ Atrophy as result of drug therapy or unknown reasons
gonadal cells. ❍ Mutations in a number of hormones, hormone recep-
tors, transcription factors, enzymes and channels can
Control of hormone levels also lead to hormone deficiencies.
❍ Affected by diurnal fluctuations that vary with the Autoimmune damage to the thyroid gland (Hashimoto’s
sleep–wake cycle thyroiditis) and pancreatic islet -cells (type 1 diabetes
– GH and ACTH mellitus) are prevalent causes of endocrine disease.
534
Hyperfunction ❍ Corticotropin-releasing hormone (CRH)

❍ Gonadotropin-releasing hormone (GnRH).
An organ is in the state of hyperfunction if there is exces-
sive hormone production. Function of hormones produced by the anterior
Causes pituitary
❍ Autoimmune disorders ❍ Body growth and metabolism (GH)
❍ Hormone-producing tumor of the gland ❍ Function of the thyroid gland (TSH)
❍ Excess hormone administration. ❍ Glucocorticoid hormone levels (ACTH)
❍ Function of the gonads (FSH and LH)
Hormone resistance ❍ Breast growth and milk production (prolactin).
Hormone resistance is a state when the production of hor- Disorders associated with hypothalamic and
mone is optimal but the end organs are resistant to anterior pituitary insufficiency
hormonal effects.
Hypopituitarism is a result of impaired production of one or
Causes more anterior trophic hormones or impaired synthesis and
❍ Inherited defects in membrane receptors, nuclear recep- secretion of hypothalamic hormone. More often than not the
tors, or in the pathways that transduce receptor signals. etiology is acquired disease like tumors, inflammation or
vascular changes rather than inherited disorders. The clin-
These disorders are characterized by defective hormone ical manifestations depend on which hormone is deficient.
action, despite the presence of increased hormone levels.
Common acquired forms of functional hormone resistance
include insulin resistance in type 2 diabetes mellitus. The GROWTH HORMONE
pathogenesis of functional resistance involves receptor
downregulation and post-receptor desensitization of sig- The growth hormone (GH), produced by somatotropes in
naling pathways; functional forms of resistance are gener- the anterior pituitary is necessary for linear bone growth
ally reversible. in children. It stimulates cells to increase in size and divide
more rapidly by enhancing amino acid transport across cell
Endocrine disorders also can be categorized as membranes; increases the rate at which cells use fatty acids
❍ Primary disorders—those that originate in the target and decreases the rate at which cells use carbohydrates.
gland responsible for producing the hormone
❍ Secondary disorders—where the target gland is essen- Growth Hormone Deficiency
tially normal, but its function is altered by defective
levels of stimulating hormones or releasing factors Growth Hormone in Children—Dwarfism
from the pituitary system
In children GH deficiency, GHRH receptor mutations, GH
❍ Tertiary disorders—which result from hypothalamic
insensitivity or nutritional deficiency which interferes with
dysfunction where both the pituitary and target organ
linear bone growth result in Dwarfism. A familial mode of
are understimulated.
inheritance—autosomal dominant or recessive or X-linked
is seen. Isolated GH deficiency is characterized by short
Pathophysiology of Hypothalamic–Pituitary stature, micro penis, increased fat, high pitched voice, pro-
Axis (HPA) pensity to hypoglycemia.
The HPA axis can be considered the master of ceremony in Etiology of hypopituitarism
regulating the endocrine functions. The secretions of the
❍ Developmental/structural—pituitary dysplasia, con-
hypothalamus act on the pituitary which further releases
genital hypothalamic defects like Kallmann syndrome
hormones to act on the end organs.
❍ Traumatic—radiation, head injury, surgery
❍ Neoplastic—pituitary adenoma, leukemia, pituitary
Hypothalamic hormones regulating the secretion
metastasis
of anterior pituitary hormones
❍ Infiltrative/inflammatory—hemochromatosis, sarcoido-
❍ GH-releasing hormone (GHRH) sis, Langerhans cell disease
❍ Somatostatin ❍ Vascular—pituitary apoplexy, sickle cell disease
❍ Dopamine ❍ Infections—histoplasmosis, toxoplasmosis, Pneumocystis
❍ Thyrotropin-releasing hormone (TRH) carinii, tuberculosis.
535
Causes of short stature Investigations

❍ Variants of normal Imaging may reveal reduced bone mineral density, structural
❍ Low birth weight damage of the pituitary and abdominal scan may show
❍ Chronic illness and malnutrition excessive omental adiposity.
❍ Functional endocrine disorders
❍ Chromosomal disorders Laboratory findings
❍ Skeletal abnormalities
❍ Unusual syndromes. Evoked GH is less than 3 ng/ml, IGF-I level is low or nor-
mal, Concomitant gonadotropin, TSH, ACTH reserves may
Orodental manifestations be deficient, LDL—cholesterol is increased.
In pituitary dwarfs eruption rate and shedding of teeth is
delayed for 1–3 years for teeth that normally erupt during Managment
1st decade of life and for 3–10 years for teeth erupting Replacement therapy with GH at 0.15–0.3 mg/day, maxi-
in the 2nd decade. Lack of third molar development is a mum 1.25 mg/day to maintain IGF-I levels in the mid-
common finding. Clinical crowns are smaller because of normal range for age and gender matched controls. It is
incomplete eruption of teeth. The dental arches are smaller contraindicated in the presence of an active neoplasm,
resulting in malocclusion. The anatomical crowns are nor- intracranial hypertension, uncontrolled diabetes and reti-
mal in size. The roots of teeth are shorter and supporting nopathy. Replacement with adrenocortical and thyroid
structures are retarded in growth. Mandibular growth is hormones in panhypopituitarism may be necessary.
more retarded than maxilla.
Diagnosis and investigations Growth Hormone Excess

Assessment of height if short stature, i.e. if a patient’s
Gigantism
height is more than 3 SD below mean for age or deceler-
ated growth, final height can be predicted using standard Gigantism results if GH excess occurs in children or before
scales (Bayley–Pinneau or Tanner–Whithouse). the growth plates of bone fuse. This results in increased
Radiographic assessment of bone age and skeletal linear bone growth. McCune–Albright’s syndrome may
maturation, i.e. degree of growth plate fusion. GH release account for as many as 20% of cases of gigantism.
is pulsatile and is best measured in response to provoca-
tive stimuli like exercise, insulin-induced hypoglycemia Orodental manifestations
which normally increases GH to more than 7 g/l in chil-
dren. Pituitary imaging with MRI may reveal a mass or The teeth size is proportional to the size of the jaws and
structural defect. the body. Roots may be larger than normal.
Management
Growth Hormone Excess in Adults
Replacement therapy with recombitant GH (0.02–0.05 mg/
kg per day SC) GH insensitivity and in mutations of GH Acromegaly
receptor IGF-I is given which bypasses the dysfunctional
Growth hormone hypersecretion after the closure of
GH receptor.
epiphyses characterized by disordered somatic growth and
proportion.
Adult GH Deficiency Excess GH secretion may be a result of pituitary adenomas
like GH cell carcinoma, multiple endocrine neoplasia 1,
Growth hormone deficiency in adults is caused by hypo- or McCune–Albright syndrome; or due to extra pituitary
thalamic or pituitary somatotrope damage. Infarction of tumor like pancreatic islet cell tumor.
the pituitary leading to hypopituitarism in adults is called
Simmond’s disease. Panhypopituitarism, loss of weight,
Causes of acromegaly
diminished sexual function, reduced basal metabolic rate,
atrophic skin, thin eyebrows, loss of eyelash, sharp features; Most common cause (95%) is a somatotrope adenoma.
thin lips with immobile expressions are the features. Other Other causes ( 5%) include excess secretion of GHRH by
clinical features include impaired quality of life, increased hypothalamic tumors, ectopic GHRH secretion by non-
body fat mass, central fat deposition, reduced exercise endocrine tumors such as carcinoid tumors or small cell
capacity, CVS risk factors with impaired cardiac structure lung cancers; ectopic secretion of GH by non-endocrine
and function, abnormal lipid profile and atherosclerosis. tumors.
536
Excess GHRH secretion ❍ Enlargement of paranasal sinus especially the frontal

sinus.
❍ There central and peripheral causes for excess GHRH
❍ Thickening of the outer table of the skull vault leading
secretion
to enlargement and deformity.
❍ Central—hypothalamic hamartoma, choristoma, gan-
glioneuroma Radiographic features of the jaws
❍ Peripheral—bronchial carcinoid, pancreatic islet cell
tumor, small cell carcinoma, medullary thyroid carci- ❍ Enlargement of the mandible, excess condylar growth
noma, pheochromocytoma. and height of the ascending ramus
❍ Increased angle between ramus and body of mandible
Clinical features with the loss of antegonial notch
❍ Spacing and flaring of anterior teeth with anterior
The condition is insidious in onset and usually occurs open bite
between 6.6 and 10.2 years of life. Voice deepening, struc- ❍ Class III skeletal relationship
tural changes like facial and skeletal disfigurement, frontal ❍ Increase in thickness and height of alveolar process
bossing and cranial ridges, enlarged hand and feet; arthrop- ❍ Radiographic features of the teeth include hyperce-
athy with joint swelling, hypermobility and cartilaginous mentosis and supraeruption of posterior teeth to com-
thickening; periarticular fibrous thickening with joint stiff- pensate for the growth of mandible.
ening or deformities; nerve entrapment; generalized vis-
ceromegaly including the tongue, lips, nose, bones, salivary
glands, thyroid, heart, liver, spleen; abnormalities of the Investigations
spinal column like osteophytosis, disk space widening and Age and gender matched serum IGF levels is increased.
dorsal kyphosis, carpal tunnel syndrome, hyperhidrosis and Single GH level estimation is not useful. Confirmation is
oily skin, facial wrinkles, nasolabial pads and heel pads by the failure of GH suppression to less than 1 g/l within
thicken. Skin tags are the markers for adenomatous colonic 1–2 hour of an oral glucose load. Prolactin levels are
polyps. increased.
The patient is medically compromised due to symp- CT and MRI play a pivotal role in the evaluation of these
tomatic cardiac disease, hypertension and left ventricular patients. MRI is now the investigation of choice in diagnosis
hypertrophy. There is an excessive deposition of muco- of pituitary adenomas. About 80–85% of micro-adenomas
polysaccharides around heart muscle fibers. Increased GH are visible on unenhanced T1 weighted MRI and 33–50%
and decreased glucose tolerance leads to mild-to-moderate are seen as areas of hyperintensity on T2 weighted MRI.
diabetes mellitus. Increased blood glucose levels with MRI is better than CT in assessment of sella in the presence
increased HBA1c (used to assess the efficacy of interven- of bony skull base thickening due to fibrous dysplasia as
tional therapy in DM), accelerated aging of arteries lead to in cases of McCune–Albright’s syndrome. The distinction
atherosclerosis. between pituitary gland and abnormal fibrous bone tissue
Other endocrine complications include: at skull base is better made on MRI. The combination of
❍ Increased serum prolactin level with/without galactor- pre- and post-contrast images is useful in this regard.
rhea However, CT of skull base plays a useful role in some cases
❍ Hypopituitarism with amenorrhea/impotence, second-
for detailing neural foraminal compression, especially if
ary adrenal and thyroid failure surgery is being contemplated.
❍ Glucose intolerance with diabetes mellitus
In one study (Akira Hagiwara et al) on T2-weighted MRI,
❍ Hypertriglyceridemia, hypercalciuria.
hypointensity was seen more commonly in adenomas that
produced GH than in those which did not; hypointensity
The oral manifestations include mandibular overgrowth was nearly exclusive to densely granulated GH-producing
with prognathism in adults, maxillary widening and teeth adenomas.
separation, jaw malocclusion and overbite; enlarged lips, Octreotide scintigraphy, in combination with other imag-
tongue with teeth indentations on the lateral border are ing modalities, is useful in the diagnosis and follow-up of
common features. Soft palate hyperplasia causes sleep pituitary tumors. It allows scar tissue to be differentiated
apnea and airway obstruction. Patients usually present for from tumor recurrence after surgical treatment and ensures
flaring of teeth and orthodontic treatment. better selection of patients who will benefit from medical
treatment with somatostatin analogs.
General radiographic features Management of acromegaly
❍ Enlargement or ballooning of sella turcica if an Synthetic somatostatin analog—octreotide acetate suppress
enlarged pituitary adenoma is present. integrated GH levels to 5 g/l in ⬃70% of patients and
537
2 g/l in ⬃60%. It normalizes IGF-I in ⬃75% of patients. ❍ Primary polydipsia—Secondary deficiency of ADH due
It is given at a dose of 50 g/day 3 times a day up to to inhibition of secretion by excessive intake of fluids.
1,500 g/day.
Lanreotide, a slow-releasing preparation suppresses GH Clinical features
for 10–14 days after 30 mg IM.
❍ Polyuria and polydipsia
GH antagonists—pegvisomant at 10 mg/day for 2 weeks
❍ Urinary output: 5–20 l/day
reduces IGF-I levels.
❍ Low specific gravity: 1.010
Dopamine agonists like bromocriptine, cabergoline,
❍ Low osmolality: 300 mmol/kg.
high energy stereotactic radiation therapy for tumor mass
reduction and trans-sphenoidal surgery are the modalities
Investigations
of treatment.
❍ Fluid deprivation test—to distinguish between cranial
Oral health considerations DI and psychogenic DI
❍ Response to ADH—to distinguish between cranial and
Patients may seek orthodontic treatment for malocclusion. nephrogenic DI
Bilateral growth of mandible and new onset of diabetes ❍ Plasma electrolytes, calcium
mellitus is suggestive of acromegaly. ❍ Investigation of renal tract and suprasellar anatomy.
Management
Disorders of Posterior Pituitary
(Neurohypophysis) ❍ Desmopressin 1–2 mg q day by injection or 10–20 mg
b.i.d. intranasally or 100–400 mg b.i.d. orally
The neurohypophysis secretes oxytocin and vasopressin ❍ Chlorpropamide 125–250 mg/day enhances the renal
(antidiuretic hormone). Oxytocin is released rendering responsiveness to vasopressin
uterine contraction during child birth and release of milk ❍ Thiazide diuretis—only drug therapy for nephrogenic DI.
during breast feeding. The antidiuretic hormone aids in
conservation of body water. It acts on permeability of col-
lecting ducts and tubules reabsorbing water. It also causes
arteriolar smooth muscle contraction, vasoconstriction THYROID GLAND
and increases arteriolar blood pressure.
The thyroid produces two hormones, thyroxin (T4) and tri-
iodothyronine (T3). Acting through nuclear receptors,
Diabetes Insipidus these hormones play a critical role in maintaining thymo-
genic and metabolic homeostasis; help in cell differentia-
Diabetes insipidus (DI) is caused due to the decreased tion influencing growth and development in children and
secretion or action of ADH. It is characterized by produc- mental development and attainment of sexual maturity.
tion of abnormally large volumes of dilute urine. Thyroxin stimulates eruptive movement and tooth size, but
has little effect on alveolar growth. After diabetes mellitus,
Etiology thyroid disease is the most common endocrine problem in
the general population and most commonly seen in women
Cranial DI—deficient production of ADH. It is caused by
with higher rate after the age of 50 years.
genetic defect, such as dominant, recessive DIDMOAD syn-
drome (DI, diabetes mellitus, optic atrophy, deafness).
Hypothalamic or high stalk lesion—histiocytosis X, sar-
Pathophysiology of Hypothalamic–Pituitary–
coidosis, craniopharyngioma, suprasellar pituitary adenoma,
Thyroid Axis
basal meningitis, head injury, surgery, encephalitis.
It is a classic example of endocrine feedback loop.
Idiopathic causes
TRH + Anterior TSH +
Nephrogenic DI—renal tubules unresponsive to vasopressin. Hypothalamus Thyroid
pituitary
Causes for nephorgenic DI are:
(−)
❍ Genetic defect—Sex linked recessive, cystinosis (−)
T4→T3
❍ Metabolic—Hypokalemia, hypercalcemia
❍ Drug—Lithium, demeclocyline, amphotericin B, amino-
glycosides, cisplatin, rifampin, foscarnet Thyroid hormone and cortisol provide negative feedback
❍ Poisoning—Heavy metals to the pituitary and hypothalamus. A small hypothalamic
538
peptide most specific to thyroid function thyrotropin- Etiology

releasing hormone (TRH); TSH, a larger peptide from the
Primary
pituitary, predominantly associated with thyroid function.
Together, these peptides control three major functions of ❍ Iodine deficiency
the thyroid: the production of thyroid hormone (T4), the ❍ Autoimmune hypothyroidism: Hashimoto’s thyroid-
growth and proliferation of thyroid cells, and the produc- itis, atrophic thyroiditis
tion of thyroglobulin (a large protein that acts as a binding, ❍ Iatrogenic: Treatment, subtotal or total thyroidectomy,
storage and maturation protein for T4). As a consequence external irradiation of neck for lymphoma or cancer
of driving thyroid hormone production with TSH, circulat- ❍ Drugs: Iodine excess (including iodine—containing
ing systemic blood levels of T4 are elevated. The increased contrast media and amiodarone), lithium, antithyroid
blood levels of T4 result in decreased pituitary secretion of drugs, p-aminosalicylic acid, interferon-
and other
TSH, forming a negative feedback loop. The negative feed- cytokines, aminoglutethimide
back mechanism causes decrease in the specific releasing ❍ Congenital hypothyroidism: Absent or ectopic thyroid
and stimulating hormones of the hypothalamus and pitu- gland, dyshormonogenesis, TSH-R mutation.
itary. Decreases in the pituitary production of the corre- ❍ Infiltrative disorders: Amyloidosis, sarcoidosis, hemo-
sponding stimulating hormones, TSH and ACTH, result in chromatosis, scleroderma, cystinosis, Riedel’s thyroiditis
the downregulation of the hormone produced by the spe- ❍ Transient hypothyroidism
cific endocrine gland, T4. ❍ Silent thyroiditis, including postpartum thyroiditis
❍ Subacute thyroiditis.
Laboratory evaluation
Secondary
Categorized as:
❍ Hypopituitarism: Tumors, pituitary surgery or irradia-
1. Screening of newborns for congenital hypothyroidism tion, infiltrative disorders, Sheehan’s syndrome, trauma,
2. Screening of adult patients seen for non-thyroid related genetic forms or combined pituitary hormone defi-
reasons. ciencies
❍ Measures of T3, T4, and TSH-TSH immunoradiometric ❍ Isolated TSH deficiency or inactivity
assays followed by measurement of unbound circulat- ❍ Hypothalamic disease: Tumors, trauma, infiltrative
ing thyroid hormone. disorders, idiopathic.
TSH should not be used to assess thyroid function in Hypothyroidism in children is termed cretinism and that
patients suspected or known pituitary disease because manifesting in adults called myxedema.
secondary hypothalamic pituitary disease is associated
with a variable (low-high) TSH level which is inap- Congenital hypothyroidism—cretinism
propriate for the low T4 levels.
❍ Radioiodine uptake and thyroid scanning: Thyroid Congenital hypothyroidism if undetected early results in
gland selectively transports radioisotopes of iodine hypothyroidism in children termed cretinism.
(123I, 125I, 131I) and 99mTc pertechnetate, allowing thy- Congenital hypothyroidism is due to thyroid gland dys-
roid imaging and quantification of radioactive tracer genesis or inborn errors of thyroid hormone synthesis,
fractional uptake. TSHR antibody mediated.
❍ Tests to determine etiology of thyroid dysfunction— Females have a 2:1 prediliction. The infant appears
Autoimmune thyroid disease is detected by measuring normal at birth. The condition is diagnosed because of
circulating antibodies against thyroid peroxidase and prolonged jaundice, feeding problems, hypotonia, macro-
thyroglobulin. glossia, delayed bone maturation, umbilical hernia, sparse
❍ Resin uptake test. and brittle hair and fingers, atrophic sweat glands. Diag-
❍ Ultrasonographic scanning and ultrasonography- nosis is established by heel prick blood estimation of T4.
guided FNA biopsy of a thyroid nodule. If treatment is delayed permanent neurologic damage
❍ CT and MRI scans. occurs. T4 is instituted at a dose of 10–15 g/kg per day
❍ Serum or tissue thyroglobulin determination. with close monitoring of TSH levels.
Disorders Associated with Thyroid Gland Oral manifestations

Shortened base of the skull leading to retraction of the
Hypothyroidism
bridge of the nose with flaring. The face looks wider as
Primary hypothyroidism is an intrinsic disorder of the thy- longitudinal growth fails. Accumulation of glycosamino-
roid gland causing low levels of thyroid hormone with glycans leading to enlarged lips and enlarged tongue with
raised TSH. continuous protrusion leading to malocclusion, delayed
539
eruption, retained deciduous teeth but no impairment of every 6 hourly in case of profound hypothyroidism to
tooth formation. counter depleted adrenal reserve.
Hyperthyroidism
❍ In children delayed closure of epiphysis and skull sutures
with the production of numerous wormian bones. Hyperthyroidism is the result of excessive thyroid function
❍ Delayed eruption, short roots and thinning of lamina whereas thyrotoxicosis is a state of thyroid hormone excess
dura. the etiology of which is hyperthyroidism.
❍ Relatively small maxilla and mandible.
Causes of thyrotoxicosis
General signs and symptoms in myxedema Primary hyperthyroidism
Patient complains of tiredness, weakness, difficulty in 1. Graves’ disease
concentrating and poor memory. Dryness of skin with hair 2. Toxic multinodular goiter
loss and intolerance to cold. Weight gain with poor appe- 3. Toxic adenoma
tite, constipation, menorrhagia (later oligomenorrhea or 4. Functioning thyroid carcinoma metastases
amenorrhea), dyspnea, hoarse voice, paresthesias, impaired 5. Activating mutation of the TSH receptor (autosomal
hearing are the symptoms. dominant)
The signs include dry coarse skin; cool, peripheral 6. Struma ovarii
extremities, puffy face, hands and feet (myxedema), dif- 7. Drugs; iodine excess (Jod–Basedow phenomenon).
fuse alopecia, bradycardia, peripheral edema, delayed ten-
don reflex relaxation, carpal tunnel syndrome, serous Thyrotoxicosis without hyperthyroidism
cavity effusions, decrease in renal function. 1. Subacute thyroiditis
2. Silent thyroiditis
Oral health considerations 3. Other causes of thyroid destruction; amiodarone, radia-
tion, infarction of adenoma
❍ Delayed eruption of permanent teeth.
4. Ingestion of excess thyroid hormone (thyrotoxicosis
❍ Myxedema—respiratory depression, CVS depression.
factitia) or thyroid tissue.
❍ Mild hypothyroidism—exaggerated effects of analgesics
and sedatives in routine doses. Secondary hyperthyroidism
❍ Increased subcutaneous mucopolysaccharides—decrease
1. TSH—secreting pituitary adenoma
the ability of small vessels to constrict—prolonged
2. Thyroid hormone resistance syndrome; occasional
bleeding.
patients may have features of thyrotoxicosis
❍ Decreased metabolic activity in fibroblasts—delayed
3. Chorionic gonadotropin—secreting tumors
wound healing.
4. Gestational thyrotoxicosis.
❍ Drug interactions with lithium, amiodarone, etc. may
induce hypothyroid stages.
Clinical features
Radiographic features Symptoms Hyperactivity, irritability, dysphoria, heat
intolerance and sweating, palpitations, fatigue and weak-
Radiographic features of the teeth include evidence of
ness, weight loss with increased appetite, diarrhea, poly-
periodontal disease, loss of teeth, separation of teeth and
uria, oligomenorrhea, loss of libido.
external root resorption.
Signs Tachycardia, atrial fibrillation in the elderly, trem-
Treatment ors, goiter, warm, moist skin, muscle weakness, proximal
myopathy, lid retraction or lag and gynecomastia.
Daily replacement dose of levothyroxin 1.6 g/kg (100–
150 g).
Oral manifestations
Thyroxin sodium 1.5 mg/kg, i.e. 100–150 mg/day.
Ideal dose restores TSH to normal. Alveolar atrophy is seen in advanced cases as a conse-
Supportive therapy to correct metabolic disturbance. quence of osteoporosis. Periodontitis and dental caries
External warming if temperature falls below 30C which can appear more rapidly. The jaws and teeth development is
result in cerebral vascular collapse. Correction of hypona- accelerated and earlier shedding of deciduous teeth and
tremia and hypoglycemia with hypertonic saline and IV accelerated eruption of permanent teeth is seen. Euthyroid
glucose. Hypotonic infusions should be avoided which can infants of hyperthyroid infants may have natal teeth. Lin-
lead to water retention. Parenteral hydrocortisone 50 mg gual thyroid is sometimes seen. A lingual thyroid if seen
540
in a euthyroid patient should not be excised unless the Clinical features

presence of the thyroid gland is confirmed with radioactive
Patients present with very high fever, extreme cardiovascu-
iodine scanning. Pain associated with subacute thyroiditis
lar effects (tachycardia, congestive failure and angina) and
radiates to the ear, jaw and occipital region. Horseness of
severe CNS effects (agitation, restlessness and delirium).
voice and dysphagia are other features.
Management
Graves’ Disease
❍ Propylthiouracil 600 mg initially and 200–300 mg/
Hyperthyroidism with diffuse thyroid enlargement, oph- 6 hour. Propranolol to reduce tachycardia.
thalmopathy and dermatopathy. ❍ Glucocorticoids and antibiotics if infection present.
An autoimmune disorder characterized by abnormal
stimulation of the thyroid gland by thyroid-stimulating Orodental treatment evaluation of a patient with thy-
antibodies (thyroid-stimulating immunoglobulins [TSI]) roid disorder Any patient who is asymptomatic and has
that act through the normal TSH receptors. had a physical examination and normal thyroid function
This is associated with human leukocyte antigen test within the past 6 months is at low risk for complica-
(HLA)-DR3 and HLA-B8. Familial tendency is evident due tions during dental therapy. If otherwise the patient is
to immunologically mediated activation of fibroblasts in considered as at moderate risk because an asymptomatic
extraocular muscles and skin with accumulation of lid lag hypothyroid patient under thyroid replacement therapy
or retraction, proptosis, exophthlamos, diplopia, corneal may become hypothyroid again because of lapses in med-
involvement, periorbital edema, chemosis, optic nerve ication or subtle metabolic shifts. Similarly, a hyperthy-
damage. roid patient on propyl thiouracil or radioactive iodine
Dermatopathy in the form of pretibial myxedema, skin remain euthyroid only in about 30% of cases or as a com-
appearance appears like orange and clubbing may be seen. plication of radioactive iodine therapy insidiously may
become hypothyroid.
Radiographic features A hypothyroid patient has pre-existing CNS depression
and is acutely sensitive to drugs with CNS depressing side
In children early development and eruption of teeth with
effects. Narcotic analgesics and sedatives can precipitate
premature loss of primary teeth is seen. In adults general-
respiratory and cardiovascular depression. Patients with
ized decrease in bone density or loss of some areas of
hyperthyroidism are particularly susceptible to catechol-
edentulous alveolar bone is seen.
amines (epinephrine as vasoconstrictors) used in local
anesthetics and gingival retraction cords. Along with den-
Investigations
tal stress it can precipitate thyroid storm. Short of thyroid
❍ Increased T3 storm it can exacerbate underlying cardiovascular pathology.
❍ Increased or upper limit normal range—T4 If no evaluation has been done in the past 6 months the
❍ Decreased TSH patients have to be referred to the physician and for labo-
❍ Non-specific or increased serum glutamic oxaloacetic ratory evaluation especially when type V and VI procedures
transaminase (SGOT), serum glutamic pyruvic trans- are indicated.
aminase (SGPT), alkaline phosphatase. Patients with hyperthyroidism may have elevated blood
❍ Routine thyroid 99mTc radionuclide imaging is helpful pressure and heart rates on the basis of the effects of
in identifying patients with an unsuspected cause of thyroid hormone on sympathetic nervous system activity.
hyperthyroidism other than Graves’ disease. Patients with high arteriolar pressures may require increased
attention and a longer duration of local pressure to stop
The treatment of Graves’ disease is aimed at
bleeding. Hyperthyroidism patients who are on warfarin
1. Reducing thyroid hormone synthesis using antithyroid (Coumarin) may have an increased metabolism of this drug,
drugs like thionamides—propyl thiouracil 100–200 mg leading to decrease in previously therapeutic coagulation
6–8 hourly, carbimazole and methamizole 10–20 mg indices.
8–12 hourly. Patients with long-standing hypothyroidism may have
2. Reducing the amount of thyroid tissue with 131I radio- increased subcutaneous mucopolysaccharides due to de-
iodine or subtotal thyroidectomy. crease in the degradation of these substances. The presence
of excess subcutaneous mucopolysaccharides may decrease
the ability of small vessels to constrict when cut and may
Thyrotoxic Crisis/Thyroid Storm
result in increased bleeding from the infiltrated tissues,
Thyrotoxic crisis is a medical emergency because of exac- including mucosa and skin. Local pressure for an extended
erbation of hyperthyroidism precipitated by infection, stroke, time will probably adequately control the small-vessel
trauma, diabetic ketoacidosis or thyroid surgery. bleeding.
541
PARATHYROID GLANDS Clinical features

It was described by Jackson and Frame (1972) as the tetrad
Parathormone (PTH) is an 84 amino acid single chain pep- of ‘bones, stones, abdominal groans and psychic moans
tide and is the primary regulator of extracellular calcium with fatigue overtones’.
concentration. It acts directly on bone inducing calcium Mostly occur after 60 years of age and women are
resorption and on the kidney where it stimulates calcium affected 2–4 times more often than men. About 80% of
reabsorption and synthesis of 1,25-dihydroxy vitamin D the cases are asymptomatic and the primary involvement
(1,25(OH)2D) a hormone that stimulates GI calcium is in the kidneys and skeletal system. Stones refer to the
reabsorption. increased deposition of calcium in renal parenchyma and
Continuous exposure to elevated PTH leads to increased a tendency to develop recurrent nephrolithiasis (renal cal-
osteoclastic mediated bone resorption. However intermit- culi) with resulting complications like urinary tract obstruc-
tent administration of PTH, elevating levels for 1–2 hour/ tion, infection, loss of renal function and uremia. Metastatic
day leads to net stimulation of bone formation rather than calcifications are also seen in blood vessel walls, subcutane-
breakdown. Thus the effects of PTH are: (i) calcium removal ous soft tissues, dura and the region around the joints. Band
from bone, and (ii) bone remodeling by acting on both keratopathy, where calcification occurs as a narrow band at
osteoblasts and osteoclasts. the limbic margin of the cornea of the eye is also seen.
Bones refer to the distinctive involvement of the bone.
Hyperparathyroidism Ostetitis fibrosa cystica, bone lesions which on histopath-
ological examination show multinucleated giant osteo-
Hyperparathyroidism (HPT) causing bone disease was first clasts in scalloped areas of the bone surface (Howship’s
described by von Recklinghausen in 1891 and so-called as lacunae) and replacement of normal cellular and marrow
von Recklinghausen disease of bone. It is a generalized dis- elements with fibrous tissue.
order of calcium, phosphate and bone metabolism due to Abdominal groans refer to subtle vague GI disturbances
increase in PTH secretion. such as nausea, vomiting, anorexia, pancreatitis, duodenal
Hyperparathyroidism occurs in three clinical forms: and peptic ulcers.
Psychic moans may be due to CNS manifestation rang-
❍ Primary HPT is the uncontrolled production of PTH as
ing from mild personality problems to severe psychiatric
a result of a parathyroid adenoma (80–90%) or chief
disorders due to hypercalcemia.
cell parathyroid hyperplasia (10–15%) or a parathyroid
Other manifestations include neuromuscular problems
carcinoma (2%), (multiple endocrine neoplasia I and
with proximal muscle weakness, easy fatigability and
MEN 2a).
muscle atrophy (differentiated from other neuromuscular
Two syndromes are associated with HPT, namely,
disorders by regression after surgical removal of the
hereditary hyperthyroid jaw tumor syndrome and
glands).
familial isolated primary hyperthyroidism with
carcinoma.
Orodental manifestations
❍ Secondary HPT is a compensatory mechanism result-
ing from a primary condition producing hypocalcemia One of the first signs is development of malocclusion
such as rickets, osteomalacia, pregnancy, chronic renal because of drifting of teeth. Giant cell tumors and pseudo-
insufficiency, calcium deprivation or maternal hypo- cysts of the jaws are the other possible lesions found.
parathyroidism. Padbury et al demonstrated a significantly greater inci-
Hypocalcemia prompts increased PTH production dence of torus/tori in the Caucasian population with the
with liberation of calcium from bone. incidence of tori nearly three times higher in HPT patients.
Renal osteodystrophy occurs in chronic renal HPT leads to a preferential loss of cortical bone and pres-
diseases. ervation or increase in trabecular bone. It has been pro-
In the kidney, the hydroxylation of 25-OH vitamin posed that the presence of hypercalcemia in HPT patients is
D3 occurs which is necessary for calcium absorption preceded by longer periods of elevated PTH levels. It could
from the gut. Therefore in patient with end stage renal be that the tori represent an expansion of trabecular bone
disease active vitamin D is less—less calcium is at the expense of cortical bone in response to elevated PTH
absorbed from gut—hypocalcemia compensatory levels with possible contribution from the mechanical
PTH production. forces present in the oral cavity. Furthermore, PTH mediated
❍ Tertiary HPT may occur after long-standing second- endosteal resorption has been shown to be compensated
ary hyperparathyroidism, characterized by the develop- by PTH-mediated periosteal apposition with an increase in
ment of a functional parathyroidism adenoma causing overall bone size.
increased production of PTH. The most common cause Periodontal ligament (PDL) width in patients with a torus
is chronic renal failure. was higher than those without a torus, and this intriguing
542
relationship remained when issues of gender and age were density of the root and is rounded at apex. Some authors
taken into account. This is consistent with the HPT patient feel that the loss of lamina dura occurs only in about 10%
population having reduced radicular lamina dura density case and is the overrated feature of HPT.
and may reflect the localized impact of PTH in the PDL Metastatic calcification may occur in salivary gland.
space to evoke cortical bone loss in the area adjacent to
Brown tumors These occur in pelvis, ribs and femurs but
the tooth, whereas compensatory mechanisms are taking
most common in the jaw bones. These are either peripheral
place for increased bone growth in the form of the tori
or central, monostotic or polyostotic. These may be uni-
more laterally.
locular or multilocular without marginal scalloping. The
borders may be poorly defined and there may be a zone of
Radiographic features reactive bone outlining the lesion. Histologically, it is
The classic generalized features: Most important and reli- identical to central giant cell granuloma of the jaws. If a
able sign of HPT is subperiosteal erosion along the radial giant cell granuloma is found later than the second decade
margin of the middle phalanges. Initially, only the outer the patient should be screened for serum calcium, PTH and
surface of the cortex appears lace like but then the endos- alkaline phosphatase.
teal surface is also affected. Generalized demineralization Teeth The teeth stand out because of the osteopenic
shows an unusual radiolucency. nature of the bone. In developing or erupting tooth: (i) loss
Osteitis fibrosa generalisata It is also called as osteitis of crypt wall, (ii) pointed and tapered roots especially at
fibrosa cystica as they appeared cyst-like on radiographs. apical third, and (iii) large pulp chambers are seen.
It refers to the pattern of generalized rarefaction seen in In erupted teeth, the pulp chambers appear large in
the skeleton in approximately 13% of patients with hyper- younger people but in adults they are narrower. Pulpal
parathyroidism. It appears as radiolucent area with thin cor- calcifications are seen in pulp chambers.
tices and hazy indistinct trabeculae or a moth eaten image.
In regions where the trabeculae are completely missing a Histopathologic features
cyst-like appearance is seen. Histopathologically a sparse Brown tumors are characterized by a proliferation of exceed-
narrow trabeculae scattered throughout a fibrous stroma ingly vascular granulation tissue with accumulation of
with few osteoclasts are seen. hemosiderin pigments giving the lesion a brown appear-
Brown tumors Peripheral or central tumors of bone are ance. Numerous multinucleated osteoclast type giant cells
seen in the late phase of the disease in 10% of the cases. are seen and some lesions are proliferative showing paral-
Metastatic calcification of soft tissues appearing punc- lel arrangement of spicules of woven bone set in a cellular
tuate or nodular and occur in kidneys and joints. fibroblastic background with multinucleated giant cells
often associated with secondary HPT related to chronic
Radiographic features of skull Skull appears osteopo- renal disease.
rotic and this change may occur before jaw changes.
Deossification appears granular caused by loss of diploie Investigations
and thinning of cortical plates and is prominent in the
outer third of the skull termed pepper-pot skull which may ❍ Immunoassay for parathormone.
be interrupted by one or more cystic areas representing ❍ Multiple markers of bone tumors such as formation
brown tumors. Radiographically, they appear rounded with indices (bone specific alkaline phosphatase, osteocalcin
smooth or irregular borders and with sclerotic reactive and type I collagen peptides) and bone resorption indi-
bone margins. ces (including hydroxypyridinium collagen crosslinks
and telopeptides of type I collagen).
Radiographic features of jaw bones Generalized demin- ❍ CT and dual energy X-ray absorptiometry (DEXA) of
eralization characterized by lack of sharpness of trabecu- spine produce reproducible quantitative estimates of
lar pattern with a more granular appearance described as bone density. Non-invasive imaging like ultrasonog-
ground glass appearance or osteoporosis fibrosa generalisata raphy, CT scanning, MRI and parathyroid (PT) scintig-
sometimes may have a mottled or moth-eaten appearance raphy may be used.
due to variation in density. Subperiosteal erosion at the
mandibular angle, resorption of the inferior cortex of the
Management
mandible, lining of mandibular canal, mental foramen,
walls of antrum and nasal cavities. Resorption of the lam- Primary HTP: In symptomatic patients removal of hyper-
ina dura is the first sign of deossification in the jaws. Two plastic/functional tumor is done after PTH assay.
99m
relative changes are observed, namely, thickening of PDL Tc-sestamibi/99mTc-pertechnetate subtraction scin-
space and tapered appearance of roots because the lamina tigraphy (SS) as initial test for patients undergoing para-
dura provides a definitive edge effect accentuating the thyroidectomy and helical CT anatomical detail, such as
543
the relationship with surrounding tissues and detection of ❍ Substitution therapy—1 hydroxycholecalciferol or
concomitant thyroid nodules, guiding the surgical explo- vitamin D 40,000–120,000 g/day (1–3 mg daily) with
ration especially in patients with ectopic PT glands. 1 g elemental calcium.
In asymptomatic patients who do not undergo surgery
the management is as suggested by Bilezikian et al. Oral health considerations
Assessment It occurs during odontogenesis; pitting enamel hypoplasia
and failure of tooth eruption may occur. Presence of per-
❍ Serum calcium—annually
sistent oral candidiasis in a young patient may signal the
❍ 24-Hour urinary calcium only during initial evaluation
onset of endocrine—candidiasis syndrome.
❍ Creatinine clearance only during initial evaluation
❍ Serum creatinine annually
❍ Bone density—annually Tetany
❍ Abdominal radiographs with or without ultrasono-
graph at the time of initial evaluation Tetany is defined as an increased excitability of peripheral
❍ Secondary HTP nerves due to hypocalcemia or alkalosis.
❍ Restriction of dietary phosphate, administration of
bisphosphonates and vitamin D metabolite (calcitrol) Causes
and renal transplantation.
❍ Hypocalcemia: Malabsorption, osteomalacia, hypo-
parathyroidism, acute pancreatitis.
Hypoparathyroidism ❍ Alkalosis: Repeated vomiting of gastric juice, excess
intake of alkalis, hyperventilation, primary hyperaldo-
Hypoparathyroidism could be hereditary or acquired. steronism.
Decreased amount of PTH leads to hypocalcemia and
hyperphosphatemia.
Clinical features
Children present with carpopedal spasm, stridor and con-
Hereditary
vulsions. Main d’ accoucheur sign is seen (metacarpopha-
Hypoparathyroidism associated with conditions like langeal joints flexed, interphalangeal joints of fingers and
DiGeorge syndrome—defective development of thymus thumb extended with opposition of the thumb). Adults pres-
and parathyroid glands; and polyglandular syndromes. ent with tingling in hands, feet and around the mouth.
Latent tetany is characterized by Trousseau’s sign. When
the sphygmomanometer cuff is inflated on upper arm more
Acquired
than systolic BP carpal spasm occurs within 3 minutes.
Postoperative hypoparathyroidism Damage to parathy- Chvostek sign is seen which is characterized by twitching
roid gland or inadvertent removal during thyroid surgery. of upper lip when the facial nerve is tapped below the
zygomatic process.
Infantile hypoparathyroidism Transient, associated with
maternal hyperparathyroidism or calcium deficiency.
Management
Idiopathic hypoparathyroidism Autoimmune disease of
adrenal, thyroid or ovary in young people. Tetany is managed with 20 ml of 10% calcium gluconate IV
or 10 ml IM. Alkalosis is treated with IV isotonic saline.
Clinical features
Calcifications of basal ganglia and extrapyramidal syn-
dromes like choreoathetotic movement and dystonia are HYPOTHALAMUS–PITUITARY–ADRENAL
more common and appear earlier. Tetany as consequence AXIS
of hypocalcemia, grand mal epilepsy, psychosis, cataracts,
papilledema, increased intracranial pressure and chronic The main function of hypothalamus–pituitary–adrenal
changes in finger nails, hair, alopecia and hypomagnese- (HPA) axis is to maintain metabolic homeostasis and to
mia are seen. May be associated with autoimmune poly- mediate the neuroendocrine stress response. An understand-
glandular—candidiasis syndrome. ing of the axis is important especially for management of
conditions which need long-term corticosteroid therapy
Management
and management of patients on long-term corticosteroid
❍ Usually asymptomatic—no treatment. therapy and adrenal insufficiency.
544
Hormone Released from Type Actions

Corticotropin releasing hormone (CRH) Hypothalamus Peptide Controls function of adrenal cortex
Adrenocorticptropic hormone (ACTH) Anterior pituitary Pro-opiomelanocortin (POMC) Controls function of adrenal cortex, controls
medullary rennin and aldosterone mineralocorticoid
action in controlling blood volume and pressure
Melanocyte stimulating hormone (MSH) Anterior pituitary Pro-opiomelanocortin (POMC)
B-endorphins Anterior pituitary Pro-opiomelanocortin (POMC)
Mineralocorticoids (aldosterone) Function in sodium, potassium and water balance
Glucocorticoids (cortisol) Aid in regulating the metabolic functions of the
body and in controlling the inflammation.
Essential for survival in stress situations
Adrenal sex hormones (androgens) Serve mainly as a source of androgens for women
Pathophysiology of adrenal diseases and Tests for mineralocorticoid reserve and stimulation of
conditions renin–angiotensin system is by programed volume deple-
tion such as sodium restriction, diuretic administration or
Acute inflammatory or septic insults activate HPA axis
upright posture.
through the integrated actions of proinflammatory cytokines,
Suppression tests are done to document hypersecretion
bacterial toxins and neural signals resulting in cortisol ele-
of adrenal hormones by measuring the target hormone
vation, restraining inflammatory response and providing
response after standardized suppression of its tropic hor-
host protection. Thus, the neuroendocrine stress response
mone. Examples are the pituitary-adrenal suppressibility
reflects the net result of highly integrated hypothalamic,
and an overnight dexamethasone suppression test as screen-
intrapituitary and peripheral hormone and cytokine signals.
ing test.
Disorders affecting the adrenal glands resulting excess
or insufficient production of adrenal products. Excess pro-
duction due to pathophysiologic processes as due to ACTH Cushing’s Syndrome
secreting pituitary tumors (70% of cases) is called Cushing’s
disease. Iatrogenic glucocorticoids, adrenal tumors or ecto- The diagnosis of the condition presents two great chal-
pic secretion of ACTH (malignant tumor of pulmonary ori- lenges:
gin) and rarely CRH producing ectopic tumor Cushing’s 1. To distinguish between pathologic and physiologic/
syndrome. other cortisol production
Insufficient adrenocortical function may occur primarily 2. To determine etiology of cortisol excess.
due to destruction of adrenal cortex called Addisons’s dis-
ease or secondarily due to hypothalamic-pituitary disease The condition is commonly seen in the age group of 20–50
or administration of exogenous steroids. years with a 5:1 female predilection. Central obesity, ple-
In clinical dentistry, cortisol deficiency or excess is thoric moon facies, cutaneous atrophy, purple striae with
overwhelmingly an iatrogenic disease, caused either by easy bruising, proximal muscle wasting, osteoporosis, hyper-
treatment of the patient with glucocorticoids—Cushing’s tension, diabetes mellitus, immunosuppression (including
syndrome or by patient withdrawal from previous treat- delayed hypersensitivity reaction), psychiatric problems,
ment—adrenal insufficiency. gonadal dysfunction and hypergonadism.
Characteristic central distribution of fat, thin skin with
Laboratory evaluation of adrenocortical function striae and bruising and proximal muscle weakness; osteo-
porosis in children and young females suggest pathologic
Urine levels: A 24-hour urine free cortisol measurement is causes rather than physiologic causes for excess cortisol
a reliable screening test. It is higher in the day time (7 AM– production.
7 PM) than at night (7 PM–7 AM)/urinary creatinine should
also be measured to determine the accuracy and adequacy
Actions of cortisol
of collection procedure.
Stimulation tests: These are useful in hormone deficiency ❍ Glucose metabolism
states. ACTH stimulation test is done to determine the func- ❍ Protein metabolism
tional reserve of adrenal gland for production of cortisol. ❍ Fat metabolism
545
❍ Anti-inflammatory action and not on the basis of glucocorticoid therapy. Patients with
❍ Psychic effect Cushing’s syndrome are also more likely to have Candida
❍ Permissive effect. and fungal infections, possibly due to abnormal flora on
the skin and mucosa.
Rapid onset with skin hyperpigmentation with severe
myopathy suggests ectopic causes of ACTH secretion. The
primary cause of death is cardiovascular disease followed Diagnosis
by infections and suicides. The diagnosis of Cushing’s syndrome is based on labora-
tory documentation of endogenous hypercortisolism.
Endocrinopathic pigmentation Bronzing of the skin and
Hematopoietic features are leukocytosis, lymphopenia and
patchy melanosis of the oral mucosa are signs of Addison’s
eosinopenia.
disease and pituitary-based Cushing’s syndrome. The cause
of hyperpigmentation is oversecretion of ACTH, which has
melanocyte-stimulating properties. In Addison’s disease, Radiographic features
adrenocortical insufficiency evolves as a consequence of Primary radiographic feature is generalized osteoporosis
granulomatous infection of the cortex or autoimmune cor- which shows a granular pattern. This demineralization
tical destruction. As steroid hormones decrease, the feed- leads to pathologic fractures. The skull shows diffuse thin-
back loop is stimulated with excess secretion of ACTH by ning with mottled appearance. The teeth may erupt pre-
the neurohypophysis. With a decrease in mineralocorticoids maturely and partial loss of lamina dura may be seen.
and glucocorticoids, the patient develops hypotension and
hypoglycemia, respectively.
In Cushing’s syndrome, adrenocortical hyperactivity is Adrenal Insufficiency
observed, and if such activity is caused by a cortical secre-
Primary Adrenal Cortical Insufficiency
tory adenoma or cortical hyperplasia of adrenal origin,
ACTH secretion will be shut down. Alternatively, if the (Addison’s Disease)
hypercorticism is the consequence of a pituitary ACTH- Primary adrenal cortical insufficiency is caused by a pro-
secreting tumor that secondarily induces an adrenal hyper- gressive destruction of adrenal cortex. The etiology is idio-
secretion, then melanocyte-stimulating effects may evolve. pathic or may result from hemorrhage, sepsis, infectious
Patients with Cushing’s syndrome may be hypertensive diseases, malignancies, adrenalectomy or drugs leading to
and hyperglycemic and may show facial edema (‘moon deficiency of the major hormones cortisol and aldosterone.
face’). In both cases, the skin may appear tanned, and the The original description of Addison’s disease—general lan-
gingiva, palate, and buccal mucosa may be blotchy. These guor and debility, feebleness of heart’s action, irritability
changes in pigmentation are due to an accumulation of of the stomach, and a peculiar change of the color of the
melanin granules as a consequence of increased hormone- skin.
dependent melanogenesis. Endocrinopathic disease should Another specific symptom of primary adrenocortical
be suspected whenever oral melanotic pigmentation is insufficiency is a craving for salt. Although hyponatremia
accompanied by cutaneous bronzing. Serum steroid and occurs in both primary and secondary adrenal insuffi-
ACTH determinations aid the diagnosis, and the pigment ciency, its pathophysiology in the two disorders differs. In
will disappear once appropriate therapy for the endocrine the primary condition, adrenocortical insufficiency is
problem is initiated. mainly due to aldosterone deficiency and sodium wasting,
whereas in the secondary form, adrenal insufficiency is
Dental considerations due to cortisol deficiency, increased vasopressin secretion
and water retention.
Patients who are on chronic glucocorticoid therapy have
decrease in subcutaneous collagen and the production of
Signs and symptoms of adrenal insufficiency
other extracellular proteins by fibroblasts, which may
explain the tendency of patients with Cushing’s syndrome ❍ Anorexia and weight loss
to bleed and to bruise easily. There may also be related ❍ Fatigue and weakness
defects in the walls of small blood vessels, resulting in defec- ❍ Gastrointestinal symptoms, nausea, diarrhea
tive constriction of these vessels during bleeding. Wound ❍ Myalgia, arthralgia, abdominal pain
healing is also impaired, and scar formation is less timely ❍ Orthostatic hypotension
and less vigorous than in a normal subject. ❍ Hyponatremia
Patients who are on chronic glucocorticoid therapy are ❍ Hyperkalemia
considered to be immunocompromised and more than nor- ❍ Hyperpigmentation
mally susceptible to infection. Antibiotic prophylaxis is ❍ Secondary deficiency of select hormones
decided on the basis of the underlying disease, however, ❍ Associated autoimmune conditions.
546
Secondary Adrenal Cortical Insufficiency Glucocorticoid replacement equivalent to the normal

physiologic output of the adrenals, i.e. in the range of 12.5
This is a more common condition which occurs as a result to 50 mg. Cortisol 25–30 mg or prednisone 7.5 mg per day
of hypothalamic/pituitary disease, surgical removal of would be adequate. In case of emergency inject contents
pituitary gland or administration of exogenous steroids. of prefilled dexamethasone (4-mg) syringe should be
Withdrawal of glucocorticoids after therapy increases the injected intramuscularly and physicians help sought as
release of CRH from hypothalamic peptidergic neurons. quickly as possible. Mineralocorticoid replacement in the
The pituitary is stimulated to increase ACTH secretion into form of 0.05–1 mg of fludrocortisone and adequate sodium
the blood. After glucocorticoid therapy, however, the intake can help addisonian patients lead a normal life.
adrenal glands may be atrophied and incapable of respond- Monitoring lying and standing blood pressure and pulse,
ing appropriately to increased ACTH. The inappropriate edema, serum potassium and plasma renin activity is
response to the adrenal glands as a consequence of the essential.
negative feedback mechanism and suppression of endog-
enous cortisol production through suppression of ACTH, Secondary adrenal insufficiency During stress in normal
POMC and CRH resulting in adrenal insufficiency. The individuals, plasma cortisol levels may double, suggesting
production of aldosterone which is independent of ACTH an inherent ability of the adrenal glands to increase corti-
is not appreciably affected. sol production by 100%. In the patient with adrenal insuf-
ficiency, adrenal function is inadequate to produce adequate
Acute Adrenal Insufficiency cortisol in the face of stress.
Secondary adrenal insufficiency due to hypothalamic/
Acute adrenal insufficiency could be a result of fulminat- pituitary disease may be managed as an ACTH dependent
ing septicemia caused by Pseudomonas and meningococci disorder and replaced with the missing glucocorticoid.
leading to bilateral adrenal hemorrhage in children called The clinical manifestation of adrenal insufficiency usu-
Waterhouse–Friderichsen syndrome. In adults, it could be ally occurs when a patient on gluococorticoids is being
use of anticoagulants or a coagulation disorder or a chronic withdrawn from glucocorticoid therapy or when a patient
insufficiency precipitating to acute stage because of stress with a previous history of glucocorticoid therapy is chal-
or profound infection. lenged by a stressful event. Subnormal cortisol production
during acute severe illness has been termed functional or
Diagnosis of adrenal insufficiency
relative adrenal insufficiency.
In patients in whom adrenal insufficiency is merely to Many patients with chronic inflammatory and autoim-
be ruled out, cortisol can be measured between 0800 and mune diseases such as oral lichen planus, pemphigus group
0900 hours. Hormonal pattern of morning plasma cortisol of diseases, pulmonary fibrosis, rheumatoid arthritis and
concentrations of less than 3 g/dl (83 nmol/l) are indica- severe asthma are on an alternate-day dosage regimen for
tive of clinical adrenal insufficiency whereas concentra- glucocorticoids.
tions of more than 19 g/dl (525 nmol/l) rule out the Alternate-day therapy (ADT) is usually reserved for sta-
disorder. ble chronic disease and permits the reactivation of adrenal
Measurement of plasma corticotropin can be used to dif- and pituitary function on the days during which no oral
ferentiate between primary and secondary adrenal insuf- glucocorticoid is given. For patients on long-acting gluco-
ficiency. In patients with primary adrenal insufficiency, corticoids, especially the most potent agents, no stimula-
plasma corticotropin concentrations invariably exceed tion of adrenal and pituitary function may occur on the
100 pg/ml (22 pmol/l), even if the plasma cortisol levels are off therapy day because of a long drug half-life. Pituitary
in the normal range. Normal plasma corticotropin values ACTH production takes place mainly at night. If circulat-
rule out primary, but not mild secondary, adrenal insuf- ing glucocorticoid levels are still elevated in the evening
ficiency. In primary adrenocortical insufficiency, basal hours, the ACTH produced will be insufficient to stimulate
plasma aldosterone concentrations are low or at the lower the adrenal glands, even during ‘off’ days. This is a particu-
end of normal values, whereas the PRA or concentration is lar problem with multiple daily dosing with glucocorticoids
increased because of sodium wasting. such as dexamethasone, which are most potent and which
have long half-lives.
Medical management Alternate-day therapy is a strategy for limiting adrenal
insufficiency and cushingoid effect. It is best managed
Primary adrenal insufficiency The primary medical needs
by using a high morning dose (7–8 AM) of prednisone or
of addisonian patient are:
another short-acting glucocorticoid. Initially, the total
1. Management of adrenal disease—elimination of infec- dose for the 2 days should be additive; if 20 mg is given
tious disease or malignancy every day, then 40 mg will be required on the ‘on’ day.
2. Hormonal replacement therapy. Physicians usually initiate ADT by gradually increasing
547
the ‘on’ day dosage while tapering the ‘off’ day dosage, intravenously thereafter every 8 hourly for 48–72 hours.
thus maintaining the total dose as a constant. In patients The dosage is then tapered to the preoperative main-
with recognized adrenal insufficiency, alternate-day dos- tenance level.
age can be life-threatening if significant stress occurs on
The steroids given are within 2 hours of surgery and read-
the ‘off’ day. If the patient cannot respond to stress and
iness to face any post-surgical complications exacerbating
does not increase the oral glucocorticoid dosage, then
adrenal insufficiency, liver dysfunction, sepsis and drugs
severe hypotension, nausea and shock may result. The
is warranted. Certain drugs like etomidate (anesthetic),
problem of an adequate response to stress is even more
aminoglutethimide (adrenolytic), ketaconazole lower plasma
significant in children with severe asthma; in who inhaled
cortisol levels. Inducers of hepatic cytochrome P45 oxygen-
glucocorticoid therapy has been shown to cause acute
ases like barbiturates, rifampicin and phenytoin accelerate
adrenal insufficiency.
cortisol degradation. Oral anticoagulants can be potenti-
Hence, three things have to be considered during an
ated by IV high doses of methylprednisolone.
ADT:
Stressful conditions that can precipitate adrenal crisis
1. ADT may be approached through transition schedule
that allow the patient to adjust gradually. ❍ Invasive surgical procedure
2. Supplementary non-steroid medications may be ❍ The onset of infection
needed on the off day to minimize symptoms of the ❍ An exacerbation of an underlying disease
underlying disease. ❍ Serious life event such as the death of a family member.
3. Symptoms occurring on the off day represent relative
adrenal insufficiency rather than exacerbation of Orodental treatment evaluation of patient with adrenal
underlying disease. dysfunction Before the dental procedure the kind and
degree of adrenal dysfunction is to be determined in con-
Known severe adrenal insufficiency usually requires the sultation with the patient’s physician. The risk of blood
premedication of the patient with 100 mg of hydrocorti- pressure variation, osteoporosis and peptic ulcers in long-
sone acetate intramuscularly 30 minutes before an inva- term steroid users should be considered.
sive procedure. Subclinical adrenal insufficiency may be The BP should be taken at baseline and monitored at
suspected after as little as 5 days of high-dose glucocorti- every appointment. Fractures are a risk in osteoporotic
coid therapy ( 60 mg of prednisone). patients and periodontal bone loss is common. Excessive
Salem et al (1994) have recommended the following: neck manipulation should be avoided in such patients.
Long-term use of NSAIDs to be considered in patients on
1. Minor surgical stress: 25 mg of hydrocortisone equiv- steroids considering the risk of developing peptic ulcers.
alent (refer to Table 5) on the day of surgery. For Use of appropriate sedation techniques minimizes stress.
instance, a patient on 5 mg of prednisone every day
may be given 5 mg of prednisone or 25 mg of hydro- Antibiotic prophylaxis A short course of antibiotic ther-
cortisone or equivalent preoperatively. apy is useful to prevent infections and delayed wound heal-
2. Moderate surgical stress: 50–75 mg of hydrocotisone ing due to immunosuppressive effects of steroids when
equivalent may have to be given for 2 days. For excessive manipulations of tissues are anticipated.
instance, a patient on 10 mg of prednisone daily should
Patients on daily steroid therapy for non-adrenal diseases
be given 10 mg of prednisone (or parenteral equiva-
Patients under these circumstances are usually on a main-
lent) preoperatively and 50 mg of intravenous hydro-
tenance dose of 5–60 mg of prednisone or equivalent.
cortisone intraoperatively. On the first postoperative
Oral examination does not require additional steroid
day 20 mg intravenous hydrocortisone can be given
supplements. Non-surgical Type II and some Type III and
8 hourly (60 mg/day). On the second postoperative day,
minor surgical procedures Type IV may induce mild to
the dosage is then tapered to the preoperative mainte-
moderate stress for which doubling the maintenance dose
nance level.
to a maximum of 60 mg prednisone or equivalent on the
3. Major surgical stress: 100–150 mg/day of hydrocor-
day of procedure tapered back to maintenance dose in
tisone equivalent may be required for 2–3 days. For
2 days would be adequate.
instance, a patient on long-term 40 mg of prednisone/
Invasive surgeries Type V and Type VI would require
day should receive 40 mg of prednisone (or parenteral
60 mg of prednisone on the day of surgery, equivalent dose
equivalent) preoperatively and 50 mg of hydrocorti-
of parenteral hydrocortisone intraoperatively and 50% of
sone intravenously thereafter every 8 hourly for
the dosage the day after, tapered to maintenance dose over
48–72 hours.
next 2–3 days.
But a patient on long-term 5 mg of prednisone/day
should receive 25 mg of prednisone (or parenteral Patients on alternate day steroid therapy for non-
equivalent) preoperatively and 25 mg of hydrocortisone adrenal diseases Patients under these circumstances have
548
Table 6 Protocol for supplementation of patients on glucocorticoid therapy who are undergoing dental care
Protocol
Dental Previous systemic steroid use Current systemic steroid Daily alternating systemic Current topical steroid
procedure use steroid use use
Routine If prior usage lasted for 2 weeks No supplementation Treat on steroid dosage day; No supplementation
procedures and ceased 14–30 days ago, give needed no further supplementation needed
previous maintenance dose needed
If prior usage ceased 14–30 days
ago, no supplementation needed
Extractions, If prior usage lasted 2 weeks and Double daily dose on day Treat on steroid dosage day, No supplementation
surgery, or ceased 14–30 days ago, give of procedure and give double daily dose on needed
extensive previous maintenance dose day of procedure
procedures If prior usage ceased 14–30 days Double daily dose on first Give normal daily dose on first
ago, no supplementation needed post-operative day when postoperative day when pain is
pain is anticipated anticipated
comparatively less adrenal suppression than on patients Treatment

on daily dosage.
Aldosterone antagonists—spironolactone—25 to 100 mg TDS.
Operative procedures should be scheduled on the ‘on’
day. No alterations are required for non-surgical Type II Secondary aldosteronism
and some Type III and minor surgical procedures Type IV.
For Type V and Type VI procedures doubling the dose to a Increased production of aldosterone in response to activa-
maximum of 60 mg prednisone and a single dose the day tion of the renin–angiotensin system. It occurs in associa-
after may be required followed by resumption of normal tion with the accelerated phase of hypertension or on the
schedule. If general anesthesia is indicated an ACTH stim- basis of underlying edema disorder due to overproduction
ulation test to determine the response of adrenals to exog- of renin.
enously administered steroids (Table 6). It may present in many forms of edema—cirrhosis,
nephrotic syndrome or cardiac failure and is characterized
Mineralocorticoids The main mineral corticoid is aldo- by hypokalemic alkalosis, moderate to severe increase in
sterone; cortisol has primarily a glucocorticoid effect and plasma renin activity and moderate to marked increase in
partially mineralocorticoid effect. aldosterone.
Function of aldosterone is to increase renal tubular
reabsorption of sodium and secretion of potassium thus
regulating the K concentration in ECF, renin–angiotensin Disorders of Adrenal Medulla
system, Na concentration and ACTH.
Pheochromocytoma
Aldosteronism Tumors of chromaffin tissue that secretes catecholamines,

90% of which are derived from adrenal medulla and rest
It is the hypersecretion of the mineralocorticoid aldosterone. in sympathetic ganglia.
The familial tendency is seen along with MEN type IIA
Primary Aldosteronism (Conn’s Syndrome) (pheochromocytoma medullary Ca of thyroid hyper-
parathyroidism) MEN type II B (multiple mucosal neuroma
Etiology resides within the adrenal gland. It is twice com- syndrome, von Hippel–Lindau syndrome and neurofibro-
mon in women aged 30–50 years. Clinical features include matosis I.
diastolic hypertension, headaches, muscle weakness and
fatigue. Other findings include polyuria, polydipsia and Clinical features
absence of edema. Hypertension is the most common sign and hypertensive
paroxysms or crises are alarming. It occurs in young to
Lab findings
mid adult life.
Hypokalemia less than 2 mEq/l, hypernatremia, metabolic Pallor, palpitations, sweating, headache, anxiety, abdom-
alkalosis and the assessment of ratio of serum aldosterone inal and chest pain, vomiting, constipation, weight loss,
to plasma renin activity. glucose intolerance are seen.
549
Most important aspect is the precipitation of hyper- ❍ Increased metabolic breakdown of folate (a deficiency
tensive crisis during surgery or trauma or any activity that can inhibit tissue repair).
displaces abdominal contents. Orthostatic hypertension
Estrogen and progesterone
and shock are expected. Any drug is not given unless a
thorough review is done. ❍ Effect ground substance of connective tissue by increas-
Adverse reactions are seen with opioid, histamines, ing fluidity
adrenocorticotropin and glucagons that directly release ❍ Concentrations increase in saliva and fluid with increased
catecholamine from the tumor; drugs like tricyclic antide- concentrations in serum.
pressants that block neuronal uptake of catecholamines, and
indirectly acting sympathomimetic drugs like methyl dopa. Oral manifestations
❍ Pregnancy epulis
Sex Hormones ❍ Recurrent aphthous ulcers
❍ Herpes labialis lesions
Androgens and estrogens are the essential hormones for ❍ Candida infections.
sexual development and fertility. The secretion of these
hormones is controlled by the H–P axis, GnRH, LH and Oral changes in menopause
FSH release.
❍ Thinning of the oral mucosa
The predominantly male sexual hormone, namely, the
❍ Burning mouth syndrome
androgens and testosterones regulate sexual differentia-
❍ Gingival recession
tion, virilization and hormonal changes accompanying
❍ Xerostomia
puberty, development of testes, ultimately leading to sper-
❍ Altered taste sensation
matogenesis and puberty.
❍ Alveolar bone loss
The female sex hormones, namely, the estrogen and
❍ Alveolar ridge resorption
progesterone are essential for development of sexual char-
❍ Osteoporosis.
acters, menstrual cycle and ovulation.
Geriatric patient and endocrine related
Causes of precocious sexual development
manifestations
❍ Idiopathic
❍ Impaired glucose tolerance—diabetes mellitus
❍ Gonadal disease
❍ Reduced thyroxin clearance and production
❍ Adrenal disease
❍ Increased ADH, reduced renin and aldosterone leading
❍ Hypothalamic disease
to decrease in Na and increased K
(e.g. Gonadotropin independent/dependent precocious
❍ Decreased testosterone leading to impotency
puberty, McCune–Albright’s syndrome).
❍ Decreased estrogen—burning mouth syndrome and
Hypogonadism, disorders of male reproductive system and osteoporosis
sexual differentiation are seen in chromosomal abnormalities ❍ Decreased vitamin D absorption and activation lead-
like Klinefelter syndrome, Turner syndrome, cryptorchi- ing to osteopenia and fractures.
dism among a few. Klinefelter syndrome shows dental
anamolies like taurodontism.
PREGNANCY
Effects of estrogen and progesterone on oral tissues
Estrogen Pregnancy is one condition where endocrine changes are
the most significant alterations due to production of
❍ Increases cellular proliferation in blood vessels (known
maternal and placental hormones.
in the endometrium)
❍ Decreases keratinization, while increasing epithelial
Dental management guidelines during pregnancy
glycogen.
❍ Developing rapport with the patient thereby reducing
Progesterone
anxiety since it is always a very emotional experience
❍ Increases vascular dilation, thus increases permeabil- for an expectant mother.
ity (result in edema and accumulation of inflamma- ❍ Detailed medical history and consultation with the
tory cells) patient’s obstetrician and physician.
❍ Increases proliferation of newly formed capillaries in ❍ Monitoring vital signs and blood examination because
gingival tissues (increased bleeding tendency) of development of anemia and folate deficiency during
❍ Alters rate and pattern of collagen production. pregnancy.
550
❍ Patient in the third trimester if positioned in a supine Anxiolytics Benzodiazepines and barbiturates are best
position may suffer from supine hypotensive syndrome avoided because of cleft palate development on prolonged
due to compression of inferior vena cava by the uterus. exposure or neonatal respiratory depression.
A left lateral positioning of the patient in such situation Nitous oxide is better used only in the second and third
is advisable. Always a short appointment semi-reclined trimesters and not more than 30 minutes with at least 50%
position, often changing of position is advisable. oxygen to prevent hypoxia.
❍ Pregnant patients of abnormal food habits, high sugar
Corticosteroids Prednisone can be safely used.
diet, high gag reflex and vomiting especially in the
first trimester causing high rate of caries and enamel
Oral manifestations
erosion.
❍ Preventive program to develop healthy oral environ- ❍ Pregnancy gingivitis and pyogenic granuloma mostly
ment. Studies have shown that reducing streptococci in the labial aspect of interdental papilla. It starts
count in mother reduces incidence of caries in the around the 2nd month and continues until after par-
child. turition and regresses.
❍ Prenatal fluoride 2.2 mg/day in the second and third ❍ Dental caries and enamel erosion and halitosis.
trimesters reduces incidence of caries in the child. ❍ Because of abnormal food habits and gastric regurgi-
❍ Odontogenic infections, sepsis and febrility have known tation.
to cause miscarriage. ❍ Chloasma gravidarum, pigmented lesion of the skin
❍ Elimination of periodontal irritants reduces chances and mucosa may have to be differentiated from other
of pregnancy gingivitis and pregnancy tumor. More- pigmentation disorders.
over, patients with periodontal infections have higher
chances of miscarriage, preterm birth and low birth Radiographs in pregnancy
weight.
Exposing the patient to X-radiation should be avoided dur-
❍ Second trimester is the safest period for elective dental
ing pregnancy because of the possible stochastic effects
treatment.
(uncertain effects) especially during the first trimester because
the developing fetus is susceptible to radiation damage.
Prescription of drugs
Under unavoidable circumstances exposing the patient
It is always safe to avoid any drug during pregnancy espe- for dental radiographs is considered safe provided precau-
cially during first trimester when the organs are in the for- tionary measures in minimizing radiation are established,
mative stage. The drugs may cross the placental barrier such as use of F-speed films, digital sensors.
and may be toxic or teratogenic to the fetus since the liver Long cone technique (200 mm FSD) filtration and rect-
and kidneys are still immature. angular collimation and use of lead aprons.
In unavoidable circumstances the drugs that are When a lead apron is used during contemporary dental
approved only may be given. radiography the gonadal and fetal effective is less than
0.01 Sv (microsieverts). No increase in gross congenital
Anesthetics Local anesthetics with adrenaline are rela-
anamolies or intrauterine growth retardation due to expo-
tively safe with required amount eventhough they cross the
sure during pregnancy has been observed up to less than
placental barrier. Lidocaine and prilocaine are safer than
5–10 cGy.
bupivacaine, which can cause fetal bradycardia.
Autoimmune polyendocrinopathy-candidiasis-ectodermal
Analgesics The analgesic of choice is paracetamol. Ibu- dystrophy (APECED), also called autoimmune polyendo-
profen is the next choice but is best avoided in the second crine syndrome (APS) type I, is a rare autosomal recessive
trimester. Other NSAIDs can constrict ductus arteriosus, disease with a complex picture discovered over decades.
postpartum hemorrhage and delayed labor especially in Chronic mucocutaneous candidiasis (MC), hypoparathy-
the third trimester. Prolonged high dose of opioids leads to roidism (HP) and adrenocortical failure (AF) are its most
congenital abnormalities and respiratory depression. common components. To define APS type 1, at least two of
the three major components need to be present.
Antibiotics Penicillins, erythromycin (except in etiolate
form) second and third generation cephalosporins, metroi- 1. Chronic candidiasis and T cell defect: In most cases of
nidazole and clindamycin are considered safe. However, APS type 1, chronic candidiasis is the first manifesta-
an increased dose or more frequent administration may tion of the disease, often occurring before the age of
be needed as the volume of distribution is increased in 5 years.
pregnancy. 2. Chronic hypoparathyroidism and parathyroid autoan-
Tetracycline is contraindiacated as it chelates to tibodies: In the course of APS type 1, candidiasis is
hydroxyapatite of developing teeth and causes enamel followed by chronic hypoparathyroidism, which usu-
hypoplasia. ally appears before the age of 10 years and affects
551
70–100% of patients. When chronic hypoparathyroid- occasionally predate any overt endocrine neoplasia, the
ism develops during the neonatal period, it is impor- recognition of these oropharyngeal lesions as possible
tant to differentiate this from genetic diseases such evidence of MEN III can lead to the early and sometimes
as DiGeorge’s syndrome (caused by a 22q11 deletion) successful treatment of the associated malignancies like
Kenney–Caffey disease (locus mapped to chromosome thyroid carcinoma which has a poor prognosis occurring
1q42–q43), or the Barakat syndrome (caused by in 18 to 25-year-old individuals. Thickening of the lip—
GATA3 haploinsufficiency). a characteristic ‘bumpy’or ‘blubbery’ lip appearance is seen.
3. Addison’s disease (AD) and adrenal cortex autoimmu- Marfanoid habitus, café au lait spots, lentigines and a his-
nity: In the course of APS type 1, AD tends to be the tory of diverticulosis or lower-bowel surgery are the features.
third disease to appear after chronic candidiasis and/ The finding of oral mucosal neuromas in association with a
or hypoparathyroidism, and it develops usually before family history of carcinoma of the thyroid or pheochromo-
15 years of age and affects 22–93% of patients. In most cytoma clearly indicates a need to search for other evidence
cases the disease is heralded by the presence of adre- of this syndrome. Endocrine abnormalities are also some-
nocortical autoantibodies (ACA), frequently found at the times present in patients who have other inherited syndromes
onset of the other main clinical manifestations of this with neoplastic associations, such as neurofibromatosis 1,
type of APS (candidiasis and/or hypoparathyroidism). McCune–Albright’s syndrome and von Hippel–Lindau syn-
drome. These conditions are usually excluded from the
Systemic lupus erythematosus definition of multiple endocrine neoplasias.
A hormonal component to SLE is suggested, by its high
Diffuse endocrine system
incidence in women in their child bearing years, the many
reports of remission during pregnancy, and the finding of This group of widely scattered endocrine tissues consisting
increased estrogen levels in SLE patients suggest an endo- of AUPD cells, are called so because of their properties—
crinal role. a high content of amines; the capacity of amine precursor
uptake; the presence of amino acid decarboxylase. The
cells of these tissue contain characteristic granules and
Multiple Endocrine Neoplasia Type III secrete polypeptides and amines. The origin of the tissues
(Multiple Mucosal Neuroma Syndrome) is believed to be from the neural crest cells. Neuroendocrinal
tumors are rare in the oral cavity.
Multiple endocrine neoplasia types I to III (MEN I, II,
and III) is a group of familial syndromes in which neoplas- Merkel cell carcinoma or (MCC) or neuroendocrine car-
tic change occurs in several endocrine glands in one cinoma It is a rare but aggressive malignancy of the skin
individual. with a rare intraoral occurrence. Only 14 cases of intraoral
MEN I involves lesions in some combination of pancre- Merkel cell carcinoma have been reported (Yoshida et al,
atic islets, adrenal cortex, and parathyroid and pituitary 2001).
glands; it includes Zollinger–Ellison (or gastrinoma) syn- Factors strongly associated with the development of
drome, in which multiple primary gastrin-secreting ade- MCC are:
nomas or adenocarcinomas are located in the pancreas,
❍ Age more than 65 years
duodenum, or even extra-abdominal sites such as the
❍ Sex predilection—males
parathyroid gland. Stomach ulceration and hyperplasia of
❍ Fair skin
the pancreatic islets and parathyroid glands develop sec-
❍ History of extensive sun exposure
ondary to the excess gastrin release and account for the
❍ Chronic immune suppression (e.g. kidney or heart
characteristic presenting symptoms. Between one-quarter
transplantation or HIV)
and one-half of gastrinomas have other features of the
❍ Association with other premalignant and malignant
MEN I syndrome, which is not associated with any skin or
skin lesions
oral phakomatosis.
❍ Common sites in the head and neck region are the
MEN II A which involves medullary carcinoma of the
cheeks, nose, mouth, eyelids and periocular region.
thyroid gland, pheochromocytoma of the adrenal medulla,
and parathyroid hyperplasia or adenoma, is not associated
Clinical presentation
with any phakomatosis.
MEN II B or MEN III, a subgroup of these patients The common clinical findings include a nodule with or
exhibits multiple neuromas of the commissural mucosa without ulceration. The nodule may be usually firm and
(which is characteristic) lips, tongue, and buccal, conjunc- erythematous. Pain may or may not be an associated fea-
tival, nasal, and pharyngeal mucosae, in association with ture. MCC very rarely occurs on mucous membranes of the
their endocrine neoplasia (this is referred to as multiple head and neck region, and it carries a particularly poor
mucosal neuroma syndrome). Since these neuromas may prognosis.
552
SALIVA AND MONITORING OF HORMONE salivary testosterone levels may also be useful in behav-
LEVELS ioral studies of aggression, depression, abuse, violent and
antisocial behavior.
Saliva can be analyzed as part of the evaluation of endo- Estradiol can be detected in saliva in concentrations
crine function. The majority of hormones enter saliva by that are only 1–2% of serum concentrations. These con-
passive diffusion across the acinar cells. Most of these hor- centrations are similar to the serum concentrations of free
mones are lipid soluble (i.e. steroids). Small polar molecules estradiol, which can diffuse into saliva. Salivary estradiol
do not readily diffuse across cells and instead enter saliva levels followed the same trends as serum estradiol levels
through the tight junctions between cells. The molecular- during a menstrual cycle. Furthermore, salivary estriol
weight cut-off for ultrafiltration is 100–200. This rela- levels showed a very high correlation with serum levels of
tively small molecular size prevents many hormones from free estriol in pregnant women, and salivary estriol levels
entering saliva from serum by means of ultrafiltration. In were suggested as a means for the assessment of feto-pla-
addition, active transport does not appear to facilitate hor- cental function.
mone transfer into saliva. Measurements of salivary hor- Salivary progesterone levels showed good correlation
mone levels are of clinical importance if they accurately with serum levels during the menstrual cycle and reflected
reflect the serum hormone levels or if a constant correlation the free serum progesterone levels. Salivary progesterone
exists between salivary and serum hormone levels. For levels can be useful for the prediction of ovulation, with
neutral steroids which diffuse readily into saliva, salivary serum progesterone levels, and salivary estradiol and pro-
hormone levels represent the free serum hormone levels. gesterone levels can be used for the evaluation of ovarian
Conversely, due to their size, protein hormones do not enter function. Decreased salivary estriol is suggested as a marker
saliva through passive diffusion, but primarily through of fetal growth retardation. Furthermore, an increased sal-
contamination from serum as a result of outflow of gingi- ivary estriol-to-progesterone ratio may be a predictor of
val crevicular fluid (GCF) or from oral wounds. Furthermore, pre-term delivery.
some steroid hormones can be metabolized in the salivary In general, serum and salivary levels of protein hor-
epithelial cells by intracellular enzymes during transcel- mones are not well correlated. These hormones are too
lular diffusion, which can affect the availability of these large to reach saliva by means of passive diffusion across
hormones in saliva. cells or by ultrafiltration, and the detection of these hor-
Due to their lipid solubility, steroid hormones can be mones in saliva is primarily due to contamination from
detected in saliva. Salivary cortisol levels demonstrate serum through GCF or oral wounds. Therefore, serum lev-
excellent correlation with free serum cortisol levels. How- els of protein hormones such as gonadotrophins, prolactin
ever, the actual salivary cortisol levels are lower than the and thyrotropin cannot be accurately monitored by means
serum-free cortisol levels, possibly due to enzymatic deg- of salivary analysis (Vining and McGinley, 1986, 1987).
radation in the salivary epithelial cells during transcellular Salivary monitoring of hormone levels has many advan-
diffusion. Salivary cortisol levels were found to be useful in tages over the more conventional serum analysis. Hormone
identifying patients with Cushing’s syndrome and Addison’s evaluation often necessitates multiple sample collection in
disease and also for monitoring the hormone response to a relatively short time interval, which makes the non-
physical exercise and the effect of acceleration stress. invasive collection of saliva ideal for this purpose. However,
Contrary to cortisol, salivary cortisone, prednisone and it is important to consider the possible limitations of sali-
prednisolone levels do not accurately reflect serum corti- vary analysis for hormone evaluation. Hormones enter
sone levels. Cortisone is a neutral steroid and therefore saliva by passive diffusion and ultrafiltration, and active
readily diffuses into saliva; however, cortisol is converted transport of hormones into saliva does not exist. Therefore,
to cortisone by an enzyme present in the salivary glands mostly lipid-soluble and hormones with small molecular
(11 ß-hydroxysteroid dehydrogenase). Thus, cortisone lev- weight can be detected in saliva. Most hormones are
els in saliva are higher than in serum and do not bear any protein-bound in serum, and thus salivary hormone levels
diagnostic significance. represent the free hormone levels which are available for
Salivary aldosterone levels have demonstrated a high diffusion into saliva. This may provide more clinically use-
correlation with serum aldosterone levels with the use of a ful information, since free serum hormone levels are the
solid-phase enzyme immunoassay. Increased aldosterone biologically active fraction of hormone in serum. For accu-
levels were found in both the serum and saliva of patients rate results, a constant and predictable correlation must
with primary aldosteronism (Conn’s syndrome). exist between salivary and serum hormone levels. However,
Testosterone and dehydroepiandrosterone have also been different hormones are bound to similar serum carrier pro-
identified in saliva. Salivary concentrations were found teins, and thus changes in levels of one hormone may
to be 1.5–7.5% of the serum concentrations of these hor- affect the free levels of others. For hormones that demon-
mones with direct radioimmunoassay technique. Monitoring strate a constant but low salivary-to-serum ratio, a suffi-
ciently large sample volume or a more sensitive analysis
553
method is required. In addition, many hormones exhibit perceptions of the public, media, arts and the society at
marked circadian variations. Therefore, timing of saliva large toward the ‘mad’ among us.
collection may affect the results. The salivary flow rate For centuries mental health problems and their treat-
can also affect the concentrations of certain hormones. An ments has been the realm of not only ‘physicians’ but also
increase in salivary flow rate will usually result in reduced a range of other professionals including ‘faith healers’. Till
concentrations of molecules that reach saliva by diffusion. the 18th century the mentally ill were secluded in ‘institu-
However, the rate of diffusion of steroid hormones, par- tions’ and ‘asylums’. In the next stage, mid 18th century,
ticularly cortisol, is usually high enough to maintain a con- some physicians in England and France started recogniz-
stant relationship between salivary and serum levels of the ing themselves as having a special interest in dealing with
hormone regardless of the salivary flow rate. The concen- mental health. But still there was no treatment available
trations of hormones that reach saliva by ultrafiltration, and the mentally ill were ‘chained’ at large. It was Philippe
such as dehydroepiandrosterone sulfate, are more affected Pinel, a French physician who in 1793 started an initiative
by changes in salivary flow rate. Changes in salivary flow toward the ‘moral treatment’ of the mentally ill and ‘liber-
rate may lead to changes in salivary pH. This may affect ated’ the insane from their chains.
the entry into saliva of molecules according to their pKa. The first half of 20th century saw the accidental discov-
The stability of hormones in saliva is important as well for ery of malaria being therapeutic for psychosis in tertiary
accurate evaluation. Hormones in saliva can be degraded, syphilis. This led to the dangerous practice of injecting
among other ways, by enzymes native to saliva, enzymes milk to induce fever. Inducing seizures by ‘insulin coma
derived from oral microorganisms, and enzymes derived therapy’ or injecting ‘camphor oil’ which was considered
from leukocytes that enter the oral cavity from the gingi- a successful treatment for ‘schizophrenia’ then is largely
val sulcus. In addition, molecules that reach saliva by pas- ‘barbaric’ this day in age. The biggest breakthrough for
sive diffusion across cells, like unconjugated steroids, may Psychiatry came in the 1950s with the discovery of ‘chlor-
be subjected to enzymatic degradation within the salivary promazine’ and the First World Congress of Psychiatry at
glands, prior to entering saliva. These factors have to be Paris. Since then Psychiatry has probably seen the inven-
considered when saliva is evaluated as an alternative for tion of numerous new medications, perhaps much more
the evaluation of serum hormone levels. than most other branch of medicine. However, present day
psychiatry has outgrown the medical model and has incor-
porated a ‘bio-psycho-social’ model both for understand-
ing and treating mental illness.
MENTAL HEALTH AND ITS RELEVANCE TO
DENTISTRY
Concepts of Mental Illness and
This section aims at equipping the reader with skills to its Classification
recognize the commonly seen mental illness. In addition
Similar to using the word ‘illness’ loosely in everyday
to giving a broad overview of mental illness and present
speech, the word ‘mental illness’ is used with little preci-
day psychiatric practice we intend to focus on the inter-
sion in psychiatric practice, and often synonymously with
face between ‘dentistry’ and ‘psychiatry’. We will also give
‘mental disorder’. In this context, ‘mental’ and ‘psychiatric’
you a broad overview of the treatment options available
are also used interchangeably. A very diverse discussion of
in psychiatry. In addition to making you confident in rec-
the concepts of mental illness can be found in the works
ognizing the mental illness to refer them to psychiatry,
of Lazare (1973), Kendell (1975), Hafner (1987) and Clare
this section will give you confidence in dealing with the
(1997). It is probably beyond the scope of this discussion
mentally ill when they develop dental problems.
to go into the depths of these concepts.
History of Psychiatry
Definitions of Mental Illness
Tracing the timeline backwards, in 10,000 BC there was no
There have been numerous attempts to define mental illness
difference between medicine, magic and religion. Looking
but none have been satisfactory and uniformly accepted.
at the history of medicine it may seem that Psychiatry as
One of the common approaches is to examine the concept
a specialty is relatively new. However the history of Psy-
of illness as in general medicine and to identify any analo-
chiatry dates back to more than 2,000 years when medi-
gies with mental illness. In general medicine there are five
cine itself had its birth as ‘Science’ in ancient Greece. In
types of definitions:
the ancient world, Psychiatric illness was believed to come
from the Gods and the curse of the devils. People have ❍ Absence of health
always been fascinated and puzzled, not being able to ❍ Disease is what doctor treats
understand ‘insanity’. It is interesting to note the varying ❍ Biological disadvantage
554
❍ Pathological process
The five axes of DSM IV
❍ Presence of suffering.
Axis I Clinical syndrome and ‘conditions not attributable to mental
Efforts to classify psychiatric disorders have been tried by disorder that are the focus of attention and treatment’
many over the years. Since there are no definite diagnostic Axis II Personality disorders
tests such as radiograph for fracture or blood test for malaria Axis III Physical disorder and conditions
we need different tools to provide a conceptual framework
Axis IV Severity of psychological stressors
to the psychiatric illness. Having a classificatory system
helps us to communicate with other professionals and to Axis V Highest level of adaptive functioning in the last year
decide on treatment and prognosis.
Criticisms of classifications
There is no doubt that classification is needed in psychia-
Current Psychiatric Classifications
try and it helps the clinicians to communicate with one
The two major classificatory systems in use for the psychi- another about the diagnoses given to a patient and it helps
atric disorders are the ICD (International Classification to understand the implications of the diagnoses, in terms
of Diseases)-10 and the DSM (Diagnostic and Statistical of their symptoms, prognosis, treatment and sometimes
Manual) IV and it is important to have the basic under- etiology. However, such classifications, on many occasions
standing of both. are criticized as being inappropriate or even harmful. It can
be argued that allocating patients to a diagnostic category
International Classification of Diseases (ICD)-10, distracts form the understanding of their unique personal
Chapter V difficulties. It is important to combine the two because these
qualities can modify prognosis and these personal difficul-
The ICD is proposed by the World Health Organization ties should be taken into account for a holistic treatment
(WHO) as an aid to the collection of international statistics of the disorder. It is also worth noting that some conditions
about disease. The system is revised every few years and could be sub-threshold and resemble the disorders in the
the current edition is the tenth (ICD-10). It has 22 chapters classification but do not meet full diagnostic criteria. It is
in it and the fifth chapter is devoted to psychiatry. not infrequent to see such patients presenting to a variety
of services other than psychiatry. These sub-threshold con-
Main categories of ICD-10 Chapter V (F) ditions are however clinically significant and it is impor-
F00–09 Organic including symptomatic mental disorders tant to recognize these conditions to reduce the psychiatry
morbidity or comorbidity.
F10–F19 Mental and behavioral disorders due to psychoactive
substance use
F20–F29 Schizophrenia, schizotypal and delusional disorders Diagnostic Criteria and Screening Questions
F30–F39 Mood (affective) disorders
We intend to introduce the readers to the common psychi-
F40–F49 Neurotic, stress related and somatoform disorders
atric illnesses. This section should familiarize you with the
F50–F59 Behavioral syndromes associated with psychological brief epidemiological details, diagnostic criteria and screen-
disturbances and physical factors ing questions to be used in everyday practice.
F60–F69 Disorders of adult personality and behavior A functional psychiatric illness should always be diag-
F70–F79 Mental retardation nosed after ruling out any physical condition that can
F80–F89 Disorders of psychological development explain the presentation. For example, a middle-aged lady
F90–F99 Behavioral and emotional disorders with onset usually with hypothyroidism may present with depression as the
occurring in childhood or adolescence only symptom. It is important to be aware of this aspect
as any psychiatric presentation could be mimicked by an
organic condition or by alcohol/illicit drug use.
Diagnostic and Statistical Manual (DSM)
Alcohol and substance use
The DSM is produced by the American Psychiatric Associ-
ation (APA). The first edition, DSM-I was published in 1952 The available epidemiological data suggests a combined
as an alternative to the widely criticized ICD-6. It seems 1 year prevalence rate of alcohol misuse and dependence
that the DSM-I was strongly influenced by the views of to be 7–10% (Kessler et al, 1994). An understanding of the
Adolf Meyer and Karl Menninger. Its simple glossary reflects effects of alcohol and other substances of abuse (tobacco,
the acceptance of the then prevalent psychoanalytic ideas heroin, cannabis, cocaine) is important for the practice of
in the United States. The current version is the DSM IV dentists. When such patients present with oral complications
and it has five axes as shown below. of prolonged substance misuse, in addition to treating the
555
oral condition it becomes important to liaise with the men- lasts for more than 2 weeks duration. It has to be differen-
tal health services to treat the dependence. We aim to equip tiated from normal sadness. Such patients have impaired
you to diagnose these dependence syndromes with the diag- patterns of mood, thoughts and behavior which sometimes
nostic criteria which are common for all substances. lasts for a long period of time. It causes a lot of distress to
the person and impairs his/her quality of life. It is also
Three or more of the following to be present during the previous accompanied by a high rate of suicide which is at least 7%
year in men and 1% in women (Blair-West et al, 1999; Weissman
1. Strong desire or a sense of compulsion to take the substance et al, 1996). The 12-month prevalence of depression in the
(craving) community is between 2 and 5% and the lifetime rate lies
2. Difficulty in controlling substance taking behavior (loss of control) between 10 and 20% (Alonso et al, 2004). In 2000, the
WHO identified major depressive disorder as the fourth
3. Physiological withdrawal symptoms when substance use is reduced,
e.g. tremousless, anxiety and lack of sleep with alcohol withdrawal
ranked cause of disability and premature death in the
world (Murray and Lopez, 1997). WHO has projected that
4. Need to increase the intake over a period of time to get the same
by 2020, major depression will rise in disease burden to be
effect (tolerance)
second only to ischemic heart disease. This disorder is very
5. Neglect of alternative pleasures or interests (salience)
common all over the world with a lifetime prevalence rate
6. Continuing to use despite knowing it to be harmful of 17% and a recurrence rate of more than 50% (Kessler
World Health Organization, 1992. et al, 1994; Weissman et al, 1996). People with chronic dis-
ease have a higher chance of developing depression. It is
A commonly used screening tool for alcohol is CAGE which 40% for people with coronary artery disease and 25% for
can be administered in a minute or less is shown in the patients with cancer (Musselman et al, 1998; Patten, 1999).
box below. Neurological disorders associated with a higher frequency
of depression include multiple sclerosis, Parkinson’s dis-
CAGE questionnaire ease, head trauma and stroke (Patten et al, 2000; Poewe
and Luginger, 1999). Around one-third of patients with
1. Have you ever felt you should cut down on your drinking?
depression develop alcohol or illicit substance misuse in
2. Have people annoyed you by criticizing your drinking?
their lifetime (Baker and Dawe, 2005).
3. Have you ever felt bad or guilty about your drinking?
4. Have you ever had a drink first thing in the morning to steady your
nerves or to get rid of a hangover (eye opener)? Most people with depression have five or six of the following:
Each Yes is scored 1 point. Score of 2 suggests significant alcohol • Feeling sad most of the time (but may feel a little better in the
problems. evening)
• Lose interest and enjoyment in life (anhedonia)
• Feeling tired easily with reduced energy levels
List of commonly misused drugs
• Reduced concentration in day-to-day tasks
• Amphetamines • Cocaine • Find it harder to make decisions
• Speedball • Heroin • Cannot cope with things that they used to
• Cannabis, hashish • LSD • Feel restless and agitated
• Ecstasy, ketamine • Inhalants • Lose appetite and weight
• Steroids • Ether • Take 1–2 hours to get off to sleep, and then wake up earlier than
• GHB • Glue usual
• Ritalin, diet pills, rush • THC, marijuana • Lose interest in sex
• Barbiturates, Valium, Ativan • Speed • Loss of self-confidence
• Opium • Crack • Feel useless, inadequate and hopeless
• PCP, Angel dust • Morphine, methadone • Avoid other people
• MDA, MDMA • Crystal meth • Feeling guilty about trivial issues
• Grass • Freebase • Feel irritable
• Feel worse at a particular time each day, usually in the morning
Depression • Having ideas or acts of self-harm or suicide

• Have a bleak and pessimistic view of the future
Depressive disorder also known as major depressive dis-
World Health Organization, 1992.
order or unipolar depression is a psychiatric illness which
556
Psychoses including schizophrenia and out of their mind or as if the thoughts in their mind are not
delusional disorders their own, but someone else has put them in their mind.
While patients with schizophrenia experience any of the
In psychotic illnesses patients suffer from strange and
above core psychotic symptoms, delusional disorders are
unusual experiences and beliefs that are beyond our
characterized by either a single delusion or a set of related
understanding. Schizophrenia is a mental disorder that
delusions which are usually persistent and sometimes life-
affects around 1 in every 100 people. In one-third of the
long. These delusions are clearly personal rather than being
patients it can can become severe and enduring. The most
subcultural and occur in the absence of brain disease.
common symptoms are hallucinations and delusions.
Auditory hallucinations if present are only occasional
A hallucination is a phenomenon where people hear, see,
(WHO, 1992). The delusional disorders are generally more
feel or smell something when there is nothing or nobody
difficult to treat. Though most of the times psychosis is
to account for it. In functional psychotic illnesses the most
just picked up from the patient’s conversation, the box below
common is hearing voices that seem to be utterly real and
gives a list of screening questions.
coming from the outer world although other people cannot
hear them. These voice/voices can either talk directly to
the patient or talk to each other about the patient. The These questions only form a guide to clarify when you suspect
patient might report that he/she is overhearing a conversa- something abnormal and only the most relevant questions should
be asked keeping in mind the educational and cultural background:
tion. Though the voice can be pleasant they are more often
rude, critical and abusive, irritating and sometimes com- Persecution
manding. These commanding hallucinations increase the How do you get on with others?
risk of harm to self and significant others. Do you believe that people are trying to harm you or make your life
Delusion is belief that the patient holds with complete miserable?
convictions although it seems to be based on a misinterpre- Is there a plot to cause harm to you?
tation or misunderstanding of the situation or event. When Reference
we ask the patient why they believe in it, their reasons do not Do people talk behind your back?
make sense to us or they say that they cannot explain it and Do things seem specially arranged for you?
Do you see any reference to yourself in the TV or newspaper?
‘they just know it’. So it is customary to define ‘delusion’
as a false unshakable belief, which is out of keeping with the Grandiosity
patient’s social and cultural background (Hamilton, 1984). How do you see yourself compared to others?
Do you have any special powers or abilities?
The most common in this category are ‘paranoid’ delusions
Are you specially chosen in any way?
and delusions of ‘reference’ or ideas of reference. The par-
anoid delusions or delusional ideas make the patient feel Thought disorder
persecuted or harassed. For example, the patient might Are you able to think clearly?
Is there any sort of interference with your thinking process?
believe that he/she is being influenced by the neighbours
Do you think people around you can read your mind?
who are using special powers or technology. Another exam-
ple is where the patient starts to believe that his/her partner Always check for conviction, explanations and coping
How do you know this is the explanation?
is unfaithful. This belief is based on odd details that seem
Could it be your imagination or your ‘mind playing tricks’?
to have nothing to do with sex or infidelity. In such cases
What do your family and friends think about this problem?
other people see nothing to suggest that the belief might What do you intend to do about this problem?
be true. Ideas of reference occur when the patients start
getting a special meaning in ordinary day-to-day events
and believe that they are specially connected to them. For Anxiety, stress-related and somatoform
example, radio or television programs are about them or disorders
people are communicating to them in odd ways, such as Anxiety is a normal human feeling and we all would have
through the color of cars in the street. experienced it when faced with situations that we find dif-
People with a more severe form of psychosis suffer ficult or threatening. The best example would be an exam-
from ‘thought disorder’ in which their thought process is ination or interview. Often people refer to it as ‘stress’
muddled. Their ideas are disconnected in such a way that which is confusing and can mean different things. It may
it is hard for other people to understand. Some patients refer to things that make one anxious or on the other hand
experience as if their feelings or actions are controlled by it could be a reaction to being faced with anxiety provok-
an external agency as if they are being controlled like a ing situations. It is important to differentiate ‘anxiety’
puppet or a robot. So people experience as if their thought from ‘worry’ and ‘fear’. When anxiety is a result of a con-
process is being interfered with. It could be that their tinuing problem like financial problem we call it ‘worry’.
thoughts are vanishing as though someone is taking them A sudden response to an immediate threat like looking over
557
a cliff is ‘fear’. Both fear and anxiety can be helpful, help- take to avoid the fearful situation. This ‘avoidance behav-
ing us to avoid dangerous situations, making us alert and ior’ keeps expanding to include more and more situations
motivating us to deal with problems. However, if it becomes and circumstances. The sufferer usually knows that there is
too strong or goes on for too long it can interfere with our no actual danger, may feel silly about this fear but still is
daily activities and make our lives miserable. unable to control it. Such a phobia can start after a distress-
Anxiety causes numerous physiological (in the body) ing or a traumatic experience, many a times in early life.
symptoms and cognitive symptoms (in the mind) shown in Many of us worry before meeting new people and before
two separate boxes below. going to parties but once we are there we can cope and
enjoy the situation. Some people become very anxious about
Physiological symptoms of anxiety such situations, cannot enjoy them and at worse totally
• Irregular hear beats (palpitations) avoid such social situations. This condition is called ‘social
• Sweating
phobia’. Such people tend to worry about becoming the
center of attention wherever they find themselves among
• Muscle tension and pain
people. They worry that everybody is looking at them and
• Shakes and tremors watching what they are doing. At their worst, these feel-
• Butterflies in the stomach ings of fear and bodily symptoms can end up in a ‘panic
• Tightness in the chest attack’. A panic attack lasts for a few minutes during which
• Breathing heavily the person feels overwhelmingly anxious and is terrified
• Dizziness
of losing control, going mad or dying. These feelings reach
a peak and then pass off rapidly leaving the person feeling
• Faintness
weak and exhausted.
• Indigestion
• Diarrhea Panic disorder
Panic disorder is a type of mental illness with recurrent
Cognitive symptoms of anxiety and unexpected intense episodes of anxiety called as panic
• Fear of ‘going mad’ attacks. A panic attack is characterized by intense appre-
• Fear of ‘passing out’ or ‘imminent death’ hension and terror accompanied by physical symptoms
like palpitations, chest pain, dizziness, sweating and dif-
• Fear of having a ‘heart attack’
ficulty in breathing. These attacks have an abrupt onset
• Fear of a serious physical health problem
and peak in intensity within 10 minutes. These attacks are
• Feeling worried all the time not associated with any external event or situation and
• Feeling excessively tired come out of the blue. Such episodes often result in calls to
• Unable to concentrate paramedics and visits Accident and Emergency Department.
• Feeling irritable Panic disorder could sometime be a lifelong illness that
remains only partially responsive to treatment. Women
• Sleep problems
appear to have more severe form of the disease than men
(Yonkers et al, 1998) and is much more commoner in women
Phobias and social phobia (Barzega et al, 2001; Kessler et al, 1994).
Phobia is fear of a particular situation or particular things
Agoraphobia
that are not dangerous and which most people do not
find it dangerous. We all have fears about things such as The term ‘agoraphobia’ means ‘fear of open spaces’. Such
height and spiders but, for most of us, these fears do not people not only fear open spaces but also presence of a
interfere with the way we lead our lives. These fears are crowd. They fear not having an immediate and easy escape
called phobias only when they interfere with things we to a safe place (usually home). Such patients generally
would otherwise enjoy or do easily. A person with phobia have a fear of leaving home, fear of entering shops, crowds,
has the same intense symptoms of anxiety described above public places, or of traveling alone in trains, buses, or
but these arise only in the particular situations that planes. The severity of anxiety symptoms and the extent
frighten them. At other times they do not feel anxious. of avoidance varies from patient to patient. However, for
For example, a person with phobia of dogs is comfortable some people it is the most incapacitating of all phobic
when there are no dogs around. However, phobia makes disorders and they become completely housebound. Some
the person avoid situations in which they know they will people are terrified by the thought of collapsing and being
be anxious. This ‘avoidance’ actually makes the phobia left helpless in the public. The lack of an immediately
worse as time goes on. Over time the person’s life becomes available exit seems to be a key feature in these patients
increasingly dominated by the precautions he/she needs to with agoraphobia who tend to be women in early adult life.
558
Depression and social phobia may also be present but do obscene or simply because they are perceived to be senseless.
not dominate the clinical picture. The person often tries to resist these thoughts but is unsuc-
People feel grief-stricken, depressed, anxious and angry cessful. Obsessional thoughts could be in the form of ideas,
after a traumatic experience. A traumatic event is where images or impulses that enters the individual’s mind again
one is in danger; one’s life is threatened or sees other peo- and again in a stereotyped fashion. Compulsive acts are
ple dying or being injured. The condition is called post- also known as rituals. These are stereotyped behaviors
traumatic stress disorder (PTSD) when one starts re-living repeated again and again. They are not inherently enjoy-
the trauma again and again in the form of ‘flashbacks’ able and they do not result in the completion of any useful
during the day or ‘nightmares’ in sleep. In addition, they thoughts. The individual often views the compulsions to
start avoiding related situations, become emotionally be reducing the anxiety caused by the obsessions. Usually
numb and ‘hypervigilant’, unable to relax. The emotional these repetitive acts are considered to be pointless and the
reaction in stress is often accompanied by other symptoms patient tries to resist them, only to yield to them when the
of anxiety listed in the boxes on page 558. anxiety builds up. However, in very long-standing cases
The questions in the box below help you to screen for the resistance may be minimal. There is often a close rela-
the anxiety disorders. tionship between obsessional symptoms and depression.
The most common ritual seen is frequent hand washing as
Panic attacks
a result of obsessive thoughts that one might be contami-
• Have you had spells or attacks when you suddenly felt anxious, nated by germs, dirt or HIV. The other commonly seen
frightened, uncomfortable or uneasy, even in situations where most compulsion is a checking behavior.
people would not feel that way? Did the spells surge to a peak, About 1 in 50 people suffer from OCD at some point in
within 10 minutes of starting? their lives. Men and women are equally affected. It affects
• Do you feel anxious or uneasy in places or situations where you work, relationships and family life of the patient. People
might have a panic attack or panic-like symptoms, or where help with a severe OCD also cause burden on their caretakers.
might not be available or escape might be difficult: like being in Some people with mild OCD improve without any treat-
a crowd, standing in a queue, when you are away from home or ment. Some will slowly get worse and some get worse when
alone at home, or when crossing a bridge, traveling in a bus, train
they are stressed and depressed. The mainstay of treatment
or car?
for OCD continues to be talking therapies like exposure and
Social phobia response prevention and guided self-help. Antidepressant
• Have you been fearful or embarrassed of being watched, being the
medications can be used alone or in combination with
focus of attention, or fearful of being humiliated? This includes things
talking therapies for moderate to severe OCD.
like speaking in public, eating in public or with others, writing while
someone watches, or being in social situations.
Post-traumatic stress disorder Hypochondriasis
• Have you ever experienced or witnessed or had to deal with
Patients with hypochondriasis are preoccupied by a fear of
an extremely traumatic event that included actual or threatened
death or serious injury to you or someone else? Examples of
having a serious disease based on the misinterpretation of
traumatic events include serious accidents, sexual or physical their bodily symptoms (American Psychiatric Association,
assault, a terrorist attack, being held hostage, kidnapping, fire, 1994). This serious preoccupation persists despite negative
discovering a body, sudden death of someone close to you, investigations and causes distress with impaired function-
war, or natural disaster. ing of the patient. The central and diagnostic clinical fea-
• Have you had a dental appointment or a medical intervention in ture is the preoccupation with the idea of having a serious
the past which you found significantly traumatic? What was the medical condition, usually one which will lead to death or
reason you found it to be traumatic? Was it because you were not serious disability. The patient usually ruminates repeatedly
adequately prepared due to lack of information as to what was on this possibility. The minor and insignificant bodily
going to happen?
abnormalities, normal variants, normal functions and minor
ailments will be interpreted as signs of the serious disease.
The patient will consequently seek medical advice and
Obsessive compulsive disorder
investigation but is unable to be reassured by the negative
We commonly use phrases like, ‘He’s an obsessive cricket results. Such patients may be able to accept that their wor-
fan’, and ‘She’s a compulsive liar’. We use these phrases ries are groundless but nonetheless are unable to stop dwell-
when people do things again and again, and others can- ing and acting on them. Such beliefs are more often than
not see any reason for it. The essential features of this not over-valued ideas. However, this belief can be of a delu-
disorder are recurrent obsessional thoughts and compul- sional intensity when the patient should be treated as for
sive acts. Obsessions are patient’s own thoughts which are a delusional disorder. Otherwise antidepressants, behav-
repetitive and intrusive. These thoughts are almost invari- ioral therapy and cognitive behavioral therapy (CBT) are
ably distressing to the patient, because they are violent or the mainstay of the treatment for hypochondriasis.
559
Body dysmorphic disorder (Marbach, 1996). Though this urgency is almost palpable,
such urgent situations create the potential for failure. It is
Body dysmorphic disorder (BDD) is characterized by
thus essential to be aware of the three putative neuro-
‘devastating’ preoccupation with an imagined defect in
pathologic facial pain disorders:
appearance and marked distress over the supposed defor-
mity (American Psychiatric Association, 1994). Though 1. Phantom tooth pain
the focus of patients with BDD is by definition on physical 2. Intraoral stump pain
appearance, data exists on patients with BDD having obses- 3. Phantom bite syndrome.
sional concerns with odor (Hollander and Aronowitz, 1999).
1. Phantom tooth pain Reports on tooth pain of obscure
BDD is also known as dysmorphophobia. It is an intrigu-
origin are relatively recent (Harris, 1974). The term phan-
ing and sometimes difficult to treat condition that often
tom tooth pain was first used in 1978 (Marbach, 1978), and
mystifies the clinicians. This disorder is characterized by
since then the condition has been validated extensively as
distressing and impairing preoccupation with a non-existent
a clinical entity (Marbach, 1993a, 1993b; Pollmann, 1984;
or slight defect in appearance. Although BDD symptoms
Rees and Harris, 1979). Phantom tooth pain is the most
may sound trivial the disorder can cause severe distress
common type of orofacial phantom pain reported. It usually
and can lead to suicide. Individuals with BDD are preoc-
follows dental or surgical procedures such as pulp extirpa-
cupied with the idea that some aspects of their appearance
tion or tooth extraction. It is characterized by persistent
is unattractive, deformed or ‘just not right’ in some way.
toothache with no identifiable cause. Neither repeated end-
Concerns usually focus on their face or head but can involve
odontic treatment nor tooth extraction renders the affected
any area of the body (Phillips and Diaz, 1997; Phillips et al,
area free of pain. On the contrary, procedures and other
1993). Concerns with bodily asymmetry, for example,
surgical interventions such as trigeminal rhizotomy and
‘uneven’ buttocks are also common. BDD by proxy consists
microvascular decompression frequently exacerbate pain
of a preoccupation with supposed flaws in another person’s
and may even enlarge its distribution. Other terms for
appearance, which may lead to insistence that the other
phantom tooth pain, such as atypical odontalgia (Brooke
person should have surgery or dermatological treatment.
and Merskey, 1994), idiopathic odontalgia and atypical
The majority of BDD patients seek often costly non-
facial pain, also are in use (Bates and Stewart, 1991).
psychiatric treatments (Phillips, 1996) and may also present
The pain is described as a constant, dull, deep ache with
to the dental practitioners in the first instance for surgical
occasional spontaneous sharp pains. There are no refractory
treatment. However, most BDD patients appear dissatisfied
periods. Peripheral stimuli can momentarily exacerbate
with such treatment and many dislike their new appear-
the pain but have no prolonged influence. Radiographic
ance even more (Andreasen and Bardach, 1977). Multiple
and laboratory tests are negative. Sleep is undisturbed by
procedures may be received in search for a cosmetic solu-
pain or other phantom sensations. Many patients report a
tion to this psychiatric problem before it comes to a psy-
brief symptom-free period on awakening in the morning.
chiatrist. Occasionally, dissatisfied patients sue, or even
It resembles other phantom pain syndromes that commonly
become violent toward the physician. There are rare reports
arise following amputation and injury (Marbach, 1996).
of patients trying to perform their own surgery, as did one
Phantom tooth pain has only been reported in adults and
man who cut his nose open and tried to replace his own
none in children (Marbach, 1996).
cartilage with chicken cartilage. The mainstay of evidence
based treatments for BDD includes selective serotonin 2. Intraoral stump pain Stump pain is a frequent squeal
reuptake inhibitors (SSRI) and CBT. of limb amputations. Davis cited this pain as a major cause
of prosthetic limb rejection among amputees (Davis, 1993).
Sherman and colleagues suggested that denture pain is the
INTERESTING INTERFACE BETWEEN intraoral equivalent of limb stump pain (Sherman, 1989).
‘DENTISTRY’ AND ‘PSYCHIATRY’ Stump pain does not disappear with time or with adjustments
or replacements of the prostheses (Marbach, 1996). The
health and financial conditions of these patients improve
Disorders on the Psychotic Spectrum
on treatment directed toward stump pain which is focused
Orofacial phantom pain away from mechanically based etiologies (Marbach, 1985).
The onset of pain is usually associated with an injury to
Current day dentists are often faced with challenges when
a peripheral nerve. Pain is often worse at the site of the
patients present with orofacial pain. In the current day
original trauma, although in chronic cases they have dif-
competitive practice there is always an urgency to treat con-
ficulty in localizing the pain.
ditions associated with the pain. More often than not the
clinical dilemma needs to be resolved quickly and decisively. 3. Phantom bite syndrome Phantom bite syndrome is
Such situations impel the clinician to perform procedures often associated with an inability to adapt to changes
designed to eliminate pain such as endodontic therapy or in dental occlusion (Marbach, 1978, 1985). Non-painful
tooth extractions or to construct new and different dentures phantom limb phenomena are common among recent
560
amputees but usually fade with time (Jensen et al, 1984). those seen as a consequence of other disorders such as
Recently orofacial phantom bite syndrome has been viewed schizophrenia, depression or temporal lobe epilepsy (Pryse,
as a psychiatric disorder (Bonica, 1991; Jensen et al, 1984; 1971). It is still controversial whether ORS is truly a unique
Marbach, 1978, 1985). This interpretation has been revised disorder or merely a part of the symptomatology of other
in light of current research (Jensen et al, 1984; Melzack, psychiatric conditions (Lochner and Stein, 2003). However,
1993; Sherman, 1989). Though phantom bite syndrome it has been noted that most patients with primary ORS are
can occur at any stage of dental treatment, it is typically young without concurrent psychiatric disorders (Pryse,
associated with the construction of extensive prosthesis in 1971). Arguably, the principal symptomatology of ORS has
all age groups and the beginning of orthodontic treatment sufficient overlap with anxiety and somatic disorders.
in adolescents (Marbach, 1996). They usually complain of
continuous discomfort and are frequently distressed by Case vignette
the lack of familiarity of their own bite (Marbach, 1996). A 22-year-old male presented with the belief that he had malodorous
Seeking relief from phantom bite syndrome often becomes breath (halitosis) and a foul odor emanating from his armpit, feet,
an expensive and lengthy effort toward restoration of one’s and anal region. This persistent preoccupation had begun in early
original but ‘lost’ bite (Marbach, 1978, 1985). Success is adolescence, but the intensity had increased significantly over the past
rarely if ever obtained. Treatment therefore should be focused 7 months. Halitosis was his main concern. But collateral information
on prevention, early detection and patient education. from his parents confirmed his excessive washing and frequent
change of clothes. His embarrassment about the perceived halitosis
gradually caused increasing social withdrawal and isolation and also
Delusional bad odor
resulted in depressive symptoms. He remained convinced that the
Bad breath is also known halitosis; it is a common concern halitosis had persisted, despite reassurance to the contrary by his
found in millions of people. There seems to be no reliable physician and close family members.
way to assess breath odor. Some people develop faulty
perceptions about having a bad breath. Sometimes it Schizophrenia—dental implications
affects their entire life. On the other hand, some people
Dentists who are familiar with the signs and symptoms of
who have halitosis remain unaware of their condition.
schizophrenia are likely to feel more secure while treating
Every patient has a breath odor self-image. This self-image
patients with schizophrenia. It makes the dentist more con-
ranges from little or no distortion to severe psychopathol-
fident while obtaining consultative advice from the patient’s
ogy (Eli et al, 2001). Because these people go to a dentist
psychiatrist. Dentists can provide the full range of service
with a complaint of oral malodor, it becomes the respon-
to such patients and constitute to the psychotherapeutic
sibility of dental practitioners to identify this psychopa-
aspect of management. In addition to being able to commu-
thology. It is important for dentists to consider both
nicate effectively with such patients, the dental treatment
psychological and physiological factors while diagnosing
may need to be modified because of the patient’s impaired
and treating such cases.
ability to think logically. It is very important for all dentists
Various researchers have attempted to understand the dis-
to be aware of the local and systemic effects of psychiatric
tortions in self-perception of odors, including oral malodor,
medications and the adverse interactions between these
in the context of various psychiatric disorders (Davidson
drugs and medications used in dentistry.
and Mukherjee, 1982; Pryse-Phillips, 1971). One relevant
Schizophrenia can be conceptualized as a group of dis-
example is patients who complain of various body odors
orders, with variable presentations, best described in three
(auxiliary, fecal or genital) that appear to have no objective
different dimensions: positive symptoms, disorganized symp-
basis. These patients may have somatic delusions or an
toms and negative symptoms. The positive symptoms are
olfactory reference syndrome (ORS) (Pryse-Phillips, 1971).
the exaggeration or distortion of normal functions, most
Causes of ‘delusional halitosis’ were presented in the litera-
commonly delusions and hallucinations which are described
ture by Davidson and Mukherjee (1982), Iwu and Akpata
earlier in the chapter. ‘Disorganized’ symptoms are inferred
(1990) and Oxtoby and Field (1994). Halitophobia also
from patient’s speech and are also known as ‘thought dis-
may be considered in the context of BDD. Halitophobics
order’. It is manifested as rapid shifts between topics that
often display other psychological phenomenon, such as
either have a loose logical association or are completely
compulsive toothbrushing and withdrawal from social
unrelated. They may also exhibit inappropriate affect man-
interactions (Rosenberg, 1996).
ifested by childish silliness and unpredictable agitation.
(Andreasen et al, 1995; Marder, 1996). They also exhibit a
Olfactory reference syndrome
range of neurocognitive impairments in the areas of mem-
Patients sometimes present with persistent olfactory con- ory, attention and executive functions, which in turn impairs
cerns or preoccupations with personal odor (Malasi et al, their vocational and social adjustment (Green, 1996).
1990; Pryse, 1971; Stein et al, 1998; Videbech, 1966). The Negative symptoms are less dramatic but equally debilitat-
term ‘olfactory reference syndrome’ (ORS) has been intro- ing. They include reduced emotional responsiveness (flat
duced to differentiate primary olfactory concerns from affect); reduced speech output and poor thought content;
561
inability to plan, initiate and persist in goal directed activ- Pseudoparkinsonian side effects generally develop in 1–3 months
ities (avolition) and poor self-care in terms of washing, after therapy:
bathing and cooking. They also show social withdrawal • Reduced eye blinking
and loss of pleasure in previously enjoyed activities (anhe- • Monotonous and soft speech
donia) (Kaplan, 1994 and Sadock, 1995). • Mask-like face
Substance abuse frequently accompanies schizophre- • Cogwheel rigidity
nia. The prevalence rate of alcohol abuse in schizophrenia Tardive dyskinesia is slow to develop and is generally seen in
is as high as 63% in some studies (Dixon, 1999). Other 7–10 years of antipsychotic use. It is characterized by rhythmical
commonly abused drugs are cannabis and cocaine. Some involuntary movements of:
patients seem to self-medicate their positive symptoms • Mouth (ex, puckering)
with these illicit drugs which in turn makes the psychosis • Face (ex, puffing of cheeks)
worse. Abuse of these substances strongly correlates with • Jaw (ex, chewing movements)
medical and psychosocial problems like inadequate diet, • Tongue (ex, protrusion)
homelessness, poor compliance with medications and
multiple episodes of the disorder (Dixon et al, 1999). More Clozapine is another medication to be aware about. It is an
than three-fourths of patients with schizophrenia smoke atypical antipsychotic used in patients unresponsive to other
and this is frequently associated with emphysema, lung medications (Breier, Malhotra, Su, et al, 1999). Clozapine
cancer, cardiac disease and oral cancer (McEvoy and has a unique added advantage of reducing the cravings for
Brown, 1999). alcohol and illicit drugs (Volavka, 1999). About 0.5 to 1%
Patients with schizophrenia are often having a disinter- of patients on clozapine develop bone marrow suppression
est in performing appropriate preventive oral hygiene causing agranulocytosis and granulopenia (Worrel, Marken,
techniques (Friedlander and Liberman, 1991; Thomas et al, Beckman, et al, 2000). This prevents clozapine from being
1996). This in turn leads to the development of advanced used as a first-line drug. Thus all patients on clozapine
oral disease. This is compounded by reduced access to should have periodic WBC count (Hammad, 1998). Although
care, prolonged hospitalizations, impaired finances and clozapine is a potent anticholinergic agent, it has a unique,
paucity of clinicians who are comfortable in caring such unexpected side effect of sialorrhea in 85% of patients
people. Some antipsychotics, namely, chlorpromazine and (Clark, 1992).
thioridazine cause profound hyposalivation through their
anticholinergic effects (Sreebny, 1989). This is worsened
by the co-administration of antiparkinsonian agents to Anxiety and Depressive Disorders
counteract the extrapyramidal side effects (EPSE) of the
conventional (dopamine agonist) antipsychotics. The resul- Depression and dental implications
tant hyposalivation causes intensification of periodontal A depressive disorder may be associated with extensive
disease and rapid caries progression (Gupta et al, 1993). dental disease (Baker and Dawe, 2005). Patients may seek
Therefore, a lot of people with schizophrenia have a higher dental treatment before becoming aware of their psychiat-
requirement for periodontal treatment, dental restorations ric illness (Gatchel et al, 1996). Depression is often associ-
and dental extractions (Velasco et al, 1997). The conven- ated with a disinterest in performing appropriate and
tional antipsychotics have adverse side effects, especially timely oral hygiene techniques. It can also be associated
the movement disorders that mimic neurological diseases. with a cariogenic diet, diminished salivary flow, rampant
These movement disorders frequently have an orofacial dental caries, advances periodontal disease and oral dys-
component and are seen to develop in a time-dependent esthesias (Friedlander and Mahler, 2001). Side effects of
fashion. These movement disorders are caused by the block- antidepressant medications increase the incidence of den-
ade of basal ganglia dopamine D2 receptors in the extra- tal diseases including xerostomia (Gerber and Lynd, 1998).
pyramidal system (Holloman and Marder, 1997). An appropriate dental management in such patients would
include a good dental education, the use of saliva substi-
Effects of antipsychotic drugs on orofacial movements
tutes and anti-caries agents containing fluoride. Special
Acute diagnoses which generally manifest within the first 5 days of precautions should be taken and every potential drug inter-
starting treatment: action should be kept in mind while prescribing analgesics
• Oculogyric crisis (eye turned upward) and local anesthetics (Callahan, 1996).
• Torticollis (head turned sideways)
• Retrocollis (head turned backward)
Depression in chronic facial pain
• Trismus (forced closing of the mandible)
• Laryngospasm Depressive illness is a common and a serious disorder that
• Spasm of neck muscles affects at least 20% of women and 10% of men during their
• Tongue protrusion lifetime (Kessler et al, 1994). Approximately 15% of these
562
people eventually commit suicide (Angst and Hochstrasser, changes in salivary viscosity (Astor et al, 1999). Xerostomia
1994; Coppen, 1994). Depression has a high comorbity (Peeters et al, 1998), orthostatic hypotension (Peeters et al,
with chronic facial pain, making it important for dental 1998) and cardiotoxicity (Roose et al, 1998) are significant
practitioners to be able to identify depressive symptoms in adverse effects of certain antidepressant medications espe-
dental patients presenting with facial pain. The rate of cially when they are taken in combination with other med-
depression has been shown to be as high as 40–80% in ications (Fox, 1998). Chronic xerostomia can cause oral
patients with chronic facial pain (Gallagher et al, 1991; mucosal changes, increased coronal and root caries suscep-
Korszun et al, 1996). More than 40% of patients with tibility, candidiasis, partial loss of taste acuity, periodontal
chronic facial pain are refractory to treatment (Friedlander disease, and difficulty in swallowing and functional pros-
and Mahler, 2001). Thus early identification and optimal thetic problems (Astor et al, 1999). In patients aged 60 years
treatment of co-morbid depressive disorder will have a sig- or older, Thomson and colleagues (1995) reported a higher
nificant impact on the treatment outcome for these patients. root caries index value for those on antidepressant therapy.
Blumer and Heilbronn (1982) suggested that patients Precautions related to orthostatic hypotension include
with chronic pain have an underlying depression that is shorter dental interventions, positioning the patient up
masked and represented only by somatic symptoms that right in the dental chair, avoiding sudden postural changes,
are not accompanied by the usual mood symptoms, making using caution in prescribing medications which cause
it more difficult for the dental practitioners to recognize orthostatic hypotension.
depression. On the other hand, some authors have concep- The injection of local anesthetics containing vasocon-
tualized depression as a secondary maladaptive response strictors often causes an increase in blood pressure. A rare
to chronic pain (Dworkin, 1991). paradoxical hypertensive reaction may occur when patients
As a dental practitioner the most important factor is hav- taking drugs with alpha-1 adrenergic blocking activity
ing awareness of depression as a medical disorder and hav- (such as tricyclic antidepressant) are given with a local anes-
ing an openness to recognize the less obvious presentations thetic containing vasoconstrictor (such as epinephrine).
of a depressive disorder. If clinicians themselves conceptu- This reaction occurs as epinephrine is unable to bind to
alize depression as a mental disorder or a character defect the blocked alpha-1 receptors, instead interacts with avail-
and do not understand the neurological basis and treatability able beta-2 receptors causing vasodilatation and a resul-
of depressive conditions, they will not only fail to recognize tant paradoxical hypotensive reaction (Keene et al, 2003).
this serious condition but will also convey this prejudice Dental precautions include using a minimal quantity of local
to their patients. Patients may also be resistant to even con- anesthetic with sympathomimetic vasoconstrictor and tak-
sidering a diagnosis of depression particularly when they are ing care to prevent intravascular injections. Use of epineph-
consulting a dentist for what they perceive to be a physical rine containing homeostatic agents is contraindicated. All
condition such as jaw pain. Because of the time constrains these precautions are only in addition to routine monitor-
in a busy dental practice it might be worth while administering of blood pressure and other vital signs (Yagiela, 1999).
ing a depression rating scale such as the Beck Depression
Inventory (Beck et al, 1961) for all patients presenting with
Eating disorders—dental implications
chronic facial pain. Recognizing depressive symptoms in
patients with chronic pain is particularly challenging as Dentists are likely to encounter patients who have eating
many symptoms secondary to chronic pain are also prime disorders and they have an important part to play in the
symptoms of depressive illness. For example, patients with overall care of these patients. There are two major catego-
chronic pain have a resulting poor sleep, lethargy, irritability ries of eating disorders namely anorexia nervosa and buli-
and weight loss due to pain while eating, which are the mia nervosa. Anorexia occurs in upper and middle class
core symptoms of depression. It is also important to screen families while bulimia presents across all social classes
for co-morbid alcohol and substance abuse in such patients. (Hugo and Lacey, 1996).
A broad multidisciplinary approach to the diagnosis and Anorexia is aversion to food, which can be conceptual-
treatment will improve treatment outcomes of such patients. ized as resulting from a complex interaction between bio-
logical, individual and family factors. This aversion to food
leads to severe weight loss and its complications, both
Antidepressant use—importance for dental practice
physiological and psychological. There are two subtypes of
Many dental patients are prescribed antidepressants for anorexia, ‘restricting’ and ‘binge/purge’ types. The differ-
diverse therapeutic reasons such as pain control, insom- ence between these two sub-categories is based on whether
nia, smoking cessation, substance abuse and eating disor- the person regularly engages in binge eating or self-induced
ders. However, antidepressants taken with other drugs may vomiting (SIV), excessive exercise or misuse of laxatives,
increase the risk of complications that require special den- diuretics or enemas. Bulimia is even more common and is
tal precautions and care. Patients receiving antidepressant characterized by overeating followed by inappropriate com-
therapy commonly complain of decreased salivation and pensatory behaviors with normal body weight.
563
Patients with bulimia nervosa often present with bilat- assist in making the initial diagnosis and can influence
eral and occasional unilateral parotid gland swelling. The progress of the medical and psychological management of
incidence of parotid gland swelling is 10–15% in people the disorder.
with bulimia (Brady, 1985). The submandibular salivary
gland is involved infrequently. The exact pathogenesis of
these glandular enlargements has not been determined. It MANAGEMENT OF PSYCHIATRIC
is generally accepted that multiple emetic episodes cause DISORDERS
an autonomic neuropathy (Ascoli et al, 1993). With sym-
pathetic nerve impairment, individual acinar cells enlarge
and lead to clinically visible gland swelling (Ascoli et al, Broad Principles
1993). Such an asymptomatic bilateral parotid enlargement Some patients who receive psychiatric treatment for men-
often presents a diagnostic dilemma to the dentists. As these tal health problems may be reluctant to admit it. This is
patients with bulimia nervosa and parotid gland swelling most often because of the perceived stigma associated with
are usually secretive about their self induced vomiting (SIV) mental illness. It is important and could be quite tricky for
(purging), the diagnosis will have to be made by conduct- a dentist to overcome such barriers and obtain the neces-
ing a thorough clinical examination and serum electrolyte sary information. It always helps to take a supportive and
study. Early recognition and prompt diagnosis when such non-judgmental attitude, and advise patients that such
patients present to the dentists can avoid the later serious information will be held confidential and also that it is
medical complications. The need for these patients to seek indispensable to provide a safe dental care.
psychiatric care and discontinue SIV is mandatory. Patients with mental health problems may be uncoop-
Eating disorders have various orodental adverse effects. erative and irritable during dental treatment. They may
Holst and Lange in 1939 coined the term ‘perimylolysis’ to appear unappreciative and may seem to have numerous
describe the distribution of erosion on the upper palatal complaints that are inconsistent with the objective findings
surfaces secondary to vomiting, reflux and regurgitation (Korszun and Ship, 1997). It is always beneficial to liaise
(Holst and Lange, 1939). Several research studies till date with the patient’s psychiatrist before beginning any dental
have shown that SIV results in increased frequency of treatment. Information requested should include at least
erosion on palatal surfaces (Hellstrom, 1977). Whether the the latest mental state, risk assessment and the list of psy-
caries experience in eating disorder individuals is greater chotropic medications. In addition, a history of alcohol
than in normal population remains unclear (Hurst et al, and illicit drug use is always useful. Such patients should
1977). Salivary flow increases dramatically prior to vomit- undergo a liver function test, full blood count and coagu-
ing because the medullary center that controls vomiting is lation profile before commencing the dental treatment.
connected to salivary nuclei (Edgar, 1992). With respect to
SIV, the stimulated salivary flow should therefore be altered.
Research has discovered reduced bicarbonate in bulimics Importance of Interpersonal Communication Skills
along with increased salivary viscosity (Edgar, 1992). One with Special Emphasis on Dental Phobia
study has found increased frequency of periodontal dis-
Communication skills—why bother?
ease in patients with eating disorders (Touyz et al, 1993).
Angular cheilitis, candidosis, glossitis and oral mucosal From personal experience of one of the authors, we state
ulceration are possible sequelae of nutritional deficiency. the obvious that dentists should remember that the patient
There has not been any report of malignant change associ- cannot speak with a wide open dry mouth and with dental
ated with SIV (Brady, 1980). instruments in their mouth. They should probably ask ques-
The dental care demanded by individuals with eating tions that generate a yes/no type of response. Moreover,
disorders is very challenging. Although the dentist might at the outset, it is useful to lay down the ground rules such
suspect vomiting as the cause of erosion, these patients will as, ‘if in pain, raise your left hand’.
not readily admit to such behavior because they can be Patients seeking dental treatment have apprehensions
highly secretive and embarrassed by it. The patient’s moti- about the nature of their problem and the procedure
vation to reduce the frequency of SIV will increase once involved. One of the common misconceptions that people
the dentist is able to openly relate the progress of dental have is that all dental procedures are painful. Moreover,
erosion with the vomiting. Toothbrushing after vomiting the fear of injections and the sound and sight of drills and
is generally regarded as inadvisable because the softened, other instruments keep many patients away. This may lead
demineralized surface is more susceptible to toothbrush to patients postponing visits to their dentists or totally avoid-
abrasion (Milosevic et al, 1997; Robb et al, 1995). ing it. The estimates of dental anxiety in general population
Patients whose teeth have been damaged as a conse- varies between 6% and 20% (Rouse and Hamilton, 1990).
quence of an eating disorder are most likely to present first Dentist–patient relationship rests a lot on the interper-
to the dentists. In many cases the dentist is in a position to sonal communication. This also has a significant impact in
564
allaying dental anxiety. Corah et al (1985) have reported more likely to report pain (Maggirias and Locker, 2002).
that anxiety during treatment was influenced by patient’s This again brings us to the fact that explaining the
satisfaction with the dentist’s technical competence, under- procedure as highlighted in the previous paragraph, gives
standing and communication skills (Corah et al, 1985). patients a sense of control and is useful in allaying their
It needs to be stressed that every doctor has to approach anxiety.
his/her patients with warmth and have a basic respect for Study by Eli et al suggests that the level of patient’s
them and acknowledge the distress that the person seeking anxiety was affected by evaluation of the present dentist by
help may be going through. the patient and memories of anxiety from childhood and
also by the personality traits of the patient (Eli et al, 1997).
Importance of explaining the procedure to the Some training centers have communication skills train-
patients ing programs for students in dentistry to teach students
micro-skills such as dealing with anxious patients. It has
Dentists often have to deal with anxious clients. Helping
been shown that communication skills training has an effect
patients overcome such anxiety can reduce the rates of
on the knowledge and a substantial effect on the behavior
missed appointments and also better compliance on the
of the students (van der Molen et al, 2004).
part of the patient during the treatment session. Various
ways of doing it can be employed such as using models,
Note:
charts or diagrams and video clippings. It is important to
check that the patient understands at every step. The very ❍ Clear communication involves making simple and
step of explaining the procedure helps patients to be men- short statements asking only one question at a time.
tally prepared and allays their anxiety. It is also helpful to ❍ Active/attentive listening is a key feature of good
obtain a verbal consent from the patient ensuring that they communication. It involves making a good eye con-
are ready for the procedure. tact and also by using non-verbal modes of com-
In addition, creating a conducive environment for the munication (head nodding).
patient also helps. A survey by Bare and Dundes has ❍ Avoid interrupting when the other person is talking.
shown that a majority of anxious patients found it helpful ❍ Ask questions to clarify what the person actually
to have gentle music in the background, magazines and meant when unclear.
books, adorned walls and a slightly cool temperature in the ❍ It is important to confirm that the person has under-
waiting area (Bare and Dundes, 2004). stood what the doctor had to say. ‘Have you under-
stood/Am I clear?’, ‘Do you have any questions?’
Dealing with dental anxiety/phobia ❍ Reassure, explain that the procedure can be stopped
at any time if the patient cannot tolerate it.
Bare and Dundes have summarized from past research the
reported causes of dental anxiety (Bare and Dundes, 2004).
Some of the common ones are: Medications
❍ If patients have had previous painful experiences Antidepressants
❍ Belief that painful treatment is inevitable
Antidepressants are drugs that relieve symptoms of depres-
❍ If they feel that they lack control over the situation,
sion. They were first developed in 1950s and have been used
including the inability to stop a procedure they find
regularly since then. There are almost 30 different kinds of
unpleasant
antidepressants which belong to the four major categories:
❍ Lack of understanding of the procedure that the dentist
tricyclics, mono amine oxidase inhibitors (MAOIs), SSRIs
performs
and serotonin noradrenaline reuptake inhibitors (SNRIs).
❍ Patients having a general fear of the unknown
Antidepressants work by increasing the activity of certain
❍ Media coverage or hearing from acquaintances of
neurotransmitters mainly serotonin and noradrenaline.
frightening tales of dentists or procedures
Antidepressants are used to treat moderate to severe depres-
❍ Experienced detached treatment by a dentist
sive illness, severe anxiety and panic attacks, OCD, chronic
❍ Having fears of experiencing ridicule because of how
pain, eating disorders and PTSD.
they react to situations arising during their visit.
The tricyclic antidepressants such as imipramine, ami-
It is important to provide information to the patients as triptyline, nortryptiline and dosulepin cause side effects
soon as possible about the nature of the problem and what such as dry mouth, slight tremor, tachycardia, constipation,
they can expect during and after the treatment. It is known sleepiness and weight gain. In addition to these anticho-
as ‘informational control’ and it reduces dental anxiety linergic side effects, men may experience delayed ejaculation
(Levitt et al, 2000). and difficulty in getting or keeping an erection. Tricyclic
Maggirias and Locker reported that patients who per- antidepressants are less commonly used as they are dan-
ceive that they have little control over the procedure were gerous in overdose.
565
SSRIs are the commonly used first line treatment and antipsychotics include risperidone, olanzapine, quetiap-
include drugs such as fluoxetine, sertraline, citalopram ine, amisulpiride and clozapine. Risperidone is the only
and paroxetine. SSRIs are generally well tolerated and less atypical antipsychotic which is available in the form of
harmful in an overdose which make them the most popu- long acting injection.
lar. SNRIs are very similar to SSRIs although venlafaxine Clozapine is an atypical antipsychotic medication and
should not be used in patients with heart problem and the only one that has shown to be more effective for peo-
needs monitoring of blood pressure. MAOIs are rarely pre- ple who do not respond to other sorts of antipsychotics.
scribed these days as they cause dangerously high blood In addition to the side effects of the atypicals mentioned
pressure on eating food containing ‘tyramine’ known as above, clozapine also produces increased salivation. The
‘tyramine reaction’. It is important to check for the danger- main drawback is that it can affect the bone marrow reduc-
ous drug interactions before prescribing if a patient is on ing the white cell count causing agranulocytosis which
MAOIs. It is generally best to taper off the dose of an anti- can be fatal. For this reason, patients taking clozapine need
depressant as stopping it suddenly may cause withdrawal weekly full-blood count for the first 6 months, 2 weekly for
such as stomach upset, anxiety, dizziness and flu-like the next 6 months, and 4 weekly thereafter.
symptoms. The current recommendation is that antide- Most patients with schizophrenia need to take antipsy-
pressant should be continued for at least 6 months after chotics for a long time. On stopping the treatment, symptoms
complete remission of symptoms. If one has suffered from of schizophrenia usually come back, if not immediately,
two or more episodes of depression then treatment should often within 6 months. It is advisable to reduce the dose of
be continued for at least 2 years. the medication gradually, only in discussion with a psy-
chiatrist who will monitor for early signs of relapse.
Antipsychotics
Anti-manic agents
Starting from the mid 1950s several medications were
discovered that reduce the symptoms of schizophrenia Medications used to treat mania include mood stabilizers
and other psychotic disorders. They came to be known as such as lithium and valproate. Other medications used to
‘antipsychotic’ medications. These older drugs are called treat mania include antipsychotics and benzodiazepines.
‘typical’ or ‘first generation’ antipsychotics. They work by Medications used to prevent the relapse of manic episodes
reducing the action of dopamine in the brain (dopamine in manic depression (bipolar disorder), include drugs such
antagonist). Some typical antipsychotics are chlorproma- as lithium, valproate, carbamazepine and lamotrigine.
zine, haloperidol, pimozide, trifluoperazine and sulpiride. Lithium has over the last 40 years been the most com-
Some of these typical antipsychotics are available in long monly used drug to prevent relapse. Lithium is a safe drug
acting depot injection form which can be administered when taken at the correct dose, however it has a narrow
once in 2 to 4 weeks for people who do not comply with therapeutic window (0.6 to 0.8 mmol/l) and becomes unsafe
oral medications. The typical antipsychotics have more above this level in the blood. The common side effects
side effects than atypicals. The side effects include stiff- include fine tremors, metallic taste in the mouth, tiredness,
ness and shakiness as in Parkinson’s disease along with weight gain and underactive thyroid gland. Long-term treat-
feeling sluggish and slow in their thinking. Other side ment with lithium can cause renal impairment. It is thus
effects include uncomfortable restlessness (akathisia) and important to periodically check for serum lithium level,
sexual side effects. A long-term side effect is ‘tardive thyroid functions and renal functions including creatinine
dyskinesia’—persistent movements generally of the mouth clearance. Lithium is an ion and is excreted unchanged from
and tongue which is difficult to treat and very disabling. the kidneys. Drugs such as diuretics and NSAIDs can dan-
Over the last 10 years, several newer medications have gerously increase the serum lithium levels and so it is very
been in use. They work on a different range of chemical important to check for drug interactions before prescribing
messengers in the brain including the serotonin system for a patient on lithium.
(serotonin dopamine antagonists). These came to be known Valproate and semi-sodium valproate are becoming
as ‘atypical’ or ‘second generation antipsychotics’. These widely used treatment for mania and bipolar disorder. The
atypical antipsychotics are less likely to cause parkinso- common side effects of valproate include sleepiness, diz-
nian side effects although they may cause weight gain ziness, increased appetite and weight gain, skin rashes and
and problems with sexual functions. They may also help irregular periods. Very rare side effects include pancreati-
the negative symptoms on which the older drugs have tis and liver failure. It is again very important to check for
very little effect. They also seem to have much less pro- drug interactions as valproate is a hepatic enzyme inhibi-
pensity to cause ‘tardive dyskinesia’. The common side tor and reduces the metabolism of other medications.
effects of atypicals include weight gain, sexual side effects, Carbamazepine is usually used as a second line treatment
glucose intolerance and increased chance of developing for bipolar disorder. Unlike valproate, carbamazepine is
Type 2 diabetes and sedation. The commonly used atypical a hepatic enzyme inducer and increases the metabolism
566
of other medications and thus reduces their efficacy. Inter- doing things together. Behavioral psychotherapy tries to
estingly it reduces its own level and needs bigger doses with change the behavioral patterns of the patients and helps
longer treatment. Lamotrigine also helps to prevent mood to overcome fears by spending more and more time in the
swings particularly of severe depressive episodes. It is how- situation they fear. They are also given homework exercises
ever not used as a monotherapy for bipolar disorder. and are asked to keep a diary to practise the new skills in
between the sessions. This type of behavioral psychother-
Electroconvulsive therapy apy is effective for panic, phobias, anxiety, OCD and vari-
ous kinds of social and sexual difficulties. Results are seen
Electroconvulsive therapy (ECT) is a treatment used in quite quickly. CBT aims at changing the thinking patterns.
psychiatry for severe mental illnesses. It was originally It emphasizes on how the thinking, behavior, emotions
developed in the 1930s and was used widely during the and physiological symptoms are all related in a particular
1950s and 1960s for a variety of conditions. Since then its instance and is influenced by the environment. It encour-
use was declined. ECT remains a controversial treatment, ages a discussion on how we think and helps us to get rid
which some people have strong feelings about. There are of unhelpful ways of thinking. It focuses on the present
those who claim it can be a lifesaving procedure, while and not on the past. CBT have achieved particular success
others feel it should be banned. ECT is a way of causing in the treatment of certain types of depression. Psychody-
someone to have a seizure and it is this seizure that is namic psychotherapy focuses on the feelings one has about
needed for the treatment to work. The seizure is made to other people, family and those close to the individual. This
happen by passing an electric current across the person’s mode of treatment involves discussing the past experiences
brain in a carefully controlled way from a specially devel- and how these may have led to the present situation and
oped ECT device. The current can be administered to the affecting the individual’s life. It may involve a brief therapy
whole brain when it is called ‘bilateral ECT’ or just to the for a specific difficulty or may be long-standing with daily
non-dominant hemisphere called the ‘right unilateral ECT’. sessions lasting for many years. Family therapy focuses
The seizure itself is very similar to the seizures that occur very clearly on the relationships, boundaries and commu-
in people with generalized epilepsy, but it is caused on nication styles in the family of the concerned individual.
purpose in very controlled circumstances using general- Marital therapy is for relationship problems in a marriage,
ized anesthesia and muscle relaxant, just like for a surgical partnership or family. In all the above forms of therapy, the
operation. The aim of ECT is to cause a ‘generalized cere- therapist may be a psychiatrist, psychologist or the mental
bral seizure’ between 10 and 50 seconds long using the health professional who has had an in-depth training in
right dose of electricity. The current recommendation is to psychotherapy. These therapies are usually done under
use ECT for treatment resistant severe depression, severe supervision.
mania and catatonia (NICE, 2003). ECT is used more as a
lifesaving treatment for quick resolution of very severe When to refer to a psychiatrist
symptoms and is always supplemented with a continua-
tion of the most appropriate pharmacotherapy. As soon as you suspect a mental disorder and realize that
the patient is not seeing a psychiatrist, it is good to think
about referral. In general the earlier the mental illness is
Psychological (talking therapies)
detected the better the final outcome. Always try to reas-
There are several types of ‘talking treatments’ also known sure and encourage the patient to seek help for the mental
as ‘psychotherapy’. These are different ways of helping peo- health problems. Be non-judgmental and explain to the
ple to overcome stress, emotional problems, relationship patient that treatment is available, and it is possible to
problems and troublesome habits. The commonality in these improve the quality of life with help from the mental
treatments is talking to another person and sometimes health services.
567
CHAPTER
Bone Diseases and
19 Fibro-osseous Lesions
Manish Juneja, Ravikiran Ongole
➧ Fibro-Osseous Lesions ➧ Bone Diseases

Periapical Cemento-osseous Dysplasia Osteogenesis Imperfecta
Focal Cemento-osseous Dysplasia Osteopetrosis
Florid Cemento-osseous Dysplasia Cherubism
Gigantiform cementoma Infantile Cortical Hyperostosis
Ossifying Fibroma, Cementifying Fibroma and Idiopathic Osteosclerosis
Cemento-ossifying Fibroma Gorham’s Disease
Juvenile Ossifying Fibroma (Psammomatoid and Paget’s Disease of Bone
Trabecular)
Fibrous Dysplasia
FIBRO-OSSEOUS LESIONS most fibro-osseous jaw lesions can be assigned with rea-
sonable certainty into one of the several categories:
Fibro-osseous lesions of the jaw comprise a diverse group I. Fibrous dysplasia
of conditions which are characterized by replacement of II. Reactive (dysplastic) lesions arising in the tooth-bearing
normal bone by a tissue composed of collagen fibers and area
fibroblasts that contain varying amounts of mineralized A. Periapical cemento-osseous dysplasia
substance which may be bone, cementum or both in B. Focal cemento-osseous dysplasia
appearance. These lesions present a wide range of clinical C. Florid cemento-osseous dysplasia
and radiographic patterns. The precise diagnosis of fibro- III. Fibro-osseous neoplasms: Cementifying fibroma, ossi-
osseous lesions depends on good clinical, radiological and fying fibroma or cemento-ossifying fibroma
histological correlation.
Another classification which attracted many pathologists
Numerous terminologies have been used to designate
was given by Fowler. He classified fibro-osseous lesions as
these lesions. In 1940s and early 1950s, these lesions were
follows:
commonly termed as localized osteitis fibrosa, osteofibroma
or fibrous osteoma. The lesions in the skull were termed as I. Osseous dysplasia
leontiasis ossea because of its lion-like appearance. With A. Non-hereditary
advances in the understanding of these lesions a number 1. Periapical osseous dysplasia
of classifications have been proposed by many investigators. 2. Focal osseous dysplasia
Waldron (1985, 1993), Makek (1987), Slootweg (1996), 3. Florid osseous dysplasia
Fowler (2005) are a few among those who have been B. Hereditary
accepted, although no universally approved classification 1. Familial gigantiform cementoma
system has been presented. II. Fibro-osseous neoplasms
The classification system that has been considered to be A. Conventional ossifying fibroma
most useful was given by Waldron in 1993. Waldron sug- B. ‘Juvenile’, ‘active’, or ‘aggressive’ forms of ossify-
gested that with adequate clinical and radiographic data, ing fibroma
568
Chapter 19 – Bone Diseases and Fibro-osseous Lesions
III. Fibrous dysplasia present greater problems in diagnosis than do multiple

A. Monostotic fibrous dysplasia lesions, particularly when the solitary lesion is in the pre-
B. Polyostotic fibrous dysplasia molar area and the patient is male or a white female.
C. Polyostotic fibrous dysplasia with endocrinopathy
(McCune–Albright)
D. Craniofacial fibrous dysplasia. Focal Cemento-osseous Dysplasia
The term focal cemento-osseous dysplasia was first sug-
gested by Tomich and Summerlin in 1989. The disease is
Periapical Cemento-osseous Dysplasia
seen in the edentulous posterior areas of females who are
The term periapical cemento-osseous dysplasia is used to in their 4th and 5th decades of life.
designate a lesion which occurs in the periapical areas and
shows cementum-like and osseous areas on histopatho- Clinical features
logic examination. The term periapical cemental dysplasia Focal cemento-osseous lesions are invariably asymptomatic
which was used earlier to designate these lesions is some- and are discovered on routine radiographic examination.
what incorrect as the lesion shows both cementum-like There is no swelling associated with it unless the lesions
and osseous areas. The etiology is unknown, but they appear are old and have caused bony expansion. On surgical
to originate from the periodontal ligament. exploration the tissue occupying the defect is gritty, hem-
orrhagic and is removed by curettage in small fragments,
Clinical features often with some difficulty. This helps in differentiating the
The periapical cemento-osseous dysplasia is a disease which lesion from ossifying fibroma which is well circumscribed
involves the periapical areas of the vital teeth in black and avascular and easy to enucleate.
female patients who are older than 30 years of age. The
lesions are invariably asymptomatic and are discovered on Radiographic features
routine radiographic examination. These are well-demarcated radiolucent or mixed radiolucent/
radiopaque area or highly sclerotic. Most lesions are less than
Radiographic features 2 cm but larger lesions may be seen. Simple bone cysts also
may occur with focal cemento-osseous dysplasia.
The early lesions show well-defined, circumscribed radio-
lucent lesions involving the apices of one or several teeth. Histopathologic features
Individual lesions are seldom more than 1.0 cm in diameter
and most are less than 0.5 cm in size. The lesions show an Microscopically areas of cellular fibrous tissue containing
increasing degree of calcification with time, which may numerous small blood vessels, irregular trabeculae of
appear as mixed radiopaque and radiolucent lesions. The woven bone and/or cementum-like calcifications are seen.
older lesions show radiopaque masses. These lesions do Scattered foci of multinucleated giant cells may be seen.
not show any bone expansion.
Histopathologic features The lesion shows no tendency to recur after removal.
Not many reports appear that review the histopathologic Partial removal of the lesion is also advocated in cases
findings of periapical cemento-osseous dysplasia. The where the lesion is very large and limits the total excision.
lesions which are misdiagnosed as inflammatory periapical The partial removal does not show any tendency to enlarge
pathologies and are subjected to histopathologic findings or recur and the long-term prognosis seems to be excellent.
have shown to be composed of cementum-like and/or
osseous trabeculae in a fibroblastic stroma. As the lesions Florid Cemento-osseous Dysplasia
become older the calcified component appears to be more
prominent. The term florid cemento-osseous dysplasia (FCOD) was
first suggested by Melrose et al in 1976 to describe a con-
dition of exuberant multiquadrant masses of cementum
and/or bone in both jaws and in some cases, simple bone
The diagnosis is done mainly on the basis of radiographic cyst-like lesions in affected quadrant. Sometimes familial
presentation and clinical findings. Surgical intervention is tendencies have been observed. In past this condition has
contraindicated. Early lesions showing radiolucent areas been designated as sclerosing osteitis, multiple enostoses,
are often misdiagnosed as periapical cysts or granulomas. diffuse chronic osteomyelitis and gigantiform cementoma.
Accurate pulp testing should be done to avoid such errors. The etiology of florid cemento-osseous dysplasia is
Isolated lesions involving the apical areas of vital teeth unknown. Waldron et al have proposed that reactive or
569
dysplastic changes in the periodontal ligament might be a of massive sclerotic masses of disorganized mineralized
cause for the disease. material. Both sporadic and familial occurrence has been
reported. The etiopathogenetic mechanism of gigantiform
Clinical features cementoma is unknown.
The disease is seen exclusively in middle aged, black female The term gigantiform cementoma has been used syn-
and has a striking tendency for bilateral occurrence, often onymously with the terms sclerotic cemental masses, florid
presenting symmetrically in the jaws. The disease is lim- osseous dysplasia, multiple enostoses and diffuse sclerosing
ited to the tooth bearing areas of the jaws. Many patients osteomyelitis. It was Norberg in 1930 who first described
are partially or completely edentulous when the condi- gigantiform cementoma. In 1971, the World Health Organi-
tions are detected. Many cases are completely asymptom- zation (WHO) classified gigantiform cementoma in the cat-
atic and the disease is diagnosed during routine radiographic egory of cemental lesions.
examination. When the lesions are large, jaw expansion
Clinical features
may be noted and symptoms of dull pain or drainage are
always associated with exposure of the sclerotic calcified Gigantiform cementoma is an autosomal dominant disor-
mass to the oral cavity as a result of progressive alveolar der having high penetrance and variable expressivity. No
atrophy after a denture or after extraction. Laboratory sex predilection has been observed. It typically presents
investigations show no biochemical abnormalities. as a slow-growing, multifocal/multiquadrant and expans-
ile lesions involving both the jaws. The lesions are usually
Radiographic features seen in younger age, although older patients have also
Bilateral densely sclerotic lesions often symmetrically pres- been reported. The size of the lesions seen in older patients
ent in various areas of the jaws. These are usually mixed was comparable to those seen in younger individuals.
with less well-defined areas of a mixed radiolucent/radi- Thus it has been suggested that these lesions may have pla-
opaque pattern. Majority of the patients are edentulous at teaued in their growth, probably after cessation of skeletal
initial presentation. If the patient is having remaining growth. The characteristic clinical manifestation is of a
anterior teeth, they will often show circumscribed typical painless maxillary and mandibular swelling with associ-
lesions of periapical cemento-osseous dysplasia. Well-defined ated facial deformity. Tooth impaction, malpositioning of
radiolucency representing simple bone cyst is not uncom- teeth and malocclusion are also seen associated with the
monly seen with florid cemento-osseous dysplasia. swelling. The enlargement of the swelling finally stops
during the 5th decade.
Microscopically florid cemento-osseous dysplasia shows
Radiographically, gigantiform cementoma exhibits multiple
an admixture of woven bone trabeculae and droplets of
circumscribed, expansile and lobular mixed radiolucent-
cementum-like calcifications in a fibroblastic stroma. The
radiopaque lesions that usually cross the midlines of the
cementum-like calcifications often fuse to form coalescing
jaws. Sometimes large masses are seen in posterior areas
masses.
of the jaws and the lesions combining in the midline. This
suggests that lesions might have started as separate posterior
masses; however, with anterior progression they became
The diagnosis of the disease is mainly dependent on the confluent and crossed the midlines of the jaws.
radiographic and clinical presentation. However, the method
of treatment is not very satisfactory. In the asymptomatic Histopathologic features
patient, no treatment should be considered and patient Microscopically it presents a spectrum of histopathologic fea-
should be kept under observations. In symptomatic patients, tures, mainly characterized by variable degrees of cellularity
where exposure of the sclerotic masses to the oral cavity is and variation in the amount and size of mineralized deposits.
seen, biopsy or elective extraction of teeth in the involved The mineralized component is present as admixture of
area should be avoided. Antibiotic therapy should be insti- small and large psammomatoid and spherical hematox-
tuted. Sequestration of cementum-like masses will occur ylinophilic, cementum-like calcified deposits and irregular
slowly, followed by healing. Saucerization or surgical exci- bony trabeculae in a fibroblastic proliferative background.
sion of the sclerotic masses is not successful. The fibrous stroma may show fascicular or storiform pat-
terns with spindle-shaped or stellate-shaped fibroblasts.
The lesions are generally hypovascular, with occasionally
Gigantiform Cementoma
focally rich vascular areas exhibiting aggregates of thick-
Gigantiform cementoma is a rare, benign fibro-cemento- walled small blood vessels. Mitoses, hyperchromatism and
osseous disease of the jaws. It is characterized by formation pleomorphism are not seen.
570
Management and prognosis to the tooth-bearing areas of the jaws, although posterior
mandibular lesions may extend upward into the ascending
The surgical management of gigantiform cementoma is usu-
ramus. The lesion appears as hard, localized and slow grow-
ally difficult because of the extensive involvement of the
ing, painless mass that may displace adjacent structures
jaws with these tumors. Surgical inaccessibility, particularly
and cause root resorption. Exfoliation of teeth may also be
in the posterior regions of the jaws seems to be responsible
seen. Expansion is seen commonly in the inferior border
for recurrence of the tumors. These lesions seem to have a
of the mandible, followed by buccal plate expansion. On
tendency toward recurrence when they are treated with
surgical exploration, the tumor is found to be relatively
incomplete surgical removal. It is therefore recommended
hypovascular and well demarcated from the surrounding
that these lesions should be managed with conservative but
tissue, permitting relatively easy separation from the sur-
complete surgical excision whenever feasible.
rounding bone. This demarcation from the surrounding
structure is not seen in fibrous dysplasia and thus can be
Ossifying Fibroma, Cementifying Fibroma and used to distinguish the two.
Cemento-ossifying Fibroma
Ossifying fibroma is the most common fibro-osseous neo-
The lesion appears well circumscribed in contrast to fibrous
plasm of the jaw. It is a bone producing, slow growing,
dysplasia, the borders of which are ill-defined. Initial lesions
asymptomatic, well demarcated, benign lesion common in
are radiolucent representing an osteolytic image followed by
maxilla and mandible. The tumor is defined as a demarcated
gradual transformation into a mixed lesion and eventually
and occasionally capsulated lesion consisting of fibrous
becoming radiopaque. The periodontal ligament space of
tissue containing variable amounts of mineralized material
the involved teeth is clearly seen unlike fibrous dysplasia.
resembling bone and/or cementum or both. The term ossi-
Eversole et al have described two basic radiological pat-
fying fibroma is used if the predominant component is
terns: a unilocular radiolucency with or without radi-
bone, while cementifying fibroma is defined by the presence
opaque foci, and a multilocular radiolucency. The former
of cementum-like spherical calcifications. The lesions char-
presentation is found to be more common. Expansion of
acterized by the presence of bone and cementum are referred
the cortical plates is a common finding. Teeth displacement
to as cemento-ossifying fibroma.
and root resorption may be seen (Figure 1).
The 1972 WHO classification separated the cementifying
fibroma which was considered to represent odontogenic Histopathologic features
tumors, from ossifying fibroma, which was considered to
be osseous tumors. However, it has been agreed by all that Ossifying fibroma shows a range of histologic appear-
the two represent the same pathological condition with dif- ances. It shows fibrous and osseous tissues with the former
ferent histological presentation. Thus, in 1992, WHO clas-
sified the lesion as cemento-ossifying fibroma. The origin Figure 1
of the amorphous cementum-like calcifications commonly
seen in these lesions is still uncertain. Many of the fibro-
osseous lesions of the skull which were excised from the
regions far away from the jaws showed cementum-like cal-
cifications which make their cemental origin very unlikely.
It is also known that bone and cementum cannot be dis-
tinguished histologically. Thus, the nomenclature represents
only a variation in the histologic presentation. The prog-
nosis and course of the lesion remains the same.
Etiology
Ossifying fibroma occurring in the jaw seems to arise from
the periodontal membrane, which contains pluripotential
cells capable of forming cementum, bone and fibrous tissue.
Clinical features Lateral cephalograph in a patient suffering from ossifying

Ossifying fibroma shows a definite female predilection, fibroma reveals an extensive expansion of the cortical
with the mandible (premolar–molar area) involved more plate in the mandible. Multilocular radiolucency is evident.
than maxilla in most of the cases. These occur mostly in Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
the 3rd and 4th decades of life. The lesions are restricted
571
tissue predominating. The fibrous stroma is highly cellular, of osteoblastoma and termed it psammous desmo-osteo-
with spindle-shaped fibroblastic cells arranged in whorls. blastoma.
A fibrous capsule may be seen in some cases. The osseous Trabecular variant of juvenile ossifying fibroma was
tissue consists of rounded or lobulated basophilic masses described by Reed and Hagy, in 1965. Makek described the
(cementum-like), trabeculae of osteoid, woven or lamellar trabecular variant as trabecular desmo-osteoblastoma.
bone, or combination of the two. Osteoblastic rimming
around the trabeculae can be seen. Focal clusters of giant Etiology
cells (osteoclasts) can be seen to be arranged haphazardly
or adjacent to the mineralized material. Juvenile ossifying fibroma is considered to develop from
The histologic differentiation of ossifying fibroma undifferentiated cells of the periodontal ligament.
from osteogenic sarcoma may occasionally cause diffi-
culty for the pathologist. Ossifying fibroma shows osseous Clinical features
tissue which is relatively uniform and regularly arranged. The lesion characteristically occurs under the age of 15 years
On the other hand, in osteogenic sarcoma, the bone in 79% of cases. The psammomatoid variant occurs exclu-
spicules and osteoid usually demonstrate irregularity and sively in the extragnathic craniofacial region especially in
haphazard arrangement along with significant cellular the orbital bones and paranasal sinuses (61.6%), and less
anaplasia. commonly in the jaws, maxilla (19.7%) and mandible
(7%). Few cases have been reported to occur in calvarium,
Management parietal, temporal and frontal bones. In mandible, the tumor
The ossifying fibroma can usually be excised in one piece occurs more commonly in the ramus than in the body of
or removed in several large fragments. Prognosis is excel- the mandible. There seems to be no predilection for either
lent and recurrence after removal is seldom seen. There is sex. Patients often present with symptoms such as exoph-
no evidence of malignant transformation. thalmus, bulbar displacement and proptosis when affect-
ing the orbital bones and paranasal sinuses. In the jaws,
the affected region shows a painless expansive swelling of
Juvenile Ossifying Fibroma several months. Development of aneurysmal bone cyst in
(Psammomatoid and Trabecular) psammomatoid juvenile ossifying fibroma is commonly
reported. The cyst tends to occur more commonly in the
Juvenile ossifying fibroma is an uncommon lesion that younger patients in the 1st and 2nd decades of life.
affects the jaw of children under 15 years of age. Histo- The trabecular variant is characterized by a progressive
logically it is composed of cell-rich fibrous tissue contain- and sometimes rapid aggressive growth. The tumor expands
ing bands of cellular osteoid, trabeculae of bone and the affected bone, leading to facial asymmetry. It occurs
aggregates of giant cells. There are two histologic variants more commonly in maxilla as compared to mandible. Few
of juvenile ossifying fibroma: psammomatoid and trabec- cases have also been reported in sinonasal region. Slight
ular variants. The lesion is characterized by the early age male predominance has been observed with an average
of onset, the bone pattern, the high tendency to recurrence age range of 8.5–12 years. In maxilla, tumor may lead
and the aggressive local behavior. The lesion has been to nasal obstruction, epistaxis and eye displacement. The
variously described as juvenile ossifying fibroma, active duration of the lesion is usually a few months.
juvenile ossifying fibroma, aggressive ossifying fibroma,
reticular desmo-osteoblastoma or active fibrous dysplasia.
The term juvenile (aggressive) ossifying fibroma was
used in the second edition of the WHO classification of Radiographically, the psammomatoid variant shows well-
odontogenic tumors to describe a lesion affecting the jaws defined expansion of the affected bone having mixed radio-
of children under the age of 15 years. This term is used to lucent, radiodense and ground-glass appearance. The lesion
describe two distinct histopathologic variants of ossifying is partially or completely surrounded with thin, corrugated
fibroma of the craniofacial skeleton. These are referred to margins. Sometime multilocular appearance may be seen
here as psammomatoid juvenile ossifying fibroma and tra- representing the small cystic spaces.
becular juvenile ossifying fibroma. Psammomatoid juve- The trabecular variant is expansive, well-defined and
nile ossifying fibroma was first reported by Benjamins, unilocular or multilocular with cortical thinning and
in 1938, who gave it the designation osteoid fibroma with perforation. The tumor mass is radiolucent with variable
atypical ossification of the frontal sinus. It was later calcification-induced radiopacities, occasionally demon-
termed psammomatoid ossifying fibroma of the nose and strating fine specks and occasionally producing a ‘ground-
paranasal sinuses by Gögl, in 1949. The same lesion was glass’ appearance. Increase in radiodensity may be observed
later termed juvenile active ossifying fibroma by Johnson over time. Root resorption and displacement of involved
et al in 1952. Makek considered the lesion to be a variant teeth are also observed.
572
Histopathogic features Malignant change has not been reported. Fatal consequences
are extremely rare and usually caused by complications
Microscopic examination of psammomatoid juvenile ossi-
arising from direct intracranial extension with resultant
fying fibroma shows well demarcated but unencapsulated
encephalitis and meningitis.
lesion composed of numerous small rounded mineralized
Trabecular juvenile ossifying fibroma should be conser-
collagenous bodies (psammomatoid ossicles) uniformly
vatively excised in its entirety. Recurrences are seen in
distributed within a cellular fibroblastic stroma. The cel-
30–50% of cases. More than one excision may be required
lularity of the fibroblastic stroma varies in different tumors
to achieve cure. No malignant transformation has been
and at different sites within the same lesion. Occasional
reported.
shrunken cells, representing nuclei of osteocytes, are embed-
ded within the ossicles, and there is usually a collagen outer
band to these mineralized bodies. Some of the ossicles Fibrous Dysplasia
may show a basophilic center and eosinophilic fringe. In
the periphery of the lesions, some of the ossicles show a Fibrous dysplasia of bone is an uncommon congenital skel-
transition into small bone trabeculae. The tumor infiltrates etal disorder. It is characterized by the replacement of nor-
and destroys the adjacent bone with focal induction of mal bone and marrow by fibrous tissue, within which
reactive bone formation. Development of aneurysmal cyst irregular trabeculae of woven bone are haphazardly distrib-
is followed by focal myxoid change in the stroma with hem- uted. The maturation of bone is arrested at the woven bone
orrhage and osteoclastic giant cells, with gradual expan- stage. It may affect single (monostotic) or multiple bones
sion and formation of cysts with thin fibrous walls. The (polyostotic) and may be associated with endocrinopathies.
differential diagnosis with other fibro-osseous lesions of A paper by von Recklinghausen in 1891 was probably
the jaw, such as cemento-ossifying fibroma, osteoid oste- the first citation of the disease. Albright pointed out two
oma or bone dysplasia, should be made with a mandatory cases that were described by von Recklinghausen as
pathological study, and is largely based on the nature of examples of osteitis fibrosa generalisata due to hyperpara-
the calcified products of the tumor. The mineralized tissue thyroidism were almost certainly examples of fibrous dys-
of central ossifying fibroma is composed of a variable plasia. The term fibrous dysplasia was first suggested by
combination of mature and immature bony trabeculae and Lichtenstein in 1938 as a designation for multiple bone
lobulated basophilic masses of cementum-like material. lesions that were described by Albright et al as osteitis
There may be occasional concentrically laminated particles, fibrosa generalisata. The lesions of fibrous dysplasia were
called cementicles, but these are not a prominent feature initially considered to be primarily polyostotic. Lichtenstein
of psammomatoid entity. and Jaffe later expanded this concept and noted that iso-
Microscopic features of trabecular variant of juvenile lated (monostotic) form of disease also occurred and was
ossifying fibroma shows an unencapsulated tumor mass by far more common than the polyostotic. McCune and
infiltrating into the surrounding bone and reactive bone Albright in 1936 and 1937 respectively showed an associa-
formation at the periphery. The tumors show a character- tion of polyostotic fibrous dysplasia with abnormal skin
istic loose structure with cell-rich stroma composed of pigmentation, and precocious puberty because of which
fibroblastic spindle cells that produce little collagen. this association has been termed as McCune–Albright’s
Anastomosing trabeculae of osteoid is seen in a pattern syndrome. Jaffe–Lichtenstein described the association of
that resembles ‘paintbrush’ strokes. The osteoid areas polyostotic fibrous dysplasia with abnormal skin pigmen-
mature into woven bone trabeculae that are rimmed with tation; thus it was named as Jaffe–Lichtenstein syndrome.
osteoblasts and show osteocytes embedded in it. Aggregates
of osteoclastic giant cells are commonly present and seen Etiology
in association with the bony trabeculae and in separate The molecular mechanism responsible for fibrous dysplasia
foci in the fibrous stroma, usually—but not always—at sites is a postzygotic activating mutation of the GNAS1 gene
of hemorrhage. Mitotic figures may be observed in the that encodes for the Gs␣ subunit of the heterotrimeric
stroma but are never numerous. Cystic degeneration and G protein complex. The result is constitutive activation of
aneurysmal bone cyst formation have been described in a the adenylyl cyclase enzyme and overproduction of
few cases. 3⬘,5⬘-cyclic adenosine monophosphate. The most common
GNAS1 gene mutations are a replacement of arginine by
either cysteine or histidine at codon 201 (R201C or R201H),
Recurrence after surgical management is common and is but other mutations have also been identified. The severity
reported to range from 30 to 56% in psammomatoid juve- of the disease phenotype is thought to depend on when the
nile ossifying fibroma. Recurrence may be attributed to mutation occurs during embryogenesis. If the mutation
difficulty in proper resection caused by the location of occurs during the formation of the inner cell mass, all three
the lesion and the infiltrative nature of tumor borders. germ cell layers will be affected and the phenotype will be
573
McCune–Albright’s syndrome. If it occurs later in develop- of those with the polyostotic fibrous dysplasia. Similarly,
ment, only one or two germ cell layers will be affected and maxillofacial fibrous dysplasia is the term recently described
the phenotype is less severe. Fibrous dysplasia is con- by Mahajan et al for the lesions which are limited to the
sidered a disease of cells of the mesenchymal stem cell/ facial bones. Painless enlargement of the affected bone is
osteoblastic lineage in which excess cyclic adenosine mono- most commonly seen. The patient usually is not able to
phosphate impairs the ability of the stem cell to differentiate recall the onset of the swelling. Maxillary and mandibular
into a mature functioning osteoblast. fibrous dysplasia is associated with significant facial and
palatal asymmetries, heterogeneous dental anomalies (rota-
tion, oligodontia, displacement, enamel hypoplasia and
Clinical features
hypomineralization, taurodontism, and others), malocclu-
Fibrous dysplasia is mainly diagnosed before the age of sion, and a high caries index score.
30 years and is equally seen in both the sexes. It occurs
both in polyostotic and monostotic forms. Monostotic Radiographic findings
form is at least six times more common than polyostotic
The diagnosis of fibrous dysplasia can be made by radio-
form.
graphs although it is not unusual for bone biopsies to be
In polyostotic form of disease two or more bones are
required because of uncertainty based on radiographs alone.
affected generally the long bones, ribs and skull, although
The characteristics, which are somewhat variable, include
any bone may be affected. The lesions are often found
a ground-glass appearance, which occurs due to superim-
unilaterally. Painless expansion of the affected area is most
position of a myriad of thin, poorly calcified trabeculae.
common complaint of the patient. Pathological fracture with
Ground-glass appearance can be best appreciated in a
resultant pain and bone deformity are other symptoms.
good quality periapical or occlusal view (Figure 2). Early
Presence of abnormal skin pigmentation with polyostotic
lesions may be radiolucent or mottled. Mandibular lesions
fibrous dysplasia is termed as Jaffe–Lichtenstein syn-
sometimes may appear multilocular on the radiograph
drome. The abnormal hyperpigmentation seen resembles
which is an optical illusion occurring due to endosteal
café-au-lait spots which mean coffee with milk. The hyper-
cortical erosion caused by growth of the lesion contrasting
pigmentation tends to be present on the same side and on
with areas of preserved normal cortex. The lesion of
the skin overlying the lesions of fibrous dysplasia. The
fibrous dysplasia is usually ill-defined radiographically;
hyperpigmented macules are well-defined and have irreg-
ular borders which sometimes distinguish them from the
lesions associated with neurofibromatosis. The color can Figure 2
be medium to dark brown.
Association of endocrine abnormalities with skin
hyperpigmentation and polyostotic fibrous dysplasia is
termed as McCune–Albright’s syndrome. A variety of
endocrine abnormalities such as accelerated skeletal growth,
acromegaly, gigantism, hyperprolactinemia, Cushing’s
syndrome, hyperthyroidism, hyperparathyroidism, diabe-
tes mellitus, hypothalamic hypogonadism and hypophos-
phatemic rickets, gynecomastia, spermatogenesis in young
boys and sexual precocity in girls have been associated
with the disease. Sexual precocity is the most common
endocrine abnormality seen in the affected individual.
Male children exhibit enlarged genitalia and advanced
secondary sex characteristics. Female children manifest
estrogen excess.
Monostotic form of the disease is seen in 80–85% of
cases. The jaws are the most common areas affected, with
the maxillary jaw being more common than the mandibu-
lar jaw. The maxillary lesions frequently involve a group
of contiguous bones separated by sutures (i.e. maxilla,
zygoma, sphenoid and occiput) and thus are not strictly
monostotic lesions. Such lesions are more appropriately Mandibular occlusal radiograph showing expansion of the
classified as craniofacial fibrous dysplasia. The craniofa- cortical plates and ground-glass appearance in fibrous
dysplasia. Courtesy: Department of Oral Medicine and
cial region is involved in up to 25% of the patients with
monostotic fibrous dysplasia, and in approximately 40–60%
574
they tend to blend imperceptibly into the adjacent bone. to be delicate and are not connected to one another. The
Foci of irregular or denser calcification may be seen super- trabeculae are not sharply defined and along their margins
imposed on ground-glass appearance. Waters’ view of the bone matrix streams out along the collagen fibers that
maxillary lesions frequently shows a radiodense area that extend from the trabeculae into the neighboring stroma.
largely obliterates the maxillary sinus and involves the Under polarizing microscope irregular birefringent fibers
zygoma and lower rim of the orbit. Most common mani- of immature or woven bone can be demonstrated. The
festation of fibrous dysplasia in the skull is the thickening bone formed is metaplastic in nature thus the trabeculae
of the occiput and base of skull. Involved dentition shows are not seen to be surrounded by osteoblasts. This form of
narrowing of the periodontal ligament space with an ill- metaplasia is called a fibro-osseous metaplasia. Older
defined lamina dura that blends with the abnormal bone. lesions may show lamellar maturation. At the periphery of
Taurodontism of the teeth may also be seen. Involvement the lesion pre-existing host lamellar bone may be seen
of the mandible results in expansion of the cortical plates without a clear demarcation between the lesion and the
and bulging of the lower border of the mandible. When host bone. The fibrous stroma comprises immature appear-
long bones are affected in an advanced stage, there may ing small, slender spindle cells in loose and whorled
be a Shepherd’s crook deformity of the proximal femur. arrangement. Giant cells are usually not seen in lesions of
Computerized tomography (CT) of fibrous dysplasia is fibrous dysplasia but if seen are usually associated with the
useful in demonstrating the ground-glass texture of the pre-existing mineralized tissue. Presumably, they are
lesion and is particularly helpful in defining the extent of responsible for spread of the lesion.
craniofacial disease (Figure 3). The microscopic features of macules show excessive depo-
Magnetic resonance imaging (MRI) does not provide a sition of melanin, despite a normal number of melanocytes.
characteristic appearance of fibrous dysplasia and there-
fore is a less useful imaging modality. Laboratory investigations
Serum calcium and phosphorus levels are normal. The serum
alkaline phosphatase levels may be elevated, roughly cor-
Grossly, the tissue is calcified, firm, gritty or granular. The responding to the extent of the bone lesions. The levels
characteristic microscopic features of fibrous dysplasia do not increase as seen in Paget’s disease. Urinary hydro-
comprises a fibrous stroma in which spicules of woven xyproline, specific index of bone collagen resorption, can
bone are found. The woven bone is present in the form of be elevated. In patients with McCune–Albright’s syndrome
irregular shaped trabeculae which can be resembled to the various endocrinopathies are associated with eleva-
Chinese script writing (Figure 4). The bone trabeculae tend tions of circulating hormones depending on which glands
are affected. High levels of growth hormone, prolactin,
Figure 3 and thyroid hormones and, less commonly, testosterone,
adrenocorticosteroids and parathyroid hormone have
been reported. In patients with rickets or osteomalacia,
hypophosphatemia and hyperphosphaturia are usually
present.
Figure 4
Computed tomographic image showing expansion of the

cortical plate and invasion of the maxillary sinus in fibrous The irregular (Chinese letter-shaped) trabeculae of bone in
dysplasia. Courtesy: Department of Oral Medicine and fibrous dysplasia. Courtesy: Department of Oral Pathology,
Radiology, MCODS, Mangalore MCODS, Mangalore
575
Management and prognosis is also seen in individuals suffering from osteogenesis

imperfecta.
In most cases, the disease tends to stabilize and essentially
Mutations that interfere with expression of the collagen
stops growing when skeletal maturation is reached. Small
gene, formation of the triple helix (amino acid sequencing),
lesions, particularly in mandible can be removed by com-
or procollagen secretion, affect the structure and function
plete resection. However diffuse and larger lesions preclude
of collagen fibrils, resulting in osteogenesis imperfecta.
their removal without extensive surgery. Cosmetic defor-
Electron microscopic studies in osteogenesis imperfecta
mity, with associated psychological problems or functional
reveals a smaller diameter of the collagen fibril and smaller
deformity may require surgical intervention. Re-growth
than normal apatite crystals when compared to that of nor-
of the lesion is seen in 25–50% of the patients after a
mal individuals.
shave-down procedure. Re-growth after surgical proce-
Mutations in COL1A1 gene on chromosome 17 and the
dures appears to be more common in younger patients
COL1A2 gene on chromosome 7 (genes that encode for the
suggesting that surgical intervention should be delayed as
synthesis and/or structure of type I collagen) may cause a
long as possible.
combination of production of abnormal collagen and
Malignant change in fibrous dysplasia, usually an
decreased production of normal collagen. The variability in
osteosarcoma rarely has been reported. This risk increases
combinations results in the different phenotypic expressions
in patients who receive radiation therapy. Thus radiation
of osteogenesis imperfecta.
therapy is definitely contraindicated in fibrous dysplasia.
Milder forms of osteogenesis imperfecta are caused
Patients should be kept under long-term follow-up to rule
primarily by the decreased production of normal collagen,
out any malignant changes. Patients with fibrous dyspla-
while more severe forms are caused primarily by the pro-
sia do not have any complications associated with the
duction of abnormal collagen. These abnormalities may be
routine dental care.
dominantly inherited or the result of sporadic mutation.
Classification
BONE DISEASES
Dominant or classical osteogenesis imperfecta Majority
of the cases of osteogenesis imperfecta (up to 90%) are
The bones of the facial skeleton particularly the maxilla
caused by a dominant genetic defect. These individuals
and mandible are affected by various diseases. These dis-
have a 50% chance of passing on the disorder to each of
eases present with a myriad of clinical features and char-
his/her progeny. Though most children in the dominant
acteristic oral manifestations. This section will attempt to
form inherit the disorder from a parent, some are born
highlight common bone diseases affecting the maxillofacial
with the dominant form of osteogenesis imperfecta even
skeleton.
though there is no family history of the disorder. In these
children, the genetic defect occurs as a result of a sponta-
Osteogenesis Imperfecta neous mutation.
Osteogenesis imperfecta has also been referred to as Recessive osteogenesis imperfecta It is believed that
brittle bone disease, Vrolik syndrome and Ekman–Lobstein approximately 10–15% of cases of osteogenesis imperfecta
syndrome. are caused by a recessive mutation. The parents do not have
Osteogenesis imperfecta is an inherited disorder of the the disorder but their genes are mutated. To inherit the
connective tissue having an autosomal dominant pattern recessive condition the individual must receive a copy of
of inheritance. However, autosomal recessive and non- the mutation from both parents. If one parent has osteo-
hereditary types have also been known to occur. The con- genesis imperfecta because of a recessive mutation, 100%
dition is characterized by fragile bones that tend to break of their children will be carriers of the recessive mutation.
easily, often from the mildest of trauma. Whether any of these children will have the condition will
depend on their inheritance from the other parent.
Based on the clinical features and molecular studies
Pathophysiology
osteogenesis imperfecta is currently divided into eight
The primary pathology in osteogenesis imperfecta is the types.
disturbance in the synthesis of type I collagen (predominant It is estimated that approximately 85–90% of osteo-
protein of the extracellular matrix of most tissues). In bone, genesis imperfecta are caused by a dominant mutation in
this defect of extracellular matrix causes osteoporosis. The a gene coding for type I collagen. Osteogenesis imperfecta
affected bones become weak and fragile thereby making Types I, II, III and IV belong to this category. Types V and
them susceptible to fracture. Type I collagen is also a VI do not have a type I collagen mutation, but the genes
major constituent of dentin, sclerae, ligaments, blood ves- causing them have not yet been identified. Types VII and
sels and skin; therefore, abnormalities of these structures VIII are inherited in a recessive manner.
576
Clinical features long bones with broad and beaded ribs. Type IIB shows
broad and short long bones with thin ribs that have little
Type I It is the most common and mildest form of osteo-
or no beading. Type IIC group shows thin and longer long
genesis imperfecta. These patients present with normal or
bones with thin and beaded ribs.
close to normal stature. They may have muscle weakness
and hypermobility of joints. Since the bones are fragile Type III In Type III osteogenesis imperfecta the bones
they tend to fracture easily and most of the fractures occur are extremely fragile and hence have an increased ten-
before puberty. Fragility of the walls of the blood capillar- dency to fracture. Fractures are often present at birth.
ies may be seen. The patients may present with a triangu- These individuals are usually short statured. The sclera has
lar shaped face. Some authors describe the facies to be a bluish tint. It has been reported that the mortality rate is
similar to that of cleidocranial dysostosis. The skull may generally higher at adolescence. Kyphoscoliosis causes
be disproportionately large with a temporal bulge thereby cardiopulmonary distress resulting in death. A barrel-
causing the ears to be pushed outward and forward. The shaped rib cage is seen. The patients may have a triangular
forehead is broad with frontal bossing giving rise to a face. Hearing loss can be seen; some patients may present
mushroom-shaped skull. with brittle opalescent teeth. Histologically the collagen is
Hearing loss due to osteosclerosis, may be usually seen improperly formed.
in the 2nd and 3rd decades of life. These patients typically
present with blue, purple or gray tinted sclera (Figure 5). Type IV Individuals suffering from Type IV osteogenesis
The sclera in these individuals tends to be extremely thin, imperfecta may show mild to moderate bone fragility. It is
therefore allowing the color of the underlying choroid to considered that the severity of this form of the condition
be transmitted. Most of the patients have normal teeth is in between the severity of Type I and Type III osteogen-
whereas some may present with opalescent brittle teeth. esis imperfecta. These individuals may present with a mild-
Histologically collagen has normal structure but the quan- to-moderate bone deformity and a slightly shorter than
tity of the collagen present is usually less than normal. average stature. Bones are fragile and fractures are usually
present before puberty. The sclera is generally white (nor-
Type II It is the most severe and lethal form of this con- mal) in color. Some patients may present with a faint blue
dition. Most infants are still born or die shortly after birth. tint. Triangular face and barrel-shaped rib cage is usually
The death may occur mostly due to respiratory distress or seen. Teeth may be brittle and appear opalescent in some
intracerebral hemorrhage. It is estimated that almost 90% patients. Hearing deficits may be encountered. Histologi-
of the infants do not live longer than a month. Severe bone cally there is improper formation of collagen.
deformity and multiple fractures are evident. These indi-
Type V It has a dominant inheritance pattern. Individuals
viduals have a small stature with underdeveloped lungs.
of this form of the condition do not exhibit mutations in
Blue colored sclera is seen. The collagen that is formed is
type I collagen genes. It mimics the symptoms and signs of
insufficient in quantity and is of poor quality. Dentino-
Type IV osteogenesis imperfecta.
genesis imperfecta may be seen.
The site of fracture shows hypertrophic calluses. Radio-
Based on the radiographic findings of the long bones
graphs of long bones reveal a radiodense band adjacent to
and ribs, Type II osteogenesis imperfecta is subdivided into
the growth plate. The sclera is white and teeth are normal.
groups A, B and C. Type IIA demonstrates broad and short
The characteristic microscopic finding is the presence of
‘mesh-like’ pattern of bone.
Figure 5
Type VI This subtype of osteogenesis imperfecta mimics
the symptoms and signs of Type IV. It is believed to be
inherited as a recessive trait.
The characteristic ‘fish scale’ appearance of bone under
a microscope is unique for this type of osteogenesis imper-
fecta. Blood investigations reveal minimally elevated lev-
els of alkaline phosphatase.
Type VII It is inherited in a recessive manner. It is
believed to originate from mutation to the cartilage asso-
ciated protein (CRTAP) gene.
This type of osteogenesis imperfecta mimics Type IV or
lethal Type II. Infants may present with a small head and
round face. The sclera are white; other features include
Blue colored sclera. Courtesy: Department of Oral Medicine
short stature, short humerus and femur. Another typical
and Radiology, KLE Institute of Dental Sciences, Bangalore
feature is a deformation of the hip joint (coxa vara).
577
Type VIII It is inherited in a recessive manner. It is of multiple joints usually with flexion deformities, with or
believed to be caused by the mutation of LEPRE1 gene. The without pterygia or webbing at the joints involved) and
clinical features in this type are similar to those of osteogen- osteogenesis imperfecta.
esis imperfecta lethal Type II or Type III. However, infants
present with severe growth deficiency and white sclera. Management
Exercises such as walking, swimming and water therapy,
Orofacial manifestations
to strengthen bones and muscles are recommended.
Patients suffering from this condition can present with tri- Patients should be advised to refrain from smoking, alco-
angular facies as a result of the soft craniofacial bones hol consumption, excessive use of caffeine and use of ste-
along with a large and thin calvarium. roids as these can accelerate the rate of bone depletion.
Osteogenesis imperfecta can be associated with den- Bisphosphonates such as palmidronate have been used
tinogenesis imperfecta. The color of teeth affected by successfully in managing osteogenesis imperfecta. It is
dentinogenesis imperfecta may vary from blue to gray to believed that palmidronate minimizes the rate of bone
brown. The outer enamel may often chip off to reveal the loss. It can be administered intravenously in a dosage of
underlying soft dentin. 7.5 mg/kg/year at 4–6 month intervals.
Other skeletal and dental disturbances noted are the fre-
quent presence of a skeletal class III malocclusion (especially
in type III and type IV osteogenesis imperfecta) and anterior Osteopetrosis
and posterior crossbites. Osteopetrosis is also known as marble bone disease
and Albers–Schönberg disease. It was first described by
Radiographic features Albers–Schönberg, a German radiologist in 1904.
General radiographic findings The common feature in Osteopetrosis is a rare disorder of bone that arises from
all forms of osteogenesis imperfecta is osteoporosis. In the defective differentiation and function of osteoclasts and
milder forms of the disease, the bones exhibit thin cortices reduced generation of superoxide by leukocytes. As the
and minimal fractures. However, in severe forms of the osteoclasts fail to resorb bone, the bones become dense
disease the long bones are stunted and exhibit extensive and fragile. Bone fragility occurs because very few colla-
fractures. The skull is poorly mineralized and exhibits gen fibers connect osteons adequately and the remodeling
wormian bones. Spinal deformities such as flattened spinal of woven bone to compact bone is defective. This, in turn,
bones (platyspondyly) and S-shaped scoliosis. Other char- will make bones vulnerable to fracture.
acteristic features associated with osteogenesis imperfecta The actual incidence of osteopetrosis is unknown.
include cod fish vertebrae, popcorn calcifications in the However, Beighton et al (1979) reported that the overall
metaphyseal-epiphyseal region of long bones, especially incidence of osteopetrosis was approximately one case in
of the knee and ankle caused due to repeated microfrac- 100,000–500,000 population.
tures at the growth plate, basilar invagination or impres-
Pathophysiology
sion (projection of the tip of the odontoid process above
the McGregor line) and Tam O’Shanter skull (caused by The gene for adult osteopetrosis has been mapped to chro-
large and thin cranium with platybasia). mosome 1p21 (Van Hul, 1997).
Whyte (1999) described few probable reasons for the
Radiographic findings specific to oral cavity Teeth may
functional failure of osteoclasts such as abnormalities in
have bulbous crowns constricting at the cervical portion
the osteoclast stem cell or its microenvironment, osteoblast
and stunted roots. The pulp chamber and radicular portion
precursor cells or the mature heterokaryon or in the bone
of the pulp may be partially or totally obliterated.
matrix.
Other factors that may predispose to altered bone
Dental considerations resorption that may influence osteoclastic activity include
Since most patients especially in the younger age group synthesis of abnormal parathyroid hormone and defective
have high susceptibility for fractures, excessive force should production of interleukin-2 (IL-2). Hofbauer (1999) reported
best be avoided during extraction of teeth. Tooth wear osteopetrosis in cathepsin K (cysteine protease important
may be managed esthetically with restorations or crowns. for osteoclast function) deficient mice.
Types
Three distinct clinical forms of osteopetrosis are recognized:
Datta and others (2005) reported a 34-week pre-term baby
suffering from Bruck syndrome, which is a combination of 1. Adult form of osteopetrosis (osteopetrosis tarda)—auto-
arthrogryposis multiplex congenita (congenital contractures somal dominant
578
2. Infantile form of osteopetrosis (osteopetrosis con- Other features of abnormal endosteal bone formation are
genita)—autosomal recessive evident such as tortuous lamellar trabeculae replacing the
3. Intermediate form of osteopetrosis (marble bone cancellous portion of bone, globular amorphous bone depo-
disease)—autosomal recessive. sition in marrow spaces and osteophytic bone formation.
The other rare varieties of osteopetrosis are transient The number of osteoclasts may be increased, normal or
infantile, lethal and post infectious. decreased, but there is no evidence of functional osteo-
clasts, as Howship’s lacunae are not visible.
Clinical features In the adult form of osteopetrosis, there is increased
amount of osteoid. The osteoclasts are usually few in number
Adult form of osteopetrosis The adult form of osteope- and they lack ruffled borders. Occasionally, the osteoclasts
trosis is usually discovered later in life on radiographic may be large in size and may be present in large number.
examination. It is believed to be asymptomatic in about Presence of woven bone may be a common feature.
60% of the patients. The usual complaint could be related In the infantile form of osteopetrosis, osteoclasts are
to bone pain, fractures and osteomyelitis. The typical radio- usually abundant and found at the bone surfaces. Osteoclast
graphic finding in osteopetrosis tarda is sclerosis of the nuclei are especially numerous, but the ruffled borders or
axial skeleton. The long bones are generally spared or clear zones that characterize normal osteoclasts are absent
minimally affected. and fibrous tissue usually crowds the marrow spaces.
The adult form has two subtypes:
1. Type I: Occurrence of fractures is rare, cranial nerve Radiographic features
compression, normal serum acid phosphatase levels. There is generalized, homogeneous increase in density
2. Type II: Fractures are commonly seen, cranial nerve is throughout the skeleton. The increased density obscures
rarely compressed, high levels of serum acid phospha- the trabecular pattern such that the cortical bone and can-
tase levels. cellous bone cannot be differentiated. Long bones may
A prominent dental consideration is the susceptibility to appear club-like or bone within bone (endobone).
develop osteomyelitis following dental extractions. Other The skull shows increased radiodensity, especially at
oral findings include increased susceptibility to carious the base. Sinuses are small and not fully pneumatized. The
lesions, delayed eruption of teeth and/or malformed teeth. extremely radiopaque vertebrae may exhibit alternating
Osteopetrosis congenita It is a severe form in which the bands which is referred to as known as the rugger-jersey
patient suffers from osteopetrosis at birth or in the first few sign. The pelvic bones show subcrestal sclerosis.
years of life. This condition is usually fatal due to bleeding,
severe anemia or uncontrollable infection. The skeleton Radiographic findings of jaws and teeth
reveals generalized sclerosis. Osteopetrosis results in severe The maxilla and mandible exhibit increased radiodensity.
bone marrow failure. Subsequently hepatosplenomegaly is Owing to the increased radiodensity, internal structures such
seen to compensate for hematopoiesis. Involvement of the as teeth and other normal anatomic landmarks are not
cranial foramina results in blindness, deafness and hydro- readily visible.
cephalus. The child shows extreme growth retardation. The increased density of bone compromises the vascu-
Other orofacial features include frontal bossing, hyper- larity thereby making the jaws susceptible to infections
telorism and paranasal sinus malformations. Like other (such as osteomyelitis).
forms of osteopetrosis the jaws are susceptible to osteomy- Osteopetrosis may cause delayed eruption of teeth.
elitis following dental extractions. Other dental findings include relatively thickened lamina
Marble bone disease The intermediate form of osteope- dura, premature loss of teeth and malformed teeth. Teeth
trosis is not as severe as osteopetrosis congenita. In this may be susceptible to carious lesions as they are poorly
form of the disease, bone marrow failure is not seen. These calcified.
individuals have retarded growth and may present with
fractures in the 2nd decade of life. However, they may Management and prognosis
show intracranial calcifications, macrocephaly and cranial
Infantile osteopetrosis if untreated will usually result in
nerve deficits. These individuals also show increased sus-
death in the first few years of life due to bone marrow
ceptibility to infections.
failure.
The only cure for children with severe osteopetrosis is
allogenic hematopoietic stem cell transplantation.
The presence of remnants of mineralized primary spongiosa Askmyr et al (2008) propose the possibility for gene
that persists as islands of calcified cartilage within mature replacement therapy to manage osteopetrosis. In their article,
bone is typical of osteopetrosis. they describe that the hematopoietic stem cell-targeted
579
gene therapy in a mouse model of infantile malignant Genetic basis

osteopetrosis has reportedly shown to correct many signs
The locus for the cherubism gene is 4p16. The most reason-
and symptoms of osteopetrosis.
able conclusion from the linkage data obtained in a study
Key (1995) conducted a 6-month trial to study the effi-
of four families by Tiziani and others is that the locus for
cacy of recombinant human interferon gamma on osteope-
cherubism is located on the telomeric side of D4S1582.
trosis. In the study comprising 14 patients suffering from
Ueki et al detected point mutations causing amino acid
severe osteopetrosis, they administered subcutaneous
substitutions in the SH3-binding protein SH3BP2.
injections of recombinant human interferon gamma-1b
(1.5 ␮g/kg of body weight per dose) three times per week
Clinical features
6 months. There were no side effects reported. They con-
cluded that long-term therapy with interferon gamma Cherubism appears to have 100% penetrance in males and
increases bone resorption and hematopoiesis and improves only 50–70% penetrance in females. Although the condi-
leukocyte function. tion is known to be hereditary, in some cases there has
Corticosteroids have also been used in managing osteo- been no detectable family history, and although it usually
petrosis, though it is not a preferred modality of treatment. occurs bilaterally, there have also been cases of unilateral
Steroids have known to increase the red blood cell mass and involvement, perhaps because of incomplete penetrance or
platelet count, but they do not improve the bone mass. new mutations.
In another independent study, children suffering from Typically, the jaw lesions of cherubism remit spontane-
osteopetrosis were administered 1,25-dihydroxy vitamin D. ously when affected children reach puberty, but the reason
The study showed no improvement in the clinical condition for this remission is unknown. The reduction in osteoclast
of these children. However, bone biopsies showed evi- formation caused by sex steroids and the increase in plasma
dence of increased osteoclastic bone resorption. concentrations of estradiol and testosterone at puberty
Key and others (1984) suggested that large doses of both suggest that the genetic defect responsible for the
calcitriol (up to 32 ␮g/day) along with the use of calcium localized increase in osteoclasts in cherubism is overridden
deficient diet will stimulate bone resorption by activating and normalized by the increased synthesis of sex steroids.
the dormant osteoclasts. Affected children are normal at birth and are without
clinically or radiographically evident disease until 14 months
to 3 years of age. At that time, symmetric enlargement of
Cherubism
the jaws begins. Typically, earlier the lesion appears, the
Cherubism is a non-neoplastic hereditary bone lesion that more rapidly it progresses. The self-limited bone growth
is histologically similar to central giant cell granuloma. usually begins to slow down when the patient reaches
It affects the jaws of children bilaterally and symmetri- 5 years of age, and stops by the age of 12–15 years. At
cally, producing the characteristic cherubic appearance. puberty the lesions begin to regress. Jaw remodeling con-
Cherubism has also been referred to as familial or heredi- tinues through the 3rd decade of life, at the end of which
tary fibrous dysplasia, bilateral giant cell tumor and familial the clinical abnormality may be subtle.
multilocular disease. The signs and symptoms depend on the severity of
Cherubism was first described by Jones in 1933. He the condition and range from clinically or radiographically
termed it familial multilocular disease of the jaws. How- undetectable features to grotesquely deforming mandibu-
ever, as the cystic nature of the condition was invalidated lar and maxillary overgrowth with respiratory obstruction
Jones and others were the first to use the term cherubism. and impairment of vision and hearing. Cherubism was
The word ‘cherub’ originally designated a member of the reportedly fatal in one case, where aspiration occurred
second order within the Christian celestial chorus. These because of the grotesque facial deformity.
were creatures with severe, staring eyes (including eyes on The jaw lesions are usually painless and symmetric
the wings and the body) and a wheel below the feet. swellings, having florid maxillary involvement (Figures 6
Angels constituted another order within the celestial cho- and 7). The lesions, which are firm to palpation and non-
rus, and angels with childish, full-cheeked faces, often tender, most commonly involve the molar to coronoid
gazing upward, were widely depicted in baroque art. Thus, regions, the condyles usually being spared, and are often
the term ‘cherubism’, is actually inappropriate for the dis- associated with cervical lymphadenopathy. Enlargement of
ease, because the typical clinical picture does not resemble the cervical lymph nodes contributes to the patient’s full-
a classical cherub but a baroque angel. faced appearance and is said to be caused by reticuloendo-
According to the WHO classification, cherubism belongs thelial hyperplasia with fibrosis. The lymph nodes become
to a group of non-neoplastic bone lesions affecting only the enlarged before the patient reaches 6 years of age, decrease
jaws. It is a rare, benign condition with autosomal domi- in size after the age of 8 years, and are rarely enlarged
nant inheritance, and it is one of the very few genetically after the age of 12 years. Intraoral swelling of the alveolar
determined osteoclastic lesions in the human body. ridges may occur. When the maxillary ridge is involved,
580
Figure 6 Figure 7
Extraoral photograph of a 7-year-old girl with cherubism. Extraoral photograph of the girl’s 14-year-old brother
Reproduced with permission from editor, JCDA. Ongole R, also suffering from cherubism. Reproduced with
Pillai RS, Pai KM. Cherubism in siblings: a case report. permission from editor, JCDA. Ongole R, Pillai RS, Pai KM.
J Can Dent Assoc 2003;69(3):150–54 Cherubism in siblings: a case report. J Can Dent Assoc
2003;69(3):150–54
the palate assumes a ‘V’ shape. A rim of sclera may be vis-

ible beneath the iris, giving the classic ‘eye to heaven’ In order to overcome the limitations of Arnott’s classi-
appearance. fication, Kalantar Motamedi developed a different classifi-
Numerous dental abnormalities have been reported, such cation system, which addresses both the involvement and
as agenesis of the second and third molars of the mandible, aggressive behavior of the disease.
displacement of the teeth, premature exfoliation of the
primary teeth, delayed eruption of the permanent teeth, ❍ Grade I (divided into five classes): Lesions of the man-
and transpositions and rotation of the teeth. In severe dible without signs of root resorption
cases, tooth resorption occurs. ❍ Grade II (divided into three classes): Lesions of the
Although cherubism was initially described as a familial mandible and maxilla without signs of root resorption
disease affecting the jaws, cases without any apparent hered- ❍ Grade III (divided into five classes): Aggressive lesions
itary origin have been reported. In a few cases cherubism has of the mandible with signs of root resorption
been described as being associated with other diseases and ❍ Grade IV (divided into three classes): Lesions involv-
conditions such as Noonan’s syndrome, gingival fibromato- ing the mandible and the maxilla and showing signs
sis, psychomotor retardation and obstructed sleep apnea. of root resorption
❍ Grade V: Massively growing, aggressive and exten-
Radiographic staging sively deforming juvenile cases involving the maxilla
and the mandible and which may include the coronoid
Arnott suggested the following grading system for the process and condyles.
lesions of cherubism:
❍ Grade I: Involvement of both mandibular ascending Radiographic features
rami Cherubism is characterized by bilateral multilocular cystic
❍ Grade II: Involvement of both maxillary tuberosities
expansion of the jaws (Figures 8 and 9). Early lesions occur
as well as the mandibular ascending rami in the posterior body of the mandible and the ascending
❍ Grade III: Massive involvement of the whole maxilla and
rami. Maxillary lesions may occur at the same time but
mandible except the coronoid process and condyles. escape early radiographic detection because of overlap of
Arnott’s staging was not exhaustive and some authors the sinus and nasal cavities. Displacement of the inferior
found it difficult to stage their cases. alveolar canal has been reported.
581
Figure 8
Orthopantomograph showing bilateral multilocular radiolucencies involving the mandible and displacement of teeth in
cherubism in a 7-year-old patient. Reproduced with permission from editor, JCDA. Ongole R, Pillai RS, Pai KM.
Cherubism in siblings: a case report. J Can Dent Assoc 2003;69(3):150–54
Figure 9
Orthopantomograph showing bilateral multilocular radiolucencies involving the mandible and displacement of teeth in
cherubism in a 14-year-old patient. Reproduced with permission from editor, JCDA. Ongole R, Pillai RS, Pai KM.
Cherubism in siblings: a case report. J Can Dent Assoc 2003;69(3):150–54
Destruction of the alveolar cavity may displace the and tartrate-resistant acid phosphatase, which is charac-
teeth, producing a radiographic appearance referred to as teristic of osteoclasts. The collagenous stroma, which con-
‘floating tooth syndrome’ (Figure 10). With adulthood, the tains a large number of spindle-shaped fibroblasts, is
cystic areas in the jaws become re-ossified, which results considered unique because of its water-logged, granular
in irregular patchy sclerosis. There is a classic (but non- nature.
specific) ground-glass appearance because of the small, The capillaries exhibit large endothelial cells and peri-
tightly compressed trabecular pattern. vascular capillary cuffing. The eosinophilic cuffing is con-
sidered to be a characteristic feature of cherubism. However,
these deposits are not present in many cases, and their
absence does not exclude the diagnosis of cherubism.
Histologic examination of the lesions usually reveals Resolving lesions of cherubism show an increase in fibrous
numerous multinucleated giant cells. These multinucleated tissue, a decrease in the number of giant cells and forma-
cells show strong positivity for monoclonal antibody 23c6 tion of new bone.
582
Figure 10
Orthopantomograph showing bilateral multilocular radiolucencies affecting the body and ramus of the mandible in cherubism.
The radiograph also shows the lower right second molar appearing to be floating. Courtesy: Department of Oral Medicine and
Differential diagnosis Treatment

Giant cell granuloma of the jaws, osteoclastoma, aneurys- The condition is self-limiting and generally regresses
mal bone cyst, fibrous dysplasia and hyperparathyroidism by puberty. Surgery to correct the jaw deformities of
are considered in the differential diagnosis of cherubism. cherubism is rarely indicated. If necessary, surgery is
Giant cell granuloma and osteoclastoma are histologi- usually undertaken after puberty or when severe func-
cally similar to cherubism. However, giant cell granuloma tional and esthetic problems are reported. Although exac-
is usually unilateral and usually affects patients between erbation has sometimes been reported after surgery, it is
20 and 40 years of age, whereas cherubism is a symmetric believed that surgery ultimately accelerates the involution
lesion affecting children. process.
Osteoclastoma rarely occurs in the jaws, unlike cherubism. Liposuction has been used to change the contour of the
Aneurysmal bone cyst may also exhibit giant cells, but jaws in a patient with cherubism. The procedure involves
its main feature is a cavity lined with tissue other than removal of the fibro-osseous tissue (consistency of firm
endothelium. Jell-o). This consistency of the tissue makes the removal
Both fibrous dysplasia and hyperparathyroidism contain difficult with bone ronguers and curettes. A blunt suction
large numbers of osteoclasts. However, histologic exami- lipectomy cannula is used to curette the abnormal tissue
nation of the classic form of fibrous dysplasia reveals tra- and then aspirated by a high-suction apparatus.
beculae of immature bone resembling Chinese characters Radiation has been used successfully, but it is discour-
within the proliferating stroma. These trabeculae are not aged because of possible retardation of jaw growth as
rimmed by osteoblasts. Furthermore, polyostotic fibrous well as the risks of osteoradionecrosis and induction of
dysplasia first presents in the 2nd or 3rd decade of life. malignancy.
Hyperparathyroidism rarely affects the jaw in an iso- The treatment of choice is curettage, but equally good
lated manner. Its histologic features differ from those of results have been obtained with simple contouring to pro-
cherubism in which it does not contain the mononuclear duce a more cosmetically acceptable appearance.
stromal cell population that is characteristic of the latter. Southgate and others (1998) proposed the possibility
Finally, peritrabecular fibrosis is a feature of hyperpara- of managing cherubism with calcitonin. de Lange et al
thyroidism but not cherubism. Serum concentrations of (2007) report a case of cherubism treated with calcitonin.
parathyroid hormone and calcium also help to distinguish
these lesions. Levels of serum alkaline phosphatase are
Infantile Cortical Hyperostosis
generally elevated in cases of fibrous dysplasia. In cases of
hyperparathyroidism, levels of serum calcium are elevated, Infantile cortical hyperostosis was first described by Caffey
levels of serum phosphorus are decreased and levels of (1945) and Silverman. It is also known as Caffey’s disease,
serum alkaline phosphatase are generally within normal Caffey–Silverman syndrome, familial or sporadic infantile
levels. cortical hyperostosis.
583
It is a self-limiting, inflammatory process with unknown with the underlying cortical bone. Over time the bone
etiology characterized by hyperostosis or thickening of undergoes remodeling to assume normal morphology.
cortices of various bones in the skeleton. Some authors The mandible (unilaterally or bilaterally) is affected in
believe that it is transmitted as an autosomal dominant almost every patient. The cortices exhibit thickening and
trait with incomplete penetrance. Other proposed etiologi- sclerosis.
cal factors include a primary arterial abnormality and an Bykov and others (2003) describe a characteristic radio-
allergic phenomenon. tracer uptake in the mandible in bone scan of a child
suffering from localized mandibular Caffey disease. They
described the finding as ‘bearded infant’ appearance.
Clinical features
As the name suggests the condition is first noticed at birth Management
or in the first few months of life. It has been reported that
the average age of onset varies from about 7 to 11 weeks. Caffey disease is almost always self-limiting. It usually
Infants are usually very irritable and present with tender, regresses without any complications by about 9 months.
deep seated and firm swellings. The swellings are evident Literature review shows use of corticosteroids and NSAIDs to
on the scalp, face, neck, thorax and extremities overlying manage symptoms and signs of pain and inflammation. Other
the affected bones. Swellings in the mandible may be seen drugs that have been used with some success are prosta-
in the ramus and angle of the mandible. glandin inhibitors such as naproxen and indomethacin.
Hyperostoses of various bones of the skeleton are seen.
The mandible and clavicular bones are reported to be most
commonly affected. However, the ribs, skull and long Idiopathic Osteosclerosis
bones have been reportedly involved. Idiopathic osteosclerosis has also been termed dense bone
Other associated clinical features that have been reported island and enostosis.
in literature include facial nerve palsy (Challapalli et al, It is believed that enostosis is a counterpart of exostoses
1998) and Hotlzman (1972) reported Erb’s palsy (form of that occur in the inner surface of the cortical plates within
brachial plexus palsy in which there is paralysis of the the cancellous bone. It represents a focus of mature compact
muscles of the upper arm and shoulder girdle) in a patient (cortical) bone within the cancellous bone (spongiosa).
with infantile cortical hyperostosis affecting the scapula. The etiology for their occurrence is unknown. These are
asymptomatic, benign and usually found on routine radio-
Histopathologic features graphic investigations. Greenspan (1995) in a review of
enostosis described that enostosis is probably congenital or
Initial stages of the condition show periosteal and soft developmental in origin and represents failure of resorption
tissue inflammation. In the subsequent stages thickening during endochondral ossification.
of the periosteum and immature lamellar bone beneath the
periosteum is seen.
Characteristic features
However, in the advance stage of the disease, no signs
of inflammation or subperiosteal changes are evident. Idiopathic osteosclerosis have known to occur anywhere
Hyperplasia of the lamellar cortical bone is appreciated. in the skeleton. However, the sites that are commonly
involved are the pelvis, femur, other long bones, spine and
the mandible.
Laboratory findings
McDonnell (1993) in a review of 107 patients reported
Though laboratory findings are not specific for infantile that the average age at which idiopathic osteosclerosis
cortical hyperostosis, elevated erythrocyte sedimentation was discovered was 36 years and women exhibited enos-
rate and serum alkaline phosphatase levels are evident. tosis twice as much as men. Mandible was most commonly
Some patients exhibit leukocytosis and anemia. Kumar affected. However, he reported the presence of enostosis
et al (2008) reported a patient with Caffey disease having in the maxilla in a few individuals. The common site of
raised immunoglobulins (IgG and IgM) and thrombocytosis. occurrence is the mandibular first molar region. Almost
10% of the enostosis occurring in the mandibular molar
region tend to resorb the roots of the mandibular first molar.
The typical radiographic appearance is usually a solitary
Radiographic evaluation plays an important role in diag- radiopacity with relatively ill-defined or diffuse border.
nosing infantile cortical hyperostosis. The long bones, clav- However, occasionally the internal structure of the sclerotic
icle and mandible are most frequently involved. These area may show variable radiopacity (Figure 11).
bones initially reveal periosteal bone formation. With Kawai et al (1996) reported a gigantic dense bone island
time, the periosteal bone density increases and integrates of the jaws. The dimensions of these large enostoses
584
measured between 2.5 and 7 cm on an orthopantomograph. Bone islands are usually ‘cold’ on bone scintigraphy.
They concluded that these gigantic dense bone islands However, one should be aware of the fact that some harm-
were simply larger versions of the dense bone islands seen less bone islands can mimic aggressive lesions on a bone
routinely. scan.
Greenspan and Stadalnik (1995) reported that the char- No treatment is required. The clinician should differen-
acteristic findings on a plain radiograph were a homoge- tiate these dense bone islands from other bone pathologies
neously dense, sclerotic focus in the cancellous bone with that may require active treatment.
distinctive radiating bony streaks (‘thorny radiation’) that
blend with the trabeculae of the host bone to create a
feathered or brush-like border (Figure 12). Gorham’s Disease
Gorham’s disease is also called Breschet–Gorham syn-
drome, Gorham’s osteolysis, Gorham’s syndrome, Gorham–
Figure 11 Stout syndrome, massive osteolysis, vanishing bone
disease and phantom bone disease.
Gilbert Breschet, Lemuel Whittington Gorham and
Arthur Purdy Stout were the first few people who described
this condition. However, JBS Jackson in 1838 published
the first case report. Subsequently Gorham and coworkers
(1954) and Gorham and Stout (1955) reported patients suf-
fering from massive osteolysis.
Gorham’s disease is a rare condition characterized by
proliferation of vascular channels that result in destruction
and resorption of the osseous matrix.
Etiopathogenesis
In this condition, the normal bone is replaced by an
aggressively expanding, non-neoplastic vascular tissue.
The aggressively proliferating neovascular tissue causes
massive bone loss. In a study by Graham and Stout, they
Cropped orthopantomograph revealing small radiopaque foci showed an association between vanishing bone disease
approximating the apices of the mandibular first premolar and and the presence of hemangiomatous or lymphangioma-
second molar. Courtesy: Department of Oral Medicine and
tous tissue. However, they were not able to demonstrate
osteoclasts in the region of bone resorption.
Figure 12
Orthopantomograph revealing enostoses in relation to the mandibular right second molar, second premolar and mandibular left
first premolar. The radiopaque foci are homogeneously dense and blend into the normal trabecular bone pattern producing a
brush like border. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
585
Many other investigators however demonstrated osteo- pattern nor an associated nephropathy. The disease is
clasts in the lytic front of the lesion. The role of osteoclasts is benign and the osteolysis usually stops after a few years.
supported by the fact that the use of calcitonin and bisphos- Type 5 Winchester syndrome with autosomal recessive
phonates (inhibitors of osteoclastic activity) to treat patients transmission. Rare childhood carpotarsal osteolysis in asso-
suffering from Gorham’s disease arrests bone resorption. ciation with contractures, shortness of stature, skin lesions,
Devlin and others (1996) proposed that interleukin-6 corneal clouding and osteoporosis without nephropathy.
(IL-6) can be a potential mediator of the massive osteolysis.
They found that the levels of cytokine IL-6 increases almost Diagnosis and investigations
by seven times when compared to the levels in a normal
patient. The typical radiographic features will help in definitive
They believe that there are multiple sources of interleu- diagnosis of Gorham’s disease. Laboratory tests are gener-
kins such as the blood vessels or dilatation of the vascular ally not diagnostic and usually the blood tests are within
marrow or the osteoclasts or cells in the bone marrow normal limits. However, the serum alkaline phosphatase
microenvironment, including monocytes, fibroblasts, and level may be slightly elevated.
lymphocytes are potential sources of IL-6. Plain radiographs, bone scans, CT and MRI can be used
to evaluate the osteolysis. The initial changes include radio-
lucent foci in the medullary portion of the bone with ill-
Clinical features defined margins. As the disease progresses these foci
Gorham’s disease is usually discovered in the first 4 decades coalesce together and involve the cortical plates and subse-
of life. However it may affect any age group and exhibits quently large portion of bone undergo dissolution or ‘disap-
no sex predilection. pear’. Orthopantomographs may reveal thinned-out
The disease commonly affects the pelvis and shoulder. cortical plates and loss of lamina dura.
Other sites that are affected are the scapula, clavicle, max-
illofacial skeleton, ribs, spine, skull and the bones of the Histologic features
extremities. Histologically, proliferation of thin walled vessels is seen.
Severe complications include paraplegia, when the spine As the disease advances, extensive osteolysis is seen.
is involved and respiratory difficulty when the thorax Subsequently the osseous tissue is replaced by fibrous tissue.
involved.
It is believed that the maxillofacial skeleton in involved Management and prognosis
in about 25 to 30% of the patients. Deformed mandible or
maxilla causes malocclusion, mobility of teeth and occa- The prognosis of massive osteolysis is relatively good
sionally results in pathological fracture. unless vital structures are involved. Generally massive
osteolysis has poor prognosis when the spine and viscera
are affected. The major cause for mortality or morbidity in
Classification of idiopathic osteolysis Gorham’s disease is the development of chylothorax (pres-
(Hardegger et al, 1985) ence of lymphatic fluid in the pleural space) due to direct
Type 1 Hereditary multicentric osteolysis with dominant extension of the dilated lymphatic vessels into the pleural
transmission. Usually seen between the age of 2 and 7 years. cavity.
Associated with spontaneous pain and swelling beginning Though various treatment modalities have been used in
in the hands and feet. Carpotarsal osteolysis occurs over the management of massive osteolysis, no single treatment
the period of a few years. The progression ceases normally modality has proven to be effective in controlling the dis-
in adolescence. ease process.
Various non-surgical methods have been tried such as radi-
Type 2 Hereditary multicentric osteolysis with recessive
ation therapy (40–45 Gy in 2 Gy fractions), anti-osteoclastic
transmission similar to type 1, but may be associated with
drugs (bisphosphonates) and alpha-2b interferon.
severe generalized osteoporosis.
Surgical management includes resection of the lesion
Type 3 Non-hereditary multicentric osteolysis with followed by reconstruction using bone grafts. Surgical defects
nephropathy. It appears in childhood. There is a gradual can also be cosmetically masked with prostheses.
disappearance of the carpus with the tarsal bones involved,
but to a less degree. Proteinuria is seen. Death occurs usu-
ally due to renal failure and malignant hypertension. Paget’s Disease of Bone
Type 4 Gorham’s massive osteolysis (Gorham–Stout syn- Paget’s disease was first described by Sir James Paget in
drome). Monocentric occurrence in any part of the skele- 1877. He called this condition osteitis deformans as he
ton may start at any age. Normally ‘hemangiomatous tissue’ believed it was an inflammatory bone condition. Rhodes
is found in the osteolytic region. It has neither a hereditary and Jawad suggested that as the pathology is now known
586
to be a disorder in bone turnover and not an inflammatory (almost around 100 nuclei compared to five to ten in nor-
condition, the term ‘osteodystrophia deformans’ is more mal osteoclasts).
appropriate needs wider recognition. In the intermediate phase, as the bone is rapidly resorbed,
Paget’s disease of bone is characterized by excessive there is an increased amount of bone formation with numer-
osteoclastic bone resorption in a focal area that is accom- ous osteoblasts which are morphologically normal. The new
panied by an increased osteoblastic bone deposition. This bone formed is abnormal with the collagen fibers being
abnormal resorption and deposition of bone results in laid down in a haphazard manner.
weakened and distorted architecture of bone. In the sclerotic phase bone formation occurs at a rapid
pace. The bone thus formed is disorganized (woven bone)
Etiology and pathophysiology which is structurally weak. Fibrous connective tissue and
blood vessels invade the woven bone leading to a state
The etiology of Paget’s disease is still unknown. Some of
of hypervascularity of the bone. As time progresses there
the well accepted causes include viral infection and genetic
is diminished hypercellularity resulting in the formation
predisposition.
of a pagetic bone. This state is referred to as burnt-out
Helfrich and Hockling (2008) describe that the disease
Paget disease.
has a genetic basis and mutations in SQSTM1 gene have
been associated with familial and sporadic disease in up to
40% of cases. SQSTM1 gene encodes sequestosome 1, Clinical features
also known as p62, which is an ubiquitin-binding protein
Paget’s disease of bone affects individuals over the 4th decade
that is involved in the IL-1, TNF and RANKL signaling
of life. The incidence increases with the advancing age. It is
pathways.
believed that males are twice as more likely to be affected
In Paget’s disease, osteoclast precursors have also been
than females. It is common in Europe, North America,
shown to be hyperresponsive to the RANK ligand (RANKL),
Australia and New Zealand and almost always affects whites.
a member of the tumor necrosis factor-alpha superfamily,
Paget’s disease is rare among Asians and Africans.
which promotes osteoclast genesis. One possibility is that
A series of articles by Joshi et al (2006), Anjali et al
increased expression of RANKL contributes to the localized
(2006), Bhadada et al (2006) studied the prevalence and
nature of the disease.
presentation of Paget’s disease in various parts of India.
Macrophage-colony stimulating factor (M-CSF) may play
They reported the mean age at which the condition was
a role in Paget’s disease. M-CSF is a growth factor pro-
diagnosed was in the 5th decade. The male-to-female ratio
duced by many cells, including osteoblasts and marrow
of occurrence was 2.5:1.
fibroblasts. Significantly high levels of M-CSF have been
Paget’s disease follows a chronic course. It is estimated
found in patients with untreated Paget’s disease.
that about 80% of the patients are asymptomatic and the
Several genetic theories also suggest that human leuko-
disease is recognized on routine radiographic examinations.
cyte antigen (HLA) on chromosome 6 and the gene on
Though the disease affects multiple bones, almost 17%
chromosome arm 18q may play important roles in Paget’s
are unifocal (monostotic). The common bones that are
disease.
involved are the bones of the axial skeleton (spine, pelvis,
The other popular hypothesis for the etiology of Paget’s
sacrum, femur and the skull).
disease is the action of slow virus (slow viruses are those
The common signs and symptoms are bone pain,
that produce diseases with extended or prolonged incubation
deformed bones, neurological deficits secondary to com-
periods).
pression of the neural tissues. Other patients present with
The measles virus messenger RNA sequences have been
a range of symptoms that may include pathologic frac-
found in osteoclasts and other mononuclear cells of pag-
tures, congestive heart failure, hearing loss (compression
etic bones. Canine distemper virus nucleocapsid antigens
of eighth cranial nerve), and dysesthesias and weakness
have also been found in osteoclasts from patients with
from nerve root compression.
Paget’s disease. According to this hypothesis, bone marrow
Patients present with bowed legs and associated abnor-
cells (the progenitors of osteoclasts) are infected by a virus
malities in the gait. Some authors describe the gait as
that causes an abnormal increase in osteoclast formation.
waddling or simian gait. Spinal deformities such as spinal
stenosis or kyphosis are seen.
Progression of disease
Some individuals exhibit increased size of the skull with
The progression of Paget’s disease passes through three or without frontal bossing and enlargement of the middle
phases, namely, the lytic phase, mixed or intermediate phase third of the face, mimicking the face of a lion (leontiasis
and the sclerotic phase. ossea). Patients generally find the need to change hats to
In the lytic phase, there is increased bone resorption. accommodate the growing size of the head.
There is increased number of osteoclasts at the site of Pathologic fractures are common in Paget’s disease.
resorption. These osteoclasts have typically multiple nuclei However, the healing is normal.
587
Oral manifestations On radionuclide imaging, the radiopharmaceutical is

markedly taken up by the pagetic bone. Extensive involve-
The maxilla and mandible can be affected. It is estimated
ment of the mandible may occasionally reveal the uptake
that the maxilla is almost twice as commonly affected than
of the radiopharmaceutical from condyle to condyle giving
the mandible. The involved jaw will be enlarged. Enlarged
rise to the Lincoln’s sign or the ‘black beard’ sign.
maxilla will cause widening of the alveolar ridge and flat-
tening of the palatal vault. Spacing between teeth may be
a secondary complication. Histologic features
In advanced cases, the lips fail to cover the enlarging
The histologic features depend on the phase of the disease
jaws resulting in an open mouth state.
process. The pathognomonic feature in Paget’s disease is the
presence of the basophilic resting and reversal lines which
Laboratory investigations
are indicative of the junction between the resorptive and
Patients with Paget’s disease may demonstrate very high formative phases of bone that results in the appearance
levels of serum alkaline phosphatase (helps to estimate of mosaic pattern or jigsaw puzzle pattern of bone. The
bone formation), especially when multiple bones are affected. bony trabeculae will reveal prominent osteoblastic and
It is believed that the total alkaline phosphatase levels osteoclastic activity.
have only 78% sensitivity of detecting Paget’s disease.
Therefore, bone specific alkaline phosphatase levels are Dental considerations
more accurate. During the active phases of the disease process, invasive
However, serum calcium and serum phosphorus levels are oral surgical procedures may result in extensive bleeding
normal. Other investigations that can help in the identifi- due the vascularity of the diseased bone. Hypercementosis
cation of the extent and severity of bone turnover include of teeth may make dental extractions difficult. Patients on
estimating levels of deoxypyridinoline and N-telopeptide treatment with bisphosphonates are more likely to develop
of type I collagen (help in estimating amount of bone chemo-osteonecrosis even with minimal trauma. Dental
resorption). Urinary excretion of deoxypyridinoline and extractions should be best avoided during the treatment.
N-telopeptide are elevated. Alpha-alpha type I C-telopeptide Similarly, in the advanced phases of the disease, the bone
fragments are sensitive markers of bone resorption for assess- is susceptible to infections and pathologic fracture.
ing disease activity and monitoring treatment outcome. Patients may require frequent replacement of oral
prosthesis to accommodate for the ever increasing size of
Radiographic features the jaws.
The radiographic findings in Paget’s disease depend on the

Management
phase of the disease. Radiographs may exhibit areas of
osteolysis and areas of bone deposition or an intermediate In symptomatic patients, bone pain can be managed with
phase. NSAIDs. The drugs routinely used are calcitonin (osteoclast
There are very unique radiographic features of Paget’s inhibitor) and bisphosphonates (anti-resorptive agents).
disease such as osteoporosis circumscripta, blade of These drugs minimize the bone turnover rate.
grass lesion, brim sign, framed vertebrae and cotton-wool New generation bisphosphonates such as pamidronate,
appearance. etidronate, tiludronate, alendronate and risedronate are
Osteolytic zones in the skull represented by large cir- preferred over calcitonin.
cumscribed radiolucent areas are referred to as osteoporo- The recommended dosages of these drugs are etidro-
sis circumscripta (usually seen in the frontal and occipital nate (400 mg/day for 6 months), pamidronate (single IV
bones). In the intermediate phase, as bone deposition occurs, infusion of 60 mg to a maximum of 90 mg per day for
areas of patchy sclerosis are seen in the skull and the jaws, 3–4 days diluted in saline or glucose solution for 46 hours),
giving rise to the cotton-wool appearance. Hypercementosis alendronate (given orally 20–40 mg/day for 6 months),
and loss of lamina dura around the roots of teeth is usu- tiludronate (400 mg/day for 3 months) and risedronate
ally seen in Paget’s disease. Rarely, root resorption may (30 mg/day for 2 months). All patients treated with
be seen. bisphosphonates should be given calcium and vitamin D
Long bones of the skeleton reveal a V-shaped pattern supplements.
that divides the healthy bone from its pagetic counterpart. Keating and Scott (2007) described the efficacy of
This unique finding is termed ‘blade of grass’ lesion. Thick- zoledronic acid, a third generation, nitrogen containing
ening of the iliopectineal line in the pelvic bone is termed bisphosphonate in the management of Paget’s disease.
‘brim sign’. Enlargement of the vertebral bodies with thick- They describe that a single intravenous dose of zoledronic
ened cortices and vertical striations gives rise to the appear- acid 5 mg is effective and well tolerated in the treatment
ance of ‘framed vertebrae’. of Paget’s disease of bone. A single IV dose of zoledronic
588
acid was associated with a significantly higher thera- Surgical management may be required mainly for decom-
peutic response rate and a more rapid reduction in bone pression of the nerves.
turnover than that achieved with 60 days of oral rise-
dronic acid. Moreover, biochemical remission was sus-
Prognosis
tained after 24 months of follow-up in zoledronic acid
recipients. It is believed that about 1% of the patients develop osteo-
Cytotoxic drug such as mithramycin (plicamycin) has sarcoma as a complication of Paget’s disease. Benign and
also been tried where the disease is refractory to bisphos- malignant giant cell tumors have also known to occur in
phonates. However, it is seldom used these days. these patients.
589
CHAPTER
20 Autoimmune Disorders
Balaji Rao B, Sumanth KN
➧ Concepts of Immunity and Autoimmunity Pemphigus Foliaceous

Sjögren’s Syndrome Brazilian Pemphigus
Mikulicz’s Disease (Benign Lymphoepithelial Lesion) Pemphigus Erythematosus
Aphthous Stomatitis (Aphthous Ulcers, Canker Sores, Paraneoplastic Pemphigus
Recurrent Aphthous Stomatitis) Bullous Pemphigoid
Giant Cell Arteritis Cicatricial Pemphigoid
Periodontal Disease ➧ Epidermolysis Bullosa Acquisita
Rheumatoid Arthritis ➧ Systemic Lupus Erythematosus
➧ Autoimmune Polyendocrinopathy–
Systemic Autoimmune Diseases with
Orofacial Manifestations Candidiasis-Ectodermal Dystrophy
➧ Diabetes Mellitus Type I (IDDM)
➧ Pemphigus
Pemphigus Vulgaris ➧ Systemic Sclerosis
Pemphigus Vegetans ➧ Myasthenia Gravis
CONCEPTS OF IMMUNITY AND of acquired immunity is achieved through the formation of

AUTOIMMUNITY large umbers of activated lymphocytes that are specifically
designed to destroy the foreign agent. This type of immu-
The human body has the ability to resist almost all types nity is called cell mediated immunity or T-cell immunity
of organisms or toxins that tend to damage the tissues and (because the activated lymphocytes are T-lymphocytes).
organs. This capacity is termed immunity. A specific type of
immunity that arises from general processes, rather than
Autoimmunity
from specific disease organisms is called innate immunity.
In addition, the human body has the capability to develop Failure of the immune system to tolerate (antigen-specific
extremely powerful specific immunity against individual immunological unresponsiveness) self-tissues. It is a con-
invading agents such as lethal bacteria, viruses, toxins, etc. dition in which structural or functional damage is caused by
This is called acquired immunity. Acquired immunity is the action of immunologically competent cells or antibodies
caused by special immune system that forms antibodies against normal components of the body.
and activated lymphocytes that attack and destroy specific When the concept of autoimmunity came to be accepted
organisms or toxins. as a pathogenic mechanism, a large number of diseases
Two basic forms of acquired immunity occur in the body. were suggested to have an autoimmune etiology, based
In one of them the body develops circulating antibodies, on the finding of autoantibodies in the patients. However,
which are globulin molecules in the blood that are capable autoantibodies have also been found in the serum or tis-
of attacking the invading agent. This type of immunity is sues of otherwise normal and healthy elderly individuals.
called humoral immunity or B-cell immunity (because Autoantibodies are also formed following tissue injury and
B-lymphocytes produce the antibodies). The second type may serve a role in the removal of the products of tissue
590
Chapter 20 – Autoimmune Disorders
breakdown. This lead to the need to formulate criteria for orofacial region. Sumanth and Balaji Rao have proposed a
diagnosing autoimmune diseases. simpler classification of autoimmune diseases that affect
the orofacial region.
Diagnostic criteria for autoimmune disorders 1. Autoimmune disorders affecting orofacial region pre-
The first criterion for recognizing autoimmune disease was dominantly
given by Witebsky in 1957. This criterion was extended by a. Sjögren’s syndrome (Gougerot–Sjögren syndrome)
many authors. Robert H Waldman documented the follow- b. Benign lymphoepithelial lesion (Mikulicz’s disease)
ing criteria: c. Aphthous stomatitis
d. Periodontal disease
1. a. Circulating or localized populations of cells sensi- e. Giant cell arteritis (temporal arteritis)
tized to antigen, or products of immunologically 2. Systemic autoimmune disorders with orofacial mani-
activated cells, active under physiologically con- festations
ditions should be demonstrated, or a. Pemphigus
b. Circulating or localized populations of cells sensi- b. Bullous pemphigoid
tized to antigen, or products of immunologically c. Cicatricial pemphigoid
activated cells, active under physiological condi- d. Epidermolysis bullosa acquisita
tions, should be present e. SLE
2. Sensitizing antigen should be identified and f. Bullous SLE
characterized g. Autoimmune polyendocrinopathy candidiasis ecto-
3. Antibodies or sensitized lymphocytes should be pro- dermal dystrophy (APECED)
duced in vitro or in animals against the same antigen h. Myasthenia gravis
4. Pathological events in vitro or in the experimental i. Dermatomyositis
animal should correspond to those in human disease j. Systemic sclerosis
5. Transfer of disease from immunized to normal animal k. Idiopathic thrombocytopenic purpura.
by cells or serum.
Classification of autoimmune diseases Sjögren’s Syndrome

(Gougerot–Sjögren Syndrome)
Fergusson (1995) categorized autoimmune diseases as
organ specific and non-organ specific. Described in Chapter 11 (Diseases of Salivary Glands) on
page 265.
1. Organ specific autoimmune diseases
a. Hashimoto’s thyroiditis
b. Primary myxedema
Mikulicz’s Disease
c. Thyrotoxicosis
(Benign Lymphoepithelial Lesion)
d. Pernicious anemia
e. Autoimmune atrophic gastritis Described in Chapter 11 (Diseases of Salivary Glands) on
f. Autoimmune Addison’s disease page 265.
g. Type I diabetes mellitus
h. Goodpasture’s syndrome
i. Myasthenia gravis Aphthous Stomatitis
j. Sympathetic ophthalmia (Aphthous Ulcers, Canker Sores, Recurrent
k. Autoimmune hemolytic anemia Aphthous Stomatitis)
l. Idiopathic thrombocytopenic purpura
m. Primary biliary cirrhosis Described in Chapter 7 (Vesiculobullous Disorders) on
n. Chronic active hepatitis page 174.
o. Sjögren’s syndrome
2. Non-organ specific autoimmune diseases
a. Rheumatoid arthritis Giant Cell Arteritis
b. Dermatomyositis
Giant cell arteritis is a relatively common form of large
c. Systemic sclerosis
vessel vasculitis occurring predominantly in elderly indi-
d. Systemic lupus erythematosus (SLE).
viduals. The mean age of onset is 70 years with a 2:1
Various classifications in literature do not necessarily female-to-male ratio. Temporal artery is the common
group autoimmune diseases that specifically affect the artery that is involved in cranial arteritis (Figure 1).
591
inflamed gingival tissue, stimulated pooled saliva and

Figure 1
serum of patients suffering from chronic moderate peri-
odontitis. They correlated the presence of rheumatoid
factors to the periodontal disease and concluded an auto-
immune origin of the disease.
Ftis et al (1986) found increased levels of antibodies to
type I collagen in patients with periodontal disease than in
control subjects.
Hirsch et al (1988) used enzyme-linked immuno spot
test to enumerate antibody secreting cells to human col-
lagen types I–IV by cells isolated from gingival and
peripheral blood of individuals suffering from adult peri-
odontitis. Analysis revealed the presence of high numbers
of cells that secreted antibodies to type I collagen when
compared to type III. They suggested that autoimmunity
may contribute to the pathogenesis of this common
disease.
Anusaksathien and Dolby (1991) postulated many pos-
The prominent temporal artery in a patient suffering sible explanations to explain the presence of autoantibod-
from temporal arteritis. Courtesy: Dr C Stephen Foster, ies in periodontal disease.
The Ocular Immunology and Uveitis Foundation,
1. Enhanced presentation of self-antigens through increased
Cambridge, Massachusetts, USA
expression of the molecule associated with antigen
presentation, namely, Ia antigen.
2. Altered T helper or T suppressor cell function.
Muki proposed an autoimmune basis for giant cell arte- 3. Polyclonal activation of cells which have the ability,
ritis because it occurred more frequently in patients with for reasons which may not be clear, to produce auto-
other autoimmune disorders such as thyroid disease or antibodies.
rheumatoid arthritis. 4. Idiosyncrasies of the antigen-idiotype network.
5. Bacterial or viral cross-reactivity with self-antigen
Clinical features leading to the production of cross-reactive antibodies
❍ The temporal artery is extremely tender on palpation. 6. Genetic predisposing factors.
❍ The individual may complain of deep aching or throb-
bing pain and burning sensation over the muscles.
❍ The pain may radiate to the maxilla, mandible, neck or Rheumatoid Arthritis
face. Rheumatoid arthritis (RA) is a chronic systemic inflamma-
tory disorder that may affect many tissues and organs—
Diagnosis skin, blood vessels, heart, lungs and muscles—but principally
The definitive diagnosis is based on temporal artery biopsy, attacks joints, producing a non-suppurative proliferative
which demonstrates giant cell arteritis and elevated eryth- synovitis that often progresses to destruction of the articu-
rocyte sedimentation rate. lar cartilage and ankylosis of joints. Although the cause
for RA remains unknown, there is convincing evidence
Management that autoimmunity plays a pivotal role in its chronicity
and progression, likening this condition to other so-called
The condition once recognized should be treated aggressively connective tissue diseases. RA can affect the TMJ.
with corticosteroids. Approximately 50% of the patients who
have not been treated in time may develop blindness. Pathogenesis
It is currently believed that RA is triggered by the expo-
Periodontal Disease sure of an immunogenetically susceptible host to an arth-
ritogenic microbial antigen.
For almost two decades the concept of autoimmune patho-
The involved mechanisms in the causation could be:
genesis for periodontal disease was considered.
Alphonse et al (1981) have detected rheumatoid factor 1. Genetic susceptibility: Rosenberg and Nuki have
by latex slide agglutination in subgingival plaque, shown that 65–80% of the individuals suffering from
592
RA have human leukocyte antigen (HLA)-DR4 and 2. Unusually affected joints—carpometacarpal joints,
HLA-DR1 or both. Other haplotypes like DW4, DW10, cricoarytenoid joint, sacroiliac joints and sternocla-
DW13, DW14 and DW15 have also been implicated. vicular joint.
2. Primary exogenous arthritogen: Roudier et al pro- 3. Rarely affected joints—distal interphalangeal joints,
posed Epstein–Barr virus as the primary microbial lumbar spine.
agent for the causation of RA. Other microbial agents
like retroviruses, parvoviruses, mycobacteria, borrelia The changes of RA most useful for diagnosis are noted in
and mycoplasma could also initiate the disease. the hands and wrists. They include swan neck deformity,
3. Autoimmune reaction: Warder in 1940 proposed ulnar deviation, dorsal interosseous muscle atrophy and
the presence of rheumatoid factor, which was the swelling of wrist.
first human autoantibody described in association Temporomandibular joint manifestations It is believed
with RA. that TMJ symptoms develop in more than one-third of the
Although T cells played a primary role in the patients during the first year of RA.
pathogenesis of RA, B cells were also involved. In According to Tegellerg and Kopp (1987) and Votalia
1993, Panayi reported that approximately 80% of (1964), the most characteristic clinical signs of RA affect-
patients have rheumatoid factors which were directed ing the TMJ were palpatory tenderness of the joint, crepi-
against Fc portion of IgG, present in serum and syno- tus from the joint and swelling in relation to the TMJ
vial fluid. The presence of rheumatoid factors in the region.
joints is believed to contribute to the inflammatory Almost 60% of the individuals present with bilateral
reaction. Williams in 1996 observed that rheumatoid involvement of joints.
factors showed principal specificity for structures on Severe RA involving the TMJ can result in a progres-
C3 and C2 (Fc) domains of IgG, which thus defined sive anterior open bite due to bilateral destruction of man-
rheumatoid factor as an autoantibody associated dibular condyles.
with RA. Hugh Ogus described a possible course of rheumatoid
4. Mediators of tissue damage: Panaji in 1993 reported involvement of the TMJ. He proposed that the TMJ
that the rheumatoid synovium was heavily infiltrated involvement progresses through three phases:
with lymphocytes, most of which were CD4 helper
T cells. They generate cytokines, which in turn could 1. Active phase (acute symptoms, synovial effusion,
activate other immune cells and macrophages. destruction of fibrocartilage and erosion of underly-
ing bone)
Clinical features 2. Healing phase (remodeled and flattened articular
surface)
❍ It can occur at any age, but bimodal peaks are seen at 3. Secondary osteoarthrotic phase (marginal proliferations
40 and 60 years of age. and further destruction of the condyle, predominant
❍ Women are two-and-half times more likely than men clinical finding of crepitus).
to develop disease.
❍ Patients complain of polyarthralgias, fatigue and mus-
cle aches. Radiographic features of rheumatoid arthritis
❍ Prolonged morning stiffness of joints. affecting the TMJ
❍ Joints of the hands, feet, elbows, shoulders, knees and 1. Formation of new bone (marginal proliferation) was
TMJ are affected. described in 1975 by Hugh Ogus. It is reported that
❍ Within weeks of affliction, the involved joints become the erosions of the articular surface were the most
tender and marked swelling is seen. important diagnostic finding of RA in other joints and
❍ Low-grade fever, malaise, anorexia and weight loss is not the TMJ.
seen. 2. Subcondylar cystic destruction and severe destruction
of bone eventually lead to complete loss of condyle.
Joint manifestations Such destruction will give the remaining condylar bone
the shape of the mouth piece of a flute.
Katz classified the joint manifestations as:
3. Anterior open bite and reduced joint space (due to
1. Joints that are usually affected—ankles, cervical spine, destruction of articular cartilage).
elbows, hips, knees, metacarpophalangeal joints, meta- 4. The most characteristic radiographic signs of RA in
tarsophalangeal joints, proximal interphalangeal joints, the TMJ are erosions of cortical outline and reduced
shoulders, tarsal joints, temporomandibular joints and joint space (Figure 2) as proposed by Akerman et al
wrists. in 1988.
593
Figure 2
Erosions of the cortical outline of the condyle and reduced joint space. Courtesy: Department of
Kopps in 1995 stated that the radiographic diagnosis of F. Pulmonary

RA in TMJ was difficult. However, absence of radiographic
1. Pleural effusions
changes in the TMJ will not exclude the possibility of
2. Fibrosing alveolitis
early RA in TMJ.
3. Bronchiolitis
4. Caplan’s syndrome
Extra-articular manifestations of rheumatoid
disease G. Cardiac
A. Systemic manifestations 1. Pericarditis
1. Fever 2. Myocarditis
2. Weight loss 3. Endocarditis
3. Fatigue 4. Coronary vasculitis
4. Increased susceptibility to infections 5. Granulomatous aortitis
B. Musculoskeletal manifestations H. Ocular

1. Osteoporosis 1. Episcleritis
2. Bursitis 2. Scleritis
3. Muscle wasting 3. Scleromalacia
4. Keratoconjunctivitis sicca
C. Hematological manifestations
1. Anemia I. Amyloidosis.
2. Thrombocytosis
3. Eosinophilia Laboratory findings
D. Vasculitis ❍ None of the multiple blood, synovial fluid or biopsy
1. Visceral arteritis studies is specific for RA.
2. Pyoderma gangrenosum ❍ These individuals exhibit increased ESR levels and
3. Digital arteritis anemia.
❍ 50% of the patients show presence of antinuclear anti-
E. Neurological body (ANA).
1. Cervical cord compressions ❍ 80% of patients exhibit significant elevations of rheu-
2. Compression neuropathies matoid factor (not diagnostic).
3. Peripheral neuropathies ❍ Rheumatoid synovial fluid is generally cloudy and
4. Mononeuritis multiplex tinged green.
594
Diagnosis surrounded by a palisade of proliferated macrophages,

and peripheral fibrosis and chronic inflammatory cell
The diagnosis of RA is based on a carefully obtained his-
infiltration, predominantly perivascular.
tory and a diligent physical examination to unravel subtle
signs of inflammation. One should strive to detect the con- B. Definite rheumatoid arthritis This diagnosis requires
dition as early as possible to prevent crippling deformities. five of the above criteria. In criteria 1 through 5, the joint
The American Rheumatism Association diagnostic cri- signs or symptoms must be continuous for at least 6 weeks.
teria aid in clinical diagnosis. These criteria were designed
C. Probably rheumatoid arthritis This diagnosis requires
principally for research and literary purposes.
three of the above criteria and total duration of joint
A. Classical rheumatoid arthritis This category requires symptoms must be at least 3 weeks.
seven of the following criteria to match. In criteria 1 through D. Possible rheumatoid arthritis This diagnosis requires
5, the joint signs or symptoms must be continuous for at two of the following criteria and total duration of joint
least 6 weeks. symptoms must be at least 3 weeks:
1. Morning stiffness. 1. Morning stiffness
2. Pain on motion or tenderness in at least one joint 2. Tenderness or pain on motion (observed by a physician)
(observed by the physician). with history of recurrence or persistence for 3 weeks
3. Swelling (soft tissue thickening or fluid, not bony 3. History or observation of joint swelling
overgrowth alone) in at least one joint (observed by a 4. Subcutaneous nodules (observed by a physician)
physician). 5. Elevated erythrocyte sedimentation rate or C-reactive
4. Swelling (observed by a physician) of at least one protein
additional joint (symptom free period between the two 6. Iritis (of dubious value as a criterion except in the
joint involvements may not be more than 3 months). case of juvenile RA).
5. Symmetrical joint swelling (observed by a physician)
with simultaneous involvement of the same joint on Diagnostic criteria for local involvement of TMJ
both sides of the body (bilateral involvement of prox-
imal interphalangeal, metacarpophalangeal or meta- The criteria for diagnosing TMJ involvement with RA were
tarsophalangeal joints is acceptable without absolute given by Kopp in 1995.
symmetry). Involvement of the terminal phalangeal 1. Bilateral joint involvement
joint will also satisfy this criterion. 2. Tenderness to adaptation of lateral or posterior aspects
6. Subcutaneous nodules (observed by a physician) over of the joint
bony prominences, on extensor surfaces or in juxta- 3. Joint crepitus
articular regions. 4. Radiographic signs of erosions of the articular cortical
7. Radiographic changes typical of RA (which must bone of the TMJ
include at least bony decalcification localized to or 5. Swelling over the TMJ (acute phase)
most marked adjacent to the involved joints and not 6. Temperature change in or over the TMJ. Increase in
just degenerative changes). Degenerative changes do the acute phase and decrease in the chronic phase
not exclude patients from any group classified as RA. 7. Anterior open bite
8. Positive agglutination test—demonstration of the 8. Abundance of polymorphonuclear leukocytes in the
‘rheumatoid factor’ by any method which, in two joint fluid (acute phase) or mononuclear leukocytes
laboratories, has been positive in not over 5% of nor- (lymphocytes and plasma cells, chronic phase)
mal controls—or positive streptococcal agglutination
test (the latter is now obsolete). The presence of four of those eight criteria should be able
9. Poor mucin precipitates from synovial fluid (with shreds to determine the local diagnosis.
and cloudy solution).
10. Characteristic histologic changes in synovium with three Differential diagnosis
or more of the following marked villous hypertrophy, Differential diagnoses include ankylosing spondylitis,
proliferation of superficial synovial cells, often with pal- psoriatic arthritis, Reiter’s syndrome, arthritis of chronic
isading, marked infiltration of chronic inflammatory inflammatory bowel disease, polytendinitis, osteoarthritis
cells (lymphocytes or plasma cells predominating) and sarcoidosis.
with tendency to form ‘lymphoid nodules’, deposition
of compact fibrin either on surface or interstitially,
Management
foci of necrosis.
11. Characteristic histologic changes in nodules show The successful management of RA lies in a comprehensive
granulomatous foci with central zones of necrosis, multidisciplinary approach. Systemically administered
595
drugs, local injections of corticosteroids, physical therapy, foliaceous. Oral mucosal involvement is of little consequence
occupational therapy, psychosocial support, patient edu- in the foliaceous and erythematosus forms.
cation, family involvement, surgical intervention and
vocational rehabilitation are all part of the routine daily
management program of patients with RA. Pemphigus Vulgaris
1. Non-steroidal anti-inflammatory drugs Piroxicam Described in Chapter 7 (Vesiculobullous Disorders) on
20 mg once a day, diclofenac 25–75 mg twice a day, page 174.
naproxen 250–500 mg twice a day, ibuprofen 400–
800 mg four times a day or ketoprofen 50–75 mg thrice
Pemphigus Vegetans
a day.
2. Remittive drugs used in the treatment of rheumatoid Described in Chapter 7 (Vesiculobullous Disorders) on
arthritis page 174.
– Antimalarials (chloroquine 250 mg, hydroxychloro-
quine 200 mg)—one tablet twice a day followed by
once a day. Pemphigus Foliaceous
– Gold parenterally (sodium aurothiomalate or urothio- Described in Chapter 7 (Vesiculobullous Disorders) on
glucose)—test dose of 10 mg followed by 25–50 mg page 174.
weekly for 4–6 months.
– Gold given orally (auranofin 3 mg)—one tablet twice
a day Brazilian Pemphigus (Fogo Selvagem)
– Methotrexate given orally (2.5 mg)—one tablet thrice
Described in Chapter 7 (Vesiculobullous Disorders) on
a day, once or twice in a week
page 174.
– Penicillamine (125–250 mg)—initial dose of 250 mg
once a day, raised by 125 mg increments, every 6–8
weeks as tolerated if active arthritis persists, maximal Pemphigus Erythematosus
daily dose 1,500 mg. (Senear–Usher Syndrome)
Physical and occupational therapy, surgery, occlusal splints, Described in Chapter 7 (Vesiculobullous Disorders) on
occlusal adjustments, prosthetic treatment can increase page 174.
the patient comfort and help in mastication.
Paraneoplastic Pemphigus
SYSTEMIC AUTOIMMUNE DISEASES WITH Described in Chapter 7 (Vesiculobullous Disorders) on

OROFACIAL MANIFESTATIONS page 174.
PEMPHIGUS Bullous Pemphigoid

Described in Chapter 7 (Vesiculobullous Disorders) on
It is an autoimmune disease involving the skin and the
page 174.
mucosa and characterized by intraepidermal bullae forma-
tion. It is caused by antibodies directed as the intercellular
intercementing substances. Cicatricial Pemphigoid
Classification Described in Chapter 7 (Vesiculobullous Disorders) on

page 174.
❍ Pemphigus vulgaris
❍ Pemphigus vegetans
❍ Pemphigus foliaceous EPIDERMOLYSIS BULLOSA ACQUISITA
❍ Pemphigus erythematosus (Senear–Usher syndrome)
❍ Brazilian pemphigus (Fogo selvagem)
Epidermolysis bullosa acquisita (EBA) is a chronic subepi-
❍ Benign familial chronic pemphigus (Hailey–Hailey
dermal blistering disease associated with autoimmunity to
disease)
the collagen within the anchoring fibrils (type VII colla-
❍ Paraneoplastic pemphigus.
gen) that are located at the dermal–epidermal junction.
Pemphigus vegetans is a variant of pemphigus vulgaris The first diagnostic criterion was putforth by Raenigk
and pemphigus erythematosus is a variant of pemphigus et al in 1971.
596
characteristically seen within the flexural areas and skin

Figure 3
folds. Some patients may present with erythema and flat
urticarial plaques in the absence of blisters.
About 10% of the patients exhibit severe mucous mem-
brane involvement may present a picture clinically similar
to cicatricial pemphigoid with erosions and scarring in
the oral cavity, conjunctiva, upper esophagus and anus or
vagina.
Diagnostic criterion for EBA was proposed by Yaoita
et al (1981). This is still the most widely followed diagnostic
criterion. These criteria, with some updated modifications
are as follows:
1. Bullous disorder with the clinical spectra outlined above
2. No family history of a bullous disorder
3. A subepidermal blister by histologic examination
4. Deposition of IgG deposits within the dermal-epider-
mal junction (a positive direct immunofluorescence of
perilesional skin)
5. Junctional IgG deposits localized to the lower lamina
densa and/or sublamina densa zones of the basement
membrane when examined by direct immunoelectron
Extensive erosions over the lower extremity and some
microscopy or indirect or direct salt split skin immu-
areas of healing with scar formation in epidermolysis nofluorescence and/or western blotting.
bullosa. Courtesy: Dr Ajit Auluck
Histologic features
Routine histologic examination of lesion on skin obtained
1. A negative family and personal history for a previous from EBA patients show a subepidermal blister and a clean
blistering disorder separation between the dermis and the epidermis with
2. An adult onset of eruption inflammatory infiltrate.
3. Spontaneous or trauma-induced blisters that resemble
those of hereditary dystrophic epidermolysis bullosa Immunologic parameters
4. The exclusion of all other bullous diseases.
Patients with EBA have IgG deposits within dermal–
Clinical features epidermal junction of their skin. This is detected by direct
immunofluorescence. IgG is the predominant immunoglob-
Epidermolyis bullosa acquisita may have at least three dif- ulin class, but deposits of complement IgA, IgM, factor B
ferent clinical presentations: the ‘classical presentation’, and properdin may also be detected.
‘bullous pemphigoid like presentation’ and a ‘cicatricial The localization of immune deposits within the dermal–
pemphigoid like presentation’. epidermal junction of the skin of epidermal junction of the
The classical presentation is a non-inflammatory, bul- skin of the patients is the ‘gold standard’ for the diagnosis.
lous disease with an acral distribution that heals with
scarring and milia formation. It is a mechanobullous dis-
ease marked by skin fragility. These patients have ero- SYSTEMIC LUPUS ERYTHEMATOSUS
sions, blisters and scars over trauma prone surfaces (back
of the hands, knuckles, elbows, knees, sacral area and toes)
Systemic lupus erythematosus (SLE) is a systemic disease
(Figure 3).
affecting multiple organ systems that is characterized by
A scarring alopecia and some degree of nail dystrophy
the presence of antibodies to nuclear antigens. Lupus is a
may be seen. The lesions heal with scarring and frequently
Latin word for ‘wolf’. It is called so because of the way it
with the formation of pearl like, milia cysts within the
eats away skin.
scarred areas.
Types of lupus:
The bullous pemphigoid like presentation is a wide-
spread inflammatory vesiculobullous eruption involving 1. SLE
the trunk, central body, skin folds and the extremities. The 2. Discoid lupus erythematosus
bullous lesions are surrounded by inflamed or urticarial 3. Drug-induced lupus erythematosus
skin. The distribution of lesions like bullous pemphigoid is 4. Lupus nephritis
597
5. Subacute cutaneous lupus erythematosus

Figure 4
6. Neonatal lupus erythematosus.
Neonatal lupus erythematosus is a rare disease affecting
babies born to women suffering from SLE. Subacute cuta-
neous form is characterized by non-scarring skin lesions
on the areas of skin exposed to sunlight.
Epidemiology
Systemic lupus erythematosus is the most common connec-
tive tissue disease. The reported prevalence of SLE is 50.8 per
100,000 individuals above 17 years of age. The prevalence
of SLE in individuals in the age group of 18–65 years is
about 1:1,000 in white women and 1:250 in black women.
In this age group, SLE is 10 times more frequent in females
than in males. Among children and elderly, SLE is only
twice as frequent in females than in males.
Etiology and pathogenesis

Genetic About 10% of the patients with SLE have a first
degree relative with the disease. Multiple HLA associations
have been reported, particularly for B8, DR2, DRW52,
DQW1 and DQW2. The null gene for C4 occurs more often Maculopapular rash on the face and chest of a patient
in patients with SLE in their families. suffering from SLE. Courtesy: Dr Ashok
Environmental A significant exposure to sunlight

clearly precede the onset of the disease in about one-third
2. Cutaneous lesions Patients present with a typical ery-
of the patients.
thematous rash over the malar region, which is popu-
Medication Drugs that have been implicated in causing larly referred to as the butterfly rash. It is evident as
SLE are categorized into: an erythematous and slightly edematous lesion located
❍ High risk drugs (hydralazine, procainamide and on the cheeks, extending across the bridge of the nose
isoniazid) (Figure 4). It is generally the first manifestation of the
❍ Moderate risk to low risk drugs (quinidine, D- disease. It frequently occurs after exposure to sun. The
penicillamine, carbamazepine, oral contraceptive pills). rash heals well without scarring or pigmentation.
Patients may also present with a pruritic maculo-
Pathogenesis papular rash, which may resemble lesions of drug
eruption.
The immune system is clearly abnormal in SLE. T cells do 3. Joint manifestations About 95% of the patients show
not control the activity of the B cells, which produce a large joint involvement. Arthritis with pain on movement,
number of autoantibodies. Some of the autoantibodies tenderness, or effusion is present in about 80% and
(anti-DNA) participate directly in the renal disease forming arthralgia is present in about 15% of the patients with
immune complexes in the glomeruli. Other autoantibodies SLE. The proximal interphalangeal, knee, wrist and
form complexes with their antigens to lead to vasculitis. metacarpophalangeal joints are most often involved.
At present, SLE is thought to be a disorder in which Swan neck deformities occur in 15% of the individu-
genetic defects are present but not yet delineated. These als but unlike rheumatoid arthritis, no bony erosion
genetic defects lead to a defective homeostasis between is seen.
B cells and T cells. When the individual is challenged by 4. Renal disease Renal involvement in SLE ranges from 29
certain factors like ultraviolet light, infection or by an to 53%. The WHO grading of type of renal lesions are
unknown stimulus, the B cells become hyperactive leading a. Type I: normal
to a variety of autoantibodies. b. Type II: mesangial hypercellularity and immune
deposits confined to mesangium. These patients
Clinical features
have mild proteinuria
1. General manifestations Fever (low grade or spiking), c. Type III (focal proliferative lupus nephritis):
fatigue, weight loss and generalized malaise are gen- proteinuria, hematuria and occasionally nephrotic
eral manifestations in patients suffering from SLE. syndrome
598
d. Type IV (diffuse proliferative nephritis): signifi-

Figure 5
cant proteinuria, hematuria, renal insufficiency,
severe nephritic syndrome and hypertension may
be present
e. Type V (membraneous nephritis): significant pro-
teinuria, hematuria and nephritic syndrome.
5. Cardiopulmonary manifestations The most common
symptom is pleuritic chest pain, which is present
in about 40% of the patients. Pleural effusions are
rarely evident. The common cardiac manifestation
is pericarditis which occurs in about 25% of the
patients.
6. Nervous system manifestations Approximately 14% of
the patients exhibit peripheral neuropathy and mixed
sensory motor disturbances. Guillain–Barre syndrome
may occasionally be present. Grand mal seizures are
seen in about 15% of the patients in the early stages
of the disease. Organic brain syndromes are charac-
terized by impaired orientation, perception, memory
and intellectual function.
CSF studies revealed significant elevation of pro-
tein levels and WBC count in 32% of the cases.
7. Gastrointestinal manifestations Nausea, vomiting and White plaque with dark reddish purple margins on the palate
anorexia are present in about 20% of the patients. in a patient with SLE. Courtesy: Dr Ashok
Dysphagia may be present and is usually associated
with Raynaud’s phenomenon. Hepatomegaly occurs
in about 30% of the patients and splenomegaly is
seen in 20% of the patients and is usually not associ- examined 22 patients with SLE and reported that 50%
ated with hemolytic anemia. had oral lesions. The most common sites affected by
8. Ocular manifestations Approximately 15% of the SLE are buccal mucosa, lips and palate.
patients exhibit conjunctivitis and episcleritis. Rarely The typical intraoral lesions are multiple white plaques
occlusion of the central retinal artery may be seen. with dark, reddish purple margins (Figure 5). Hyperemia
Blindness from retinal arteritis may ensue. and edema are marked, increasing the tendency for bleed-
9. Hematologic abnormalities One or more hemato- ing and superficial ulceration. The prevalence of ulcer-
logic abnormalities are present in nearly all patients ation (most commonly reported oral manifestation of SLE)
of SLE. Most common is a mild to moderate normo- has been reported to be between 7 and 41%.
cytic normochromic anemia. Hemolytic anemia and Rhodes and Johnson in 1989 reported xerostomia,
reticulocytosis occur in only 10% of the patients. angular cheilitis, mucositis, glossitis, dental caries, peri-
Leukopenia with WBC counts less than 4,000/l. Mild odontitis, dysphagia and glossodynia as the oral manifes-
thrombocytopenia is present in 1/3rd of patients tations of SLE.
with SLE.
Circulating anticoagulant in SLE produces a slight
Laboratory investigations
prolongation of the thromboplastin time or partial
thromboplastin time. Bleeding and clotting times are 1. Presence of ANA: A wide spectrum of autoantibodies
normal. In contrast, antibodies directed against factors may be found in patients with SLE. ANA are usually
VII, IX and XII may prolong bleeding times and result detected for screening purposes by the indirect fluo-
in clinically significant state of anticoagulation. rescence antibody technique. ANA are positive in
10. Salivary gland manifestation Acute enlargement of one nearly all patients with SLE who have clinical evidence
or both parotid glands occurs occasionally in patients of active disease. But the diagnosis of SLE should
with SLE. The swelling may be painful and usually never be made in a patient with a positive ANA and
returns to normal as the systemic disease is controlled. absence of multisystem disease.
The enlargement may be associated with the presence 2. Polyclonal hypergammaglobulinemia is common in
of Sjögren’s syndrome. patients with SLE. Serum complement levels are gen-
11. Oral manifestations Oral manifestations of SLE were erally decreased in active SLE, especially in patients
first described by Bazin in 1861. Monash in 1931 suffering from active nephritis.
599
3. Rheumatoid factor: Approximately 18% of the patients AUTOIMMUNE POLYENDOCRINOPATHY–

exhibit rheumatoid factor in conjunction with non- CANDIDIASIS–ECTODERMAL DYSTROPHY
erosive deforming arthritis.
4. Lupus erythematosus (LE) cell phenomenon: A spe- Autoimmune polyendocrinopathy–candidiasis–ectodermal
cific test for the disease was established with the discov- dystrophy (APECED) is a rare condition which requires at
ery of the ‘LE cell’ inclusion phenomenon by Hargraves least two of the following conditions to make the diagno-
and associates in 1969. In this test, blood serum from sis: hyperparathyroidism, Addison’s disease and chronic
a suspected person is added to the buffy coat of normal mucocutaneous candidiasis.
blood. If the patient is suffering from SLE, typical LE Porter et al (1995) and Perniola et al (1998) described
cells will develop. The LE cell phenomenon consists of developmental defects of teeth and oral candidiasis as the
a rosette of neutrophils surrounding pale nuclear characteristic oral findings in these individuals.
mass apparently derived from a lymphocyte.
LE cells are rarely found in discoid lupus erythematosus. Clinical features
Management 1. Girls are more commonly affected.

2. Hypoparathyroidism is the most common endocrinopa-
The basis of treatment is the use of corticosteroids, given thy that is seen. However, addisonian adrenocortical
in a dose adequate to control the multisystem disease and hypofunction, autoimmune thyroid disease, vitiligo,
to return the anti-DNA antibodies and complement levels diabetes mellitus and other autoimmune disorders can
to normal. also be associated with APECED.
Depending on the severity and nature of the disease 3. Oral manifestations include pseudomembranous can-
the dose of corticosteroids can vary from 10 mg to 1 g didiasis and recurrent angular stomatitis.
intravenously per day. 4. Enamel hypoplasia may be seen secondary to hypo-
In patients with repeated exacerbations of the disease parathyroidism, usually affecting permanent dentition,
at moderately high doses of corticosteroids, azathioprine reflecting the early postnatal onset of autoimmune
may be useful to allow the patient to be maintained on hypoparathyroidism.
lower doses of corticosteroids. Antimalarial drugs are use-
ful in patients with skin abnormalities, alopecia or muco- Management
sal ulcers. In the recent times, severe and unresponsive
APECED management requires antifungal therapy and the
cases have been managed successfully with plasmaphere-
management of endocrinopathy.
sis or leukapheresis.
DIABETES MELLITUS TYPE I (IDDM)
1. Platelet count estimation may be required prior to any
oral surgical procedure, to evaluate the severity of Diabetes mellitus is a clinical syndrome characterized by
thrombocytopenia. hyperglycemia due to absolute or relative deficiency of
2. Bacterial endocarditis—Libman–Sacks vegetations may insulin.
be found under mitral valve leaflets in SLE. These Among the various types of diabetes, insulin-dependent
vegetations rarely affect function but can lead to bac- diabetes mellitus (IDDM) has an autoimmune basis.
terial endocarditis. Patients with SLE have to be consid- Edwards et al in 1995 proposed the following explana-
ered for antibiotic prophylaxis before dental treatment tions as evidence to suggest autoimmune etiology for
that is likely to cause bacteremia. IDDM:
3. According to Roura et al (1991), drugs that have a pho-
tosensitizing potential like penicillin, sulfonamides 1. HLA-linked genetic predisposition
and NSAIDs should be used judiciously as they tend to 2. Association with other autoimmune disorders
exacerbate SLE. 3. Circulating islet cell cytoplasmic and surface, insulin
4. SLE patients are susceptible to shock and infection as autoantibodies in new patients.
they are medicated with adrenal suppressing doses of 4. Mononuclear cell infiltration of pancreatic islets result-
corticosteroids or cytotoxic drugs. ing in selective destruction of insulin secreting cells.
5. Recurrence of insulinitis and selective destruction of
Prognosis insulin-secreting cells in pancreatic grafts.
Literature review reveals a 5-year survival rate in almost
95% of the patients of SLE. However, these patients need
to be constantly medicated with the lowest possible doses Median rhomboid glossitis is considered as a specific
of corticosteroids. pathognomonic oral manifestation associated with diabetes.
600
However, various oral conditions are worsened in a dia- seen. The characteristic claw-like deformity of fingers is
betic individual such as gingivitis and periodontal dis- seen due to flexural contractures and resorption of termi-
eases, oral candidiasis, delayed socket healing after extrac- nal phalanges (Figure 7). Some patients present with neu-
tion and burning tongue. ralgia or paresthesia.
Dermal involvement can be divided into three distinct
Specific considerations pertaining to dental treatment
phases:
1. Local anesthetics without epinephrine should be used 1. Edematous phase (stiff, puffy fingers)
because even minimal doses of epinephrine can elevate 2. Indurative phase (hard, tight, hidebound)
blood glucose concentration. Reduced blood supply in 3. Atrophic phase (softened skin, burned out).
diabetics due to atherosclerosis may be accentuated fur-
ther by vasoconstriction activity of epinephrine and
can lead to dry socket. Figure 6
2. Complicated dental procedures are best delayed in
uncontrolled diabetics.
3. Post-surgical infections are common in uncontrolled
diabetics. This can be managed with appropriate
antibiotics.
However, all types of routine dental treatment may be per-
formed safely in the dental setting in individuals whose
blood glucose levels are under control.
SYSTEMIC SCLEROSIS
Systemic sclerosis can be described as chronic, progressive

dermatosis characterized by board-like hardening and
immobility of the affected skin, with visceral involvement,
especially of lungs, esophagus, kidneys and heart. It is char-
acterized by alterations of the microvasculature, distur-
bances of the immune system and by massive deposition of
collagen. Curzio in 1973, published the first detailed descrip-
tion of a scleroderma-like disease. A variant of systemic
Linear ribbon-like marking on the forehead resembling
sclerosis is termed acrosclerosis, which is a combination of mark produced by blow of a saber in systemic sclerosis.
Raynaud’s phenomenon and scleroderma affecting the Courtesy: Dr Ajit Auluck
extremities.
Epidemiology
Figure 7
The annual incidence of systemic sclerosis is approximately
6–12 patients per one million individuals. Individuals
between the 3rd and 5th decades of life are mostly affected.
Females are affected seven times more than males.
Types of systemic sclerosis

1. Localized form
a. Circumscribed morphea form (yellowish-white patches
on the skin surface surrounded by a violaceous halo
of varying size and shape).
b. Linear ‘en coup de sabre’ form (linear ribbon-like
markings that resemble marks produced by blow of a
saber are seen) (Figure 6).
Claw-like deformity in systemic sclerosis.
In the initial stages, indurated edema of skin followed by
fixation of epidermis to the deeper subcutaneous tissues is
601
2. Diffuse form Diffuse form of systemic sclerosis is Orofacial manifestations

characterized by fibrosis of internal organs with loss of
❍ Mask-like appearance of the face (due to loss of skin
smooth muscle and loss of visceral function. More than
folds) (Figure 8).
50% of the patients exhibit involvement of the gastroin-
❍ Alae of the nose will gradually atrophy to produce the
testinal tract (mainly distal esophagus) resulting in dyspha-
characteristic ‘mouse-like facies’.
gia. Lung involvement results in dyspnea and hypoxia, due
❍ Fibrosis of the buccal mucosa may be seen.
to interstitial inflammation and fibrosis. Involvement of
❍ Tongue and lips may turn rigid.
the kidney and heart results in renal and cardiac failure
❍ Mucogingival periodontal problems (loss of attached
respectively. Occasionally, soft tissue calcifications may
gingiva, gingival recession) are seen.
also be seen.
❍ Mouth opening is significantly reduced, giving rise to
a ‘purse string’ appearance to the mouth.
❍ Involvement of the minor salivary glands may mimic
Figure 8 signs and symptoms of Sjögren’s syndrome.
❍ Some patients may complain of pain, paresthesia, or
hyperesthesia along the course of the trigeminal nerve.
❍ The most commonly seen radiographic finding in
patients is the resorption of the terminal phalanges of
the hand and the distal portions of the radius and ulna.
❍ Generalized widening of the periodontal ligament
space (two to four times the normal width) is seen in
10–37% of the patients. In comparison to anterior
teeth, widening of periodontal ligament space is more
marked in the posterior teeth (Figure 9).
❍ Resorption of the mandible in patients suffering from
scleroderma was first reported by Taveras in 1959.
Literature review reveals that the incidence of mandibu-
lar resorption varies from 10 to 33%. The sites that are
resorbed include the condyle, coronoid process, ascend-
ing ramus, posterior border of the ramus and the angle
Mask-like facies in progressive systemic sclerosis. of the mandible. The sites of resorption predispose
Courtesy: Dr Ajit Auluck to pathological fractures, osteomyelitis, trigeminal
Figure 9
Generalized widening of periodontal ligament space in systemic sclerosis. Courtesy: Dr Ajit Auluck
602
neuropathy (associated with severe pain) and aperto- it involves muscles that control facial expression, chewing
gnathia. and swallowing. In advanced stages, respiratory muscles
are affected leading to acute respiratory failure.
Diagnosis
Epidemiology
The diagnosis of systemic sclerosis is made by the presence
It affects approximately 2 out of every 100,000 people and
of the characteristic clinical and radiographic features.
can occur at any age. It is most common in women
However, some non-specific serological changes such as the
in the 2nd and 3rd decades of life. In men, the condition
presence of antinuclear antibodies and rheumatoid factor
usually develops between 6th and 8th decades of life.
are seen.
Types of MG
Syndromes associated with systemic sclerosis
Generalized MG: Approximately 85% of the patients
CREST syndrome was originally called Thibierge– develop generalized form of this condition within 1 year of
Weissenbach syndrome in honor of two French physicians the onset of the disease. Patients report progressive weak-
who described this condition in 1910. Winterbauer renamed ness in the trunk, arms and legs.
this syndrome as CREST syndrome in 1964.
CREST syndrome is a combination of calcinosis cutis, Ocular MG: Muscles that control eye movements are typi-
Raynaud’s phenomenon, esophageal hypomobility, sclero- cally affected. Approximately 10–15% are affected by this
dactyly and telangiectasia. condition.
Congenital MG: Inherited condition caused by genetic
Dental considerations defect, develops at birth or shortly after birth.
All forms of dental treatment can be safely carried out in Transient neonatal MG: It occurs in infants born to moth-
these patients. However, since the mouth opening gradu- ers who have MG. Around 10–20% of the neonates develop
ally reduces, mouth opening exercises, use of increased this condition. It occurs due to transfer of maternal anti-
number of tongue blades between posterior teeth to stretch AChR antibodies through the placenta to the newborn
facial tissues and bilateral commissurotomy are done to reacting with the AChR of the neonate.
manage these patients.
These patients can be instructed to practice good oral Etiology
hygiene practices as the gradual reduction in mouth open-
The exact cause of MG is still unknown. Females and indi-
ing will hinder maintenance of oral hygiene.
viduals with certain HLA have a genetic predisposition to
autoimmune diseases. It is proposed that there is a dys-
Course and prognosis regulation of the immune system. Sensitization to a for-
The prognosis is better when there are only dermal mani- eign antigen that has cross-reactivity with the nicotinic
festations. Involvement of internal structures has an unfa- ACh receptor has been proposed as etiology for MG.
vorable prognosis. On an average the 5-year survival rate
is about 70%. Clinical features
1. Patient’s face may appear expressionless due to the
weak and slack facial muscles.
MYASTHENIA GRAVIS 2. Inability to hold the head upright.
3. Mandibular movements are sluggish and slack. Patient
The term myasthenia gravis (MG) was coined by combin- may have to support his/her mandible with a finger.
ing two Greek words for muscle and weakness to form the The patient’s mouth may remain open all the time.
word myasthenia and adding the Latin word gravis, which 4. Patient is said to have a ‘snarling face’ when he/she try
means severe. The autoimmune etiology for MG was pro- to smile. When the patient attempt to smile, the corners
posed for the first time in 1959–1960 by two independent of the mouth are not drawn up and outward, and the
workers, Simpson and Nastuck. levator muscles expose the canines.
Myasthenia gravis is an autoimmune disorder of the 5. Gag reflex is often absent, and such patients are at risk
neuromuscular junction, in which autoantibodies are for aspiration of oral secretions.
directed toward acetylcholine receptors (Ac in the motor 6. Dysphagia is seen because of the weakness of the
end-plate units of skeletal muscle, resulting in a progres- muscles associated with chewing and swallowing.
sive fatigability of the skeletal muscle. In the initial stages, 7. Patient may complain of nasal regurgitation.
extraocular muscles are affected. As the disease progresses 8. Patient’s voice may acquire a nasal quality.
603
9. In severe cases patient is unable to clear bronchial 10–20 seconds. After 20 seconds the patient is advised to
secretions which may predispose to pneumonia. bring his/her gaze to the normal position. In patients suf-
10. Ptosis is seen in almost all patients. Patients have fering from MG, the upper eyelid elevates and may either
difficulty in eye closure. Characteristically, pupils are slowly begin to droop or twitch several times before assum-
unaffected in MG. ing a stable position. This phenomenon is brought about
by rapid recovery and easy fatigability of muscles. How-
Diagnostic tests ever, this test can be positive in patients with brain stem
Myasthenia gravis can be diagnosed with tests like edro- or ocular disorders.
phonium test, ice-pack test, test to assess eyelid twitch
response, anti-AChR antibody test, anti-MuSK antibodies, Management of MG
electrophysiological tests (repetitive nerve stimulation test
and single fiber electromyography), CT/MRI of chest (to The current management of MG includes the use of anti-
screen for associated thymic tumors). Patients should also cholinesterase drugs for temporary improvement of neuro-
be evaluated for diseases associated with MG such as thy- muscular transmission, removal of anti-AChR Abs by plasma
roid disease, diabetes mellitus, rheumatoid disease, perni- exchange or specific immunoadsorption procedures, use of
cious anemia, systemic lupus erythematosus, sarcoidosis, non-specific immunosuppressants or immunomodulators
Sjögren’s disease and polymyositis. to curb the anti-AChR response, and thymectomy.
MG when not treated with adequate dosage of medica-
Simple tests that can be performed in a dental tion can present with myasthenic crisis and when treated
setting with excessive medication can exhibit cholinergic crisis.
Myasthenic crisis may cause bronchospasm with
Ice-pack test Patients having ptosis secondary to MG will wheezing, cyanosis and in extreme conditions respiratory
improve when an icepack is applied to the eyelid. However, failure can occur.
ptosis secondary to other conditions will not improve on Cholinergic crisis may be associated with miosis and
application of icepack. SLUDGE syndrome, which is characterized by salivation,
Eyelid twitch response It is also referred to as Cogan’s lacrimation, urinary incontinence, diarrhea, gastrointen-
lid twitch. Patient is instructed to gaze downward for stinal upset, hypermotility and emesis.
604
CHAPTER
Granulomatous Diseases
Adel Kauzman, Iona Leong 21
➧ Tuberculosis ➧ Foreign Body Granulomas
➧ Leprosy (Hansen’s Disease) ➧ Wegener’s Granulomatosis
➧ Syphilis ➧ Sarcoidosis
➧ Deep Fungal Infections ➧ Orofacial Granulomatosis
Blastomycosis ➧ Crohn’s Disease (Regional Ileitis, Regional
Histoplasmosis Enteritis)
Aspergillosis
Zygomycosis (Mucormycosis, Phycomycosis)
Granulomatous diseases are a heterogeneous group of dis-

Figure 1
orders that are characterized by a specific pattern of chronic
inflammation called granulomatous inflammation, in which
granulomas are formed. Granulomas usually develop as a
result of a cell-mediated hypersensitivity reaction to a
non-degradable antigen. The clinical significance of a histo-
logical finding of granulomatous inflammation is that this
type of inflammation is associated with a relatively limited
number of conditions.
A granuloma is a focal aggregate of inflammatory cells,
composed predominantly of epithelioid macrophages,
which may be surrounded by a rim of lymphocytes and/or
plasma cells. Epithelioid macrophages resemble epithelial
cells, having abundant pale pink cytoplasm, vesicular and
oval nuclei and indistinct cell borders (Figure 1). Some
activated macrophages may fuse to form multinucleated
giant cells which may be located within the center or
periphery of the granuloma (Figure 2). In Langhans type Photomicrograph of a granulomatous inflammatory reaction
giant cells the nuclei are arranged in the periphery, often in showing multiple granulomas surrounded by lymphocytes.
Each granuloma is composed predominantly of epithelioid
the shape of a horseshoe. The nuclei of foreign body type
macrophages and multinucleated giant cells. Epithelioid
giant cells are arranged haphazardly in the cytoplasm.
macrophages show abundant pale pink cytoplasm,
Some granulomas may show central areas of necrosis. vesicular and oval nuclei and indistinct cell borders.
Caseous necrosis, in which the dead tissue is soft and dry (Hematoxylin and eosin, original magnification 200)
and resembles cheese, is classically associated with tuber-
culosis. Long-standing granulomas may be surrounded by
a rim of fibrous connective tissue and fibroblasts. Immune-mediated granulomatous inflammation represents
Granulomas can be classified as immune or foreign body a form of delayed type (cell-mediated) hypersensitivity
type. Foreign body type granulomas develop in response to reaction and may be secondary to specific types of bacte-
inert endogenous or exogenous foreign material (Table 1). rial, fungal and parasitic infections. Immune granulomas
605
Figure 2 TUBERCULOSIS
Tuberculosis is a common worldwide infection caused by

bacteria belonging to the Mycobacterium tuberculosis
complex. Although tuberculosis usually affects the lungs, it
may also involve other organs in up to one-third of cases.
Initial infection, also known as primary infection, is followed
by a long latency period characterized by absence of symp-
toms in most individuals. Latent infection progresses to
active disease in a minority of individuals, due to reacti-
vation or exogenous re-infection with a second strain of
M. tuberculosis. Therefore infection must be distinguished
from active disease. Active tuberculosis caused by drug-
susceptible strains is curable if properly treated, but may
be fatal within 5 years in more than half the cases if
untreated.
The M. tuberculosis complex includes M. tuberculosis,
Photomicrograph showing a multinucleated giant cell the most frequent and important agent of human disease.
in the center of a granuloma. The nuclei of the giant cell are
M. bovis is transmitted by unpasteurized milk and is the
arranged in the periphery of the cytoplasm. (Hematoxylin and
cause of a small percentage of cases in developing coun-
eosin, original magnification 250)
tries. M. tuberculosis is an aerobic, non-spore-forming,
rod-shaped bacterium. The organism is classified as an
acid-fast bacillus because the high mycolic acid and lipid
content in the cell wall prevents decolorization by acid
Table 1 Granulomatous diseases that may affect the oral tissues
alcohol with acid-fast stains such as the commonly used
Infections Foreign body Diseases of unknown Ziehl–Neelsen stain.
etiology Tuberculosis is a disease of poverty associated with
Bacterial
overcrowding and undernutrition. Other risk factors for
Tuberculosis Silica Wegener’s granulomatosis tuberculosis include human immunodeficiency virus (HIV)
Leprosy Beryllium Sarcoidosis infection, diabetes, smoking, excessive alcohol consump-
Syphilis Talc Orofacial granulomatosis tion, end-stage renal failure, and therapy with tumor
Others Suture material Crohn’s disease necrosis factor antagonists and corticosteroids.
Starch Based on World Health Organization (WHO) estimates,
Fungal approximately two billion people or one-third of the world’s
Blastomycosis total population were infected with M. tuberculosis in 2010,
Histoplasmosis with 1.4 million deaths caused by tuberculosis yearly.
Aspergillosis Tuberculosis occurs world-wide, but the majority of cases
Zygomycosis occur in low-income countries and in countries with emerg-
Others ing economies. The developing countries of Africa and Asia
Parasitic have the highest prevalence of tuberculosis, with the most
Leishmaniasis cases occurring in India, China, South Africa, Indonesia and
Schistosomiasis Pakistan. Tuberculosis is uncommon in North America and
Europe, where it occurs predominantly in HIV-infected
persons, immigrants from high-prevalence countries, dis-
advantaged and marginalized populations. Although
are mostly seen with mycobacterial and fungal infections intense efforts at disease control have resulted in stabiliza-
because the infectious organisms typically have antigens tion or decreasing incidence of tuberculosis in most coun-
which are poorly degraded by macrophages. Antigen tries, there has been a rapid increase in Africa related to
exposure initiates a cell-mediated immune response, the spread of the HIV epidemic.
resulting in activated CD4 T lymphocytes of the Th1 type
which secrete cytokines such as interleukin (IL)-2, IL-12,
Pathogenesis
interferon (IFN)-, and tumor necrosis factor (TNF)-. The
cytokines stimulate activation of macrophages to form The disease is usually transmitted by the airborne spread
granulomas. Some immune-mediated granulomatous dis- of droplet nuclei produced by patients with infectious pul-
eases are of unknown etiology (Table 1). monary tuberculosis. Droplets aerosolized by coughing,
606
Chapter 21 – Granulomatous Diseases
sneezing or speaking are inhaled. Thus transmission of

Figure 3
infection is enhanced by crowded conditions in poorly ven-
tilated rooms and prolonged, close contact with patients
with active tuberculosis. Inhaled bacilli reaching the alveoli
are ingested by non-specifically activated alveolar macro-
phages, which may contain the bacillary multiplication or
be killed by the multiplying bacilli. Chemotactic factors
released by lysed macrophages attract non-activated mono-
cytes from the bloodstream to the site. These initial stages
of infection are usually asymptomatic.
Specific immunity develops about 2–4 weeks after
infection. Large numbers of activated macrophages accu-
mulate at the site of the primary lesion, forming granulomas
or tubercles. Granuloma formation is mediated by cytokines
released by alveolar macrophages, including TNF-. This
delayed-type hypersensitivity reaction to bacillary antigens
destroys macrophages and causes necrosis in the center of
the tubercles. The necrotic material resembles soft cheese,
hence the designation ‘caseous necrosis’. Its low oxygen
tension and low pH inhibit growth of M. tuberculosis. Viable
organisms may however remain dormant within the mac- Gross appearance of Ghon lesion in the lung of a patient
rophages or in the necrotic material for years or even with a history of healed primary pulmonary tuberculosis.
throughout the patient’s lifetime. Some tubercles in the lung It presents as a calcified spherical mass
parenchyma and hilar lymph nodes may heal by fibrosis
and calcification, while others undergo further evolution.
Figure 4
Clinical features
Tuberculosis is classified as pulmonary or extrapulmonary.
Pulmonary tuberculosis can be primary or secondary.
Primary pulmonary disease results from an initial infec-
tion with M. tuberculosis in previously unexposed indi-
viduals and is usually asymptomatic. The lesion is usually
peripheral and localized to the middle and lower lung
zones and is accompanied by hilar or paratracheal lymph-
adenopathy. In most cases, the lesion heals spontaneously
and may later be evident as a small calcified nodule (Ghon
lesion) (Figure 3). Approximately 5–10% of patients prog-
ress directly from initial infection to active disease, usually
because of an existing state of immunosuppression. This is
especially seen in children and in persons with impaired
immunity, such as those with malnutrition or HIV infec-
tion. The initial lesion enlarges, cavitates, invades and
destroys bronchial walls and blood vessels. Large numbers
of bacilli spread into the airways and the environment
through expectorated sputum. Patients may develop pleu-
ral effusion and progressive primary tuberculosis.
Hematogeneous dissemination may result in fatal miliary Gross appearance of miliary tuberculosis in the lung
tuberculosis (Figure 4) or tuberculous meningitis.
Secondary pulmonary tuberculosis, also known as post-
primary disease, is usually due to endogenous reactivation oxygen tension favors mycobacterial growth. The extent
of latent infection. Triggers for reactivation include immu- of lung involvement varies greatly, from small infiltrates
nosuppression, especially AIDS, malnutrition and vitamin D to extensive cavitatory disease. Massive involvement of
deficiency. The disease usually occurs in the apical and the lungs, with coalescence of lesions, produces tubercu-
posterior segments of the upper lung lobes, where the high lous pneumonia. The result may be death, spontaneous
607
Figure 5
Panoramic radiograph of a patient with a history of tuberculosis showing calcified cervical and submandibular lymph nodes
remission or chronic disease with a progressively debilitat- leukoplakic areas. Most oral lesions represent secondary
ing course (‘consumption’). Individuals with chronic disease infection from the initial pulmonary lesions, either from
continue to discharge tubercle bacilli into the environment. hematogeneous spread or from exposure to infected spu-
Symptoms and signs are often non-specific and insidious tum. Secondary oral lesions usually occur on the tongue,
early in the course of secondary pulmonary tuberculosis, palate and lip.
consisting mainly of fever, night sweats, weight loss, Primary oral tuberculosis without pulmonary involve-
anorexia, general malaise and weakness. Cough eventu- ment is rare. It usually involves the gingiva, mucobuccal fold
ally develops in most individuals, often initially non-pro- and areas of inflammation adjacent to teeth or in extraction
ductive and subsequently accompanied by the production sites. Primary oral lesions are frequently accompanied by
of purulent sputum. The sputum may be blood-streaked enlarged regional lymph nodes. Tuberculous osteomyelitis
due to blood vessel involvement. has been reported in the jaws and appears as ill-defined
Virtually any organ system may be affected by extra- areas of radiolucency.
pulmonary tuberculosis, but the most commonly involved
sites are the lymph nodes, pleura, genitourinary tract, bones Histopathologic features
and joints, meninges, peritoneum, pericardium, and the
head and neck region. In the head and neck, tuberculosis can Affected tissues show multiple granulomas called tubercles
involve the larynx, middle ear, nasal cavity, nasopharynx, which consist of aggregates of epithelioid histiocytes, lym-
oral cavity, parotid gland, esophagus and spine. As a result phocytes and Langhans multinucleated giant cells (Figure 1).
of hematogeneous dissemination in HIV-infected individu- The tubercles often show central caseous necrosis (Figure 6).
als, extrapulmonary tuberculosis is seen more commonly Special stains such as Ziehl–Neelsen or other acid-fast
nowadays than in the past. stains are required to demonstrate the bacteria (Figure 7).
Lymph node tuberculosis (tuberculous lymphadenitis) Because of the relative scarcity of bacilli within tissue,
is the most common form of extrapulmonary tuberculosis organisms may not be successfully demonstrated in all
and is especially common in HIV-infected persons. It usually cases, and a negative result therefore does not completely
presents as painless swelling of the lymph nodes, most eliminate the possibility of tuberculosis.
commonly at cervical and supraclavicular sites (scrofula). Granulomas may be absent or poorly formed in people
Lymph nodes are usually discrete in early disease but may with poor immune responses, especially those infected
develop caseous necrosis and form fistulas through the over- with HIV.
lying skin draining caseous material. Involved nodes may
radiographically appear calcified (Figure 5). Pulmonary Diagnosis and laboratory findings
tuberculosis is unusual in patients with scrofula.
A presumptive diagnosis of active tuberculosis is com-
monly made on the finding of acid-fast bacilli on micro-
Oral manifestations
scopic examination of a diagnostic specimen such as a tissue
The most common manifestation of tuberculosis in the oral biopsy or a smear of expectorated sputum. Definitive diag-
cavity is a chronic painless ulcer. Less frequently tubercu- nosis requires the isolation and identification of M. tuber-
lous lesions present as nodular, granular or rarely firm culosis from a diagnostic specimen, usually sputum in a
608
Due to inconsistent results and high rates of false-

Figure 6
negative and false-positive results, serological tests for the
diagnosis of active tuberculosis are not recommended.
Screening for latent M. tuberculosis infection can be
performed with skin testing with purified protein deriva-
tive (PPD). However, the test is of limited value in the
diagnosis of active disease because of its low sensitivity
and specificity. False-negative reactions are common in
immunosuppressed patients and in those with overwhelm-
ing tuberculosis. Positive reactions are elicited from indi-
viduals who have been infected with M. tuberculosis but
do not have active disease and from persons sensitized by
non-tuberculous mycobacteria or Bacille Calmette–Guérin
(BCG) vaccination.
The gold standard for identifying M. tuberculosis
infection is an interferon- release assay which measures
interferon- titers released by T cells in response to stimu-
Photomicrograph of a caseating granuloma from a lation with certain TB-speific antigens, such as ESAT-10
tuberculous lesion. The center of the granulomas shows and CFP-10.
caseous necrosis. Multinucleated giant cells and lymphocytes
are seen in the periphery of the granuloma. (Hematoxylin and
eosin, original magnification 5) Management protocols and prognosis
Because of drug resistance, cure of tuberculosis requires
prolonged concomitant administration of at least two agents
to which the organism is susceptible. Four major drugs are
Figure 7 considered as first-line treatment agents: isoniazid, rifampin
(rifampicin), pyrazinamide and ethambutol. Second-line
drugs include streptomycin, kanamycin, amikacin, capreo-
mycin, ethionamide, cycloserine, para-aminosalicylic acid
and fluoroquinolone antibiotics such as ofloxacin. Second-
line agents are used in patients resistant to first-line ther-
apy, because of their lower efficacy and higher toxicity.
Infectious cases should be diagnosed rapidly and appro-
priately treated until cure. The development of drug-resis-
tant tuberculosis is primarily the result of monotherapy or
failure of the healthcare provider to prescribe at least two
drugs to which the tubercle bacilli are susceptible. Resistance
occurs also if the patient fails to take properly prescribed
therapy. Multidrug-resistant tuberculosis (MDR-TB) is a
form of tuberculosis that does not respond to the standard
Photomicrograph showing a positive Ziehl-Neelsen
drug treatment, i.e. the two main first-line drugs: isoniazid
stain in a case of pulmonary tuberculosis.
and rifampicin. The term extensive (extreme) drug resistant
(Ziehl–Neelsen, original magnification 400)
tuberculosis (XDR-TB) has been used to describe a form of
MDR-TB resistant to any fluoroquinolone and one of three
injectable aminoglycosides (capreomycin, kanamycin and
patient with productive cough. Specimens are cultured on amikacin).
egg- or agar-based medium and incubated at 37C under 5% Strategies for prevention and disease control include
CO2. The organisms grow slowly in culture and 4–8 weeks BCG vaccination and treatment of persons with latent tuber-
may be required before growth is detected. culosis infection who are at high risk of developing active
Automated liquid culture systems are faster and show a disease, such as those infected with HIV, close contacts of
greater yield than solid media-based systems. persons with known or suspected active tuberculosis, persons
New automated molecular tests such as XpertMTB/RIF with medical risk factors associated with reactivation of
which is a nucleic acid amplification test allow rapid diag- tuberculosis, medically underserved and low-income pop-
nosis not only of tuberculosis, but also of multidrug-resistant ulations, alcoholics, injection drug users, persons with abnor-
tuberculosis (MDR-TB). mal chest radiographs compatible with past tuberculosis,
609
and residents of long-term care facilities. BCG vaccine is long, and is thought to be between 3 and 5 years, with a
recommended for routine use at birth in countries with range of a few months to 30 years.
high tuberculosis prevalence. However, general use of BCG Interactions between host genetic factors and the micro-
may not be recommended in countries with low risk of organism determine the clinical pattern of the disease.
transmission due to the wide variation in efficacy of the Some genes control the overall susceptibility and resis-
vaccines available. tance of the individual to the disease (innate resistance),
while HLA-associated genes influence the ability and the
pattern of the immune response mounted against the
microorganism (through the function of T lymphocytes
LEPROSY (Hansen’s Disease) and antigen-presenting cells).
Tuberculoid and lepromatous leprosy represent the two
Leprosy is a chronic granulomatous disease affecting the main clinical patterns of the disease situated at either end of
skin and peripheral nerves caused by Mycobacterium a spectrum with borderline forms situated along this spec-
leprae. Impairment of nerve function results in major dis- trum. Tuberculoid leprosy is seen in patients who are able
abilities which have physical, social and psychological to mount a vigorous immune response to the mycobacterium,
consequences and are the basis of the stigmatization his- and is characterized by low bacillary counts (paucibacillary
torically associated with the disease. M. leprae is an acid- leprosy), few skin lesions and involvement of a small num-
fast-staining, gram-positive, obligate intracellular bacillus ber of nerve trunks. It shows a predominant Th1 cytokine
with tropism for macrophages and Schwann cells. The response with high levels of IL-2, IL-12, IFN- and TNF-.
organism grows preferentially in areas of lower tempera- Lepromatous leprosy is characterized by a limited cellular
ture, which is why the skin seems to be a target site for immune response, uncontrolled bacillary proliferation within
infection. M. leprae does not grow in culture media. Animal host macrophages (multibacillary leprosy), diffuse skin
reservoirs of M. leprae include the nine-banded armadillo lesions and extensive involvement of peripheral nerves. In
(due to its low core body temperature), mice footpads and this form, there is a defective Th1 or a dominant Th2 cyto-
some non-human primates. kine response. High levels of IL-4, IL-5 and IL-10 are
According to the WHO, about 720,000 new cases of detected in these lesions. Most patients, however, fall into
leprosy are reported each year, and about two million per- a broad borderline category between these two ends of
sons suffer from leprosy-related disabilities. The disease is the spectrum, and are classified as borderline-tuberculoid,
endemic in several developing tropical countries, including mid-borderline (or dimorphous) and borderline-lepromatous.
India (which accounts for the majority of reported cases These forms are clinically unstable, and affected patients
worldwide), Brazil, Myanmar, Madagascar and Mozambique. can experience a shift of the disease toward any end of the
More than 80% of all new cases are reported in these geo- spectrum.
graphic locations. Moreover, geographic and ethnic differ-
ences in disease distribution are noted within each of these
countries. In contrast to other mycobacterial infections, Clinical features
leprosy does not seem to be more frequent in patients Leprosy is twice as common in males as in females, espe-
infected with HIV, and co-infection with HIV and M. leprae cially after puberty. The age at diagnosis varies between 10
does not appear to alter the course of either disease. and 20 years. Leprosy can affect the skin, peripheral nerves,
eyes and bone. Peripheral nerve lesions can cause numb-
ness and weakness, which results in traumatic injuries to
Pathogenesis
affected organs.
The precise mechanism of transmission of leprosy is still Tuberculoid leprosy, which corresponds to the pauci-
unknown. Leprosy is not very contagious and has low bacillary form of the disease, presents initially on the skin
infectivity. Leprosy is not spread by touching, and expo- as hypopigmented, well-demarcated macules that enlarge
sure to the microorganism rarely results in clinical disease. slowly and develop elevated margins. The number of lesions
Transmission seems to require frequent and prolonged is usually limited. Loss of dermal appendages (hair folli-
contact with an infected person and there is an eight-fold cles and sweat glands) also is seen, especially in fully
increased risk of disease development in household con- developed skin lesions. Nerve involvement occurs early in
tacts. Disease transmission is thought to occur by inhala- the course of the disease and sensory, motor and autonomic
tion of bacilli-laden aerosols from lesions in the respiratory nerves are affected, with resulting hypoesthesia, muscle
tract. The inhaled microorganisms are disseminated by weakness and anhidrosis. Involved nerves are enlarged and
alveolar macrophages to different body organs. Growth can be visible clinically. Some patients can present with
occurs mainly in cooler body sites, such as the skin, mucosa severe painful neuritis. Sensory loss in the hands and feet
and peripheral nerves. The incubation period between often leads to severe trauma and burns. When the facial
exposure to the M. leprae and disease development is very nerve is affected, patients can experience lagophthalmos
610
(inability to close eyelids completely) and facial paralysis. In lepromatous leprosy, sheets of foamy macrophages
When the ophthalmic branch of the trigeminal nerve is called lepra cells are seen in the dermis. These cells contain
involved, anesthesia of the cornea and conjunctiva can very large numbers of acid-fast bacilli, best demonstrated
increase the risk of corneal trauma and ulceration. These with the Fite method. Well-formed granulomas are rare in
ocular lesions can result in blindness. lepromatous leprosy.
Lepromatous leprosy, which corresponds to multibacil-
lary form of leprosy, shows extensive, symmetric and bilateral
Diagnosis and laboratory findings
skin lesions. The skin of the face is commonly affected,
becoming thickened and corrugated. Earlobes become pen- The diagnosis of leprosy is based on a positive history of
dulous and the lateral portions of the eyebrows are lost. prolonged contact with a known infected person, living in
Involvement of the nose causes nasal stuffiness and nasal endemic areas of the disease, clinical presentation and
bleeds, while perforation of the nasal septum results in laboratory findings. Important diagnostic signs include:
saddlenose deformity. Nerve involvement is less prominent (i) hypopigmented or reddish patches with definite loss of
in lepromatous leprosy. sensation, (ii) thickened peripheral nerves, and (iii) acid-
Leprosy reactions are acute inflammatory complica- fast bacilli on skin smears or biopsy material.
tions presenting as medical emergencies during the course Skin smears obtained to detect the microorganism are
of the disease. These can occur in untreated patients, but highly specific, but not very sensitive, because bacilli may
may often represent a complication of therapy. not be identified in tuberculoid leprosy. This test is not
used routinely as a diagnostic aid because laboratory ser-
vices are not readily available. Intradermal injection of
Oral manifestations
heat-killed M. leprae (lepromin test) has no diagnostic
Oral lesions are not common in patients affected by lep- utility in individual cases. A positive lepromin test reflects
rosy, nor are they considered pathognomonic of the dis- the ability of a person to develop a granulomatous response
ease. Their prevalence ranges from 0 to 60% of cases. This to the microorganism, but does not indicate infection
may decrease further with recent advances in therapy. Oral with, or exposure to M. leprae. Major advances in the
lesions seem to be more frequent in the lepromatous form laboratory diagnosis of leprosy were made by the develop-
of the disease and include papules, plaques, nodules, non- ment of genetic probes and polymerase chain reaction
specific erosions and ulcerations involving the tongue, (PCR) that can identify M. leprae DNA in clinical speci-
buccal mucosa and palate. mens. These molecular techniques are highly sensitive and
A triad of changes affecting the facial bones and termed specific, but are not currently used in clinical practice, in
facies leprosa was described through archaeological studies. part due to the complexity of the procedures. The gold
It consists of atrophy of the anterior nasal spine, atrophy standard for the diagnosis of leprosy is a full thickness
and recession of the maxillary alveolar process, and endo- skin biopsy obtained from the advancing margin of an
nasal inflammatory changes. The alveolar destruction lim- active lesion.
ited to the maxillary anterior region can result in loosening
and loss of teeth in the area. Granulomatous inflammation
Management protocols and prognosis
of the nasal cavity can result in palatal perforation and
oronasal communication. The WHO recommends prolonged multidrug therapy for
In lepromatous leprosy, M. leprae can infect the dental leprosy to overcome the problem of drug-resistant M. lep-
pulp and cause pulp necrosis. The microorganisms can rae. Paucibacillary leprosy is treated with dapsone and
accumulate within myelinated nerves in the pulp. Infected rifampin while multibacillary leprosy is treated with a
macrophages can be seen around capillaries. The resulting regimen of dapsone, rifampin and clofazimine. The dura-
vascular damage may cause a reddish discoloration of the tion of treatment varies depending on the form of the dis-
tooth. ease. Other multidrug protocols with varying periods of
treatment are currently being tested.
Complications of leprosy include crippling of the hands
and feet, facial deformities and blindness. Recovery
Tuberculoid leprosy demonstrates well-developed non- from neurologic impairment is limited. Leprosy reactions,
caseating granulomas composed of histiocytes, lympho- which can occur during treatment, require special and
cytes and giant cells. Acid-fast bacilli are absent or difficult emergency care.
to find. Peripheral nerves become enclosed within and are No highly effective vaccine has yet been developed against
destroyed by the granulomas. Tuberculosis, sarcoidosis and leprosy, but there is evidence of a protective effect of BCG
other granulomatous inflammatory reactions may show vaccine. The efficacy of this vaccine ranges between 20 and
similar microscopic features and must be excluded by 80% in different trials. Other vaccines are being tested
clinical and laboratory investigations. including BCG with heat-killed M. leprae.
611
SYPHILIS with or without treatment. Because the ulcers are usually

painless and can occur at sites where they are not visible
Syphilis, also known as lues, is a chronic sexually transmit- or recognized, many individuals are not diagnosed at this
ted disease caused by Treponema pallidum. The disease stage.
has diverse clinical presentations and is characterized by Hematogeneous dissemination of the pathogen in the
periods of active disease and latency. primary stage results in the manifestations of secondary
The causative agent of syphilis, T. pallidum subspecies syphilis, approximately 6–8 weeks later. The most com-
pallidum, hereafter referred to as T. pallidum, is a micro- mon manifestations of this stage are a maculopapular rash
aerophilic spirochaete which cannot be grown in vitro. affecting the flank, shoulder, arm, chest, back, hands and
The only known natural host for T. pallidum is the human. soles of feet. A generalized non-tender lymphadenopathy
The genome has been fully sequenced and is small, ren- also is present. Less common features of secondary syphilis
dering T. pallidum an obligate parasite with limited meta- are mucous patches, condyloma lata, alopecia, meningitis,
bolic capabilities. Other pathogenic treponemes also can myalgia, ocular complaints, hepatic, pulmonary and neu-
cause disease in humans, such as yaws and pinta. rologic involvement. The secondary lesions resolve, with
Syphilis occurs worldwide and usually is transmitted by or without treatment, and the infection enters a ‘latent’ stage
sexual contact or from mother to infant. T. pallidum can in which there are no clinical manifestations. Transmission
be transmitted as a blood-borne infection, but this is infre- of syphilis is by lesion contact and occurs through indi-
quent. Syphilis is most common among the poor, those who viduals with the primary or secondary stages of the disease.
lack access to healthcare and in those with many sexual After many years, approximately one-third of individu-
partners. According to WHO estimates, approximately als with latent syphilis progresses to tertiary syphilis, which
12 million new cases of venereal syphilis occurred in 1999, is characterized by cardiovascular disease, neurosyphilis
most of them in developing countries. Congenital syphilis or gummas. Cardiovascular syphilis is the result of endar-
is a leading cause of perinatal and neonatal death in many teritis obliterans of the vasa vasorum which provides the
of these countries. In North America and western Europe, blood supply to large vessels, leading to aortitis, aortic
syphilis is less common and disproportionately affects minor- regurgitation, aortic aneurysm or coronary ostial stenosis.
ity populations, men who have sex with men, and persons Neurosyphilis may take the form of general paresis, insan-
using cocaine and other drugs. The individuals at highest ity and tabes dorsalis. Nearly all direct mortality is due to
risk for syphilis also are at increased risk for HIV infection, cardiovascular or neurologic complications and is seen in
and the two frequently coexist, as syphilis facilitates the a relatively small proportion of patients, years after infec-
transmission of HIV. tion. Gummas are foci of granulomatous inflammation which
may occur in any tissue and present as nodular ulcerative
Pathogenesis lesions. The most commonly involved sites include skin and
skeletal system, mouth and upper respiratory tract, larynx,
T. pallidum rapidly penetrates intact mucous membranes liver and stomach.
or microscopic abrasions in skin. It then enters the lymphat- In pregnant women, syphilis may lead to stillbirth,
ics and blood to produce systemic infection. Despite spontaneous abortion or congenital infection of the neo-
induction of a strong humoral and cell-mediated immune nate. Features of early congenital syphilis include rhinitis,
response, T. pallidum is able to survive in the untreated mucocutaneous lesions, periostitis, hepatosplenomegaly,
human host for decades and may continue to be transmit- lymphadenopathy, anemia, jaundice, leukocytosis and
ted or cause end-organ damage. T. pallidum can be trans- thrombocytopenia. Later features of congenital syphilis
mitted from a syphilitic woman to her fetus across the are seen at least 24 months after birth and include the
placenta at any stage of pregnancy. The manifestations of hutchinsonian triad of interstitial keratitis of the cornea, sen-
congenital syphilis are thought to be due to the immune sorineural hearing loss, and the dental anomalies described
response of the host, rather than a direct toxic effect of the below.
pathogen.
Clinical features Oral manifestations

Untreated syphilis is a chronic illness which is tradition- Although the oral cavity is the most common extragenital
ally divided into primary, secondary and tertiary stages. In site of infection, oral manifestations of syphilis are rare.
the primary stage a chancre appears at the site of inocula- Primary syphilis results from orogenital or oroanal contact
tion about 21 days post-infection. The chancre begins as a with an active lesion and typically manifests as a solitary
painless papule that becomes ulcerated. Primary lesions ulcer (chancre) with indurated margins on the lip, tongue
most commonly occur on the genitalia and are frequently or palate. The ulcer is usually deep, and is accompanied by
accompanied by regional lymphadenopathy. The primary cervical lymphadenopathy. The chancre usually heals spon-
lesion usually resolves spontaneously within 4–6 weeks, taneously within 7–10 days.
612
In secondary syphilis, maculopapular and nodular muco- exudate is of limited value in oral lesions because other com-
sal lesions are common. Superficial mucosal erosions, called mensal Treponema species found in the human mouth can
mucous patches, may be seen on the lips, oral mucosa, be confused with T. pallidum. There are two types of sero-
tongue, palate and pharynx. The mucous patches are typi- logical tests for syphilis: treponemal and non-treponemal.
cally painless, oval to crescentic erosions, surrounded by a Commonly used non-treponemal tests include the rapid
red periphery. Snail track ulcers are serpiginous lesions plasma reagin (RPR) and venereal disease research labo-
that may arise de novo, or form by coalescence of a num- ratory (VDRL) tests. The non-treponemal tests measure IgG
ber of mucous patches. Condylomata lata are broad-based and IgM directed against the cardiolipin–lecithin–choles-
verrucous plaques which can be seen in secondary syphilis terol antigen complex and are used for screening or for
of the oral cavity. quantification of serum antibody. Treponemal tests are used
The oral manifestations of tertiary syphilis are mostly due for confirmation of reactive non-treponemal results and
to gumma formation. Gummas are caused by endarteritis include fluorescent treponemal antibody-absorbed (FTA-
obliterans and tend to involve the hard palate, tongue or ABS) test and T. pallidum particle agglutination assay
lower alveolus. The gummas form swellings which may (TPPA). The treponemal tests are qualitative tests and are
coalesce to form serpiginous lesions and eventually ulcerate, not used in assessing treatment responses.
resulting in bone destruction, palatal perforation and oro-
nasal fistula. Intraosseous gummas appear as ill-defined Treatment
radiolucent lesions. Interstitial glossitis is rare and is the
Parenteral long-acting penicillin G is the drug of choice
result of contracture of the tongue musculature after healing
for all stages of syphilis. Resistance to penicillin has not
of a gumma. An association between interstitial glossitis
been described. Other antibiotics effective against syphilis
and oral squamous cell carcinoma of the tongue has been
include the macrolides such as erythromycin and azithro-
suggested, but this may be due to the carcinogenic agents
mycin, as well as tetracycline antibiotics, such as tetracy-
formerly used to treat syphilis (arsenicals and heavy metals)
cline and doxycycline. Unfortunately, with increased use
rather than the infectious agent. Tertiary syphilis can also
of azithromycin for many infections, there has been an
give rise to both unilateral and bilateral trigeminal neuropa-
increased prevalence of macrolide-resistant T. pallidum.
thy and facial nerve palsy.
Vaccines are not available for this disease. Testing for HIV
Oral manifestations of congenital syphilis include
status in affected patients is also recommended.
Hutchinson teeth which are screwdriver-shaped incisors that
may show notching of the incisal edge. ‘Mulberry’ molars
have multiple poorly developed cusps. The facies of patients
with congenital syphilis show frontal bossing, saddle nose, DEEP FUNGAL INFECTIONS
and poorly developed maxillae. Rhagades are linear scars at
the angles of the mouth caused by secondary bacterial infec- Fungal infections or mycoses usually are superficial and
tion of a facial rash occurring in early congenital syphilis. involve the skin, hair, nails and mucous membranes. In
some cases, especially in immunocompromised patients,
Histopathologic features disseminated or deep fungal infections can cause extensive
tissue destruction. In immunocompetent hosts, deep fungal
Primary and secondary syphilis are characterized by dense infections can heal or remain latent for prolonged periods
plasma cell infiltrates, especially in a perivascular distribu- of time. Many fungal infections can initiate a granulomatous
tion, and by capillary endothelial proliferation and oblit- inflammatory response in the host as discussed below.
eration of small blood vessels. However, the microscopic
features are not specific for syphilis. Plasma cells are com-
mon in oral biopsies, especially those taken from the gingiva. Blastomycosis
The Warthin–Starry method is a silver impregnation tech-
Blastomycosis is caused by Blastomyces dermatitidis, a
nique which can be used to demonstrate T. pallidum, but
dimorphic fungus that grows in soil and decaying wood.
the stain is not specific and may label other spirochaetes
The organism is endemic in some parts of the United States
which inhabit the oral cavity. Gummas are composed of
and Canada, but infections are also seen in Mexico, Middle-
aggregates of epithelioid and giant cells, forming granulomas,
East, Africa and India. Infection occurs through inhalation
usually with a prominent plasma cell infiltrate. T. pallidum
of the conidiae (asexual spores). In the lungs at body tem-
is rarely demonstrated in gummas.
perature, the conidiae are transformed into yeasts that
multiply through budding. Yeasts represent the pathogenic
Diagnosis
forms of the organism. The infection usually is contained
The diagnosis of syphilis is usually based on clinical signs in the lungs and most patients are either asymptomatic or
and symptoms and serologic tests. T. pallidum cannot be develop mild non-specific flu-like symptoms. In some
detected by culture. Dark-field microscopic examination of patients, hematogeneous spread can occur.
613
Figure 8 Figure 9
Photomicrograph showing Blastomyces dermatitidis. The

organism appears as round yeast with thick refringent cell
wall. (Hematoxylin and eosin, original magnification 200) Periodic acid-Schiff (PAS) stain showing Blastomyces
dermatitidis in the lung of a patient who died of disseminated
blastomycosis. (Original magnification 400)
Three clinical forms of blastomycosis are recognized:
pulmonary blastomycosis, disseminated blastomycosis and
cutaneous blastomycosis which is very rare. Pulmonary
disease can be acute or chronic. Acute pulmonary blasto-
mycosis presents clinically with productive cough, chest Figure 10
pain, dyspnea, fever and night sweats. Chronic pulmonary
blastomycosis can be mistaken for tuberculosis, sarcoid-
osis, or even malignancy. Oral involvement is rare and can
present as ulcers or exophytic mucosal lesions. The clini-
cal differential diagnosis includes oral squamous cell car-
cinoma and biopsy usually is needed to exclude this
possibility.
The diagnosis of blastomycosis is based on the identifi-
cation of B. dermatitidis in a tissue biopsy or a cytological
smear of an infected body fluid. The organism appears as
a round yeast cell which divides by broad-based budding.
It has a thick, refringent cell wall (Figure 8). Biopsy from
lesional tissue shows a mixed acute and granulomatous
inflammation. Special stains such as periodic acid-Schiff
(PAS) (Figure 9) and methenamine silver (Figure 10) can be
used to identify the organism in tissue samples. Skin or Methenamine silver stain of the same case shown in
mucous membrane lesions show marked pseudo-epitheli- Figure 9. Note the broad-based budding of the yeast.
omatous hyperplasia. Diagnosis can be confirmed by cul- (Original magnification 400)
ture as well, but this may take several weeks. Highly
specific and sensitive DNA probes can be used to identify
the organism, a few days after culture.
Treatment is based on the severity of the disease. Patients bat droppings, and hence rich in nitrogen. The organism is
with mild to moderate illness are treated with itraconazole. endemic in some areas of the United States, especially
Those with severe disease, meningeal lesions and immuno- in the Mississippi and Ohio River Valleys. It can also be
compromised patients are managed with amphotericin B. seen in other parts of the world, including central America,
Asia and some Mediterranean countries. Disease occurs
through inhalation of the microconidia, the infectious
Histoplasmosis
form of the fungus. Macrophages, the main target of infec-
Histoplasmosis is caused by Histoplasma capsulatum, a tion, can be destroyed by the intracellular multiplication
dimorphic fungus found in soil contaminated with bird or of the pathogen.
614
The severity of the disease is proportional to the amount

Figure 11
of inhaled spores and to the integrity of the immune response
of the host. Therefore, immunocompromised patients, such
as those with AIDS, organ transplant recipients, and those
with hematological malignancies may develop dissemi-
nated histoplasmosis, with involvement of extrapulmonary
sites.
Acute pulmonary infection presents with fever, dys-
pnea, productive cough and anterior chest discomfort.
Granuloma formation and coagulative necrosis can result
in cavitation of lung tissues. Healing of the granuloma-
tous lesions can cause fibrosis and concentric calcifi-
cations. A chronic progressive form of histoplasmosis
exists as well and resembles tuberculosis clinically. The
most common form of histoplasmosis, however, is an
asymptomatic pulmonary infection or a mild respiratory
illness.
Oral involvement is seen mainly in disseminated dis- Aspergillosis of the maxillary sinus in an
ease in the form of ulcers, red or white lesions. Oral ulcers immunocompromised patient. Note the septate hyphae
secondary to histoplasmosis may resemble squamous cell that branch at acute angles. (Hematoxylin and eosin,
carcinoma or tuberculous ulcers. original magnification 400)
The diagnosis of histoplasmosis is based on culture,
identification of the organism in tissue samples, and sero- responsible for the majority of invasive infections in
logic tests. Growth of the organism in culture can take immunocompromised patients.
several weeks. More rapid identification can be achieved Diagnosis of aspergillosis is based on the clinical signs
with specific DNA probes. Microscopic examination of and symptoms, individual patient risk, culture and histo-
lesional tissue can show granulomas and multinucleated pathology. Microscopically, multiple septate hyphae,
giant cells or a diffuse, mixed inflammatory infiltrate dom- 3–4 m in diameter, with a tendency to branch at acute
inated by macrophages. Special stains confirm the pres- angles and with occasional fruiting bodies can be seen
ence of small, uniform and oval budding yeast. (Figure 11). Aspergillus has a tendency to invade blood
Treatment is based on the severity of the disease. vessels, which explains the presence of areas of hemor-
Mild-to-moderate forms of histoplasmosis are treated with rhage and infarction in involved tissues. A non-caseating
itraconazole, while disseminated disease and immunocom- granulomatous inflammatory response with giant cells can
promised patients require treatment with amphotericin B. be seen in immunocompetent patients. It can be intense
enough to cause pressure necrosis of adjacent bone.
Aspergillosis Until recently, amphotericin B and itraconazole were
the standard therapeutic regimen for patients with inva-
Aspergillosis is a fungal infection caused by members of sive aspergillosis. A recent trial showed that voriconazole
the Aspergillus species which are ubiquitous saprophytic is more effective in these cases and is associated with bet-
molds growing on organic matter. Sporulation of the mold ter survival and fewer side effects. Patients with allergic
produces large numbers of conidia that can be inhaled by fungal sinusitis may require surgical debridement, systemic
humans without adverse consequences in the majority of antifungal treatment and systemic or topical steroids.
cases. In some patients, the inhaled organisms can cause Aspergilloma requires surgical debridement.
allergic fungal sinusitis, allergic pulmonary aspergillosis
or asthma. A mass of fungal hyphae, called aspergilloma,
Zygomycosis (Mucormycosis, Phycomycosis)
can form in the sinuses or the lungs of patients suffering
from cavitary tuberculosis, bronchiectasis or lung abscesses. Zygomycosis is a fungal infection caused by zygomycetes,
Immunocompromised patients, such as individuals with especially those belonging to the order of Mucorales. The
AIDS, bone marrow or organ transplant recipients, and Rhizopus species and the Mucor species belonging to the
patients with hematological malignancies, can develop Mucoraceae families of Mucorales are the most common spe-
invasive disease, where primary disease of the lungs is fol- cies causing human zygomycosis. Rare human infections can
lowed by widespread hematogeneous dissemination and be caused by the enthomophthorales order of zygomycetes.
multiple organ involvement. Mucorales fungi are ubiquitous and can grow on organic
Aspergillus fumigatus and A. flavus are the most com- matter including bread, fruits, vegetables and soil. They pro-
mon species to cause human aspergillosis. A. fumigatus is duce abundant spores that can be spread in the environment
615
Figure 12 FOREIGN BODY GRANULOMAS
A number of foreign materials of endogenous or exogenous

origin, which cannot be phagocytosed by macrophages
because of their size or composition, can induce a granulo-
matous inflammatory response in tissues. Examples include
silica, beryllium, glass, talc, starch, suture material, and a
large number of dental materials, such as pumice, gutta
percha, endodontic sealers, and some impression materials.
Some endogenous substances such as hair, keratin, amy-
loid and calcifications can also induce granulomatous
inflammation in tissues. Macrophages accumulate in the
site, become activated, and differentiate into epithelioid
cells. Some of the macrophages fuse to form foreign body
giant cells. Granulomas form and envelop the foreign
material, which may be seen in the cytoplasm of some
macrophages and giant cells.
Zygomycosis of the mandible in a patient undergoing
chemotherapy. The photomicrograph shows multiple Clinical features
broad, thin-walled, aseptate hyphae that tend to branch
irregularly or at right angles. (Hematoxylin and eosin, The clinical manifestations of foreign body reactions are
original magnification 400) usually non-specific and vary from a localized mass to
superficial erosions and ulcerations. In the oral cavity, the
gingiva seems to be the most common site of involvement
and inhaled by humans. Infection rarely occurs in and the condition has been termed granulomatous or for-
immunocompetent patients. The most common underlying eign body gingivitis. Dental materials are the most com-
risk factors for zygomycosis are uncontrolled insulin- mon causative factors in these cases, but other substances
dependent diabetes mellitus (IDDM) with metabolic acido- including hair and nails have been reported. The condition
sis, immunosuppressive treatment, prolonged systemic presents clinically as a localized change in gingival color,
corticosteroid use, solid-organ and hematopoietic stem ulceration or diffuse gingival erythema. Lesions start at
cell transplantation, persistent neutropenia and iron- the interdental papilla and extend laterally. An important
chelating treatment. diagnostic clue is that the condition does not regress with
The most common human manifestation of this infec- improvement of oral hygiene measures.
tion is rhinocerebral zygomycosis that affects the nose,
maxillary sinus, midface with frequent extension into the Histopathologic features
brain. Sinonasal disease causes nasal obstruction, rhinorrhea,
Biopsy material from the gingiva can show granulomas and
facial pain and facial swelling. Oral mucosal involvement
foreign body giant cells in the absence of microorganisms.
presents with ulceration. Typically, the base of the ulcer is
The foreign substance can be identified in hematoxylin
black and massive tissue destruction and necrosis can be
and eosin stained sections (Figure 13) or by polarized light
seen in untreated patients.
microscopy. Energy-dispersive radiographic microanalysis
The diagnosis of zygomycosis relies on the identifica-
can help confirm the presence of foreign material in tissue
tion of the broad, thin-walled, irregularly-branching and
sections and identify its nature. In the absence of identifi-
mostly aseptate hyphae in affected tissues. Branching
able foreign material, other causes of granulomatous
occurs at right angles (Figure 12). The organism has high
inflammation have to be investigated. In some cases, the
affinity for blood vessel invasion, which explains the mas-
inflammatory response is lichenoid in appearance and the
sive tissue infarction and necrosis observed clinically and
foreign body is too small to be identified microscopically.
microscopically.
A diagnosis of lichen planus can therefore be rendered,
Treatment of zygomycosis necessitates management of
but the lesions will not respond to conventional treatment
the underlying medical condition, use of effective antifun-
of lichen planus.
gal treatment and surgical debridement. Amphotericin B is
the treatment of choice. Posaconazole seems to provide
encouraging results, especially in prophylaxis. Debridement
is necessary because antifungal treatment cannot reach The treatment of foreign body reactions includes the elim-
infected tissues, and correction of the surgical defects is ination of the offending agent and excision of involved
needed once active disease is controlled. tissues. This results in adequate clinical response in the
616
The role of genetic factors is suggested based on disease

Figure 13
association with distinct HLA alleles. Other genes involved
in controlling the immune response can play a role in dis-
ease pathogenesis as well.
Granulomatous vasculitis of the upper airways and lungs
suggests that the disease may represent a cell-mediated
reaction to an inhaled antigen of bacterial, viral or envi-
ronmental origin. Chronic nasal carriage of S. aureus has
been linked to the initiation and relapse of WG through a
number of molecular mechanisms implicating B cells,
T cells and neutrophils.
Clinical features
WG affects mainly the upper and lower respiratory tracts.
In generalized WG, renal disease develops rapidly. Limited
WG refers to disease restricted to the respiratory system
without rapid renal involvement. In some cases, the disease
Photomicrograph of a foreign body reaction following
reconstructive surgery in the temporomandibular joint
is restricted to the skin and mucosa. This has been termed
showing a large number of multinucleated giant cells superficial WG.
engulfing a foreign material. Typical granulomatous Pulmonary involvement is seen in more than 80% of
inflammation was seen elsewhere in the specimen. patients who may present with cough, hemoptysis, dys-
(Hematoxylin and eosin, original magnification 100) pnea and chest discomfort. In asymptomatic patients, abnor-
mal chest radiograph may be the only manifestation of the
disease. Renal disease develops in the majority of patients
(77%), and is the most common cause of morbidity and
majority of cases. If a diagnosis of granulomatous gingivitis mortality. Early glomerulonephritis presents with protein-
is made in the absence of an identifiable foreign material, uria, hematuria and red blood cell casts in urine. Disease
the patient should be investigated for other causes of progression leads to renal failure, especially if no treatment
granulomatous inflammation. is administered. Active disease is characterized in general
by constitutional symptoms such as malaise, fever, night
sweats and weight loss. Other organs can be involved by the
WEGENER’S GRANULOMATOSIS granulomatous vasculitis, including peripheral and cranial
nerves, heart, eyes and skin.
Wegener’s granulomatosis (WG) is a systemic autoimmune Involvement of the head and neck region is very com-
disease that is invariably fatal if left untreated. The disease mon in WG. The nasal cavity and paranasal sinuses are
may occur in generalized and limited forms, and consists affected in 60–90% of the cases, with symptoms of nasal
of the classical triad of (i) necrotizing granulomatous obstruction, sinusitis and headache, purulent nasal dis-
inflammation involving the upper respiratory tract, the charge, epistaxis, and mucosal ulceration. Nasal septal
lower respiratory tract, or both, (ii) necrotizing glomeru- perforation can cause saddle nose deformity. The ears, the
lonephritis, and (iii) systemic vasculitis involving small to eyes, the larynx and the oral cavity can be affected as well.
medium-sized vessels (capillaries, venules, arterioles, or Eustachian tube blockage may result in persistent serous
arteries). otitis media. Laryngeal disease can result in subglottic steno-
WG is an uncommon disease, with an estimated preva- sis which can lead to upper airway obstruction requiring
lence of 3.0 per 100,000 persons in the United States. It is emergency tracheostomy. Salivary gland involvement is
more common in whites than in blacks and affects both uncommon, but has been reported in WG.
sexes equally. The mean age at onset is approximately
40 years, but the disease can occur at any age, with 15% Oral manifestations
of cases occurring in patients younger than 19 years.
Oral involvement is quite common in WG and it may be
the first sign of the disease. Strawberry gingivitis, one of
Etiology and pathogenesis
the characteristic signs of WG, is thought to be an early
The etiology of WG has not been fully elucidated. Genetic manifestation of the disease, occurring before renal involve-
factors, exposure to environmental antigens such as silica ment. Clinically, strawberry gingivitis appears as hyper-
and infection (in particular with Staphylococcus aureus) plastic granular and friable gingival lesions with multiple
seem to play an important role in disease pathogenesis. surface petechiae. The buccal surface of the gingiva is more
617
frequently involved. The process originates in the inter- diseases. Furthermore, a negative c-ANCA titer does not
dental papillae and extends laterally to involve the rest of exclude the diagnosis of WG. Changes in ANCA titers
the gingiva. Periodontal bone loss has been described and appear to reflect disease activity and rising titers can predict
can result in tooth mobility and extraction. Non-healing relapse in treated patients although this is not a consistent
of extraction sockets is possible. Oral ulcers affecting any finding.
mucosal surface can be seen in WG, but are non-specific
and seem to develop at an advanced stage of the disease, Management protocols and prognosis
usually after renal involvement occurs. Necrosis and ulcer-
Wegener’s granulomatosis is typically treated with long-
ation of the palate can be seen.
term cyclophosphamide given in oral doses together with
glucocorticoids which produces rapid and marked improve-
ment in the majority of patients. Recently, it was shown
Wegener’s granulomatosis is characterized microscopi- that rituximab can play a role in the induction of remis-
cally by granulomatous inflammation, geographic necro- sion. Less toxic drugs such as azathioprine, methotrexate
sis and vasculitis. The granulomas are ill-defined, surround and leflunomide can be used to maintain remissions.
areas of necrosis, and are not as compact and as defined Approximately half of remissions are followed with one
as those seen in tuberculosis and sarcoidosis. In lung biop- or more relapses, and many patients develop some degree
sies, areas of necrosis are surrounded by a shell of fibro- of morbidity either from irreversible features of their dis-
blastic, inflammatory and giant cells, producing palisading ease or from the toxic side effects of treatment. Patients may
granulomas. The inflammatory infiltrate can be dense, suffer from varying degrees of renal insufficiency or impaired
mixed, and non-specific and overshadow the underlying upper respiratory tract function. Cyclophosphamide-related
pathology. Special stains do not show microorganisms and toxicities include cystitis, bladder cancer, myelodysplasia
foreign body cannot be identified by polarizing light and infertility. Diabetes mellitus, cataracts, infectious com-
microscopy. Affected vessels show a transmural inflam- plications and osteoporosis are some of the glucocorticoid-
matory reaction, with focal or diffuse polymorphonuclear associated side effects.
infiltrates causing vessel wall destruction and fibrin depo-
sition within the walls and the lumen. The necrosis seen
seems to be secondary to the vasculitis. SARCOIDOSIS
Oral mucosal biopsies show an inflammatory infiltrate
composed mainly of neutrophils and eosinophils that occa-
Sarcoidosis is a relatively common multisystem immune-
sionally form microabcesses. Giant cells are common and
mediated disease primarily affecting the lungs and lym-
ill-defined granulomas can be seen. Vasculitis is less prom-
phatic systems. It is characterized by the presence of
inent in mucosal biopsies from the head and neck region,
non-caseating granulomas in affected organs. The etiol-
including the mouth, mainly due to the small size of these
ogy of sarcoidosis is unknown, but familial, spatial (e.g.
biopsies and lack of larger vessels in mucosa. Strawberry
among people in the same household), seasonal and occu-
gingivitis shows pseudoepitheliomatous hyperplasia and a
pational clustering of the disease have been reported, sug-
prominent vascular component associated with red blood
gesting multiple underlying factors, including genetic
cell extravasation.
predisposition, infectious agents and environmental expo-
sures. Possible microbial triggers include mycobacterial
and propionibacterial organisms, with the mycobacterial
The diagnosis of WG is based on history, clinical presenta- catalase-peroxidase (mKatG) protein as a potential candi-
tion, laboratory test results and microscopic findings of date antigen. Sarcoidosis likely develops from an exagger-
necrotizing granulomatous vasculitis in a biopsy specimen. ated cellular immune response (acquired, inherited or both),
Laboratory tests show an elevated erythrocyte sedimenta- to a limited class of persistent antigens or self-antigens.
tion rate (ESR), high C-reactive protein, leukocytosis and Space-occupying granulomas form from the accumulation
thrombocytosis. Renal involvement can result in urinary of mononuclear inflammatory cells, mostly CD4 Th1 lym-
sediment, erythrocyturia, proteinuria and high serum cre- phocytes and mononuclear phagocytes in affected organs.
atinine levels. Chest radiographs and computerized tomog- Organ dysfunction and tissue injury result from physical
raphy can confirm the presence of pulmonary infiltrates or distortion of the tissue by the granulomas.
nodules. Detection of c-ANCA by indirect immunofluores- Sarcoidosis is found throughout the world, but is most
cence and confirmation of the specificity of ANCA against prevalent in United States and Scandinavian populations.
proteinase-3 (PR3-ANCA) by ELISA yields a specificity of In the United States there is a three-fold to five-fold increased
99% and a sensitivity of 73% in the diagnosis of WG. incidence in blacks compared to whites. Geographic, eth-
Identification of c-ANCA is an adjunctive test, as false pos- nic and genetic factors are linked to the specific clinical
itive results can be seen in some infectious and neoplastic characteristics of patients with sarcoidosis. Blacks are more
618
likely to have severe musculoskeletal or constitutional

Figure 14
symptoms on presentation, while whites appear to have
higher rates of asymptomatic disease, disease limited to
the chest and erythema nodosum.
Clinical features
Sarcoidosis is more common in young and middle-aged
adults, with approximately 75% of the cases in individuals
younger than 40 years. Females appear to be slightly more
susceptible than males. Sarcoidosis has a variable clinical
presentation and course, and can affect many organs and
tissues, but the lungs are most commonly affected. Unlike
many lung diseases, sarcoidosis favors non-smokers. Other
organ systems that may be affected by sarcoidosis include
the heart, liver, spleen, bones, skin, eyes, lymph nodes,
parotid glands, and uncommonly, the oral cavity. The
majority of patients with sarcoidosis are asymptomatic,
with the disease being discovered on routine chest radio-
graphs. Symptomatic sarcoidosis may develop abruptly
over a period of a few weeks. Less frequently sarcoidosis Photomicrograph of a granulomatous inflammatory
arises insidiously over months or years without significant lesion involving the lung of a patient with sarcoidosis.
symptoms. Symptomatic patients usually present with Well-formed non-caseating granulomas are noted and
respiratory and skin manifestations or with constitutional are surrounded by a rim of lymphocytes. (Hematoxylin
and eosin, original magnification 200)
and non-specific symptoms such as fever, night sweats,
fatigue and malaise. Dry cough, dyspnea and chest pain
are frequent respiratory complaints. Cutaneous manifesta-
tions occur in approximately 25% of patients and include
sicca, can mimic Sjögren’s syndrome. Intraosseous lesions
erythema nodosum and lupus pernio. Erythema nodosum
are less common than soft tissue lesions, accounting for
are scattered tender erythematous nodules occurring fre-
approximately one-fourth of all reported intraoral cases.
quently on the lower legs, while lupus pernio represents
Either jaw may be affected. Intraosseous lesions typically
chronic, violaceous lesions involving the face, limbs, back
appear as non-expansile ill-defined radiolucent areas which
and buttocks. Ocular symptoms may be due to anterior
may be accompanied by tooth mobility due to alveolar
uveitis or lacrimal gland involvement resulting in kerato-
bone loss.
conjunctivitis sicca. Two distinctive clinical syndromes
are associated with acute sarcoidosis. Löfgren syndrome, a
form of acute sarcoidosis usually found in white females,
consists of erythema nodosum, bilateral hilar lymphade- Lesions of sarcoidosis consist of tightly clustered aggre-
nopathy and arthralgia. Heerfordt syndrome (uveoparotid gates of epithelioid histiocytes surrounded by a rim of lym-
fever) is characterized by parotid enlargement, anterior phocytes (Figure 14). Multinucleated giant cells of the
uveitis, facial paralysis and fever. Langhans type or foreign body type are intermixed with
the histiocytes. Necrosis is typically absent (Figure 15). The
Oral manifestations giant cells may contain laminated calcified structures called
Schaumann bodies or stellate inclusions known as asteroid
Excluding salivary gland and lymph node involvement,
bodies. Special stains for fungal and bacterial organisms
oral manifestations of sarcoidosis are uncommon, and the
are negative. Polarizable, dissolvable and pigmented foreign
disease is typically diagnosed before oral symptoms appear.
materials are not detectable.
Sarcoidosis may involve any oral mucosal site, most com-
monly buccal mucosa, followed by gingiva, lips, floor of
mouth, tongue and palate. The lesions most commonly pres-
ent as submucosal masses, which may vary in color from The diagnosis is based on clinical and radiographic find-
normal to brownish-red or violaceous or they may be hyper- ings, histopathologic evidence of non-caseating epitheli-
keratotic. Sarcoidosis of the major and minor salivary oid granulomas, and exclusion of other known causes of
glands may result in xerostomia. Sarcoidosis can cause granulomatous inflammation. Chest radiographs may show
bilateral enlargement of the major salivary glands which, bilateral hilar lymphadenopathy, diffuse parenchymal infil-
in conjunction with xerostomia and keratoconjunctivitis trates, or both, and 90% of patients have abnormal chest
619
The overall prognosis of sarcoidosis is good. Spontane-

Figure 15
ous resolution is common and in most patients symptoms
resolve spontaneously within 2 years without treatment.
Poor prognostic indicators include chronic disease, older age
at onset, black race, lupus pernio, neurosarcoidosis, cardiac
involvement and advanced pulmonary disease. Approxi-
mately 4–10% of patients die of progressive respiratory,
central nervous system or cardiac involvement.
OROFACIAL GRANULOMATOSIS
Orofacial granulomatosis (OFG) is a clinical and patho-

logic term introduced by Wiesenfeld in 1985 to describe
a group of conditions affecting the oral and maxillofa-
cial region, and characterized microscopically by non-
caseating granulomatous inflammation. The spectrum of
Photomicrograph of sarcoidosis showing absence of necrosis. OFG includes cheilitis granulomatosa (CG) of Miescher,
Scattered giant cells are noted within the granulomas. Melkersson–Rosenthal syndrome (MRS), Crohn’s disease,
(Hematoxylin and eosin, original magnification 100)
sarcoidosis and other granulomatous inflammatory condi-
tions that could affect this region. Some cases of OFG can
radiographic findings sometime during the course of their develop secondary to a chronic dental infection or to a
disease. Lung function abnormalities typically found include contact hypersensitivity reaction while others appear to be
decreased lung volumes and diffusing capacity. Elevated idiopathic. A preliminary diagnosis of OFG should be fol-
serum angiotensin-converting enzyme (ACE) levels support lowed by a thorough clinical and laboratory investigation
the diagnosis of sarcoidosis, but this test lacks sensitivity to identify and treat any possible underlying local or sys-
and specificity. Elevated serum calcium concentration and temic disease and thus idiopathic OFG is a diagnosis
urinary calcium level may be found. The Kveim test, a skin acquired by exclusion.
test for sarcoidosis involving intradermal injection of The etiology of OFG is unknown and the disease is
human spleen extract of sarcoid tissue, is no longer used thought to represent an abnormal immune reaction. Infec-
because of concerns of transmission of infectious diseases. tion, allergy and genetic predisposition have been sug-
Minor salivary gland biopsy may be helpful in suspected gested. Monoclonal lymphocytic expansion, which may be
cases of sarcoidosis, with success rates between 19% and secondary to chronic antigenic stimulation, has been iden-
58%. However, parotid biopsy provides a better diagnostic tified in OFG. Cytokine production by the monoclonal lym-
yield, with confirmation of sarcoidosis reported in 93% of phocytic proliferation could stimulate granuloma formation.
patients following this procedure. The spectrum of OFG encompasses cheilitis granuloma-
tosa (CG) of Miescher and Melkersson–Rosenthal syn-
Management protocols and prognosis drome (MRS). Systemic conditions such as tuberculosis,
Crohn’s disease, sarcoidosis and other granulomatous
The diagnosis of sarcoidosis usually is followed by a inflammatory conditions could present with granuloma
3- to 12-month period of observation to define the general formation in the oral and maxillofacial region.
course of the disease. Immediate medical treatment is indi-
cated for patients with neurological, cardiac, severe ocular,
Clinical features
advanced pulmonary and disfiguring cutaneous disease, as
well as persistent hypercalcemia. The standard treatment The most consistent finding in OFG is a painless, persistent
for sarcoidosis is systemic corticosteroids. Other immuno- diffuse swelling involving one or both lips (macrochelia).
suppressive and immunomodulating drugs including tumor The swelling can be unilateral or involve the whole lip
necrosis factor antagonists, have been used, but definitive (Figure 16). In the early phases of the disease, the swelling
evidence of their efficacy is lacking. Xerostomia second- is usually soft, intermittent and recurrent. Later, the swell-
ary to sarcoidosis of the major salivary glands predisposes ing becomes permanent and fibrotic. Generalized edema,
patients to caries, periodontal disease and candidiasis, neces- erythema and non-specific erosions and ulcerations may
sitating preventative measures, salivary stimulants, topical be seen in the mouth. Gingival swelling can be seen in some
fluoride and antifungal medications. Systemic corticoste- cases. Some patients develop swelling elsewhere in the face,
roid therapy can result in adrenal suppression requiring with or without lip involvement, making the diagnosis
special precautions before oral surgical interventions. more difficult. Other reported manifestations of OFG include
620
Figure 16 Figure 17
Diffuse lip swelling in a patient with orofacial granulomatosis.

Slight erythema was noted on the facial skin
Photomicrograph of a buccal mucosal biopsy showing
multiple non-caseating epithelioid granulomas with
multinucleated giant cells. An intense lymphoplasmocytic
fissures of the tongue, taste alterations, decreased salivary infiltrate is associated with the granulomas. (Hematoxylin
production and a cobblestone appearance of the buccal and eosin, original magnification 100)
mucosa.
When the swelling is limited to the lips, the term CG is
used by some clinicians and when it is associated with a exclusion of possible causes of granulomatous inflamma-
fissured tongue and a history of recurrent facial paralysis tion should be followed by a systematic work-up, including
a diagnosis of MRS can be applied. It is better, however, to clinical, laboratory and radiographic investigations, to
limit the use of these terms since they are considered rule out underlying local or systemic disease.
as subsets of OFG rather than specific disease entities. It is All oral foci of infection should be identified and
also useful to use terms such as OFG in the context of treated. Elimination diet and patch testing against poten-
Crohn’s disease or sarcoidosis, OFG secondary to contact tial allergens such as food additives, cosmetics, fragrances
hypersensitivity reaction, or idiopathic OFG to standardize and dental hygiene products should be performed if con-
terminology. tact hypersensitivity reaction is suspected. Chest radio-
graphs and serum levels of angiotensin-converting enzyme
Histopathologic features can be obtained to screen for evidence of sarcoidosis.
Complete blood count, ESR, and serum levels of folic acid,
Orofacial granulomatosis is characterized by the presence
vitamin B12, and iron are useful in patients with unusual
of non-caseating epithelioid granulomas, usually with
gastrointestinal manifestations. These patients also should
multinucleated giant cells (Figure 17). The granulomas may
undergo specialized gastrointestinal examination to assess
be concentrated around blood vessels, or they may be scat-
for Crohn’s disease. If this investigation is negative, it can
tered in the connective tissue and lamina propria. Sometimes,
be repeated later, especially if clinically indicated and in
minor salivary gland involvement can be seen. A perivascu-
younger patients with OFG as these patients are more
lar lymphocytic infiltrate with marked dilation of lymphatic
likely to have concomitant intestinal disease. Tuberculin
channels and marked edema of the superficial lamina pro-
test and chest radiographs should be performed even if
pria is seen in some cases, and can be useful when typical
acid-fast stains were negative on histology to exclude
granulomas are absent. Acid-fast bacilli and fungal organ-
tuberculosis.
isms cannot be identified with special stains and foreign
material is not seen with polarized light microscopy.
Intralesional corticosteroid injection is the treatment of
Special stains, such as the PAS stain, Grocott methena- choice in OFG. The response to intralesional treatment is
mine silver stain, Ziehl–Neelsen and Gram stain applied to usually fast, but relapses are common, and therefore
the lesions of OFG do not show fungal or specific bacterial repeated injections are needed. Systemic corticosteroids
organisms. Foreign body is not demonstrable within the are effective, but their long-term use is limited by the
lesions with polarized light microscopy. The microscopic recurrent and chronic nature of the disease and by their
621
serious side effects. Other therapeutic measures have been endoplasmic reticulum and metabolic stress, regulation of
used with variable success, including hydroxychloroquine, adaptive immunity and inflammation.
methotrexate, clofazimine, metronidazole, minocycline alone The role of genetics in disease development is actively
or in combination with oral prednisone, thalidomide, dap- investigated and susceptibility genes such as NOD2/CARD15,
sone and danazol. One study showed that the impact of IL23R, ATG16L1 and others have been identified. Of these,
dietary manipulation in patients suffering from OFG can the NOD2/CARD15 gene (caspase recruitment domain fam-
be significant, especially in the presence of oral mucosal ily member 15) is the most replicated and understood. It
inflammation. Other investigators reported improvement plays a role in regulating innate immune responses, bacte-
of oral manifestations upon removal of amalgam restora- rial killing, immune responses to endogenous microbial
tions, but this has not been reproduced in controlled stud- antigens and epithelial function. NOD2 (nucleotide-bind-
ies. Surgical intervention (cheiloplasty) is performed when ing oligomerization domain containing 2) is an intracel-
the response to medical treatment is weak and in severely lular protein that senses bacterial products and activates
disfiguring cases. It is not a first-line therapy because of components of the innate immune system.
the high risk of recurrence.
The treatment of OFG is difficult and often disappoint- Clinical features
ing due to the chronic nature of the disease and the high
Crohn’s disease is more prevalent in western countries than
risk of recurrence. Identifying the underlying causative factor
in developing countries and is more prevalent in northern
is not feasible in many cases, which increases the risk of
regions compared to southern regions. Urban areas have a
recurrence. Spontaneous remissions have been reported
higher prevalence of Crohn’s disease than rural areas. The
but do not seem to be very common.
disease frequency is highest in Ashkenazi Jews. Higher
economic status and active smoking increase the risk for
Crohn’s disease. The peak age of onset is between 15 and
CROHN’S DISEASE (Regional Ileitis, Regional 30 years, and a second peak occurs between the ages of
Enteritis) 60 and 80. The male-to-female ratio is 1:1 to 1.8:1.
Crohn’s disease can affect any part of the gastrointesti-
Etiology and pathogenesis nal tract from the mouth to the anus, with the terminal
ileum being involved in the majority of patients. Symptoms
Crohn’s disease is a chronic, relapsing, immunologically
commonly include long-standing diarrhea, abdominal pain,
mediated inflammatory bowel disorder. Its etiology and
weight loss, and some patients may experience non-specific
pathogenesis have not been clearly defined. Crohn’s disease
symptoms such as malaise, anorexia or fever. Transmural
is thought to be due to an inappropriate acquired T-cell
inflammation (inflammation affecting all layers) of the gut
immune response to certain commensal enteric bacteria
may result in fissures, abscesses, fistulas, thickening of the
developing in genetically susceptible hosts. Environmental
bowel wall and limited distensibility. Aphthous-like super-
factors seem to play a role in disease pathogenesis as well.
ficial ulcerations can be seen. These can fuse longitudinally
T cells implicated in Crohn’s disease are primarily acti-
and transversely around normal tissue to produce a ‘cobble-
vated CD4 Th1 lymphocytes which secrete cytokines
stone’ appearance of the bowel mucosa.
such as IL-12, IFN- and TNF. Although Crohn’s patients
Extraintestinal manifestations are relatively common
show loss of tolerance to enteric commensal bacteria, no
and include dermatologic conditions such as erythema
specific infectious etiology has been identified. The role of
nodosum, pyoderma gangrenosum, pyodermatitis vegetans,
environmental triggers such as smoking, use of antibiotics
and Sweet syndrome, a neutrophilic dermatosis. Rheuma-
and non-steroidal anti-inflammatory drugs (NSAIDs), stress
tologic manifestations include asymmetric polyarticular
and infection is not well understood, but these factors
migratory polyarthritis most often affecting the large
appear to play a role in precipitating the onset or the reac-
joints of the upper and lower extremities, and ankylosing
tivation of disease. Evidence for hereditary factors in the
spondylitis. Ocular complications include conjunctivitis,
pathogenesis of Crohn’s disease includes a concordance
anterior uveitis/iritis and episcleritis.
rate of approximately 50% in monozygotic twins and an
increased risk of the disease in relatives of patients with
Oral manifestations
Crohn’s disease.
Genes associated with innate immunity, such as Oral lesions of Crohn’s disease can vary and be relatively
NOD2 (nucleotide-binding oligomerization domain 2) and non-specific. These do not necessarily reflect intestinal
ATG16L1 (autophagy-related, 16-like) genes, have been disease activity. Oral involvement can occur at any time
implicated in Crohn’s disease. NOD2 is an intracellular during the course of the disease and may precede gastro-
protein that senses bacterial products and activates com- intestinal manifestations in as many as 30% of the cases.
ponents of the innate immune system. Other genetic loci Findings include diffuse or nodular swelling of the oral
linked to Crohn’s disease include several genes involved in and perioral tissues, a cobblestone appearance of the oral
622
Figure 18 Figure 20
A linear ulcer in the upper left vestibule of a young

Clinical appearance of pyostomatitis vegetans, presenting
patient with Crohn’s disease
diffuse erosive and erythematous lesions involving the labial
mucosa, the gingiva and the vestibule
Figure 19
Figure 21
Diffuse hyperplastic gingivitis in the same patient

shown in Figure 18. Biopsy confirmed the presence
of non-caseating granulomas
Photomicrograph of a gingival biopsy from the patient
shown in Figure 19 demonstrating multiple non-caseating
mucosa and deep linear ulcers involving the buccal vesti- granulomas with a large number of multinucleated giant cells.
(Hematoxylin and eosin, original magnification 100)
bule (Figure 18). Aphthous ulcers can be seen, but their
significance in Crohn’s disease is uncertain, as they occur
as frequently in the general population and in the same
and is characterized by the development of multiple yel-
age group affected by Crohn’s disease. Other reported
lowish, serpiginous pustules on erythematous oral mucosa
oral manifestations include fibroepithelial hyperplasia,
(Figure 20). The pustules often rupture, leading to erosions
granulomatous gingivitis (Figure 19), angular cheilitis,
and fissuring.
persistent submandibular and superficial cervical lymph-
adenopathy, and metallic dysguesia. Less than 1% of
patients with Crohn’s disease may develop diffuse stoma-
titis, with some cases caused by Staphylococcus aureus, In the bowel, Crohn’s disease is characterized by transmu-
and others being non-specific. A rare condition called ral non-necrotizing granulomatous inflammation. Oral
pyostomatitis vegetans may be associated with Crohn’s dis- lesions show non-necrotizing granulomas in the submucosa
ease. Pyostomatitis vegetans is usually seen on the buccal, similar to those seen in OFG (Figure 21). The severity of the
labial mucosa, soft palate, ventral tongue and facial gingiva granulomatous inflammation may vary tremendously from
623
patient to patient and from various sites in the same shows a variable and unpredictable response to topical
patient. Therefore, a negative biopsy at one site and time and systemic glucocorticoid therapy. Topical treatment
may not necessarily rule out a diagnosis of Crohn’s dis- may yield remission in almost 50% of patients and should
ease. Special stains should be performed to rule out the be used as first-line therapy in patients with asymptomatic
possibility of deep fungal or mycobacterial infection. intestinal disease, while intra-lesional corticosteroids and
systemic treatment should be considered when topical
Diagnosis treatment fails to control symptoms. Crohn’s patients have
an increased risk of colorectal cancer, non-Hodgkin’s lym-
A single gold standard for the diagnosis of Crohn’s disease is
phoma, squamous cell carcinoma of the skin and small
not available. The diagnosis is confirmed by clinical eval-
bowel cancer.
uation and a combination of endoscopic, histopathologic,
The management of Crohn’s disease is based on con-
radiographic and biochemical investigations. Serological
trolling acute flares followed by maintenance of clinical
tests for anti-Saccharomyces cerevisiae antibodies/perinuclear
remissions. This is achieved by the use of corticosteroids
ANCA (ASCA/ANCA) have a high specificity for the diag-
as first-line therapy followed by immunosuppressive drugs
nosis of Crohn’s disease if the pattern is positive (ASCA/
such as azathioprine, 6-mercaptopurine and methotrexate
ANCA). High serum levels of C-reactive protein are use-
for maintenance. Sulfasalazine and 5-ASA can be used to
ful for assessing a patient’s risk of relapse and may cor-
maintain remissions or for the treatment of mildly active
relate with disease activity.
disease, but their role is questioned and considered to be
modest. Budesonide, a locally active steroid with limited
systemic activity, can be used as single agent or in combi-
Current therapeutic options include anti-inflammatory and nation therapy to induce remissions in mild and localized
immunosuppressive medications, such as sulfasalazine, ileocaecal disease. Its role in maintenance is questioned.
5-ASA agents, prednisone, budesonide, azathioprine and TNF antagonists such as infliximab and adalimumab seem
6-mercaptopurine. Anti-TNF therapies such as infliximab, to be extremely effective against Crohn’s disease as they
adalimumab and certolizumab are effective against Crohn’s induce and maintain mucosal healing and reduce surgery
disease as they block TNF, a key inflammatory cytokine and hospitalization rates. However, in current treatment
and mediator of intestinal inflammation. Antibiotics, espe- protocols, they are reserved for patients resistant to ste-
cially metronidazole and ciprofloxacin, are used for their roids and immunosuppressors as well as for those with
anti-inflammatory and anti-infectious properties. Surgery severe fistulizing disease. This concept is challenged and
is required for most patients and is related to the duration use of TNF antagonists is tested for the treatment of early
of disease and site of involvement. Oral Crohn’s disease Crohn’s disease and for the maintenance of remissions.
624
CHAPTER
Sexually Transmitted
Diseases
Praveen BN, Nagaraj A, Ravikiran Ongole
22
➧ Fellatio Syndrome ➧ Condyloma Acuminatum
➧ Traumatic Lesions of Lingual Frenum ➧ Oropharyngeal Gonorrhea
➧ Syphilis or Lues ➧ Oropharyngeal Chlamydial Infection
➧ Human Immunodeficiency Virus Infection ➧ Oropharyngeal Trichomonal Infection
➧ Intraoral Molluscum Contagiosum
Sexually transmitted diseases (STDs) or sexually transmitted FELLATIO SYNDROME

infections (STIs) or venereal diseases are infections that
can be transferred from one person to another through sex- It is described as submucosal hemorrhage secondary to
ual contact. repetitive negative pressure and/or blunt trauma associ-
Sexually transmitted diseases have been a part of ated with fellatio (Terezhalmy et al, 2000).
human existence from ages. However, in the last couple of These oral lesions are typically found in sexually active
decades with changing sexual practices among hetero- adults. However, presence of such lesions in children may
sexual and homosexual individuals, the incidence of STDs be associated with sexual abuse.
have exponentially increased.
Moreover, with the emergence of various multidrug Clinical features
resistant strains of pathogens, newer spectra of diseases
such as acquired immunodeficiency syndrome (AIDS) and The submucosal hemorrhages are characteristically seen at
the evidence of venereal spread of various pathogens, the the junction of the soft and hard palate without involve-
need to prevent, recognize these diseases and manage them ment of the uvula, pharyngeal wall or other oropharyngeal
effectively is important. structures.
According to the statistics of the Centers for Disease These lesions may appear bilaterally as solitary lesions,
Control and Prevention (CDC), approximately 19 million occasionally connected hemorrhagic bridge. They may also
STD cases are reported annually in the United States. Among appear as a well-defined band of ecchymosis stretching
these 19 million cases about 50% of these are seen in people across the soft palate.
in the age group of 15–24 years. These non-ulcerated hemorrhagic areas are painless and
It is believed that there are more than 25 diseases that do not blanch on palpation.
are transmitted through sexual activity. The most common
Diagnosis and differential diagnosis
STDs are HIV, chlamydia, gonorrhea, syphilis, genital herpes,
human papillomavirus, hepatitis B, trichomoniasis and bac- A good personal history will help in the diagnosis. However,
terial vaginosis. other conditions that may mimic such lesions are frequent
As most of these venereal diseases have characteristic use of drinking straws, habit of sucking on candies (pro-
oral findings, the oral physician needs to keep abreast of the duces negative pressure), petechiae secondary to forceful
latest updates about the whole range of clinical manifesta- sneezing, vomiting or coughing, upper respiratory tract
tions of these diseases. infections, blood dyscrasias, infectious mononucleosis, naso-
This chapter will deal with common STDs associated pharyngeal tumors and anticoagulant or antithrombotic
with oral manifestations. medications.
625
Management movement under dark-field microscopy in a wet prepara-

tion. Organisms can be demonstrated in tissue/tissue fluids
Patient should be made aware of the nature and cause of
by silver staining and immunofluorescence. Pathogenic
the lesion. Hemorrhagic diathesis should be considered when
T. pallidum (Nicholas strain) has not yet been able to cul-
the lesions do not subside in about 10 days time following
ture but organisms can be maintained in rabbit’s testicular
cessation of the habit.
tissue with retaining its pathogenicity. In refrigerated blood
the organisms die within 5 days and can be easily killed
with soap and water.
TRAUMATIC LESIONS OF LINGUAL FRENUM
Pathogenesis
Lesions on the lingual frenum are usually seen in individuals
who practice cunnilingus (tongue projected into the vaginal The organisms gain entry through mucous membrane or
area). During such tongue thrusting, the ventral surface of abraded skin during sexual contact. It is estimated that only
the tongue and particularly the lingual frenum rubs against about 50% of people who come in contact develop syphilis.
the incisal edges of the mandibular anterior teeth. It is believed to be due to the presence of local factors and
‘immobilins’ in the blood which immobilize T. pallidum.
Clinical features Incubation period varies from 9 to 90 days.
Natural immunity to syphilis does not occur in humans
Patient may complain of pain and soreness in relation to and vascular changes appear to be more significant, char-
the ventral surface of the tongue. On clinical examination, acterized by endarteritis and periarteritis. Fibroblastic pro-
the ventral surface of the tongue and the lingual frenum may liferation leads to fibrosis and scar formation.
exhibit an ulcerative lesion usually covered by a fibrinous
exudate. The ulcer is typically bounded by an erythematous Clinical features
halo. In chronic cases irritation fibroma may be evident on
the lingual frenum. Primary syphilis Primary syphilis is typically acquired
via direct sexual contact with the infectious lesions of a
Management person with syphilis. Approximately 10–90 days after the
initial exposure (average 21 days), a skin lesion appears at
In most patients the ulcer will resolve in about a week’s the point of contact, which is usually the penis, vagina or
time. Topical anesthetic agent can be prescribed for symp- rectum, but can occur anywhere on the body.
tomatic relief. Irritation fibroma will have to be surgically It is estimated that only about 2% of the primary lesions
excised. are evident on extragenital sites such as the rectum, fingers,
lips, tongue, palate, tonsils, nipple and chin.
Lee et al (2006) reported a case of syphilitic chancre
SYPHILIS OR LUES presenting as a solitary nodule of the nipple. Primary lesions
on the fingers usually result from contact with genital lesions
Syphilis is a chronic venereal infection caused by fragile during sexual foreplay or as an occupational exposure
spirochete Treponema pallidum. in physicians and nurses as a result of direct contact with
The term ‘syphilis’ was coined by the Italian physician and infectious ulcers in their patients. Little (2005) suggested
poet Girolamo Fracastoro (1530). Other names which have that syphilis can be spread by direct contact with mucosal
been used in literature are French disease, Italian disease, lesions of primary and secondary syphilis or blood and
Christian disease, British disease, lues venerea and Cupid’s saliva from infected patients.
disease. In the 16th century syphilis was recognized as This lesion, called a chancre, is a firm, painless ulcer-
‘Great Pox’ in Europe. ation localized at the point of initial exposure to the spi-
Diseases caused by other species of Treponema include rochete. The chancre begins as a papule that subsequently
Yaws (caused by T. pertenue), Pinta (caused by T. carateum) ulcerates. The chancre may persist for 3–8 weeks and usu-
and bejel (endemic syphilis, caused by T. endemicum). ally heals spontaneously. Localized lymphadenopathy may
The major source of transmission of syphilis is by sexual be evident.
contact (acquired). The other modes of transmission include The oral primary syphilitic lesion (chancre) like elsewhere
transplacental spread (congenital/neonatal) or accidental in the body, is generally seen after about 3 weeks after the
inoculation of the causative organism (non-sexual modes exposure at the site of inoculation of the virus. Initially a
of transmission). papule is formed which subsequently ruptures to form a
T. pallidum is a delicate fastidious spirochete whose painless ulcer. The ulcer is generally punched out and may
only natural hosts are humans. It is of 10–15 ␮m in length be indurated. Regional lymphadenopathy is usually a char-
and 0.2 ␮m in thickness, tapering ends and possesses acteristic feature. The chancre resolves in about 4 weeks
10–15 spirals. It has a graceful ‘to and fro’ and angulating leaving a scar.
626
Chapter 22 – Sexually Transmitted Diseases
Secondary syphilis Though it is often believed that the and lateral aspects of the tongue, they tend to ulcerate or
secondary lesion of syphilis appears about 2 months after manifest as irregular fissures. The mucous patches may
the primary lesion has healed, an estimated 30% of the indi- coalesce to give rise to, or arise de novo as serpiginous
viduals may present a chancre along with the secondary lesions, which are popularly referred to as snail track ulcers.
lesion.
The secondary lesions of syphilis affect the skin, liver, Maculopapular lesions
kidneys, genitalia, oral mucosa, eyes, ears and bones. The macular lesions are seen on the hard palate as flat-to-
Skin rashes may be seen which may have varied presen- slightly raised, firm, erythematous lesions.
tations such as papules, macules and pustules. These lesions The papular lesions are seen as erythematous, raised,
are typically copper colored and sometimes referred to as firm round nodules with a gray center that may ulcerate.
‘raw-ham’ colored lesions. The palms and soles are com- The papules may be evident at the commissures and con-
monly affected. These lesions are generally not pruritic. dyloma lata (papillary outgrowths) on the buccal mucosa.
It is estimated that about 5–10% of the patients present
with patchy or ‘moth-eaten appearance’ alopecia. Nodular disease
Systemic signs and symptoms associated with secondary
syphilis include malaise, prostration, cachexia, low-grade Occasionally lesions of secondary syphilis present as nodules.
fever (seldom exceeding 100⬚F), headache, asymptomatic These lesions may mimic keratoacanthoma or squamous cell
meningitis, cranial nerve palsies (nerves II to VIII), painless carcinoma when they occur on the vermillion border of
lymphadenopathy, vague bone pain, jaundice, syphilitic the lip. These nodular lesions are usually seen on the face,
hepatitis, proteinuria, nephrotic syndrome, rapidly progres- mucous membranes, palms of the hands and soles of the feet.
sive glomerulonephritis, renal failure and ulcers affecting Latent syphilis Latent syphilis is defined as having sero-
the antral and pyloric areas of the stomach. logic proof of infection without signs or symptoms of
Ophthalmic signs and symptoms include episcleritis, disease.
scleritis, interstitial keratitis, posterior uveitis, papillary Latent syphilis is further classified as early or late. Early
abnormalities and optic nerve involvement. latent syphilis is defined as having syphilis for 2 years
Patients can also complain of bilateral tinnitus and or less from the time of initial infection without signs or
deafness. Genital mucosa reveals the presence of macules, symptoms of disease. Late latent syphilis is infection for
papules, ulcers or condylomata. These lesions usually resolve more than 2 years but without clinical evidence of disease.
in about 3 weeks. Early latent syphilis is considered more infectious than
It is estimated that about 30% of the patients with sec- the late latent syphilis.
ondary syphilis present with oral mucosal involvement.
Secondary syphilis in the oral mucosa can exhibit two char- Tertiary syphilis The evidence of the tertiary stage of
acteristic features: mucous patches and maculopapular syphilis may occur anywhere from a year of the primary
lesions. Occasionally nodular lesions may be seen. infection to about 10 years. The pathognomonic feature of
A severe generalized form of secondary syphilis is this phase is the presence of gummas. These soft masses of
referred to as ulceronodular disease (lues maligna). This form inflammation (granulomas) can occur anywhere in the
of syphilis is characterized by fever, headache and myalgia, body. Other clinical manifestations of tertiary stage of
followed by a papulopustular eruption that rapidly trans- syphilis include neuro- and cardiovascular syphilis.
forms into necrotic, sharply demarcated ulcers with hem- Neurologic complications include generalized paresis of
orrhagic brown crusts on the face and scalp. The incidence the insane or general paresis (chronic dementia along with
of lues maligna in AIDS patients is high. progressive personality changes and memory loss). Other
Intraorally crateriform or shallow ulcers are seen on the neurologic manifestations are hyperactive reflexes and
gingivae, palate or buccal mucosa, with multiple erosions Argyll Robertson pupil (small and irregular pupils con-
on the hard and soft palates, tongue and lower lip. strict in response to focusing the eyes, but not to light) and
tabes dorsalis (locomotor ataxia).
Cardiovascular complications include syphilitic aortitis,
Mucous patches aortic aneurysm and aortic regurgitation. Syphilitic aortitis
Mucous patches are evident as oval or crescent-shaped can cause de Musset’s sign (bobbing of the head). Trophic
erosions or shallow ulcers of about 1 cm diameter, covered lesions such as Charcot’s joints and distal extremity neu-
by a grayish-white pseudomembrane surrounded by an ropathic perforating ulcers are seen in some patients.
erythematous halo.
Oral manifestations
These may be seen bilaterally on the mobile surfaces of
the mouth. However, the gingiva, hard palate, tonsils and Gumma formation, syphilitic leukoplakia and neurosyphi-
pharynx may be involved. At the commissures, the mucous lis are the oral manifestations seen in the tertiary stage of
patches may appear as split papules, while on the distal syphilis.
627
The initial gummatous lesion is evident as a painless Early manifestations The earliest clinical finding is per-
swelling that commonly occurs on the hard palate and sistent rhinitis. Other associated findings include hepa-
tongue. However, occasional reports of the gumma occurring tomegaly, splenomegaly, glomerulonephritis and nephrotic
on the lower alveolus, parotid gland and soft palate have syndrome.
been described. These swellings subsequently ulcerate. Palms and soles of these neonates show erythematous
Gumma on the palate may cause palatal perforation and maculopapular rashes or vesiculobullous lesions. General-
formation of an oronasal fistula formation. Radiographically, ized lymphadenopathy is evident. Bone lesions such as
gumma involving bone show ill-defined destruction of bone osteochondritis and osteomyelitis.
mimicking malignant lesions. These gummatous lesions
heal by scarring over a period of time. Late manifestations Hutchinson’s triad (interstitial ker-
Gumma involving tongue is referred to as interstitial atitis, peg-shaped upper incisors, and eighth cranial nerve
glossitis. The tongue appears irregular, enlarged and lobu- deafness), saber shins, irregular thickening of the sterno-
lated. Occasionally, papillary atrophy may be appreciated clavicular portion of the clavicle (Higoumenakis sign), flar-
on the dorsal surface of the tongue resulting in a condition ing scapulas, mental retardation and hydrocephalus are the
termed luetic glossitis. late manifestations of syphilis.
Bilateral hydrarthrosis (Clutton’s joints) and neuro-
Syphilitic leukoplakia syphilis are other common late manifestations of syphilis.
Syphilitic leukoplakia appears as a homogeneous white Orofacial manifestations in congenital syphilis Local-
patch on the dorsum of the tongue. Some studies have ized periostitis of the frontal and parietal bones is mani-
shown that the prevalence of syphilis in patients with fested as frontal bossae of parrot. Appearance of the
squamous cell carcinoma of the tongue was about 60%. ‘Olympian row’ is seen when the supraorbital region is
involved.
Inflammation of the nasal mucosa may destroy the
Neurosyphilis underlying bone and cartilage, perforate the nasal septum
and manifests as saddle nose.
Tertiary syphilis can cause unilateral and bilateral trigem-
A short maxilla along with the saddle nose may present
inal neuropathy and facial nerve palsy. It is also believed
as concave or shallow-dish appearance of the middle third
that syphilitic osteomyelitis may give rise to trigeminal
of the face. A relative mandibular prognathism results in a
neuropathy.
‘bulldog jaw’.
Other oral changes include high palatal arch. The dental
Congenital Syphilis anomalies of congenital syphilis only arise in teeth in which
calcification occurs during the first year of life, namely,
Congenital syphilis or prenatal syphilis is caused when the permanent incisors and first molars.
T. pallidum is transmitted to the offspring by an infected Hutchinson’s teeth are short, barrel-shaped or peg-shaped
mother. T. pallidum crosses the placenta only after the widely spaced central incisors. Other findings include
16th week of intrauterine life. notching of incisal edges. The incisors have a screwdriver-
Untreated mothers can drastically affect the status of the shaped morphology (convergence of the lateral margins
offspring. Most of the pregnancies result in spontaneous toward the incisal edge). Mulberry molars, hypocalcifica-
abortion, stillbirth, premature delivery or perinatal death. tion of enamel and presence of rhagades extending from
Prematurity and low-birth weight have also been reported. the angle of the mouth are other prominent features. Occa-
Kassowitz’s law is an empirical observation used in sionally, atrophic glossitis and facial neuropathies may
context of syphilis. It states that greater the duration between be seen.
infection of mother and pregnancy, better is the outcome
for the infant. Better outcome includes lesser chances of
Diagnosis
stillbirth and of developing congenital syphilis.
The rate of vertical transmission in untreated women is Along with the typical history and clinical picture of
70–100% for primary syphilis, 40% for early latent syphilis syphilis, serological tests are necessary for an accurate
and 10% for late latent disease. diagnosis.
These statistics show very clearly that the longer the Dark-field microscopy has been used extensively for
interval between infection and pregnancy, the better is the the diagnosis of primary syphilis. However, it cannot dif-
prognosis for the newborn. ferentiate T. pallidum from the other treponemal species.
Based on the time of presentation of the signs and Serological tests can be used effectively to diagnose
symptoms, the clinical manifestations can be categorized secondary, latent and tertiary syphilis. The serologic tests
as early manifestations (occurring in the first 2 years of include non-treponemal tests (Venereal Disease Research
life) and late manifestations occur after 2 years of age. Laboratory [VDRL] test and the Rapid Plasma Reagin [RPR]
628
test) and treponemal tests (serum fluorescent treponemal 5. Other STIs increase the risk of HIV transmission and
antibody absorption test [FTA-ABS] and microhemagglu- infection, because they cause the disruption of the
tination test for T. pallidum [MHA-TP]). normal epithelial barrier by genital ulceration and/or
microulceration.
Management 6. HIV transmission through oral exposure to semen or
vaginal fluids have been documented. Fellatio (mouth
Parenteral penicillin G is the drug of choice for the to penis contact) and cunnilingus (mouth to vulva con-
treatment of all stages of syphilis. Patients allergic to pen- tact) cannot be considered to be totally safe practices.
icillin can be treated with doxycycline, tetracycline or 7. Transmission appears to be highest during the early
erythromycin. and late stages of HIV when the viral load is at its peak.
HIV and AIDS are described in detail in Chapter 4 on
Bacterial, Viral and Fungal Infections.
HUMAN IMMUNODEFICIENCY VIRUS
INFECTION
INTRAORAL MOLLUSCUM CONTAGIOSUM
In 1981, homosexual men with symptoms of a disease that
now are considered typical of the AIDS were first described Molluscum contagiosum is a DNA poxvirus that generally
in Los Angeles and New York by the CDC. In 1983, research- affects the skin or mucous membranes. Juliusburg (1905)
ers in the United States and France described the virus that discovered the viral nature of this condition. Molluscum
causes AIDS, now known as the human immunodeficiency contagiosum has also been known as epithelioma conta-
virus (HIV) and belonging to the group of viruses called giosum and dimple wart.
retroviruses. It is estimated that about 33.2 million people Molluscum contagiosum is primarily caused by MCV-1
are affected by AIDS worldwide. and MCV-2 (molluscum contagiosum virus subtypes 1
and 2).
Modes of transmission The virus is transmitted by direct contact and autoin-
HIV is transmitted through direct contact of a mucous mem- oculation. Upon entering the host cell the virus replicates
brane or the bloodstream with a body fluid containing HIV, in the cytoplasm of epithelial cells producing cytoplasmic
such as blood, semen, vaginal fluid, preseminal fluid and inclusions and cause enlargement of the infected cells.
breast milk. The transmission of HIV can result from vagi- Molluscum contagiosum is more common in patients who
nal, anal or oral sex, infected needles, blood transfusion, are on steroid therapy or in those who have atopic derma-
maternal-fetal transmission during pregnancy, birth and titis, immunodeficiency or lymphoproliferative disorders.
breast feeding.
Clinical features
Sexual mode of transmission The infection affects both adults and children. In children,
It is estimated that the transmission through sexual contact the transmission is via non-sexual skin contact whereas in
accounts for 75–85% of all HIV infections. Sexual trans- young adults it occurs as an STD. In children, the lesions are
mission occurs with the contact between sexual secretions generally seen on the face, trunk, extremities (especially in
of one person with the rectal, genital or oral mucous the axillae), and sometimes on the mucous membranes of
membranes of another. the lips, tongue and buccal mucosa. In adults, involve-
ment of the pubic, genital and perineal areas is common.
Some facts about sexual mode of transmission The typical lesion is an umbilicated or waxy papule
associated with itching and pain. Some patients develop
1. Unprotected receptive sexual acts are riskier than
eczema around the papule.
unprotected insertive sexual acts.
The papules are flesh-colored and occasionally
2. Anal sex is the most efficient means of sexual HIV
yellowish-white in color. These papules can occur as soli-
transmission compared to vaginal intercourse or oral
tary lesions and sometimes several in number. These are
sex (the rectal mucous membranes seem to have more
generally about 2–5 mm in diameter. Large sized lesions
receptors to bind HIV and the tissue is more easily
(⬎5 mm) and a greater number of lesions (⬎30 in number)
traumatized).
are usually seen in AIDS patients.
3. HIV can be transmitted through both insertive and
receptive oral sex.
Oral manifestations
4. The risk of HIV transmission from exposure to saliva
is considerably smaller than the risk from exposure to Oral lesions are rarely encountered in molluscum conta-
semen. giosum. These lesions mimic the skin lesions.
629
The common sites of involvement include the lips, to form bulbous/papillomatous lesions. These papillomatous
buccal mucosa, hard palate, retromolar area and tongue. lesions can affect any part of the oral cavity. The common
Fornatora et al (2001) described a rare case of molluscum sites of involvement are the dorsal surface of the tongue,
contagiosum occurring on the gingiva. palate, alveolar ridge, buccal mucosa and gingiva.
The nodules proliferate and coalesce to form soft, red
Histologic features or dirty gray, sessile or pedunculated papillary growths.
Lesions develop rapidly to form discrete single or exten-
Hematoxylin and eosin-stained biopsy sections reveal
sive clusters of granular or cauliflower-like neoplasms.
thickening (almost six times more than normal) and down-
growth of epithelium. Presence of intracytoplasmic inclu- Diagnosis
sion bodies (molluscum bodies or Henderson–Patterson
bodies) is typical of molluscum contagiosum. Diagnosed indirectly by cytology, biopsy or the appear-
Alternatively, smears taken from scrapings of the lesion ance of white areas after the application of acetic acid
show inclusion bodies when stained with Papanicolaou, (acetowhitening).
Wright, Giemsa or Gram stain. The affected area is wrapped with a gauze piece soaked
An advanced technique to identify the antigen of MCV with 5% acetic acid for 5 minutes. A magnifying lens (10⫻)
by fluorescent antibody technique may be employed. or a colposcope can be used to visualize the warts which
appear as minute white colored papules.
Management A more definitive diagnosis can be made by detecting
the viral nucleic acid or capsid protein.
Most of the lesions are self-limiting and usually resolve in
about 9–12 months. The goal of the treatment is to limit Management
the transmission and autoinoculation.
Podophyllin (cytotoxic agent) is the drug of choice. Alter-
Topical application of cantharidin, podophyllin or treti-
natively, topical trichloroacetic acid and 5-fluorouracil can
noin cream may be beneficial.
be used. Surgical modalities include the use of cryother-
Cryotherapy with liquid nitrogen is a popular manage-
apy and carbon dioxide laser ablation.
ment strategy. Surgical excision of the lesion is also an
accepted treatment modality.
Antiviral medications such as ritonavir, zidovudine
have been used to manage the molluscum contagiosum in OROPHARYNGEAL GONORRHEA
immunocompromised individuals.
Gonorrhea is a caused by Neisseria gonorrheae. The dis-
ease is characterized by purulent inflammation of mucous
CONDYLOMA ACUMINATUM membrane surfaces.
Clinical features
Condyloma acuminatum commonly termed genital or vene-
real wart occurs commonly on external genitalia; however, The bacterial infection spreads through sexual contact.
oral lesions can occur either through orogenital sex or by Gonococcal infections are 1.5 times more common in men
auto-inoculation of genital lesions. Condyloma acumina- than in women. It is usually seen in sexually active ado-
tum is caused by human papilloma virus types 6, 11 and lescents and young adults. Like most STDs, the presence of
16. Condylomata acuminata are now thought to be one of gonorrhea in a child can be considered as an indicator for
the four most common STDs. sexual abuse of the child.
Men may present with urethral discomfort, dysuria and
Clinical features purulent discharge. Other notable feature is epididymitis.
Women may complain of thin, purulent and mildly
Typical lesions are evident as solitary or multiple papular
odorous discharge from the vagina. Another important
eruptions. These eruptions are usually flesh colored and
complication of gonorrhea in women is pelvic inflamma-
appear pearly, filiform, fungating, cauliflower or plaque like.
tory disease (PID). PID indicates that the offending organ-
The most commonly affected areas are the penis, vulva,
ism has ascended to involve the endometrium, fallopian
vagina, cervix, perineum and perianal area. Rarely, muco-
tubes, ovaries and peritoneum. PID is characterized by mid-
sal lesions in the oropharynx, larynx and trachea have
line mild or severe pain and cramps.
been reported.
Right upper quadrant pain from perihepatitis (Fitz–
Hugh–Curtis syndrome) may occur following the spread of
Oral manifestations
organisms upward along peritoneal planes.
The typical oral lesions of condyloma acuminatum are min- Occasionally rectal discharge, bloody stools and pruritus
ute, multiple pink colored nodules which tend to coalesce may be evident.
630
Many of the patients may suffer from dysphagia sec- Oral manifestations
ondary to pharyngitis.
Patients may present with pharyngitis. The oral mucosa
An extensive form of gonorrhea, disseminated gonor-
appears intensely erythematous. However, painless muco-
rheal infection (DGI) is seen in some patients. The dis-
sitis is the characteristic feature. Lips, buccal mucosa, floor
seminated form is characterized by joint or tendon pain
of mouth, tongue, tonsillar regions and uvula are the com-
(gonococcal arthritis), skin rash, gonococcal meningitis
mon sites affected. Occasionally, cervical lymphadenopathy
and gonococcal endocarditis.
may be present.
Oral manifestations Diagnosis

In the initial stages of the infection, patients may com- Cultures are usually difficult to obtain and expensive.
plain of burning sensation, drying of the mouth and foul However, direct immunofluorescent antibody and enzyme
breath. immunoassay can be used. Alternatively, PCR can be
This phase may be followed by the evidence of painful used.
ulcers usually seen on the lips, gingival, tongue, palate
(hard and soft) and the tonsillar regions. Management
The ulcers are typically punched out in the interdental
gingiva. The tongue may exhibit papillary atrophy. The The drugs of choice are doxycycline or azithromycin with
ulcers are usually covered by grayish-white or yellowish- ofloxacin.
white pseudomembrane. Pharyngitis is a characteristic
feature.
Regional lymphadenopathy may be evident. In some OROPHARYNGEAL TRICHOMONAL
patients, the TMJ may be affected (gonococcal arthritis of INFECTION
TMJ).
Trichomonas vaginalis is the causative agent of trichomonia-
Diagnosis sis. It is a parasitic protozoan with humans as the only natu-
ral host. It infects the squamous epithelium of the genital
The evidence of gram-negative diplococci in a Gram stained tract. Incubation time is generally between 4 and 28 days.
smear of exudates from the gonococcal lesions is sugges- It is believed that T. vaginalis infections are a marker for
tive of N. gonorrheae infection. high risk sexual behavior as they are generally associated
with other STDs, especially gonorrhea.
Management
Clinical features
Oropharyngeal gonorrhea is best managed with intramus-
cular injection of ceftriaxone. Other drugs that have been Women complain of foul smelling, frothy vaginal dis-
used effectively include cefixime and ciprofloxacin. charge. However, the pathognomonic sign is the presence
of ‘strawberry cervix’ or ‘colpitis macularis’ on colposcopy.
Patients may also complain of lower abdominal pain and
dysuria. However, some patients may remain asymptomatic.
OROPHARYNGEAL CHLAMYDIAL Male patients may remain asymptomatic or present
INFECTION with urethritis.
Chlamydial infection is caused by Chlamydia trachomatis, Oral manifestations

an obligate intracellular bacterium that infects the urethra, The characteristic feature is strawberry-like inflammation
epididymis, uterus and cervix. The bacterium is usually of the oral mucosa secondary to vasodilation. The mucosal
spread through sexual activity. surface may show presence of exudate.
However, it can also spread vertically to cause pneumo-
nia and conjunctivitis in neonates. Diagnosis
Chlamydial infection is usually seen in the 2nd and 3rd
decades of life. The gold standard for diagnosing trichomonads is by cul-
ture. The Diamond’s medium is the ideal medium for this
procedure.
Clinical features
Few simple diagnostic tests that may be used are the
Women may present with dysuria, vaginal bleeding, vagi- immediate examination of a wet slide and whiff test.
nal discharge and lower abdominal pain. Men may exhibit The easiest method to visualize motile trichomonads
dysuria, rectal and/or urethral discharge and proctitis. is by placing a small amount of vaginal discharge on a
631
microscope slide and mixing with a few drops of saline of a strong fishy odor is indicative of a positive result.
solution within 20 minutes of obtaining the sample. The This test is referred to as the whiff test or amine odor test.
slide is then examined under a microscope at low or
medium power. The presence of flagellated, pyriform pro-
Management
tozoa indicates a positive result.
Alternatively, several drops of 10% potassium hydroxide Metronidazole and tinidazole are the drugs of choice. The
are mixed with a sample of vaginal discharge. Presence recommended dose is 2 g orally in a single dose.
632
CHAPTER
Nutritional and Metabolic
Disorders
Shubha Sairam, Praveen BN
23
➧ Nutritional Requirements of Indians ➧ Metabolic Disorders
➧ Carbohydrates Lysosomal Storage Diseases
➧ Proteins Lipoid Proteinosis
Hand–Schuller–Christian Disease or Multifocal
➧ Lipids
Eosinophilic Granuloma
➧ Vitamins Letterer–Siwe Disease
Vitamin A
➧ Lipid Reticuloendothelioses
Vitamin D
Gaucher Disease
Vitamin K
Niemann–Pick Disease
Vitamin C
Vitamin B Complex
Nutriology as per Dorland’s Medical Dictionary is the sci- even further, leading to subsequent inadequate dietary
ence of nutrition. It is the science of how the body utilizes intake and compromised nutritional status.
food to meet requirements for development, growth, repair, There are six classes of nutrients: water, carbohydrates,
and maintenance. Nutrients are biochemical substances that proteins, fats, minerals and vitamins. Nutrients work
can be supplied only in adequate amounts from an outside together and interact in complex metabolic reactions.
source, usually from food. Proteins, carbohydrates and fats provide energy for body
The relationship between nutrition and oral health is needs. However, the body cannot use this energy without
multifaceted. Nutrition has both local and systemic adequate amounts of vitamins and minerals.
impacts on the oral cavity. While diet and eating patterns
have a local effect on the teeth, saliva and soft tissues, the
systemic impact of nutrition also has considerable impli-
cations and it too merits assessment as a component of NUTRITIONAL REQUIREMENTS OF INDIANS
comprehensive care. The systemic effect is the impact of
the nutrients consumed as they assume their biological Energy requirement is defined as the amount that will bal-
functions in relation to the development and maintenance ance the energy expenditure of the individual (as deter-
of the extra- and intraoral structures and secretions. The mined by body size and composition and level of physical
oral cavity is often one of the first sites where nutrient activity) consistent with long-term good health. This
deficiencies can be clinically noted. intake will allow for the maintenance of economically
Clinical manifestations of nutrient deficiencies can necessary and socially desirable physical activity. In chil-
have a significant impact on the function of the oral cav- dren and pregnant/lactating women, the energy require-
ity. Functional properties of the oral cavity include taste, ment will include energy needed for deposition of tissue
salivation, mastication and swallowing food. Any altera- and secretion of milk at the rate consistent with good
tions in the structure and function of the oral cavity may health. All estimates of requirement are based on habitual
compromise intake and contribute to the development of a intakes though these are expressed as daily intake.
nutrient-deficiency state. When the associated oral struc- Recommended dietary allowance (RDA) is the amount of
tures are affected, these alterations may be compounded selected nutrients considered adequate to meet the known
633
nutrient needs of healthy people. The Canadian equivalent ❍ Carbohydrates are required for the formation of non-
is the recommended nutrient intakes (RNIs). The energy essential amino acids.
needs of men and women for different activity levels ❍ Non-starch polysaccharides constitute dietary fibers
computed on the basis of recommendations made by a that are required to prevent constipation, reduce blood
Joint Expert Consultation of the World Health Organization cholesterol and give satiety.
(WHO)/Food and Agricultural Organization (FAO)/United ❍ It is recommended that 55–60% of total energy intake
Nations University (UNU) in 1985 and by an Expert should be obtained from carbohydrates.
Committee constituted in 1988 by the Indian Council of
Medical Research (ICMR) are as shown in Tables 1 and 2. Sources
The ICMR's RDA is higher than those recommended by the Grains (wheat, corn, rice, oats, rye, barley, buckwheat and
WHO/FAO/UNU. millet) provide complex carbohydrates and starches. Veg-
For computing RDA, the ICMR has taken body weight etables, especially root and seed varieties (potatoes, sweet
of ‘reference man’ as 60 kg and that of ‘woman’ as 50 kg. potatoes, beet and peas) contain considerable amounts of
Average weight of Indian men is 52 kg and women 44 kg. starch. Milk is a good source of lactose. Dietary fiber can be
For children and adolescents, weight for age from National obtained from whole grain breads and cereals and legumes.
Center for Health Statistics (NCHS), USA/well-to-do Indian
children have been utilized by ICMR for deriving the RDA Dental considerations
so that energy intake enables optimum growth. However, as
in adults, majority of children and adolescents weigh sub- For decades, dietary carbohydrates have one of the major
stantially less and hence their energy requirement is lower. constituents incriminated in the causation of caries. The
occurrence of caries depends upon the frequency of con-
sumption, chemical constitution, route of administration,
CARBOHYDRATES and physical properties of carbohydrates.
Sugar substitutes have been claimed to be non-cariogenic
Carbohydrates have been the major sources of energy due to their inability to act as substrates for the enzyme
since the dawn of history and furnish up to 90% of energy glucosyltransferase. Xylitol is one such commonly used
needs. Carbohydrates provide about 4 kcal/g. The average sugar substitute. It has been found to be non-cariogenic.
adult stores about 300 g of carbohydrate in the liver and Some studies also show that chewing xylitol gum has
muscle tissue as glycogen.
Table 2 Recommended dietary allowance of infants and
Functions
children
❍ Mainly provides fuel for the body, especially the cen- Age of infant Energy (kcal) Weight (kg)
tral nervous system.
❍ Adequate consumption has a protein sparing function, 3–6 months* 700 7
i.e. proteins consumed are used for anabolic function 6–9 months* 810 8.5
rather than as an energy source. 9–12 months* 950 9.5
❍ In the absence of carbohydrates, fat metabolism is 6 months† 583 5.4
incomplete and leads to formation of ketone bodies. 6–12 months† 844 8.6
❍ Carbohydrates are required for the formation of structural
Source: *WHOIFAO/UNO (1985); †ICMR (1988).
components such as cartilage, nervous tissue and bone.
Table 1 ICMR's recommended dietary allowance for energy
Sex Ref. body Actual body Energy RDA

weight (kg) weight (kg)
Activity For ref. body For actual Percentage
category weight body weight difference
Man 60.0 52.0 Sedentary 2,425 2,115 13
Moderate 2,875 2,492 13
Heavy 3,800 3,293 13
Woman 50.0 44.0 Sedentary 1,875 1,740 12
Moderate 2,225 1,958 12
Heavy 2,925 2,594 11
Source: Dr BS Narasinga Rao-Gopalan Oration (2001).
634
Chapter 23 – Nutritional and Metabolic Disorders
cariostatic properties. Other sugar substitutes, sorbitol, Dental considerations

aspartame, sucralose have been found to have a negligible
During tooth development, mild-to-moderate protein defi-
effect on the development of caries.
ciency results in smaller molars, chemical alterations of
the exposed enamel surface, significantly delayed eruption
and retardation of mandibular development. Smaller sali-
PROTEINS
vary glands develop resulting in decreased salivary flow.
This saliva has a different protein, amylase and aminopep-
Second to water, proteins are the most abundant sub- tidase activity, thereby compromising its immune function.
stances in the body. The structural units of proteins are Delayed eruption and decreased salivary flow lead to
amino acids. Of the 20 amino acids present in nature, nine increased incidence of dental caries. Epithelium, connective
are considered essential, i.e. these are to be supplied by tissue and bone may be poorly developed. Insufficient
the diet. intake of protein results in negative nitrogen balance,
decreased levels of secretory IgA. This leads to a lowered
Functions resistance to infections, reduced ability to withstand the
❍ Generation of new body tissues stresses of injury or surgery, and prolonged recovery time.
❍ Repair of body tissues PEM may be a major reason for the occurrence of necro-
❍ Production of enzymes, hormones, immunoglobulins tizing ulcerative gingivitis (NUG) and noma.
❍ Regulation of fluid balance
❍ Transport of insoluble fats
❍ Provide energy—4 kcal/g. LIPIDS
Sources Lipids provide more energy per gram than either carbohy-
drates or proteins and are an essential component of tooth
Meat and milk food groups provide most of the dietary enamel and dentin.
proteins. Soy is also a good source of proteins.
Chemistry and sources
Deficiency
The structural units of lipids are fatty acids. Saturated fatty
Protein energy malnutrition This term covers the spec- acids contain only single bonds. Examples include pal-
trum of clinical conditions seen in undernutrition. The two mitic and stearic acids found in animal fat, butter, coconut
clinical forms of protein energy malnutrition (PEM) oil, chocolate, etc.
include kwashiorkor and marasmus. Monounsaturated fatty acids contain only one double
Kwashiorkor occurs when a child is fed on a diet with bond. The most abundant of these is oleic acid found in
very low protein content relative to energy. This results in olive, peanut oil and animal products.
a high level of plasma insulin and low levels of plasma Polyunsaturated fatty acids (PUFAs) contain two or
cortisol. This hormonal pattern leads to an uptake of more double bonds. Three PUFAs that are considered to be
amino acids in muscle, diverting these from liver, leading essential fatty acids include linoleic, linolenic and arachi-
to decreased albumin synthesis and therefore edema. donic acids. These are found in safflower, soybean, fish
Thus a child with kwashiorkor shows apathy, lethargy and corn oils, nuts and seeds.
and severe anorexia. There is generalized edema, muscle Deficient consumption of fats leads to loss of weight.
wasting in shoulders and upper arms. The child may have Deficiency of linoleic acid leads to growth retardation,
a ‘moon face’. Potbelly due to weakness of abdominal skin lesions, and reproductive failure.
muscles occurs. Skin changes in the form of thickening, Over consumption of fats leads to excessive fat stores
cracking and areas of denudation occur. Hair changes and obesity. Other conditions related to fat consumption
color and becomes sparse. include diabetes mellitus, hyperlipidemia, fatty infiltration
Marasmus occurs when there is inadequate food intake of liver, and certain types of cancer.
resulting in energy deficiency. This leads to low insulin
and high plasma cortisol levels. This results in amino acids Dental considerations
being released from muscles making them available for
Epidemiological and laboratory studies indicate that fats
protein synthesis.
have a cariostatic effect. Dietary fats probably have local
A child with marasmus presents with no subcutaneous fat
rather than systemic influence. Hypotheses for anticario-
and wasted muscles. The body weight is severely reduced.
genicity include:
There is no edema, and skin and hair changes are mild or
absent. 1. Some fatty acids, such as oleic acid act as growth fac-
Studies have shown that the incidence of PEM among tors for lactobacilli, others, such as lauric acids inhibit
Indian children could be as high as 51.6–70%. the growth of streptococci.
635
2. Long-chain fatty acids may decrease the dissolution The growth and differentiation of epithelial cells
of hydroxyapatite by acids. throughout the body is especially affected by vitamin A
3. Oral food retention decreases with increased fat intake. deficiency. In addition, goblet cell numbers are reduced in
4. Fats may lubricate the tooth and prevent acid pene- epithelial tissues and as a consequence, mucous secretions
tration into enamel. (with their antimicrobial components) diminish. Cells lin-
5. Fats may produce a film on food particles and prevent ing protective tissue surfaces fail to regenerate and dif-
partial digestion of food particles in the mouth. ferentiate, hence they flatten and accumulate keratin. Both
6. Dietary fat delays gastric emptying, enhancing fluoride factors—the decline in mucous secretions and loss of cel-
absorption and increasing tissue fluoride concentration. lular integrity—reduce the body’s ability to resist invasion
from potentially pathogenic organisms. Pathogens can also
compromise the immune system by directly interfering
VITAMINS with the production of some types of protective secretions
and cells. Classical symptoms of xerosis (drying or non-
Vitamins are catalysts for all metabolic reactions using wettability) and desquamation of dead surface cells as
proteins, fat and carbohydrates for energy, growth and cell seen in ocular tissue (i.e. xerophthalmia) are the external
maintenance. Vitamins are vital to life, but are required in evidence of the changes also occurring to various degrees
minute amounts. in internal epithelial tissues.
Vitamins are classified as fat soluble—vitamins A, D, E, K Current understanding of the mechanism of vitamin A
and water soluble—vitamins B, C. action within cells outside the visual cycle is that cellular
functions are mediated through specific nuclear receptors.
Characteristics of fat-soluble vitamins: These are soluble Binding with specific isomers of retinoic acid (i.e. all-trans-
in fats or fat solvents. Chemically, these are organic sub- and 9-cis-retinoic acid) activates these receptors. Activated
stances and do not contain nitrogen. These are absorbed in receptors bind to DNA response elements located upstream
the intestines in the presence of bile. These are fairly stable of specific genes to regulate the level of expression of those
to heat, as in cooking. Larger amounts of these vitamins genes. These retinoid-activated genes regulate the synthe-
can be stored in the body and for long periods of time. Thus, sis of a large number of proteins vital to maintaining nor-
symptoms of deficiencies appear late. Increased intake may mal physiologic functions. There may, however, be other
lead to toxicity. mechanisms of action that is as yet undiscovered.
Vitamin A Dietary sources
Vitamin A (retinol) is an essential nutrient needed in small Preformed retinal is found in milk, cheese, butter, eggs,
amounts by humans for the normal functioning of the visual meat, cod liver oil and liver. -Carotene is also present in
system; growth and development; and maintenance of yellow, orange and green leafy vegetables (spinach,
epithelial cellular integrity, immune function, and repro- turnip greens, broccoli).
duction. These dietary needs for vitamin A are normally To express the vitamin A activity of carotenoids in diets
provided as preformed retinol (mainly as retinyl ester) and on a common basis, a Joint FAO/WHO Expert Group in
provitamin A carotenoids or -carotene. 1967 introduced the concept of the retinol equivalent (RE)
and established the following relationships among food
Functions sources of vitamin A:
1 g retinol 1 RE
Vitamin A functions at two levels in the body: the first is 1 g -carotene 0.167 g RE
in the visual cycle in the retina of the eye; and the second 1 g other provitamin A carotenoids 0.084 g RE.
is in all body tissues where it systemically maintains the
Older literature describes the International Unit of vitamin A.
growth and soundness of cells.
This may be converted to RE as depicted in Table 3:
In the visual system, carrier bound retinol is transported
1 IU retinol 0.3 g retinol
to the retina. Rhodopsin, the visual pigment critical to
1 IU -carotene 0.6 g -carotene
dim-light vision, is formed in rod cells after conversion of
1 IU retinol 3 IU -carotene.
all-trans-retinol to retinaldehyde, isomerization to the
11-cis-form, and binding to opsin. Alteration of rhodopsin
Deficiency
through a cascade of photochemical reactions results in
the ability to see objects in dim light. The speed at which Vitamin A deficiency leads to xerophthalmia and night
rhodopsin is regenerated is related to the availability of blindness due to degeneration of epithelial cells. Xeroderma
retinol. A deficient intake of vitamin A thus leads to night can also occur. Mild deficiency may be related to a
blindness. depressed immune response.
636
Table 3 Estimated mean requirement and safe level of

Retinoids have been used in oral precancer, cancer and
intake for vitamin A
immunologically mediated diseases such as lichen planus.
Retinoids have a potent growth inhibiting effect on cancer
Group Mean requirement Recommended safe in vivo and in vitro. They can induce apoptosis and regu-
(mg RE/day) intake (mg RE/day)
late the function of the immune system. On this basis, reti-
Infants and children noids have been used as chemopreventive agents in oral
0–6 months squamous cell carcinoma (SCC). However, clinical trials of
7–12 months 180 375 retinoids have not yielded significant results. Retinoids
1–3 years 190 400
have been used in the treatment of oral leukoplakia, and
4–6 years 200 400
has been shown to cause temporary remission, but also
7–9 years 200 450
250 500
causes toxicity. Retinoids have been used as an adjunctive
treatment in the management of oral lichen planus.
Adolescents
Retinoids eliminate reticular and plaque-like lesions but
10–18 years 330–400 600
these recur following withdrawal of therapy.
Adults
Females Toxicity
19–65 years 270 500
65 years 300 600 Because vitamin A is fat soluble and can be stored, pri-
Males marily in the liver, routine consumption of large amounts
19–65 years 300 600 of vitamin A over a period of time can result in toxic symp-
65 years 300 600 toms, including liver damage, bone abnormalities and joint
Pregnant women 370 800 pain, alopecia, headaches, vomiting, and skin desquama-
Lactating women 450 850 tion. Other clinical symptoms include diplopia, alopecia,
Source: Vitamin and mineral requirements in human nutrition, WHO. dryness of mucous membranes, reddened gingiva, thinning
of epithelium, cracking and bleeding lips, and increased
activity of osteoclasts. Overconsumption of -carotene leads
Dental considerations to hypercarotenemia and yellow pigmentation of skin.
Vitamin A is necessary for growth of both soft tissue and

bone. It is required for resorption of old bone and synthe- Vitamin D
sis of new bone, formation of ameloblasts, odontoblasts,
Functions
and maintenance of the integrity of epithelial tissues.
Severe deficiency of vitamin A may result in enamel Vitamin D is required to maintain normal blood levels of
hypoplasia and defective dentin formation in develop- calcium and phosphate, which are in turn needed for the
ing teeth. Odontoblasts lose the ability to arrange them- normal mineralization of bone, muscle contraction, nerve
selves in normal parallel linear formation leading to conduction and general cellular function in all cells of
altered dentin deposition. This further leads to degenera- the body. Vitamin D achieves this after its conversion to
tion and atrophy of ameloblasts. This results in enamel the active form 1,25-dihydroxy vitamin D (1,25-(OH)2D),
hypoplasia characterized by defects in enamel matrix and or calcitriol. This active form regulates the transcription
incomplete calcification. of a number of vitamin D-dependent genes that code for
calcium-transporting proteins and bone matrix proteins.
Clinical applications Vitamin D also modulates the transcription of cell cycle
proteins, which decrease cell proliferation and increase
Carotenoids possess antioxidant properties and are very effi-
cell differentiation of a number of specialized cells of the
cient in scavenging singlet oxygen and peroxyl radicals.
body (e.g. osteoclastic precursors, enterocytes, keratino-
These free radicals are known to damage the structure and
cytes). This property may explain the actions of vitamin D
function of cell membranes. Thus a diet rich in antioxidants
in bone resorption, intestinal calcium transport and skin.
is associated with a lower risk of cancer and heart disease.
Stich and colleagues gave large quantities of -carotene
Sources
and sometimes vitamin A to chewers of betel quids in Kerala,
India, and to Canadian Inuits with pre-malignant lesions The most physiologically relevant and efficient way of
of the oral tract and witnessed reductions in leukoplakia acquiring vitamin D is to synthesize it endogenously in the
and micronuclei from the buccal mucosa. skin from 7-dehydrocholesterol by sunlight (UV) exposure.
However, the amount of supplements used in these stud- In most situations, approximately 30 minutes of skin expo-
ies have been large and hence, not advisable other than sure (without sunscreen) of the arms and face to sunlight can
increasing consumption of fruits and vegetables. provide all the daily vitamin D needs of the body (Table 4).
637
Table 4 Recommended nutrient intakes (RNIs) for vitamin D

fractures. Muscle weakness and a waddling gait may be
present.
Group RNI (mg/day)
Infants and children
0–6 months 5 Patients with rickets also have involvement of alveolar bone
7–12 months 5 resulting in its weakening. Patients with severe vitamin D
1–3 years 5 deficiency develops enamel hypoplasia. This is characterized
4–6 years 5
by pitting of surface enamel. These surfaces are prone to
7–9 years 5
adherence of plaque. However, studies of susceptibility to
Adolescents caries among these teeth reveal conflicting results.
10–18 years 5
Adults
19–50 years 5 Vitamin K
51–65 years 10
Vitamin K is an essential fat-soluble micronutrient. Thus far,
65 years 15
the only unequivocal role of vitamin K in health is in the
Pregnant women 5 maintenance of normal coagulation. The vitamin K-dependent
Lactating women 5 coagulation proteins are synthesized in the liver and com-
Units: For vitamin D, 1 IU 25 ng, 40 IU 1 mg, 200 IU 5 mg, 400 IU prise factors II, VII, IX and X, which have a hemostatic role
10 mg, 600 IU 15 mg, 800 IU 20 mg. (i.e. they are procoagulants that arrest and prevent bleed-
Source: Vitamin and mineral requirements in human nutrition, WHO. ing), and proteins C and S, which have an anticoagulant
role (i.e. they inhibit the clotting process). Despite this
duality of function, the overriding effect of nutritional
Skin synthesis of vitamin D may be influenced by: vitamin K deficiency is a bleeding tendency caused by the
relative inactivity of the procoagulant proteins.
❍ Latitude and season—both influence the amount of UV
light reaching the skin Chemistry and function
❍ The aging process—thinning of the skin reduces the
efficiency of this synthetic process Vitamin K is the family name for a series of fat-soluble
❍ Skin pigmentation—the presence of darker pigments in compounds which have a common 2-methyl-1,4-naphtho-
the skin interferes with the synthetic process because quinone nucleus but differ in the structures of a side chain
UV light cannot reach the appropriate layer of the skin at the 3-position. These are synthesized by plants and bac-
❍ Clothing—virtually complete covering of the skin for a teria. In plants, the only important molecular form is phyl-
medical, social, cultural or religious reasons leaves loquinone (vitamin K1). Bacteria synthesize a family of
insufficient skin exposed to sunlight compounds called menaquinones (vitamin K2).
❍ Sunscreen use—widespread and liberal use of sun- The biological role of vitamin K is to act as a cofactor
screen, though reducing skin damage by the sun, del- for a specific carboxylation reaction that transforms selec-
eteriously affects synthesis of vitamin D. tive glutamate (Glu) residues to -carboxyglutamate (Gla)
residues. The reaction is catalyzed by a microsomal enzyme,
It is recommended that individuals not synthesizing vita- -glutamyl, or vitamin K-dependent carboxylase.
min D should correct their vitamin D status by consuming
the amounts of vitamin D appropriate for their age group. Dietary sources
Other food sources include cod liver oil, catfish, salmon,
Phylloquinone is distributed ubiquitously throughout the
turnip greens, tuna, milk, egg yolk and butter.
diet, and the range of concentrations in different food cat-
egories are very wide. In general, the relative values in
Deficiency
vegetables confirm the known association of phylloqui-
Rickets is a clinical syndrome that occurs when there is a none with photosynthetic tissues, with the highest values
deficiency of vitamin D in the growing skeleton. Infants with being found in green leafy vegetables. The next best sources
rickets exhibit delayed development and muscle hypoto- are certain vegetable oils (e.g. soybean, rapeseed and
nia, craniotabes (small non-calcified areas in skull bones), olive); other vegetable oils, such as peanut, corn, sun-
bossing of frontal and parietal bones, swelling of the rib flower and safflower. Menaquinones seem to have a more
costochondral junctions (rickety rosary). Severe rickets may restricted distribution in the diet than does phylloquinone.
be associated with hypocalcemic tetany, giving rise to laryn- Menaquinone-rich foods are those with a bacterial fer-
geal stridor when the vocal cords are affected. mentation stage.
Vitamin D deficiency in the adults is termed osteomala- Intestinal microflora synthesize large amounts of
cia. Osteomalacia is characterized by bone pain, pathologic menaquinones, which are potentially available as a source
638
of vitamin K. Most of these menaquinones are present in Dental considerations

the distal colon. It is noteworthy that menaquinones with
Oral manifestations of vitamin K deficiency include gingi-
very long chains are known to be synthesized by members
val bleeding, petechiae and ecchymoses. Bleeding on brush-
of the anaerobic genus Bacteroides.
ing occurs when prothrombin levels fall below 35% and
It is commonly held that animals and humans obtain a
spontaneously when the levels fall below 20%.
significant fraction of their vitamin K requirement from
direct absorption of menaquinones produced by microflo- Characteristics of water-soluble vitamins: These are
ral synthesis, but conclusive experimental evidence docu- water-soluble organic substances. The B vitamins also
menting the site and extent of absorption is lacking. The contain nitrogen. These vitamins are readily absorbed in
most promising site of absorption is the terminal ileum, the jejunum. The body stores very small quantities of each
where there are some menaquinone-producing bacteria as of these vitamins and hence, daily intake is important.
well as bile salts. However, the balance of evidence suggests
that the bioavailability of bacterial menaquinones is poor
because they are for the most part tightly bound to the Vitamin C
bacterial cytoplasmic membrane and also because the larg- Vitamin C (chemical names: ascorbic acid and ascorbate)
est pool is present in the colon, which lacks bile salts for is a six-carbon lactone which is synthesized from glucose
their solubilization (Table 5). by many animals. Vitamin C is synthesized in the liver in
some mammals and in the kidney in birds and reptiles.
Deficiency
However, several species including humans are unable to
Primary deficiency of vitamin K is uncommon, but may synthesize vitamin C. When there is insufficient vitamin C
occur due to impaired fat absorption in celiac disease, sprue, in the diet, humans suffer from the potentially lethal defi-
ulcerative colitis and jaundice. The gut remains sterile for ciency disease scurvy.
the first few days after birth and hence newborns may suf-
fer from vitamin K deficiency. Deficiency also occurs sec- Functions
ondarily following antibiotic therapy.
Vitamin C is an electron donor (reducing agent or anti-
oxidant), and probably all of its biochemical and molecu-
lar roles can be accounted for by this function.
Table 5 Recommended nutrient intakes (RNIs) for vitamin K
Group RNIa (mg/day) Dietary sources

Infants and children Ascorbate is found in many fruits and vegetables. Citrus
0–6 months 5b fruits and juices are particularly rich sources of vitamin C
7–12 months 10 but other fruits including cantaloupe and honeydew mel-
1–3 years 15 ons, cherries, kiwi fruits, mangoes, papaya, strawberries,
4–6 years 20 tangelo, tomatoes and water melon also contain variable
7–9 years 25 amounts of vitamin C. Vegetables such as cabbage, broc-
Adolescents coli, Brussels sprouts, bean sprouts, cauliflower, mustard,
Females, 10–18 years 35–55 greens, red and green peppers, peas and potatoes are also
Males, 10–18 years 35–55 important sources of vitamin C (Table 6).
Adults
Females Deficiency
19–65 years 55
65 years 55 Deficiency of vitamin C leads to scurvy, which can manifest
Males in as little as 20 days. From the 15th century, scurvy was
19–65 years 65 dreaded by seamen and explorers forced to subsist for months
65 years 65 on diets of dried beef and biscuits. Scurvy was described by
Pregnant women 55 the Crusaders during the sieges of numerous European cities,
Lactating women 55 and was also a result of the famine in 19th century Ireland.
a Three important manifestations of scurvy, namely, gin-
The RNI for each group is based on a daily intake of approximately 1 mg/kg
gival changes, pain in the extremities, and hemorrhagic
body weight of phylloquinone. bThis intake cannot be met by infants who
manifestations precede edema, ulcerations, and ultimately
are exclusively breast-fed. To prevent bleeding due to vitamin K deficiency,
death. Skeletal and vascular lesions related to scurvy prob-
it is recommended that all breast-fed infants should receive vitamin K
ably arise from a failure of osteoid formation. In infantile
supplementation at birth according to nationally approved guidelines.
scurvy, the changes are mainly at the sites of most active
Source: Vitamin and mineral requirements in human nutrition, WHO.
bone growth; characteristic signs are a pseudoparalysis
639
Table 6 Recommended nutrient intakes (RNIs) for vitamin C Vitamin B Complex
Group RNI (mg/day) Vitamin B1—Thiamine

Infants and children It functions as a coenzyme, thiamine pyrophosphate (TPP)
0–6 months 25 in the metabolism of carbohydrates and branched-chain
7–12 months 30 amino acids. It is required for the normal functioning of the
1–3 years 30 brain, nerves, muscles and the heart. Carbohydrate metab-
4–6 years 30 olism is impossible without thiamine.
7–9 years 35
Adolescents Sources
10–18 years 40
Whole grains, nuts, legumes, pork are good sources of
Adults
19–65 years 45
thiamine.
65 years 45
Pregnant women 55 Deficiency
Lactating women 70 Intake of polished rice, alcoholism and ingestion of raw
Source: Vitamin and mineral requirements in human nutrition, WHO. fish lead to the deficiency of thiamine.
Mild deficiency of thiamine leads to depression,
anorexia, fatigue and inability to concentrate. Hence, this
of the limbs caused by extreme pain on movement vitamin has also been called as ‘morale vitamin’.
and caused by hemorrhages under the periosteum, as Severe deficiency leads to beriberi. Beriberi occurs in
well as swelling and hemorrhages of the gums surround- two forms: wet and dry. Dry beriberi is characterized by
ing erupting teeth. In adults, one of the early principal impairment of sensory and motor function and muscle wast-
adverse effects of the collagen-related pathology may be ing. Wet beriberi presents with edema, cardiac enlargement
impaired wound healing. Vitamin C deficiency can be and tachycardia.
detected from early signs of clinical deficiency, such as Wernicke–Korsakoff syndrome occurs in the deficiency
the follicular hyperkeratosis, petechial hemorrhages, swol- of thiamine and is characterized by nystagmus, ataxia and
len or bleeding gums and joint pain, or from the very mental confusion in alcoholics.
low concentrations of ascorbate in plasma, blood or
leukocytes. Dental considerations
Tongue changes in the form of an enlarged, flabby, red
Dental considerations and edematous appearance with enlargement of fungiform
One of the earliest manifestations of scurvy is gingivitis papillae have been associated with thiamine deficiency.
leading to periodontitis and resulting in tooth mobility and The gingiva becomes inflamed and presents with an ‘old
loss of teeth. However, vitamin C deficiency per se does rose’ color (Table 7).
not lead to gingivitis. Gingivitis is caused by bacterial
plaque. Vitamin C deficiency may aggravate the gingival Vitamin B2—Riboflavin
response to plaque and worsen the edema, enlargement
Riboflavin is a flavoprotein and is part of the oxidation
and bleeding.
chain in the mitochondria, acting as a coenzyme in oxidation-
Acute vitamin C deficiency results in edema and hem-
reduction reactions. It is required for the metabolism of
orrhage in periodontal ligament, osteoporosis of alveolar
carbohydrate, proteins and fat. It is also required for the
bone, tooth mobility and degeneration of collagen fibers.
maintenance of mucous membranes (Table 8).
Vitamin C deficiency accentuates the destructive effect of
gingival inflammation on the underlying periodontal liga-
ment and bone. Vitamin C influences the metabolism of col- Sources
lagen in the periodontium, affecting the ability of the tissue Milk and milk products, grains, meat, poultry and fish.
to regenerate and repair itself.
Scurvy also leads to changes in teeth in the form of
Deficiency
hypoplastic teeth. Vitamin C deficiency leads to the atro-
phy of ameloblasts and odontoblasts leading to a disrup- Riboflavin (vitamin B2 ) deficiency results in the condition
tion in their orderly polar arrangement. Thus, dentin formed of hypo- or ariboflavinosis. As riboflavin deficiency almost
resembles osteodentin, with a lack of parallel arrangement invariably occurs in combination with a deficiency of other
of dentinal tubules. Severe deficiency of vitamin C leads to B complex vitamins, some of the symptoms (e.g. glossitis and
hypercalcification of predentin. dermatitis) may result from other complicating deficiencies.
640
Table 7 Recommended nutrient intakes (RNIs) for thiamine resection of the small bowel, and decreased gastrointestinal
passage time.
Group RNI (mg/day)
Infants and children Dental considerations
0–6 months 0.2 Oral symptoms are a prominent feature of riboflavin defi-
7–12 months 0.3
ciency. These are characterized by angular cheilitis and
1–3 years 0.5
glossitis.
4–6 years 0.6
7–9 years 0.9
In the initial stages, there is a reddish appearance of the
tip and lateral margins of the tongue. The tongue appears
Adolescents
to be coarsely granular due to atrophy of filiform papillae
Females, 10–18 years 1.1
Males, 10–18 years 1.2
and swelling of fungiform papillae. Severe cases lead to
atrophy of all papillae causing the tongue to appear smooth
Adults
and glazed. Riboflavin deficiency also leads to increased
Females, 19 years 1.1
Males, 19 years 1.2
vascularization causing the tongue to appear magenta in
color.
Pregnant women 1.4
Angular cheilitis initially presents as pallor of the lips
Lactating women 1.5 at the angles of the mouth. This is followed a few days
Source: Vitamin and mineral requirements in human nutrition, WHO. later by reddening of lips due to the desquamation of the
epithelium and fissuring at the angles of the mouth. There
may be only one or multiple fissures. These fissures develop
a yellow crust, which can be removed without bleeding.
Table 8 Recommended nutrient intakes (RNIs) for riboflavin,
If the deficiency is not treated, the fissures become deep
by group
and may bleed and become painful. Secondary infection
Group RNI (mg/day) with oral and/or skin microorganisms may occur. The
Infants and children incidence of the orolingual and dermal lesions in India is
0–6 months 0.3 high about 5–10%, particularly in pregnant women and in
7–12 months 0.4 school-going children.
1–3 years 0.5 Angular stomatitis, however, may be associated with
4–6 years 0.6 iron deficiency anemia. Angular cheilitis, however, is
7–9 years 0.9 often associated with fungal infections, lip-sucking and
Adolescents dehydration.
Females, 10–18 years 1.0 Patients with riboflavin deficiency also reveal a scaly,
Males, 10–18 years 1.3 greasy dermatitis of the alae of nose and nasolabial folds.
Adults Corneal vascularization, superficial and interstitial kerati-
Females, 19 years 1.1 tis have also been reported in ariboflavinosis. Impairment
Males, 19 years 1.3 of psychomotor performance tests in riboflavin deficiency
Pregnant women 1.4 has been reported to occur since physical work increases
Lactating women 1.6 riboflavin requirement. Riboflavin deficiency has also been
Source: Vitamin and mineral requirements in human nutrition, WHO.
implicated in the etiology of cataract.
The major cause of hyporiboflavinosis is inadequate METABOLIC DISORDERS

dietary intake as a result of limited food supply, which is
sometimes exacerbated by poor food storage or processing. Lysosomal Storage Diseases
Children in developing countries will commonly demon-
Lysosomes are subcellular organelles containing specific
strate clinical signs of riboflavin deficiency during periods
hydrolases that allow targeted processing or degradation
of the year when gastrointestinal infections are prevalent.
of proteins, nucleic acids, carbohydrates and lipids. There
Decreased assimilation of riboflavin also results from
are more than 40 different types of lysosomal storage
abnormal digestion, such as that which occurs with lactose
diseases.
intolerance. This condition is highest in African and Asian
Metabolic diseases can be categorized as:
populations and can lead to a decreased intake of milk, as
well as an abnormal absorption of the vitamin. Absorption 1. Disorders of protein metabolism
of riboflavin is also affected in some other conditions, for 2. Disorders of carbohydrate metabolism
example, tropical sprue, celiac disease, malignancy and 3. Disorders of lipid metabolism
641
Disorders of Protein Metabolism Investigations A diagnosis of amyloidosis is first made

on the basis of clinical suspicion; a tissue biopsy is used to
Amyloidosis It is a rare disease characterized by the establish a definitive diagnosis. Biopsy followed by Congo
deposition of an insoluble extracellular fibrillar protein in red staining is considered the gold standard for diagnosis.
various tissues of the body. This protein called amyloid is Amyloid shows an apple-green birefringence when viewed
not a single entity, but has three major and several minor with polarized light microscopy. Biopsy is generally from
biochemical forms. The major biochemical forms include the organ suspected to be involved. Gingival biopsy may
AL (amyloid light chain), derived from plasma cells; AA be taken in generalized amyloidosis. After the sample has
(amyloid-associated) protein synthesized by liver; and A yielded a positive result, the type of amyloidosis must be
amyloid found in the cerebral lesion of Alzheimer's dis- determined. Immunofixation electrophoresis of serum or urine
ease. The characteristic physical nature of amyloid is its will enable the detection of monoclonal immunoglobulins
crossed -pleated sheet conformation as demonstrated by or light chains in 90% of patients with AL amyloidosis.
X-ray crystallography and infrared spectroscopy. In patients whose light chains are not detected, a bone mar-
Classification Amyloidosis can be primary systemic, row biopsy can be used to detect the clonal dominance of
secondary systemic, localized, myeloma-associated or plasma cells by immunohistochemical staining techniques.
hereditary-familial. These categories constitute 56%, 8%, If no evidence of plasma cell dyscrasias is noted, other types
9%, 26% and 1% of cases respectively. The primary systemic of amyloidosis should be considered, such as the hereditary-
form affects mainly mesenchymal tissues such as the heart, familial type. Patients suspected of having AA amyloidosis
tongue, and gastrointestinal tract and is characterized by should undergo immunohistochemical staining of their
the deposition of AL amyloid. The secondary form is how- serum for the detection of the AA protein.
ever associated with destructive chronic inflammatory dis-
eases such as tuberculosis, leprosy, rheumatoid arthritis, Treatment The treatment of amyloidosis is directed both
ankylosing spondylitis and osteomyelitis and is character- toward the affected organ and the specific type of the dis-
ized by the deposition of AA amyloid. This form affects ease. Nephrotic involvement may necessitate the need for
primarily the kidneys, adrenals, liver and spleen. About diuretics and dialysis, and cardiac involvement may dic-
12% of myeloma patients develop amyloidosis. Various tate the need for diuretics as well. Colchicine, intermittent
heredofamilial types have been reported including familial oral melphalan, prednisone, vincristine and adriamycin, in
Mediterranean fever and transthyretin-related amyloidosis combination with dexamethasone have met with consid-
(ATTR) type. erable success. Treatment with high-dose intravenous
melphalan with autologous blood stem cell support results
Clinical features Generally, peak age incidence is in the in complete remission of the plasma cell dyscrasia. Renal
fifties, with a male-to-female ratio of 2:1 and no racial transplantation is extremely successful in providing dra-
bias. Clinical manifestation depends on the organ or site matic symptom relief for patients with dialysis-related
of involvement and the severity of involvement. Its mani- amyloidosis. The definitive therapy for ATTR amyloidosis
festations range from asymptomatic deposition to serious is liver transplantation.
clinical impairment to even death. Initial symptoms include
fatigue, weakness and weight loss. Later, the disease may Prognosis The prognosis for patients with generalized
manifest as renal disease, hepatomegaly, splenomegaly or amyloidosis is poor if left untreated. The disease is rap-
cardiac abnormalities. idly fatal within 1–3 years of diagnosis. Diagnosis is mostly
made at postmortem; the 5-year survival rate has been put
Oral considerations Oral manifestations of systemic at 20%. Prognosis of secondary amyloidosis depends on the
amyloidosis have been well documented. The tongue is the control of the underlying condition. Patients with myeloma-
most frequently reported location of intraoral amyloid associated amyloidosis have a poorer prognosis.
deposition. Macroglossia commonly develops, and diffi-
culty with speaking and chewing may ensue. Dental inden- Porphyria The term ‘porphyria' is derived from the
tations of the lateral borders of the tongue may be evident Greek word porphuros, meaning ‘purple pigment’, after
owing to pressure against the teeth. Other sites in the oral the purple-red urine that some patients produce during an
cavity that may have amyloid deposition include the buc- acute attack.
cal mucosa, palate, gingiva and floor of the mouth. A rare Porphyrias are a group of inherited or acquired disor-
instance of amyloid deposition of amyloid in the parotid ders of certain enzymes in the heme biosynthetic pathway
gland has been reported. (also called porphyrin pathway).
In the absence of clinical symptoms, oral tissues have Porphyrias can be categorized into acute porphyria and
been advocated for biopsy to detect amyloid deposition. cutaneous porphyria.
Oral biopsy sites that have demonstrated presence of amy- Acute porphyrias are further subdivided into acute
loid are numerous, including the gingiva, parotid gland, intermittent porphyria, hereditary coproporphyria, varie-
minor salivary glands of the lower lip and buccal mucosa. gate porphyria and ALA dehydratase-deficient porphyria.
642
Cutaneous porphyria is divided into congenital erythro- Congenital erythropoietic porphyria Congenital erythro-
poietic porphyria (Gunther’s disease), porphyria cutanea poietic porphyria or Gunther’s disease is a rare autosomal
tarda and erythropoietic protoporphyria. recessive disorder that usually presents with marked skin
photosensitivity, hypertrichosis, blistering, scarring, milia
Clinical features formation and dyspigmentation of the photo-exposed areas.
Acute intermittent porphyria It is an autosomal domi- The enzyme URO-3-synthetase is deficient. It typically
nant disorder caused by the deficiency of uroporphyrino- begins in infants or young children.
gen I synthetase. Acute attacks in this disease are triggered Sun-exposed regions of the body such as the hands, neck
by alcohol consumption, tobacco use, stress and hormonal and face may exhibit vesicular or bullous lesions. These ves-
changes such as in luteal phase of menstrual cycle and icles subsequently rupture and heal with scar formation.
pregnancy. Men and women are equally affected. The Owing to its physical affinity to calcium phosphate, por-
symptoms of the disorder are frequently manifested after phyrin binds to teeth, nails and bones. Both the deciduous
puberty. and permanent dentition appear red or brown in color
In acute episodes the urine turns purple-red following (erythrodontia).
exposure to sunlight. Acute attacks are characterized by
Porphyria cutanea tarda It is an autosomal dominant
nausea, vomiting, malaise, myalgia and chest pain. Some
disorder and the most common of all the porphyrias. It is
individuals may present with depression, confusion, hal-
caused by the deficiency of URO-decarboxylase.
lucinations and seizures. Severe forms of the condition
Alcohol consumption, hepatitis C and medications such
may result in respiratory paralysis.
as iron supplements and hormonal replacements can pre-
Downey (1992) reported a patient who complained of
dispose to porphyria cutanea tarda.
severe abdominal pain, seizures, diarrhea, drooling of saliva
Cutaneous photosensitivity is the most striking feature.
associated with a metallic taste immediately after placing
Exposure to sun leads to the formation of vesicles and bul-
a fixed partial denture containing pallidum (76%), copper
lae which subsequently heal forming crusts. These indi-
(10%) and gold (2%). The symptoms subsided on removing
viduals may exhibit hyperpigmentation and hirsutism.
the denture. This patient was diagnosed as suffering from
acute intermittent porphyria. Erythropoietic protoporphyria It is an autosomal domi-
Acute intermittent porphyria can be diagnosed by the nant disorder caused by the deficiency of ferrochelatase.
qualitative assessment of urine for porphobilinogen. The striking feature of this type of porphyria is the devel-
opment of cutaneous manifestations of pain, redness, itch-
Hereditary corpoporphyria It is the rarest form of acute ing and burning sensation within a few minutes of sun
porphyria characterized by the deficiency of enzyme corpo- exposure. Thickening of the skin and nail deformities are
oxidase. It is an autosomal dominant condition which is seen in chronic cases. Unlike other forms of porphyria
usually more common in women. The trigger factors are the hyperpigmentation and hirsutisms are rare.
same as that of the acute intermittent porphyria. However,
acute attacks are less common and less severe.
The clinical symptoms are the same as that of the acute Orofacial manifestations and dental
intermittent type. These patients may exhibit cutaneous considerations
photosensitivity. Individuals suffering from cutaneous porphyria may pres-
Elevated levels of coproporphyrins in stool analysis is ent with long eyelashes, bushy eyebrows and facial hair
diagnostic of hereditary corpoporphyria. (facial hirsutism).
Oral mucosa is very vulnerable even to the mildest trauma
Variegate porphyria The term ‘variegate' is used to
due to the accumulation of porphyrin precursors. Intraoral
describe the variety of clinical manifestations that this form
erosions and bullae may be seen.
of porphyria exhibits. It can manifest as an acute form, cuta-
Patients suffering from congenital erythropoietic porphyria
neous form, both or occasionally appear clinically latent.
(CEP) may exhibit pallor of the oral mucosa. Occasionally,
Protocoproporphyria hereditaria or South African por-
the alveolar mucosa may appear bluish purple owing to
phyria (widely prevalent in the white population of South
the discolored underlying alveolar bone.
Africa) are other names by which variegate porphyria is
Deciduous dentition of CEP patients may appear deep
known.
red-brown in color. Permanent teeth may not be severely
Vareigate porphyria is diagnosed based on the marked
discolored. The cervical regions of teeth are more intensely
increase in the levels of protoporphyrin in stool.
discolored compared to the occlusal region. These discol-
ALA dehydratase-deficient porphyria It is an acute ored teeth tend to fluoresce with Wood’s light (near UV
form of porphyria whose pattern of inheritance is still not light of 365 nm wavelength).
known. The clinical presentation mimics that of acute It is believed that the concentration of porphyrin is higher
intermittent porphyria. in the dentin compared to the enamel.
643
Fayle and Pollard (1994) reported that the enamel MPS I or Hurler syndrome is the most common. All of
showed marked thinning in a 4-year-old child suffering them are inherited as autosomal recessive traits except
from CEP. They believed that the thinning of enamel can MPS type II—Hunter syndrome, which is inherited as an
increase the caries risk in these individuals. X-linked trait.
Use of carbohydrate in the management of acute por-
phyria can cause extensive periodontal disease and high Mucopolysaccharidosis I (MPS I) It is caused by defi-
caries incidence. cient or absent activity of the lysosomal hydrolase-L-
Oral B carotene which is used to improve tolerance to iduronidase. This enzyme is responsible for the degradation
light in CEP and erythropoietic protoporphyria patients of heparan sulfate and dermatan sulfate, and a deficiency
can induce carotenemia, leading to yellowish-orange disin the enzyme leads to accumulation of these substances
coloration of the oral mucosa and tongue. in various tissues.
As heavy metals can provoke an acute episode of por- MPS I includes three subtypes:
phyria, dental amalgam has been recommended to be ❍ MPS I-H (Hurler syndrome): The most severe form in
avoided in these patients. which the onset is in infancy.
Light in the operation theatre have known to cause ❍ MPS I-H/S: The intermediate form, characterized by men-
burns in some patients. Hence, the light on the dental chair tal retardation and other features of Hurler syndrome.
should be considered a potential hazard to these patients. ❍ MPS I-S (Scheie syndrome): The least severe form
It is advisable to use filters that remove light in the wave- having its onset in adulthood and presenting no fea-
length of 400–550 nm. tures of mental retardation.
Various medications that can be used and those that
have to be avoided are listed in Table 9. Hurler syndrome (Gargoylism) The worldwide incidence
of MPS I-H has been reported to be 1:100,000. The chro-
mosomal abnormality in this syndrome has been mapped
Disorders of Carbohydrate Metabolism to 4p16.3.
Mucopolysaccharidoses (MPS) These are normal com- Clinical features The disease becomes apparent in the
ponents of the cornea, cartilage, bone, connective tissue first 2 years of life, progressing in childhood. Death usu-
ground substance and the reticuloendothelial system. ally occurs before the 2nd decade of life, unless affected
MPS are characterized by the storage of mucopolysaccha- patients are treated by bone marrow transplantation.
rides in various tissues. Theses are caused by the deficiency Patients are characterized by dwarfism, mental retardation,
of the enzymes required for their degradation. The overall skeletal malformations, hernias, cardiac disease and sys-
prevalence of MPS is about 1 in 25,000. The variants of temic hypertension, hepatosplenomegaly and flexion con-
MPS have been classified as types I through VII. Of these, tractures. The facial features are characteristic, with patients
having a large head, prominent forehead, a broad saddle
Table 9 Drug recommendations in patients suffering from nose, hypertelorism, puffy eyelids and an open mouth.
porphyria The oral and dental findings of MPS I-H include hyper-
plastic gingiva, macroglossia, high-arched palate, short man-
Safe drugs Contraindicated drugs
dibular rami with abnormal condyles, spaced hypoplastic
(commonly used) (commonly used)
peg-shaped teeth with retarded eruption; and localized den-
Acetaminophen Alcohol tigerous cyst-like radiolucencies. Gingival hyperplasia has
Acetyl salicylic acid Clindamycin been reported in some patients. Guven et al reported that
Acyclovir Diclofenac MPS I-H—affected dentin was characterized by extremely
Amoxicillin Erythromycin
narrow dentinal tubules, whose direction followed an irreg-
ular wave-like pattern. The enamel–dentin junction had
Amphotericin B Lidocaine
microgaps, and the enamel displayed irregular arrangement
Bupivacaine Mepivacaine of prisms. There was a decrease in the protein content of
Codeine Miconazole enamel and dentin.
Dexamethasone Prilocaine The mucopolysaccharides accumulate in fibroblasts, giv-
Gentamicin Pyrazolones ing them an appearance of ‘clear’ or ‘gargoyle’ cells, and
Ibuprofen Carbamazepine
hence the term ‘gargoylism’. Elevated levels of mucopoly-
saccharides are found in urine.
Iron Phenytoin sodium
Nitrous oxide Dapsone Mucopolysaccharidosis II (Hunter syndrome) The bio-
Penicillins Sulfonamides chemical cause of Hunter syndrome is a deficiency in the
Streptomycin Oral contraceptives (some)
activity iduronate-2-sulfatase and is characterized by the
deposition of dermatan sulfate and heparan sulfate in tissues.
644
It has an estimated prevalence of 1 in 170,000 male live Clinical features The syndrome is characterized by severe
births. Its prevalence among Ashkenazi and Oriental Jews skeletal changes including hypoplasia of odontoid process,
is reported to be approximately twice that reported in other short neck, pectus craniatum, kyphoscoliosis and dwarfism.
populations. Hunter syndrome is an X-linked, recessive Other findings include laxity of joints, neurosensory deaf-
inherited disease that affects males nearly exclusively. The ness and corneal clouding. Mental retardation is absent.
gene responsible for the syndrome is located at Xq28. Characteristic dental findings are found in MPS IV A,
but not in MPS IV B. These have been described as miner-
Clinical features In its severe form, clinical features
alization disturbances of enamel leading to the appearance
appear between 2 and 4 years of age in the form of severe
of dull grayish to yellowish crowns, small teeth, severe
mental impairment. Death usually occurs in the 1st or 2nd
attrition, and pointed cusps. The enamel, however, had a
decade of life, following obstructive airway disease and/or
normal radiodensity. Damage to TMJ has been reported in
cardiac failure. The disease may have a slightly later onset,
the form of irregularity of cortical bone of the head of con-
with minimal neurologic dysfunction. These patients have
dyle, possibly aggravated by severe attrition of teeth.
normal intelligence and survive into adulthood.
Diagnosis of the disease is by the estimation of keratan
Clinical features include coarsening of facial features,
sulfate levels in urine and plasma.
broad noses with flared nostrils, prominent supraorbital
ridges, large jaws, and thick lips, enlarged protruding tongue. Mucopolysaccharidosis V: absent Mucopolysaccharido-
Mobility is restricted because of joint stiffness and consis VI (Maroteaux–Lamy syndrome). It occurs due to a
tractures. The skeletal findings of Hunter syndrome are col- deficiency of arylsulfatase B (N-acetylgalactosamine-4-
lectively known as dysostosis multiplex. Corneal clouding sulfatase), which results in the accumulation of dermatan
is not a feature of Hunter syndrome. There may be progres- sulfate within lysosomes. An incidence of 1:100,000 to
sive hearing loss, sleep apnea, hepatosplenomegaly, val- 1:1300,000 has been reported.
vular heart disease, progressive airway obstruction, and
distinctive skin lesions, described as ivory-white papules Clinical features Patients suffering from Maroteaux–
that are 2–10 mm in diameter, often coalescing to form Lamy syndrome have similar somatic features resembling
ridges. Diagnosis is established by measurement of urine those of the other mucopolysaccharidoses, but distin-
mucopolysaccharide levels and enzyme activity. guished by the presence of normal intelligence, prominent
metachromatic inclusions in leukocytes and a deficient
Mucopolysaccharidosis III (Sanfilippo syndrome) It is activity of arylsulfatase B. Characteristic features of this
characterized by the deposition of heparan sulfate in tis- disease include growth retardation, hernias, a large deformed
sues. Based on the deficient enzyme, this syndrome is clas- head, typical facies, a short neck and spinal abnormalities.
sified into four subtypes A, B, C and D. The incidence of Hepatosplenomegaly results from mucopolysaccharide
the combined MPS III subtypes has been estimated at 1 in deposits in several organs. Severely affected MPS VI patients
24,000 births to 1 in 66,000 with MPS III A being the most are usually diagnosed in infancy or early childhood and the
common subtype. MPS III A is characterized by the defi- condition usually results in death in the 2nd decade of life.
ciency of heparan-N-sulfatase, MPS III B by N-acetyl-- Death is usually a result of either respiratory tract infection
glucosaminidase, MPS III C by acetyl-CoA-glucosaminide or cardiac disease, which are caused by the deposition of
N-acetyltransferase deficiency, and MPS III D by the defi- mucopolysaccharides.
ciency of N-acetylglucosamine-6-6-sulfate sulfatase. The craniofacial features in this syndrome are similar
Clinical features Symptoms begin in late infancy with to Hurler syndrome. The head is large, with a prominent
various patterns of neurodegeneration resulting in severe forehead, often marked supraorbital ridges and temporal
mental retardation characterized by rapid loss of social bulge. Rhinitis with rhinorhea is common and corneal
skills with aggressive behavior and hyperactivity, and dis- opacity is also observed. At rest, the mouth is open and the
turbed sleep patterns. In profoundly affected patients, enlarged tongue tends to protrude. The teeth have been
delayed speech development is often noticed at 2 years of reported as being short or stubby, misshapen, malformed,
age. Hirsutism, coarse facies and diarrhea are found. Mild peg-shaped, poorly formed, abnormal, poorly calcified,
hepatosplenomegaly and mild skeletal disturbances may and hypoplastic. Usually there is an anterior open bite
be found. Diagnosis depends on enzymatic analysis and relationship in association with macroglossia. Localized
heparan sulfaturia. radiolucent areas resembling dentigerous cyst-like folli-
cles are also reported in Maroteaux–Lamy syndrome. The
Mucopolysaccharidosis IV (Morquio syndrome) This margins of the radiolucencies are usually smooth and
syndrome is characterized by the deposition of keratan clearly defined. These radiolucencies are caused by accu-
sulfate and chondroitin-6-sulfate in tissues. It is subclassi- mulation of mucopolysaccharides in the tissues.
fied into Morquio type A, characterized by the deficiency
of N-acetyl galactosamine; and Morquio type B, caused by Mucopolysaccharidosis VII (Sly syndrome) It is caused
the deficiency of -galactosidase. by a deficiency in the enzyme -glucuronidase. This
645
results in the accumulation of heparan sulfate and dermatan mental retardation and neuropsychiatric illnesses. Intestinal
sulfate in tissues. bleeding has been reported following hyaline deposits in
the small bowel.
Clinical features The syndrome is highly variable, with
clinical presentation ranging from severe lethal hydrops
Oral manifestations
fetalis to mild forms with survival into adulthood. Sewell
et al suggested that MPS VII comprises three main clinical The oral mucosa appears thickened and nodular. The sites of
groups: an early severe lethal form, an intermediate form involvement are the labial, buccal and palatal mucosa, pos-
with slight organomegaly and moderate skeletal anoma- terior tongue, uvula, tonsillar region and lingual frenulum.
lies and a very mild form in which patients present later The typical oral lesions are seen as yellowish-white
and show longer survival. Most patients show hepato- papules or plaques.
splenomegaly, and skeletal anomalies such as dispropor- Involvement of the lingual frenum causes difficulty in
tionate dwarfism, sternal protrusion, kyphosis, scoliosis, tongue protrusion. Macroglossia and ‘wood-hard’ tongue
and a broad-based gait. Craniofacial dysmorphism charac- are other characteristic features. Lips may be enlarged,
terized by epicanthus, anti-mongoloid eyelids, short nose nodular and may exhibit fissuring.
with anteversion of the nostrils, macroglossia, and coarse Occasionally, patients may report dry mouth secondary
facies may be seen. A variable degree of mental impair- to infiltration and obstruction of the parotid duct by the
ment and delayed speech is present. hyaline material. Gingival hyperplasia has also been
reported.
Management of mucopolysaccharidoses Methods of
Oezarmagan et al (1993) in their report of lipoid pro-
replacing the enzyme missing in MPS have included
teinosis in two sisters, describe the findings of overreten-
bone marrow transplantation, human amnion membrane
tion of deciduous teeth and oligodontia.
implantation, fibroblast transplantation, serum or plasma
infusion, white blood cell infusions, gene therapy, intra-
peritoneal implantation of myoblasts overexpressing I2S
and enzyme replacement therapy. Future prospects include Bean-shaped radiopacities in the temporal lobe or hippo-
gene therapy, newborn screening and prenatal screening. campus on skull radiographs are typical findings in lipoid
proteinosis.
Lipoid Proteinosis
Lipoid proteinosis also known as hyalinosis cutis et muco-
sae or Urbach–Wiethe disease. It is transmitted as an auto- On histopathologic evaluation the hyaline deposits exhibit
somal recessive disorder. Lipoid proteinsosis is characterized positivity for periodic acid-Schiff (PAS) and Congo red.
by generalized thickening and scarring of the skin, muco- However, the material is diastase resistant.
sae and occasionally some viscera secondary to diffuse Ultrastructural features include concentric rings of
deposition of a hyaline-like substance. excess basement membrane surrounding blood vessels,
Recent studies have shown that lipoid proteinosis is and irregular reduplication of lamina densa at dermoepi-
caused by the mutations in a glycoprotein (extracellular dermal junction resulting in onion-skin appearance.
matrix protein 1) that is expressed in various tissues. The Dyer et al (2006) studied the lesional epidermis, cultured
mutation was mapped to a locus on chromosome 1q21. monolayer keratinocytes and raft keratinocyte cultures
from blistering lesions of a child with lipoid proteinosis.
Clinical features Their ultrastructural studies showed the dissociation
of relatively intact desmosomes from keratinocytes, with
Deposits in the vocal cords may manifest as inability of
desmosomes which were ‘free-floating’ in the intercellular
babies to cry at birth and hoarseness of voice from early
spaces or attached by thin strands to the cell membrane.
infancy or childhood.
They suggest that these ‘free-floating’ desmosomes could
The skin characteristically shows whitish, moniliform
be a result of an inherent defect in keratinocyte adhesion.
papules on the eyelids (blepharosis moniliformis, eyelid bead-
ing), and yellowish or waxy papules or nodules on the lips,
Management
knuckles, hands, knees, elbows, axillae and the perineal
regions. Lipoid proteinosis to date has no definitive treatment. How-
Occasionally, the face may exhibit vesiculobullous erup- ever, Wong and Lin (1988) successfully used oral dimethyl
tions which later form scars. Patchy alopecia has also been sulfoxide in a 41-year-old man. They initially used a dose
reported. of 40 mg/kg/day, which was subsequently increased pro-
Other features include dyspnea, dysphagia, corneal opac- gressively to 60 mg/kg/day. In their follow-up after 3 years
ities and glaucoma. Calcifications of intracerebral para- of treatment, the patient’s skin lesions, hoarseness of voice
sellar or hippocampal gyri may be associated with epilepsy, and abnormal esophageal function improved to a great
646
extent. The only side effect they encountered was the hand and feet are usually spared. Mandibular posterior
garlic-like smell on the patient’s breath. region is usually the site to be affected.
Intralesional heparin and ketretinate have also been used
Radiographic features Alveolar bone may show irregu-
with limited success. Other treatment options used include
lar radiolucent lesions. Larger lesions may cause destruc-
CO2 laser surgery of vocal cords and beaded eyelid pap-
tion of the cortical plate resulting in pathological fracture.
ules, and dermabrasion of skin for aesthetic and functional
However, lesions within the mandible appear as
reasons.
well-defined radiolucent areas mimicking cysts.
In severe forms of the condition, resulting in respiratory
These varied radiographic appearances of eosino-
embarrassment, tracheostomy is performed.
philic granuloma can be mistaken for odontogenic cyst,
osteomyelitis or a malignancy.
Disorders of Lipid Metabolism
Management The currently accepted methods of man-
Non-lipid reticuloendothelioses It involves diseases such aging eosinophilic granuloma include surgical curettage,
as unifocal eosinophilic granuloma, multifocal eosinophilic low dose radiation (not more than 10 Gy) and chemother-
granuloma (Hand–Schuller–Christian disease) and Letterer– apy used alone or in combination.
Siwe disease. These diseases are collectively referred to as
histiocytosis X disease.
Histiocytosis X is the term used to describe a group Hand–Schuller–Christian Disease or Multifocal
of conditions affecting the reticuloendothelial system. Eosinophilic Granuloma
Lichtenstein in 1953 coined the term histiocytosis X. It is
The condition was named after Henry Asbury Christian,
a non-neoplastic lesion of unknown etiology character-
Alfred Hand Jr and Artur Schüller.
ized by an intense proliferation of histiocytes along with a
It is a chronic form of eosinophilic granuloma charac-
number of eosinophilic leukocytes, neutrophilic leuko-
terized by disseminated form of skeletal and extraskeletal
cytes, lymphocytes, plasma cells and multinucleate giant
(soft tissue) lesions.
cells.
However, this term has now been changed to Langerhans
cell histiocytosis (LCH) by Risdall et al (1983), as these Clinical features
diseases are known to involve Langerhans cell precursors. Facial asymmetry secondary to involvement of the facial
Eosinophilic granuloma is considered to be the chronic bones is a characteristic feature. Patients may complain of
localized form of LCH which also includes Hand–Schuller– pain, swelling and progressive facial asymmetry.
Christian disease (the chronic disseminated form) and Ribs, vertebrae, femur and pelvis are other bones that are
Letterer–Siwe disease (the acute disseminated form). commonly affected. It is believed that about 25% of the
Radiographic features of histiocytosis X Dagenais et al patients present with the classical triad of signs and symp-
(1992) described the characteristic radiographic features of toms that include punched-out radiolucent areas in the
histiocytosis X. They reported that seven characteristics, skull on a radiograph, exophthalmos and dwarfism, infan-
either alone or in combination, are useful in the identifica- tilism or polyuria.
tion of histiocytosis X. These include the appearance of
Oral manifestations
solitary ‘intraosseous’ lesions, the multiplicity of ‘alveolar
bone’ lesions, the ‘scooped-out’ effect in the alveolar pro- The oral manifestations in these patients may mimic man-
cess, the well-defined periphery, sclerosis in the alveolar ifestations of advanced periodontitis such as halitosis, gin-
bone lesions, periosteal new bone formation and slight gival inflammation with purulent discharge, spontaneous
root resorption. exfoliation of teeth and extensive loss of alveolar bone.
Other symptoms include loss of taste sensation and oral
Eosinophilic granuloma It affects men twice as more
mucosal ulceration or soreness.
than women. It is usually seen in young adults. The condi-
Radiographs may reveal displacement of teeth, and
tion may have varied presentations. Some patients may
sometimes teeth ‘floating in air’ appearance may be appre-
complain of pain whereas in some others it may be inci-
ciated. Bone scan may help in identifying multifocal dis-
dentally evident on routine radiographic evaluation.
ease. The bone destruction may be round, oval or irregular,
It is believed that oral involvement is usually associated
occasionally with a periosteal reaction
with pain, swelling, mucosal ulcerations and gingival
inflammation. Ardekian et al (1999) in their study found
Diagnosis
that 44% of patients complained of pain and about 48%
also complained of swelling in the affected area. The bones Radiographic features, histopathologic features and
that are commonly affected are the skull bones, mandible, laboratory investigations are sufficient to diagnose this
ribs, femur, humerus and pelvis. However, the bones of the condition.
647
Histologically the lesion progresses through the phase LIPID RETICULOENDOTHELIOSES

of proliferative histiocytic phase, vascular granulmatous
phase, diffuse xanthomatous phase (abundance of foam Gaucher Disease
cells), and finally the fibrous or healing phase.
Laboratory studies reveal normal serum cholesterol Gaucher disease is a rare autosomal recessive disorder
level and an elevated tissue cholesterol level. characterized by defective function of the catabolic
enzyme -glucocerebrosidase, leading to an accumulation
Management of its substrate, glucocerebroside, in the mononuclear
phagocyte system, lymph nodes, bone marrow; Kupffer
Surgical curettage or excision is the ideal mode of treat- cells in the liver; osteoclasts in bone; microglia in the cen-
ment. Lesions that are not amenable to surgery can be tral nervous system; alveolar macrophages in the lungs;
irradiated or drugs such as vinblastine or cyclophospha- and histiocytes in the gastrointestinal tracts, genitourinary
mide can be used. tracts, and the peritoneum. Cells with this deposition are
However, almost 50% of the patients exhibit spontane- termed Gaucher cells.
ous remission over a period of time.
Classification
Letterer–Siwe Disease There are three clinical variants caused by distinct allelic
It is named after Erich Letterer and Sture August Siwe. mutations. Type I, also called as chronic non-neuronopathic
Unlike the other forms of Langerhans cell histiocytosis, form, accounts for 99% of cases of Gaucher disease and
Letterer–Siwe disease is an acute disease and almost has an incidence of 1:100,000 in general population. It is
always seen in infants. It usually runs a short course and most common among Ashkenazi Jews and is compatible
ends fatally. with long life. Type II is termed acute neuronopathic form
and is highly lethal. Type III, termed chronic/subacute
neuronopathic form has a course intermediate between
Clinical features
types I and II.
In Letterer–Siwe disease, recurrent pyoderma-like lesions
with crusting and scaling, vesicopustular and purpuric Clinical features
eruption occurs in crops over the face, scalp and trunk,
resembling seborrheic dermatitis. Type I is characterized by the absence of neurologic symp-
toms. Patients may present with hepatosplenomegaly, leu-
kopenia and thrombocytopenia due to marrow replacement
Oral manifestations
and cortical erosion. Extensive skeletal disease and patho-
In most of the cases, oral manifestations may not be logic fractures may be seen.
appreciated owing to the rapid course of the disease. Neurologic symptoms (in types II and III) include convul-
However, mucosal ulceration associated with hemorrhage, sions, dementia, ocular muscle apraxia, mental retardation
gingival inflammation, extensive loss of the maxilla and and myoclonus.
mandible and spontaneous exfoliation of teeth may be
seen. Oral manifestations
Oral mucosa has a yellow pigmentation and presents pete-
Diagnosis
chiae. Jaw involvement is often asymptomatic and may be
Tzanck smear of the vesicopustules shows pale histiocytes detected on routine dental radiographs. Generalized osteo-
which can be used as a rapid screening test. penia, loss of trabecular structure, effacement of lamina
Skin biopsy shows a proliferative reaction with exten- dura, displacement of mandibular canal, pseudocystic radio-
sive upper dermal infiltration and epidermal invasion lucent lesions and apical root resorption of teeth adjacent
with histiocytes, some of which are atypical, along with to lesions have all been reported. Involvement of maxil-
erythrocytes. lary sinus has been reported in a few cases. Delayed erup-
Typically foam cells and fibrosis are appreciated tion has been reported in more than 50% of cases.
histologically.
Diagnosis
Bone marrow aspiration or biopsy of liver and spleen
The prognosis of this condition is very poor. It is believed reveal the presence of Gaucher cells, which appear as large
that the presence of purpura is a lethal sign. Chemotherapy cells having abundant granular, or fibrillary blue-gray
is usually the only mode of treatment. cytoplasm with a wrinkled tissue paper-like appearance
648
with abundant lightly PAS–positive fibrillary material in Niemann–Pick Disease

the cytoplasm. The level of glucocerebrosidase in leuko-
cytes or cultured fibroblasts is also helpful in diagnosis. It is characterized by the deficiency of the enzyme, sphingo-
myelinase, and accumulation of sphingomyelin in tissues.
Prognosis and treatment
Classification
The infantile form (type II) of Gaucher disease may lead to
It can be classified into two categories based on biochem-
early death. Most affected children die before the age of
ical and molecular criteria. The first category includes types
5 years. Type I of Gaucher disease requires close medical
A and B, the second, type C.
follow-up with periodic assessment. With the availability
of recombinant enzyme, most patients with the adult-
Clinical features
chronic form can look forward to normal or near-normal
life expectancy. Enzyme replacement therapy with a The organs most commonly involved are spleen, liver,
recombinant glucocerebrosidase known as imiglucerase bone marrow, lymph nodes and lungs. The entire central
(Cerezyme) given intravenously is the mainstay of treat- nervous system also becomes involved. The patient pres-
ment for Gaucher disease. Hepatosplenomegaly, anemia ents with massive visceromegaly and severe neurologic
and thrombocytopenia usually improve within 6 months. deterioration.
Unfortunately, it is not effective on neurologic symptoms, Diagnosis is achieved by the estimation of sphigomye-
because it does not cross the blood–brain barrier. Future linase activity in leukocytes or cultured fibroblasts.
treatments may include gene therapy and intervention Prognosis is poor and affected infants die before the
with chemical chaperones. age of 3 years. Antenatal diagnosis is possible.
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SECTION Forensic Dentistry
VIII
24 Clinical and Radiological Perspective 653
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Chapter-24.indd 651 10/3/2012 10:17:10 AM


CHAPTER
Clinical and Radiological
Perspective
Ashith B Acharya
24
➧ Introduction, History and Relevance ➧ Facial Approximation
➧ Dental Identification Clay Modeling
What is Identification? Computer-assisted Approximation
Importance of Teeth in Postmortem Identification ➧ Age Estimation Methods
Principle of Dental Identification Clinical Methods of Age Assessment
Dental Identification Procedure Radiographic Methods for Age Assessment
➧ Challenges in Postmortem Examination Estimating Age in Children and Adolescents
Rigor Mortis Third Molars in Age Estimation
Incineration A Combined Clinical and Radiographic Method
Jaw Resection Estimating Age in Adults
Tooth Colored Restorations ➧ Bite Mark Procedures
➧ Postmortem Alterations to Teeth and Oral How Does a Bite Mark Look?
Tissues Type of Injury
Incinerated Teeth Site of Injury
Postmortem Pink Teeth Differential Diagnosis of Human Bite Marks
Tongue Hemorrhage Bite Mark Investigation
➧ Social Profiling Collecting Bite Mark Evidence
Demographics of the Case
➧ Identifying the Edentulous
Visual Examination and Documentation
Denture Marking Systems
Photographs
Palatal Rugae in Identification
Saliva Swab
➧ Craniofacial Identification Obtaining Dental Evidence from a Suspect
Cranial Suture Pattern Analyzing and Comparing Bite Mark Evidence
Frontal Sinus Configuration Conclusions of Bite Mark Analysis
Skull-Photograph Superimposition
➧ Lip Print Investigation
Challenges in Superimposition
Three-Dimensional Digital Superimposition
INTRODUCTION, HISTORY AND RELEVANCE black anterior tooth. In 1193 AD the Maharaja of Kannauj,
Jai Chandra Rathor, was recognized by his false teeth fol-
Forensic odontology is the branch of dentistry which deals lowing death in a battle. The English duke Charles of
with the law. The last half-century has seen forensic den- Burgundy, who also died in a battle in 1477 AD, was identi-
tistry make tremendous progress globally, both in terms fied from his dental features courtesy, the court physician
of research as well as application in routine casework. who recognized two recently extracted teeth (Furness,
Forensic odontology primarily involves identification. 1972). Paul Revere is credited as being the first dentist to
Right through history, the human dentition has been used identify a person from dental features (Luntz, 1970). He
several times in identifying individuals. Harvey (1973) has identified a friend—who died in the American Revolution
traced one of the earliest recorded incidences of dental in 1775—from a silver and ivory bridge prepared by him.
identification to 66 AD, when the severed head of the wife Subsequently, intermittent cases of forensic dental
of Roman emperor Nero was identified by a rival from her identification in 19th century Europe and America have
653
Section VIII – Forensic Dentistry
been reported (Pedersen, 1965). However, Gustafson (1962) entire chapter to ‘love bites’, with its detailed classification,
believed that the role of forensic odontology in human iden- in the treatise Kama Sutra. The unique arrangement of the
tification came to prominence toward the end of the 19th dental arch was recognized in medieval Britain during the
century after two major fires in Europe: the first occurred reign of William I (1027–1087 AD), green wax seals with
in 1881 when the ‘Ring Theatre’ in Vienna was destroyed the impression of the king’s teeth were implanted on state
during a performance with 449 casualties; and the second— documents to avoid falsification and indicate the authen-
the Charity Bazaar fire in Paris in 1897—resulted in 127 ticity of the seal.
deaths. In both tragedies, dental features were used for The use of bite marks as evidence in court can be traced
identification. According to Harvey (1973), one of the back to 1692 in the United States. Harvey (1973) cites a
dentists who assisted the identifications in Paris was a 1906 case where a burglar was convicted in Britain because
Cuban named Oscar Amoedo. In 1898, he authored one of his dental models fit the marks left in cheese found at the
the first books on forensic odontology, L’art Dentaire en crime scene which was probably the first successful convic-
Medicine Legale, and is considered a pioneer of modern tion based on bite mark evidence in Europe. Subsequently
forensic dentistry. It is noteworthy that Yasutami Kojimahara there have been numerous cases that made use of bite
of Japan had published a similar work titled Dental marks but, with varying degrees of success. Therefore, bite
Jurisprudence a few years earlier in 1894 (Suzuki, 1996). marks remained a contentious area of forensic sciences.
The routine use of dental evidence in human identification However, over the latter half of the 20th century, bite mark
gained impetus during the 1950s and 1960s, and forensic procedures have greatly advanced and it is now routinely
dentistry gained organized status in 1972 with the forma- used in court proceedings of Europe, America and Asia
tion of the International Organization for Forensic Odonto- Pacific. Its objective application as evidence in crime can
Stomatology (IOFOS). have far reaching implications for the society in general
With the evolution of criminal investigation in the and criminology in particular.
industrialized world, scientific methods of detecting both The potential of teeth as legal evidence has rendered
the victim and culprit in a crime concurrently developed. forensic dentists as an integral part of the forensic team of
Fingerprinting was one such method—the dermal ridges on experts. Interest in the subject is not only confined to special-
our fingers are so unique even twins do not share an iden- ist forensic dentists but also oral physicians and radiolo-
tical pattern. The human dentition is considered to be as gists, oral pathologists, pedodontists, prosthodontists and
unique to an individual as fingerprints are. The adult den- forensic medical professionals. This chapter attempts to
tition normally consists of 32 teeth of which some may be address relevant clinical and radiological aspects of foren-
missing, decayed or treated. Effects of various environmen- sic odontology and it is intended to further stimulate the
tal factors such as fluorosis may also manifest in the teeth. reader’s inquisition in this fascinating area.
Hence, it is extremely rare to have two identical dentitions,
and this concept makes use of identifying the deceased.
Moreover, teeth are one of the most durable parts of the DENTAL IDENTIFICATION
body and can withstand physical, chemical and biological
insults. As a result, teeth can be used to build the profile of
What is Identification?
skeletal remains. For example, race and sex differences in
tooth morphology are known to occur; age estimation using Identity refers to the characteristics by which a person
tooth eruption, calcification and histological methods is fre- may be recognized (Acharya and Taylor, 2003) and identi-
quently applied. Dental age estimation gained prominence fication is the establishment of a person’s individuality.
following a case in Britain in 1935, where the age of two Accurate identification of the dead is required both for
badly mutilated bodies was estimated from the calcifica- legal and humanitarian reasons (Brown, 1984). It enables
tion of third molars (Taylor, 1963). This contributed to the the settlement of insurance and property, permits remar-
eventual identification of the bodies using photographic riage of the surviving spouse and facilitate last rites of the
superimposition. In an age when heightened social, emo- body in accordance with appropriate religious customs.
tional and legal importance is placed on identifying the Haglund and Morton (1994) believe that a majority of
dead, dental identification methods has great relevance. individual identifications in forensic settings are non-
Apart from identification, forensic odontology is applied problematic since most individuals die in the company of
in the investigation of crimes where traces of the teeth, such family and friends. These deaths may occur at home or in
as bite marks, are observed. The ability to match a bite mark a hospital, allowing visual identification. However, in cases
to the uncommon arrangement of the dental arch can prove where the body is burned, traumatized or decomposed,
important in investigating sex crimes and violent fights. visual identification can prove unreliable. Hence, Sopher
It is interesting to note that as early as the 6th century AD, (1972) believes that visual recognition and use of personal
the Indian sage Vatsy āyana had recognized the prevalence effects are the least dependable methods of identification
of bites during love-making. Consequently, he devoted an in such circumstances. Therefore, the safest option is for
654
Chapter 24 – Clinical and Radiological Perspective
the forensic expert to analyze physical features present in Dental Identification Procedure
the body.
Having ascertained the value of teeth in identification and
the principle for the same, one can proceed with the pro-
Importance of Teeth in Postmortem Identification cedure. Dental identification may be required in establishing
the identity of a single person, a small group of individu-
Every individual has a variety of physical features that als or in large fatalities such as accidental or natural disas-
include built, height, epidermal ridges and dental characters. The method for the different scenarios is essentially
teristics. Most of these, however, are prone to change over the same and is addressed together in the following columns
an individual’s lifetime. Epidermal ridges (which produce although, considering the magnitude of the latter, the
fingerprints) are exceptions but, like other soft tissues, are approach may vary. The identification procedure includes
susceptible to postmortem decomposition. Teeth are con- a number of steps, namely:
sidered as the hard tissue analog to fingerprints and gain
importance in identification as the time period since death 1. On-site evidence recovery
increases. Although teeth are susceptible to disease during 2. Postmortem dental examination and radiography
life, they are the most durable part of the body after death. 3. Obtaining dental records
Apart from teeth being relatively resistant to postmortem 4. Comparing post- and antemortem data
change, practically all materials used by the dentist are also 5. Preparing the identification report and conclusion
resistant to postmortem destruction. Therefore, the use of
dental evidence is considered essential for establishing iden- On-site evidence recovery
tity in traumatized, decomposed and incinerated remains. To begin with, it is essential to collect all dental evidence
from the dead body (Haglund and Morton, 1994). This may
require the dentist’s presence at the site where the dead body
Principle of Dental Identification is recovered (Brown, 1984). Dental evidence present at the
site where the body was found may be overlooked and not
The underlying principle of dental identification is that
recovered—decomposition and skeletonization often leads
combinations of dental characteristics are never the same
to loosening of teeth and the anterior teeth, which have con-
in any two individuals. Several authors believe that the
ical roots, may get dislodged. Oliveira and associates (2000)
human dentition is unique (Johanson and Lindenstam,
and Ðuric and coworkers (2004) have reported that inci-
1961; Keiser-Nielsen, 1977; Fellingham et al, 1984; Phillips
sors, especially maxillary, are most prone to getting lost post-
and Scheepers, 1990). Human beings normally have 32
mortem. Incinerated bodies have fragile bones and teeth,
permanent teeth which vary in morphology and arrange-
which can easily be mishandled by an untrained individual;
ment. Although teeth are relatively resistant to environmen-
trauma may result in scattering of dental evidence around
tal factors and postmortem proteolysis, during life they are
a large area. Acharya and Taylor (2003) have found that the
susceptible to disease such as caries. As a result, teeth may
inability to recover complete and intact postmortem den-
have undergone treatment in the form of fillings, crowns,
tal data can undermine successful identification. Therefore,
etc. Those teeth that cannot be restored may have been
to maximize success, one must try to recover all dental
extracted and, thus, missing from the oral cavity. According
evidence that may be present at the site. Hence, care must
to Keiser-Nielsen (1977), the number of combinations 16
be taken to document and photograph the scene at which
missing teeth can produce is approximately 60 crore.
the body is recovered; a forensic odontologist should be
Sixteen filled teeth produce a similar combination. Four
present to ensure proper search and recovery of all material
missing and four filled teeth combined can produce about
of dental significance needed for identification.
70 crore combinations. Every tooth has five surfaces and
taking the 160 tooth surfaces individually would produce
Postmortem dental examination and radiography
astronomical variations. In addition to the features visible
on oral examination, radiographs reveal distinct shape and The postmortem dental examination is usually conducted in
outline of various restorations of the crown and root and a mortuary. Case numbers allotted to the body should be
anatomic features of the teeth and bone (Sopher, 1972). verified and entered in the modified Interpol postmortem den-
Hence, the permutations resulting from observing the teeth tal odontograph, also referred to as the ‘pink form’ (Figure 1)
clinically and radiographically would be phenomenal. It before the commencement of examination. A visual appraisal
has been calculated that there are 1.8 1019 possible combi- of the body may enable preliminary assessment of the eth-
nations of 32 teeth being intact, decayed, missing or filled nicity, gender and approximate age of the victim. The
(Fellingham et al, 1984). Therefore, dental identity can be body bag used to transport the deceased should be thor-
considered as the sum total of all characteristics of teeth oughly searched for dislodged teeth or dental appliances.
and associated structures which, while not individually Postmortem dental examination is similar to routine
unique, equal a unique totality (Acharya and Taylor, 2003). examination of the oral cavity. All oral and dental features,
655
Figure 1
Modified Interpol postmortem form
including intact and missing teeth, caries and other pathol- The dental examination must be complemented with post-
ogy, restorations, attrited and rotated teeth should be care- mortem dental radiography as this will reveal what cannot
fully charted on the postmortem form. This will be the be observed clinically. Radiographic films may be stabilized
basis for later comparison and verification. Brown (1984) in the mouth using readily available materials such as gauze
suggests that postmortem examination should be under- or cotton rolls. Whenever possible, postmortem radiographs
taken by dentists working in pairs—one examining and should be taken to replicate the type and angle of the ante-
the other recording. The roles are then reversed and the mortem radiographs (Goldstein et al, 1998). According to
procedure repeated ‘as an added check’. Any dental these authors, if the difference in horizontal angulation
evidence missing should be reported to the forensic between the ante- and postmortem radiographs is more than
pathologist or accompanying law enforcement personnel. 10, comparison will not facilitate identification. However,
Subsequent dental evidence recovered must be labeled changes in the vertical angulation or the focal point-film
properly. distance do not affect the outcome of comparison. Pretty and
656
Sweet (2001) have suggested that ante- and postmortem Therefore, the authors examined whether radiographs with
radiographs could be marked using a rubber-dam punch to no restorations could be used in postmortem identifica-
differentiate the two—‘one hole for antemortem films and tion. Their results indicated that dental anatomy observ-
two holes for postmortem films’. The exposure time may able on the radiographs, especially ‘root morphology and
need to be increased or decreased respectively in bloated alignment’, facilitated correct identification in more than
bodies recovered following drowning and in skeletal remains. 85% of cases.
Obtaining dental records Identification report and conclusion
Dental records contain information on treatment and den- Subject to the availability of adequate postmortem data
tal status of a person during life. These antemortem records and quality antemortem records, Haglund and Morton
can be obtained from the local dentist, specialist or hospi- (1994) have stated that ‘the comparison and eventual
tal records. Whenever possible, the original records should dental identification are straightforward and routine’.
be examined. Records include hand-written dental charts, These authors believe that the quality and quantity of
radiographs, study casts and photographs. An individual information required for establishing positive dental
may have visited more than one dentist for therapy and identification has not been established. In fingerprinting,
multiple dental records may exist. Relevant information differences in the ante- and postmortem data rule out a
from the dental records should be transcribed onto the positive match. This concept, however, does not apply to
modified Interpol antemortem odontograph (the ‘yellow dental identification as long as the inconsistencies are
form’) (Figure 2). explainable. For example, the postmortem data may
Contribution of dental records in successful identification reveal a restoration on a tooth but the dental records indi-
depends on the availability of original records, their com- cate that the same tooth is intact. This difference, how-
pleteness and quality (Acharya and Taylor, 2003). By main- ever, can be explained since the restoration may have
taining good dental records, dentists reflect their awareness been placed on a date after the last available dental
and responsibility to forensic identification (note: quality record. On the other hand, if the postmortem data shows
dental records are also important as a counter to malprac- an intact tooth but the same tooth is indicated as being
tice lawsuits). It is also important that these records are restored in the dental record, this would probably suggest
relatively recent—records made for a 6-year-old child may a mismatch. Having compared the ante- and postmortem
not be useful 20 years later in adulthood. data, one should address whether the similarities are sig-
nificant and the differences can be explained. Based on
this, a range of conclusions can be reached which have
Comparing the dental data been modified below from McKenna (1986), Silverstein
The comparison of known antemortem data with the (1995) and Acharya and Taylor (2003).
obtained postmortem data is called comparative identifica-
Positive identification This indicates that the ante- and
tion. Sholl and Moody (2001) have stated that ‘the basic
postmortem data match each other with no unexplainable
principle of any method of identification is to determine
differences. The identity is proven ‘beyond reasonable doubt’
that individual characteristics observed postmortem are con-
and should include radiographic support.
gruent with those known to have been present in life’. Once
the postmortem findings and antemortem records are avail- Probable identification The ante- and postmortem data
able, the data can be compared. Comparison should be have a high level of concordance but a lack of quality
qualitative rather than quantitative in nature; it must also be information precludes positive identification. Explainable
systematic and methodical. Features compared include den- differences may exist between the two sets of data and
tal restorations, pathology, crown, root and pulp anatomy, radiographic support is, usually, not available.
associated bony structures, artifacts and dental anomalies.
An individual with multiple dental treatment and uncommon Possible identification The ante- and postmortem data
dental features has a better probability of being identified are in agreement but the available information is insuffi-
than someone with no extraordinary dental characteristics. cient both in terms of quantity and quality. The available
information neither permits a definitive identification nor
Radiographic morphology in dental identification enables the identity to be excluded.
Presence of restorations are an important component of
Insufficient evidence The available ante- and/or postmor-
comparative dental identification. Sholl and Moody (2001)
tem information are insufficient to form the basis for a
have suggested that the incidence of restorative work
conclusion.
among younger age groups in Europe and North America
appears to be declining, probably a long-term benefit of Excludes identity The ante- and postmortem data are
fluoridation and improved oral hygiene. Hence, there may clearly inconsistent; the data contain unexplainable dif-
be the necessity to rely on other features of the dentition. ferences that comprehensively indicate a mismatch.
657
Figure 2
Modified Interpol antemortem form
It is necessary for the forensic odontologist to be abso-

presents certain advantages to forensic investigation. Since
lutely certain for an identification to be ‘positive’. One also
the images can be viewed immediately after exposure, the
needs to remember that any attempt at establishing identity
operator has the choice to either accept or reject the image
is addressed to the legal authorities. Hence, the report and
depending on its quality. Further, postmortem images may
conclusion should be as definitive and clear as possible.
be retaken to replicate the antemortem radiographic posi-
Digital radiography in dental identification Traditional tion and angulation. This could be particularly relevant
methods of radiography involve the use of film. The advent in postmortem settings where access to radiographic pro-
of digital radiography over the past decade, which replaces cessing may be unavailable or the ability to retake the
the radiographic film with an image capturing sensor, radiographs of the decedent at a future date is unlikely.
658
Hanaoka and colleagues (2001) have successfully used the dental examination. Their method prescribes an intraoral
technology in postmortem dental identification and pre- incision to expose the coronoid process followed by the
dicted its routine application in the future. They have stated insertion of a curved Cooper type scissors through
that receiving, sending and storing digital radiographs the incision to dissect temporalis fibers that are inserted to
through computer networks is effortless. According to the top of the coronoid process (Figure 3A, B). The deep
Hubar and Carr (1999) and Du Chesne and associates (1999), fibers, which may also insert to the mandibular notch, are
the radiographic image may be enlarged, rotated and its drawn forward with a channel retractor for dissection
brightness and contrast modified. Several images may be (Figure 3C, D). The myotomy is carried out on both the left
viewed together which allow convenient comparison of and right sides.
ante- and postmortem images. ‘Subtle features difficult to
visualize on conventional radiographs such as trabecular
bone patterns are seen with greater detail digitally and Incineration
may further help to corroborate identification of a dece-
Due to the fragility of dental tissues consequent to fire, it
dent’ (Hubar and Carr, 1999). However, manipulations
is essential to maintain their integrity during examination
which distort the structures visible on the radiograph by
of charred human remains. According to Delattre (2000),
changing their angular relationship are not admissible by
‘charred dental remains are by nature rather delicate’ and
the courts of law (Du Chesne et al, 1999). Hence, these
can crumble if not handled cautiously. The color of the teeth,
authors have stated that it must not be the objective of
apparently, gives an indication of its relative fragility—
postmortem radiology to manipulate the images ‘to such an
blackened teeth are less fragile than those that are ashen
extent that optimal or even misleading results are presented’.
gray (the appearance of incinerated teeth is described later
While it is reasonable to expect forensic experts to avoid
in this chapter). The lips and cheek may contract in pro-
such alterations on postmortem radiographs, one cannot
longed or intense fire and withdraw exposing the anterior
be so sure about antemortem digital images. Therefore, the
teeth. As a result, these teeth are the most exposed to fire
conclusions based on comparison of post- and antemortem
and the enamel and dentin on their anterior surface can
digital radiographs may be brought into question by the
simply crack off when touched. To prevent the loss of tooth
courts of law, which will probably give greater weight to
structure, Mincer and associates (1990) have listed a vari-
documentary evidence such as conventional radiographic
ety of materials that can stabilize incinerated teeth. They
films than electronic data files.
endorse clear acrylic spray paint and cyanoacrylate glue
as the best agents since these are readily available, econom-
ical, portable, permeate the teeth well and set fast.
CHALLENGES IN POSTMORTEM However, in their study, not all specialists surveyed con-
EXAMINATION sidered reinforcing incinerated teeth essential. Many felt
problems could be circumvented by very careful handling
Examining dental remains may be challenging in cases of of the teeth or photographing the teeth prior to handling.
rigor, incineration and other circumstances. Described Intraoral radiography without jaw opening can be accom-
below are some techniques to overcome difficulties, as well plished by removing the tongue through the floor of the
as precautions to be taken while using them. mouth and inserting the films through the opening. This
has been referred to as the ‘tunneling technique’ by
Griffiths and Bellamy (1993), who have also suggested the
Rigor Mortis use of material such as bubble plastic or a sheet of foam to
protect the fragile skeletal and dental remains during
Limited opening of the mouth due to severe postmortem
transportation.
rigor will affect dental examination since access to the
oral cavity is limited and viewing the dentition is impaired.
In such cases, where the muscles are extremely rigid, the
Jaw Resection
mouth will have to be forced open. The use of trismus
screws (made from acrylic) for obtaining initial opening in Depending on the circumstances of death and condition of
the anterior dentition followed by Fergusson or Heister the body, the forensic dentist can decide to cut open the soft
mouth gags to increase jaw separation at the posterior tissue of the cheek to reach the teeth, or remove the jaws
region has proven useful in the author’s experience. entirely. Resected jaws are easier to examine and radiograph.
However, one must be careful not to fracture the teeth. However, such ‘invasive’ procedures can pose obstacles,
Nakayama and coworkers (2001) have advocated an intra- especially when the body needs to be viewed by relatives.
oral approach for myotomy of the temporalis muscle. They Furthermore ethical and legal hurdles may be faced.
contend that mouth opening demands relaxation of the Therefore, one must consider jaw resection as a last option
temporalis and, hence, its myotomy will facilitate easy and obtain prior permission from the forensic pathologist.
659
Figure 3
A B
C D
(A, B) Intraoral approach for myotomy: when the temporalis is attached to the top of the coronoid process, myotomy is
accomplished with scissors only; (C, D) in case the temporalis is attached both to the coronoid and mandibular notch,
a channel retractor is inserted to draw forward the deep end of the muscle to facilitate dissection. (Reprinted from Nakayama Y,
Aoki Y, Niitsu H, et al (2001), ‘Forced oral opening for cadavers with rigor mortis: two approaches for the myotomy on
the temporal muscles’, Forensic Science International, Vol. 118, pp. 37–42, with permission from Elsevier)
Tooth Colored Restorations Incinerated Teeth

Tooth colored restorations such as composites and glass The identification and recovery of burnt teeth subsequent to
ionomers may be missed during examination. These can be accidents, arson or terrorist activities—where human remains
disclosed using dyes such as mercurochrome or tinted ben- may only be recovered in parts—can assist in establishing the
zalkonium chloride, which are applied on the teeth with origin of the remains and help reconstruct the scene.
cotton swabs. However, using dyes may leave the oral cav-
ity stained and messy (Clark and Meeks, 1989). Therefore,
Crown
Carson and colleagues (1997) have suggested the use of com-
mercially available alternative light sources. They discovered Muller and associates (1998) have undertaken one of the
that tooth colored restorations are best revealed at wave- few detailed works on changes that teeth undergo when
lengths of 415–530 nm. This range of wavelength also exposed to high temperatures. They observed that enamel
revealed restorations when the teeth were burnt, although shows crazing (fine surface cracks) at 150C but retained
standard UV light (350 nm) may have more practical value its normal color although at 200C, enamel loses its gloss.
in fire-damaged bodies. Further increase in temperature causes more crazing—
initially over the neck, then more coronally—followed by
a few frank cracks.
POSTMORTEM ALTERATIONS TO THE TEETH Initial separation of enamel from dentin occurs at 400C
AND ORAL TISSUES and the enamel shell separates totally at 450C. At 500C,
Harsányi (1975) has observed deep longitudinal furrows on
While teeth are known for their relative postmortem stabil- the crown which almost divide it into several pieces. At this
ity, their appearance may be altered, and this can provide temperature, the pulp chamber and root canal are preserved
clues about the circumstances of death. and not narrowed. Above 500C, the crown undergoes
660
fragmentation and consistently becomes smaller with fur- resulting from the dry distillation of organic matter, can
ther rise in temperature. The narrowed cavities of the pulp lead to observational errors’.
chamber and root canal are recognizable even at 1,100C.
The coronal dentin is exposed at 450C (following total Root
separation of the enamel shell). Cracks appear at 600C with
The root shows a light yellow color at 150C and exhibits
disintegration from 800C onward. While enamel is con-
a calcinated appearance at 300–400C when exposed directly
verted to powder, dentin is not. This has been attributed to
without protection of the alveolar bone. Further rise in
hypermineralized pretubular zone of the dentin. Myers
temperature displays crazing of the coronal one-third of
and colleagues (1999) believe that the mineral content of
the root. This progresses to the apical part at a temperature
dentin, located within its organic matrix composed of type I
beyond 800C and the root begins to fragment at 900C.
collagen, helps stabilize the collagen against thermal dena-
turation and shrinkage. The color changes in the crown Effects of temperature on restorative materials Like
and root at different temperatures has been demonstrated teeth, restorative materials used in treatment are equally
by Muller and associates (1998) (Table 1). However, these resistant to the effects of high temperature. According to
authors have cautioned that in real-life scenarios, it may be Rossouw and associates (1999), composites, compomers
much more difficult to observe the color changes since teeth and glass ionomers can withstand 250–500C for a short
found at the site of fire need to be carefully cleaned. They time but, beyond 5 minutes their compressive strength is
added that ‘the presence of a superficial metallic layer, reduced to almost zero. Hence, they need to be handled
very carefully. Compomers (14.3%) and glass ionomers
Table 1 Color changes in burned teeth (18.6%) have higher volume shrinkage when compared to
composites (6–10%). This means that the former restor-
Temperature (C) Appearance ative materials may get dislodged, especially from the
150 Crown Normal color and shiny appearance buccal and lingual preparations. Most tooth colored
Root Off-white to light yellow restorative materials turn grayish-black at the aforemen-
tioned temperatures. Their change in color means that it is
200 Crown Dull, light brown surface
difficult to spot them visually. However, the radiodensity
300 Crown Light brown to grayish with dark brown
is not altered and, therefore, they can be viewed on radio-
spots
graphs. Metallic restorative materials melt and distort at
Root Black different temperatures: gold alloys melt at 915–1,090C;
400 Crown Dark brown/gray silver amalgam’s range depends on the mercury content
Root Shiny black (some amalgams may resist up to 870C). Porcelain den-
500 Crown Enamel is dark brown or gray; dentin is ture teeth have been cited as resisting temperatures as
gray high as 900C (Taylor et al, 2002); however, acrylic den-
Root Gray/brown ture material depolymerizes to monomer at 450C.
600 Crown Dark gray enamel and blue-tinted gray
dentin Postmortem Pink Teeth
Root Blue-tinted gray with white spots
Pink colored teeth are encountered on occasions during
700 Crown Dark gray enamel and blue-tinted white postmortem dental examinations. This intrinsic pigmenta-
dentin; the cusp tips are blue
tion is believed to be associated with victims of sudden
Root Blue-tinted white or white only death (Padayachee, 1988). Van Wyk (1989) cites a number
800 Crown Dark gray enamel with white spots; of studies which suggest that the phenomenon requires
white dentin increased blood supply to the pulp. This may be the result
Root White with gray spots of raised venous pressure caused by an event such as
900 Crown White dentin strangulation (Whittaker et al, 1976) although van Wyk
Root White with dark gray or black spots at CEJ
(1988) could not correlate the cause of death or the time
since death to the presence of pink teeth. Padayachee
1,000 Crown White dentin
(1988) has stated that pink teeth show ‘conspicuous bleed-
Root White with pink tinge at apex ing in the dental pulp and the resultant pigmentation is
1,100 Crown Chalky white thought to be due to an increased pressure in the pulpal
Root Chalky white circulation’. The phenomenon of postmortem pink teeth is
1,150 Crown Chalky white believed to be analogous to postmortem lividity produced
Root Chalky white
by the flow of blood into a dependent part of the body
after death (Whittaker et al, 1976; van Wyk, 1987).
Source: Modified from Muller and associates (1998).
According to van Wyk (1988), the blood vessels in the
661
pulp become distended due to pooling of blood. The red smokers, a typical elliptical type of attrition of the anterior
cells undergo hemolysis followed by autolysis of the blood teeth corresponding to the pipe stem is visible. In musi-
vessel walls and release of pigment in solution which dif- cians also, specific form of attrition on the anterior teeth
fuses into the dentinal tubules. The degree of dentinal caused by a musical instrument’s mouthpiece can be seen.
staining varies and van Wyk (1988) concludes that teeth Padayachee (1988) has highlighted certain forms of occu-
appear pink clinically only when the dentin is stained pational staining, e.g. extrinsic staining in industrial
extensively. The pigment responsible for the pink discolor- workers has been attributed to exposure to metals and
ation is undegraded hemoglobin (van Wyk, 1989). their salts. He also cited black/gray pigmentation of teeth
in anemics taking liquid iron supplements.
Tongue Hemorrhage Sociocultural practices such as betel nut chewing prev-
alent in India also result in characteristic brown deposits
Hemorrhage of the tongue can be observed associated with on the teeth (Padayachee, 1988). On the other hand, van
strangulation deaths. Bockholdt and Maxeiner (2002) con- Wyk (1976) draws attention to an unusual sociocultural
sider tongue hemorrhage to be the result of either cranial custom among a particular group of people from South
congestion, compression by the dental arches or the hyoid Africa—the deliberate extraction of the maxillary incisors,
bone pressing onto the base of the tongue following neck especially in females, to facilitate oral sex.
compression. These authors have reported tongue hemor- Probably more unusual is a purported case of self-
rhage in 71% of homicidal strangulations, 19% of which were extraction of the teeth among drug abusers reported by
the result of biting caused by compression of the tongue Pretty and Hall (2004). These authors have suggested that
between the teeth. However, tongue injury was observed ‘dentists should consider self-injury as an explanation for
in only 5% of suicidal strangulations. A protruded tongue, intra- and peri-oral injuries of unknown origin’. They add
compressed between the jaws, is observed in a significant that the presence of extraction sockets due to self-mutilation
number of suicidal hangings although the number of grossly could be a finding in mentally imbalanced individuals or
visible tongue bites with hemorrhages is small; a similar among those with an altered state of mind resulting from
small number of internal hemorrhages ‘restricted to the tip drug abuse. Bécart and coworkers (1997) have reported
or anterior part of the tongue (development during a pro- that heroin users have a greater incidence of decayed
trusion of the tongue in the course of the hanging agony)’ teeth, especially atypical carious lesions on the labial/buc-
are evident. However, extensive internal hemorrhages in cal surfaces. These lesions may appear larger, darker and
suicidal strangulations should be treated with some degree of less painful than usual cervical caries, convincing some
caution—Bockholdt and Maxeiner (2002) have cited reports authors to consider them as a pathognomonic sign of drug
of internal tongue hemorrhage in victims who were first addicts (Lowenthal, 1967; Colon, 1972). Pretty and Sweet
strangulated and then hung by the killer (‘simulated sui- (2001) cited a number of studies that also indicate the
cide’), i.e. extensive internal tongue hemorrhage could indi- presence of dental erosions in drug abusers and alcoholics.
cate a presumed sucide as being a homicide. But they may Cattaneo and coworkers (2003) have detected morphine
also be the result of highly atypical suicidal strangulation. and codeine in the teeth of skeletal remains found 2 years
after death. They believes that such findings suggest—but
do not prove—a drug related death. This not only throws
SOCIAL PROFILING light on the social antecedents of the unidentified indi-
vidual but also gives clues to the circumstances surrounding
According to van Wyk (1976), information about habits and the death (e.g. drug overdose).
occupational antecedents can be obtained from the dentition.
This can provide valuable information on the person’s social
background and prove useful in identification. Based on IDENTIFYING THE EDENTULOUS
personal observation and epidemiological studies, van Wyk
(1976) categorized such features as: (i) lesions of the teeth Borrman and coworkers (1999) have cited studies that project
only, (ii) lesions of the teeth and soft tissues, and (iii) lesions an increase in the percentage of elderly around the world in
of the soft tissues only. This section, however, will empha- the coming decades. While the percentage of edentulousness
size on the lesions of the teeth, since they are more likely among this group may decrease, the number of edentate indi-
to survive postmortem vagaries than soft tissues. viduals may actually remain the same or even increase. From
According to Gustafson (1966), grooving of the upper a forensic odontology point of view, problems arise when
anterior teeth may be encountered in cobblers, carpenters attempting to identify edentulous cases. The absence of a
and electricians. Such a groove commonly results from the ‘dental combination’ may render conventional comparative
habit of opening hairpins with an upper central incisor— identification of little use. In fact, it is believed that most prob-
the constant abrasion of the metal pin against the tooth lems in postmortem identification are encountered in com-
edge eventually leaves a shallow groove. Among pipe plete denture cases (Gustafson, 1966). The denture, however,
662
can be a crucial piece of evidence in identifying the edentu- Denture Marking Systems
lous as they remain intact or only slightly damaged following
traumatic accidents. Dentures may have been fabricated to A widely recommended technique for identifying the eden-
replicate an individual’s dentition. For example, Taylor and tulous is to mark dentures with the individual’s name and/or
coworkers (2002) were able to positively identify an individ- a personal number. Markers such as printed onion skin paper
ual based on, among other evidences, distinct features of a and adhesive labels, electronically inscribed clear lami-
maxillary denture which contained a midline diastema and nated tape, photochemically etched ‘microchips’ (Figure 5)
gold inlays on two of the teeth (Figure 4). In addition, the type, or typewriter-punched stainless steel bands ‘can be placed
size and shape of teeth used in the denture can be used for either in the tissue surface during trial packing or in the
identification. This, however, warrants detailed dental records. polished surface after processing’ (Borrman et al, 1999). The
Therefore, use of denture-marking systems may be more prac- materials and the mode of imprint should be strong enough
tical; comparison of palatal rugae may also hold some use. and preferably resistant to high temperatures expected in
a fire. Moreover the marker should not compromise the den-
Figure 4 ture strength. However, these forms of markings—where
identity can be revealed easily and publicly—may raise pri-
vacy issues. Hence, Rötzscher and associates (1999) have
suggested ‘writing’ and ‘reading’ personal data on a micro-
chip by means of a handheld electronic scanner. This per-
sonal information pertaining to the individual cannot be
directly viewed but may be accessed using specific computer
hardware and software. Issues of privacy notwithstanding,
marking a denture enables a simple mean to identification
when other methods are unavailable. However, Borrman and
coworkers (1997) highlighted that the ‘denture per se does
not prove identity since it is not a fixed appliance but it is
a significant link in a chain of evidence of personal identity’.
Characteristic features in a maxillary denture. (Reprinted from Palatal Rugae in Identification

Taylor PTG, Wilson ME, Lyons TJ (2002), ‘Forensic odontology
lessons: multishooting incident at Port Arthur, Tasmania‘,
Since denture marking is still an uncommon practice,
Forensic Science International, Vol. 130, pp. 174–82, alternatives for identifying the edentulous need to be
with permission from Elsevier) explored. The use of palatal rugae pattern could prove use-
ful for this purpose. The rugae on the maxilla or maxillary
Figure 5
A B
Denture marking ‘microchip’ with inscription (A) and the ‘microchip’ incorporated in a maxillary denture (B).
(Reprinted from Rajan M, Julian R (2002), ‘A new method of marking dentures using microchips’, Journal of Forensic
Odonto-Stomatology, Vol. 20, No. 1, pp. 1–5, with permission from authors)
663
denture of the deceased can be compared to previous den- records or unidentified skeletal remains, craniofacial
tures recovered from the decedent’s home, or plaster mod- methods of identification may be applied. The utility of
els that may be available with the dentist or laboratory these methods is variable and may result in establishing
technician. Palatal rugae are well protected by the orofa- the identity of the deceased or providing broad clues.
cial tissues in fires and traumatic accidents. In a recent
pilot study, Muthu, Subramanian and colleagues (2005)
Cranial Suture Pattern
have found that rugae were unaltered in 93% of victims of
third-degree burns; they also discovered that the rugae The cranial sutures are seam-like joints of bone exclusive to
withstood decomposition and were recognizable in more the skull. As the cranial bones grow and meet, the margins
than 80% of the cases. Furthermore, rugae patterns are become highly complex and irregular. In a study of ectocra-
unique to an individual and can be used in identification— nial suture patterns on 320 skulls, Sekharan (1985) observed
Limson and Julian’s study (2004), which obtained high- that their configuration differed in every individual (Figure 7),
percentage accuracy in identifying individuals in a simu- including twins. He categorized the suture patterns into ser-
lated ante- and postmortem comparison of the palatal rated (saw-like junctions), denticulate (characterized by small
rugae, bears reasonable testimony to this premise. These tooth-like projections), squamous, limbous and plane types.
authors employed a software—RUG FP-ID Match—for the For example, the sagittal, lambdoid and coronal sutures on
comparison. First, rugae outlines on the casts were demar- the cranial vault can be divided into a number of subdivi-
cated using a graphite pencil and photographed. The sions and within these subdivisions one may appreciate ser-
image was transferred to a computer where the medial and rations and denticulations. Therefore, the patterns produced
lateral ends of the rugae were marked manually (Figure 6). by cranial sutures are highly variable and individualistic and
Based on the principle commonly employed in fingerprint may be used in identification. To accomplish this, however,
analysis, the software compared the marked points antemortem radiographs of the lateral and anteroposterior
between ante- and postmortem cast images to ascertain a view (preferably Townes’ view) should be available. Sekharan
match. (1985) observed that the suture patterns, which are visible in
the majority of such radiographs, can be compared with
sutures on forensic skull specimens in situ. Alternatively, he
CRANIOFACIAL IDENTIFICATION suggests taking postmortem radiographs and comparing the
suture pattern on these to those on the antemortem radio-
In cases where comparative dental identification is not graph. However, the cranial vault reaches adult dimen-
possible due to reasons such as unavailability of dental sions at about 7 years of age. Therefore, one needs to be
cautious while comparing postmortem radiographs of an
Figure 6 adult to those taken during childhood. Recently, Rogers
and Allard (2004) have pointed to the success of viewing
sutures on computerized tomography (CT) scans and rec-
ommend this technology over conventional radiography.
Frontal Sinus Configuration

The frontal sinuses are irregular shaped, pneumatic cavities
located along the inferior aspect of the frontal bone, deep
to the superciliary arches (Harris et al, 1987). Although absent
or insignificant at birth, it increases in size and complex-
ity with age and attains maximum size by about 20 years
(Schuller, 1943). In fact, Schuller (1943) is attributed having
stated that the radiographic appearance of these sinuses
show individual variation. In a pilot study, Harris and
associates (1987) have also observed that ‘no two frontal
sinuses were alike’ in terms of height, width and number
of edge loculations, but Schuller (1943) has warned that
Characteristic points are plotted manually on the rugae certain diseases and disorders may alter the size and shape
pattern image. (Reprinted from Limson KS, Julian R (2004), of the sinuses. For example, acromegaly renders the
‘Computerized recording of the palatal rugae pattern and sinuses extremely large while sinusitis can also cause some
an evaluation of its application in forensic identification‘, changes. Moreover, the sinus walls become thinner in old
Journal of Forensic Odonto-Stomatology, Vol. 22, No. 1,
age and look larger (Nambiar et al, 1999), although these
pp. 1–4, with permission from authors)
changes are minor (Kullman et al, 1990).
664
Figure 7
A B
C D
Cranial suture pattern of different skulls. (Reprinted from Sekharan PC (1985), ‘Identification of skulls from its suture pattern‘,
Forensic Science International, Vol. 27, No. 3, pp. 205–14, with permission from Elsevier)
The radiograph which best reveals frontal sinuses is the shape, symmetry and septa. However, these categories
anteroposterior view (especially occipitomental view), a may hold little practical value since investigators resort to
record which may be available on occasion. Depending on simple visual comparison of radiographs placed side-by-
the condition of the postmortem remains, radiographs from side or superimposed. Harris and associates (1987) have
‘several views with different angulations and densities’ may suggested tracing the sinus outlines visible on radiographs
need to be taken for proper comparison with antemortem onto orthodontic tracing paper for comparison although,
radiographs (Nambiar et al, 1999). Kullman and coworkers (1990) obtained near 100% accuracy
Schuller (1943) attempted to describe frontal sinus con- in a study that compared simulated ante- and postmortem
figuration and defined seven characteristics: radiographs per se. The latter also state that successful com-
parison is not influenced by the experience of the observer.
1. Septum and its deviation
The frontal sinuses have been used to establish identity
2. Upper border (scallop, arcades)
in numerous cases and it has seen an increase in its use
3. Partial septum
and acceptance among forensic anthropologists and radi-
4. Ethmoidal and supraorbital extensions
ologists (Christensen, 2004). However, successful identifi-
5. Height from planum
cation using frontal sinuses is contingent to standard
6. Total breadth
methods of comparison including uniform angulation and
7. Position of sinus midline.
orientation of radiographs taken ante- and postmortem
Yoshino and coworkers (1987) have provided a simpler and substantial empirical evidence pertaining to their
classification of the frontal sinuses based on the size, uniqueness.
665
Skull-Photograph Superimposition ❍ Camera-object distance: Differences in the focal lengths

of camera lens can alter the contour of high-curvature
Superimposing the facial photograph (of the suspected areas on the face
deceased) and skull was first successfully put to use in a ❍ Scaling factors: Appropriate magnification of the pho-
criminal case by Glaister and Brash (1937). They superim- tograph may be essential to obtain equivalence in size
posed antemortem photographic negatives of the heads of vis-à-vis skull specimen.
two homicide victims to the respective postmortem skull
radiographs and obtained positive identification. Since the
anterior dentition may be visible on many personal photo- Figure 8
graphs, the use of tooth size, shape, angulations may be of
particular relevance in superimposition. However, adjust-
ments need to be made to orient the ante- and postmortem
images. It is advisable to use superimposition as a corrobo-
rative tool, rather than the sole method, for identification.
Actually, it may have more value in exclusion rather than
identification per se. Nevertheless, it gains prominence in
the Indian context since research and case work have been
reported on a regular basis. The infrequent availability of
quality dental records has probably encouraged its use in
forensic identification. It includes photographic superimpo-
sition, video superimposition and newer digital techniques.
Photographic superimposition
The conventional superimposition method, photographic
superimposition utilizes antemortem photographs, radio-
graphs or portraits (Nickerson et al, 1991) for comparison Superimposition of the putative deceased’s photograph and
with the skull specimen at hand. The skull is photographed skull. (Reprinted from Bilge Y, Kedici PS, Alakoç YD, et al
and its tracing laid over that of the antemortem photograph. (2003), ‘The identification of a dismembered human body:
The superimposed images are carefully studied to determine a multidisciplinary approach‘. Forensic Science International,
Vol. 137, No. 2–3, pp. 141–6, with permission from Elsevier)
how closely the skull and face fit each other. The general size
and shape of the skull and bony landmarks such as the orbits,
nasal cavity, dentition, etc. are used to indicate the proximity
of the fit. The facial soft tissue thickness should also be con- Figure 9
sidered during superimposition. Recently, Bilge and coworkers
A B
(2003) have reported a case where identification was estab-
lished by superimposing a photograph of an unidentified skull
with the antemortem photograph (Figure 8) using commer-
cially available software such as Adobe Photoshop. They
state that ‘the photo-to-photo superimposition technique
is quite a supportive system for positive identification, espe-
cially when the questioned person displays uncommon fea-
tures, and good quality photographs are achieved’.
As an aid to enhance the success of photographic
superimposition, Jayaprakash and colleagues (2001) have
suggested that the skull surface details be studied carefully
and correlated to that of the face photograph before draw-
ing a conclusion. The similarity between the dry skulls and
photographs with respect to, for example, the frontal emi-
nence or nasal bone morphology (Figures 9 and 10) can (A) Broad and raised nasal ridge and nasomaxillary
be correlated adding due weight to the superimposition. notches in a male skull and (B) the nasal morphology in
Nevertheless, a number of practical problems may under- the corresponding face photograph. (Reprinted from
mine the outcome of photographic superimposition: Jayaprakash PT, Srinivasan GJ, Amravaneswaran MG (2001),
‘Cranio-facial morphoanalysis: a new method for enhancing
❍ Angular positioning: The skull and the facial image reliability while identifying skulls by photo superimposition‘,
Forensic Science International, Vol. 117, pp. 121–43, with
should have reasonably equal horizontal and vertical
permission from Elsevier)
angulations
666
Figure 10 image and anatomical landmarks such as the glabella,

nasion, ectocanthion and maxillary dentition compared to
A B determine the fit of the images. The authors claim ‘gains in
terms of image restoration and enhancement and near-
optimal superimposition’ as potential advantages of this
technique.
Challenges in Superimposition
One of the challenges to proper superimposition is to obtain
life-sized enlargement of the photograph to the correspond-
ing skull. The presence of an object of known size on the
photograph is helpful in reaching this end. However, in its
absence, a number of reference points have been suggested.
Sekharan (1971) has recommended measuring the distance
between various anatomical landmarks (gonion, nasion,
(A) Narrow, prominent and wavy nasal ridge, conspicuous
nasomaxillary notches and asymmetric bulge in the lateral menton, etc.) independently on the skull and photograph.
aspects of the nasal ridge in a male skull and (B) the nasal Their ratio are calculated and compared between the skull
morphology in the corresponding face photograph. (Reprinted and photograph for concordance. However, the use of teeth
from Jayaprakash PT, Srinivasan GJ, Amravaneswaran MG to achieve life-sized enlargement may be more reliable.
(2001), ‘Cranio-facial morphoanalysis: a new method for Teeth are the only craniofacial components visible clini-
enhancing reliability while identifying skulls by photo cally during life and may be evident on antemortem pho-
superimposition‘, Forensic Science International, Vol. 117, tographs where the subject is smiling. Hence, dental features
pp. 121–43, with permission from Elsevier) on the photograph, such as the outlines of teeth or inter-
canine width, can be compared to the same on the skull to
obtain a 1:1 magnification (McKenna et al, 1984).
Video superimposition
The advent of videography led to improvements in con-
ventional still photography superimposition. Video super-
FACIAL APPROXIMATION
imposition usually makes use of two video cameras, one
focussing on the skull and another on the antemortem When virtually no information is available on the identity
photograph. The resulting images are ‘mixed into one of skeletal remains, an attempt at ‘reconstructing’ the face
composite image’ (Nickerson et al, 1991) using image mix- from the skull may be considered. Since such a reconstruc-
ing devices. The advantage of video superimposition over tion usually results in an approximate reproduction of the
photographic superimposition is that the skull can be target face, the term ‘facial approximation’ is believed to be
placed on a flexible mounting device and its angular posi- more appropriate. Facial approximation is used as a tech-
tion and distance from the camera adjusted according to nique of last resort for identifying individuals in forensic
the position of face on the photograph. In addition, image cases. It can involve 2D sketches using the artist’s perception
mixers enable added advantage of fade-in and fade-out, or, as described in the next section, 3D methods which use
and vertical and horizontal ‘sweep’ mechanisms. This clay modeling techniques and computer-generated images.
allows different images to be progressively superimposed
upon each other.
Clay Modeling
Three-Dimensional Digital Superimposition According to Phillips and coworkers (1996), facial approx-
imation is a combination of art and science in which a 3D
The use of three-dimensional (3D) superimposition imag- representation of the facial features is produced using the
ing techniques has been suggested by Nickerson and skull as foundation. These authors have described the fol-
coworkers (1991). The antemortem photograph is digitized lowing steps to manually approximate a face: first, one
to produce a two-dimensional (2D) image whereas the needs to make an assessment of the ethnicity, sex and age
skull is laser-scanned to produce a 3D image. The 2D dig- using the skull bones and teeth. This information can add
itized facial image is mapped onto a polygon—a process value to, and assist in, the approximation. The mandible is
known as texture mapping. The texture-mapped image articulated with the skull and jaws and the soft tissue pro-
can be manipulated as easily as the 3D skull model. This file is drawn around the underlying bone using average
texture-mapped image is then projected onto the 3D skull facial tissue thickness; the nasal profile can be constructed
667
at this stage using a cephalograph. The skull cavities (orbits, the landmarks of the face lie on top of those of the skull;
nasal fossa, etc.) are blocked with modeling wax and an soft tissue thickness is selected to give the face a thin,
impression of the entire skull is made using rubber-base medium or fat appearance. The computer is now ready
impression material. The cast is poured in plaster and refer- to fit the face over the skull which is achieved using a
ence points marked on it; shallow holes are then drilled at 3D transformation termed ‘warp’, resulting in an approxi-
these points. Thin dowel sticks are placed in the holes to mated face (Figure 13). A limitation of this method is that
indicate the thickness of facial tissues at these points. The it relies on the operator’s ability to select the ‘average’
muscles of facial expression and mastication are repro- facial template—a procedure that may be inaccurate and
duced on the plaster cast using modeling clay. Once the prone to bias. To overcome this, Claes and associates
basic face is sculpted, finishing touches such as wrinkles, (2006) have recently developed a facial template that is
hair pattern, eyes, etc. are added. The authors claim that based on the features of 118 individuals. This would be a
artistic creativity developed during the method may result more realistic average, although the database could be
in a more life-like reproduction of the face. However, the further expanded. Certain advantages of the computer-
method has a variable success rate, is subjective and, at based approximation over clay modeling include:
best, merely replicates ‘certain facial features which may
❍ A plaster cast of the skull need not be prepared.
be characteristic’ (Phillips et al, 1996).
Therefore, it circumvents potential damage to the skull
arising from impression making (Vanezis et al, 2000).
Computer-assisted Approximation ❍ Faster, easier and more efficient generation of approx-
imations (Vandermeulen et al, 2006).
Vanezis and associates (2000) have developed a computer-
❍ Superimposition of the skull and approximated face
assisted 3D facial approximation technique. To begin with,
allows the operator to assess soft tissue to skull alignment
the defects and natural orifices of the skull are covered
and check for any obvious errors (Vanezis et al, 2000).
with cotton and the skull placed on a turn-table. The table
❍ Multiple approximations, that can take into account
is rotated 360 and scanned under a low-intensity laser
differences in facial appearance due to subject-specific
beam. This results in the production of 200–250 skull pro-
attributes such as race, gender, age and body mass
files which are fed to a computer system to enable viewing
index, is possible (Claes et al, 2006; Vandermeulen
of a 3D skull image (viewing is achieved by means of a
et al, 2006).
‘Facial Reconstruction’ software). The same software is then
used to mark landmarks on the skull image at which the Facial approximation is by no means a definitive form of
facial depth has been previously determined (Figure 11). identification, rather a means to it. It can be difficult to
From a database of facial templates, a face is chosen obtain facial models which reflect the exact likeness of a
which has average features and matches the skull anthro- missing person. However, an image approximated may be
pologically; landmarks corresponding in location to those published or broadcast in the media to revive the public’s
of the skull are placed on the face (Figure 12). The facial memory about a victim and obtain further leads. As
template is then carefully placed on the skull image so that Vanezis and associates (2000) point out, the purpose of
Figure 11
A B C
Views of digitized image of the skull with landmarks in position. (Reprinted from Vanezis P, Vanezis M,
McCombe G, et al (2000), ‘Facial reconstruction using 3D computer graphics‘, Forensic Science International,
Vol. 108, pp. 81–95, with permission from Elsevier)
668
Figure 12
A B C
Anthropologically matching facial template with landmarks in position. (Reprinted from Vanezis P, Vanezis M,
McCombe G, et al (2000), ‘Facial reconstruction using 3D computer graphics‘, Forensic Science International,
Figure 13
A B C
The facial template has been ‘warped’ on the skull-image to produce an approximated face. (Reprinted from Vanezis P,
Vanezis M, McCombe G, et al (2000), ‘Facial reconstruction using 3D computer graphics‘, Forensic Science International,
facial approximation is ‘to trigger the recognition process histologic age has been presented elsewhere (Acharya and
to see whether a name can be put to a face. Once this is Sivapathasundharam, 2006). In this chapter, a few clinical
achieved, one can proceed to a more definitive identifica- and radiographic methods for dental age estimation are
tion using comparative antemortem data’. described.
Clinical Methods of Age Assessment

AGE ESTIMATION METHODS Estimating age by observing the teeth clinically is a con-
. venient and economical method. In clinical set-ups, the
Krogman and Işcan (1986) considered age, sex, popula- age of children is routinely estimated by examining the
tion affinity and stature to be the four essential parame- type of tooth that has emerged into the oral cavity
ters in identification of the deceased when no clue is although, this method falls short of scientific credibility. A
available. The dentition can reveal the first three of these more objective ‘clinical count’ of the number of erupted
parameters. An overview of estimating sex, ethnicity and teeth has also been developed for assessing age in young
669
individuals (Foti et al, 2003), which is described later. In Table 2 Tooth-calcification stages and their coded symbols
adults, however, where all the teeth have emerged other
parameters, such as dental attrition, may be utilized. Stage Coded symbol
Initial cusp formation Ci
Dental attrition Coalescence of cusps Cco
According to Solheim (1988), dental attrition has been Cusp outline complete Coc
used to assess age in archeological and forensic contexts. Crown 1/2 complete Cr½
An advantage of using attrition in age estimation is that it Crown 3/4 complete Cr¾
is one of the few regressive changes of the teeth that can Crown complete Crc
be clinically examined. However, the degree of attrition in
Initial root formation Ri
modern humans may have reduced as a result of diet that
comprises processed food. Moreover, attrition can be aggra- Initial cleft formation Cli
vated in certain pathological conditions such as bruxism. Root length 1/4 complete R¼
Solheim (1988) assessed attrition in different types of teeth Root length 1/2 complete R½
by measuring the surface area, length of the crown and Root length 3/4 complete R¾
certain other criteria, and observed a weak correlation with Root length complete Rc
age. He further developed multiple regression formulas
Apex 1/2 closed A½
which showed that attrition may best be applied for age
assessment using the premolars. However, the author adds Apex completely closed Ac
that age estimates from attrition should be based on Source: Modified from Moorrees et al (1963).
examination of several teeth or the entire dentition.
Demirjian’s method
Radiographic Methods for Age Assessment Demirjian and coworkers (1973, 1976) developed a method
of age estimation which, over the years, has become the
Gleiser and Hunt (1955) first stated that ‘the calcification
most widely used technique to assess age from the dentition
of a tooth may be a more meaningful indication of somatic
of children and adolescents. The method utilized seven per-
maturation than is its clinical emergence’. Calcification is
manent mandibular teeth on the left side (incisor to second
a continuous process which can be assessed from radio-
molar) and did not make use of the third molar. Conse-
graphs. This relies on stages of tooth formation ranging
quently, it was not applicable to individuals older than 16
from the actual formation of crypt to the fully mature
years (since the second molar is fully developed by this
tooth. There is a continuous change in the shape and size
age). A recent modification of the method (Chaillet and
of the teeth with each tooth following the same sequence.
Demirjian, 2004) incorporated the third molars allowing
The radiographic appearance of the teeth may be used for
its application to just over 18 years of age. In addition,
age estimation even after tooth development is complete,
the development of each tooth was divided into 10 stages,
e.g. deposition of secondary dentin can be viewed indi-
instead of the original eight. These stages were numbered
rectly in terms of the size of the pulp chamber.
‘0’ (no visible tooth calcification) to ‘9’ (complete closure of
the root apex). Each of the eight teeth on the radiograph is
Estimating Age in Children and Adolescents compared to a chart that contains a diagrammatic represen-
tation of the 10 stages and assigned an appropriate devel-
Moorrees’ method opmental stage (any one of 0–9). Next, corresponding to the
Moorrees and colleagues (1963) categorized permanent developmental stage, a ‘maturity score’ is allotted to each
tooth calcification into 14 stages (Table 2). The developing tooth. The maturity score assigned for the eight teeth are
tooth is compared to a chart and an appropriate develop- added and a ‘total maturity score’ obtained. The total matu-
ment stage assigned. The selected stage is compared to rity score (S) is substituted in regression formulas and the
chronological age-conversion charts. The charts are com- chronological age derived. Owing to differences in dental
posed of segments, one for a specific tooth, in which the development in males and females, different formulas for age
chronology of its development is recorded graphically by estimation were developed. In females the formula used is:
horizontal bars for each stage. On the horizontal bar, the Age (0.0000615 S3) – (0.0106 S2)(0.6997 S)
mean age of attainment of that stage and the range (1 and – 9.3178
2 SD) are indicated. A scale is provided at the top and
bottom of the chart for indicating chronological age. The In males, the formula used is:
assessment of tooth formation is recorded by ticking the Age (0.000055 S3) – (0.0095 S2)(0.6479 S)
appropriate stage for a particular tooth. – 8.4583
670
The estimated age should accommodate an age range of respects and not an ideal development marker however, in
2.65 years for females and 2.28 years for males. While the absence of other biological indicators of age in the late-
the method is convenient to use and applied extensively, it teens and early adulthood, they are used to estimate age
has a few limitations: (Mincer et al, 1993). Arany and associates (2004) have
adapted Demirjian’s developmental stages exclusively to
❍ If a tooth is missing, the method may not be used
the third molar and assessed if its calcification can be used
(unless the tooth is present on the right side).
to estimate ages with legal implications (e.g. 14, 16 and 18
❍ The 10 developmental stages may not describe the entire
years). They calculated a ~97% probability that an indi-
gamut of stages of tooth development.
vidual is at least 14 year old if the root length crown
❍ Choosing a tooth developmental stage may have inher-
height (Demirjian’s ‘Stage 7’); ~93% probability of 16-years
ent subjectivity.
if the root development is complete but apex patent
Demirjian’s 8-teeth method was recently tested on an (Demirjian’s ‘Stage 8’); and ~98% probability of 18-years
Indian sample (n 100) by Rimal and Sumanth (2006) if the root apex is closed (Demirjian’s ‘Stage 9’). The latter
who revealed that the method underestimated age by has important implications in India and other countries
1.14 0.34 years. This reinforces long-held observations since 18 years is the age of majority, and the consequent
that the accuracy of the method varies from one popula- change in legal outlook toward a person’s rights and actions.
tion to another, possibly due to regional variations in Interestingly, third molar develops faster in males, which
tooth development. Demirjian’s method was developed on is in contrast to the earlier development of all other teeth
children of French ancestry and will need local modifica- in females. One must remember that the probabilities
tions on large samples if it is to be reasonably reliable in described above are based on a study on the Japanese and
the population to be tested. can vary in different populations.
Open apices method A Combined Clinical and Radiographic Method

A recent age estimation method suggests measuring the
To facilitate clinical age assessment, Foti and coworkers
open apices of developing permanent teeth (Cameriere et al,
(2003) have suggested a method which utilizes the number
2006). This method also examined seven mandibular teeth
of erupted teeth. In addition, they also give provision for
on the left side, probably to test its effectiveness in age
using tooth calcification visible on radiographs. Using mul-
assessment vis-à-vis Demirjian’s original method (1973,
tiple regression analysis, they presented formulas for age
1976). For assessing age, orthopantomographs are digitized
estimation for different clinical and forensic scenarios. For
using a flat-bed scanner and the images measured with the
example, when the teeth are visible clinically as well as radio-
aid of the software Adobe Photoshop. Since measurements
graphs are available for observing the calcifying tooth germs,
of the open apexes may be influenced by factors such as
the following formula is recommended:
radiographic magnification and angulation errors, the ratio
of each tooth apex in terms of the respective tooth length Age 16.088 (0.226 number of erupted permanent
was obtained. A multiple linear regression equation was upper 1st molars) (1.564 number of erupted
developed from these ratio which estimated age, on the permanent upper 2nd molars) (0.832 number of
average, to within 0.04 years of the actual age. The high- erupted upper 3rd molars) (0.912 number of erupted
precision level in age estimation has been verified by Rimal lower 3rd molars) (1.699 number of tooth germs on
and Sumanth (2006) in an Indian sample where, chronologi- radiographs excluding 3rd molar germs).
cal age was marginally underestimated (0.32 years). Accuracy
There may be instances when partial skeletal remains,
of the method notwithstanding, the relative effort involved
e.g. only the mandible, are recovered and one may need to
in measuring the apices and tooth length may preclude its
estimate the age based solely on clinically visible teeth.
widespread use, and it is unlikely to replace the acceptance
The formula for estimating age from a mandible in such a
enjoyed by Demirjian’s method in the near future.
scenario is:
Age 9.726 (0.571 number of erupted deciduous
Third Molars in Age Estimation lower incisors) (0.378 number of erupted permanent
lower canines) (0.579 number of erupted lower
With the completion of development of all teeth by about
premolars) (1.056 number of erupted permanent
16 years, the third molars remain the only tooth that con-
lower 2nd molars) (2.236 number of erupted lower
tinues to develop. Consequently, radiographic evaluation of
3rd molars).
third molars is one of the two parameters for age estima-
tion in adolescents and young adults (Arany et al, 2004), Using this method, the authors claim reasonable accu-
the other being morphological examination of skeletal fea- racy in age estimation comparable to those obtained with
tures. The third molar is the most variable tooth in many Demirjian’s method. While the technique is promising,
671
it is as yet untested and needs to be validated on diverse

Figure 14
populations.
The accuracy of dental aging is not uniform from birth to T
maturity. Age estimation in younger ages tends to be more
accurate since more teeth are calcifying and the intervals P
between morphologic stages are shorter and more precise;
in older children, ‘the biological variation increases con-
siderably’ (Hägg and Matsson, 1985). These authors have
reported an age range of 12 months in younger children A R
while the same increases to 20 months in older children.
Moreover, the diversity in the rate of dental development
among children and adolescents of different populations B
undermines accuracy of the preceding age estimation meth-
ods. Development of population-based dental development
C
standards could render the age estimates more acceptable.
Estimating Age in Adults

In adults, the teeth are completely developed and one cannot
rely on tooth emergence or stages of calcification for assess-
ing the age. Traditionally, histological methods have been
Diagram showing measurements made on the radiograph of
used for estimating age in this group, most notable of these
each tooth. The ratio of these lengths and widths were used
being Gustafson’s pioneering work (1950) and its subsequent
in age assessment. (Reprinted from Kvaal SI, Kolltveit KM,
modification by Johanson (1971). A major disadvantage of Thompsen IO, et al (1995), ‘Age estimation of adults from
these methods, however, is the necessity to extract and sec- dental radiographs‘, Forensic Science International, Vol. 74,
tion the teeth. While this may be practical in dead individu- pp. 175–85, with permission from Elsevier)
als, it is neither possible nor ethical among living adults.
Kvaal’s radiographic method the efficacy of the method on digitized radiographs and
A method developed by Kvaal and associates (1995) side- obtained lower correlation with age. However, more recently,
steps the necessity for extracted teeth in adult age esti- Bosmans and coworkers (2005) have obtained accurate age
mation. Their method used the tooth, root and pulp size estimates on digital orthopantomographs. Further testing
measurement of six teeth (maxillary central and lateral inci- on conventional and digital radiographs in different popu-
sor and second premolar; mandibular lateral incisor, lation groups will reinforce the validity of this method.
canine and first premolar) observed on periapical radio-
graphs. The measurements included several length and
width ratio (Figure 14) such as pulp/root length (P), pulp/ BITE MARK PROCEDURES
tooth length (R), tooth/root length (T), pulp/root width at
CEJ (A), pulp/root width at mid-root level (C), pulp/root Dental identification makes use of the randomness of intact,
width at mid-point between levels A and C (B), mean value diseased, restored and missing teeth. Bite mark investiga-
of all ratio excluding T (M), mean value of width ratio B tion, on the other hand, uses the configuration of teeth to
and C (W), mean value of length ratio P and R (L). These establish the identity of a biter. The size, shape, angulation
ratio were used to compensate for magnification and angu- and pattern of the biting edges of the anterior teeth in the
lation errors of teeth on the radiograph. When six teeth upper and lower dental arches can be arranged in 1.36 1026
(right or left side) from both jaws are available, the follow- different ways (Rawson et al, 1984). But the dynamics
ing regression formula can be used: involved during production of bite marks—such as movement
of the biter’s jaw, movement of the victim and flexibility of
Age 129.8 316.4 (M) 66.8 (W – L).
the bitten tissue—renders the appearance of the mark highly
The length measurements may be recorded using a calliper variable (Figure 15) and the presumed uniqueness of the den-
however, the widths need to be measured using a stereo- tition may not be depicted on the mark (Pretty, 2006a).
microscope—an equipment that may not be available rou- Therefore, investigating bite marks, perhaps, provides the
tinely in Indian dental set-ups. Hence, applying this method greatest challenge to forensic odontologists (Brown, 1985).
on digitized radiographs and measuring the teeth on a com- Bite marks have been described as marks made by the
puter monitor with accompanying software tools may be teeth either alone or in combination with other mouth parts
more convenient. Kolltveit and colleagues (1998) first tested (MacDonald, 1974). Bite marks may be caused by humans
672
Figure 15
A B
Two distinct bite marks (A, B) on the back of a victim made by the same dentition. In both photographs, mandibular arch mark is
further from the scale. (Reprinted from Sheasby DR, MacDonald DG (2001), ‘A forensic classification of distortion in human
bitemarks‘, Forensic Science International, Vol. 122, pp. 75–8, with permission from Elsevier)
or animals; these may be on tissue, edible items or objects. surface as a result of tooth pressure during a bite causes
Biting is considered to be a primitive type of assault and indentations. Indentations, while ideal for collecting as evi-
results when teeth are employed as a weapon of attack, dence and subsequent analysis, seldom persist for long dura-
dominance or self-defense. As a result, bite marks are usu- tions unless the victim is deceased. Owing to the elastic nature
ally associated with sex crimes and violent fights. Hence, of skin, indentations soon disappear and the skin regains
matching a bite mark to a suspect’s dentition may enable its original contour. This is followed by a brief period
law enforcers to implicate the suspect in the crime. of edema over the bitten area, which usually obscures the
bite mark completely. Once the edema subsides, subcuta-
neous bleeding becomes apparent. These are referred to as
How Does a Bite Mark Look? contusions or bruises and are the most common presenta-
Features of a bite mark tion of bite marks. These appear as reddish/purplish discol-
oration on the skin surface, a result of blood escaping into
The human bite mark may be identified by its gross, class the subcutaneous tissue from ruptured vessels. With increased
and individual features (Sweet, 1995): intensity of the bite, there may be abrasions and lacerations
Gross features Grossly, a bite mark usually appears as a of the skin. The most extreme form of bite mark injury is
circular or elliptical mark with a central ecchymosed area. avulsion, where part of the tissue is bitten off. However, the
The circular/elliptical mark is caused by the dental arches forensic significance of a bite mark does not necessarily
while the central area of ecchymosis is apparently due to increase with the severity of the injury. Pretty (2006b) has
sucking action or negative pressure created by the mouth. proposed a ‘bite mark severity and significance scale’ to assist
forensic dentists in determining how valuable a bite mark is
Class features The marks produced by different types of as forensic evidence. The proposed index has two parts—a text-
teeth are usually distinct allowing one to differentiate the based index (Figure 16) and a pictorial guide (Figure 17). As
tooth class. For example, incisors produce rectangular, canines illustrated in these figures, well-defined bruising and lac-
triangular and premolars spherical-shaped patterns. erations are usually best suited for forensic investigation.
Individual features Class features may, in turn, have
characteristics such as rotations, fractures, etc. Such fea- Site of Injury
tures are known as individual features and render the bite Bite marks may be found on any part of the body (Pretty
mark distinct. and Sweet, 2000). However, females are bitten most often
on the breast followed by the arms, legs and thighs while
males are bitten on the arm, hands/fingers and the back.
Type of Injury
Interestingly, many of the male ‘victims’ received bites dur-
A bite mark may appear as an indentation, contusion, abra- ing the course of a crime committed by them, i.e. they were
sion, laceration or avulsion. Initially, compression of the skin bitten by their victims, probably in self-defense.
673
Figure 16
1. Very mild bruising, no individual tooth marks present,
diffuse arches visible, may be caused by something other
than teeth—low forensic significance
2. Obvious bruising with individual, discrete areas associ-

ated with teeth, skin remains intact—moderate forensic
significance
3. Very obvious bruising with small lacerations associated

with teeth on the most severe aspects of the injury, likely
to be assessed as definite bite mark—high significance
High
Increasing severity
forensic
4. Numerous areas of laceration, with some bruising, significance
some areas of the wound may be incised. Unlikely to
be confused with any other injury mechanism—a high
forensic significance
5. Partial avulsion of tissue, some lacerations present indi-

cating teeth as the probable cause of the injury—moderate
forensic significance
6. Complete avulsion of tissue, possibly some scalloping

of the injury margins suggested that teeth may have
been responsible for the injury may not be an obvious
bite injury—low forensic significance
The bite mark severity and significance scale. (Reprinted from Pretty IA (2006), ‘The barriers to achieving an evidence base for
bitemark analysis’, Forensic Science International, Vol. 159S, pp. S110–20, with permission from Elsevier)
Differential Diagnosis of Human Bite Marks ❍ Does the age of the bite mark correspond to the time
and type of crime?
Grey (1989) has reported a case where defibrillator paddles ❍ Are there unique, individual characteristics in the bite
used in resuscitation caused artifacts that resembled mark?
bite marks. He urges caution while investigating what may ❍ Can these characteristics be compared to the teeth of
appear as a bite mark to the casual eye—‘things are not the suspect?
always what they seem to be; care must always be taken not
to misinterpret findings’. Other bite mark-mimicking inju- Once these questions have been answered satisfactorily,
ries may be caused by: one may proceed with the next step—collecting bite mark
evidence.
❍ Hair curling iron
❍ The ends of a pipe
❍ Jewellery Collecting Bite Mark Evidence
❍ Shoe heel.
When a suspected case of bite mark is presented, immedi-
These, however, are usually differentiated from true bite ately report it to the law enforcement agency and collect
marks by the absence of class characteristics. necessary evidence. However, the primary concern is
patient care—at no time should collection of bite mark evi-
dence interfere with timely patient treatment. This is espe-
Bite Mark Investigation cially important since Pretty and associates (1999) have
Any attempt at bite mark investigation should begin with reported that human bites have a great potential for infec-
the following questions (Drinnan and Melton, 1985; Sweet, tion (including HIV, hepatitis B and syphilis), particularly
1995): when the bite injury presents as an abrasion or laceration.
Drawing from the experience and suggestions of accred-
❍ Is the injury a bite mark? ited forensic dentists, the American Board of Forensic
❍ If a bite, was it caused by human teeth? Odontology (1995) has suggested the following protocol
❍ Was it caused by an adult or a child? for bite mark evidence collection.
674
Figure 17
A B
C D
E F
Visual index of the bite mark severity and significance scale. (Reprinted from Pretty IA (2006), ‘The barriers to achieving an
evidence base for bitemark analysis‘, Forensic Science International, Vol. 159S, pp. S110–20, with permission from Elsevier)
Demographics of the Case Visual Examination and Documentation

Vital information pertaining to the case should be noted Visually examine the bite mark and document the following:
first, e.g. name, age and sex of the victim; case number, ❍ Location, shape, color and size
date of examination and name of the examiner(s). ❍ Type of injury
675
❍ Contour, texture and elasticity of the bite site should be taken. In case of a living victim, photographs can
❍ Differences between upper and lower arches, and be repeated every 24 hours for 3–4 days to record progres-
between individual teeth sive changes in the pattern of bruising (Brown, 1985).
Note: If the victim is dead, examine the bite mark prior

to autopsy (Brown, 1985). Saliva Swab
It is reasonable to assume that a bite cannot be inflicted
without leaving saliva behind. Saliva deposited in the bitten
Photographs
area may be a source of cellular DNA, enabling a direct link
High-quality photographs provide a permanent record of to the suspect. Care should be taken not to wash the bite
the bite mark appearance. No time should be lost in area before saliva swabbing. According to Clift and Lamont
obtaining photographs as the injury rapidly changes (1974), the amount of saliva deposited in a bite mark is
appearance due to healing in living victims or postmor- about 0.3 ml, distributed over an area of 20 cm2. However,
tem change in the deceased. Color and black-and-white by the time of evidence collection, the bite area may have
photographs may be taken using off-angle light source. dried up. Therefore, a cotton swab moistened with distilled
Orientation and close-up photographs (Figure 18) should or clean tap-water should be used for swabbing. This
be taken: orientation photographs depict the location of rehydrates the dry cells in the bite area. The swab is held
the bite mark on the body; close-up photographs of the vertical along its long axis making circular motion with
bite mark should be made with a rigid reference scale—a moderate pressure over the bitten surface. A second swab,
ruler, such as the ABFO No. 2 scale, should be placed on which is dry, is used to collect the moisture left on the skin
the same plane as the bite mark. The entire scale and bite by the first swab and cells that remain. This constitutes the
mark must be visible on the photograph. A second close- ‘double swab technique’ recommended by Sweet and asso-
up photograph depicting the bite mark without the scale ciates (1997) and is supposed to yield higher amounts of
can be made to indicate that no portion of the mark has salivary DNA. Both swabs are then air-dried at room tem-
been concealed by the scale. The camera should be posi- perature for about 30 minutes. Following this, the swabs
tioned directly over the injury site with the long axis of the should be placed in paper envelopes to facilitate continued
lens perpendicular to the surface of the bitten skin—this air circulation around the swab tips (Sweet and Pretty,
decreases perspective distortion of the image due to off- 2001). These are labeled and stored under refrigeration.
angle camera position (Sweet and Pretty, 2001). If the bite The latter prevents degradation of salivary DNA and bac-
is on a curved surface and the upper and lower arch marks terial growth in case of delay in lab processing. A third
are widely spaced, separate photographs of each mark control swab may be taken from an anatomically similar
Figure 18
A B
(A) Anatomical location of bite mark on victim’s left shoulder; (B) close-up photograph of the bite mark.
(Note: The use of flexible scales is not recommended for bite mark evidence collection and ABFO No. 2 scale (Figure 17)
is preferred). (Reprinted from Pretty IA (2006), ‘The barriers to achieving an evidence base for bitemark analysis’,
Forensic Science International, Vol. 159S, pp. S110–20, with permission from Elsevier)
676
location outside the bite area and bagged separately. Clark testimony that proves that the evidence has not been altered
(1992) has suggested that if the bite has occurred through or tampered with in any way since it was obtained. This is
clothing, the clothes must also be swabbed. The use of necessary both to assure its admissibility in courts of law
high-intensity alternative light source (such as UV light) to and its probative value in preceding investigations.
locate stains from body fluids enable saliva traces to be
recovered even in the absence of visible bite marks. There
Analyzing and Comparing Bite Mark Evidence
are some disadvantages to salivary DNA analysis, includ-
ing the need for extensive laboratory equipment and Ideally, bite mark analysis should begin with a qualitative
expertize, high cost and possible degradation of DNA. and quantitative analysis in situ. This should be followed
Nevertheless, ‘there is an increasing uptake of such tech- by the analysis of life-sized or enlarged photographs.
nology’ among forensic dentists (Pretty, 2006a). A separate qualitative and quantitative analysis of the
models and occlusal registrations of the suspect’s denti-
Note: Saliva swabs may also be collected according to tion can be performed at this stage. Rather than relying on
the recommendations of the testing laboratory. the number of teeth depicted in the mark, analyze uncom-
mon characteristics such as presence or absence of a par-
Some important steps in managing bite mark cases: ticular tooth, mesiodistal dimension of the teeth and dental
arch, rotation, fracture and diastema. Following this, one
❍ Notify concerned legal authorities and consult a forensic may proceed with the comparison.
odontologist. According to Sweet (1995), the protocol for bite mark
❍ Record history and perform a detailed physical exam- comparison is made up of two broad categories: (i) metric
ination. analysis, and (ii) pattern association.
❍ Obtain adequate records of the bite marks including:
– Documentation Metric analysis The measurement of specific features of
– Photographs the bite mark and suspect’s dentition is known as ‘metric
– Saliva swab. analysis’. Measurements may be made on life-sized photo-
graphs of the bite mark and include:
❍ Overall size of the mark
Obtaining Dental Evidence from a Suspect
❍ Intercanine distance
The bite mark evidence collected should be complemented ❍ The length and width of the tooth
with dental evidence from the suspect who has purport- ❍ Spacing between tooth marks
edly caused the bite. Evidence must be obtained in the ❍ Rotation from normal arch form.
presence of law enforcers, using a signed and witnessed
A similar procedure is employed on the suspect’s dental
informed consent or a warrant; infection control and
casts. The measurements thus obtained from the mark and
asepsis must be adhered to. Begin by obtaining history of
the cast are compared to one another. Simple instruments
any dental treatment subsequent to, or in proximity of, the
like callipers may be used for the measurements. Recently,
date of bite mark; follow it up with a detailed extra- and
computer-based measurements using software such as
intraoral clinical examination. Other evidence recovered
Adobe Photoshop have also been used (Johansen and
should include:
Bowers, 2000). Metric analysis, however, must be applied
❍ Photographs of the suspect’s teeth in occlusion and in in conjunction with ‘pattern association’.
open bite
Pattern association Matching the configuration of the
❍ Maxillary and mandibular impressions made with rub-
bite injury to the arrangement of teeth on the suspect’s
ber-base impression material or irreversible hydrocolloid
dental cast is called ‘pattern association’. This can be done
and models poured in dental stone. It is useful to pour at
by hand-tracing the incisal and occlusal edges of the sus-
least one additional cast of the suspect as backup
pect’s teeth on transparent acetate sheets and superimposing
❍ Bite registration on a sheet of wax may be obtained in
these ‘overlays’ on life-sized bite mark photographs. Sweet
the absence of impressions
and coworkers (1998) developed a computer-based tech-
❍ If saliva has been recovered from the bite mark,
nique to produce overlays, which was found to be the
swab should be obtained from the suspect for DNA
most accurate tracing method (Sweet and Bowers, 1998).
comparison.
Johansen and Bowers (2000) have elaborated on this tech-
All evidence obtained must be labeled and stored appro- nique to develop a digital method for bite mark compari-
priately—the case number, date, time, place, as well as any son and newer methods have since been reported (Thali
witnesses involved must be recorded at every step of evi- et al, 2003) (Figure 19). The trend today appears to be mov-
dence collection to maintain the chain of custody. In prac- ing toward the use of computer-based methods (Pretty,
tical terms, a chain of custody is the documentation and 2006a); nevertheless, standardization of the comparison
677
However, in a recent review of bite mark analysis,

Figure 19
Pretty (2006a) has warned against considering such a sys-
tem of conclusion as an absolute interpretation of bite
mark evidence. He believes that existing research data on
physical bite mark comparison is not infallible and has
stressed that ‘to prevent miscarriages of justice it is essential
that odontologists are highly trained, undergo proficiency
testing and aim to collect biological evidence whenever
possible’. Further, he adds that while presenting conclu-
sions to the courts of law one must avoid overstating the
level of certainty and acknowledge the subjectivity of the
analysis.
LIP PRINT INVESTIGATION
Lip print—the study of which is called cheiloscopy—can

be important in crime investigation (Caldas et al, 2007).
A bite mark is compared to digitized casts of the suspect using
According to Ehara and Marumo (1998), lipstick smears
3D/CAD program. (Reprinted from Thali MJ, Braun M,
Markwalder TH, et al (2003), ‘Bitemark documentation and
are frequently encountered in forensic investigations as an
analysis: the forensic 3D/CAD supported photogrammetry important form of transfer evidence. These researchers
approach‘, Forensic Science International, Vol. 135, No. 2, have stated that ‘lipstick smears on the suspects’ clothing
pp. 115–21, with permission from Elsevier) can indirectly prove a link between the suspect and a
female victim, and smears left on cigarette butts, glasses
or cups can prove a link between a suspect and a crime
process is yet to be achieved and conclusion of the analy- scene’.
sis is usually based on the expert’s level of personal expe- Snyder (1950) is believed to have first pointed out that
rience and judgment (Rothwell, 1995). the lines and fissures on the lips have individual varia-
tions, much like fingerprints. Renaud (1972) examined lip
prints on 4,000 individuals and found them to be singular.
Conclusions of Bite Mark Analysis The uniqueness of lip grooves has also been confirmed by
Levine (1982) has suggested three likely outcomes or con- Tsuchihashi (1974), who studied more than 1,300 lip prints.
clusions to bite mark comparison, which has been modified These researchers and a few others studied lip prints using
as follows: similar classification, a composite of which is:
1. Vertical grooves*
Positive identification 2. Branched grooves*
3. Bifurcated grooves*
There is reasonable dental certainty to indicate that the
4. Intersected grooves
bite mark has been produced by the suspect’s dentition—this
5. Reticular grooves
implies that there are characteristic matches between the
6. Other grooves (comma, ellipse, triangle, horizontal,
bite mark dimensions/pattern and that of the suspect’s teeth.
etc.)
Possible identification Both Renaud (1972) and Tsuchihashi (1974) recorded the
lip pattern of an individual quadrant-wise, i.e. the lips
The bite mark and the suspect’s dentition are consistent—
were divided into four quadrants, much like the dentition—
although the suspect’s teeth could have made the bite mark,
a horizontal line dividing the upper and lower lip and a
there are no characteristic matches to be absolutely certain.
vertical line along the sagittal plane dividing the lips into
right and left sides. Grooves in each quadrant of the lip
Excludes identification
were recorded according to the above classification.
The bite mark and the suspect’s dentition are not consis- Suzuki and Tsuchihashi (1975) applied the classification
tent—features on the bite mark indicate that the suspect’s in two criminal cases, both of which enabled the exclusion
teeth have definitely not caused them. of the suspects—in effect proving the innocence of the
*These grooves may traverse the lips completely or partially.
678
Figure 20
A B
(A) Invisible lip print prior to and (B) after developing with lysochrome powder. (Reprinted from Navarro E,
Castelló A, López JL, et al (2006), ‘Criminalisitic, effectiveness of lysochromes on the developing of invisible
lipstick-contaminated lipmarks on human skin—a preliminary study‘, Forensic Science International,
suspected perpetrators, which is of equal importance as However, further studies on whether the lip prints devel-
proving the identity of criminals. These two cases investi- oped can be accurately compared to those of a suspect are
gated lip prints caused by the use of lipsticks that leave warranted. According to Tsuchihashi (1974), lip grooves
behind a visible mark. More recently, however, the cos- are on the zone of transition of the lips which, being
metics industry has developed lipsticks that do no leave a extremely mobile, are affected by the pressure applied by
visible trace on contact with a surface (Seguí et al, 2000). the lips and their direction. Consequently, there is the pos-
Such colorless lip prints, too, can be visualized using sibility that lip prints of one individual may be mistaken
developing reagents such as metallic powders (Seguí et al, for another’s. However, the classification provided and
2000) or lysochromes (Navarro et al, 2006) (Figure 20). quadrant-based recording of the lip grooves will probably
However, developing lip prints on colored or multicolored render a successful comparison. The suggested method for
surfaces using these reagents may not prove useful due to comparison is to examine lip prints closest to the mid-line
contrast problems (e.g. Sudan Black—a lysochrome pow- and then work laterally (Tsuchihashi, 1974). Whether lip
der of the same color—may not be useful in developing lip print evidence is admissible in court and can stand the
prints on a black ceramic cup). Therefore, some authors rigors of legal scrutiny is a matter of debate. According to
have advocated the use of fluorescent reagents such as Ball (2002), there is very little science and research to sup-
Nile Red (Castelló et al, 2005). This reagent can either be port a methodology for collecting and comparing lip print
used in powder form or in solution (0.1 mg Nile Red dis- evidence which has gained acceptance within the forensic
solved in 100 ml ethanol). The reagent powder or solution community. Further research to verify the uniqueness of
is applied to the area of interest (with a brush or piece of lip prints, develop protocols for lip print investigation and
cloth, respectively) and visualized under UV or blue light. establish its accuracy in comparative analysis is essential,
The shape, outline and grooves of the lips were discernible without which it ‘would fail to meet any scientific stan-
and the authors believe that it has value in identification. dards of reliability’ (Ball, 2002).
679

PART Oral Radiology
II
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SECTION Basics of
IX Radiology
25 Basics of Radiation Physics 685

26 Radiation Biology 697
683
Chapter-25.indd 683 10/3/2012 10:48:27 AM


CHAPTER
Basics of Radiation Physics

Ravikiran Ongole 25
➧ Pioneers in Dental Radiology ➧ Intraoral X-ray Units
➧ Fundamentals of Radiation Physics Components of an Intraoral X-ray Machine
Radiation Filters and Collimators
Particulate Radiation ➧ Interaction of X-rays with Matter
Electromagnetic Radiation Scattering
Ionizing and Non-ionizing Radiation Absorption
Production of X-rays
Properties of X-rays
PIONEERS IN DENTAL RADIOLOGY the University of Würzburg for 6 years as Rector, he took
over as Director of a new physical institute in the University
Wilhelm Conrad Röentgen (1845–1923) of Munich. For his outstanding work, Röentgen was
awarded an honorary MD degree by the University of
Wilhelm Röentgen, the physicist from Germany is consid- Würzburg. He was awarded the Rumford Gold Medal from
ered the father of diagnostic radiology. Röentgen discov- the Royal Society (1896) and a gold medal from the
ered X-rays in 1895. Franklin Institute of Philadelphia. He was honored with
the first Nobel Prize in Physics in 1901. The International
Personal life and early years Union of Pure and Applied Chemistry (IUPAC) in honor of
Röentgen, named element number 111 as roentgenium (Rg)
Wilhelm was born to a German father Friedrich Conrad
in 2004.
Röentgen and Dutch mother Charlotte Constanze on March
27th, 1845 in the small town of Lennep in Rhineland. When
Publications on X-rays
Röentgen was 16 years old he enrolled in Utrecht Technical
School, Netherlands. In 1868, he obtained a diploma in 1. ‘On a New Kind of Rays, A Preliminary Communi-
mechanical engineering from the Polytechnic School in cation’. Journal of Würzburg Physical-Medical Society,
Zurich. Under the tutelage of AEE Kundt, Wilhelm studied December 28, 1985.
the properties of gases and obtained a doctoral degree in 2. ‘On a New Kind of Rays, Continued’. Journal of
1869. After obtaining his PhD, he worked as Kundt’s assis- Würzburg Physical-Medical Society, March 9, 1896.
tant at the University of Würzburg and later elevated to the 3. ‘Further Observations on a New Kind of Rays’. Journal
post of an associate professor in theoretical physics. Wilhelm of Prussian Academy of Science, March 10, 1897.
married Anna Ludwig in 1872. She was the daughter of an
inn-keeper and 6 years elder to him. His wife died in 1919,
following a prolonged illness and Wilhelm died due to William Herbert Rollins (1852–1929)
intestinal cancer at the age of 73 years on February 10th,
William Herbert Rollins was an American scientist and
1923. They were buried in Giessen.
dentist. He was a pioneer in radiation protection and is
known as the father of radiation protection. It is believed
Recognition and awards
that he had published over 200 scientific articles regarding
The University of Würzburg appointed Röentgen as pro- the hazards of radiation. Rollins, although a practicing
fessor of physics in 1888. Just 6 years later in 1894, he was dentist, also had a medical degree from Harvard Medical
chosen as the Rector of the same university. After serving School. He called X-rays ‘X-light’ and documented them
685
Section IX – Basics of Radiology
extensively in 1904. He is called the ‘Forgotten Man’ of used to help him to mix materials and take the X-rays.
dentistry. William Rollins proposed the use of filters to In 1986, Edmund Kells and Brown Ayres devised a technique
remove the low energy X-rays from the primary beam and for radiographing the teeth and jaws. Dr Kells, in Dental
introduced collimation. He recommended a long target- Cosmos, mentioned the importance of keeping the film and
film distance to improve image quality. He pioneered the object at right angles to the source using a film holder. In
sandwiching of the X-ray film between two intensifying 1903, he introduced processing tanks and time-tempera-
screens to increase the film speed. Dr Rollins advocated the ture processing. In 1909, Kells cut, wrapped and used roll-
need to determine a safe and harmless dose of radiation. In type photographic film.
1901, he advocated the use of leaded glasses for radiation
personnel and a lead shield to cover all areas on the
patient’s body that were not being imaged. Rollins also felt Friedrich Otto Walkhoff (1860–1934)
the need to construct a lead hood that would cover the
Friedrich Otto Walkhoff was a Berlin dentist, a pioneer of
X-ray tube head.
dental X-ray diagnostics and a dedicated fighter for civil
interests of dentists. It was barely 14 days after the
Howard Riley Raper (1887–1978) announcement of the discovery of roentgen rays that
Walkhoff wrapped a photographic glass plate in a rou-
In 1909, Dr Howard Raper, was the first to introduce tinely used rubber dam material and held it with his teeth.
radiology as a subject in the dental school. He also had He then laid still on the floor for an X-ray exposure that
three other ‘firsts’ to his credit—first Professor of Radiology, lasted a full 25 minutes. This was considered the first den-
first academician to teach dental radiology and published tal radiograph. A year later, he managed to take extraoral
the first textbook dealing with oral radiology titled radiographs with an exposure time of 30 minutes.
Elementary and Dental Radiography, in 1913. In 1917, he
proposed the first model of angle meter used with a chart
of vertical angles for various teeth to avoid distortion. Gordon Fitzgerald (1907–1981)
In 1925, in co-operation with Eastman Kodak Company,
Dr Raper developed the bitewing technique for the detec- In the 1940s, Dr Fitzgerald designed the long cone for the
tion of interproximal caries. dental X-ray machine. He published four papers on long
cone technique in the Journal of American Dental Associa-
tion between 1947 and 1950.
Weston A Price (1870–1948)
Weston Andrew Valleau Price was a dentist known pri- Frank Van Woert (1856–1927)
marily for his theories on the relationship between nutri-
tion, dental health, and physical health. Dr Price, in 1897, Dr Frank Van Woert is credited for his work on the use of
a founding member of the American Roentgen Ray Society, films, film holders and processing of films. He was the first
designed and patented lead-lined gloves for protection to use films as image receptors which were developed by
against X-ray burns, but placed his innovation in the public Kodak. He also engineered a metallic holder which could
domain instead of commanding fees from users. In 1900, be used to hold films. His other inventions include the
Price designed a celluloid-based dental film. In 1904, he daylight film processing tank, an improved angle meter
proposed two techniques for film positioning in the oral for bisecting angle technique and an automated exposure
cavity, namely, the paralleling technique and the bisecting timer switch.
angle technique.
Franklin W McCormack
Edmund C Kells (1856–1928)
Franklin McCormack, an American medical X-ray techni-
Edmund Kells was born in New Orleans, Louisiana, to a cian, employed paralleling technique principles in intra-
dentist Charles E Kells. He became a dentist, researcher, oral radiography. He wrapped the film with a black paper
and inventor. His experiments caused the loss of most of and used a flat metal plate to make the film packet rigid.
his left arm. In 1899, he set up the first X-ray laboratory He was also known for using bite blocks and hemostat as
(The New Orleans X-Ray Laboratory) for radiographs and film holders to stabilize the film in the mouth.
fluoroscopic examinations. He demonstrated the radio- Readers are encouraged to visit the website of the
graph of chest and hip, a bullet in the head and the mea- roentgen museum for more information on the chronol-
surement of a root canal. He hired the first acknowledged ogy of events in origin of radiology (http://www.roent-
dental assistant—Malvina Cuera, before which, his wife genmuseum.de).
686
Chapter 25 – Basics of Radiation Physics
Particulate Radiation
Films and Processing of Films
Particulate radiation consists of a stream of atomic or sub-
1896: The exposure time varied between 5 and
atomic particles that transmit kinetic energy by means of
15 minutes and the processing time var-
their small masses moving at very high velocities. They may
ied between 30 and 60 minutes
carry a positive charge (alpha particles), negative charge
1913: Hand-wrapped, moisture-proof, dental
(beta particles) or no charge (neutrons). Examples of par-
packet containing two films
ticulate radiation are alpha rays, beta rays and cathode rays.
1919: First machine-wrapped dental X-ray
film packet called Regular film (Kodak)
with emulsion on only one side Electromagnetic Radiation
1920: Film hangers were introduced
Early 1920s: Cellulose nitrate as base, emitted poison- There are two concepts to understand electromagnetic
ous gases on burning radiation, namely, classical theory and modern quantum
1924: Radia-tized film (Kodak) with double theory. According to the classical theory, the flow of
side emulsion energy at the universal speed of light through free space or
1924: Non-flammable cellulose triacetate film through a material medium is in the form of the electric
base and magnetic fields that make up electromagnetic waves
1940: Ultra speed (improved Radia-tized film) such as radio waves, visible light, and gamma rays. In
doubled the film speed of the 1924 film such a wave, time-varying electric and magnetic fields are
Early 1960s: Polyester film base mutually linked with each other at right angles and per-
1980s: Ektaspeed film introduced by Kodak pendicular to the direction of motion (Figure 1).
In terms of the modern quantum theory, electromag-
netic radiation is the flow of photons (also called light
quanta) through space. Photons are packets of energy (h␯)
that always move with the universal speed of light. The
FUNDAMENTALS OF RADIATION PHYSICS symbol h is Planck’s constant, while the value of ␯ is the
same as that of the frequency of the electromagnetic wave
of classical theory. The spectrum of frequencies of electro-
Radiation
magnetic radiation extends from very low values over the
Radiation is the transmission of energy through space and range of radio waves, television waves, and microwaves to
matter. There are two basic forms of radiation, namely, visible light and beyond to the substantially higher values
particulate radiation and electromagnetic radiation. of ultraviolet light, X-rays, and gamma rays.
Figure 1
Propagation vector
Electric field
Magnetic field
Electromagnetic spectrum
687
Figure 2 Production of X-rays

1 X-ray photons are produced within the X-ray tube by
10, 000 accelerating electrons with a high voltage and allowing
1 X-ray therapy them to collide with a metal target. This collision results
1000 in the production of X-rays by two basic mechanisms:
1 Bremsstrahlung radiation and characteristic radiation.
100
Measured in angstrom units
Bremsstrahlung radiation is the major source of X-ray

1 photons from the X-ray tube. Characteristic radiation con-
10 X-ray radiography
tributes only to a small amount of X-ray photons that are
1
produced from the X-ray tube.
10
100 Ultraviolet ray
Sun lamp Bremsstrahlung radiation
1000
Light ray It is also referred to as braking radiation or deceleration
10,000 radiation (Figure 3). Bremsstrahlung is derived from the
photography
100,000 Infrared ray German words bremsen meaning ‘to brake’ and strahlung
toaster meaning ‘radiation’. The sudden ‘braking’ of high speed
1,000,000
electrons at the target produces bremsstrahlung radiation.
1
Microwave When high speed electrons are directed toward the
1000
oven tungsten target, they interact with the nuclei of the atoms
1
100 in the tungsten target in two ways, namely, direct hit and
1 near miss/wide miss.
Measured in meters
10
1 Direct hit interaction In this interaction the high speed
Radar
10 electrons collide head on with the nuclei of the atoms in
100
the tungsten target. However, very few electrons display
such direct collisions. When such a collision takes place,
1000
the total kinetic energy of the high speed electron is con-
10,000 Television verted into a single X-ray photon whose energy is numer-
100,000 ically equal to the energy of the high speed electron.
1,000,000 Radio Near miss/wide miss interaction Majority of the inter-
10,000,000 actions of the high speed electrons with the nuclei of the
atoms of the tungsten target are near/wide misses. Because
100,000,000
of the attractive force of the nucleus, the high speed nega-
tively charged electrons are drawn toward the nucleus
Various energy levels of the electromagnetic spectrum
(however, does not collide head on), resulting in the deflec-
tion of the electron from its original path. This results in
the electron losing some of its kinetic energy, which is
given out in the form of an X-ray photon. The closer the
Ionizing and Non-ionizing Radiation
high speed electron is drawn toward the nucleus greater
Radiation has a wide range of energies that form the elec- will be the stopping/braking effect and resultant energy of
tromagnetic spectrum (Figure 2). The spectrum has two the photon.
major divisions:
1. Non-ionizing radiation Characteristic radiation (Figure 4A, B)
2. Ionizing radiation.
Apart from the direct hit and near/wide misses many of
Radiation that has enough energy to move around atoms the high speed electrons interact with the electrons in the
in a molecule or cause them to vibrate, but not enough to outer orbit around the nucleus. This interaction causes
remove electrons, is referred to as ‘non-ionizing radiation’. removal of the electron from its shell, thereby ionizing the
Examples of this kind of radiation include radio waves, atom. The vacant site in the inner shell produced by the
infrared, ultraviolet, visible radiation and microwaves. displaced electron is quickly filled up by another electron
Radiation that falls within the ‘ionizing radiation’ range from an outer shell. When the displaced electron is replaced,
has enough energy to remove tightly bound electrons from a photon is emitted with the energy equivalent to the dif-
atoms, thus creating ions. ference in the two orbital binding energies.
688
Figure 3
X-ray photon
Direct hit Near miss
X-ray photon
High speed electron
Target
Bremsstrahlung radiation
Bremsstrahlung radiation. Courtesy: Dr Jaideep Shekhar
Figure 4
A B
Characteristic radiation. (A) Interaction with inner shell electron. (B) interaction with outer shell electron.
Courtesy: Dr Jaideep Shekhar
Properties of X-rays
(Inverse square law: For a given beam, the intensity is
1. X-rays are forms of electromagnetic radiation. inversely proportional to the square of the distance
2. They have short wavelength and hence exhibit great from the source).
penetrating power. 12. They are invisible to eye and cannot be heard or smelt.
3. They travel at the speed of light (3 ⫻ 108 meters/second 13. They exhibit properties of interference, diffraction and
or 186,000 miles/second). refraction similar to visible light.
4. They affect (produce images) photographic plates and 14. They produce an electric field at right angles to their
X-ray films. path of propagation.
5. They travel in straight lines in the form of waves. 15. They produce a magnetic field at right angles to the
6. X-rays are made up of small packets of energy called electric field and path of propagation.
photons or quanta. 16. They do not require any medium for propagation.
7. They can ionize gases. 17. X-rays can penetrate liquids, solids and gases. The
8. They cannot be focused using a lens. degree of penetration depends on quality, intensity
9. They cannot be reflected, refracted or deflected by and wavelength of X-ray beam.
magnetic or electric field. 18. They are absorbed by matter, the absorption depends
10. They can penetrate opaque objects. on the anatomic structure of the matter and the wave-
11. They follow the inverse square law. length of the X-ray beam.
689
Figure 5 Figure 6
Wall mounted intraoral radiographic unit Mobile intraoral radiographic unit mounted on
a stand with casters
19. X-rays interact with materials they penetrate and

cause ionization. Figure 7
20. When X-rays fall upon certain materials, visible light
will be emitted called fluorescence.
21. X-rays have the property of attenuation, absorption
and scattering.
22. They exhibit heating effect.
23. They can stimulate or destroy living tissues.
24. They have a germicidal or bactericidal effect.
Other biological effects of X-rays are discussed in Chapter 26
on Radiation Biology.
INTRAORAL X-RAY UNITS
Various types of dental X-ray (intraoral radiography) units

are available to suit the needs of the maxillofacial radiology Photograph depicting the X-ray unit mounted on the wall
department. behind the patient’s head
Majority of the intraoral X-ray units are meant for wall
mounting (Figure 5) or simply fixed to the floor. However,
mobile versions are also widely used. These mobile units room, it eliminates the need for multiple X-ray units, and
comprise of a standard intraoral X-ray unit mounted on these can be used effectively in remote rural areas. Studies
a stand with casters (Figure 6). These are referred to as have shown that these systems help in reducing the num-
‘stand-type’ units. These units can be freely moved between ber of repeat radiographs by 50% and are also twice as fast
multiple operatories. A few dental clinics prefer the a ceil- in taking radiographs compared to the conventional den-
ing mounted version of the X-ray unit. tal X-ray units.
The ideal location of the X-ray unit (Figure 7) is on Another recent development in the dental X-ray sys-
the wall right behind the patient’s head (12 o’clock wall). tems is the use of direct current (DC) for X-ray generation.
Alternatively, mounting the lateral wall is also acceptable Until relatively recently, many of the dental X-ray genera-
as this allows the X-ray unit to be shared between two tors applied alternating current (AC) to the tube when
treatment rooms via a lead-lined pass through cabinet. generating X-rays. Constant potential generators produce
Over the last few years, portable handheld X-ray units a relatively constant stream of radiation and a greater per-
have gained popularity. These are battery operated (usu- centage of higher energy ‘useful’ radiation.
ally rechargeable Ni–Cd), weighing 5–9 pounds (Figure 8). With an AC generator, voltage across the tube goes
Since these handheld devices can be taken from room to from zero up to the maximum kilovolt peak (kVp), then
690
back to zero. This produces X-ray photons of varying lower contrast conventional films compared to AC units
energies. The lowest energy photons are filtered out, but at any given kVp, but these units typically operate at a
the average photon energy produced by an AC tube for a slightly lower kV than AC units, which decreases this dif-
given kVp is still lower than the average photon energy ference. Constant potential units may reduce patient expo-
produced by a constant potential tube at that same kV. sure slightly and may produce more consistent exposures
Lower energy photons are more readily absorbed by the at the very short exposure times associated with digital
patient, so the more homogeneous beam of higher energy radiography.
photons produced by constant potential units may slightly
reduce patient exposure.
Components of an Intraoral X-ray Machine
When using conventional film, the lower average pho-
ton energy of an alternating current unit will produce The intraoral X-ray machine is made up of the following
films of higher contrast than a constant potential unit (for parts:
a given kVp). However, constant potential units typically
1. X-ray tube head (Figure 9A, B)
operate at 60 or 65 kV compared to the 70 kVp of an alter-
2. Control panel
nating unit which brings the contrast levels closer to each
3. Swivel arms for maneuverability.
other. While most X-ray units operate at a single, fixed kVp,
some models have a facility to vary the kVp settings.
X-ray tube head
In summary, AC and DC units are both capable of pro-
ducing diagnostic images whether using conventional film It is made up of an evacuated (vacuumed) borosilicate
or digital radiography. Constant potential units (DC) produce glass envelope, which encloses the anode and cathode,
power supply circuit, protective lead housing and insulat-
ing (coolant) oil between the glass envelope and the outer
Figure 8 protective housing (Figure 10).
Glass envelope (Figure 11) It is made of borosilicate
glass that is evacuated. The glass tube is evacuated in
order to:
❍ Prevent collision of the electrons with gas molecules,
which might reduce their speed.
❍ Prevent oxidation ‘burn out’ of the tungsten filament.
Anode (Figure 12) The anode is positively charged. It

comprises of a tungsten target that is embedded in a cop-
per stem.
Tungsten Tungsten is chosen as an ideal target material
because of the following reasons:
❍ It has a high atomic number (74)—very efficient for
Portable handheld X-ray unit
production of X-rays.
Figure 9
A B
(A) X-ray tube head with a long cone. (B) X-ray tube with a short cone
691
Figure 10
4
5
3
6
10 8
11 7
2 12 13 9
14
15
1. Swivel arms
2. Yoke 10. Electron beam
3. External housing 11. Anode
17 12. Copper stem
4. Step down transformer
16 13. Tungsten target
5. Step up transformer
6. Borosilicate glass housing 14. Aluminum filter
7. Cathode 15. Collimator
8. Molybdenum focusing cup 16. Primary X-ray beam
9. Tungsten filament 17. Position indicating device
X-ray tube—schematic diagram. Courtesy: Dr Jaideep Shekhar
❍ Low vapor pressure—it helps in maintaining the vac-

Figure 11
uum in the tube at high temperatures during the oper-
ation of the machine.
The tungsten target is angulated at about 20⬚ to the central
ray of the X-ray beam.
Copper stem The tungsten target is embedded in a cop-
per stem. In order to compensate for the poor thermal con-
ductivity of tungsten, the target is embedded in a copper
stem, which is a good conductor of heat. It carries away
the heat from the tungsten target thereby preventing tung-
sten from melting at high operating temperatures.
Cathode The cathode is negatively charged. It comprises
of a filament and focusing cup.
Filament The filament is made up of tungsten. The tungsten
Borosilicate glass tube housing the anode and cathode filament is in the shape of a coil, which is about 1 cm in
length, and the coil of wire used is about 2 mm in diameter.
The coil is mounted on supporting rods that carry the power
❍ High melting point (3360⬚C)—it is a known fact that supply to the filament. To produce X-rays, the filament is
almost 99% of the kinetic energy of electrons is con- heated to incandescence using current from a low voltage
verted to heat, therefore making it necessary for the source. The tungsten filament in turn gives out electrons at
target to withstand high temperatures. a rate that is proportional to the temperature of the filament.
692
Figure 12 Figure 13
Anode and cathode of the X-ray tube head Control panel of an intraoral radiographic unit
Focusing cup The filament lies in the focusing cup. The Working mechanism of the X-ray machine
focusing cup is made up of molybdenum. It is a negatively
To ensure a sufficient rate of electron emission, the tung-
charged concave reflector, which electrostatically focuses
sten filament should be pre-heated to an optimum operat-
the electrons into a narrow beam that is directed toward
ing temperature. The timer circuit initially sends a current
the focal spot.
through the filament for about half a second to heat
Power supply circuit The power supply circuit of the X-ray the filament and bring it to an optimum operating tem-
tube head mainly consists of a step down transformer, perature. Once the filament is heated to an optimum tem-
high voltage transformer and electrical insulating oil sur- perature, the timer applies power to the high voltage
rounding the transformers. The transformers are encased circuit.
in an electrically grounded metal housing. Other parts of However, it is not practical to keep the filament pre-
the power supply circuit are a filament current (mA) con- heated continuously as it shortens the lifespan of the
trol switch and a kilovolts peak (kVp) selector dial. tungsten filament. Accordingly, it is not advisable to leave
Step down transformer It helps to reduce the voltage of the X-ray machine switched on all through the working
the incoming alternating current to about 10 volts. This is hours.
controlled by the mA switch. The step down transformer
Dissipation of heat from the X-ray machine It is under-
provides a low voltage current to heat the filament of the
stood that 99% of the electrons emitted, contribute to the
X-ray tube.
heat generated within the X-ray machine and only 1%
High voltage transformer It helps in generating a high contribute to X-ray production.
potential difference between the anode and cathode, thereby Various methods are employed to dissipate heat from
accelerating the electrons from the cathode toward the the X-ray machine:
anode to generate X-rays.
1. The X-ray machine should be operated in an air
Electrical insulating oil This oil acts as a coolant as well conditioned environment with the temperature about
as an electrical insulator. The insulating oil aids in dissipa- 18—20⬚C.
tion of heat produced by the X-ray tube. 2. Presence of an insulating oil or coolant between the
X-ray tube and the outer protective housing.
Control panel (Figure 13)
3. Embedding the tungsten target in a copper stem.
Most of the intraoral machines available have the facility to Copper being a good conductor of heat carries away
adjust the tube voltage and tube current. However, most of the heat from the target.
the machines operate at fixed parameters. X-ray machines 4. Angulating the target to about 20⬚ helps in minimiz-
used for taking intraoral radiographs are calibrated at ing the heat generated at the focal spot.
about 60–65 kVp and 8 mA. 5. Using a rotating anode minimizes the heat generated
The exposure time is usually one parameter that the during the production of X-rays. A rotating anode
dentist can control. The exposure time in most machines provides a large surface area at the focal spot thereby
can be adjusted from 0.1 to 3 seconds. aiding in the dissipation of heat.
693
Figure 14 components of the X-ray tube such as the borosilicate

glass envelope, insulating oil and the sealant that prevent
the leakage of the insulating oil, by way of design prevent
low energy X-ray photons to leave the X-ray tube hous-
ing. This inherent filtration is equivalent to 0.5–2 mm of
aluminum.
2. Added filtration Aluminum disks of various thick-
nesses as per the requirement are placed at the exit
portal.
3. Total filtration It is the sum of both the inherent filtra-
tion and added filtration. The amount of filtration required
is based on the tube voltage of the machine that is used.
The quantity of filtration required is expressed as ‘half
value layer’.
Photograph showing the aluminum filter at the far end Half value layer It is the thickness of aluminum required
of the PID to reduce by half the number of X-ray photons passing
through it. For a machine operating between 30–70 kVp,
total filtration should be about 1.5 mm and a machine
operating at 90 kVp the total filtration required is about
Filters and Collimators 2.5 mm of aluminum.
X-rays produced in the X-ray tube exit the glass envelope
through the exit port. Before the X-rays reach the object Along with aluminum filters, rare earth materials like
to be imaged they pass through the filter and then the erbium, yttrium and samarium have been used as fil-
X-ray beam is collimated and directed at the area of inter- ters. These filters used in conjunction with aluminum
est using the position indicating device (PID). filters further reduce the patient exposure. However, the
exposure time has to be increased by almost 50% and
Filters the contrast, resolution and sharpness of the resultant
image is reduced.
X-ray photons produced in the X-ray tube consists of
photons of various energy levels. It is a known fact that
photons of the maximum energy contribute to the produc- Collimators
tion of the latent image on an image receptor system film Collimator is a metallic barrier with a central aperture,
and photons of low energy contribute to exposure of the which is used to contain the size of the X-ray beam to the
patient. Studies have shown that the surface exposure lev- required site of exposure. The collimator is generally made
els reduce to about 20% when the X-ray beam is filtered. up of a thick plate of lead (radiopaque substance). It is
Photons of low energy levels have a low penetrating desirable that the collimated beam should be contained
power and hence do not aid in the formation of an image within a circle having a diameter of 7 cm or 2¾ inches.
of diagnostic quality. It becomes necessary to prevent
photons of low energy and low penetrating power from Functions of the collimator
reaching the patient. This is achieved by using a filter at 1. Reduces patient exposure by containing the size of
the exit port of an X-ray tube (Figure 14). the X-ray beam to the site that has to be exposed.
2. Prevents scattered radiation from reaching the film,
Design: The filter is made up an aluminum disc. It selec- that add to film fog and thereby degrading the diag-
tively allows the passage of high-energy X-ray photons nostic quality of the radiographic image.
and prevents the low energy X-ray photons from leaving
the X-ray tube. The aluminum disk is about 0.1 mm in Types of collimators Based on the shape collimators can
thickness. be classified as:
Types of filtration 1. Rectangular collimators
1. Inherent 2. Diaphragm collimators (Figure 15)
2. Added 3. Tubular collimators.
3. Total.
A rectangular collimator (Figure 16) reduces the skin sur-
1. Inherent filtration As the name suggests it is the fil- face exposure by almost 60% compared to that of a dia-
tration offered by the design of the X-ray tube. Various phragm collimator.
694
Figure 15 Figure 17
Coherent scatter
Compton scatter
Photoelectric
absorption
Interaction of X-rays with matter
Photograph of a diaphragm collimator with the Figure 18

central aperture
Figure 16
Coherent scatter. Courtesy: Dr Jaideep Shekhar
Scattering
Coherent scatter
Coherent scattering is also referred to as classic scattering.
It accounts for only about 8% of the total interactions of
X-rays with matter, therefore has a very small role in pro-
ducing film fog.
Rectangular collimator When a low energy X-ray photon passes near an atom’s
outer electron, it may not be absorbed but scattered with-
out loss of energy. The incident photon interacts with the
electron by causing it to vibrate momentarily at the same
INTERACTION OF X-RAYS WITH MATTER frequency as the incoming photon. Following this interac-
tion, the incident photon ceases to exist (Figure 18).
The X-ray beams that exit out of the X-ray tube interact The vibration causes the electron to radiate energy in
with matter that they encounter. Such encounters result in the form of another X-ray photon with the same frequency
absorption of scattering of X-ray photons. It is believed and energy as in incident photon. This secondary photon is
that almost 9% of the X-ray photons pass through matter emitted at an angle to the path of the incident X-ray photon.
without any interaction.
Compton scatter
Types of Interaction (Figure 17) Compton scatter accounts for about 62% of the interac-
tions of X-rays with matter. It occurs when a X-ray pho-
Scattering
ton interacts with an outer electron. The incident photon
❍ Coherent scatter
collides with the electron, which receives kinetic energy
❍ Compton scatter
and recoils from the point of impact. The incident photon
Absorption is deflected by its interaction and scattered from the site of
❍ Photoelectric effect. collision (Figure 19).
695
Figure 19 Figure 20
Compton scatter. Courtesy: Dr Jaideep Shekhar Photoelectric absorption. Courtesy: Dr Jaideep Shekhar
Energy of the scattered photon equals energy of the

incident photon minus the kinetic energy gained by the by the absorption of X-rays by matter. It occurs when
recoil electron plus its binding energy. Compton effect incident photon collides with a bound electron in an
results in loss of the electron, thus ionization of absorbing atom. During this interaction, the photon ceases to exist
atom. The scattered photons travel in any direction. On (Figure 20).
reaching the film, they cause film fog. The electron is ejected from its shell and becomes a
recoil electron (photoelectron). The kinetic energy of recoil
electron is equal to the energy of photon minus that required
Absorption to overcome the electron binding energy.
Photoelectric effect results in electron deficiency, which
Photoelectric effect
is instantly filled by an electron from outer orbit with
Photoelectric effect accounts for about 30% of all the the release of characteristic radiation, which is absorbed
interactions of X-rays with matter. It is characterized within the object.
696
CHAPTER
Radiation Biology
Shubhasini AR, Bhanushree R, Praveen BN 26
➧ Effects on Living Systems ➧ Dose and Risk in Radiography
➧ Molecular and Cellular Radiobiology Patient Exposure and Dose
➧ Deterministic and Stochastic Effects Reducing Dental Exposure
Deterministic Effects on Tissues and Conducting the Examination
Organs Protecting Personnel
Effects of Total Body Radiation X-ray Equipment Installation and Related Factors
Effects of Radiation on Oral Tissues Shielding
Radiation in the CT Suite
Radiation Safety and Protection ➧ Radiation Detection and Measurement
➧ Sources of Radiation Personnel Dosimetry
Natural Radiation Wearing the Dosimeter
Man-made Radiation ➧ Film Exposure and Processing
X-rays were discovered in 1895, and since then, these rays EFFECTS ON LIVING SYSTEMS
were applied in several fields such as physics, chemistry
and biology. In the beginning, people using these rays Living tissues may be affected by radiation in two ways—
were not aware of the biological effect of X-rays. X-ray directly by the action on cellular molecules and indirectly
tubes were calibrated based on the erythema produced on by the action on water. While direct effects account for
skin when the operator placed his hand in front of the one-third of damages, indirect effects account for the
X-ray beam. There were numerous episodes of injury to remaining two-thirds.
operators and patients. The first biological effects of X-rays
were reported in 1896 and included skin burns, epilation Direct effect X-rays interact with biological molecules
and eye irritation. In 1906, two French radiobiologists, causing excitation and ionization. This results in the
Bergonie and Tribondeau described a law which states that breakage of chemical bonds in these molecules and results
‘the radiosensitivity of a cell is directly proportional to its in the formation of free radicals.
reproductive activity and inversely proportional to its These reactions may be represented as follows:
degree of differentiation’.
X-ray photon ⫹ RH → R* ⫹ H⫹ ⫹ e⫺,
X-rays interact with atoms and molecules in the body
within pico to femto second (10⫺13 to 10⫺15 s) of exposure. where RH represents a biological molecule such as a car-
This reaction may occur directly on biological molecules bohydrate and R* is a free radical formed after the loss of
or indirectly through the action on water molecules. This an electron e⫺. Both R* and H⫹ immediately react with other
results in the formation of free radicals. Free radicals are biological molecules either by dissociation:
fragments of molecules which have unpaired electrons, and
hence high reactivity. They react with surrounding mole- R* → X ⫹ Y*
cules and become stable by cross-linking or dissociation. or cross-linking:
Thus, there is an alteration of cellular molecules which
further results in biological effects. R* ⫹ S* → RS
697
The altered molecules differ structurally and functionally structure remains unaffected. Exposure of enzymes, how-
from the original molecules resulting in a biological change ever, has a cascading effect, since altered enzymes may not
in the irradiated tissues. perform their physiological actions, resulting in intracellu-
lar molecular alterations. These changes occur at radiation
Indirect effect Water is the most abundant molecule in
doses much higher than that which causes cell death.
living tissues and hence X-rays passing through the body
The nucleus is more often affected due to radiation, but
readily encounter water molecules. A series of complex
changes can occur in mitochondria on exposure to higher
chemical reactions then occur in water and finally result
doses of radiation. Mitochondria become swollen with dis-
in the formation of highly reactive molecules. This is
organization of internal cisternae.
termed radiolysis of water.
In the first step, X-ray photons displace an electron result-
ing in the formation of positively charged water molecule. Radiation Effects in DNA
X-ray photon ⫹ H2O → H2O⫹ ⫹ e⫺ Radiation causes a wide range of lesions in DNA such as:
This positively charged water molecule reacts with another ❍ Single strand breaks
water molecule, thus: ❍ Double strand breaks
❍ Base damage
H2O⫹ ⫹ H2O → H3O⫹ ⫹ OH*
❍ Protein–DNA crosslinks
The free electron produced in the first reaction combines ❍ Protein–protein crosslinks involving nuclear proteins
with a water molecule: such as histones and non-histone proteins.
e⫺ ⫹ H2O → H2O⫺ → OH⫺ ⫹ H* Most base damages, protein–DNA crosslinks and protein–
protein crosslinks are usually minor damages which can be
The electron may also be dissolved in the solution to form
repaired and probably play a minor role in carcinogenesis.
aqueous electron, e⫺aq.
Single strand breaks are characterized by damage to a sin-
e⫺ → e⫺aq gle strand of the helix of DNA and are repaired easily using
X-ray photons may act directly on water molecules to the intact strand as a template. However, double strand
produce electronically excited water molecules, which in breaks, characterized by damage to both strands at the
turn breakdown to hydroxyl and hydrogen radicals: same site or in close proximity, are more difficult to repair.
Thus, they frequently result in cell death. Fortunately, these
X-ray photon ⫹ H2O → H2O* → OH* ⫹ H* are less common in occurrence. An exposure of 1–2 Gy to
The end result of the radiolysis of water is the production a cell produces approximately more than 1,000 base dam-
of OH*, H*, and e⫺aq. All these are highly reactive radicals ages, about 1,000 single strand breaks and only about 40
and react readily with cellular molecules such as DNA and double strand breaks.
lipids.
Cell cycle effects
RH ⫹ OH* → R* ⫹ H2O
Dividing cells participate in cell division which, although a
RH ⫹ H* → R* ⫹ H2
continuous process, can be described in the following
The hydrogen free radical combines with dissolved oxygen phases:
to form hydroperoxyl free radicals:
❍ First Gap (G1) phase or phase characterized by division
H* ⫹ O2 → HO2* of synthesis of organelles
These hydroperoxyl free radicals form hydrogen peroxide: ❍ Synthetic (S) phase characterized by division of DNA
❍ Second Gap (G2) phase characterized by preparation
HO2* ⫹ H* → H2O2 for mitosis
HO2* ⫹ HO2* → H2O2 ⫹ O2 ❍ Mitotic (M) phase in which the actual cell division occurs.
These hydroperoxyl free radicals and hydrogen peroxide Cells not undergoing division remain in G0 phase. Typical
react with biological molecules, alter them and cause cell cell division times are 10–40 hours with the G1 phase taking
destruction. about 30%, S phase 50%, G2 phase 15% and M phase 5% of
the cell cycle time. There are checkpoints at the G1/S and
G2/M boundaries that monitor the accuracy of cell division.
MOLECULAR AND CELLULAR Radiosensitivity differs throughout the cell cycle with
RADIOBIOLOGY late S phase being most radioresistant, G2/M being most
radiosensitive and G1 phase taking an intermediate posi-
Exposure of proteins to radiation leads to changes in their tion. In G2/M phase, chromatin is tightly compacted and a
secondary and tertiary structures although the primary poor repair capacity. This explains the high radiosensitivity
698
Chapter 26 – Radiation Biology
of this period. DNA synthesis is almost complete by late mucous membrane are all cells which divide regularly and
S phase. Damage by X-rays at this phase can be repaired, do not undergo differentiation. These cells are extremely
and thus the cell is radioresistant at this phase. DNA has an radiosensitive.
open structure in G1 phase having a better repair capacity;
2. Differentiating intermitotic cells Intermediate pre-
this makes G1 phase radioresistant.
cursors of blood cells, spermatocytes and oocytes, dividing
cells in inner enamel epithelium are cells that divide less
Bystander effect
frequently and undergo some differentiation. These cells
Cells close to irradiated cells but not themselves exposed are less radiosensitive than the first group.
to radiation may also exhibit DNA damage and reduced sur-
3. Multipotential cells Connective tissue cells such as
vival. This phenomenon is termed bystander effect. This
fibroblasts, vascular endothelial cells, and mesenchymal
may occur due to the damaging signals communicated by
cells are cells that divide when there is a need for more
irradiated cells through gap junctions, or through damag-
cells. These cells have an intermediate radiosensitivity.
ing molecules released into the surrounding medium.
4. Reverting post mitotic cells Cells such as acinar and
ductal cells of salivary glands, and parenchymal cells of
DETERMINISTIC AND STOCHASTIC EFFECTS liver, kidney and thyroid are cells which are differentiated
and specialized in their function. These are divided infre-
Radiation exposure can lead to many harmful health effects. quently and hence are usually radioresistant.
Such effects were classified by International Commission on 5. Fixed post mitotic cells Cells such as neurons, striated
Radiological Protection (ICRP) in 1990 into deterministic muscle cells, epithelial cells close to the surface and eryth-
and stochastic effects. rocytes are cells which are highly differentiated and are
Deterministic effects Deterministic effects also called incapable of division. These cells are most resistant to
‘tissue reactions’, refer to those effects in which the sever- radiation.
ity of response is proportional to the dose. These effects,
usually cell killing, occur in all people when the dose is Effects of radiation on tissues
large enough. Deterministic effects have a dose threshold The effects of radiation treatment on normal tissues have
below which the response is not seen. Examples of deter- been divided into the following:
ministic effects include oral changes after radiation therapy.
❍ Early (or acute) response—when clinical symptoms are
Stochastic effects Stochastic effects also called ‘cancer/ seen within a few weeks of radiation treatment. This
heritable effects’, refer to those effects for which the prob- response occurs in more radiosensitive tissues.
ability of the occurrence of a change, rather than its sever- ❍ Late response—when clinical symptoms take many
ity, is dose dependent. Stochastic effects are all-or-none, months or years to develop. It occurs in organs where
i.e. a person either has or does not have the condition. For parenchymal cells divide rarely.
example, radiation-induced cancer is a stochastic effect
because greater exposure of a person or population to radi-
ation increases the probability of cancer but not its severity. Effects of Total Body Radiation
Stochastic effects are believed not to have dose thresholds. Acute radiation syndrome
ICRP introduced another term ‘detriment’ to measure the
harmful health effects of low-dose radiation to individuals It refers to the various effects on the health of an individ-
and their offsprings. The detriment in a population is defined ual exposed to high doses of radiation. These effects pres-
as the mathematical expectation of the induction of cancer ent within 24 hours of irradiation and may last for months.
and hereditary damage caused by an exposure to radiation. These have been classified into gastrointestinal, hemato-
Detriment is a complex concept combining the probability, poietic and neural/vascular presentations.
severity and time of expression of radiation harm. Prodromal symptoms of total body radiation include
nausea, vomiting, fever, headache, fatigue and a brief skin
erythema. These changes occur on exposure to minimum
Deterministic Effects on Tissues and Organs dose of 1.5 Gy.
Cellular and tissue response Hematopoietic syndrome It occurs after exposure to
2–7 Gy of radiation. It occurs due to the exposure to active
Based on the law of Bergonie and Tribondeau, cells may be
hematopoietic areas such as sternum and pelvis. This results
classified into five groups based on their radiosensitivity.
in destruction of the blood cell precursors, which in turn
1. Vegetative intermitotic cells Early precursor cells of leads to lower levels of peripheral blood cells. Circulating
blood cells and spermatogenic cells, and basal cells of oral blood cells themselves are not affected by radiation.
699
Granulocytes which have a short lifespan, are not replaced non-threshold hypothesis, according to which, at low doses
by maturation of precursor cells. This predisposes to infec- and dose rates, total radiation-related cancer risk is propor-
tions. As thrombocytes disappear in peripheral blood, bleed- tional to dose. The mechanism of carcinogenesis is thought
ing ensues. As erythrocytes, which have a long lifespan, to be due to gene mutations. These may not be repaired
disappear, anemia occurs. and the unrepaired genes may be transmitted to daughter
cells, which may lead to the development of cancer. It has
Gastrointestinal syndrome It occurs after an exposure
been suggested that radiation acts as both an initiator and
of about 7–15 Gy. Exposure of the stomach and intestines
a promoter of carcinogenesis.
to radiation results in injury to the rapidly dividing basal
epithelial cells. This results in ulceration of the mucosa,
resulting in loss of plasma and electrolytes, bleeding, and Effects of Radiation on Oral Tissues
diarrhea. The normal microbial flora invades the mucosa
One of the modalities used in the treatment of cancer is
resulting in septicemia. The combined effects of gastroin-
radiotherapy. Irradiation of a high dose given to patients suf-
testinal and hematopoietic syndromes result in death within
fering from cancer in the orofacial region invariably exposes
2 weeks.
the oral tissues. The source of radiation is usually gamma
Neurovascular syndrome It occurs after exposure to rays from external source, while brachytherapy with inserted
more than 50 Gy of radiation. It is uniformly fatal and radon or iodine-125 implants may occasionally be used.
death occurs in 1 or 2 days. It occurs due to extensive The dose of radiation is about 50 Gy, given in divided doses
injury to the central nervous system and the vascular sys- of 2 Gy/day.
tem. There may be dizziness, headache, incoordination, This results in several effects which are described
convulsions, and stupor. below.
Radiation effects in the developing embryo and Mucositis

fetus
The basal layer of the oral mucosa consists of dividing
Cells and tissues in embryos and fetuses are more radio- cells which are susceptible to radiation damage. Death of
sensitive than adult cells. In the early fetus, radiation these cells results in mucositis. Mucositis begins 12–17 days
exposure has an all-or-none effect, i.e. there is either spon- after initiation of radiotherapy.
taneous abortion or normal development. The most sensi- Based on severity, mucositis has been classified into
tive period for the occurrence of developmental anomalies four grades:
is between 18 and 45 days of gestation. The period of brain
development, from 8 to 15 weeks post conception is also Grade 1 Begins at the end of first week of radiotherapy.
a very sensitive period. Radiation exposure during this Characterized by the presence of white areas in the oral
period may result in microcephaly and mental retardation. mucosa due to hyperkeratosis. This stage is usually asymp-
Exposure after this period may result in general growth tomatic.
retardation and an increased risk for childhood cancer. Grade 2 Occurs around the end of second week of radio-
However, all these changes occur after exposure to radiation therapy. Small areas of ulceration are noted. Patient can
levels of about 1 Gy, whereas full mouth dental radiography take a soft diet.
using a lead apron results in an exposure of just 0.25 ␮Gy.
Grade 3 Severe mucositis characterized by large ulcers
Radiation-induced heritable diseases covered by pseudomembrane. Patient has pain and diffi-
culty in eating and can tolerate only liquid diet.
Radiation exposure to germ cells may result in chromo-
somal alterations which are heritable. The major genetic Grade 4 Even more severe mucositis characterized large
effect of radiation is due to microdeletions, which refer to areas of ulceration covering almost the entire oral mucosa.
the deletion of multiple, functionally unrelated but contigu- Patient can consume only liquids, or may need nasogastric
ous genes. These effects do not cause death of the indi- intubation.
vidual but result in several malformations such as mental Mucositis continues up to the completion of radiother-
and physical retardation and various malformations. These apy. After this, the mucosa heals and healing is complete
diseases are rare. It has been estimated that the risk of these by 2 months. Later, the mucosa tends to become atrophic.
multi-organ congenital disorders after exposure to 1 Gy is
approximately 0.1%. Xerostomia
Salivary glands are frequently in the path of radiation
Mechanism of radiation-induced cancer
and are exposed during radiotherapy. The parenchyma of
Exposure to ionizing radiation is an established cancer risk these glands is radiosensitive, which finally results in a
factor. Cancer risk has been described based on the linear fibrosis of the gland. This results in a progressive decrease
700
in salivary secretion, termed xerostomia. This decrease is radiation and have adequate knowledge regarding the
dose dependent and secretion reaches zero at about 60 Gy. methods to minimize unnecessary exposure to radiation.
Patients complain of dry mouth and difficulty in swallow- This section will attempt to highlight various measures to
ing. As lubricating properties of saliva are lost and its pH protect the patient, radiation personnel and public from
decreases, demineralization of enamel begins. the long-term hazards of diagnostic radiology.
Teeth Mature teeth are not affected by radiation, how-
ever, radiation doses as low as 400 cGy may retard the
development of incompletely formed teeth. Irradiation to Historical Events in Radiation Safety and
developing teeth may result in short roots, incomplete cal- Protection
cification, dilaceration, and delayed or arrested eruption. ❍ X-ray induced case of severe dermatitis was pub-
Radiation caries It is a form of rampant caries that occurs lished in July 1896. The first dose limit of 10 rad
in individuals exposed to curative radiation. Radiation per day (or 3,000 rad per year) was recommended in
causes a change in saliva by decreasing its secretion, low- 1902 based on fogging observed on a photographic
ering its pH, and reducing its buffering capacity. This plate.
reduces the cleansing action of saliva on teeth and results ❍ The destructive effect of X-rays on living tissues,
in accumulation of debris, demineralization, and finally, such as skin, blood forming-organs and reproduc-
rampant caries. tive organs was first evident by the animals studies
Three types of radiation caries have been described: performed in 1903.
Type 1: The most common type. It is characterized by car- ❍ In 1928, the first formal radiation unit—the roentgen,
ies occurring around the teeth in the cervical was adopted. In 1953 and 1954, the bone marrow
region. As caries deepens, there is amputation of dose limit of 300 millirem (mrem) per week and skin
the crown. dose limit of 600 mrem per week was adopted by
Type 2: It is characterized by the initiation of caries on all ICRP and NCRP (National Council on Radiation
crown surfaces, eroding away the entire coronal Protection and Measurements), respectively.
structure. ❍ An annual occupational dose limit of 5 rem per year
Type 3: The least common type. It is characterized by color was recommended by ICRP in 1957. A lifetime occu-
changes in dentin resulting in a diffuse blacken- pational dose limit of 235 rem for individuals in the
ing or dark brown discoloration of the crown. age group between 18 to 65 years and an annual
dose limit of 500 mrem per year to public was rec-
Osteoradionecrosis ommended by NCRP in 1958. The three parts of
dose control—justification, optimization and limita-
It refers to the secondary infection of irradiated bone. tion was put forth by ICRP, in 1961 (Table 3).
Radiation exposure to bone affects the vascularity of bone, ❍ The ALARA (As Low As Reasonably Achievable)
destroys osteoblasts and some osteoclasts. The normal guidelines for radiologic protection for radiation
vascular bone marrow is converted to fatty and fibrous personnel, the maximum annual radiation dose limit
marrow. Thus, the marrow tissue becomes hypoxic, hypo- of 5 rem per year was recommended.
vascular and hypocellular. ❍ In 1980, ICRP limited the radiation exposure to 10 rem
If secondary infection is then superimposed on irradi- over any 5-year period and 5 rem in any 1 year. The
ated bone, the bone readily undergoes necrosis. Infection public limiting dose as 100 mrem per year averaged
may occur from sequelae of caries, periodontitis, denture- over any 5-year period.
sore or a tooth extraction. Osteonecrosis is more common
in mandible than the maxilla, presumably because of the
rich vascular supply of maxilla, and also since the man-
dible is irradiated more frequently. SOURCES OF RADIATION (Table 1, Figure 1)
Thus, before the initiation of radiotherapy, the dental
status of a patient should be assessed. Any pre-existing
Radiation is the transmission of energy through space and
disease such as caries, periodontal disease, third molar
matter. It is natural and part of our lives. The radioactive
pathology, defective restoration, etc. should be treated.
materials present naturally in the earth’s crust can be
encountered in the building construction materials, food
substances and the air we breathe. Muscles, bones, and
RADIATION SAFETY AND PROTECTION tissues of our own bodies contain naturally occurring
radioactive elements. It is estimated that about four-fifths
It is imperative that dental professionals as well as the of the average annual radiation dose worldwide is the
public are aware of the potential hazards of the ionizing contribution from natural radiation.
701
Table 1 Average effective dose of ionizing radiation from Cosmic sources

different sources It is estimated that cosmic radiation accounts for about
Source Dose (␮Sv) 8% of the annual total body exposure.
In different parts of the world the cosmic radiation varies
Natural
in dosage due to difference in altitude (radiation doubles for
Cosmic 0.4
every 6,000 feet) and magnetic field. The sources of cosmic
Terrestrial
0.5 radiation are protons, electrons, X-rays and gamma rays.
External
Radon 1.2
Other 0.3 Terrestrial sources
2.4
Total Terrestrial sources originate from both external and internal
Man-made sources; external sources being soil and internal sources,
Medical (estimated) including radon and other nuclides that are inhaled or
Diagnostic X-ray 2 ingested. Radon contributes 1.2 mSv to natural radiation
Nuclear medicine 0.5 and the average whole body radiation from it is 228 mrem
Other consumer products 0.08 per year. Potassium-40 and carbon-14 are two of the main
Other enlisted internal sources of radiation. The entrapment of
0.01
Professional radioactive materials by the body’s own tissues through
0.01
Fallout the soil, water and air constitute about 11% of our total
0.01
Nuclear fuel cycle annual exposure.
0.01
Dental radiology
2.5
Total
Man-made Radiation
Exposure to man-made radiation increases depending on
its usage; place of residence, lifestyle, pattern of house
Figure 1 construction, kind of traveling and smoking also affects
radiation exposure. People residing in the coastal regions
Consumer
Nuclear
products
are more protected than airline crew due to the thick cover
medicine of earth’s atmosphere.
3% Other
4%
1%
Cosmic rays Medical and dental diagnosis and treatment
8%
Terrestrial Medical and dental X-rays account for an average of about
8% 11% of our total annual exposure. But dental X-ray exam-
Sources of radiation inations account for less than 1% of the average annual
Internal exposure radon exposure from man-made exposures. Annually worldwide,
sources 54% over one billion medical examinations and 300 million den-
11% Medical X-rays tal examinations are performed which include radiation
11%
therapy, nuclear medicine and diagnostic medical exposure.
The average dose received from all medical exposures is
300 mrem which represents six-fold increase in average
medical exposures over the past 25 years. Half of the average
Sources of radiation exposure background medical exposure (147 mrem) is from computed
tomography (CT) examinations, a relatively new technology
that has significantly improved diagnostic imaging.
For a professional medical or dental radiologist, technol-
Natural Radiation ogist or assistant, the occupational dose limit is 5,000 mrem
per year. For the non-medical or dental public, the dose
The natural background radiations are from three sources— limit is 100 mrem per year.
cosmic radiation, terrestrial radiation and internal radiation. Although the risk involved in dental radiography is
The annual effective dose of radiation from cosmic and very less, it becomes imperative for the practitioner to avoid
terrestrial sources is about 2.4 millisieverts (mSv) world- even the smallest unnecessary dose of radiation in work-
wide. The average whole body radiation to an individual ing area. For this, developing basic concepts on radiation
from background radiation is estimated to be around units and measurements and applying the same for safety
620 mrem. and protection measurement is indispensable (Table 2).
702
Table 2 Radiation unit conversion factors Dose limits
Radiation units of measurements

The regulatory bodies at the international and national
levels, namely, ICRP, its counterpart the NCRP in the United
Unit of measure Conversion equivalent States, and the Atomic Energy Regulatory Board (AERB) in
1 curie 3.7 ⫻ 1010 disintegrations/second India have laid down recommendations for radiation safety
and protection to humans without limiting their beneficial
1 becquerel 1 disintegration/second
application. In spite of low dosage in dental radiography,
1 millicurie (mCi) 37 megabecquerel (MBq) radiation should be minimized wherever practicable. The
1 rad 0.01 gray (Gy) efficacy of radiology department lies in wise choice of
1 rem 0.01 sievert (Sv) investigative procedures which aims at reduced radiation
exposure and diagnostic accuracy.
1 roentgen (R) 0.000258 coulomb/kilogram (C/kg)
1 megabecquerel (MBq) 0.027 millicurie (mCi)
1 gray (Gy) 100 rad Patient Exposure and Dose
1 joule/kilogram
Risk estimates
1 sievert (Sv) 100 rem
1 Gy ⫻ Wr Man-made as well as naturally occurring radiation can
1 coulomb/kilogram (C/kg) 3,880 roentgen result in birth defects. Six out of one hundred live-births
can present birth defects from natural radiation. This fact
may be explained with the concept that the fetus which
For diagnostic and therapeutic purposes of a patient, has a rapid rate of development and a yet to be formed
NO LIMIT has been set for the exposure. immune mechanism is vulnerable to hazardous chemicals
and infections.
Consumer and industrial products The severity of the complication exhibited by the fetus
depends on the radiation dose as well as the stage of growth
Consumer products account for 3% of annual exposure (particularly, specific organ defects) at the time of expo-
(0.10 mSv). To name a few—smoke detectors, color televi- sure. Severe birth defects including mental and physical
sion, domestic water supply, tobacco products, combusti- growth retardation can occur if a pregnant lady is exposed
ble fuel, dental porcelain, pocket watches are all included to a radiation dose higher than 100 rem.
in this group. The ignorance period of radiation exposure to birth
defect will be between 8–12 weeks of gestation period, the
Other man-made sources time matching with the non-conformity with pregnancy.
Workers in mining, milling, nuclear plants including Small head size can result if the radiation received in the
weapons are occupationally exposed to radiation. Among womb is greater than 10 rad. If a dose of 25 rad is received
these sources strontium-90 and iodine-131 are important. after 8 weeks of gestation period, mental retardation along
Strontium-90, a beta emitter gets easily assimilated in bones with a small head size is seen.
and teeth of children and young adults due to its chemical
For public The whole body’s maximum permissible dose
similarity to calcium. Iodine-131, a gamma emitter gets
is 100 mrem (1 mSv) in a year or the average dose for
accumulated in the thyroid gland. Nuclear power contrib-
5-year period should not exceed 100 mrem (1 mSv).
utes 0.01 mSv annual exposure.
For occupational exposure The whole body’s maximum
permissible dose is 2 rem (20 mSv) and over 5 years should
DOSE AND RISK IN RADIOGRAPHY not exceed 100 mSv. The effective dose should not exceed
30 mSv in any single year.
The goal of health physics is prevention of deterministic
effects and reduction of stochastic effects by minimizing
Reducing Dental Exposure
the exposure of radiation workers and patients during
radiographic examinations. This is usually performed by ICRP is a non-governmental, independent organization,
the government agency, regulatory bodies, advisory bod- a registered charity, created in 1928 by International
ies or registered bodies by setting up regulations. This limits Congress of Radiology, the primary body in protection
the individuals who are occupationally exposed, patients against the unwise usage of ionizing radiation in different
and general population, from unnecessarily being exposed fraternity including medicine, dentistry, industry, nuclear
to radiation. This section deals with radiation dose, risk enterprise and also from naturally occurring radiation
and limitations in medical and dental radiography. sources.
703
The reports and recommendations of ICRP are published (American Dental Association) and FDA (Food and Drug
four times a year in the journal Annals of ICRP. The key Administration) for dental radiographic examination which
principles of ICRP are: include the following:
1. Principle of justification This means the situation 1. Consideration to be given to patient’s susceptibility to
dependent alteration of radiation exposure should be ben- dental caries and periodontal disease, growth and
eficial for the receiver. development stage and other unambiguous status.
2. Principle of optimization of protection This means to
follow ALARA principle for individual or mass radiation Dose limits
protection.
Application Dose limits
3. Principle of application of dose limit This implies for
Occupational Public
usage of radiation in different field area, the ICRP dose limit
recommendation should be followed with exception in med- Effective dose 20 mSv per year, 1 mSv in a year
ical fraternity and unexpected or urgent attention situations. averaged over a period
of 5 consecutive years
Patient selection criteria Annual equivalent dose

Lens of the eye 150 mSv 15 mSv
Clinical examination is a must irrespective of the kind of Skin 500 mSv 50 mSv
receptor and the imaging operating technique. Radiographic Hands and feet 500 mSv
exposure in an asymptomatic patient is non-evidence sup-
portive. But in a symptomatic patient, the images should
be limited to diagnosis and treatment plan in addition to Effective doses from conventional dental imaging techniques in Sv
adherence to published patient selection criteria by ADA Examination Effective dose (␮Sv)
Intraoral radiograph ⬍1.5
Annual allowable occupational dose
Panoramic radiograph 2.7–24.3
Part of the body Rem Sievert Cephalometric radiograph ⬍6
Whole body 5 0.05 Maxillomandibular MSCT 280–1,410
Individual organ or tissue other than 50 0.5 MSCT: multislice CT.
the lens of the eye
Lens of the eye 15 0.15
Skin 50 0.5 Typical doses from common diagnostic radiology procedures
Extremity 50 0.5 Examination Effective dose Equivalent period of
(mSv) natural background
radiation
Skull radiograph 0.18 2.6 weeks
Estimated loss of life expectancy from health risks
PA chest radiograph 0.029 3.4 days
Health risk Estimate of reduced life 8
Computed tomography: head 2 1 year
expectancy (average)
Computed tomography: chest 88 4 years
Cigarette smoking—20 per day 6 years 8
Barium meal 5 2.5 years
Overweight (15%) 2 years
PA: posteroanterior.
Alcohol ingestion 1 year
All kinds of accidents 1 year
Motor vehicle accidents 207 days Dose guidance levels for computed tomography for a typical
Home accidents 74 days adult patient
Drowning 24 days Guidance levels of dose for diagnostic radiography for a typical
Natural hazards (earthquake, lightning, 7 days adult patient
flood, etc.)
Examination Multiple scan average dose (mGy)
Medical radiation 6 days
Head 50
Occupational exposure
0.3 rem/yr from age 18 to 65 15 days Lumbar spine 35
1 rem/yr from age 18 to 65 51 days Abdomen 25
704
Effects resulting from acute whole body external exposure of radiation to men
0–25 r 25–100 r 100–200 r 200–300 r 300–600 r 600 or more

No detectable Slight transient Nausea and Nausea and vomiting Nausea, vomiting and diarrhea Nausea, vomiting and
clinical effects change in fatigue with on first day in first few hours diarrhea in first few
lymphocytes and possible vomiting hours
neutrophils above 125 r
Latent period up to Latent period with no definite Short latent period with
2 weeks or perhaps symptoms, perhaps as long as no definite symptoms in
longer 1 week cases during first week
Delayed effects Disabling sickness Reduction in Following latent period Epilation, loss of appetite, Diarrhea, hemorrhage,
may occur not common, lymphocytes and symptoms are mild: general malaise and fever, purpura, inflammation
exposed individuals neutrophils with loss of appetite, during second week followed of mouth and throat,
should be able to delayed recovery malaise, sore throat, by hemorrhage, purpura, fever toward the end of
proceed with usual pallor, petechiae, petechiae, inflammation of first week
duties diarrhea, moderate mouth and throat, diarrhea and
emaciation emaciation in the third week
Delayed effects Delayed effect Recovery in likely Some death in 2–6 weeks, Rapid emanciation and
possible, but may shorten life about three months, possible eventual death to death as early as the
serious effects on expectancy in the unless complicated by 50% of the exposed second week with
average individual order of 1% poor previous health, individuals for about possible eventual death
very improbable superimposed injuries 450 r of up to 100% of
or infections exposed individuals
r: Rads.
NCRP and ICRP radiation protection recommendations
Recommended annual limits for human exposure to ionizing radiation
Recommendation NCRP ICRP
Occupational dose limits

Relative to stochastic 50 mSv annual effective dose limit and (10 mSv) 50 mSv annual effective dose limit and 100 mSv in 5 years
effects (age [yr]) cumulative effective dose limit cumulative effective dose limit
Relative to deterministic 150 mSv annual equivalent dose limit to lens of eye 150 mSv equivalent dose limit to lens of eye and 500 mSv
effects and 500 mSv annual equivalent dose limit to skin and annual equivalent dose limit to skin and extremities
extremities
Non-occupational (public) dose limits
Relative to stochastic 5 mSv annual effective dose limit for infrequent 1 mSv annual effective dose limit and if higher, not to exceed
effects exposure and 1 mSv annual effective dose limit for annual average of 1 mSv over 5 years
continuous exposure
Relative to deterministic 50 mSv annual equivalent dose limit to lens of eye, 15 mSv annual equivalent dose limit to lens of eye and 50 mSv
effects skin and extremities annual equivalent dose limit to lens of eye, skin and extremities
Embryo/fetus 0.5 mSv equivalent dose limit per month after 2 mSv equivalent dose limit after the pregnancy has been
pregnancy is known declared
Negligible individual dose 0.01 mSv annual effective dose None established
2. Radiographs based on sound professional judgment 5. For a referred patient, the clinician’s first option should
on clinical diagnosis and treatment plan. be to obtain all the previous medical and dental records
3. To avoid routine dental radiographs for all patients at including radiographs from previous health care pro-
preset intervals. viders which help in comparing as well as future
4. Prescription of dental radiograph may be based on investigations and treatment protocols.
clinical suspicion of oral disease secondary to medical 6. For female patients under gestation period, it is best
or dental history. to adhere to FDA selection criteria guidelines. In these
705
patients, to prevent the transmission of diseases from 7. Filtration: Radiation absorbing filters, aluminum, the
mother to fetus through dental diseases and to diag- degree of attenuation should not be less than the half-
nose and manage the same, dental radiographs become value layer of the X-ray beam.
obligatory. 8. X-ray tube mechanical stability: Within the tube hous-
7. Patients under treatment for head and neck malignancy, ing, the X-ray tube should be aligned and precisely
no special consideration is necessary as the risk of devel- fixed by mechanical means without vibration, drift or
oping oral diseases is of high rate rather than the movement.
diagnostic usage of X-ray exposure for review patients. 9. X-ray tube protection: By enclosing within shielded
housing. The housing should be leakage proof and
should not exceed 0.87 mGy (100 mR) in 1 hour. The
Conducting the Examination
tube leakage is measured at a distance of 1 m in any
As the patient selection for radiographic examination is direction from focal spot.
justified, the choice and operation of the equipment, the 10. Position indicating device (PID): It must be open
radiographic technique, processing and interpretation of ended. Pointed cone or close ended applicators should
the image, will also have an impact on patient radiation not be used. It limits the minimum focal spot to skin
exposure. Conducting examination should be under pro- distance above 18 cm.
tection beneficiary for both patient and the operator. 11. Beam limiting devices: These are collimators at the end
of applicators, round or rectangular size with the size
Choice of the equipment of 7 cm or 38.5 cm2 for round and rectangular colli-
mators, respectively.
A boon to the modern dental health care is to have
qualitative diagnostic radiographs which are preliminarily
as a result of good X-ray equipment with certain basic Panoramic X-ray equipment
requirement. 1. Position-indicating device: should not be less than
1. Control panel requirements 15 cm.
a. Warning signals incorporation: (i) during unau- 2. Collimators: should be of scanning slit dimension or
thorized usage, (ii) during X-ray emission 2% of the focal spot to image receptor distance,
b. X-ray machine status indicators: should alert whichever is less.
(i) when energized to produce X-rays, (ii) when a 3. Cassette carrier: should be irradiation proof and inter-
single control panel is controlling multiple X-ray locked. Irradiation is possible only with film cassette
tubes, each X-ray tube should have a separate in the cassette carrier.
indicator during ready for usage and one usage 4. Timer: The maximum presettable irradiation time must
at a time by interlock option. be within 25 seconds, or the time required to deliver
2. Irradiation switch: Must be an intentional pressure oper- 250 milliampere-seconds, whichever is shorter.
ator. If cable mounted or wall mounted, the exposure
timer should be placed about 10–12 feet away from Cephalometric X-ray equipment
the X-ray tube. Collimator The central beam of X-rays should be fully
3. Exposure parameters indicators: Indicators like electrical blocked by the film cassette at the focal spot to film dis-
meters are advisable for adjustable X-ray tube volt- tance. The round collimator should provide the central
age, X-ray tube current and timer. For non-adjustable X-ray beam size within 30 cm in diameter or with the
indicators—permanent markers or labels can be sufficient. rectangular collimator, 800 cm2 area, at a distance of 1.5 m
4. X-ray tube voltage: To operate the X-ray tube, the or maximum focal spot to film distance, whichever is
minimum tube voltage should not be below 50 kilo- less.
volt peak (kVp). The actual kVp should not deviate 7%
the selected value from the manufacturer.
Film receptors
5. X-ray tube current: The actual value should not depart
5% by the manufacturer specification. The film speed standard was pioneered by the American
6. Timer: It should be National Standards Institute and the International
a. An automated electronic timer Organization for Standardization. A, B, C, D, E and F film
b. An automated or manual preset loader with zero speeds are available in dental radiography with A-speed
or OFF position starter set being slowest and F-speed being the fastest (Figure 2). The
c. Automated termination after each exposure exposure to the patient will be reduced by 50% without
d. The maximum pre-settable irradiation time should forfeiting the diagnostic quality by the faster films. So the
be within 5 seconds, or the time required to deliver films lesser than E-speed are not desirable to be used in
50 milliampere-seconds, whichever is shorter. dental radiography.
706
Figure 2 Typical doses from dental radiographic examinations

600 Acceptable X-ray exposure ranges speed Examination Effective Equivalent period of
groups D, E and F film dose (mSv) natural background
550
radiation
500
2 X bitewings, 70 kV, 200 mm 0.0023 8.8 hours
450 FSD2, rectangular collimation,
E-speed film
400
2 X bitewings, 70 kV, 200 mm 0.0043 17.5 hours
D
mA of 10–3 Gy
350 FSD2, round collimation,

E-speed film
300 E
2 X bitewings, 50–60 kV, 0.0083.7 1.5 days
250 100 mm FSD2, round
collimation, E-speed film
200
F 2 X bitewings, 50–60 kV, 0.0163.5 3 days
150 100 mm FSD2, round
100 collimation, D-speed film
Dental panoramic, rare earth 0.0074 1.3 days
50
intensifying screens
0 Dental panoramic, calcium 0.0144 2.6 days
50 60 70 80 90 100 tungstate intensifying screens
Kilovoltage
Relationship between surface exposures delivered to

a patient by exposure of group D, E, F intraoral films and
diagnostic density at various kilovoltages Intensifying Screen
The rare earth phosphors in modern intensifying screens
emit green light on interaction with X-rays. The rare earth
Intensifying screens used in extraoral films reduce the intensifying screens decrease patient exposure up to 55%
patient exposure to radiation. This accounts to 55% reduc- in panoramic and cephalometric radiography.
tion with rare earth intensifying screen compared to its A further reduction in patient exposure during extra-
contemporary calcium tungstate intensifying screens. The oral radiography may be achieved with the use of T-grain
phosphor crystals in the intensifying screen fluoresce when film. Introduced as T-Mat by the Eastman Kodak Company
exposed to radiation and in turn that light exposes the in 1983, this film contains silver halide grains that are
film aiding in reduced exposure. tabular or flat in shape. With its flat surface and greater
cross-section oriented toward the X-ray source, it can
Film storage gather more light from intensifying screens.
T-grain film used with rare earth screens is twice as fast
For film storage, dry and cool area is preferable. The limi- as calcium tungstate screen-film combinations and thrice
tation for radiation perception before use should not exceed as fast as conventional rare earth screen-film combinations
1.75 Gy (0.2 mR). Shielding of film storage is time depen- with retained image quality. The T-grain technology was
dent and 1.5 mm of lead shielding is sufficient for all kinds incorporated into intraoral film by Kodak to achieve the
of workload facility. faster speeds of E and F films.
Extraoral films exposed by intensifying screens achieve
a level of image resolution that is about half of the direct
Digital Radiography
exposure intraoral film. One reason for image degradation
Numerical formatting with networked computer system in in extraoral imaging systems is crossover; which refers to
obtaining high quality image constitutes digital radiography. the loss of image sharpness and resolution resulting from
This adopted software technology in imageology has the light emitted by one screen passing through the X-ray film
additional advantage of reduced exposure to both patient to expose the emulsion on the opposite side of the double
and operator, speedy recovery of the image, digital storage emulsion film. The Ultra-Vision (DuPont) and Ektavision
of the image with its electronic transmission, discontinua- (Kodak) screen film systems were designed to minimize
tion of chemical processing and its associated hazardous crossover by using phosphors that emit ultraviolet light,
wastage, elimination of lead foil, saving darkroom equip- which is less able to pass through the film base to expose
ment and adjustable diagnostic quality of the image. the opposite emulsion.
707
Results have shown that images produced by these sys- Source to skin distance
tems have higher resolution than corresponding rare earth
Distance is one of the important parameter in reducing the
screen-film systems. This allows for the use of a screen
exposure to an individual. Source to skin distance is one
one speed class higher and a 50% reduction in patient
of the parameters in reducing the exposure to an individ-
exposure.
ual. The inverse square law, ␣ I/d2 is pertinent, which states
Unlike digital intraoral imaging, there is no significant
that exposure and distance are inversely proportional. If
dose reduction to be gained by replacing extraoral screen-
the distance is doubled, the exposure is reduced by four-
film systems with digital imaging. Image resolution with
fold. ‘I’ stands for intensity of radiation and ‘d’ stands for
digital systems appears to approach that obtained with rare
distance.
earth regular-speed screens matches with T-Mat film.
Source to image receptor distance (SID)
Grids
The appropriate SID is another distance related parameter
Grids are the devices used in extraoral radiography to in reducing the patient exposure and also repeated expo-
improve the image quality by reducing the scattered radia- sure. Concentration of photons in patients has direct rela-
tion. Though the grids require slightly higher exposure, the tion with SID. Longer the SID, lesser is the exposure due
advantage of obtaining high quality radiographs, transcend to less divergent beam (Figure 3). Shorter the SID, greater
the minor risk of increased radiation dose to the patient. is the exposure secondary to concentration of photons.
This in turn reduces the repeated chance of exposure. Variation in SID results in compromised film density. So,
it is advisable to stick to recommended standardization.
The primary beam should be within 2% of the SID.
Figure 3 Collimation
Image Collimator reduces the radiation exposure by limiting the
16" distance 8" distance
Film primary and scattered beam of radiation which sequentially
helps in recuperating the image quality. The central X-ray
beam should be within the minimum coverage area. The
Federal Regulation (US) has recommended the restricted
diameter of collimator beam to 2.75 inches at the patient’s
face. To reduce the radiographic exposure in intraoral
radiographs, an inbuilt rectangular collimator in radio-
graphic machine can be a complimentary (Figure 4). This
will reduce the radiation dose up to five-fold in comparison
Source to image distance
with the circular ones.
Figure 4
Round and rectangular collimators
708
Figure 5 Skin entrance exposure
Patient examination view Skin entrance exposure

Dental bitewing (3 inch diameter 300 mrem—film
area) 90 mrem—digital radiography
140 mrem—computed
radiography
Chest X-ray (14 ⫻ 17 inch area) 20 mrem
Abdominal film (14 ⫻ 17 inch area) 300 mrem
Lumbar spine (14 ⫻ 17 inch area) 350 mrem
Extremity X-ray (8 ⫻ 10 inch area) 30 mrem
Skull X-ray (8 ⫻ 10 inch area) 100 mrem
Breast (mammogram) (glandular 175 mrad
tissue dose)
Long position indicating device
filter and for above 70 kVp, 2.5 mm aluminum filter are the
recommendations.
Figure 6
Lead aprons and collars
Tube head Cylinder tip
Lead aprons and thyroid collars are the patient protecting
Cylinder equipments used against scattered radiation. The recom-
mended lead shielding outfit for radiation workers is of
0.5 mm thickness. These protecting equipments are strongly
recommended for children and pregnant women as they are
susceptible to radiation effects but not to all adult patients
if recommended guidelines are followed stringently. As
Lead part of maintenance and long shelf life, the leaded shields
diaphragm should always be hung and not folded to prevent crack
Aluminum filter formation.
Aluminum filter Film and sensor holders

The clear-cut alignment of the collimated beam with the
teeth or jaws can be achieved through the film holder or the
Position Indicating Device digital sensor holders. In addition, PID also helps in proper
A long position indicating device (PID) with open ended alignment and prevention of cone cut images. This will pre-
and metallic lining reduces the radiation exposure by vent the repeated unnecessary unacceptable images. Among
restricting the primary beam and reducing the tissue vol- intraoral periapical radiographic techniques, paralleling
ume exposed to radiation (Figure 5). technique will reduce the exposure to more than half the
bisecting angle technique which is mainly based on the
Filtration alignment of the film holder with the PID. For safety infec-
tion control, disposable holders or sterilizable ones are
The aim of filtration is to reduce the patient exposure by recommended.
absorbing low energy photons from beam of X-rays which To reduce the radiation exposure to the clinician, he/she
do not have any role in the diagnostic image (Figure 6). should not hold the holders during exposure. Under extra-
Absorption of low energy photons increases the mean ordinary situation, the patient attender should be under
energy of the primary beam in addition to its penetrating moderation to hold the film after being prepared with
power. appropriate shielding.
There is 20% reduction in exposure with an inbuilt
X-ray machine of 3 mm of aluminum filters. So half-value
Kilovoltage
layer is designated for specific amount of filtration in dental
X-ray machines operating at a choice of kilovoltages. For Kilovoltage is the energizing exposure factor that controls the
X-ray machines operating at 50–70 kVp, 1.5 mm aluminum X-ray beam. This operating potential affects the radiation
709
dose and the backscatter radiation. Though the operating always beneficial to humans. Therefore, we should respect
potential of dental X-ray machine ranges between 50 and radiation rather being afraid of it.
100 kVp, the operating potential between 60 and 80 kVp is In spite of less radiation exposure to dental profession-
the most useful for diagnostic purposes. For less than als compared to other fraternity, it is indispensable to
60 kVp machine, an internal installation of aluminum fil- minimize occupational exposure to ionizing radiation. For
ter makes the mean beam energy to 60 kVp. a health care worker, the maximum permissible annual
As the kVp increases, there will be an increase in the dose limit is 50 mSv and maximum permissible lifetime
effective energy of the X-ray beam and image contrast dose is 10 mSv multiplied by person’s the age in years. For
will decrease. This helps in visualization of smaller differ- operating personnel, it is recommended to use protection
ence in density within the object, better resolution and barrier.
reduction in the effective dose, for example, in periodontal For this reason, the operator protection measures includ-
diseases. ing education, the implementation of a radiation protection
As the kVp decreases, there will be decrease in the program, annual and lifetime limits of exposure to ioniz-
effective energy of the X-ray beam and image contrast ing radiation, recommendations for personnel dosimeters
will increase and there will be higher entrance skin dose, and the use of barrier shielding should be incorporated.
for example, in visualization of caries and soft tissue
calcification.
The introduction of constant-potential (fully rectified), X-ray Equipment Installation and Related Factors
high-frequency or direct current (DC) dental X-ray units
1. The concluding X-ray equipment installation plan,
have made possible the production of diagnostic quality
either a new or amendment of the existing one has to
radiographs with lower kilovoltage and at reduced levels
be reviewed by an appropriate government agency.
of radiation.
The plan and supplementary papers should clearly
state the following: (i) the design and dimension of
Milliampere-seconds
the X-ray equipment operating room; (ii) the materi-
Exposure time is the most vital factor in influencing als used for construction, barrier, shielding should be
diagnostic quality. Both overexposed and underexposed mentioned; (iii) the position of doors, windows and
radiographs result in repeated exposures, thereby leading louvers; (iv) status of adjacent or surrounding area,
to needless additional patient exposure. Milliampere-second above and below the room facility; (v) concise narra-
(mAs) is the combination of milli amperage and exposure tion of the X-ray unit, manufacturer details and
time affecting the quantity of X-rays produced and sequen- working parameter; (vi) information on location and
tially the image density. orientation of dental chair, ray equipment, patient
Patient exposure is directly related to mAs. Typically a and film supporting devices.
radiograph of correct density will demonstrate very faint 2. The X-ray equipment installation should be in a safe
soft tissue outlines. Enamel and dentin will have an optical environment, providing safety for both patient and
density of about 1.0. Optimal image density can be obtained operator by directing the primary radiation always
by using values listed, after considering the age and phys- toward the shielded barrier or an unoccupied area.
ical stature of the patient. For example, 3.5 mAs is suggested 3. The setting of switching off the X-ray equipment
for an average adult when F-speed film and an operating should always be located outside the room, behind an
kilovoltage of 70 are used. This value may be arrived at adequate barrier with sufficient distance from the pri-
by using milliamperage of 10 and an exposure time of mary source.
0.35 second. If the kilovoltage is increased to reduce image
contrast, the mAs must be decreased or the radiograph will X-ray equipment room design
be overexposed.
1. First of all, the X-ray equipment installed room should
Phototiming technique uses a phototimer to measure
be designed such that the operator is not exposed to
the quantity of radiation reaching the film and automati-
the primary radiation beam.
cally terminates the exposure when enough radiation has
2. For designing the shielding of the X-ray equipment
reached the film to provide the required density. A form of
room, including the floor, wall, ceiling and door, the
this technology is currently available with some panoramic
location and dimension of the room, equipment param-
machine.
eters including workload, tube voltage, etc., should all
be taken into consideration.
3. Lead is the choice of material for room shielding. This
Protecting Personnel
should be uninterrupted and with supportive barrier
Radiation and humans are inseparable. Minimal usage to prevent drooping.
in daily activities, diagnostic and therapeutic modalities is 4. The CT room dimension should be above 25 m2.
710
Figure 7 Figure 8
Protection of the operator Lead gloves
Protection of the operator

Figure 9
1. The operator should stand at least 3 meters or 6 feet or
at an angle of 90–135 degrees from the X-ray tube or
the central X-ray beam.
2. To view the radiographic procedures by the operator
without protective barrier, leaded glass window or
shielded barrier view is an alternative option (Figure 7).
Radiation protection inspection

1. It should be on a customary basis to have an updated
and validated standard of working condition accord-
ing to parliamentary requirements.
2. Implementation and maintenance of Quality Assurance
Program.
Shielding
Radiation shielding is a mass of radiation absorbing mate-
rial placed around the radiation source to reduce the radi-
ation to a safe level for humans.
Different types of shielding in diagnostic radiology are:
Lead apron and thyroid collar
1. Operating personnel shielding
2. Patient shielding
3. X-ray equipment room shielding
4. X-ray tube shielding
5. Patient waiting room shielding. minimum 0.5 mm of lead equivalence protection device is
necessary.
Personnel shielding
Patient shielding
Appropriate personnel shielding can be achieved by having
(i) a lead protective barrier of 1.5 mm between operator and The susceptible organs, namely, thyroid, breasts and gonads
X-ray tube; (ii) lead apron and gloves of 0.25 mm thick- should be shielded in children and young adults with
ness (Figures 8 and 9); (iii) lead gonadal shield of 0.5 mm 0.25 mm of lead thickness for lead apron, collar and gloves
thickness. For any specialized radiological investigation, and gonadal shield of 0.5 mm of lead thickness.
711
X-ray equipment room shielding Patient waiting area

A few of the mandatory decisive factors about X-ray equip- 1. The patients’ waiting area should be outside the X-ray
ment room are: room.
2. A suitable alert signal in the form of red light should
1. The equipped room location should always be away
be placed at a noticeable place outside the X-ray room
from maternity and pediatric wards and with the min-
and should be kept in ON position during working to
imum dimension of 18 m2.
warn the people.
2. The wall opposing the central beam must be of mini-
3. A warning placard should be attached at the eye-
mum 35 cm thick brick.
catching place.
3. The wall opposing the scattered beam must be of min-
imum 23 cm thick brick.
4. For protection of adjacent area, the door and windows
RADIATION DETECTION AND
of the X-ray equipped room should be at least 23 cm
thick brick or 1.7 mm of lead equivalent.
MEASUREMENT
5. For ventilation or natural lightening, an unshielded
opening of 2 m above height from the finished level The principle behind detecting the radiation is the physical
outside the X-ray equipped room is permissible. and chemical effects produced by the radiation.
The following are the principal methods used to detect
Shielding of X-ray control room radiation:
It is a secondary protective measure from ionizing radia- 1. Ionization

tion. Based on the operating potential, the control room 2. Photographic effect
can be situated either within the X-ray equipped room or 3. Luminescence
outside. 4. Scintillation.
If the operating potential is up to 125 kVp, the control-
ling panel can be situated within X-ray equipped room with Ionization
a minimum distance of 3 m between fixed X-ray equipment The process of converting an atom or molecule into an ion
and the control panel. by adding or removing charged particles such as electrons
If the operating potential is above 125 kVp, then the con- or ions is termed as ionization.
trol panel should be installed outside the X-ray equipped
room with appropriate shielding for the room along with Principle Ionization in air by radiation.
accommodation of direct vision and oral communication. Radiation detection device The ionization chamber
The location of the control booth should be in such a (Figure 10).
way that the primary beam should scatter twice before
entering the room. Functioning mode It consists of an electrode positioned
In addition to this, the control booth wall and window in the middle of a cylinder that contains gas. When X-rays
shielding should be of 1.5 mm lead thickness. enter the chamber, they ionize the gas to form negative
X-ray tube shielding (source shielding)

The rationale of X-ray tube shielding is to protect the Figure 10
patient and operating personnel from leakage radiation.
This can be achieved by placing thin sheets of lead lining
Radiation
over tube housing, which helps in reducing the scattered source
radiation. − − − −
The limitation of radiation leakage exposure rate is
0.1 R h⫺1 at 1 m. The recommended maximum allowable + + + +
leakage radiation from tube housing should be less than A −
1 mGy h⫺1/100 cm2.
Ionization
Radiation in the CT Suite chamber
− +
In the CT suit, in order to reduce the scattered radiation
from 0.3 Gy/day to 1 mGy/year, an additional lead thickness
of 2.5 mm or 162 mm of concrete to shield the front and
Ionization chamber
rear reference point is recommended.
712
ions (electrons) and positive ions (positrons). The electrons

Figure 11
are collected by the positively charged rod, while the pos-
itive ions are attracted to the negatively charged wall of
the cylinder. The resulting small current from the chamber
is subsequently amplified and measured. The strength of
the current is proportional to the radiation intensity.
Photographic effect
Principle The ability of radiation to blacken the photo-
graphic film.
Application Detection of radiation exposure in films.
Luminescence
Principle The property of certain materials that emit
light when stimulated by a physiological process, a chem- Personnel dosimeter
ical or electrical action, or by heat.
Functioning mode When radiation strikes luminescence
materials, the electrons are raised to higher orbital levels. Radiation measurement By time-integrated dose, i.e.
When they fall back to their original orbital level, light is the dose summed over a period of time, usually about
emitted. The amount of light emitted is proportional to the 3 months. The dose is consequently stated as an estimate
radiation intensity. of the effective dose equivalent to the whole body in mSv
Example: Lithium fluoride will emit light when stimulated for the reporting period. Dosimeters used for personnel
by heat. monitoring have dose measurement limit of 0.1–0.2 mSv
Radiation measuring device on luminescence property (10–20 mrem).
is thermoluminescence dosimetry (TLD), a method used to
measure exposure to patients and personnel. Pocket dosimeter
It is a personnel radiation monitoring device.
Scintillation
Working mode It consists of an ionization chamber with
Principle and working mode The property of certain an eyepiece and a transparent scale, as well as a hollow
crystals such as sodium iodide and cesium iodide to absorb charging rod and a fixed and a movable fiber. When the
radiation and convert it to light. The direction of this light X-rays enter the dosimeter, ionization causes the fibers to
to a photomultiplier tube, converts the light into an elec- lose their charges and, as a result, the movable fiber moves
trical pulse. The size of the pulse is proportional to the light closer to the fixed fiber. The movable fiber provides an
intensity, which is in turn is proportional to the energy of estimate of gamma or X-ray dose rate. The pocket dosim-
the radiation. eter can measure radiation up to 50 ␮C/kg (200 mR).
Personnel Dosimetry Film badge monitoring

Radiation dosimeter is the instrument used to measure The film badge consists of small X-ray films sandwiched
radiation. between several filters, which help to detect radiation. Film
badges are inexpensive, simple mode of functioning. They are
Personnel dosimeter It is used for the monitoring of
useful for detecting radiation at or above 0.1 mSv (10 mrem).
individuals who are exposed to radiation during the course
Drawbacks of film badge are:
of their work (Figure 11).
Personnel dosimetry policies are must for all occupa- 1. They are insensitive to radiation below 0.1 mSv.
tionally exposed individuals. It is mandatory to wear per- 2. They cannot be worn longer than 4 weeks duration at
sonnel dosimeter if the annual dose is greater than 1 mSv. a stretch due to fogging as an outcome of high tem-
The values obtained from the dosimeter are honorable perature and light.
only when the dosimeters are properly worn and irradiated 3. The colossal task of chemically processing a large
only during occupational exposure and are returned on time. number of small films and subsequently comparing
Appreciation of a few personnel dosimeters are the each to some standard test films.
pocket dosimeter, the film badge or the thermoluminescent 4. The film badge cannot measure exposures less than
dosimeter. 2.6 ␮C/kg (10 mR).
713
These two positions will indicate:

Figure 12
1. Estimation of the whole body exposure at the trunk
level badge.
2. Estimation of exposures to internal organs like thy-
roid at the collar level badge.
During fluoroscopy
The protective apron should always be worn during fluo-
roscopy. Preferably two dosimeters should be worn by
radiation personnel.
One at the collar level outside the lead apron indicates
TLD badge an accurate estimate of the radiation dose to the unpro-
tected regions of head and neck; and the other at the trunk
level underneath the lead apron illustrates an accurate
In India, film badges have been recently replaced by TLD estimate of the radiation to the protected organs. If only
badges. one dosimeter is worn it must be worn at the collar outside
the lead apron, because, the neck receives 10–20 times
Thermoluminescent dosimetry (TLD) more radiation than the trunk which is protected by lead.
Principle of thermoluminescence It is the property of
certain materials to emit light when they are stimulated by
heat (Figure 12). FILM EXPOSURE AND PROCESSING
Reserves of TLD Lithium fluoride (LiF), lithium borate
Exposure parameters and film processing events can affect
(Li2B4O7), calcium fluoride (CaF2), and calcium sulfate
the quality of the radiographic image. The milliampere-
(CaSO4).
second is an important parameter set by the operator for
Working mode When an LiF crystal is exposed to radia- optimal quality of radiograph. Acquainting the skill of
tion, a few electrons become trapped in higher energy lev- film processing is another important dexterous parameter
els. For these electrons to return to their normal energy as the overexposed and underdeveloping of a film, lead to
levels, the LiF crystal must be heated. As the electrons excessive patient exposure and can produce images of
return to their stable state, light is emitted because of the poor diagnostic quality.
energy difference between two orbital levels. The amount The methods to reduce radiation exposure, result in
of light emitted is measured (by a photomultiplier tube) radiation protection. These are stated as follows:
and it is proportional to the radiation dose. 1. Stick on to proper exposure factors.
Advantages of TLD are: 2. Maintain proper record of films.
1. It can measure exposures to individuals as low as The processing recommendations include:
1.3 ␮C/kg (5 mR).
2. It can withstand a certain degree of heat, humidity, 1. Adequate ventilation for darkrooms.
and pressure. 2. Proper handling of the processing solutions.
3. Its crystals are reusable. 3. Judicious use of safe light.
4. Instantaneous readings are possible if the department 4. Following waste disposal regulations in relation to
has a TLD analyzer. processing solution and lead foil.
Disadvantage of a TLD: It is not cost effective. Darkroom

For manual processing of films, well-equipped darkroom
Wearing the Dosimeter under recommended guidelines is obligatory to get good
quality radiographs. This will indirectly reduce the expo-
During radiography
sure to the patient.
Site of personnel wearing dosimeter during radiography
1. The darkroom must be light-taut.
are at two regions:
2. The darkroom should integrate a lockable, double
1. On the trunk of the body at the level of the waist, on doors with blackened warren entrance.
the anterior side of the individual. 3. A warning light should be located outside the dark-
2. On the upper chest region at the level of the collar room at the entrance and it should be in ON position
area on the anterior surface of the individual. indicating work in progress.
714
Recordings in operational check log book
Frequency Operational check

Daily Check the quality of a film with reference radiograph
Monthly Check and analyze problems with the film, developing process, the X-ray unit or the user
Six monthly Check the light leaks in processing box
Check the condition of personnel protective equipment
Check the X-ray film storage and its shelf life
Check that the oldest X-ray film is used first
Check the expiry date of the film specified by the manufacturer
Check that exposure factors for specific examinations are readily available
Check the RSO details are displayed in a prominent location adjacent to the X-ray equipment and are correct
Check the processor maintenance procedures are displayed in a prominent location contiguous to the film processor
Check the instructions for mixing chemicals and processing films are available
Check time and temperature with timer and thermometer, respectively for manual processing
Check the warning signs of X-ray equipment being displayed and control panel are in good condition
4. Safelight bulbs of 15 W intensity must be placed 4 feet The processing chemistry should be evaluated daily, and
above the work area within the darkroom. each type of film should be evaluated monthly or when a
5. Safelight filters: Red GBX-2 filters should be placed new box or batch of films is opened.
at proper distances and must be checked regularly as Lead aprons and thyroid collars should be inspected
they may deteriorate with time or may crack. visually for damage on a monthly basis and should be
6. The darkroom must be equipped with proper stainless examined fluoroscopically on an annual basis. Lead aprons
steel processing tanks with water bath and lids, and collars in poor condition should be disposed off using
including an accurate thermometer and timing device. a recycler licensed to handle lead wastes.
Quality assurance Continuing education

Quality assurance etiquette is essential for all the user Radiation protection is a fundamental component of the
needs. This includes X-ray machine, imaging receptor, working infrastructure of any radiology department. Every
film processing, darkroom, and lead aprons and thyroid update about the radiation safety issues, recent models
collars which can be implemented in each dental health and upgradations of equipment, materials and techniques
care setting. All quality assurance procedures including should be adopted by every practitioners to improve the
date, procedure, results and corrective action, should be quality of the radiographic practices.
logged for documentation purposes. Before undertaking any radiological examination, it is
A thorough survey during installation period and important that the physician, radiologist and technologist
re-survey in every 4 years should be done by a qualified all understand the potential risks of radiation and also its
expert of state agencies. The film processor should be advantages or benefits to the patients in dentistry, medi-
evaluated every month in addition to initial installation. cine and everyday life.
715

SECTION Radiographic
X Methodology
27 Radiographic Films and Accessories 719

28 Radiographic Techniques 724
717
Chapter-27.indd 717 10/3/2012 10:51:20 AM


CHAPTER
Radiographic Films and
Accessories
27
➧ Intraoral Films ➧ Extraoral Films
Constituents of an Intraoral Film Packet Cassettes
Composition of Intraoral Film Intensifying Screens
Dimensions of Intraoral Films Grids
Radiographic films and accessories such as cassettes, film The outer jacket (on the tube side/white surface) is incor-
holders and grids are essential in every day dental prac- porated with a raised dot, which relates with a similar area on
tice. Use of appropriate accessories will help in improving the dental film inside the packet. This dot aids in orientation
diagnostic quality of the films, enhance patient care and of the film during the exposure (always places such that the
aid in improving work efficiency. dot lies closer to the incisal or occlusal aspect of the teeth).
This chapter will describe the constituents of an intra- Once the film is processed the orientation of the raised dot
oral film, cassettes and intensifying screens, grids and film is used to identify the left and right sides of the patient.
holders.
Lead foil Once the outer jacket is opened a thin sheet of
lead foil is seen. This lead foil has parallel indentations or
markings along the surface in one corner of the foil. If the
INTRAORAL FILMS film is exposed placing the wrong side toward the tube
head, these markings are seen on the resultant radiograph.
These films are used for periapical, bitewing and occlusal If the wrong side of the film is exposed, though the radio-
radiography. These are also referred to as direct action films graph will reveal an image, the image will appear light.
(primary sensitive to X-rays) and non-screen films (not used The markings on the film will help to identify the cause for
in combination with intensifying screens).
Constituents of an Intraoral Film Packet Figure 1

An intraoral film packet consists of an outer protective
jacket, lead foil, black-colored paper wrapper and the film
(Figure 1).
Film
Outer protective jacket The outer protective jacket is
essentially made of light proof and moisture proof soft Black-colored
paper wrapper
vinyl. A recent development is the use of phthalate free
film packets which are otherwise considered potentially Lead foil
harmful to health.
The outer jacket is dual colored. The tube side of the
Outer protective
film packet is white colored and the opposite side is twin
plastic jacket
colored. The cover is sealed to prevent contamination of
the film from moisture, exposure to light and ingress of
oral fluids when placed in the mouth. The packet on the
non-tube side has a flap which is pulled open in the dark-
Contents of the intraoral film packet
room to remove the exposed film for processing.
719
Section X – Radiographic Methodology
the underexposed or light radiograph. These markings silver halide grains and trace amount of sulfur compounds
(diamond markings) are seen on the resultant radiograph. and gold so as to enhance the sensitivity of the silver
Lead foil is incorporated into the film packet to: halide grains to X-rays.
❍ Minimize the amount of X-rays from passing through 1. Silver halide grains: Silver bromide makes up most of
the film and interact with the tissues beyond the film the silver halide grains that are incorporated in the
and reflect back leading to multiple exposures, thereby emulsion. However, silver iodide is added in small quan-
minimizing the quality of the final image. tities as they have crystals of larger diameter which
❍ Minimize exposure of the patient’s tissues, which are in help in increasing the sensitivity of the film.
line with the X-ray beam but beyond the imaging area. 2. Vehicle matrix: The vehicle matrix helps in the
even distribution and dispersion of the silver halide
Black paper wrapper Once the lead foil is folded back,
grains. It is made up of gelatinous or non-gelatinous
the black paper wrapper is seen in which the film is placed.
substances.
The paper wrapper protects the film from extraneous vis-
ible light and protects the emulsion of the dental film dur-
Base
ing handling and storage. It also helps in absorbing any
moisture created within the film packet as a result of humid The base of the intraoral film is made up of polyester poly-
storing conditions. ethylene terephthalate and is about 0.22 mm in thickness.
The film has a raised dot on one side and the other side It is believed that a blue tinted base will improve the view-
has a small concave depressed area. The film holder used ing characteristics of the radiograph.
for processing is clipped onto the film conforming to the Requirement of an ideal base:
depressed shallow concave area. This allows a one-point
1. It should support the emulsion.
contact between the clip and the film. The advantage of this
2. It should have enough flexibility to enable easy han-
one-point contact is that processing solutions freely flow
dling of the film.
into the shallow depression on the film, thereby exposing
3. It should be uniformly translucent and should not
every single area of the film. Holding the film in any other
hamper the diagnostic quality of the film.
area will obscure the diagnostic details on the film.
4. It should be able to withstand the effects of the chem-
icals in the processing solutions.
Composition of Intraoral Film An adhesive is usually applied in between the emulsion
Intraoral films are made up of two principal components, and base to improve the adhesion between the two.
namely, emulsion and the base (Figure 2). An additional layer of the gelatinous substance is added
to the film emulsion, which is referred to as an overcoat.
It protects the film from scratching or contamination of
Emulsion
the film during handling or contact with the processing
The emulsion is sensitive to X-rays and visible light. The tanks and it also protects the film from the pressure of
emulsion is composed of a vehicle matrix which holds the rollers of an automatic processor.
Figure 2
Overcoat
B
A
S
E
Emulsion consisting of
silver halide grains
Composition of the intraoral film. Courtesy: Dr Jaideep Shekhar
720
Chapter 27 – Radiographic Films and Accessories
Dimensions of Intraoral Films children and size 1 can be used in children. In some instances
a longer film (size 3) is used in adults, which measures
Intraoral periapical radiographs (IOPAR) 53 ⫻ 26 mm.
Intraoral periapical radiographs are available in three sizes
(Figure 3). Occlusal radiographs
Size 0—(22 ⫻ 35 mm) used for child patients The occlusal film measures 57 ⫻ 76 cm in dimension. It is
Size 1—(24 ⫻ 40 mm) these narrow films are used for imag- three times larger than a size 2 IOPAR film.
ing adult anterior teeth
Size 2—(31 ⫻ 41 mm) standard films that are used for imag-
ing adult posterior teeth. EXTRAORAL FILMS
However, when size 1 films are not available, the size 2
adult films can be placed vertically and used for imaging These films are called screen films or indirect films as the
adult anterior teeth. radiographic film is placed between two intensifying screens
within a cassette. Screen films are employed in extraoral
Bitewing radiographs radiography such as orthopantomograph, lateral cephalo-
graph, skull views, TMJ views, and lateral oblique views of
Routinely adult size 2 IOPAR is used for taking bitewing
the mandible. Extraoral films are usually available in three
radiographs. However, size 0 can be used in very small
sizes: (i) 6 ⫻ 12 inches (orthopantomography), (ii) 8 ⫻ 10
inches (all other skull radiographs: PNS, PA view, lateral
Figure 3 cephalogram, etc.) and (iii) 6 ⫻ 8 inches (TMJ views, lateral
oblique).
Cassettes
Cassettes are light tight containers, which help in maintain-
ing a uniform and intimate contact between the film and
the intensifying screens. Cassettes are available in various
sizes and shapes conforming to the various sizes of radio-
graphic film (6 ⫻ 8 inches, 8 ⫻ 10 inches and 6 ⫻ 12 inches).
IOPAR
These are usually made of plastic or thin metal. Basically
IOPAR film
size 2 pedodontic extraoral film cassettes are available in two specifications,
Occlusal film
film size 0 namely, rigid and flexible cassettes.
1. Rigid cassettes These are made of thin metal framework.
Various film sizes, namely, the occlusal, size 2 adult film and
The surface on the exposure side is made up of plastic and
size 0 child or pedo film
the non-exposure surface has metal clamps, which help in
Figure 4
A B C
(A) Intensifying screens within an extraoral cassette. (B) Extraoral film cassette of the size 8 ⫻ 10 inches.
(C) Film cassette for orthopantomography of the size 6 ⫻ 12 inches
721
Figure 5 Figure 6
X-ray beam
Phosphor layer
Film
Reflecting layer
Base
Composition of intensifying screen.

Grid
locking the cassette so as to ensure an intimate contact
between the film and the intensifying screens (Figure 4A–C).
Rigid cassettes are easy to handle and protect the screen,
are some of the salts that are used. The presence of these
however they are expensive and may break on impact.
salts has led to the naming of intensifying screens as salt
2. Flexible cassettes These are made up of flexible plas- screens.
tic and used for orthopantomography. These are delicate However, rare earth materials are used these days such
and may tear with regular use. These are also difficult to as terbium activated gadolinium oxysulfide and thulium
load and unload in the darkroom setting. However, these activated lanthanum oxybromide.
are comparatively cheaper than rigid cassettes and require
less room for storage. Coat
The coat is made up of plastic and has a thickness of 8 ␮m.
Intensifying Screens It provides protection to the underlying phosphor layer.
As the coat is made up of plastic it can be easily cleaned.
Intensifying screens are used in pairs and are positioned It should be ensured that the coat is free from scratches
on either side of a double emulsion film. and dirt.
Composition of intensifying screen Intensifying screens
are made of four principal components: the base, reflect-
Grids (Figure 6)
ing layer, phosphor layer and the coat (Figure 5).
During a radiographic exposure, almost 30% of the scat-
Base tered photons (formed as a result of Compton scattering)
leave the site imaged and exit the body. These scattered
The base of the intensifying screen is made up of the same
photons will result in the formation of dark areas on the
material that is used in an intraoral film, i.e. polyethylene
radiograph, which interfere with the diagnostic quality of
terephthalate. The base is usually 0.25 mm in thickness.
the radiographic image. However, the use of grids increases
The base acts as a supportive material.
the patient exposure by two-fold.
Therefore, grids should be used only when contrast on
Reflecting layer
a radiograph is really necessary. As the grid contains radi-
It is made up of titanium dioxide. It is placed between the opaque absorbing materials, the exposure time has to be
base and the phosphor layer. It helps in reflecting back doubled when using a grid. If the exposure time is not
on to the phosphor layer any light that is emitted by it. changed, the resultant radiograph might show multiple
However, the reflecting layer may add to the unsharpness thin white lines, which minimize the density of the radio-
of the resultant image. graph. These lines that are seen on the radiographic image
are called grid lines.
Phosphor layer
Composition
Radiosensitive phosphor salts are incorporated into this
layer of the intensifying screen. Inorganic salts such as A grid is made up of alternating strips of lead (radiopaque
calcium tungstate, zinc sulfide, and zinc cadmium sulfate material) and plastic (radiolucent material) spacers (Figure 7).
722
Chapter 27 – Radiographic Films and Accessories
The effectiveness of the grid is calculated by its grid

Figure 7
ratio. Higher the grid ratio, greater is the efficiency of the
grid in removing the scattered radiation.
Grid ratio It is the ratio of the thickness of the grid (t) to
the width of the radiolucent spacer material (d). A grid ratio
of 8 or 10 is most preferred.
Grid ratio = t/d
Patient Types of grid
t 1. Linear grid
2. Focused grid
d 3. Pseudo focused grid
Grid
4. Crossed grid
Receptor 5. Moving grid or Potter–Bucky diaphragm.
Grid—line diagram Functions

Grids are placed between the object to be imaged and the
Grids are made with varying number of pairs of spacer image receptor system (film) to preferentially minimize the
and absorber material for every square inch. Grids with 80 amount of scattered radiation reaching the film. Grids
or more line pairs per inch do not show radiolucent grid reduce film fog from scattered radiation, thereby increas-
lines on the resultant image. ing the contrast of the resultant image.
723
CHAPTER
Radiographic Techniques
28 Gail F Williamson, Kıvanç Kamburoğlu*,

Suman Jai Sanghar, Jai Sanghar N,
Rinky Nacchyon, Apeksha Mainali,
CONVENTIONAL IMAGING ➧ Lateral Oblique View

Intraoral Radiography Lateral Oblique of the Body of the Mandible
Intraoral X-ray Machine and Maxilla
X-ray Beam Angulation and Alignment Lateral Oblique of the Ramus of the Mandible
Radiographic Infection Control Bimolar Projection
Radiation Safety and Protection ➧ TMJ Radiography
Preliminary Procedures ➧ Transcranial View
Intraoral Radiographic Techniques ➧ Transpharyngeal View
Intraoral Radiographic Procedures (Parma Projection, Macqueen-Dell Technique)
Common Intraoral Technique Errors
➧ Transorbital View
Extraoral Radiography ➧ Lesser Known/Forgotten Extraoral
Technique Radiographic Techniques
Landmarks for Positioning in Skull Townes’ Method or AP Axial Projection
Radiography May Method
➧ Posteroanterior Projection Axiolateral Oblique Projection
Occipitofrontal Projection Modified Parietoacanthial Projection
PA Mandible Acanthioparietal Projection ‘Reverse Waters’
PA Cephalometric Method’
Rotated PA View ➧ Panoramic Radiology
➧ Standard Occipitomental view Origin of Panoramic Radiology
➧ 30ⴗ Occipitomental Concepts of Panoramic Imaging
➧ Parietoacanthial View (Waters’ View) Characteristics of Ghost Images
Indications of Panoramic Radiograph
➧ Parietoacanthial View
Disadvantages and Limitations
(Open Mouth Waters’ View)
➧ Modified Parietoacanthial Projection SPECIALIZED IMAGING
(Modified Waters’ View) Computed Tomography (CT)
➧ Acanthoparietal Projection Working Principle
(Reverse Waters’ Method) Computed Tomographic Scanner Assembly
➧ Submentovertex View (Base or Full Axial Advantages of CT
Projection, Schuller Method) Disadvantages of CT
Uses of CT in Dentistry
➧ Lateral View
➧ Lateral Cephalometry Dentomaxillofacial Cone-Beam Computed
➧ AP Axial Projection (Townes’ Method) Tomography (CBCT)
Historical Background
➧ Reverse Townes’ View
*Dr Kıvanç Kamburoğlu is grateful to Tomoloji Dentomaxillofacial Radiology Center and Gulhane Military Medical Academy, Dento-
maxillofacial Radiology Center, Ankara for their support.
724
Chapter 28 – Radiographic Techniques
Fundamentals of CBCT Main Indications for Ultrasound in the

Advantages and Disadvantages/Limitations of Head and Neck
CBCT Advantages Over Conventional X-ray Imaging
CBCT Clinical Applications Disadvantages
Artifacts
Magnetic Resonance Imaging
Historical Perspective Sialography
Principles of Magnetic Resonance Imaging Indications of Sialography
MR-contrast Agents Procedure
Methods for Obtaining Spatial (Tomographic) Injection of the Contrast Medium
Resolution of MR Signals Phases of Sialography
Uses for MRI Contraindications of Sialography
Advantages
Arthrography
Disadvantages
Uses of Arthrography
Common Artifacts in MRI
Types of Arthrography
Nuclear Medicine Procedure
Radiopharmaceuticals Limitations
Bone Scanning Complications
Salivary Gland Scans
Thermography (Thermal Imaging or Infrared
Ultrasonography Imaging)
Principle Liquid Crystal Thermography
Different Types of Scans Electronic Infrared Telethermography
Different Types of Scanners Used Patient Preparation
Instrumentation Procedural Requirements
Doppler Ultrasound Indications
CONVENTIONAL IMAGING or full-mouth survey consists of 14–16 periapicals and

2–4 posterior bitewings (Figure 1A, B). In addition to peri-
INTRAORAL RADIOGRAPHY apicals and bitewings, occlusal radiographs can be taken to
record a broad region of either the maxilla or mandible. In
Gail F Williamson most instances, occlusal images are supplemental views
taken to record a larger area or to localize an object.
Intraoral radiography is an important tool for proper diag- Intraoral radiographs can be combined with or aug-
nosis and treatment. Intraoral radiographs allow the clini- mented by extraoral radiographs. In extraoral radiogra-
cian to view the teeth and supporting structures revealing phy, both the receptor and X-ray source are housed outside
conditions that may not be apparent clinically. Intraoral the oral cavity. Extraoral radiography requires specialized
radiography is accomplished by placement of a small equipment and can be accomplished with intensifying
image receptor (IR) inside the mouth behind the teeth and screen-film combinations or with digital imaging systems.
structures of interest with the X-ray beam aligned externally The panoramic image is the most common extraoral pro-
over the corresponding area. Available intraoral receptors jection which provides a more complete view of the maxilla
include radiographic film, rigid digital sensors and photo- and mandible, the maxillary sinuses and the temporoman-
stimulable phosphor plates. Intraoral radiography involves dibular joints (TMJ) (Figure 2). Cephalometric radiography
the acquisition of periapical and bitewing images that can is more task-specific and includes a variety of skull pro-
be combined to form surveys. Periapical radiographs record jections. The most common application of cephalometric
the entire tooth or several teeth and the surrounding struc- radiography is the lateral skull projection used in orthodon-
tures including the apical regions, the trabecular bone, the tics. The lateral skull view is used to evaluate structures,
periodontal ligament space and the lamina dura. By con- monitor growth and development and track treatment
trast, bitewings record the crowns, proximal contacts of the progress. Cone-beam computed tomography (CBCT) is the
teeth and the alveolar bone crests. Typically, a complete most recent addition to extraoral dental imaging. CBCT is
725
indicated when three-dimensional (3D) imaging is neces- settings (Figure 3A, B). Kilovoltage controls the penetrat-
sary to evaluate and plan treatment in situations such as ing power of the X-ray beam and image contrast or differ-
implant placement, failed endodontic treatment, pathologi- ences in darkness. Milliamperage and time control the
cal conditions, difficult surgical procedures and evaluation number of X-rays and image density or the overall dark-
of craniofacial anomalies. ness of a radiographic image. Most intraoral X-ray
machines have fixed kilovoltage and milliamperage set-
tings which permit adjustment of the exposure time only.
Intraoral X-ray Machine The length of the exposure time is dependent on a number
The typical intraoral dental X-ray machine used to expose of factors including the kilovoltage and milliamperage
intraoral receptors operates at 60 or 70 kilovolts (kV), settings, collimation of the X-ray beam, patient size, area
4–8 milliamperes (mA) with variable exposure time of interest and the type of receptor.
Collimation restricts the size of the X-ray beam, reduces
patient exposure and improves image quality by reduction
Figure 1 of scatter radiation and minimizing penumbra production.
Collimation is accomplished with open-ended position indi-
A cating devices (PIDs) that are either circular or rectangular
in shape and vary in length from 20 to 40 cm (Figure 4).
Long rectangular collimation restricts the X-ray beam and
Figure 2
A typical full-mouth survey consists of 14–16 periapicals

and 2–4 bitewings. (A) Full-mouth survey taken with
size 2 film receptors. (B) Full-mouth survey taken with Panoramic image demonstrating the maxilla,
size 1 and 2 film receptors mandible, dentition and TMJ
Figure 3
A B
(A) Intraoral dental X-ray machine control panel. (B) X-ray head and PID;
HeliodentPlus Sirona Dental Systems, Inc., Long Island City, New York
726
reduces the volume of tissue exposed on the skin surface (Figure 3A). Exposure time increases as the survey pro-
the most and, therefore, is recommended for periapical gresses from the anterior to the posterior regions of the
radiography and bitewing radiography when practicable. mouth. Digital receptor exposures follow these same gen-
When selecting the exposure time, consideration should eral principles but the time settings are lower for each area
be given to the overall size of the patient as well as the when compared to film. The speed of the receptor has an
area of interest. Recommended exposure time settings are influence on the exposure time as well. Speed indicates the
designed for the average adult patient. For adult patients sensitivity of a receptor to X-rays; the faster the receptor,
smaller than average, the exposure time for each area can the less the radiation required to produce a diagnostic
be reduced by one step and for larger than average, image. Currently, F-speed is the fastest film available for
increased by one step. Exposure time settings for children intraoral radiography, providing significant dose reduction
are considerably less than for adults and frequently can and comparable performance compared to D-speed film.
be accessed by selecting a child icon on the control panel Digital receptors, both rigid sensors and phosphor plates,
provide equal or greater dose savings than F-speed film
with equivalent diagnostic utility. Intraoral X-ray machines
manufactured today are compatible with film, rigid sensors
Figure 4 and phosphor plates.
X-ray Beam Angulation and Alignment

The vertical angulation of the X-ray beam varies and is
dependent on the tooth, dental arch, oral anatomy and
intraoral technique used. Vertical angulation is adjusted by
moving the X-ray head and PID up or down. Positive vertical
angulations are used for maxillary periapicals and bitewings
and negative vertical angulations are used for mandibular
periapicals. Positive vertical angulations position the X-ray
head above horizon with the PID directed downward while
negative vertical angulations position the X-ray head below
horizon with the PID directed upward (Figure 5A, B). Most
dental X-ray machines have an angle meter on the X-ray
Collimation restricts the size of the X-ray beam and the head to indicate the degree of the vertical angulation with
area of patient skin exposure. It can be accomplished with horizon designated as 0. Vertical angulation controls the
a variety of devices including PIDs, metal devices that clip length dimension of the recorded image. Errors in vertical
into ringed instruments or that slide onto a PID.
angulation produce images that either shorten or lengthen
Rectangular collimation is optimal
the actual vertical dimension of the structures.
Figure 5
A B
Vertical angulation. (A) Positive vertical angulation is used for maxillary periapicals as demonstrated by this premolar placement.
(B) Negative vertical angulation is used for mandibular periapicals as demonstrated by this incisor periapical placement
727
Figure 6 Figure 7
Outer canthus
Pupil of eye
Ala of nose
Tip of nose
Infection control barriers for intraoral image receptors

Mentum
Central ray entry points are facial anatomic landmarks patients and the dental professional. For the clinician, this
designed to help the clinician align the central ray to the involves the use of personal protective equipment (PPE)
center of the image receptor inside the mouth including clinical attire, eyewear, a mask and gloves to
avoid contact with patient’s oral fluids. For radiographic
equipment, this involves pre-cleaning, disinfection and
preferably, coverage with plastic barriers before seating the
The horizontal angulation is adjusted by moving the patient and initiating radiographic procedures. An environ-
X-ray head and PID anteriorly or posteriorly (forward or mental protection agency (EPA)-registered hospital disin-
backward) along the horizontal plane. The horizontal fectant with intermediate-level activity should be used to
angulation is directed proximally for all intraoral views prepare radiographic equipment and environmental sur-
and follows the curvature of the dental arches. This places faces. Barriers must be changed between patients but when
the X-ray beam perpendicular to the horizontal plane of barriers are not used, the equipment and environmental
the teeth of interest. Errors in horizontal angulation result surfaces must be pre-cleaned and disinfected between
in overlapping of the proximal surfaces of the teeth and patients.
width distortion such that the teeth contacts and alveolar Disposable or radiographic instruments that can be
bone crests cannot be properly visualized. heat-sterilized should be used for intraoral radiography.
In order to expose the entire receptor, the center of the Intraoral digital receptors that cannot be heat-sterilized must
X-ray beam, known as the central ray (CR), is directed be covered with a FDA (Food and Drug Administration)-
toward the middle of the receptor which is represented cleared barrier (Figure 7) and cleaned and disinfected with
externally by facial anatomic points as illustrated in an EPA-registered hospital disinfectant with intermediate-
Figure 6. Failure to properly center the X-ray beam over level activity between patients. Protective plastic barrier
the receptor produces a partial image. The unexposed por- envelopes are used to cover radiographic film and phosphor
tion is called a ‘cone cut’, a term that dates back to the plates during placement in the mouth (Figure 7). After
time when closed cones were used rather than open-ended exposure, contaminated external barrier must be disinfected
PID collimators to direct the X-ray beam. Closed cones are before dropping the film or plate out of the barrier in prep-
no longer used as they produce excessive scatter radiation aration for processing. It is best to consult manufacturer
that significantly increases the radiation dose delivered to instructions for guidance on digital receptor handling and
the patient. disinfection practices.
Radiographic Infection Control Radiation Safety and Protection

Standard infection control procedures must be utilized ALARA (As Low As Reasonably Achievable) is the guiding
when taking intraoral radiographs. During radiographic principle in radiation safety and protection. It is the ethical
procedures, the clinician can be exposed to a variety of and professional obligation of dental professionals to keep
pathogens through direct contact with the patient’s oral the radiation dose delivered to the patient to a minimum.
fluids or by contact with contaminated radiographic Although the risk of intraoral radiography is thought to be
equipment and work surfaces. The primary goals of infec- very small, it is not risk-free and the effects of radiation
tion control are to prevent cross-contamination and the are cumulative. The measures most effective in dose reduc-
transmission of disease between patients and between tion include the use of selection criteria, fast film or digital
728
Table 1 Selection criteria guidelines
Patient type Child primary dentition Child mixed dentition Adolescent dentition Adult dentate/artially Adult edentulous
New patient Bitewings if contacts closed Bitewings/panoramic Bitewings/panoramic Bitewings/panoramic Individualized
occlusals FMS when indicated FMS when indicated Based on exam
Recall high caries Bitewings at 6–12 month Bitewings at 6–12 Bitewings at 6–12 Bitewings at 6–18 Not applicable
risk intervals month intervals month intervals month intervals
Recall low caries risk Bitewings at 12–24 month Bitewings at 12–24 Bitewings at 18–36 Bitewings at 24–36 Not applicable
intervals month intervals month intervals month intervals
Recall for periodontal Selected Selected Selected Selected Not applicable
patient PAS/BWS as needed PAS/BWS as needed PAS/BWS as needed PAS/BWS as needed
Growth and Only when indicated Only when indicated Panoramic or PAS for Not applicable Not applicable
development 3rd molars
Other Only in a special situation or circumstance
PAS: Periapicals; FMS: Full mouth survey; BWS: Bitewings.
receptors for intraoral radiography, rare earth screen-film these guidelines and taken for diagnostic and treatment
combinations or digital receptors for extraoral radiogra- purposes only. Selection criteria guidelines are based on
phy, rectangular collimation of the X-ray beam and patient the evidence of disease patterns and information obtained
shielding. from the patient’s medical and dental history, clinical signs
The clinician should practice radiation safety measures and symptoms of disease, risk factors, age and dentition,
to avoid occupational exposure and to comply with the and new or recall patient status. Only bitewing radiographs
maximum permissible dose (MPD) limit. The annual dose are recommended on time-based regimens as determined
limit is 50 millisieverts (mSv) and the lifetime dose limit by patient risk factors for caries. See Table 1 for a summary
is 10 mSv multiplied by the clinician’s age in years. However, of these guidelines. Typically, radiographic procedures are
the clinician should strive to completely avoid any occupa- delegated to qualified dental staff (dental assistant and/or
tional exposure. Compliance is easily achieved by not stand- dental hygienists) for completion.
ing in or near the X-ray beam or in its path; not holding Once the appropriate survey has been determined, the
the X-ray head or PID in place; not holding the receptor in patient should be prepared for the procedure. Facial jew-
the patient’s mouth or holding the patient in position. elry, intraoral prostheses and eyeglasses should be removed
If assistance is needed to stabilize a patient, the parent or and safely stored. The patient should be protected with a
guardian should be asked to assist, provided with a radia- lead apron and thyroid collar, provided with an explana-
tion shield and given instructions on how to restrain the tion of the purpose of the examination and given instruc-
dental patient. During exposure, the clinician should stand tions to elicit cooperation (Figure 8). Preferably, the patient
behind a wall barrier or 2 meters away and slightly greater is seated in an upright position with the midline centered
than a right angle to the X-ray beam. Personal radiation and the occlusal plane parallel to the floor for maxillary
dosimeters are commercially available to track occupational periapicals and bitewings and the chin raised so that the
exposure and are recommended for pregnant clinicians who mandibular arch is parallel to the floor for mandibular
expose dental radiographs. periapicals. The exposure time should be set for the area
before placement of the receptor inside the mouth. When
ready for exposure, the clinician should practice radiation
Preliminary Procedures
safety measures to avoid occupational radiation exposure.
Before radiographs are prescribed, a complete medical Once the survey is acquired, proper processing techniques
and dental history must be taken, reviewed and the chief should be followed as dictated by the selected image recep-
complaint identified. The dentist should examine the denti- tor. The clinician must ensure that the processed radio-
tion to determine if radiographs are indicated. If radiographs graphic images are properly arranged in the film mount or
are necessary, the type and number of projections should computer image template according to the ADA standard.
be identified. The American Dental Association (ADA) and This standard involves labial mounting and viewing of
the FDA have established guidelines for the selection of radiographic images according to the patient’s right and
dental radiographic examinations in the United States. left sides of the dentition. Attention to correct tooth order,
Dental radiographs should be prescribed according to proper location of surrounding anatomical structures and
729
Figure 8 Figure 9
Paralleling technique requires placement of the receptor

parallel to the teeth with the central ray directed at
a right angle to both the teeth and receptor
Dental patients should be shielded with a
lead apron and thyroid collar during intraoral
radiographic procedures
Figure 10
observation of restorative treatments and/or missing that

match from periapical to periapical and/or periapical to
bitewing will ensure accuracy of arrangement.
Intraoral Radiographic Techniques

There are two techniques that can be utilized for taking
A variety of ringed paralleling technique instruments are
periapicals radiographs: the paralleling technique and the commercially available. The instruments depicted here have
bisecting angle technique. The paralleling technique is bite blocks designed to hold rigid digital receptors. XCP DS®,
preferred because it produces the most accurate and repre- Dentsply Rinn, Elgin, Illinois
sentative images of the teeth and surrounding structures.
The bisecting angle technique is considered a secondary
or alternate technique when paralleling technique can-
not be accomplished due to intraoral anatomy or other horizontal and vertical angulation and centering of the X-ray
patient factors. Bisecting angle technique produces images beam (Figure 10). There are a variety of ringed instruments
with inherent distortion because the buccal and lingual commercially available with designs for all three types of
aspects of the teeth and alveolar bone are not projected intraoral receptors. Proper assembly and parallel receptor
evenly. This distortion must be taken into consideration placement to the area of interest are necessary for optimal
when interpreting the radiographs for caries and alveolar results. A standard bite-block can be used for the parallel-
bone loss. ing technique as long as the clinician has the skill to align
the PID accurately.
Paralleling technique
Bisecting angle technique
The paralleling technique requires placement of the recep-
tor parallel to the tooth or teeth of interest both in the The bisecting angle technique is based on Cieszynski’s rule
vertical and horizontal planes. The X-ray beam is directed of isometry or the geometry of equilateral triangles. This
so that it strikes both the tooth and the receptor at a right rule states that two triangles are equal if they share a com-
angle (Figure 9). Typically, the paralleling technique is mon side and two equal angles. Applied to periapical radi-
accomplished with ringed instruments that aid the clinician ography, a tooth and its projected image will be equal in
in establishing the correct object to receptor relationship, length if the X-ray beam is directed at a right angle to the
730
Figure 11 Figure 12
Many types of bisecting angle instruments are commercially

available for film, rigid sensors and phosphor plate receptors.
Snap-A-Ray® Xtra, Stabe Biteblock® and Eezee-Grip®
Digital sensor holders are shown from left to right.
Dentsply Rinn, Elgin, Illinois
Bisecting angle technique directs the central ray at Figure 13

a right angle to the plane that divides the angle
formed by the teeth and receptor
Table 2 Bisecting angle technique
Periapical view Maxillary Mandibular

Incisor 40 to 50 5 to 15
Lateral-canine 40 to 50 5 to 15
Premolar 25 to 35 10 to 15
Molar 20 to 30 5 to 5
Radiographic film ranges from size 0 to 4. Depicted in
Approximate vertical angulations
the front row are the size 2 bitewing, size 3 bitewing and
size 1 periapical films. In the back row are the size 4 occlusal,
size 0 periapical and size 2 periapical films. Carestream
Dental LLC, Atlanta, Georgia
common side or bisecting plane that divides the triangle
into two equal halves or equilateral triangles (Figure 11).
Care must be taken to accurately determine the bisecting
plane to avoid errors in vertical angulation. Approximate on the type and manufacturer of the receptor. Periapical
vertical angulations used for each periapical are listed in receptor sizes include 0, 1 and 2 and bitewing receptor
Table 2. The horizontal angulation and centering of the sizes include 0, 1, 2 and 3. Size 0 is most commonly used
X-ray beam remain the same as with the paralleling tech- in pediatric imaging and the size 4 receptor is for adult
nique. There are instruments commercially available for occlusal radiography.
the bisecting angle technique for both film and digital
Radiographic film Radiographic film is the traditional
receptors (Figure 12). Bisecting angle technique requires
medium for recording intraoral images (Figure 13). It con-
more technical skill and as such, more retakes tend to
sists of a blue-tinted plastic base material coated with a
occur than with the paralleling technique.
double emulsion of gelatin and silver halide crystals, pre-
Image receptors As previously mentioned, intraoral dominantly silver bromide. The film has a dot convexity
radiographs can be taken with three different types of that indicates the exposure side and aids in proper arrange-
intraoral receptors: radiographic film, rigid digital sensors ment and mounting of the processed radiographs. The film
or photostimulable phosphor plates. All three receptors are is wrapped with an internal black paper with a lead foil on
considered approximately equivalent in their ability to the rear or non-exposure side and covered externally with
record dental disease states; caries, periodontal disease either a plastic or paper material to protect the emulsion
and periapical pathoses. Intraoral image receptors range from light and moisture. The exposure side of the film
in sizes from 0 to 4, however, the range of sizes depends packet is white and the non-exposure side is two-toned
731
Figure 14
A B
Rigid wire dental sensors. (A) Size 2 and 1 rigid digital sensors. Schick CDR Elite,
Schick Technologies, Inc., Long Island City, New York. (B) Digital molar periapical image
with colors used to indicate speed and single or double visible image can be viewed on the monitor almost
film packet. Once exposed to radiation, the emulsion cap- instantly. Rigid digital sensors are reusable receptors that
tures and stores the latent image until the image is made cannot be sterilized. As indicated previously, they must be
visible by chemical processing. Those silver halide crystals properly disinfected and covered with a barrier to avoid
exposed to radiation are reduced to metallic silver by the direct contact with oral fluids. When positioning the
developer solution reducing agents and form the radiolu- receptor in the mouth, the wire should be oriented toward
cent components of the image. The non-exposed, undevel- the occlusal plane for vertically placed anterior periapicals
oped silver halide crystals are removed by the fixer solution and directed anteriorly for horizontally placed posterior
clearing agent and become the radiopaque components of periapicals.
the image. Typically film-based images are processed by Direct digital receptors require less radiation exposure
automatic processing systems that develop, fix and dry than film. The amount of exposure reduction is related
radiographic film in approximately 5.5 minutes. The pro- to the speed of the comparison film. Typical exposures for
cessed radiographs are ready for arrangement and mount- the average patient range from 0.04 to 0.06 second in the
ing after exiting the processor. Care must be taken to anterior sextants to 0.08–0.12 second in the posterior sex-
properly maintain the processor utilizing regular solution tants. Refer to the X-ray machine user manual for recom-
replenishment, cleaning and solution change regimens. mended exposure times.
Processing errors are a common cause of retakes in film- Errors associated with rigid digital receptors are well-
based imaging. documented in the dental literature. Common errors include
vertical angulation errors with foreshortened images and
Direct digital receptors Direct digital receptors are rigid, crown cut-offs, placement errors, horizontal overlap, cone
wired receptors in the form of the charge-coupled device cuts and problems related to patient discomfort. The rigid
(CCD) or complimentary metal oxide semi-conductor (CMOS) nature of the receptor requires strict attention to the details
detector (Figure 14A, B). The primary difference between of placement, intraoral technique and accurate alignment
the two devices is the manner in which the latent image is of the X-ray beam. A cotton roll can be placed on the
transferred to the read-out amplifier for display. The active bite-block to avoid crown cut-off and reduce patient biting
image area is smaller compared to film or phosphor plates pressure. To reduce patient discomfort, place rigid receptors
so the area of coverage is slightly diminished. These rigid closer to the midline where the mouth has greater depth.
devices are area arrays composed of a matrix of pixels Commercially available foam tissue cushions and topical
with each pixel functioning as an electron well. When the anesthetic agents are useful in reducing discomfort and
sensor is exposed to radiation, the energy that penetrates minimizing the gag reflex.
through the structures is deposited in the electron well in The primary advantages of direct digital receptors are
that location. The intensity of the energy or signal deter- rapid image acquisition and immediate image display while
mines the brightness or density of the image in each area. the disadvantages are the rigid, wired construction and
The digital data is processed by the computer and the related placement and discomfort difficulties.
732
Figure 15
A B
Phosphor plate receptors. (A) Size 0, 1, 2 and 4 phosphor plates. The opposite side or emulsion side is
directed toward the X-ray source. ScanX® Phosphor Storage Plates, Air Techniques, Inc., Melville, New York.
(B) Phosphor plate premolar periapical
Photostimulable phosphor plates Photostimulable phos- Figure 16

phor plates (PSPs) or storage phosphor plates (SPPs) are
wireless digital receptors that handle similar to film (Figure
15A, B). A separate plate is needed for each exposure and
a processing step is required before the images can be
viewed on the computer monitor. Phosphor plate receptors
have a single emulsion coated on the exposure side of the
plate composed of europium-activated barium fluorohalide.
Rather than an identification dot, plate receptors have a
letter or number to identify the exposure side of the recep-
tor. Care must be taken to prevent abrasion, crimping or
creasing of the emulsion to avoid permanent image arti-
facts and frequent plate replacement. Once the plate is
exposed, the latent image is stored in the emulsion until
the plate is scanned by a helium laser beam (Figure 16).
When the phosphor plate is scanned, it emits light in pro-
portion to the exposure received. The light detected by the
photomultiplier is converted from analog-to-digital data
and the visible image is displayed on the monitor for view-
ing. The scanning takes several seconds causing a slight Exposed phosphor plate receptors must be scanned
delay between image capture and display. Before reuse, the by a helium laser beam to digitize and display the visible
plate must be erased by white light to remove any remnant image. ScanX Intraoral Scanner, Air Techniques, Inc.,
image. Typically, the plate is erased prior to exit from the Melville, New York
scanning unit. The primary advantages of the phosphor
plate are its thin, wireless construction while the disadvan-
tages are susceptibility to emulsion scratches producing
artifacts and delayed image viewing. of the teeth and supporting structures such as carious
lesions, periodontal bone loss and periapical and bony
pathoses. Periapical radiographs can be taken in any area
Intraoral Radiographic Procedures of the mouth. There are classic placements for the incisor,
canine, premolar and molar teeth as outlined in Table 3.
Periapical radiography
When taking a full-mouth survey, it is best to take the ante-
Periapical images provide information about tooth morphol- rior periapicals first. This allows the patient time to adjust
ogy, apical proximity to anatomic structures, abnormalities to the procedure before taking the posterior periapicals
733
Table 3 Periapical and bitewing techniques
Head position: midsagittal plane perpendicular and occlusal plane parallel to floor
Projection Teeth recorded Horizontal angle Vertical angle Central ray Diagram
Maxillary incisor Central incisor teeth Proximal between Parallel to teeth long Tip of nose
periapical centered central incisors axes
Maxillary lateral-canine Lateral and canine teeth Proximal between Parallel to teeth long Ala of nose
periapical centered lateral & canine axes
Maxillary premolar Distal of canine, 1st & Proximal between Parallel to teeth long Pupil of the eye to
periapical 2nd premolar, 1st molar premolar teeth axes mid-cheek
Maxillary molar 1st, 2nd & 3rd molar; Proximal 1st & 2nd Parallel to teeth long Outer canthus to
periapical 2nd molar centered molar teeth axes mid-cheek
Premolar bitewing Canine distal, 1st & 2nd Proximals between 5 Pupil of eye to
premolar, 1st molar premolar teeth occlusal plane
Molar bitewing 1st, 2nd & 3rd molars; Proximals between 5 Outer canthus to
2nd molar centered 1st & 2nd molar occlusal plane
teeth
Head position: midsagittal plane perpendicular and mandibular arch parallel to floor
Mandibular incisor Central incisor teeth Proximal between Parallel to teeth long Center of chin
periapical centered central incisors axes
Mandibular lateral- Lateral and canine teeth Proximal between Parallel to teeth long Center of chin corner
canine periapical centered lateral & canine axes
Mandibular premolar Canine distal, 1st & 2nd Proximal between Parallel to teeth long Pupil of the eye to
periapical premolar, 1st molar premolar teeth axes mid-mandible
Mandibular molar 1st, 2nd & 3rd molar; Proximal 1st & 2nd Parallel to teeth long Outer canthus to
periapical 2nd molar centered molar teeth axes mid-mandible
734
which tend to be more difficult for some patients. Anterior Bitewing radiography
periapicals are placed in the vertical dimension with the
Bitewing radiographs are most frequently taken in the
receptor identification dot or marker located in the coro-
posterior regions of the mouth of the premolar and molar
nal aspect of the image. For adult patients, the size 1 or 2
teeth for the detection of proximal caries and alveolar
receptor can be used. The size 1 receptor is preferred for
bone loss (Figure 19A, B). For adult patients, the size 2
narrow or crowded dental arches. Anterior periapicals are
receptor is preferred. Bitewings can be oriented in either
used to record the central incisor, lateral incisor and canine
the horizontal or vertical plane with the receptor positioned
teeth (Figure 17). Posterior periapicals are oriented in the
parallel to the teeth crowns. Horizontal bitewings are the
horizontal dimension and are used to record the distal
most common but vertical bitewings are taken particularly
aspect of the canine, premolar and molar teeth (Figure 18).
when moderate or greater alveolar bone loss is present.
For adult patients, the size 2 receptor is the standard for
Anterior bitewings can be taken to view bone levels in the
posterior projections.
anterior segments of the mouth as well (Figure 20). A size 1
receptor is used to capture incisor and lateral-canine bite-
wing views. The clinician can select traditional tabs or
instrument holders to accomplish the bitewing survey.
Figure 17
Figure 18
Anterior mandibular periapicals of the incisor and Posterior maxillary periapicals of the premolar and
canine teeth molar teeth
Figure 19
Bitewings can be taken with a ringed instrument or with a tab. (A) Bitewing taken with the tab bitewing technique.
(B) Tab bitewing survey
735
Figure 20
Vertical anterior and posterior bitewing survey
Table 4 Topographical occlusal technique

Projection Head position Placement Angulation Central ray
Maxillary anterior Occlusal plane parallel & midsagittal perpendicular to Horizontal or vertical in 60 0.5 cm above tip of
the floor anterior region nose
Maxillary posterior Occlusal plane parallel & midsagittal perpendicular to Vertical toward side of 55 2 cm below pupil of
the floor interest the eye
Mandibular anterior Occlusal plane at 45 angle; midsagittal perpendicular Horizontal or vertical in 15 Center of the mentum
to the floor anterior region
The vertical angulation used for tab bitewings ranges from imaging by using a size 2 receptor and reduction of the
5 to 10. The most common error associated with bite- exposure time. In addition, anterior periapical views can
wing radiography is horizontal overlap which can render be taken using topographical occlusal technique when
the image non-diagnostic. mouth opening is limited or the arch is extremely narrow
or crowded. In these instances, a size 2 receptor is oriented
Occlusal radiography vertically. Table 4 summarizes the basic technical aspects
of topographical occlusal projections.
There are two basic types of occlusal projections that can
be taken: topographical and cross-sectional. Topographical Topographical maxillary anterior occlusal The patient’s
occlusals provide a broad view of a segment of the maxilla head is aligned with the occlusal plane parallel to the floor
or mandible. Cross-sectional occlusals are used to localize and the midsagittal plane perpendicular to the floor. The
objects or to view the buccolingual dimensions of the dental receptor can be positioned horizontally or vertically depen-
arches. The most common occlusal projections are topo- dent on the mouth size and arch width. With the mouth
graphical taken in the anterior segments of the dental opened slightly, the receptor is placed against the maxillary
arches. Adult occlusal radiographs are taken with a size 4 occlusal surfaces with the dot convexity toward the palate
intraoral receptor. The only film and phosphor plate recep- and positioned labially. The patient bites together lightly
tors available are size 4. The receptor can be positioned to secure the receptor. The approximate bisecting angle for
horizontally or vertically as needed to accommodate the this projection is 60 with the horizontal angle directed
mouth size and arch width. through the proximal contacts of the maxillary central
incisors. The central ray of the X-ray beam enters through
Topographical occlusals Topographical occlusals are based
a point located just above the tip of the nose. Generally,
on the bisecting angle technique. Topographical occlusals
the exposure time is the same time as the maxillary incisor
have the appearance of a large periapical view of the teeth
periapical for the selected receptor (Figure 21A, B).
and surrounding structures. Typical applications include
recording bilateral impactions of the permanent canine Topographical maxillary posterior occlusal As with
teeth, mesiodens or large periapical or bony lesions. Topo- the anterior occlusal, the patient’s head is positioned with
graphical occlusal projections can be applied to pediatric the occlusal plane parallel to the floor and midsagittal
736
Figure 21
A B
Topographical maxillary anterior occlusal. (A) Clinical photograph. (B) Occlusal image
Figure 22
A B
Topographical maxillary posterior occlusal. (A) Clinical photograph. (B) Occlusal image
plane perpendicular to the floor. The receptor is oriented occlusal plane is at a 45 angle above horizon. The clini-
vertically toward the side of interest and placed against cian can align the X-ray head at a 45 angle to use as a
the occlusal surfaces of the maxillary teeth with the dot comparison to check for proper head position. The occlu-
placed anteriorly. The receptor should extend approxi- sal plane should be parallel to the open end of the PID. The
mately 1 cm beyond the buccal surfaces of the posterior midsagittal plane is positioned perpendicular to the floor.
teeth and inserted posteriorly until it contacts the anterior The receptor can be positioned horizontally or vertically as
ramus. The receptor is maintained in position by light needed. With the mouth opened slightly, place the receptor
patient biting pressure. A 60 vertical angulation is used against the occlusal surfaces of the mandibular teeth with
with the horizontal angle directed through the premolar the dot convexity toward the tongue and positioned labi-
teeth contacts and the central ray entering about 2 cm ally. The patient bites together lightly to secure the recep-
below the pupil of the eye. This projection will record tor. The approximate bisecting angle for this projection is
one quadrant of the maxillary teeth and alveolar ridge 15. The horizontal angle is directed through the proxi-
(Figure 22A, B). mal contacts of the mandibular incisor teeth and the cen-
tral ray is directed through the center of the mentum. The
Topographical mandibular anterior occlusal For the man- exposure time is the same time as the mandibular incisor
dibular projection, the patient’s head is tilted back until the periapical for selected receptor (Figure 23A, B).
737
Figure 23
A B
Topographical mandibular anterior occlusal. (A) Clinical photograph. (B) Occlusal image
Table 5 Cross-sectional occlusal technique
Projection Head position Placement Angulation Central ray

Maxillary Occlusal plane parallel & midsagittal Horizontal or vertical Long axes plane of the anterior 1 cm posterior to bregma
perpendicular to the floor in anterior sectant teeth
Mandibular Occlusal & midsagittal plane perpendicular Vertical toward side of Long axes of posterior mandibular 3 cm posterior to mentum &
anterior to the floor interest teeth; 90 to receptor 3 cm lateral to midline
Mandibular Occlusal & midsagittal plane perpendicular Horizontal or vertical Long axes of anterior mandibular Midline of the floor of the
posterior to the floor in anterior sextant teeth; 90 to receptor mouth
Cross-sectional occlusals Cross-sectional occlusals are Cross-sectional mandibular anterior occlusal The patient’s
most commonly taken for the purposes of object localization head is positioned such that the occlusal plane is nearly
or to view the buccolingual aspect of the dental arches. perpendicular to the floor or as far back as possible with
Cross-sectional occlusal technique directs the central ray at the midsagittal plane perpendicular to the floor. The expo-
a right angle to the receptor. The maxillary cross-sectional sure surface of the receptor should be placed against the
occlusal requires an X-ray machine capable of 90 kV to occlusal surfaces of the mandibular teeth. Approximately
enable penetration of the calvaria. Table 5 summarizes 1 cm of receptor extends beyond the labial surfaces of the
the essential technical aspects of cross-sectional occlusal anterior teeth with the patient biting gently to secure its
projections. position. The central ray is directed perpendicular to the
receptor and through the midline of the floor of the mouth.
Cross-sectional maxillary occlusal The patient’s head
The cross-sectional anterior occlusal is useful for evaluating
and receptor are positioned in the same manner as the
expansion of the anterior mandible or localizing a salivary
other maxillary occlusal projections. The vertical angula-
stone in the sublingual gland (Figure 25A, B). The expo-
tion is determined by directing the X-ray beam through
sure time is the same time as the mandibular incisor peri-
the long axes of the anterior teeth. The horizontal angula-
apical for selected receptor.
tion is centered with the midsagittal plane and the central
ray enters the skull 1 cm posterior to bregma, the junction Cross-sectional mandibular posterior occlusal For this
of the sagittal and coronal sutures. This projection pro- projection, the patient’s head is tilted back so that occlusal
duces an unusual horseshoe-shaped image, for it depicts plane is nearly perpendicular to the floor with the mid-
all of the maxillary teeth through the long axes dimension sagittal plane perpendicular to the floor. The receptor is
end-on-end. To produce a maxillary cross-sectional image, positioned vertically toward the side of interest and as
the exposure factors should be set at 90 kVp, maximum mA, posterior as possible. The exposure surface of the receptor
and a 1-second exposure. The exposure is dependent on is placed against the occlusal surfaces of the mandibular
the selected receptor (Figure 24A, B). teeth. Approximately 1 cm of the receptor extends beyond
738
Figure 24
A B
Cross-sectional maxillary occlusal. (A) Clinical photograph. (B) Occlusal image
Figure 25
A B
Cross-sectional mandibular anterior occlusal. (A) Clinical photograph. (B) Occlusal image
the buccal surfaces of the posterior teeth with the patient eventually learn how to avoid them altogether. A diagnos-
gently biting to maintain its position. The central ray should tic periapical depicts the area of interest including the
be directed perpendicular to the receptor and centered entire length of the teeth from the crown to the root apices
3 cm posterior to the mentum and 3 cm lateral to the mid- with at least 3–4 mm of bone surrounding the apices
line. This projection records one quadrant of the mandibular and open proximal contacts. The image should have ade-
teeth through the long axes dimension. The cross-sectional quate contrast and density and be free of technical errors.
posterior occlusal is useful for evaluating mediolateral A diagnostic bitewing displays the area of interest, the
expansion of the posterior mandible or localizing a salivary proximal surfaces of the maxillary and mandibular teeth
stone in the submandibular gland (Figure 26A, B). The crowns and the alveolar bone crests. Like periapicals, ade-
exposure time is the same time as the mandibular molar quate contrast and density and absence of technical errors
periapical for selected receptor. are necessary components of a quality image. When eval-
uating a full-mouth survey, every apex and every proximal
surface should be visible somewhere within the composite
Common Intraoral Technique Errors
of images.
The clinician must have the ability to evaluate intraoral A number of technical errors can be produced when the
images for diagnostic quality and be able to identify, under- parameters of intraoral technique are not properly followed.
stand and correct errors to keep retakes to a minimum and Common errors encountered in intraoral radiography include
739
Figure 26
A B
Cross-sectional mandibular posterior occlusal. (A) Clinical photograph. (B) Occlusal image
Table 6 Common technical errors
Error Description Correction

Foreshortening Structures shorter than normal; vertical angle too steep Decrease the vertical angle of the PID
Elongation Structures longer than normal; vertical angle not steep enough Increase the vertical angle of the PID
Horizontal overlap Proximal surfaces of the teeth are superimposed onto each Direct the horizontal angle through the proximal contacts
other with width distortion of the teeth for each view
Cone cut Central ray of the X-ray beam is not aligned with the middle of Direct the central ray toward the center of the receptor
receptor producing a partial image or cut using facial landmarks or ringed instruments
Improper placement The teeth or area of interest are cut-off because the receptor is Follow the placement guidelines to include all structures
not placed in the right location required on a particular view
Underexposure Image is too light or faint because patient size was Evaluate the patient size, set the correct time for each view
underestimated, exposure time set too low or the exposure and hold down the exposure button until the exposure is
incomplete complete
Overexposure Image is too dark or dense because patient size was Evaluate the patient size, set the correct time for each view
overestimated or exposure time set too high
placement, vertical angulation errors, horizontal overlap, are dot reversal and a light image from lead foil absorp-
cone cuts and exposure errors. A discussion of these com- tion of the X-rays. Backward plate placement results in
mon errors follows. Table 6 summarizes common errors and reversal of the letter or number identifier.
their corrections.
Vertical angulation
Receptor placement
Vertical angulation errors result in either foreshortening or
The most common technical error regardless of the type of elongation of the teeth and surrounding structures. When
receptor is placement. Each periapical and bitewing place- the vertical angulation is too steep, the structures are
ment requires inclusion of specific structures on each view. diminished in length or foreshortened (Figure 28). This
When the expected structures are not recorded, retakes may error is corrected by decreasing the vertical angulation.
be necessary to gain the missing information. In addition Elongation produces the opposite result with lengthened
to incorrect location, portions of the teeth such as the structures as a result of inadequate or not enough vertical
crowns or apices can be cut-off and necessitate a retake angulation (Figure 29). Correction of elongation requires
(Figure 27). Backward placement of film and plate receptors an increase in vertical angulation to return the structures
is possible as well. Hallmarks of backward film placement to normal length.
740
Figure 27 Figure 29
Mandibular premolar periapical receptor placement error in Mandibular incisor periapical demonstrating elongation
which the apical portions were cut-off the image caused by under-angulation in the vertical plane
Figure 28 Figure 30
Maxillary incisor periapical with image foreshortening caused Premolar bitewing with proximal overlap due to improper
by over-angulation in the vertical plane horizontal angulation
Cone cuts
Horizontal overlap
A cone or PID cut is the direct result of misalignment of the
Proximal overlap is the primary error that occurs when the
central ray (Figure 32). When the central ray is not directed
horizontal angulation is misdirected (Figure 30). Diagonal
to the middle of the receptor, a portion of the receptor is
rather than perpendicular entry of the X-ray beam to the
not exposed and no image is produced in that region. Cone
horizontal planes of the teeth causes superimposition of
cuts can occur on any aspect of a radiographic image. The
the proximal surfaces from one tooth onto the next. The
use of the classic receptor placement for each view and
proximal surfaces of the teeth and alveolar bone margins
central ray alignment with external entry points will help
are obscured from view and the structures are distorted in
eliminate cone cuts. Ringed instruments with beam guides
width as well. This error is especially egregious on bite-
eliminate this error when properly assembled.
wings as it limits or prevents assessment of the teeth for
proximal caries and alveolar bone loss. The open end of
Exposure
the PID should be horizontally parallel to the buccal plane
of the teeth of interest to direct the X-rays through the A variety of exposure errors can occur that diminish
teeth contacts (Figure 31). the diagnostic quality of a radiographic image. Improper
741
Figure 31 Figure 33
The horizontal angulation should be aligned so that the A faint image of a maxillary molar periapical due to
X-rays are directed through the proximal contacts of the underexposure of the receptor
teeth on both periapicals and bitewings
Figure 34
Figure 32
A double exposure occurs when a film or plate receptor is

Cone cut error on a mandibular premolar periapical. exposed twice. It produces a dark image with superimposed
The central ray was positioned too high relative to the structures which require two retakes
center of the receptor and central entry point
Intraoral radiography is indispensable for proper diag-

selection of the exposure time for the subject or area of nosis and treatment of dental patients. A variety of intra-
interest can produce an underexposed low density image oral projections can be taken to visualize the teeth and
with a faint appearance (Figure 33) or an overexposed high- surrounding structures including periapicals, bitewings and
density image with a dark appearance. Neither is desirable occlusals. Intraoral radiographs should be prescribed by the
and both could result in retakes. With digital imaging sys- dentist according to selection criteria guidelines to ensure
tems, dark images can be corrected with the imaging soft- that the benefit of the examination outweighs the risk. The
ware as long as the image is not completely black. However, clinician must have the requisite knowledge and skill to
very light images cannot be corrected by the software effectively manage the patient and competently conduct all
because not enough exposure was received by the receptor aspects of the imaging procedure. Adherence to the ten-
to form the image. Film and plate receptors can be double- ants of radiographic technique will maximize the diagnos-
exposed producing an overly dark superimposed image tic result while at the same time minimize patient radiation
which requires two retakes (Figure 34). exposure through retake prevention.
742
EXTRAORAL RADIOGRAPHY examined. Such an amount of scatter radiation induces

a deterioration of the image quality, reducing the contrast,
Suman Jai Sanghar, Jai Sanghar N and it would eventually reduce the diagnostic quality.
Though grids minimize scatter radiation and improve the
As the term extraoral suggests, it is a technique in which quality of the resultant radiograph, they will also remove
the image receptor is placed outside the mouth during the some of the primary radiation and thus require the radia-
X-ray exposure. tion exposure to be increased.
Technique Landmarks for Positioning in Skull Radiography

Extraoral radiography, in contrast to the intraoral radiog- The basic localization points and planes of the skull (all of
raphy, is made with indirect exposure films, where the film which can be either seen or palpated) are used in radio-
is placed inside a tight cassette between intensifying screen graphic positioning (Figure 35). Accurate positioning of the
on either side and then the exposure is made after proper skull requires a full understanding of these landmarks. The
positioning of the patient with reference to the long axis planes, points, lines, and abbreviations most frequently used
of the cassette. in skull positioning are as follows:
❍ Midsagittal plane (MSP)
Image receptors
❍ Interpupillary line
Image receptors used here are otherwise known as screen ❍ Acanthion
films or indirect exposure films, as they are used along ❍ Outer canthus
with intensifying screens. These screens emit visible light ❍ Infraorbital margin
on exposure to X-rays. The emitted visible light will in ❍ External acoustic meatus (EAM)
turn expose the films. Due to the variation in the proper- ❍ Infraorbitomeatal line (IOML)
ties of intensifying screens, proper screen-film combina- ❍ Orbitomeatal line (OML)
tion must be used as recommended by the screen and film ❍ Acanthiomeatal line (AML)
manufacturer so that the emission characteristics of the ❍ Mentomeatal line (MML).
screen match the absorption characteristics of the film.
MSP The midsagittal or median plane divides the body
Dimensions of the films used in skull radiography are
into right and left halves. This plane is important in accu-
8 10 inches and for lateral oblique views, it is 5 7 inches.
rate positioning of the cranium since, for every frontal or
lateral position, the MSP is either perpendicular to, or
Intensifying screens
parallel to, the plane of the film.
Due to density of the skull, intensifying screens must be
Interpupillary or interorbital line It is the line connect-
used to minimize the amount of radiation to the patient,
ing either the pupils or the outer canthi of the patient’s
which also helps in reducing the scattered radiation. Inten-
sifying screens permit a good radiograph to be produced
with the patient receiving a much lower dose of radiation. Figure 35
Speeds of intensifying screens
Glabelloalveolar
1. Fast screens Thick layer of relatively large crystals

used, maximum speed is attained but with some sacrifice
in image detail.
line
2. Slow screens or high definition screens A thin layer of

relatively small crystals are used; detail is the best, but speed
is slow necessitating a higher dose of ionizing radiation. al l
ine Nasion
eat l line
lom
el m eata
lab o
G Orbit
3. Medium screens Medium thick layer of medium sized Infraorbitomeatal line
Acan
thiom
crystals in order to provide comprise between speed and M
en
eatal
line
External to
me Acanthion
definition. at
al
acoustic meatus lin
e
Grids
Angle of mandible Mental point
The grid is usually used to reduce scatter radiation, which (gonion)
causes a decrease of the image contrast. The amount of
scatter radiation reaching the film would be several times Illustration depicting various radiographic planes for
positioning of the skull
the primary radiation, depending on the part of the body
743
eyes. When the head is placed in a true lateral position, the ❍ Ensure that all metal objects are removed from the
interpupillary line must be exactly perpendicular to the patient, for example, hair clips and hairpins, jeweller-
plane of the film. ies, spectacles, hearing aids.
❍ Bunches of hair often produce artifacts and thus
Acanthion It is the midline point at the junction of the
should be untied.
upper lip and the nasal septum.
❍ If the area of interest includes the mouth, then denture
IOML It is the line formed by connecting the middle of (if removable) should be removed.
the infraorbital margin to EAM. It is otherwise known as ❍ The patient should be provided with a clear explana-
anthropological baseline or Frankfurt line. tion of any movements and film positions associated
OML It is a line located between the outer canthus and with the normal operation of the skull unit.
the EAM. This is also known as radiographic baseline or ❍ If needed, foam pads, Velcro straps can be used as
canthomeatal line. This line is angled approximately 10 to accessories for stabilizing the patient. Forty-five degree
the anthropological baseline. triangular pads are very useful for immobilizing children.
❍ Individual side markers are essential for skull radiography.
AML It is a line formed by connecting the acanthion to
the EAM. Radiation protection
MML It is the line formed by connecting mental point Routine radiation protection measures should be applied.
(midpoint of the triangular area of the chin) and EAM. The most effective method of dose reduction is a careful
technique to avoid the need for repeat radiograph.
Positioning terminology
To describe a skull projection, it is necessary to state the Radiographic techniques
relative positions of the skull planes to the image receptor 1. Posteroanterior (PA) projection (0)
and the central ray relative to skull planes/image receptor.
Modifications
Occipitofrontal projections The central ray enters the skull ❍ Caldwell projection (15)
through the occipital bone and exits through the frontal bone. ❍ 20 PA projection
Fronto-occipital projections The central ray enters the ❍ PA mandible
skull through the frontal bone and exits through the ❍ PA skull
occipital bone. ❍ PA cephalometric
❍ Rotated PA view
Lateral projections The central ray passes along a coronal
plane at right angles to the MSP. 2. Standard occipitomental
Oblique projections The central ray is projected at some Modifications

❍ 30 occipitomental
angle to the median plane and the coronal plane.
❍ Parietoacanthial projection (Waters’ view, PNS view)
Positioning errors ❍ Open mouth Waters’ view
❍ Modified parietoacanthial projection (modified
Rotation and tilt are two of the most common positioning Waters’)
errors (Figure 36):
3. Submentovertex (base or full axial projection, Schuller
1. Rotation—occurs when the MSP is not parallel to the film. method)
2. Tilt—occurs when the interpupillary line is not per-
pendicular to the film. 4. Lateral view
Modifications
Patient preparation ❍ Lateral cephalometric
❍ Before starting any examination, the identity of the 5. Acanthoparietal projection (reverse Waters’ view)
patient must be checked by the radiographer.
6. AP axial projection (Townes’ method)
7. Reverse Townes’ method
Figure 36
8. Lateral oblique
❍ Ramus of the mandible
❍ Body of the mandible
9. TMJ projections
❍ Transcranial
❍ Transpharyngeal
Illustrations showing the position of the mid-saggital plane
❍ Transorbital.
744
POSTEROANTERIOR PROJECTION Figure 37
Occipitofrontal Projection
Occipitofrontal projections can be taken with different
degrees of beam angulation. The choice of the angulation
depends on the anatomy, which needs to be demonstrated.
10° CR
Indications
❍ Fractures of the body, angle or ramus of the mandible,
displacement of fractures especially in a mediolateral Skull position for PA view showing the central ray directed
direction. perpendicularly through the occiput to exit the nasion
❍ Le Forte I fracture of the middle third of the face.
❍ Gross displacement of the zygomatic buttress.
❍ Displacement of the teeth in alveolar fractures.
❍ Cysts or tumors in the rami, showing expansion of the Figure 38
bone in the mediolateral direction.
❍ Deformity of the mandible or the maxillofacial area.
CR
15°
Film placement
Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device.
Patient positioning
❍ The nose and the forehead of the patient should touch
the cassette, a position which places the OML (radio- Skull position for 15 PA Caldwell view showing the central ray
graphic baseline) perpendicular to the cassette. directed at a caudal angle of 15 to the canthomeatal line
❍ Mid part of the frontal bone is positioned in the center
of the cassette.
❍ MSP is positioned perpendicular to the cassette, which
is accomplished by adjusting lateral margin of orbit or PA Mandible
EAM equidistant from the edges of the cassette. Indication
Central ray The indication of this technique is to demonstrate the

transverse or oblique fracture of the body and rami of the
For PA projections, when frontal bone is of primary inter- mandible, not evident in other projections.
est, the central ray is directed perpendicularly, through the Cassette and patient positioning is the same as that for
occiput to exit the nasion (Figure 37). PA projection except that the central ray is directed perpen-
dicular to the cassette and centered in the midline through
Variations of PA projections the cervical spine at the level of the angles of the mandible.
15 PA Caldwell method. Limitation of this technique is that the central body of
the rami is not well shown because of the superimposed
Indication For depiction of the ethmoid and frontal spine.
sinuses that include both orbits.
Patient positioning is same as that for PA projection
PA Cephalometric
except for the central ray projection, which is directed at a
caudal angle of 15 to the canthomeatal line (Figure 38). Cephalometric radiography ensures standardization and
The resultant radiograph will demonstrate the superior reproducibility of the images.
border of the petrous ridge projected onto the lower third
of the orbit. Indications
20 PA Similar to the above technique the caudal angu- ❍ To assess the position of unerupted canines.
lation alone can be changed to 20–25 which helps in bet- ❍ To evaluate the asymmetry of facial bones.
ter visualization of the orbit, as the petrous ridges will be ❍ For pre- and post-operative assessment of jaws for
projected inferiorly as the angulation is increased. both orthodontic and orthognathic procedures.
745
Film positioning Patient positioning

Cassette with the film is placed perpendicular to the floor, ❍ Patient’s nose and chin are placed in contact with the
with long axis vertically in a cassette holding device. midline of the cassette.
❍ The head is then adjusted to bring the orbitomeatal
Patient positioning baseline to 45 angle to the cassette.
❍ MSP is perpendicular to the cassette.
❍ MSP should be placed vertical and perpendicular to
the film.
Central ray
❍ Only nose should touch the film, so that the OML is
parallel with the floor which makes the radiographic Central ray is directed perpendicular to the film through the
baseline to be at 10 with the film. occiput making its exit through the anterior nasal spine.
❍ Head is immobilized using ear pieces inserted into the The final image should be such that the petrous ridge
EAM. must appear below the floor of the maxillary sinus.
❍ Film adjusted with lips centered on the film.
Central ray 30ⴗ OCCIPITOMENTAL

It is directed perpendicular to the film through MSP at the
level of the bridge of the nose. Indications
❍ To demonstrate fractures of the middle third of the face,
orbital floor.
Rotated PA View ❍ Blow-out fracture of the orbit.
Indications ❍ Fractures of the zygomatic arches.
❍ To demonstrate calculi in the parotid gland. This projection demonstrates the lower orbital margins
❍ To evaluate cysts and tumors involving the ramus of and the orbital floor. The zygomatic bones and arches
the mandible which may have mediolateral expansion. are opened out compared with the occipitomental projec-
tions as this technique uses a different angulation thereby
Technique enabling detection of certain bony displacements.
❍ The film is positioned same as that of the PA view. Film placement

❍ The head is rotated 10 toward the side of interest.
❍ This positioning rotates the bones of the back of the Cassette with the film is placed perpendicular to the floor,
skull away from the side of the face under investigation. with long axis vertically in a cassette holding device.
❍ The central ray is directed perpendicularly along the
side of the face. Patient positioning
❍ Patient’s nose and chin are placed in contact with the
midline of the cassette.
STANDARD OCCIPITOMENTAL VIEW ❍ The head is then adjusted to bring the orbitomeatal
baseline to 45 angle to the cassette.
Indications ❍ MSP is perpendicular to the cassette.
❍ Diagnosis of pathologic conditions affecting air sinuses, Central ray

especially the maxillary antra. Examples: tumors, cysts,
fluid levels. The tube head is angled 30 caudally and centered along
❍ Fractures of the middle third of the facial skeleton. the midline, such that the central ray exits at the level
of the lower orbital margins.
This position shows the floor of the orbits in profile, the
nasal region, the maxillae, the inferior parts of the frontal In a severely injured patient, a modified 30 reverse
bone, the zygomatic bone and zygomatic arches, with occipitomental view can be taken. Here the cassette is
their entire length superimposed over a small part of the positioned vertically against the vertex of the skull sup-
image. ported with foam pads so that MSP is perpendicular to
the film and to the table, also to OML.
Film placement The tube is angled 20 to the horizontal (toward
the floor) and centered to the symphysis menti in the
midline.
746
PARIETOACANTHIAL VIEW (Waters’ View) PARIETOACANTHIAL VIEW

(Open Mouth Waters’ View)
Indications
This method provides an excellent demonstration of the
This technique is useful for the evaluation of maxillary
sphenoidal sinuses. The beam is projected through the open
sinuses and it also demonstrates frontal sinuses, ethmoidal
mouth and is done for patients who cannot be placed in
sinuses, orbit, zygomaticofrontal suture and nasal cavity.
position for submentovertex view.
In Waters’ technique the neck is hyperextended enough
to place the dense petrosae immediately below the maxil- Film placement
lary sinus floor.
Film placement
Cassette with the film is placed perpendicular to the floor, Patient positioning
with long axis vertically in a cassette holding device. It is similar to the positioning in Waters’ view, except that
the patient is asked to slowly open the mouth widely. MML
Patient positioning will not be perpendicular as in Waters’.
The patient’s neck is hyperextended and centered over the
cassette perpendicular to the acanthion. MODIFIED PARIETOACANTHIAL
The patient’s chin is placed resting over the cassette PROJECTION (Modified Waters’ View)
and adjusted so that MSP is perpendicular to the plane of
the cassette.
Indication
The head is adjusted so that the OML forms an angle of
37 from the plane of cassette (Figure 39). The modified Waters’ method is a good projection to dem-
onstrate ‘blow-out’ fractures.
Central ray Although the parietoacanthial (Waters’) projection is
widely used, this technique is modified by making the patient
Perpendicular to the cassette exiting at the acanthion.
to extend his/her neck to a lesser degree. This method is
❍ Too much of angulation results in foreshortening of referred to as ‘shallow Waters’ ’.
maxillary sinus and the antral floors are not demon- In this method, OML is adjusted to form an approximately
strated. 55 angle with the plane of the cassette (Figure 40A, B).
❍ When the neck is not fully extended, the petrosae are The resulting radiograph demonstrates the facial bone
projected over the inferior portion of the maxillary with less axial angulation than with the Waters’ method.
sinus and obscure the underlying pathologic process,
if present.
ACANTHOPARIETAL PROJECTION
(Reverse Waters’ Method)
Figure 39
Indication
Reverse Waters’ method is used to demonstrate the facial bones
when the patient cannot be placed in a prone position.
37°
Patient positioning
❍ MSP is placed perpendicular to the plane and also in
the midline of the cassette.
CR ❍ Neck is extended so that the OML forms a 37 angle with
the plane of the cassette (Figure 41) by raising the chin up.
❍ If necessary, a support is placed under the patient’s
shoulders to help extend the neck.
❍ MML is approximated perpendicular to the plane of
cassette.
Skull position such that OML forms an angle of 37 Central ray
from the plane of cassette
It enters the acanthion which is parallel with MML.
747
Figure 40
A B
55°
CR
CR
35°
Illustrations showing the skull position and entry of central ray for the modified Waters’ view
Figure 41 Patient positioning

❍ The patients shoulders are raised and the neck is
hyperextended to bring the vertex of the skull in con-
tact with the cassette.
❍ The head is adjusted to bring the EAM equidistant
from the edges of the cassette (Figure 42).
❍ The MSP should be at right angles to the cassette.
❍ The OML should be as near as possibly parallel to the
cassette.
Central ray
The central ray is directed at right angles to the OML and
37°
centered midway between the EAM.
Illustration showing the skull position such that the This projection is contraindicated in patients with sus-
OML forms a 37 angle with the plane of the cassette pected neck injuries, especially suspected fractures of
odontoid region.
Though this technique is described in supine position,
Structures seen seated upright position is more comfortable because the
upright position allows a greater freedom in positioning
Reverse Waters’ demonstrates superior facial bone. The patients body to place the IOML parallel with the cassette.
image is similar to that obtained with Waters’ method, but
facial structures are considerably magnified.
Jug handle view It is a modification of submentover-
tex view (SMV) projection, wherein the whole length of
SUBMENTOVERTEX VIEW the zygomatic arch is demonstrated in profile against
(Base or Full Axial Projection, Schuller Method) the side of the skull and facial bones from which this
view derives its name.
Indications The modification done here is a decrease in the
exposure factors.
❍ To demonstrate fractures of the zygomatic arches.
❍ For visualization of the base of the skull.
❍ To evaluate the angle of inclination of the head of the
codyle. LATERAL VIEW
Film placement Indications

Cassette with the film is placed perpendicular to the floor, ❍ Backward displacement of fractures of the middle
with long axis vertically in a cassette holding device. third of the facial bones.
748
Figure 42 Figure 43
CR
CR
Skull position showing entry of central ray

Illustration depicting entry of central ray for lateral skull view
❍ Foreign body in the airway. ❍ To assess the skeletal pattern.

❍ To evaluate the posterior walls of the antrum, for its ❍ For tracing and scanning points necessary for orth-
integrity. odontic procedures.
Film placement Film placement

Cassette with the film is placed perpendicular to the floor, Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device. with long axis vertically in a cassette holding device.
Patient positioning Patient positioning
❍ The cassette is supported vertically against the side ❍ Sagittal plane is vertical and parallel to the cassette.
under examination, so that the center of the cassette is ❍ Frankfort plane should be horizontal.
2.5 cm inferior to the outer canthus of the eye. ❍ The head is immobilized by guiding the ear pieces
❍ MSP is parallel to the cassette by ensuring that the carefully into the EAM. Metal circular markers within
interorbital line is at right angle to the cassette and the the ear pieces allow the operator to rapidly recognize the
nasion and external occipital protruberance are equi- centering of the cephalostat.
distant from the margins of the cassette. ❍ The nose support is positioned over the nasion.
❍ A wedge-shaped filter is positioned so that it will be
Central ray superimposed on the face with the thick edge placed
anteriorly for demonstration of the soft tissues.
It is directed horizontally to a point 2.5 cm inferior to the ❍ The patient is instructed to close his/her mouth and to
outer canthus of the eye (Figure 43). bite together on his/her back teeth (centric occlusion).
This technique is often reserved for gross trauma as the ❍ Lips should be relaxed.
facial structures are superimposed.
If lateral view is taken for a suspected foreign body Central ray
in the eye, then additional collimation and alteration in Horizontal beam is centered on the EAM.
the centering point will be required.
AP AXIAL PROJECTION (Townes’ Method)

LATERAL CEPHALOMETRY
Indications
This is a standardized true lateral projection of the skull. ❍ To visualize the occipital area of the skull.
❍ Demonstrate the ascending rami of the mandible and
Indications condyles of both sides.
❍ To visualize the relationship of the soft and hard tis-
Film placement
sues of the face.
❍ To assess the position of the teeth in relation to each Cassette with the film is placed perpendicular to the floor,
other and to the hard tissues and adjacent structures. with long axis vertically in a cassette holding device.
749
❍ The patient is then asked to keep his/her mouth wide

Figure 44
open. By doing so, the condylar heads move out of the
CR CR glemoid fossae.
30° 37°
Central ray
The central ray is aimed upward from below the occiput
so that the central ray forms an angle of 30 to the hori-
zontal, and is centered through the condyles.
LATERAL OBLIQUE VIEW

Illustrations depicting patient head position and
entry of central ray for Townes’ method The lateral oblique radiograph is an extraoral projection that
reveals a larger area of the jaws than intraoral radiography.
Indications
Patient positioning
❍ When intraoral views are unobtainable in patients
❍ Patient is positioned such that the posterior aspect of
having trismus or severe gagging.
the skull rests on the cassette.
❍ To detect unerupted teeth or impacted molars.
❍ Patient’s head is adjusted so that the MSP is perpen-
❍ Fractures of the angle or the body of the mandible.
dicular to the midline of the cassette.
❍ Pathologic conditions affecting the jaws.
❍ Flex the patient’s neck enough to place the OML per-
pendicular to the midline of the cassette. General imaging principles
❍ When the neck cannot be flexed, the neck is adjusted
so that the IOML is perpendicular and has the central ❍ The head is rotated to ensure that the area under
ray angulation to be increased by 7 (Figure 44). examination is parallel to the film.
❍ The cassette is positioned so that its upper margin is at ❍ The film and the median plane are not parallel.
the level of the highest point of cranial vault. This will ❍ The central ray is perpendicular to the film but oblique
place the center at or near the level of foramen magnum. to the median plane.
Central ray
Lateral Oblique of the Body of the Mandible
Directed through the foramen magnum at a caudal angle of
and Maxilla
30 to the OML or 37 to IOML. Central ray enters approx-
imately 2½ inches above the glabella and through the This projection shows the dentition in the premolar/molar
level of EAM. region of the maxilla and mandible, the inferior cortex of
the mandible, and the angle and ascending ramus of the
mandible.
REVERSE TOWNES’ VIEW
Position of patient and cassette
This projection shows the condylar heads and necks. ❍ The patient is made to sit with the head supported. The
median plane is vertical.
Indications ❍ A 5 7-inch cassette is used. Film markers should be
❍ Fractures involving the condylar necks. used to indicate the side.
❍ Developmental anamolies involving the condyle, such ❍ The cassette is positioned against the patient’s cheek
as condylar hypoplasia or hyperplasia. overlying the region of the mandible under investiga-
tion, with the lower border parallel to the inferior bor-
Film placement der of the mandible but lying at least 2 cm below it.
❍ The cassette in this position is stabilized by the patient.
Cassette with the film is placed perpendicular to the floor, ❍ The patient’s head is rotated to the side of interest. This
with long axis vertically in a cassette holding device. positions the contralateral ascending ramus forward and
increases the area between the neck and the shoulder
Patient positioning
to provide space for the X-ray tube.
❍ Patient positioning is similar to that in PA projection ❍ The chin is raised slightly to increase the space between
(forehead–nose position). the posterior aspect of the mandible and the cervical spine.
❍ OML should be at right angles to the film. ❍ The patient is asked to protrude the mandible.
750
Central ray Indications

❍ Central ray is directed at a point 2 cm below and behind ❍ To demonstrate the destruction of the condyles due to
the angle of the contralateral side of the mandible. degenerative changes.
❍ The central ray is perpendicular to the plane of the ❍ To assess the degree of movement of the joint.
film.
Film placement
If the superimposition of the hyoid bone onto the body ❍ A 5 7-inch cassette is used. Film markers should be
of the mandible is to be avoided, then a downward used to indicate the side.
beam angulation of 10 can be chosen. ❍ The cassette with the film is placed on the side of
interest, with the TMJ being centered over the cassette.
❍ The cassette in this position is stabilized by the patient.
Lateral Oblique of the Ramus of the Mandible Patient positioning
This projection gives an image of the ramus from the angle ❍ MSP is parallel to the plane of the film.
of the mandible to the condyle.
Central ray
Position of patient and cassette ❍ The central ray is directed at an angle of 25 (positive
❍ The patient is made to sit with the head supported. The angulation) from the opposite side, through the cranium
median plane is vertical. and above the petrous ridge of the temporal bone.
❍ A 5 7-inch cassette is used. Film markers should be ❍ The horizontal angulation can be individually corrected
used to indicate the side. for the condylar long axis, or an average 20 anterior
❍ The cassette is positioned against the patient’s cheek angle may be used.
overlying the ascending ramus and the posterior aspect
of the condyle of the mandible under investigation. Transcranial, transpharyngeal and transorbital views
❍ The cassette is positioned such that its lower border is should be taken in both close and open mouth positions,
parallel with the inferior border of the mandible but to assess the range of condylar movements.
lies at least 2 cm below it.
❍ The patient is instructed to support the cassette in this
position. TRANSPHARYNGEAL VIEW
❍ The mandible is extended as far as possible. (Parma Projection, Macqueen-Dell Technique)
Central ray
This technique provides a sagittal view of the medial pole
❍ The central ray is directed posteriorly with upward of the condyle.
angulation of 10 toward the center of the ramus of
the mandible on the side of interest. Indications
❍ The X-ray tube is positioned on the contralateral side of ❍ To visualize the head and neck of the condyles.
the mandible at a point 2 cm below the inferior border ❍ Developmental anamolies of the condyle neck.
in the region of first or second molar. ❍ To detect grossly displaced fracture of the condyle.
Film placement
Bimolar Projection
❍ A 5 7-inch cassette is used. Film markers should be
This technique is used in orthodontic practice. It shows used to indicate the side.
both left and right oblique lateral views on one film. The ❍ The cassette with the film is placed on the side of
technique incorporates a hinged lead shield to prevent interest, with the TMJ being centered over the cassette.
exposure of the other side of the film. ❍ The cassette in this position is stabilized by the patient.
Patient positioning
TMJ RADIOGRAPHY ❍ MSP is parallel to the plane of the film.
TRANSCRANIAL VIEW Central ray

❍ The beam is directed superiorly at an angle of 5 through
It is a view that aids in visualizing the sagittal view of the the sigmoid notch of the opposite side, and 7–8 from the
lateral aspects of the condyle and temporal component. front, centered over the TMJ of the opposite side.
751
TRANSORBITAL VIEW ❍ Dorsal sellae and posterior clinoid processes

❍ Occipital bone
It provides an anterior view of the TMJ, perpendicular to ❍ Posterior portion of the parietal bone.
the transcranial and transpharyngeal projections.
Positioning of the patient
Indications The patient is asked to stand in an upright position. Arms are
❍ To visualize condylar neck fractures. placed in comfortable position. Shoulders are made to lie in
❍ To detect degenerative changes or anamolies affecting the same horizontal plane. The midsagittal plane of the body
the condyle. is centered to the center of the image receptor (Figure 45A).
Film placement Positioning of the part
❍ A 5 7-inch cassette is used. Film markers should be Patient’s head is adjusted such that midsagittal plane is
used to indicate the side. perpendicular to the midline of the IR. Patient’s neck is
❍ The cassette with the film is placed behind the skull on
flexed such that the OML is 30 to the central ray. IR
the side of interest. is positioned such that its upper margin is at the level of
the highest point of cranial vertex (Figure 45B).
Patient positioning
Central ray
The patient’s head is tilted downward 10, so that the can-
The central ray is directed through the foramen magnum
thomeatal line is horizontal.
at a caudal angle of 30 to OML. It enters approximately
Central ray 2½ inches (6.3 cm) above the glabella and passes through
the level of EAM (Figure 45C).
Central ray is oriented downward approximately by 10 Structures shown are:
and laterally approximately 30 through the ipsilateral
orbit, centered over the TMJ of interest. ❍ Symmetric image of the petrous pyramids
If the condylar motion is limited, then only the neck is ❍ Posterior portion of the foramen magnum
visible due to superimposition of the temporal component ❍ Dorsal sellae and posterior clinoid processes projected
on the condylar head. within the foramen magnum
This view is rarely being used now, due to unwanted ❍ Occipital bone
radiation exposure to the eyes. ❍ Posterior portion of the parietal bones.
In addition to the above-mentioned techniques for May Method

viewing the TMJ, reverse Townes’ view and submento-
When a patient with suspected fracture of zygomatic
vertex projections can be used to assess the TMJ.
arch comes to the department, radiographs that are usu-
ally advised for these patients are submentovertex view,
Schuller’s method and sometimes Waters’ view as well.
LESSER KNOWN/FORGOTTEN EXTRAORAL But there are always chances of superimpositions of other
RADIOGRAPHIC TECHNIQUES structures on the zygomatic arch when these methods are
used. Thus, when a radiographic view of zygomatic arch is
There are many lesser known techniques in maxillofacial required, the alternative method would be May method,
imaging which can be used as powerful tools for demon- also known as ‘tangential projection’.
strating a large variety of craniofacial structures. Although This method demonstrates:
new paradigms for X-ray imaging techniques have emerged, ❍ Zygomatic arch
they may not be economic and readily feasible to be used ❍ Temporal process of zygomatic arch
in many health centers. In such cases, some of these tech- ❍ Temporal bone
niques can still be used as a standard procedure for dem- ❍ Useful in patients who have depressed fractures or flat
onstrating various craniofacial structures. cheek bones.
Positioning of patient
Townes’ Method or AP Axial Projection
The patient is asked to stand in an upright position. Arms
This method demonstrates:
are placed in comfortable position, shoulders are made to lie
❍ The petrous pyramids in the same horizontal plane. The midsagittal plane of the
❍ Posterior portion of the foramen magnum body is centered to center of the image receptor (Figure 46A).
752
Figure 45
A B C
30°
CR
(A) Position of patient in Townes’ method. (B) Upright radiograph. (C) Upright radiograph with central ray
directed 30 degrees to OML
Figure 46
A B C
15° top of head tilt
15°
CR
(A) Position of the patient in May method. (B) Upright radiograph. (C) Central ray in May method
Positioning of the part Structures shown are:

Neck is extended such that the IOML is as parallel with ❍ Zygomatic arch free of superimposition
the plane of the IR as possible. Chin is rested on the IR. ❍ Temporal process of zygomatic arch
The midsagittal plane is rotated approximately 15. Top ❍ Temporal bone.
of the head tilted away from the side being examined 15.
It is ensured that the central ray is tangent to the
Axiolateral Oblique Projection
lateral surface of the skull. The central ray skims across
the lateral portion of the parietal bone and the mandi- Another lesser known technique is the axiolateral oblique
bular angle to project the zygomatic arch onto the IR projection, also known as original law method. This method
(Figure 46B). can otherwise be called ‘double tube angulation’.
This method demonstrates:
Central ray
❍ Mandibular condyle
The central ray is directed perpendicular to the IOML and ❍ Mastoid cells
passes through the zygomatic arch at a point approximately ❍ Lateral portion of the petrous pyramid
1½ inches posterior to the outer canthus. The IR is centered ❍ Superimposed internal and external acoustic meatuses
to the central ray (Figure 46C). ❍ Mastoid emissary veins.
753
Figure 47
A B
15° CR
(A) Position of the patient and direction of central ray in axiolateral oblique projection/
original law method. (B) Upright radiograph
Positioning of the patient ❍ Also useful for good projection demonstration of blow-
out fracture.
Patient is positioned with the head in a true lateral posi-
tion, the flexion of the patient’s head is adjusted such that Position of the patient
the interpupillary line is perpendicular to the plane of the
image receptor; and the IOML and midsagittal plane are The patient is placed in prone position. The midsagittal
parallel with the IR plane (Figure 47A). plane of the patient’s body is centered to the midline of the
image receptor (Figure 48A).
Central ray Position of the part
Central ray is directed 15 caudal and 15 anteriorly. It ❍ The patient’s head is rested on the tip of the extended
enters approximately 2 inches posterior to and 2 inches chin. The neck is extended to a lesser amount than done
above the uppermost EAM and exits the downside mastoid in Waters’ method. OML is adjusted to form approxi-
process. The IR is centered to the central ray. mately 55 angle with the plane of the IR, with the
The structures shown are (Figure 47B): average patient’s nose about 3/4 inch away from the
❍ Mandibular condyle IR. The head is adjusted so that the midsagittal plane
❍ Lateral portion of the petrous pyramid is perpendicular to the plane of the IR. The IR is cen-
❍ Superimposed internal acoustic meatus tered at the level of the acanthion (Figure 48B).
❍ Mastoid emissary veins when present
Central ray
❍ Mastoid cells.
The central ray is directed perpendicular to exit the acanthion
(Figure 48C).
Modified Parietoacanthial Projection The structures shown are:
This method demonstrates: ❍ Petrous ridges
❍ Zygomatic bone
❍ Facial bones with less axial angulation than with the
❍ Inferior orbital margin
Waters’ method
❍ Nasal septum
❍ Petrous ridges projected immediately below the infe-
❍ Mandible.
rior border of the orbits at a level midway through the
maxillary sinus
❍ Maxillary sinus Acanthioparietal Projection ‘Reverse Waters’
❍ Zygomatic bone Method’
❍ Inferior orbital margin
❍ Nasal septum This method is used to demonstrate:
❍ Mandible ❍ Superior facial bones
754
Figure 48
A B C
55°
CR
35°
(A) Position of patient in parietoacanthial projection. (B) Upright radiograph. (C) Central ray
Figure 49
A B C
CR
37°
(A) Patient positioning in acanthioparietal positioning. (B) Upright radiograph. (C) Central ray
❍ Orbit the midsagittal plane is perpendicular to the plane of the

❍ Zygomatic bone IR (Figure 49B).
❍ Maxillary sinus
❍ Petrous ridge. Central ray
The central is directed perpendicular to enter the acan-
Position of the patient thion and centered to the IR (Figure 49C).
The patient is seated in upright position. The midsagittal The structures shown are:
plane of the body is centered to the midline of the cassette ❍ Superior facial bones
(Figure 49A). ❍ Orbit
❍ Zygomatic bone
Position of the part ❍ Maxillary sinus
❍ Petrous ridge.
The patient’s chin up is brought up. The extension of
the neck is adjusted so that the OML forms 37 with the Thus, the ‘forgotten’ techniques overcome the limitations
plane of the IR. The OML is made perpendicular to the of newer techniques in terms of economics and feasibility,
plane of the IR. The patient’s head is adjusted such that and venture should be undertaken to refresh the forgotten
755
techniques and bring them into use today as well. These

Figure 50
techniques can still be used as a standard procedure for
demonstrating various craniofacial structures.
PANORAMIC RADIOLOGY
Panoramic radiology is considered a curvilinear variant of

conventional tomography, in which a single tomographic
image of facial structures includes both maxillary and
mandibular dental arches and their supporting structures.
Panoramic radiography is derived from the word ‘pan-
orama’ which refers to an unobstructed or complete view
of a region in every direction. The term ‘orthopantomog-
raphy’ was coined by Paatero and Sairenji which is derived
from ortho—straight or correct, pan (abbreviation for pan-
orama), tomo—tomography is the imaging of depth of tis-
sue layers without the interference of tissue above and
below that plane of tissue.
Origin of Panoramic Radiology

In the early days of the development of panoramic radiol- Panaromic machine with the cephalostat attachment
ogy, various experiments were carried out on the placement
of the X-ray source (intraoral/extraoral and stationary/
moving source), placement of the film (adapted intraorally,
lingual to teeth, or placed extraorally by adapting the film Concepts of Panoramic Imaging
to the jaws) and patient’s position (stationary/rotating
Technique and imaging principles
chair).
Dr H Numata, a Japanese, in 1933, devised a technique 1. Patient is advised to remove all metallic objects in the
to image the jaws by placing an intraoral film and an head and neck region such as hair clips, nose rings,
extraoral source of X-rays with a slit collimator which ear rings, chains, spectacles, removable dental appli-
rotated about the patient’s head to image the jaws. In his ances, etc.
technique the patient was stationary. 2. Patient is instructed to wear a lead apron (Figure 51).
In 1943, Horst Beger devised a prototype of the pan- 3. An extraoral panoramic film (5 12 inches) is loaded
oramic machine where the film was adapted extraorally into a designated panoramic rigid plastic backed cas-
on the jaws and the source of X-rays was placed intra- sette of the same size (Figure 52). The loaded cassette
orally. is placed into the cassette carriage assembly.
In 1946, Dr YV Paatero, experimented with a stationary 4. Patient may either be seated or standing facing the
X-ray source and the patient seated in a revolving chair. radiographer (Figure 53).
He called this technique parabolography. 5. Patient is positioned such that his/her jaws are within
In the present day panoramic imaging techniques, the the imaginary focal trough.
patient is stationary and the X-ray source and the cassette This can be achieved by asking the patient to stand
carrying the film are rotated about the patient’s head. erect with the neck extended and the chin supported
The X-ray source and cassette drive/carriage assembly are by the chin rest (this helps in establishing the vertical
incorporated into a C-arm. These machines have an optional height). The anteroposterior position can be achieved
cephalostat attachment that can be used for imaging the by asking the patient to bite in the groove in the bite-
skull such as lateral cephalograms, Waters’ views, antero- block. The bite will also help in relieving the teeth
posterior view, submentovertex view, etc. (Figure 50). from occlusion thereby preventing any cuspal over-
Many of the panoramic machines have the option of using laps. Apart from these measures, the machines have
digital receptors such as charged-couple device (CCD) and inbuilt light-beam guides that can be used for orien-
photostimulable phosphor (PSP) system instead of film- tating the head of the patient to coincide with the
based technology. midline in the sagittal plane, ala tragal line and a line
756
Figure 51 Figure 53
Patient standing upright facing the radiographer
Individual wearing a lead apron devoid of metallic

objects in the head and neck region
Figure 54
Figure 52
Patient’s chin positioned on the chin rest and

Panoramic cassette 5 12 inches anterior teeth biting in the groove on the bite rod
of reference running along the corner of the mouth

Working principle
(canine) and ala of the nose (Figures 54 and 55).
6. Exposure parameters are set. Most panoramic machines In rotational panoramic radiography, the patient is sta-
are equipped to operate between 54–85 kVp, 1–16 mA tionary and the X-ray source (tube head) and the cassette
and 16 seconds exposure for adults and 14 seconds in carriage assembly (connected to each other via a C-arm),
the child mode. rotate around the patient’s head (Figure 56).
7. Patient is instructed to place his/her tongue against the As the C-arm rotates, the X-ray tube head and the cas-
roof of the mouth and remain still during the entire sette carriage assembly (Figure 57) rotate in the same direc-
exposure cycle. tion, whereas the film along with the cassette moves in a
8. The operator stands behind the lead screen and initi- direction opposite to movement of the tube head. The rate
ates the exposure by pressing the exposure timer switch of the film movement is adjusted such that it matches with
which is also referred to as the ‘dead man’s switch’ as the speed of the slit shaped beam imaging the patient.
the timer needs to be pressed for the entire duration of A narrow beam of X-rays (facilitated by a slit collimator
the 14- or 16-second cycle. within the X-ray tube head) sweeps across in a horizontal
757
Figure 55 Figure 57
Cassette carriage
assembly
Cassette with film
Cassette carriage assembly
Light beams showing the midsagittal plane and

ala tragal line
Figure 58
Figure 56
Center of
rotation
C-arm
X-ray
Film within
Cassette source
film-cassette
carriage X-ray tube carriage
assembly head
Illustration showing moving centers of rotation
X-ray tube head and cassette carriage assembly approximating the lingual aspect of the mandibular sym-
connected via the C-arm physis (Figure 58).
With the above understanding, in the initial few sec-
onds of exposure, when the X-ray beam is imaging the
plane around an intraorally placed invisible pivot (apparent TMJ on the left side the center of rotation lies near the
intraoral source) referred to as the center of rotation. lingual surface of the body of the mandible of the right
In the vertical plane, the X-ray beam sweeps beneath side. Futhermore, as different areas of the mandible are
the occipital area with a negative angulation of 4 to imaged, the center of rotation correspondingly relocates
7. conforming to an arc shape.
Unlike older panoramic machines where there was a Objects located between the center of rotation and film
fixed center of rotation, machines these days make use of are imaged well (real images) and those between the X-ray
continuously moving center of rotation thereby allowing source and center of rotation are blurred (ghost images).
the image layer to conform to the elliptical shape of the All objects that are in the midline such as the cervical
dental arches. spine appear as double images (pair of real images).
The moving centers of rotation if plotted can be The panoramic machine is calibrated in such a way
traced into a shape of two arcs meeting at the midline that the horizontal and vertical magnification is evenly
758
Figure 59 Effect of patient positioning in the focal trough on

the resultant image
Patient position in the focal Image characteristics

trough
Patient overbites on the bite The mandibular anterior teeth
block. The incisal edges of appear narrow and fuzzy and the
teeth placed way ahead of mandibular ramus and condyles will
the groove on the bite be superimposed by the image of
the cervical spine
However, this technique may be
Dotted line enclosing employed if the area of interest is the
imaginary image layer maxillary sinus and the nasal cavity
or focal trough
Patient’s anterior teeth way Anterior teeth appear horizontally
too short of the bite magnified and blurred
Patient is positioned with the The area in the mandibular anterior
Illustration showing focal trough chin tipped down too steeply region may appear blurred. Anterior
teeth in the maxilla appear elongated
and those of the mandible will appear
stunted
Moreover, the hyoid bone may appear
matched so as to produce a final proportionate image. This
superimposing on the roots of the
is achieved by adjusting the speed of the X-ray tube head mandibular premolars and molars and
with regards to the movement of the film. the TMJ region may not be imaged
A ‘smile-line’ is created
Concept of focal trough
Patient is positioned with the The occlusal plane in the resultant
The focal trough is also referred to as the image layer chin lifted up image either appears flat or as a
which is roughly elliptical in shape to conform to the mor- reverse curve. This has been referred
phology of the dental arches. The image layer is narrower to as a ‘sad line’ or a frown
toward the anterior teeth. Some authors describe the focal However, this technique of patient
trough as the ‘plane of acceptable detail’. positioning may be used to improve
It is a three-dimensional imaginary curvilinear zone, imaging of mandibular anterior teeth
in which objects when positioned, will be imaged well. All Improper midline orientation Posterior teeth out of the focal trough
objects beyond this image layer may not be imaged or seen of the patient within the focal will appear broad and exhibit proximal
as distorted and blurred images (Figure 59). trough overlap, whereas on the contralateral
When the object of interest is positioned well within this side the teeth will appear slender
image layer, the vertical and horizontal dimensions will Patient’s neck is slumped and A vertical opaque shadow is seen
match, resulting in a sharply defined image. not straight and extended obscuring the midline structures
Patient instructed to swallow This technique will prevent
and hold the tongue against superimposition of the pharyngeal air
the hard palate space over the roots of the maxillary
Characteristics of Ghost Images
anterior teeth
All objects that lie between the source of X-ray beam
and center of rotation are projected as ghost images.
These are seen as blurred images on the opposite side of Indications of Panoramic Radiograph
the radiograph away from the real image, similar struc-
1. Used a screening radiograph to evaluate the gross
ture as the real image, positioned at the higher level on
pathologies or developmental disorders affecting the
the radiograph when compared to its real counterpart
jaws and teeth.
and they are relatively more vertically blurred than
2. Patient’s with limited mouth opening or in individuals
horizontally. Common ghost images seen on the ortho-
who cannot tolerate the intraoral film (severe gag reflex).
pantomograph (OPG) are those of metallic objects such
3. Evaluation of multiple impacted or supernumerary teeth.
as ear rings, hair clips and anatomic structures such as
4. Evaluation of the eruption status of teeth.
the cervical spine, hyoid bone, angle of the mandible
5. Evaluation of the size and extent of bony pathologies
including the lower border, etc.
such as cysts, tumors, fibro-osseous lesions, etc.
759
6. Assessment of the fractures of the jaws, especially the The intensity of the X-ray beam exiting the body is
mandible (however maxillary and mandibular midline determined by:
fractures may not be appreciated).
a. The energy of the X-ray source
7. Evaluation of the TMJ (condylar pathologies such as
b. The distance between the source of X-rays and the
fractures, hyperplasia, degeneration and changes in the
detector
morphology of articular eminence).
c. The attenuation of the beam by materials in the object
8. Although OPG is not the ideal imaging technique for
being scanned.
visualization of the maxillary sinus, the floor, medial
and posterior walls can be appreciated. Although a single source and a single-detector array can
9. Gross evaluation of the interdental bone height and produce a single scan, the efficiency of the scanning sys-
pattern in periodontal disease. tem can be increased multiple folds by using multiple X-ray
10. Evaluation of styloid process. beams and equivalent number of detectors. Each scan pro-
duces a penetration or absorption profile. However, construc-
tion of the image requires profiles obtained at different
Disadvantages and Limitations angles through the patient under study. The X-ray source
1. Midline structures and the pathologies in the region and detector assembly are rotated around the patient
may not be appreciated because of the superimposition (3,600) to produce multiple profiles of the particular site of
of the cervical spine. interest.
2. Pathologies in the premolar region may be obscured The X-ray beam attenuation is collected in a grid like
because of the proximal surface overlap in that region. pattern called matrix. Each square in the matrix is made
3. Finer details such as incipient carious lesions, minimal up of a pixel (picture element), which represents the X-ray
interdental bone loss, trabecular pattern may not be attenuation of a small finite volume of tissue called voxel
appreciated as the radiograph has poorer resolution (volume element). The typical image matrix for most CT
compared to an intraoral radiograph. scanners is 512 512 pixels.
4. Panoramic radiograph cannot be taken in individuals Each pixel represents a calculation of the actual attenu-
with cervical spine injuries and gross maxillofacial inju- ation of the X-ray beam by constituents within the body.
ries that may limit ideal position into the focal trough. A number (CT number or Hounsfield unit [HU]) is desig-
5. Young children, mentally challenged and unconscious nated for each pixel corresponding to the degree of beam
patients may not be ideal candidates for this imaging attenuation (Table 7). The CT numbers generally range
technique. from 1000 to 1000. By convention, water is designated
the number 0. However, some scanners may differentiate
between CT numbers that range from 2000 to 6000,
SPECIALIZED IMAGING but most monitors may display only 256 gray-scales and
the human eye can perceive only 64 shades of gray.
Since the CT numbers represent attenuation or density,
COMPUTED TOMOGRAPHY (CT) the computer constructs an image by printing the numbers
It is also referred to as computed axial tomography, com-

puted tomographic scanning, axial tomography and com-
puterized transaxial tomography. Table 7 CT numbers for commonly imaged tissues
Godfrey Hounsfield and Allan Cormack were instru-
Tissue CT number
mental in the development of CT and were awarded the
Nobel Prize in Medicine in 1979 for their efforts. This Air –1000
radiographic technique blends the principles of thin layer Lung –200
radiography (tomography) with the use of a computer to Fat –100
synthesize the image (computed).
Water 0
CSF 15
Working Principle Blood 20
The computed tomographic image is initiated by a process Gray matter 40

called scanning. Beams from one or several small X-ray White matter 45
sources are passed through the body and intercepted by Muscle 50
one or more radiation detectors. These radiation detectors Medullary bone 300
produce electrical impulses that are proportional to the
Cortical bone 1000
intensity of the X-ray beam emerging from the body.
760
or by assigning different shades of gray to each number contacts the detector array, thus improving CT image
thereby transforming a string of numbers into an image. quality.
Computed Tomographic Scanner Assembly Patient support couch
Though CT scanners are available in different system con- The patient support couch helps in stabilizing the position
figurations, they all have the same basic components: of a patient during a CT scan. The couch must be made of
a low-molecular-weight material such as carbon filter to
1. Gantry ensure that the path of the X-ray beam is not altered before
2. Patient support couch or after it traverses the patient. The couch is motorized so
3. Computer that the movement of the patient for slice acquisition is
4. Control console. smooth controlled and reproducible. The angle of the patient
couch can be altered to capture images in different planes.
Gantry The angulation of the patient is reported as ‘tilt’ on the
The gantry is made up of the detector array, patient sup- printout of the image.
port couch, and the X-ray tube or source. The gantry can
be tilted up to 300. The facility to tilt helps in excluding Computer
structures from the scan that may degrade the final image
The rapidity of capturing the image, acquiring data, and
(e.g., metallic dental restorations).
larger matrix size (512 512) necessitates the use of high-
Detector array The detector is made up of multiple dis- speed computers.
crete cells or detectors. Each of these detectors acquires Modern CT scans require computers that can solve up
specific information for each slice of the scan and trans- to 30,000 equations simultaneously. The time it takes the
mits it to the computer. The present fourth generation CT computer to generate a visible image after data acquisition
scanners have up to 2,400 detectors. is termed ‘reconstruction time’. Reconstruction time for a
The detectors contain either a crystal scintillation single slice is usually about 1 second. These computers can
detector or a gas-filled detector. Commonly used scintilla- constitute up to one-third the cost of a CT scanner.
tion detectors are manufactured with cesium iodide (CsI),
bismuth germanate or cadmium tungstate (CdWO4). These Control console
solid-state detectors are coupled optically to a photodiode.
Solid-state detectors exhibit detection efficiency that The control console allows the operator to select the param-
approaches 100% but cannot be packed closely together. eters of the CT scan, view the image as they are being
Gas-filled detectors contain either xenon or a xenon– generated. Many consoles have two monitors so that the
krypton mixture. These gases have a high atomic number, technician and the radiologist can manipulate the image
are inert, and display minimal afterglow. The gas is con- as the data is acquired. Image data is stored into the com-
tained under high pressure in the detector array. The indi- puter so that it can be formatted later into a number of
vidual gas-filled detectors can be placed closely together; ways. Data is stored either on magnetic tapes or disks. Most
the gas, however, in the detector only produces approxi- CT images are viewed on a film. The electronic data from
mately 50% detector efficiency. Regardless of the material each view is transferred onto a film using laser cameras.
used to capture information, the spacing of each discrete The most common film format is 14 17 inches and may
detector coupled with their detection efficiency determines contain 4–15 images.
the efficiency of the scanner and the ultimate spatial
resolution.
Advantages of CT
X-ray source The X-ray source for the currently avail-
able scanners consists of an X-ray generator and an X-ray ❍ Multiplanar imaging—image acquisition in cross-
tube. The X-ray generator is designed to produce a high sectional or in other planes.
milliampere (up to 400 mA) beam at a nearly continuous ❍ Greater geometric precision—CT solves the problem of
rate. The large amount of heat generated through continuous superimposition by allowing the clinician to view a
beam production necessitates the use of a large rotating series of thin sections (1.5–10 mm) thick depending on
anode and fairly large focal spot. the anatomic site.
The X-ray beam is collimated before it traverses through ❍ Manipulation of the acquired image—the radiographic
the patient (pre-patient collimation) and at the detector contrast and brightness can be adjusted based on the
array (post-patient collimation). The pre-patient collimation requirement.
decreases the radiation dose to the patient. The post-patient ❍ Soft tissue imaging—helps in separating subtle tissue
collimation reduces the amount of scattered radiation that contrast differences (as low as 0.5%).
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❍ Helps in distinguishing objects of subtle differences location. Panoramic radiography, in which images of both
in density such as between blood and fat, blood jaws are obtained through the synchronous rotation of
and CSF. an X-ray source and image receptor around a stationary
patient can provide broad coverage of both jaws and teeth,
but without the anatomical detail available with intraoral
Disadvantages of CT radiography. Moreover, due to the distance between the
❍ Expensive. radiation source, object and image receptor, there is a
❍ Patient’s exposure to radiation—CT is considered a high magnifying factor associated with image formation, and
radiation dose technique (depends upon region imaged, projection geometry results in image distortion and a
number of slices, thickness of slice and kVp). For a marked overlapping of tooth crowns. In order to reduce
head scan the effective dose has been calculated as the level of image distortion and ensure that image quality
2–4 mSv and 5–15 mSv for a body scan. is not affected by ghost images, accurate preparation and
A mid skull dose for a PA view (PA skull) or Townes’ positioning of patients is needed. In contrast to both intra-
view is about 3–5 mGy, whereas a mid skull dose of oral and panoramic techniques, which by their nature are
about 34–55 mGy is seen with a CT. incapable of capturing information about the third dimen-
❍ Production of artifacts, especially when metallic resto- sion of teeth and adjacent structures, and can thus provide
rations are located in the plane of tissue being scanned. only limited information about lesion origin, size and
These streak type artifacts may obscure radiographic location, medical CT devices are able to provide 3D images
findings in the CT scan and render it useless for of the dentomaxillofacial region. However, high patient
diagnosis. doses, high cost and lack of availability precludes the rou-
tine use of medical CT in dentistry. In response to the high
demand for a technique that could provide 3D data at a
Uses of CT in Dentistry lower cost and with lower radiation doses than the con-
ventional CT used in medical radiology, CBCT was devel-
❍ Evaluation of the presence and extent of clinically sus-
oped and introduced specifically for dentomaxillofacial
pected pathology in the head and neck region includ-
imaging.
ing cysts, tumors and infections.
❍ Detection of the extension of disease process into the
paranasal sinuses, base of skull and orbit. Historical Background
❍ Determination of the location, extent and displacement
Computed tomography (‘tomography’ is derived from the
of maxillofacial skeletal fractures, including detection
Greek words tomos, meaning ‘slice’ or ‘section’, and graphia,
of subdural and epidural hematomas.
meaning ‘describing’) was introduced in 1972 by the engi-
❍ Salivary gland imaging.
neer Godfrey Hounsfield and the physicist Allan Cormack.
❍ Evaluation of potential implant sites using 3D image
In 1979, Cormack and Hounsfield, who had worked inde-
reconstruction.
pendently to develop CT, were jointly awarded the Nobel
❍ Evaluation of the components of the TMJ.
Prize in Physiology or Medicine for their work on the
❍ CT-guided fine needle aspiration biopsies.
tomographic principles used in computing the spatial dis-
❍ Virtual surgeries.
tribution of a physical quantity of an object examined
from different directions and transferring this data into
easily readable images. CT represented the first practical
DENTOMAXILLOFACIAL CONE-BEAM medical application of the tomographic principle in the
COMPUTED TOMOGRAPHY (CBCT) field of medicine, providing high-contrast images without
superimposition of adjacent anatomic structures. The first
Radiology has always been a tremendous asset in clinical clinical CT scanners, installed between 1974 and 1976,
dentistry. Intraoral imaging, whether digital or film, contin- were dedicated to head-imaging only, and ‘whole body’
ues to provide the best spatial resolution of any imaging systems became available in 1976. Several hours were
method currently available. However, because spatial infor- needed just to acquire the raw data necessary to produce a
mation is lost when it is collapsed into a two-dimensional single image ‘slice’, and several days were required to
(2D) image, in many cases, two or three intraoral radiographs reconstruct an image from this raw data. Great improve-
taken from different angles are recommended. The clinical ments have since been achieved in speed, patient comfort
diagnostic capacity of intraoral radiography is influenced and resolution. Today, more anatomy can be scanned in
by a number of variables, including beam angulation, less time, which helps to eliminate artifacts from patient
exposure time, receptor sensitivity, processing, viewing con- motion and provides excellent diagnostic image quality at
ditions, superimposition of anatomic structures and lesion the lowest possible effective doses.
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Figure 60
A X-ray source B X-ray source
Translation Translation
Rotation Rotation
Object
Object
Detector
Detector
C D
X-ray source
X-ray source
Object
Helical X-ray beam path

Detector Detector
(A) First generation. (B) Second generation. (C) Third generation. (D) Fourth generation. (E) Helical CT.
Evolution of CT scanners from the original design
CT scanners have developed over four generations from system was replaced by an innovation called the ‘power
the original design in which both X-ray source and detector slip ring’, which transferred electric power from a stationary
revolved around the patient to one in which a fixed circu- source onto the continuously rotating gantry. The innova-
lar array detects the remnant beam originating from a tion of the power slip ring led to the introduction in 1989
revolving X-ray tube. First generation scanners used par- of a helical CT, in which the patient moves continuously
allel pencil beams of X-rays for data acquisition and through the gantry while the X-ray source moves simulta-
required both translation and rotation of the beam source neously down and around the patient in the same direc-
and a single-detector apparatus (Figure 60A). Second gen- tion (Figure 60E) to acquire a continuous helix of data. By
eration scanners also operated in a translation-rotation mode, combining data acquisition with continuous movement of
but employed multiple detectors that captured a small, fan- the patient through the gantry during scanning, multiple
shaped beam in an attempt to reduce scan time (Figure 60B). image slices can be captured quickly, reducing exposure
With third generation scanners, scan time was further time and artifact motion. The most recent technological
reduced through the use of a single-detector arc in con- advancement in CT acquisition occurred in 1998, when the
junction with fan-beam X-ray geometry (Figure 60C), and single row of detectors was replaced with multiple rows
with the fourth generation design, stationary detectors were arranged along the long axis of the human body. This
distributed along a full circle to form a detector ring, with increased the width of the volume acquired in each rota-
only the X-ray source in orbit (Figure 60D). tion, resulting in a more efficient use of the X-ray beam.
From 1974 to 1987, CT scanners employed high-voltage Contemporary CT multidetector scanners possess detector
cables wrapped around an elaborate set of rotating drums arrays with between 4 and 512 rows of detectors. In current
and pulleys to transfer power to the X-ray tube. A rotating practice, most systems use a combination of helical and mul-
frame or gantry, would spin 360 in one direction to pro- tidetector technology; however, despite the reduced scan
duce an image or slice, and then spin 360 in reverse to times of multidetector helical CT (MDCT), radiation doses
produce a second slice. Between each slice, the gantry would are still relatively high, as are the costs.
come to a complete stop, and the patient table would be Over time, concerns over detector costs, radiation and
moved forward by an increment equal to the slice thick- acquisition time led to the development of CT scanners in
ness. In the mid-1980s, the elaborate X-ray cable-and-drum which the fan-beam is replaced by cone-beam geometry.
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First adapted for potential clinical use in 1982 at the Mayo surfaces that can be read on a computer monitor. The use
Clinic Biodynamics Research Laboratory, the initial interest of special algorithms allows not only conventional axial
in CBCT was focused primarily on applications in angiog- plane reconstructions, but multiplanar, reformatted 2D, 3D
raphy, radiotherapy and mammography. A practical cone- and panoramic reconstructions as well. Whereas fan-beam
beam algorithm for tomographic reconstruction of 2D geometry uses primary reconstruction of data to produce
projection data was first illustrated by Feldkamp et al who axial slices from which orthogonal planar images are gen-
instead of a fan-beam reconstruction formula, used a con- erated using secondary reconstruction, cone-beam geom-
volution back-projection formula to directly reconstruct etry uses multiple-basis data projections to secondarily
a 3D density function from a set of 2D projections. The reconstruct orthogonal images. Raw primary data consists
formula provided convenient computation with useful prop- of a series of single-projection images—individually referred
erties and relatively small errors in many practical appli- to as basis, frame or raw images—that resemble cephalo-
cations, and today, a modified Feldkamp algorithm is the metric radiographic images, only each image in the series
reconstruction algorithm most frequently used in dentomax- is slightly offset from the next. An image volume is usually
illofacial CBCT. Despite the fact that the original Feldkamp calculated and constructed from several hundred 2D basis
back-projection algorithm was published in 1984, CBCT images, the complete series of which is referred to as the
units dedicated to dentomaxillofacial radiology could not ‘projection data’. The number of individual images com-
be marketed for another 15 years because economic X-ray prising the projection data is determined by the frame rate
tubes, high-quality detector systems and sufficiently pow- (number of images acquired per second), the completeness
erful personal computers were unavailable. Eventually, in of the scan arc (generally 180–360) and the speed of rota-
1999, the first dentomaxillofacial CBCT (DMCT) unit, the tion. Figure 62 shows schematic diagram of cone-beam
NewTom DVT 9000, designed by Attilio Tacconi and Piero and fan-beam imaging geometries and secondary recon-
Mozzo and produced by QR, Inc. of Verona, Italy, was struction processes.
introduced in Europe and was followed in 2001 by the intro- When the X-ray beam passes through different layers of
duction in Japan of the 3D Accuitomo-XYZ Slice View tissues, the transmitted intensity will be equal to the sum
tomography system (J Morita Mfg Corp., Kyoto, Japan). of the attenuation values of the various voxels that lie in
A spate of revolutionary CBCT applications reached the the specific straight path of the X-ray beam. An average
dental market in the 2000s, marking the beginning of a attenuation coefficient value for each voxel in the section
new era in the field of dentomaxillofacial radiology. New is derived from the different rays surrounding the area of
technological specifications and settings include multiple interest. By calculating the attenuation value of each voxel,
field of views (FOVs) and voxels that can better address a it is possible to differentiate between various tissues in
variety of specific tasks and imaging which can be con- a slice based on their computed attenuation values. Most
ducted with the patient in supine, seated, or standing posi- visualization software offer a default presentation consist-
tions. There are also several hybrid machines offering CBCT ing of a series of contiguous, interrelated 2D images in three
imaging along with panoramic and cephalometric radiog- orthogonal planes (axial, sagittal and coronal). Figure 63
raphy. Table 8 provides specifications of some contempo- shows schematic diagram of axial, coronal and sagittal
rary CBCT systems, including product, manufacturer and planes and corresponding CBCT images. In contrast to
software name; detector type; patient positioning options; medical CT, in which data is usually transferred from an
FOV and voxel size options; effective doses according to FOV acquisition workstation to a separate console for format-
(for adults); and additional features. Figure 61A, B show, ting, with CBCT, both image acquisition and visualization
respectively, a large FOV CBCT unit (iCAT Next Generation, are generally performed on the same computer.
Imaging Sciences, Hatfield, PA, USA) with a seated patient
and a small FOV CBCT unit (Kodak 9000, Carestream,
X-ray generation
Rochester, NY, USA) with a standing patient. All units are
similar in size and shape to a panoramic machine. Medical CT systems utilize high-power X-ray generators
to acquire large volumes of data at high speeds, which
requires high X-ray energy. Typically, CT systems operate
Fundamentals of CBCT
in a range of 80–140 kVp, with a maximum power of
Whereas conventional CT scanners emit a fan-shaped X-ray 20–100 kW; however, these systems can only be operated
beam and require a stack of multiple slices to obtain a com- at maximum power for a limited time, usually from 30–60
plete image, CBCT systems operate by focusing a cone- seconds. CBCT systems operate at 80–120 kVp, with most
shaped beam on a 2D detector that performs one pass or units functioning at the lower end of this range, which
less around the patient’s head to produce a series of 2D does not differ substantially from the operating parame-
images. A cone-beam algorithm is then applied to this image ters of panoramic radiographic equipment. Although CBCT
dataset, allowing the operator to extract planar and curved and CT have similar focal spot sizes (0.5–0.8 mm), unlike
reconstructions of varying thicknesses in any orientation CT, many CBCT systems possess a stationary anode, with a
and to generate accurate 3D images of bone and soft tissue tube current generally within the range of 1–20 mA, which
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Table 8 Specifications of some contemporary CBCT systems, including product, manufacturer and software name; detector type; patient positioning options; FOV and voxel
size options; effective doses according to FOV (for adults); and additional features
Company Model CBCT image Patient Field of view Voxel size Estimated effective Software Extra features
detector positioning diameter/height options doses for adult (ICRP
2007)
Quantitative NewTomVGi Amorphous Standing Five FOVs from From 0.075 to 15 15 FOV (194 Sv) NNT viewer • Safe beam*
Radiology, CBCT silicon flat Seated 6 6 cm to 0.30 mm (Pauwels et al, 2012) • Pulse system**
Verona, Italy panel Wheelchair 15 15 cm FS: 0.3 mm†
Quantitative NewTom 5G Amorphous Supine Five FOVs from Ultra high: Studies are running NNT viewer • Safe beam*
Radiology, CBCT silicon flat 6 6 cm to 0.075 mm; • Pulse system**
Verona, Italy panel, 20 18 16 cm High: 0.15 mm; FS: 0.3 mm in standard
25 cm Low: 0.30 mm mode
FS: 0.15 mm in zoom
mode†
• Ergonomic, two stage
patient chair‡
J. Morita, Veraviewepocs CMOS flat Standing Veraviewepocs 3D 0.125 mm 73 Sv (with 8 8 cm i-Dixel 2.0/One • Specifically designed for
Kyoto, Japan 3D and panel detector 4 4 cm; FOV) data viewer/ endo, perio and general
Veraviewepocs 4 8 cm; (Pauwels et al, 2012) One volume dentistry.
3De 8 8 cm viewer • Dose reduction feature
Veraviewepocs 3De
CBCT Pano/
4 4 cm;
Ceph options
4 8 cm
(Hybrid model)
J. Morita, 3D Accuitomo CMOS flat Seated Nine FOVs ranging From 0.08 to 43 Sv (with the i-Dixel 2.0/One High-quality images with

Kyoto, Japan 170 panel detector between 4 4 cm 0.250 mm smallest FOV); 50 Sv data viewer/ low dose
CBCT and 17 12 cm (with 10 5 cm FOV) One volume
(Pauwels et al, 2012) viewer
Imaging I-CAT Classic Amorphous Seated 16 13–22 cm Between 0.2 and Standard scan 69 Sv Xoran Cat • Quick launch
Sciences, CBCT silicon flat 0.4 mm (Ludlow and Ivanovic, iCATvision InVivoDental and
Hatfield, panel, 20 2008) 3DVR Dolphin software
PA, USA 25 cm
Imaging I-CAT Next Amorphous Seated Standard: 16 From 0.125 to 87 Sv (with 16 13 cm) iCATvision 720 rotation possible with
Sciences, Generation silicon flat 6, 8, 10, 13 cm 0.4 mm (Ludlow and Ivanovic, InVivoDental extended FOV
Hatfield, CBCT panel, 20 Extended: 2008) 3DVR
PA, USA 25 cm 17 23 cm
Vatech, PaX-Duo 3D Amorphous Standing From 5 5 to 0.08–0.3 mm FOV (5 5 cm) EasyDent/Ez3D • Auto-switching system
E-WOO Pano CBCT flat panel Wheelchair 15 13.5 cm 42.52 Sv between sensors
Technology, accessible FOV (12 8.5 cm) • Pulsed scan**
South Korea 120.69 Sv
Provided from company.
Unpublished
(Contd...)
765
766
Table 8 Continued
Company Model CBCT image Patient Field of view Voxel size Estimated effective Software Extra features

detector positioning diameter/height options doses for adult (ICRP
2007)
Vatech, PaX-Reve 3D Amorphous Standing From 5 5 cm to From 0.08 to FOV 5 5 cm 12.16 Sv EasyDent/Ez3D • Metal artifact removal
E-WOO CBCT/Pano/ flat panel Wheelchair 15 19 cm 0.25 mm FOV (15 15 cm) • Pulsed scan**
Technology, Ceph accessible 71.47 Sv
South Korea Provided from company.
Unpublished
Kodak Dental Kodak 9000 CMOS sensor Seated 5 3.8 cm single 0.076–0.2 mm 5 3.8 cm Kodak Imaging • Focused small FOV
Systems, 3D & 9000c 3D with optical Standing 9 7 3.75 cm 19–40 Sv Software • Stitching#
Carestream, CBCT/Pano/ fiber Wheelchair stitched (Pauwels et al, 2012)
Rochester, Ceph
NY, USA
Kodak Dental Kodak 9500 Amorphous Seated Medium: 0.2–0.3 mm Medium FOV: 92 Sv; Kodak Imaging
Systems, FOV CBCT silicon flat Standing 15 9 cm large FOV: 136 Sv Software
Carestream, panel Wheelchair Large: (Pauwels et al, 2012)
Rochester, 18.4 20.6 cm
NY, USA
Planmeca Promax 3D Flat panel Seated 8 8 cm, 8 5 cm, 0.1, 0.2, 0.4 mm 30–306 Sv Romexis • Improved artifact removal
Oy, Helsinki, CBCT/Pano/ sensor Standing 4 8 cm, 4 5 cm (Qu et al, 2010) • Extraoral bitewing
Finland Ceph/ Wheelchair • Stitching#
Planmeca Promax 3D Flat panel Seated 5.5 5 cm to 0.1, 0.2, 0.4, Studies are running Romexis • Artifact removal
Oy, Helsinki, Max CBCT sensor Standing 23 16 cm; 0.6 mm • Full skull scan
Finland Wheelchair full skull: 26 23 cm
Soredex, Scanora 3D CMOS flat Seated From 6 6 cm to 0.13–0.35 mm Largest FOV: 68 Sv On Demand Specific ear, nose, throat
Tuusulu, CBCT Pano panel 14.5 13 cm (Pauwels et al, 2012) imaging possible
Finland
Soredex, Scanora 3Dx CMOS flat Seated From 5 5 cm to 0.1–0.5 mm Not available On Demand
Tuusulu, CBCT Pano panel 24 17 cm
Finland
MyRay, Skyview CBCT Image Supine FOV diameter: 0.17, 0.23, 0.33 Largest FOV: 87 Sv Myray skyVIEW Pulsed emission**
Cefla Dental Intensifier— 11, 15, 17 cm (Pauwels et al, 2012)
Group, Imola, CCD sensor
Italy
Sirona Dental Galileos Proprietary Standing 15 15 15 cm 0.15–0.3 mm 84 Sv Galaxis, Sidexis, Galelios surgical guides
Systems, Comfort CBCT Siemens Seated (Pauwels et al, 2012) Galelios Implant
Bensheim, Cephalometric Technology
Germany
*Safe Beam technology reduces radiation level according to patient size. **Pulse system that activates the X-ray source only when needed. †Generally CBCT systems have stationary focal spot (FS) size of
0.5–0.8 mm. ‡NewTom 5G allows the patient to be seated initially and then moved comfortably into a supine position. #Separate scans can be combined to obtain the whole arc.
Data is collected from different company sites and articles. Most features are optional and effective dose measurements vary between devices according to settings used.
Figure 61
A B
(A) Large FOV CBCT unit (iCAT Next Generation, Imaging Sciences, Hatfield, PA, USA) with a seated patient.
(B) Small FOV CBCT unit (Kodak 9000, Carestream, Rochester, NY, USA) with a standing patient
Figure 62
Cone-beam Fan-beam
X-ray source
X-ray source
Detector
Secondary
reconstructions
Detector
Primary
Basis projections reconstruction
Schematic diagram of cone-beam and fan-beam imaging geometries and secondary reconstruction processes
is much lower than CT and which reduces not only genera- from an image intensifier combined with a charge-coupled-
tor power, but also heat production. Finally, most CBCT device (II/CCD) detector, making them large and bulky,
systems generate a pulsed X-ray beam that coincides with and they tended to produce circular basis image areas
detector activation, which markedly reduces total scan time (spherical volumes) rather than rectangular ones (cylindrical
as well as heat production, which translates into lower volumes). Nowadays, most CBCT units use flat panel detec-
radiation doses to patients. tors (FPD) comprising a large-area pixel array of hydroge-
nated amorphous silicon thin-film transistors or in some
more recent cases, large, complementary metal oxide semi-
Detectors
conductor technology (CMOS) arrays. X-rays are detected
All CBCT systems utilize an area detector to capture and indirectly by a scintillator such as thallium-doped cesium
record images. Initially, CBCT detectors were configured iodide or terbium-activated gadolinium oxysulfide, which
767
Figure 63
Axial
Sagittal
Coronal
Axial
Coronal
Sagittal
Schematic diagram showing axial, coronal and sagittal planes and corresponding CBCT images
spatial-resolving potential than their X-ray intensifier/

Figure 64
charge-coupled device (CCD) predecessors. For each basis
image, the detector records incident X-ray photons, collects
a charge, and sends a signal to the computer. The speed
with which a detector performs this acquisition is called the
‘frame rate’ as mentioned earlier in the section ‘Fundamen-
tals of CBCT’. For most CBCT units, a full rotation usually
requires between 5 and 20 seconds (similar to or faster than
Anisotropic Isotropic panoramic radiography), so that each basis image is acquired
x=y≠z x=y=z and sent within milliseconds, and hundreds of basis images
are acquired within a single exposure rotation. FPDs have
limitations in their performance, including linearity of
response to the radiation spectrum, lack of uniformity of
response throughout the area of the detector, and bad-pixels.
The effects of these on image quality are most noticeable
at lower and higher exposures. In order to ensure that these
inherent limitations of FPD do not affect image quality,
periodic recalibration of detectors is required.
Voxels
A comparison of volume datasets obtained anisotropically A ‘voxel’ describes the smallest distinguishable box-shaped
(left) and isotropically (right) part of a 3D image. In CBCT imaging, voxels are isotropic
(equal in all dimensions) and range from 0.4 mm to as small
as 0.076 0.076 0.076 mm. Because voxels are isotropic,
covers the FPD silicon matrix. This scintillator converts images can be constructed in any plane with high fidelity.
the X-rays to visible light, which is registered by an array of In theory, CBCT can improve the spatial resolution of
photo-diodes that produce an electrical charge proportional high-contrast structures in any chosen viewing plane. This
to the incident X-ray energy. FPD arrays afford greater superior spatial resolution, i.e. the ability to discriminate
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Figure 65
A B C
4 × 4 cm 6 × 6 cm 8 × 8 cm
D E F
10 × 10 cm 14 × 10 cm 17 × 12 cm
CBCT images of a dry skull obtained using different FOVs of Accuitomo 170 (J Morita, Kyoto, Japan).
(A) 4 4 cm; (B) 6 6 cm; (C) 8 8 cm; (D) 10 10 cm; (E) 14 10 cm; (F) 17 12 cm
objects of different attenuation separated by very small detector size and shape, beam projection geometry and
distances, is one of the most attractive qualities of CBCT beam collimation, which limit radiation exposure to a particu-
imaging and is largely the result of FPD technology and lar region of interest. The availability of different FOVs makes
isotropic data acquisition. CBCT also reduces the negative it possible to select the most appropriate FOV for a specific
effects of partial-volume averaging, a characteristic of application. CBCT units are classified based on FOV size as
both conventional fan-beam CT and CBCT imaging that small-, medium-, or large-volume units. Because the amount
occurs when the voxel resolution of the scan is greater of X-ray scatter, or ‘noise’, decreases with decrease in FOV,
than the spatial resolution of the object to be imaged. In small-volume units tend to offer the highest image resolu-
such cases, the pixel is not representative of either the tis- tion. Small-volume units are used to scan either a sextant,
sue or the boundary, but is given a weighted average of quadrant or single jaw, medium-volume units are used to
the different CT values. Figure 64 shows a comparison of scan both jaws, and large-volume units are used to scan the
volume datasets obtained anisotropically (left) and isotro- entire head. Because larger FOVs result in higher effective
pically (right). radiation doses, as a rule, smaller FOVs are recommended
for dental imaging, with the use of larger FOVs restricted to
cases where a wider view is required, such as orthodontic
Field of view
and orthognathic surgery treatment planning. Figure 65A–F
Field of view (FOV) is the term used to refer to the scan shows CBCT images of a dry skull obtained using different
volume of a particular CBCT unit. FOV is determined by FOVs of Accuitomo 170 (J Morita, Kyoto, Japan).
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Table 9 Advantages and disadvantages/limitations of CBCT in routine clinical practice
Advantages Limitations/disadvantages
Lower radiation doses than conventional CT and beam collimation Higher radiation doses than 2D imaging
Lower time, space and cost requirements than conventional CT Less soft tissue discrimination than medical CT
Greater hard tissue definition than medical CT Limited bone density measurements
Interactive display modes applicable to maxillofacial imaging Scatter and artifacts created by metal subjects
Interactive treatment planning and computer-aided surgery Liability
Advantages and Disadvantages/ basis images acquired, resolution and reconstruction

Limitations of CBCT algorithm.
In clinical practice, CBCT possesses a number of advan- Greater hard tissue definition than medical CT One of
tages over medical CT, such as lower effective radiation the most attractive qualities of CBCT imaging systems is
doses, lower costs, fewer space requirements, easier image their high spatial resolution, i.e. their ability to discrimi-
acquisition, higher image accuracy and interactive display nate between objects of different attenuations separated by
modes such as mutiplanar reconstruction that are appli- only small distances. CBCT images possess sub-millimeter
cable to maxillofacial imaging. However, the disadvantages isotropic voxel resolution ranging from 0.4 mm to as low
of CBCT include higher doses than 2D imaging; the inabil- as 0.076 mm. This superior spatial resolution—a require-
ity to accurately represent the internal structure of soft ment for the accurate depiction of highly detailed small
tissues and soft tissue lesions; a limited correlation with tooth and osseous structures—is made possible by the iso-
HUs for standardized quantification of bone density; and tropic acquisition of the CBCT image detector.
the presence of various types of image artifacts, mainly those Interactive display modes CBCT data reconstruction
produced by metal restorations, that can interfere with the capabilities make it possible to reorient images generated
diagnostic process by masking underlying structures. In in axial, sagittal and coronal planes to realign the ana-
addition, liability issues related to CBCT remain unresolved. tomical features of the patient. CBCT software also offer
Table 9 shows advantages and disadvantages/limitations various digital enhancement tools, including zoom/mag-
of CBCT in routine clinical practice. nification, window/level, ability to add annotation and
real-time measurement free from distortion and magnifi-
Advantages cation. Three distinct categories of display modes are
Lower radiation doses than conventional CT CBCT available—multiplanar reformatted (MPR), ray-sum and
scanners provide adequate image quality for dentomaxil- volumetric images. MPR modes usually include oblique,
lofacial examinations while delivering considerably smaller curved planar, i.e. distortion-free ‘simulated’ panoramic
effective doses than medical CT. Although not available and serial transplanar, i.e. cross-sectional—image reforma-
on all CBCT systems, beam collimation, which limits radi- tion. Ray-sum mode uses images of increasing sectional
ation to the area of interest, is a highly desirable function thickness to create an image slab representing a specific
that reduces doses by adjusting the irradiated field accord- volume of the patient. Ray-sum images can be used to
ing to the FOV. In general, doses range from 13 to 82 Sv for generate simulated projections, such as lateral cephalo-
small and medium FOV CBCT, compared to 474–1160 Sv metric images, that lack the magnification and distortion
for medical CT. To ensure that optimal doses are achieved, of conventional X-rays. However, the technique requires
diagnostic and image quality requirements should be eval- the use of the entire volumetric dataset, and interpretation
uated on a case-by-case basis and appropriate exposure suffers from ‘anatomic noise’, i.e. the superimposition of
parameters and FOVs selected accordingly. multiple sections. Volumetric rendering includes two dif-
ferent techniques by which 3D data can be visualized by
Lower time, space and cost requirements than conven- integrating and selectively displaying large volumes of
tional CT Dental CBCTs are compact (4 m2), easy to adjacent voxels. Whereas indirect volume rendering (IVR)
operate, reasonably affordable and require relatively low involves a complex process that requires the selection and
maintenance, making them a suitable choice not only for graphic representation of a range of gray-scale intensities at
hospitals and medical centers, but also for many dentists the voxel level, direct volume rendering (DVR) is a some-
in private practice. Scan time is comparable to that of what simpler process in which an arbitrary threshold of
panoramic radiography, varying anywhere from approxi- voxel intensities is selected and all gray values below or
mately 1–20 minutes, depending upon FOV, number of above this threshold are eliminated. The most common
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Figure 66
A B
C D
E F
CBCT image taken with (iCAT Next Generation, Imaging Sciences, Hatfield, PA, USA): (A) Axial view; (B) Simulated panoramic;
(C) Volumetric 3D representation of hard tissue; (D) Serial cross-sectional images; (E) Right generated cephalometric (MIP);
(F) Left generated cephalometric (MIP)
DVR technique is maximum intensity projection (MIP), Interactive treatment planning and computer-aided
which produces visualizations by evaluating the value of surgery CBCT datasets can be exported in standardized
each voxel along an imaginary ray projected from a par- 2D or 3D file formats for visualization and further pro-
ticular volume of interest toward the eye of the observer, cessing with third-party software. This software, as well as
selecting the highest value as the display value and elimi- software produced by CBCT manufacturers themselves,
nating voxel intensities that are below an arbitrary thresh- have increased the clinical applicability of CBCT by, for
old. Figure 66 shows simulated panoramic and serial example, simulating implant placement, bone grafts and
cross-sectional images along with volume rendering orthognathic surgical procedures in a true and accurate
images. Figure 67 shows the comparison of CBCT ray-sum virtual environment. The fusion of CBCT images with optical
generated simulated lateral cephalometric image and lat- datasets obtained with 3D optical cameras is a relatively
eral view of volumetric rendering with superimposition of new concept that provides a digital cast with an accurate
airway space of the same patient (images created using surface to be used or transferred to therapeutic applications
Invivo software [Anatomage, San Jose, CA]). via CAD/CAM (computer added design/computer added
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Figure 67
A B
Comparison of CBCT ray-sum generated simulated lateral cephalometric image (A) and lateral view of volumetric rendering with
superimposition of airway space of the same patient (B). Images created using Invivo software (Anatomage, San Jose, CA)
Figure 68
Implant placement procedure by combining optical digital impression CEREC and Galileos CBCT images
(Sirona Dental Systems, Bernsheim, Germany)
allows for more effective planning and prediction of treat-

manufacturing). Eventually, greater simplification and auto-
ment outcomes.
mation can be expected to lead to more precise dental
restorations that can be custom-fit to an individual patient.
Disadvantages/limitations
Figure 68 shows an implant placement procedure by com-
bining optical digital impression CEREC and Galileos CBCT Higher radiation doses than 2D imaging CBCT doses,
images (Sirona Dental Systems, Bernsheim, Germany). while lower than those from conventional CT, are still sig-
Several CBCT units are also now capable of integrated facial nificantly higher than those from conventional dental
scanning, whereby CBCT acquisition is accompanied by a radiography. Differences in CBCT device, FOV, exposure
concomitant 3D laser scan of facial soft tissue that is then parameters (kVp, mA) and other technical factors result in
overlaid on the bony skull image; the availability of fully substantial differences in radiation doses. Dose is strongly
integrated, accurate 3D information of both face and bone related to FOV, which varies according to indication.
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Figure 69
A B
(A) Axial CBCT and (B) MDCT images. Arrows show definitely higher soft tissue contrast in
MDCT image compared to CBCT
According to International Commission on Radiological systems in the market are typically described as having soft
Protection (ICRP) 2007, effective doses range from 19 to tissue contrast discrimination of approximately 10 HU, the
368 Sv, which is higher than the effective doses from numeric information contained in each pixel of a CT image
full-mouth radiographs (FMX) taken with photostimulable which represents tissue density, whereas modern medical
phosphor (PSP) storage and F-speed film with rectangular CT scanners have contrast resolution of 1–3 HU. This limited
collimation (34.9 Sv), FMX taken with PSP and F-speed contrast resolution remains a barrier to the extension of
film taken with round collimation (170.7 Sv) and pan- CBCT technologies into diagnostic imaging, which requires
oramic (CCD) (14.2–24.3 Sv), posterioanterior cephalo- the ability to detect the internal structure of soft tissue.
metric (PSP) (5.1 Sv) and lateral cephalometric (PSP) Figure 69 shows the difference between axial CBCT and
(5.6 Sv) radiographs. The largest contributors to effective MDCT images in soft tissue differentiation.
dose from CBCT are remainder tissues (37%), salivary gland
(24%) and thyroid gland (21%) tissues. Limited bone density measurements Radiodensity, mea-
Recent technological improvements have begun to lower sured in HU is inaccurate in CBCT scans. CBCT gray-scale
doses from CBCT. Most scanners now employ a pulse sys- values cannot provide reliable information on site-specific
tem, in which the X-ray source is activated only when bone density for purposes such as implant placement
needed, which is very useful in terms of radiation protec- because the X-ray attenuation of CBCT acquisition systems
tion. The use of newer, ‘smart-beam’ and ‘smart-sensor’ currently produces different HU values for similar bony and
technologies, in which the radiation level is set according soft tissue structures in different areas of a scanned vol-
to the patient size, should also be encouraged as a method ume (e.g. the image value of dense bone at the level of the
of radiation protection. Taking into account the higher menton differs significantly from the image value of the
radiosensitivity of children, it is imperative that the use of same bone at the level of the cranial base). In the absence
CBCT in children is fully justified over conventional X-ray of such standardization, it is difficult to interpret the gray
imaging. Needless to say, conducting ‘routine’ or ‘screening’ levels and impossible to compare the values resulting from
imaging before obtaining a medical history and perform- different machines. A method for establishing attenuation
ing a clinical examination is an unacceptable practice. coefficients from which actual HU values can be derived
from CBCT has been proposed; however, further research
Less soft tissue discrimination than medical CT ‘Dynamic is essential before this can be accepted. Despite its limita-
range’ refers to the range of incident signal intensities that tions in terms of bone quantity information, evaluation of
can be successfully detected and transmitted as image bone quality from CBCT images has a high correlation with
data. In general, the greater the dynamic range, the better structural analysis of dental implants.
the contrast resolution, i.e. the ability of an imaging sys-
tem to discriminate differences in tissue attenuation, which Scatter The amount of scatter generated and recorded by
varies according to the composition and nature of the tis- cone-beam image acquisition is substantially higher than
sue imaged. A low power configuration is often associated that of conventional medical CT. In medical CT, collimation
with increased noise and a smaller dynamic range, result- at the X-ray source restricts the z-axis coverage of the fan-
ing in lower contrast resolution. Currently available CBCT beam, allowing scatter (sinusoidal lines) from only a thin
773
axial volume of tissue to reach the detector elements during than in medical CT, mainly because of the lower kVp and
section acquisition. In contrast, CBCT expands the z-axis thus lower mean energy of the X-ray beam in CBCT sys-
coverage of the beam, allowing X-ray scatter generated tems. Figure 70 shows streak and beam-hardening artifacts
from the entire volume of coverage to reach the detector in CBCT images.
elements as the image is acquired. This scatter contribu- Patient motion is another source of image artifacts.
tion, expressed as scatter-to-primary ratio (SPR), can be as Motion artifacts appear as double-margins around a struc-
high as 3 in large-volume CBCT systems, compared to 0.2 ture and may increase with increases in scan time, which
in conventional medical CT systems. Higher amounts of can be as long as 20–30 seconds. In order to reduce the
scatter can reduce diagnostic capabilities by reducing image likelihood of motion artifacts, CBCT units, unlike medical
contrast and increasing both patient dose and image noise, CT, provide specific devices to stabilize the head during
the inhomogeneity of gray levels that occurs as a result of examination.
statistical fluctuations in the distribution of X-ray photons Nowadays, CBCT companies are actively developing
registered by the detector. artifact-reducing algorithms to be used during image
reconstruction. These processes, although contributory,
Artifacts A significant limitation to CBCT imaging is the
are rather slow and may add to the total reconstruction
presence of metallic artifacts, i.e. image flaws that are
time. More modern approaches attempt to avoid recon-
unrelated to the scanned object, which are caused by metal
struction errors either by supplementing missing or incor-
and amalgam restorations and, to a lesser extent, root-
rect information in projection images or by integrating
canal filling material and implants. Such artifacts include
some sort of meta-information into an iterative reconstruc-
streaks around materials as well as dark zones that affect
tion process. All of these methods, however, require mas-
the overall quality of the image. Streak artifacts appear as
sive computational power, which has so far prevented them
linear hyperdensities that radiate from a metallic object
from being used in daily routine work performed by com-
and may extend to the width of the field, affecting visual-
mercial scanners. Luckily, increasing computational speed
ization of areas even on the opposite side of an image.
and advances in graphics processing units can be expected
Beam-hardening artifacts, which appear as dark bands
to overcome this problem.
adjacent to high-density structures, may mimic disease.
Artifacts originating from root-canal filling material, for Liability CBCT machines are increasingly being mar-
example, may mimic root fractures, whereas dark bands keted specifically to orthodontists and implantologists or
around dental implants may mimic loss of osseointegration. dentists who place implants in private practices. Unlike
Artifacts may also occur as a result of beam-hardening, other advanced medical imaging systems, CBCT scanners
i.e. the preferential gradual absorption of lower energy X-ray are generally owned and operated by non-radiologists
photons as they traverse through layers of tissue, resulting who lack the training necessary to interpret CBCT images
in a gradual increase in the mean energy of the residual beyond the confines of their specialty or daily spheres of
beam. Beam-hardening may be more pronounced in CBCT practice. However, clinicians who order CBCT scans are
Figure 70
A B C
(A and B) Axial and (C) coronal CBCT images. Streak artifacts appear as linear hyperdensities that radiate from a metallic
object are shown with black arrows. Beam-hardening artifacts, which appear as dark bands adjacent to high-density
structures are shown with blue arrows
774
responsible for interpreting the entire image volume, given Oral implantology
the possibility that incidental findings—the likelihood of
The International Congress of Oral Implantologists,
which increase when a larger head volume is included in
Consensus Report cites four different areas in which CBCT
the scan—may have significant health consequences for
can be of use in oral implantology:
the patient. With the exception of individuals who have
completed a formal program in oral and maxillofacial 1. Diagnostics CBCT can be used in the identification
radiology, most orthodontists and other dental practitio- and evaluation of pathology, foreign bodies and defects.
ners do not have the necessary expertise to interpret CBCT 2. Implant planning CBCT images not only have been
scans, nor do they feel comfortable in doing so, yet there proven successful when used for linear measurement, but
is no informed consent process or signature waiver that also have been shown to provide reliable 3D information
would allow the clinician to interpret only a specific area for the assessment of relative bone quality and quantity, 3D
of an image volume. As a result, the clinician may be con- evaluation of ridge topography and pre-implantation
sidered liable for a missed diagnosis, even one that falls identification of vital anatomical structures such as the infe-
outside the area of his/her expertise. In case of any ques- rior alveolar nerve, mental foramen, incisive canal, maxil-
tions regarding image data interpretation, referral to a lary sinus, ostium and nasal cavity floor. This information
specialist in oral and maxillofacial or medical radiology is can be used in the treatment planning process to identify
recommended. suitable implant sites and to determine whether or not
there is a need for surgical procedures, such as sinus lifting
and bone augmentation. CBCT is also recommended in
CBCT: Clinical Applications sinus grafting operations as a mean of better predicting
Whereas early CBCT devices were dedicated to implantol- complications, thereby achieving better surgical outcomes.
ogy and dental imaging, today, applications extend to the Figure 71 shows curved and cross-sectional views used to
face and skull base as a whole. In fact, CBCT has largely assess implant sites and nerve canal in the mandible. CBCT
replaced conventional tomography for most dental diag- bone images can also be fused with soft tissue images
nostic tasks and is now commonly used for a variety of acquired with digital impression techniques to enhance
purposes in oral implantology, dentomaxillofacial surgery, planning efficiency (Figure 68).
image-guided surgical procedures, endodontics, periodon- 3. Surgical guidance CBCT images have yielded promis-
tics and orthodontics. ing results when used for surgical guidance. Commercially
Figure 71
Curved and cross-sectional views used to assess implant sites and nerve canal in the mandible
775
Figure 72
Stereolithographic guidance systems constructed for implant placement using implant software StentCad®
(Ay Tasarim Ltd., Ankara, Turkey)
available implant simulation software can be used to 1. Eruption problems CBCT images may help in identi-
process CBCT data to provide pre-operative views of ana- fying and localizing impacted or displaced permanent,
tomical structures in the jaw bone, and the use of a stereo- supernumerary or supplementary teeth, such as third
lithographic guide can ensure that pre-operatively planned molars and canine supernumeraries. In particular, CBCT
implant positions are accurately transferred to the surgical images can be used in the pre-surgical assessment of the
field. Figure 72 shows stereolithographic guidance systems relationship between third molar apices and the mandibu-
constructed for implant placement using implant software. lar canal. Figure 74 shows CBCT images taken from a
patient prior to surgical extraction of impacted third molar
4. Post-implant and/or post-grafting evaluation CBCT can
tooth.
be used to localize implants after placement; to assess bone-
implant interfaces; to evaluate demineralized bone and bone 2. Oral and maxillofacial pathologies CBCT enables the
transplants; and to identify peri-implant defects. However, surgeon to visualize oral and maxillofacial pathologic
it should be noted that metal artifacts caused by implants entities such as benign jaw bone tumors and cysts in three
may complicate assessment and measurement; moreover, dimensions, thereby assisting in diagnosis as well as in
keeping in mind concerns over dose, CBCT should only be planning appropriate treatment. Figure 75 shows CBCT
used if 2D techniques have been unsuccessful. Figure 73 images of well-defined cystic pathologic lesions.
shows cross-sectional CBCT images taken from patients
complaining of pain and discomfort in the implant region. 3. TMJ-related problems CBCT provides information
essential to the diagnosis of a variety of TMJ disorders,
including osteoarthritis, inflammatory arthritis, trauma
Dentomaxillofacial surgery
and developmental disorders. The ability to provide high-
Dentomaxillofacial surgery is important area in which 3D resolution multiplanar images with a lower dose than CT
views may become necessary, such as the following: is rapidly making CBCT the imaging modality of choice
776
Figure 73
A B C
Cross-sectional CBCT images of patients with pain and discomfort in the implant region that reveal
implants mistakenly placed in the maxillary sinus (A), mandibular canal (B) and out of cortical plate (C).
Please see arrows (Courtesy: Dr Ilker Cebeci, Tomoloji Dentomaxillofacial Radiology Center, Ankara, Turkey)
Figure 74
A B
C D
CBCT images taken from a patient before surgical extraction of impacted right third molar tooth. Conventional panoramic
radiograph showing a possible relation between right mandibular molar tooth and mandibular canal (A). CBCT generated
panoramic (B) and cross-sectional views show a relationship with third molar apex and mandibular canal (C)
for the evaluation of the osseous components of the TMJ. in adjacent structures, including the mastoid and the
It is imperative that the clinician evaluate not just the external and middle ear, and because pathological condi-
structures of the TMJ, but the entire CBCT volume, as TMJ tions such as impacted teeth, dental disease and paranasal
dysfunction may be accompanied by pathological changes sinusitis may mimic TMJ pain and result in misdiagnosis.
777
Figure 75
Patient 1
Patient 2
CBCT images of well-defined cystic pathologic lesions of two different patients
Figure 76 shows CBCT images of a patient with bone fracture, dental root fracture and anterior maxillary tooth
changes in TMJ. displacement.
4. Craniofacial fractures CBCT has been used to docu- 5. Tooth autotransplantation CBCT has recently been used
ment craniofacial fractures, including mandibular head to assist in tooth transplantation.
778
Figure 76
CBCT images of a patient with bone changes in TMJ. Images were reformatted in TMJ mode
Endodontics 3. Pre-, intra- and post-operative assessment CBCT can

support pre-surgical case planning by determining the exact
The use of CBCT in endodontics is increasing rapidly. Units
location of root apices in relation to adjacent anatomical
with small FOVs offering high-resolution images of teeth
structures. CBCT can also help in identifying endodontic
and related structures have been specifically recommended
treatment complications, such as overextended obturation
in cases where 2D systems have failed to provide sufficient
material, separated endodontic instruments, calcified canals
information regarding the following:
and perforations.
1. Root-canal morphology CBCT images can be used to
precisely determine root curvature and to definitively 4. Dentoalveolar trauma CBCT images can be used in the
identify accessory canals and other anomalies when con- diagnosis and management of dentoalveolar trauma, espe-
ventional imaging has suggested the presence of complex cially root fracture, tooth luxation and/or displacement,
tooth morphology. and alveolar fracture.
2. Periapical pathosis CBCT can assist in the diagnosis of 5. Root resorption CBCT can be used to localize and dif-
dental periapical pathosis in patients who present contradic- ferentiate between external and internal root resorption
tory or non-specific clinical signs and symptoms; who have and invasive cervical resorption and other conditions to
poorly localized symptoms associated with an untreated or identify appropriate treatment as well as prognosis.
previously endodontically treated tooth, with no evidence of Figure 77 shows CBCT images taken for endodontic
pathosis identified by conventional imaging; and in cases purposes.
where anatomic superimposition of roots or areas of the
maxillofacial skeleton are required to perform task-specific
Periodontics
procedures. CBCT can also support a diagnosis of pathosis
that is non-endodontic in origin, determines the extent of the CBCT can be used as a supplementary imaging technique
lesion and identifies its effect on surrounding structures. in situations where traditional 2D techniques have been
779
Figure 77
A B
(A) CBCT axial image showing a root fracture line and (B) CBCT sagittal image showing a periapical lesion with
bony reaction and maxillary sinusitis. Please see arrows
Figure 78
CBCT image of a cleft palate patient with eruption problems
unable to provide sufficient reliable information for peri- morphology directly influences treatment planning and
odontal assessment and treatment. CBCT may play a role prognosis. Specifically, CBCT is able to assess buccal and
in the assessment of marginal bone contours and 3D defects, lingual/palatal surface defects better, which are difficult to
especially furcations and infrabony defects, in which defect visualize in 2D imaging.
780
Orthodontics and their colleagues in 1946 and for this they were jointly
awarded Nobel Prize in 1952. Much later, the term ‘nuclear’
The use of CBCT in orthodontics has been gaining sub-
was dropped, it is now commonly referred to as magnetic
stantial popularity, although it is primarily recommended in
resonance (MR).
cases where conventional radiography cannot supply satis-
In 1971, Damadian noted that in vitro animal tumors had
factory diagnostic information. This can include assessments
elevated MR relaxation times when compared to normal
of cleft-palate patients, unerupted and supernumerary tooth
control tissue and he formed an apparatus and method for
localization, identification of root resorption caused by
detecting cancer in tissue.
unerupted teeth, evaluation of boundary conditions and
In 1973, Lauterbur published a paper and showed how
orthognathic surgery planning. CBCT imaging may also be
MR could be applied to imaging by applying a linearly
performed in other cases where it is likely to provide valu-
varying magnetic field across a liquid. Paul C Lauterbur is
able diagnostic information. Figure 78 shows CBCT images
known as the ‘Father of MRI’.
of a cleft palate patient with eruption problems.
In 1977, human Invivo images were demonstrated by
Mansfield and Maudsley.
Head and neck
In 1980, multiplanar imaging ability were first demon-
Depending on the FOV used, CBCT images may show part strated by Hawkes.
or all of the nasal cavity, paranasal sinuses, airway, cervical In 1992, functional MRI (fMRI) of the brain was intro-
vertebrae and temporal bone. In fact, specific ear, nose and duced by Ogawa.
throat imaging programs have been increasingly included
in CBCT systems, suggesting that CBCT may at some point
entirely replace medical CT imaging in certain otolaryn- Principles of Magnetic Resonance Imaging
gology-related applications. For example, an innovative 1. Nuclear magnetic dipole moment
C-arm CBCT system has been used in image-guided sur- 2. MR scanner
gery of the frontal recess; the technology is portable and 3. Proton magnetization
provides ‘near-real-time’ imaging to confirm and guide sur- 4. Resonance
gical treatment, thereby reducing the risk of disorientation 5. Relaxation
and iatrogenic injury. CBCT has also been found to pro- 6. Spin-echo phenomenon.
vide reliable and accurate 3D analysis of the upper airway
that can be of help in assessing the presence and severity Nuclear magnetic dipole moment
of obstructive sleep apnea (Figure 67). Imaging of the tem-
The individual protons and neutrons in the nuclei of all
poral bone represents another promising area for CBCT,
atoms possess a spin or angular momentum. In nuclei with
whose high-resolution and nearly artifact-free multi-planar
equal number of protons and neutrons with an electrical
reconstruction images make it possible to precisely assess
charge, a magnetic field is generated in nuclei with an
the intra-cochlear position of the electrode, including
electrical charge, a magnetic field is generated in nuclei of
visualization of each individual contact. It is this capacity
unpaired nucleons, causing these nuclei to act as magnets
for precision that makes CBCT a perfect candidate for the
with north and south poles (magnetic dipoles) and having
post-operative assessment and follow-up of cochlear implan-
magnetic momentum. The most common of these atoms,
tation electrodes.
the magnetic resonance active nuclei, are hydrogen, car-
bon, nitrogen, oxygen, fluorine, sodium and phosphorous.
Hydrogen atom is the most ubiquitous of these atoms in
MAGNETIC RESONANCE IMAGING the body. A hydrogen nucleus consists of a single unpaired
proton and therefore acts as a magnetic dipole. MRI is based
Magnetic resonance imaging (MRI) is a non-invasive method on magnetic dipole moment.
for mapping the internal structure within the body which
uses non-ionizing electromagnetic radiation and employs MR scanner
radiofrequency radiation in the presence of carefully con- The MR scanner consists of a magnet (Figure 79). Inside
trolled magnetic fields to produce high-quality cross-sectional the bore of the main magnet, there are three sets of coils
images of the body in any plane. installed to produce magnetic field gradient, one in the direc-
tion of the main field (the z-axis) and two perpendicular to
this (the x and y axes). The gradient field strengths over the
Historical Perspective
entire patient are less than 1% of the main field strength
The phenomenon of ‘nuclear induction’ later to be termed and can be rapidly varied in time. Inside the gradient coil
as nuclear magnetic resonance (NMR) was described inde- assembly, radiofrequency (RF) transmitter/receiver coil is
pendently but almost simultaneously by Bloch and Purcell mounted. Surface coil is placed directly on the surface of
781
Figure 79
Main magnet Gradient coils RF coil Couch
RF receiver/ RF pulse
transmitter amplifier
switch
RF pulse
generator
Main magnet X-gradient Z-gradient Y-gradient
power supply power supply power supply power supply
Central pulse Image
sequence controller processor
MR scanner
the body that improves the signal-to-noise ratio and the

Figure 80
resolution in the final image, but limits the volume that
can be examined. Patient is installed on a couch centrally B0
in the bore. Central pulse sequence controller operates the
Axis of precession
‘gradient coil power supplies and transmitter-receiver switch’
of the RF coil through the complex sequences used for the
various MR-imaging modes. Received radiofrequency sig-
nals are analyzed by Fourier transformation which are Axis of spin
spatially decoded in the image processor to be displayed as proton
an image, which is a map of the amplitude of radiofre-
quency signals emitted from small-volume elements, voxels,
in an imaginary slice of the patient.
Proton magnetization w
Proton exposed to a steady external magnetic field creates

External field
a force that will act on its magnetic dipole moment and
orient it parallel with the external field, but due to the Proton spin and precession
spin, it does not swing in as a compass needle would do.
Instead, it performs a maintained circular movement called
precession which has its own axis of spin and rotates at an
angle around another axis that is parallel with the exter- which is determined by the strength of the magnetic field
nal field like toy spinning top in the gravitational field and a constant. Relation between these two is expressed
(Figure 80). Magnetic dipole moment of the precessing pro- by the Larmor equation:
ton have magnitude and a direction expressed by a vector
F yB0
which is resolved in one component aligned with the axis
F precessional frequency,
of precession known as ‘the longitudinal component’.
B0 strength of the external magnetic field and
The second component which is oriented perpendicular
y gyromagnetic ratio unit of magnetic field strength-
to the external field and rotating with the frequency of
tesla (T).
precession is called ‘the transverse component’. Within a
magnetic field, all protons precess at the same frequency The precessional frequency of protons is 42.58 MHz/T.
782
Figure 81 Figure 82
Low energy state z
z Longitudinal net
magnetization vector
B0
External field x–y transversal vectors

cancel because out of
phase
High energy state
Illustration showing net magnetization vector of protons
Illustration showing proton spin levels
Larmor frequency is not exactly the same for all protons, level accompanied by a shift in the orientation of
but may differ by a few ppm (parts per million) depending their magnetic dipole moments. The magnitude of the
on the chemical bonds they have established. Larmor fre- longitudinal net magnetization vector as more protons
quency of protons in water and in aliphatic fatty acid chains shift to the high energy level. At a certain RF energy
vary. Such differences are designated chemical shifts. input, the longitudinal vector disappears. By further
input of RF energy, a surplus of proton is lifted to the
Proton spin levels When exposed to the external field, high spin-energy level, the longitudinal vector reap-
the spin of the proton may be at one of two discrete energy pears, but in the opposite direction.
levels. At low spin-energy level, longitudinal component ii. It forces the protons into coherent precession with the
of the magnetic vector points in the same direction as the appearance of transverse net magnetization vector which
external field. At the high energy level it points in the rotates with the Larmor frequency. The net magnetiza-
opposite direction (Figure 81). The fractional distribution tion vector is the resultant of longitudinal and trans-
of protons between these two states depends upon tem- verse magnetization vectors. Increase in RF energy input
perature and strength of external magnetic field. increases the longitudinal vector and decreases the
The difference manifests itself as a net magnetization of transverse vector. The angle between the direction of the
the population of protons in the material/tissue within the main field and net magnetization vector is flip angle.
field. Net magnetization vector is the statistical equilibrium
of a huge population of protons, constantly influenced by 90 pulse: RF pulse delivering energy just sufficient to tilt
thermal motion, and shifting between the two spin-energy the net magnetization vector into the transverse orientation.
levels (Figure 82). 180 pulse: RF pulse of twice 90 magnitude causes reap-
pearance of a longitudinal vector, but in the opposite direc-
Resonance tion, and will also cause the transverse vector to rotate in
When a body part or tissue is installed in a strong, steady the opposite direction relative to the main field. The dura-
and uniform magnetic field of the MR scanner, the equi- tion of such excitatory RF pulses is in the order of a few
librium state is represented by the net magnetization vec- milliseconds.
tor which is established within seconds. When disturbed
and shifted by a pulse of electromagnetic waves at the Relaxation
Larmor frequency of the protons (42.58 MHz in an IT field) When RF pulse is turned off, the excited protons return
entering perpendicular to the main field, only RF waves over a period of time to the initial equilibrium state, this is
(photons) of precisely this frequency will transfer energy called as relaxation. Recovery of longitudinal magnetiza-
by resonance to the precessing protons. This transfer of tion and the decay of transversal magnetization follow dif-
energy by resonance has two effects on the precessing ferent and independent time courses according to simple
protons: exponential functions but with different time conducted:
i. Protons at the low spin-energy level, having absorbed i. T1—Recovery of longitudinal magnetization
the energy of a RF photon, shift to the high energy ii. T2—Decay of transversal magnetization.
783
Table 10 Comparative features of T1 and T2 Table 12 Pulse sequence: basic features
T1 T2 Repetition time (TR) Echo time (TE)

This is the time at This is the time at which the induced Duration between repeat RF Time after application of RF pulse
which the longitudinal transversal magnetization has decayed pulses when the MR signal is read
magnetization has 63% of its maximum strength Time between pulse repetition Controls the amount of T2
recovered 63% of its determines the amount of T1 relaxation that has occurred when
equilibrium magnitude relaxation that has occurred at the signal is collected
Loss of energy whereby Loss of phase coherence between the the time the signal is collected
those protons that precessing protons has its origin in mutual
were lifted to the high magnetic interactions between the protons,
spin-energy level during and between the protons and local field in
Table 13 Difference between T1 weighted image and T2
magnetization give up homogeneities
weighted image
this energy and fall back
Thermal nature with Interactions between nuclei with different T1 weighted image T2 weighted image
a molecular basis in spins—‘the spin-spin relaxation time’ Emphasizes T1 value of Emphasizes differences in T2 values
random collisions with tissues of tissues
surrounding molecules, Pure liquids Solids
Accomplished by use of short Accomplished by use of long TR
collectively called ‘the • Mobile molecules • Molecules fixed
TR (300–700 ms) and TE (2,000 ms) and TE time (60 ms)
lattice-thermal relaxation • Intrinsic and local • Local intrinsic field
time (20 ms)
time’ or ‘the spin-lattice field variations are inhomogeneities
relaxation time’ rapidly fluctuating more permanent Fast T1 time, e.g. Fat-bright Short T2 time, e.g. Fat-dark
and tend to causing protons to Long T1 time, e.g. Water-dark Long T2 time, e.g. CSF/water-bright
T1 longer than T2 average out systematically To demonstrate anatomy To identify inflammatory/pathological
dephase changes
• Longer (seconds) • Short (milliseconds) • Other techniques allow the signal
from fat or water to be enhanced/
supressed
• ‘Fat saturation’—nulls the signal
Table 11 T1 and T2 relaxation times in a main field of 1.5 T from fat
Tissue type T1 time (ms) T2 time (ms)
Fat 240–250 60–80 The RF signal is analyzed and encoded to be displayed
Bone marrow 550 50 as an image. Only protons that precess in phase give rise
White matter of cerebrum 780 90 to detectable radiosignals. Thus, to detect differences in
Gray matter of cerebrum 920 100
T1 between tissues, and to fully exploit differences in T2,
complex excitatory pulse sequences are applied.
Muscle 860–900 50
CSF (water) 2,200–2,400 500–1,400 Radiofrequency pulses sequences The components are
set by the operator and determine the appearance of the
resultant image. Most basic features of a pulse sequence
The two relaxation processes reflect two types of interactions are depicted in Table 12.
between the precessing protons and their surroundings
Tissue contrast Tissue contrast is governed by intrinsic
(Table 10).
features of the tissues including proton density T1 and T2
The chemical shift (3 ppm) between protons of water and
times of the issues being imaged and how the TR and TE
fatty acids causes rapid decay of transverse magnetization
times are adjusted to emphasize these features, e.g. tissue
in tissues where fat and ‘watery’ tissue are intimately mixed,
has high proton density, strong transverse magnetization
e.g. in bone marrow. The mineralized bone tissue has few
vector at TE, strong magnetic resonance signal, appear
mobile protons which yield detectable T1 signals in diag-
bright on MR image. Conversely, low proton density low
nostic imaging. The concentration of protons detectable
transverse magnetization vector at TE weak signal dark on
by MR-imaging in a tissue is spin density/proton density.
MR-image (Table 13).
MR-imaging is directed at detection and visualization of dif-
ferences in parameters such as T1, T2 or the spin density
Spin-echo phenomenon
between different tissues and fluids within the body (Table 11).
During the period of relaxation of the magnetized tissues, Loss of phase coherence is called as ‘dephasing’ character-
an electromotive force can be induced in receiver coil as ized by loss of RF signal due to the spin-spin relaxation
an RF signal in synchrony with the precessing protons. (T2) which is an inherent property of the material/tissue.
784
Figure 83 Figure 84
B0 z
z
90° RF pulse
MZ y
y x
x w
Mxymax
w
A strong radiosignal emitting at the
Illustration showing proton magnetization vectors out of Larmor frequency
phase in the equilibrium state
Figure 85
z 180° RF pulse z
Mxymax
y y
Mz
x x
Mz
w w
Illustrations showing fanning out of the transverse component after the end of a 90 pulse and the application of
a 180 pulse reversing the longitudinal vector
The rate of decay of phase coherence (T2) is always faster

Figure 86
because of inhomogeneities in the magnetic field. The other
reason is due to ‘external’ disturbance of the measurement z
can be cancelled by the spin-echo manoeuvre (action) and
gradient-echos (reversal of the magnetic gradients). y
In equilibrium state, all the transverse (Mxy) compo- Mxy
nents of the proton magnetization vectors are out of phase Mz
(Figure 83). Sum of the longitudinal components (Mz) are x
aligned with the main field.
A 90 RF pulse flips the longitudinal vector into the
transverse plane and forces the transverse components of w
the proton magnetization vectors to precess in phase. The
single resultant Mxy vector is large and emits a strong
Illustration showing closing of the fan of vectors
radiosignal at the Larmor frequency (Figure 84).
After termination of the 90 pulse, the transverse
component begins to fan out due to small differences in precession faster precessing protons catch up with the
precessional frequency of the individual protons, i.e. T2 slower, i.e. the fan of vectors closes again (Figure 86).
relaxation. At the same time the longitudinal vector begins At time, TE, the transverse components of the proton
to grow up due to T1 relaxation (Figure 85). A 180 RF magnetization vectors regather and emit again a strong
pulse reverses the longitudinal vector and the direction of radiosignal.
785
MR-contrast Agents Figure 87

The relaxation times (expressed by T1 and T2) is shortened y
when paramagnetic substance is targeted to tissue, disturb-
ing admixture of strong magnetic dipoles due to the unpaired z
electrons in their atoms.
Gadolinium (Gd) chelated to DTPA—it is a rare earth
element which does not cross the normal blood–brain bar-
rier. This is used to detect defects in this barrier excreted in
the urine. It is utilized in urological MR-imaging. Using
additional ligands, Gd-chelates may be directed to biliary x
excretion. Agents based on manganese (Mn) having para-
magnetic properties similar to gadolinium have been
developed.
Gd and Mn based compounds are positive contrast agents
because of their influence on the relaxation constants, nota-
bly T1, is utilized to produce images (T1 weighted) where
Illustration depicting the scanner gradient
the MR signal strength is proportional to the concentration
of the agent.
Iron oxide particles effectively produce local field in
homogeneities. These are negative contrast agents which thickness of the slice location of the signal within the X
produce signal voids by strongly shortening T2. After IV and Y planes of the selected longitudinal plane derived by
administration, it shortens the relaxation time in liver, spleen switching off the Z gradient coil followed by rapidly turn-
and bone marrow, but not in tumors lodged in these tis- ing on the X and Y gradient coils. This sequence alters the
sues. This is used for gastrointestinal imaging. phase and precessional frequencies of the nuclei in the
selected slice. The resulting magnetic resonance signals
from the patient is read out while the frequency encoding
Methods for Obtaining Spatial (Tomographic) gradient is applied. The signal from the patient contains
Resolution of MR Signals many frequencies that is decomposed by the fast Fourier
The final MR-image is a square matrix of pixels contain- transformer into amplitude and frequency.
ing small-volume element called as voxel in an imaginary This information, which reflects the number of hydro-
‘slice’ of the patient. Each pixel is given a gray tone value gen nuclei and their T1 and T2 properties at each X and Y
proportional to the amplitude of the radio signal emitted location in the selected longitudinal plane is reconstructed
from the corresponding voxel in a defined period of time into MR images. The phase encoding gradient applied per-
following a sequence of RF excitations. pendicular to sIice selecting gradient is switched on for
short period of time (3–5 ms) after the excitatory RF pulse
has been switched off, produces a continuous change in
Scanner gradient
precessional phase across the slice, so that a particular
These are used to generate image that produce discrete slice phase derives from particular rows of voxels (horizontal
of tissue. It is accomplished by using two gradient coils row). The frequency encoding gradient is applied at right
which are required to modify the magnetic field surround- angles to both the slice-selecting gradient and the phase
ing the patient. X gradient refers to left to right, Y gradi- encoding gradient-switched on after the phase encoding
ant refers to anterior to posterior, Z gradient refers to head gradient has been switched off, and is maintained during
to toe (Figure 87). When one coil is turned on, it creates a the period where the RF signals are sampled establishes a
gradient in the intensity of magnetic field. Thus, in a 1.5 T continuous increase in precessional (Larmor) frequencies
scanner, when the Z gradient is turned on, the strength of from one edge of the section to the opposite edge, so that
the magnetic field at the head might be 1.4 T and that of a particular frequency derives from a particular row of
toe is 1.6 T. voxels across the slice (vertical row). The commonly used
When this gradient field is applied, the precessional fre- image matrix is 256 256 pixels. To achieve the same
quency of hydrogen nuclei will vary linearly along the mag- resolution in the X- and Y-directions, the image must be
netic variant. Accordingly, when the RF pulse is applied, constructed from 256 data samples, recorded with 256 dif-
only those nuclei precessing at the same frequency as the ferent settings of the phase encoding gradient which is the
applied signal will resonate. This allows selecting the desired main reason for the long data aquisition time in MRI com-
slice of tissue along the Z gradient slope of the gradient pared to CT imaging (Table 14). The amplitude is assigned
applied and the bandwidth of the RF pulse determine the a gray tone that is proportional to its magnitude and is
786
Table 14 Differences between CT and MRI

2. Congenital disorders: T1 weighted sequences with cor-
onal and axial images demonstrate abnormalities like
CT MRI cleft lip and palate.
Ionizing radiation Radiofrequency 3. Infections: AIDS-generalized cervical lymphadenopa-
thy with cystic lesions in the parotid.
Less expensive Expensive
4. Sinusitis: When complicated by serious condition like
Short time Long time
a tumor, venous sinus thrombosis or an intracranial
CI in pregnancy and children Can be used extension of the infection.
Suppression of image not Suppression of certain image 5. Benign tumors: Hemangiomas, lymphangiomas, neu-
possible possible rofibromas and schwannomas.
Measured in terms of density Measured in terms of intensity 6. Malignant tumors: Diagnosis, staging and monitoring
Two planes—axial and coronal Multiplanar—oblique view possible of malignant tumors affecting the head and neck region.
Indicated for trauma patients Not for trauma patients
7. TMJ: Articular disk perforations and disk displacements.
with acute bleeding
Applicable for fractures Not for fractures Advantages
Contrast used: Iodine, barium Contrast used: Gd, Mn
1. Best resolution of tissues with low inherent contrast.
For chronic stages For strokes, edema, initial stage of
2. Uses non-ionizing radiation—non-hazardous.
inflammation, cranial nerves, vessels
3. Accurate and rapid localization of intracranial patholo-
Between bones and soft tissues Between soft tissues
gies.
CI: Contraindicated 4. No streaking artifacts.
5. High-contrast images achievable.
Table 15 Image interpretation Disadvantages

Tissue T1 image T2 image 1. Some patients may complain of mild pain or tingling
Water Dark Very bright sensation.
2. Noise produced may cause discomfort to the patient.
Tumors Intermediate Bright
3. Contraindicated in patients with cardiac pacemakers.
Old blood Bright Bright
4. Not used in patients with ferromagnetic substances
Fibrous tissue Dark Dark implanted in the body.
Air Dark Dark 5. Expensive, not easily available.
Bone calcium Dark Dark 6. Room used should be free of ferromagnetic materials.
Fresh blood Dark Dark
Organs Intermediate Dark Common Artifacts in MRI
Fat Very bright Bright
Flow effect and movement artifacts in MRI
Artifacts occur due to flow in blood vessels and CSF.
Depending on the RF pulse sequences applied, the presence
displayed as the corresponding pixel in the image. High
of flow may give rise to weaker or stronger signals than
signal amplitudes appear white while low amplitudes
expected. Fast flow perpendicular to the section carry away
appear black on the gray tone scale. As in CT imaging,
those protons that should have given a signal during the RF
‘window width’ and the ‘window level’ can be varied.
signal sampling period. Blood vessel becomes ‘signal void’
and is displayed in black on the image. Protons with strong
Uses for MRI (Table 15) signals may be carried into the section by flow which dis-
turb the spatial X–Y encoding/decoding leading to artifacts.
1. For head and neck lesions: T1 weighted images— Wherever flowing blood is imaged the artifacts may be
defining anatomy of the lesion. T2 weighted images— present as streaks through a vessel, extending across the
assessing the invasion of the lesion into surrounding image in the direction of the phase encoding gradient.
structures.
Contrast enhanced MRI—extension of the lesion into
Artifacts in magnetic resonance image due to
muscles, brain or blood vessels can be studied. Coronal
motion and use of denture
scan-assessment of lesions involving the base of the
skull and the perineural extensions of tumors. See Table 14.
787
NUCLEAR MEDICINE Types of radiopharmaceuticals

1. Radioactive elements/compounds (Technetium-99m,
Nuclear medicine is a diagnostic radiation science utiliz- Fluorine-18, Iodine-131)
ing radioactive compounds or tracers having affinities for 2. Non-radioactive carrier compound with a radioiso-
particular tissues or organs in the body (iodine to the thy- tope (Gallium-67 labeled citrate, Iodine-125 labeled
roid gland); these are termed as target tissues. polyphosphate and Technetium labeled human serum
The radioactive agents are administered to the patient albumin).
either orally, intravenous or intrathecally. These agents
concentrate in target tissues and emit radiation. These are Labeling various phosphate compounds with 99Tc results in
detected and imaged by a variety of external detectors and the agents concentration in the skeleton hence used in bone
imaging systems. This helps in studying the target tissue scans. Labeling sulfur colloid with 99Tc gives an agent
under static and dynamic conditions. Such studies are called that is taken up by the reticuloendothelial cells of the liver
scintigraphic scans, scintiscans or radionuclide scans. and spleen, hence used in scans of the liver and spleen.
When these are utilized for studying bone they referred Technetium-99m methylene diphosphonate (Tc-99m
to as bone scans and when performed for salivary gland they MDP) is the most widely used radiopharmaceutical. It has
are termed as salivary gland scans. Other scans include a short half-life (6.5 h), hence there is minimal radiation
heart scans (to identify normal or abnormal blood flow to exposure and has superior physical and biological properties.
the heart muscle, measure heart function or determine the MDP is localized in bone by chemiabsorption onto immature
existence or extent of damage to the heart tissues after a hydroxy apatite crystal within newly formed inorganic
heart attack); thyroid iodine scans (to analyze the thyroid matrix and cannot be metabolized by the enzyme system.
function and show the structure of the gland. Larger doses
of radioactive iodine are used to destroy thyroid nodules
Bone Scanning
in the case of Graves’ syndrome); gallbladder or hepatobi-
liary scans (to evaluate both liver as well as gallbladder Procedure
function such as presence of gallstones); lung scans (to
Ten to fifteen millicuries of 99m-Tc labeled compound is
evaluate the flow of blood and movements into and out of
injected IV. A waiting period of 2–3 hours permits the com-
the lungs, as well as the determination of the presence of
pound to accumulate in the skeleton and to be removed
blood clots); Gallium scans (to evaluate infection and tumor),
from vascular and soft tissues via the urinary system. The
brain scans and gastrointestinal scans.
patient is then positioned under the gamma camera and
Nuclear medicine tests are extremely sensitive to abnor-
the desired bones are imaged. Whole body radiation dose
malities in body organs structure and function. Tests using
in bone scan is approximately 0.1–0.5 rad.
nuclear medicine techniques are more sensitive and specific
for disease detection than most tests because they identify
abnormalities very early in the progression of a disease, Working principle
long before the medical problem would be apparent with Following administration of the radioisotope, they reach the
other diagnostic tests. desired organs and emit gamma radiation, which is picked
In 1958, Hal Angler used scintillation camera for imag- up by the detectors placed outside the body within the
ing the entire system. In 1971, Subramanian and McFee used gamma cameras.
99m-Tc (Technetium) labeled MDP (methylene diphospho- Gamma cameras are devices used to image the radio-
nate) in bone scanning. isotope distribution in the body, within the field of view.
It consists of a sodium iodide detector of about 40 cm in
diameter. The detector assembly has a collimator which is
Radiopharmaceuticals similar to a grid in radiography. The gamma radiation emit-
ted by the patient passes through the holes in the collima-
These are radioactive agents used in nuclear medicine pro-
tor and reaches the detector, where they are converted into
cedures for imaging. They are produced using nuclear reac-
light scintillation.
tors and cyclotrons. Radiopharmaceuticals circulate and
The photomultiplier tubes convert the light into electric
concentrate to varying degrees in different organs through-
pulse which is then passed onto the computer. The com-
out the body. The localized tracer uptake is dependent on
puter constructs the distribution of the radioisotopes and
changes in regional blood flow and areas of altered bone
displays it on the monitor.
physiology and pathology which show an invariable alter-
ation in the osteoblastic and osteoclastic activity.
Interpretation of bone scans
The organs that receive the greatest exposure are referred
to as critical organs. For example, in bone scanning the blad- Abnormalities in bone are manifested in a scan mostly as
der, skeleton and bone marrow receive the highest exposure. areas of increased tracer concentration or as areas of very
788
limited uptake. Areas of increased tracer uptake seen Salivary Gland Scans
as hot spots and cold spots are areas of decreased tracer
uptake. In rare instances the entire skeleton will be dif- Procedure
fusely hot which is termed as super scans. Patient is advised to lie supine. Intravenously 40–150 mega-
In a normal scan the tracer will show bilateral, uni- becquerel (MBq) 99m-Tc pertechnetate is injected. For the
form and symmetrical distribution. The normal activity and next 25 minutes, sequential images are taken at 1 minute
uptake of the tracer depends on bone mass/density and intervals. A large field of view gamma camera equipped
bone stress in weight bearing areas. Soft tissue and the with a low-energy, high-resolution, parallel-hole collima-
urinary tract may show some background activity. tor is used. Roughly, 15 minutes following the procedure,
secretogogue like lemon juice may be given to enhance
Clinical applications of bone scans salivary secretion. The effective equivalent radiation dose
1. Trauma: Absence of tracer uptake even 24–48 hours to the patient is 0.4–1.6 mSv.
after an injury excludes a fracture. Bone scans can help
determine the age of a fracture. Clinical applications for salivary scans
2. Viability of bone grafts: An unsuccessful graft (non- Salivary scintigraphy may be used to assess obstructions
vascularized) appears as a cold spot. On the other hand, in the salivary ducts with or without parenchymal damage,
osteoblastic activity associated with a vascularized bone Sjogren’s syndrome, parenchymal impairment after radio-
graft is seen as a hot spot. iodine treatment in patients suffering from thyroid cancer.
3. TMJ changes: Early articular surface changes of the

TMJ can be determined. ULTRASONOGRAPHY
4. Osteomyelitis: Early detection (1–2 days of symptom
onset) of osteomyelitis is represented by the radionuclide The production of sound is the result of periodic changes
hyperactivity. in the pressure of air against the ear-drum. The periodicity
of these changes lie between 1500 and 20,000 cycles per
5. Metabolic bone disorders: Bone disorders such as second (Hertz). Human hearing is usually in the range of
osteomalacia, hyperparathyroidism and renal osteodystro- 20 Hz–20 kHz. Diagnostic ultrasonography uses vibratory
phy can be assessed. frequencies in the range of 1–20 MHz. Sonography comes
from a Latin word sonus meaning ‘sound’ and a Greek word
6. Detection of skeletal metastasis: Carcinoma of the
graphein ‘to write’. Sonography precisely means imaging
breast, lung and kidney which have a tendency for skeletal
with ultrasound. An ultrasound produces a visible image
metastasis appear as multiple hot spots on a bone scan.
of invisible organs inside the human body (Table 16).
7. Extraosseous localization: 99m-Tc MDP apart from local-
izing in the skeleton also localizes in several soft tissues
Principle
lesions such as muscle injuries, myocardial infarct, primary
breast lump and lung metastasis from osteosarcoma. Images with ultrasound are accomplished with pulse-echo
technique with the help of transducer. Ultrasound trans-
Advantages of bone scanning over conventional ducers convert electrical energy into ultrasound energy
radiography and vice versa. In ultrasonography, sound waves (pulse)
generated by the transducer emitted inside the patient will
A bone scan helps in detection of a lesion at the early
be reflected back (echo) at organ boundaries and within
stage (less than 5% demineralization of bone is adequate),
tissues depending on the composition of the matter. These
compared to conventional radiographs which require at
echoes then return to the transducer, where they are con-
least 30–50% demineralization of bone. Bone scans can
verted into electrical energy and then presented on the
depict the functional state of an organ or tissue whereas
display of sonographic instruments. The ultrasound instru-
conventional radiographic procedures detect the anatomical
ments processes the echoes and present them as visible
structure.
dots. The location of each dot corresponds to the anatomic
location of the echo-generating structure. Gray-scale
Limitations
image is achieved by different echo-strength and the echo
In interpreting bone scans of the skull and face, care arrival time is used to determine the depth of the structure
should be taken to avoid misinterpretation of activity in the that produced the echo. The ultrasound instruments pro-
mandible and maxillary alveolar ridges which are common cesses the echoes and present them as visible dots, which
sites of uptake due to the frequent dental infections and form the anatomic image on the display. Not all the ultra-
extraction sites. sound pulses are reflected back from any interface. Rather
789
Table 16 Terms associated with ultrasonography

2. A-scan images appear as spikes extending upward
from the baseline (point of contact of skin and the
Acoustic Product of density of the medium propagating the transducer is representative of the baseline). A-scans,
impedance sound and the velocity of propagation showing the relationship between amplitude and
Reflection The way ultrasound is reflected when it strikes an depth are used for measuring the distance between
acoustic interface is determined by the size and boundaries of tissues with different acoustic proper-
surface feature of the interface ties. These are used to distinguish between solid and
Refraction If the direction of sound changes as it crosses a cystic lesions.
boundary, then the transmission angle is different 3. M-scan used to show the motion of cardiac structures.
than the incidence angle It is a display form that presents depth versus time.
Attenuation Reduction in amplitude and intensity of sound as it Display of changes in echo is useful in evaluation of
propagates. It is the result of the combined effects rapidly moving structure like cardiac valve.
of absorption, scattering and reflection
Anechoic Without internal echoes; structure is fluid filled and
transmits sound easily Different Types of Scanners Used
Example: amniotic cavity, gall bladder
1. Linear scan These cross-sectional images have been
Hyperechoic Echo producing structure, reflects sound with a
produced with vertical parallel scan lines that are so close
brighter intensity
Example: gall stones, fat, bone
together that they cannot be identified individually.
Hypoechoic Low level echoes within a structure 2. Sector scan Each pulse originates from the same start-
Example: enlarged lymph nodes ing point, but subsequent pulses go out in slightly differ-
Isoechoic Very close to the normal parenchyma echogenicity ent directions from the previous ones. This results in a
pattern scan which is shaped like a slice of pie.
Example: muscles There is a combination of both too, where pulses origi-
nate from different starting points but each pulse travels
in a slightly different direction than the previous one.
most of the original pulse continues to be reflected back

from deeper interfaces. If process is repeated, but with dif- Instrumentation
ferent starting points for each subsequent pulse, a cross-
sectional image of the anatomy is built up. 1. Transmitter provides high amplitude voltage and con-
Velocity of sound depends on the medium. Sound is trols the rate of pulses emitted by the transducer.
attenuated through most bony structures thus velocity is 2. Transducers operate according to the principle of piezo-
faster in solids whereas it is slow in gas-filled structures electricity. This principle states that some materials
such as bowel because it impedes the sound transmission. (ceramics, quartz) produce a voltage when deformed
Contrast agent can also be injected into the circulation by an applied pressure. Piezoelectric crystal used in
to increase echogenicity. Contrast agent contain micro ultrasonography is lead zirconate titanate. When the
bubbles of gas and they produce strong echoes because transducer receives the electric impulses, the dipoles
impedence of gas is different from that of suspended liquid inside the crystals are realigned to the electric field
that enhance echogencity from perfused tissues in gray- causing the changes in the thickness of the crystal.
scale sonography and Doppler ultrasound. This leads to the formation of sound which is trans-
For many years, ultrasonography was limited to 2D mitted inside the patient. The echo reflected back to
cross-sectional scan, now it has been extended into 3D the transducers which change in the shape of crystal
scanning and imaging. This requires scanning the ultra- that results in the production of small oscillating volt-
sound through many adjacent tissue cross-sections to build ages that can be measured or recorded.
up 3D volume of echo-information. 3. Receiver and display—after amplification the echo
voltages are digitalized, i.e. they pass through analog-
to-digital converters (ADC). Electric information from
Different Types of Scans image processor drives the display, which produces a
visual image.
1. B-scan (gray-scale scan) images are produced by
scanning the ultrasound through the imaged cross-
Coupling agents
section (i.e. sending pulses through all regions of the
cross-section). B-scan converts the echo-strength into Even a very thin layer of air between the transducer and
the brightness of each represented echo on the dis- the skin surface reflects all the sound, preventing any pen-
play. (Hence B-scan or brightness scan.) etration into the tissue, thus an aqueous gel is applied over
790
the skin before application of the transducer which elimi- of the jugulodigastric and the juguloomohyoid lymph nodes,
nates the air layer and facilitates sound passage in and out which are larger and show axial diameter of 8–10 mm and
of the tissue. Coupling agent consists of: longitudinal diameter of 15–20 mm, normal nodes are dif-
ficult to detect because of their high echogenecity, which
1. Carbomer—10 g
is similar to that of the surrounding fatty tissues.
2. EDTA—0.25 g
3. Propylene glycol—75 g Reactive lymph nodes Enlarged reactive lymphnodes
4. Trolamine—12.5 g are most frequently encountered in head and neck, most
5. Distilled water up to 500 g. commonly the submandibular and lateral cervical nodes.
The typical sonographic appearance shows a longitudinal-
Doppler Ultrasound oval shape with rounded poles and smooth border. The
echogenecity is low and homogenous with hyperechoic
Echoes produced by moving objects have different fre- periphery. The axial diameter is usually less than 8 mm,
quencies than the pulses sent into the body. This is called whereas the longitudinal diameter can range from 15 to
the Doppler effect, which is put to use in detecting and 20 mm.
measuring tissue motion and blood flow. Color depends
on the flow of blood toward or away from the transducer. Inflammatory lymph nodes Shape of inflammatory
Colors are always opposite for artery and vein, so if artery lymph node is longitudinal or ovoid with rounded poles.
is red, vein will be blue or vice versa. This is used to see In addition, they may be round to spherical with smooth
tissue motion, obstruction and thrombosis. In addition, it can borders and display a hypoechoic appearance. The ratio
also detect direction, speed and character of blood flow. between longitudinal and transverse diameter of lymphnode
Color Doppler ultrasonography minimizes the need for is termed roundness index (RI). If RI 2 (longitudinal),
biopsy or fine needle aspiration cytology (FNAC). The it indicates inflammatory disease, whereas RI 1.5 (spher-
presence of blood flow signals in the center of node (this ical) favors metastatic involvement.
indirectly denotes the existence of the converging sinuses) In subatue inflammation, the lymph nodes tend to become
suggests that the node is benign. The presence of periph- smaller. In chronic inflammation, they are small, soft,
eral flow suggests a malignant nature (tumors larger than movable, slightly hypoechoic with smooth borders, and
a few millimeters in diameter stimulate the growth of new have longitudinal shape.
vessels). Specific lymphadenitis (tuberculosis) They range from
It has also been applied to automatic door openers and large, round, non-echoic to cystic necrotic nodes with blurred
to burglar alarms. borders. Fistulas appear as hypoechoic or non-echoic linear
stripes.
Main Indications for Ultrasound in the Primary lymph node disease
Head and Neck i. Sarcoidosis (benign)
ii. Hodgkin’s and non-Hodgkin’s (malignant)
Ultrasound is a very valuable tool in the diagnosis of the
head and neck. Optimal ultrasound examination of the Individual lymph nodes are round to oval and occasion-
superficial structures of the head and neck requires appro- ally appear as very large, non-echoic, simulating a cyst.
priate equipment with high-resolution small part trans-
ducers that make use of high frequency ultrasound. Lymph node metastasis Lymph nodes appear round to
Ultrasound sequence between 5 and 20 MHz is used. Either spherical, are hypoechoic but occasionally inhomogenously
the transducer is used directly on the skin or a silicon echogenic with loss of hilar definition visible. In case of
stand-off pad is placed between the transducer and the extranodular involvement, borders are poorly defined.
skin to get the ideal contact with the surface, especially in
the angle of the jaw and neck. A systematic examination 2. Salivary glands
protocol is mandatory for the evaluation of the head and Glands are typically homogenous echogenic structures.
neck. It begins with the examination of the thyroid gland Intraglandular and extraglandular ducts can only be visu-
where the instrument is adjusted. The examination is con- alized using high frequency, high-resolution transducers.
tinued along the vascular sheath to the floor of the mouth,
tongue, salivary glands and tonsils. Next step is the exam- Sialadenitis It reveals inhomogenous hypoechogenicity
ination of status of lymph nodes. which is more dominant in acute than in chronic inflam-
mation.
1. Cervical lymph nodes
Sjogren’s syndrome It reveals inhomogenous and hypo-
The majority of the normal lymph nodes in the head and echogenicity. In severe cases, multiple cystic lesions appear
neck show an axial diameter of 2–5 mm with the exception due to destruction of parenchyma.
791
Sarcoidosis of the parotid glands It reveals multiple ❍ Ultrasound-guided fine needle aspiration biopsy. Mainly
small hypoechogenic granulomatous nodules, which are used for detection of subclinical ipsilateral nodes. This
diffusely distributed throughout the gland. helps in accurate analysis of the size of nodes. 18-gauge
needle is used for FNAC.
Sialolithiasis It reveals echogenic complex with sound ❍ Determination of the relationship of vascular struc-
shadowing. Sialoliths which are smaller than 2 mm may tures and vascularity of masses with the addition of
not show shadow in sonography. Accuracy of sonography color flow Doppler imaging.
for diagnosis of sialolith is 80–94%. ❍ Detection of foreign bodies in soft tissues.
Sialadenosis It reveals homogenous, echogenic paren- ❍ Caries detection: The ultrasonic caries detector con-
chyma extending enlargement of the glands secondary to sists of a PC-controlled ultrasonic pulse receiver and a
tumor. probe (diameter 4.5 mm) attached to a handle. The dis-
play showed a correct waveform with a recognizable
Pleomorphic adenoma It reveals homogenous ultrasonic echo, and a thickness value appeared on the monitor.
pattern with decreased echogenicity and smooth borders. ❍ Measurements of the soft tissue thickness using an
But occasionally, tumor is hyperechoic with cystic areas of ultrasonic gingival-thickness meter could help practi-
calcification. tioners select the proper orthodontic miniscrew. The
Cystadenolymphoma It reveals hypoechogenic with meter’s monitor displays the soft tissue thickness.
❍ Assessment of blood flow in the carotids and carotid
smooth border. Hypoechogenicity is homogenous/inho-
mogenous with cystic areas with multiple septae. body tumor.
❍ Assessment of the ventricular system in babies by
❍ Evaluation of swelling of the neck, particularly those imaging through open frontenelles.
involving the thyroid, cervical lymph nodes or the
major salivary glands. Ultrasound is now regarded as
Advantages Over Conventional X-ray Imaging
the investigation of choice for detecting solid and cys-
tic soft tissue masses. ❍ Sound waves are not ionizing radiation.
❍ Detection of salivary gland and duct calculi. ❍ There are no known harmful effects on any tissues at
the energies and doses currently used in diagnostic
3. Primary soft tissue tumors ultrasound.
❍ Images show good differentiation between different
Hemangiomas and lymphangiomas Echogenicity is soft tissues and are very sensitive for detecting focal
variable and depends on the size of cystic compound. They disease in the salivary glands.
can be hyoechoic or isoechoic but usually are hyperechoic ❍ Technique is widely available and inexpensive.
compared to surrounding cervical soft tissues.
Cystic hygroma They appear non-echoic. Disadvantages
Lipoma Fat containing lipomas are relatively hypoechoic. ❍ Ultrasound had limited use in the head and neck
Fibrolipoma reveals high echogenicity with striated feath- region because soundwaves are absorbed by bone. Its
ery appearance. use is therefore restricted to the superficial structures.
❍ Technique is operator dependent.
Carcinoma Most frequent tumor is carcinoma of the
❍ Images can be difficult to interpret for inexperienced
tongue. It appears hypoechoic, inhomogenous, space occu-
operators because image resolution is often poor.
pying lesion with blurred borders. Sonograpy can evaluate
❍ Real-time imaging means that the radiologist must be
extension across midline and infiltration of the floor of
present during the investigation.
mouth, and relation of tumor to the mandible.
❍ Ultrasonic waves of high intensity ultrasound gener-
❍ Infections of tongue, floor of mouth. Abscess appears ate cavitation in liquids.
as circumscribed hypoechogenic or non-echogenic,
space occupying lesion with irregular border. Central
Artifacts
area of abscess contains fluid like pus, thus anechoic
and soft tissue remnants, blood vessels or foreign bod- 1. Section thickness: It results from beam width perpen-
ies appear echogenic. Aerobic bacteria may produce dicular to the scan plane. Echoes are received that
small bubbles of air, bright reflexes. originate not only from the center of beam but also
❍ Therapeutically in conjunction with the newly devel- from off center. Third dimension volume is visible.
oped sialolithotripter, to break up salivary calculi into 2. Reverberation (multiple reflection): It can occur between
approximately 2 mm fragments which can then pass the transducer and a strong reflector. Multiple echoes
out of the ductal system so avoiding major surgery. may be sufficiently strong to be detected and cause
792
confusion on display. The hyperechoic areas are sepa- 2. A set of lacrimal dilators (0000 through 0 caliber)
rated at equal interval. 3. Iodinated contrast agent (oil or water based)
3. Comet tail artifact: When the reflecting surfaces are 4. 5 ml or 10 ml syringe, guaze pads
closely spaced they appear in a form of comet tail. 5. Secretogogue to stimulate salivary flow such as fresh
Irregularities of the surface of the lung may cause lemon or 2% citric acid
transient comet tail artifacts. 6. Adequately focused lighting (a headlight unit)
4. Refraction: Refraction can cause a reflector to be 7. High powered magnifying glass.
positioned laterally (e.g. abdominal midline) produc-
ing two images of single object. The reflector is at 1 Preimaging assessment
but system places it at 2. One real structure is imaged
Patient should be asked about the following:
as two artifactual objects.
5. Ghost artifact: In transverse scan, sound rays are 1. History of allergy to iodine/contrast agents.
refracted at the muscle or fat interfaces in such a way 2. Medical history about medications.
that smaller structures in the abdomen or pelvis may 3. Previous salivary diagnostic tests and the results of
be completely duplicated. Artifact disappears if trans- the same.
ducer is angled. 4. Ask the patient whether any thyroid function tests are
6. Mirror image artifact: Air-filled lung, covered by vis- scheduled in the near future (absorption of iodine across
ceral pleura, causes a highly reflective beam into the glandular mucosa may interfere with thyroid function
chest. Thus, a sonography will produce pattern of bright tests, in such a case it is recommended that the sialog-
echoes (mirror image). raphy be deferred after the thyroid tests have been
7. Gliding sign: Longitudinal image with transducer directly performed).
on the ribs appear as curving bright interfaces that cast 5. Examine the ductal orifice, if purulent discharge is
dense acoustic shadows. The lung surface moves with evident then the procedure should be deferred until
respiration, thus appear as gliding sign in sonography. the condition has resolved.
Preimaging instructions to the patient

SIALOGRAPHY
1. Explain the procedure to the patient.
Sialography is the retrograde injection of contrast agent 2. Patient is told to expect a sense of fullness or pressure
(usually iodine based) into the ductal system of a salivary within the gland during the procedure.
gland. In 1925, Barsony introduced the technique of inject- 3. The patient should be instructed to indicate to the
ing a radiopaque medium (20% potassium iodide) into the examiner by means of a predetermined signal, when the
ductal system of salivary glands. injection of the contrast agent is becoming uncom-
fortable (this is the accepted end point for injecting
the contrast agent).
Indications of Sialography 4. Patient should be informed to expect a mild amount
1. To assess the ductal architecture of the salivary glands of discomfort and swelling of the gland in question,
to assess developmental disturbances. which usually subsides in 24–48 hours.
2. Detection or confirmation of small parotid or sub-
mandibular gland sialoliths or foreign bodies. Technique
3. Evaluation of the extent of irreversible ductal damage 1. Patient is made to lie down in the supine position.
present as a result of chronic infection. 2. Scout radiographs, comprising PA view, PA puffed
4. Differentiating between diseases such as chronic sial- cheek view and lateral views are taken for the parotid
adenitis, Sjogren’s syndrome and sialosis. gland and for the submandibular gland; AP view,
5. Evaluation of fistulas, strictures, diverticula, commu- lateral oblique view and a submentovertex view are
nicating cysts and ductal trauma. taken.
6. Rarely, as a dilating procedure for mild ductal stenosis. The scout radiographs are taken for two reasons:
7. Evaluation of masses within the submandibular or (i) to evaluate for any large calcifications within the
parotid salivary gland in conjunction with a CT scan. salivary glands and they are used as base radiographs
against which the sialograph can be compared.
Procedure 3. The salivary duct orifice is located.
4. Lacrimal dilators are used to dilate the salivary duct
Armamentarium
orifice.
1. Sialographic cannulas (Rabinov cannulas, cannulas 5. Cannulas are gently maneuvered into the orifice to
designed by Lowman and Bezella) locate the salivary duct. At this stage, asking the patient
793
to suck on freshly cut lemon will produce saliva aid- hazy (reflects the onset of acinar opacification). The ductal
ing in locating the duct orifice. phase of the normal parotid sialogram should demonstrate
6. Contrast medium is injected into the salivary duct. the main duct to be of uniform caliber extending from the
ductal orifice to the hilus of the gland. The intraglandular
portion of the parotid ductal system should demonstrate
Injection of the Contrast Medium a progressive arborization of the secondary and tertiary
Contrast medium can be injected by three techniques: ducts. This configuration is often described as a ‘leafless tree’
appearance on a sialogram.
1. Hydrostatic injection technique The ductal phase of the submandibular sialogram can
2. Distention injection technique be divided into two substages.
3. Hand injection technique. The first substage involves the filling of the deep portion
of the main duct; the second substage involves the filling of
Hydrostatic injection technique
the superficial segment perpendicular to the mylohyoid mus-
In this technique, a reservoir is used which contains the cle as well as the remainder of the ductal system. In con-
contrast media. This reservoir is placed about 70 cm above trast to the orderly arborization seen in the parotid duct, the
the patient’s head. This arrangement permits the constant submandibular ducts are often noted to terminate abruptly
perfusion of the ductal system with a water-soluble con- with poor or incomplete filling of the tertiary elements.
trast agent. This system provides for a uniform pressure
during injection without the examiner being physically Acinar phase
present in the room. This technique is useful in CT-guided
The acinar phase begins with the completion of ductal
sialography.
opacification and ends when there is a generalized increase
Distention injection technique in density of the gland. This phase was of special impor-
tance in the pre-CT era, when sialography was intended to
Distention sialography involves the hand injection of the demonstrate the presence of intraglandular and extraglan-
contrast media until the gland physically bulges. This usu- dular masses.
ally requires 2.5–3 ml of the contrast media (in normal cases
where routine hand injections are used 0.5–0.75 ml is used). Evacuation phase
This technique was popular in the pre-CT era to evaluate
and visualize small peripheral masses within the duct or The evacuation phase of a sialogram provides an estimate
the gland. This technique is not preferred any more. of the secretory function of the salivary gland as well as
demonstrating or accentuating ductal pathology that might
Hand injection technique not have been evident on other views.
The evacuation phase is divided into two subphases:
This is the most preferred technique for injecting the con-
trast media into the gland. The contrast media is injected a. Nonstimulated evacuation of the gland and ductal
gently using a steady constant pressure. The injection is system
performed under fluoroscopic observation and stopped b. Stimulated evacuation of the gland and ductal system.
immediately when the patient indicates that the injection The evacuation phase evaluated under intermittent fluo-
is becoming uncomfortable. Normally the parotid can roscopy for about one minute, while checking for sponta-
accommodate 0.5–0.75 ml of the contrast media and the neous clearing of the contrast agent from the gland. A
submandibular gland can accommodate about 0.5 ml of normally functioning, unobstructed gland should be able
the agent. to clear nearly the entire contrast agent.
The filling of the salivary duct and the gland can either be The second subphase is an evaluation of the glandular
examined under fluoroscopy in real time or routine radio- response to stimulation using a sialogogue or 2% citric
graphs can be taken after the contrast media is injected. acid drops placed on the tongue and intermittently moni-
toring the clearing of the contrast from the gland. The sec-
Phases of Sialography ond subphase is performed if a significant amount of
contrast is still present in the ductal system after the first
1. Ductal phase subphase. The nonclearing or partial clearing of the gland
2. Acinar phase may be due to a stricture, a sialolith or both or an underly-
3. Evacuation phase ing physiologic abnormality.
Post-stimulation views of the gland may demonstrate
Ductal phase
adequate clearing of the ductal system and intraparenchy-
The ductal phase begins with the injection of the contrast mal collections of contrast. Small 1–3 mm uniformly dis-
medium and terminates once the parenchyma becomes tributed collections of contrast agent may be seen as a
794
result of pseudo sialectases whereas irregularly distributed One of the more commonly used approaches involves injec-
or larger collections can result from abscess formation or tion of contrast material into the lower joint spaces, referred
tumor necrosis. to as lower joint space or single contrast arthrography.
Perforations of the disk or posterior attachment are
demonstrated by contrast material simultaneously flowing
Contraindications of Sialography into the upper joint space as the lower space is injected.
Another variation of the technique involves injecting con-
❍ Acute salivary gland infections or patients recovering
trast material into both the spaces and viewing the more
fom acute infections.
central portions of the joint with tomography. Because
❍ Malignant lesions involving the salivary gland.
contrast material is in both joint spaces, the outline of the
❍ Patient is allergic to contrast agent.
disk is profiled, showing its configuration and position.
❍ Patients who have to undergo thyroid function tests
The outline of the disk can often be enhanced by using dou-
during the period of sialography.
ble contrast arthrography. This technique involves inject-
❍ Pregnancy (high doses of radiation is used especially
ing a small amount of air along with a small amount of
for fluoroscopy).
contrast material into both joint spaces, producing a thin
coat around the periphery of both joint spaces that high-
lights the disk and the joint spaces.
ARTHROGRAPHY
Procedure
Arthrography involves injection of a radiopaque contrast
material into the joint spaces. The space occupied by the 1. The patient is placed on the fluoroscopic table in a
disk can then be visualized lying between the layers of lateral recumbent position with the head tilted on the
contrast material. tabletop. This allows the joint to project over the skull
Dr Fleming Norgaard, in 1947 was the first to success- above the facial bones in a manner similar to a tran-
fully use contrast arthrography. But it was in the 1970s that scranial radiograph.
Wilkes and others introduced and popularaized the tech- 2. Under fluoroscopic guidance the posterosuperior aspect
nique in the United States. of the mandibular condyle is identified with a metal
marker. This area is marked with an indelible pen.
Local anesthetic lidocaine is infiltrated into the super-
Uses of Arthrography ficial skin.
3. A 0.75- or 1-inch scalp vein needle and the attached
❍ Arthrography provides information regarding the soft
tubing is filled with contrast material and care is taken
tissue components, specifically the shape and position
to eliminate air bubbles. Air bubbles may simulate bod-
of the articular disk. It has been demonstrated that
ies within the joint space.
with the addition of tomography, the diagnosis of
4. In a direction perpendicular to the skin and X-ray beam,
abnormalities in the position and shape of the disk is
the 23-gauge needle is introduced in a predetermined
accurate.
region of the condyle with the jaw in the closed posi-
❍ Fluoroscopic observation of the injection may reveal
tion. Advancement of the needle is done under fluo-
the presence of adhesions, perforations and disconti-
roscopic observation to ensure proper positioning.
nuities in the capsule and provides a dynamic study of
5. When the condyle is encountered, the patient is instructed
disk movements, also any abnormal accumulation of
to open the jaw very slightly, and the needle is guided
joint fluid may be evident.
by the feel of the posterior slope of the bony condylar
❍ Synovial fluid sampling (arthrocentesis) and lavage
margin. On fluoroscopic observation, the needle will
of the joint can accompany the procedure of arthrog-
appear contiguous with the posterior condylar outline.
raphy.
6. Approximately 0.4–0.5 ml of contrast material is injected
❍ Arthrography assures a correct pre-operative diagnosis
into the lower joint compartment under fluoroscopic
of loose bodies (joint mice).
guidance. If the contrast is successfully placed into
❍ An arthrogram can clearly distinguish the synovial
the lower joint space, the opaque material will be seen
changes of an inflammatory arthritis from an internal
flowing freely anterior to the condyle in the anterior
derangement resulting from meniscal dysfunction.
recess of the lower joint compartment.
7. The needle is then withdrawn and fluoroscopic video-
tape images are recorded during opening and closing
Types of Arthrography
maneuvers of the jaws.
1. Single contrast arthrography 8. Spot radiographs are obtained during the fluoroscopic
2. Double contrast arthrography procedure.
795
Limitations periosteum of the condyle and as the joint is distended

with contrast material. This discomfort is transient in a
1. Direct medial or lateral displacements are difficult to majority of cases. If persistent joint pain occurs follow-
interpret with arthrography. ing the procedure, aspirin or acetaminophen and cold
2. Cannot be used when the disk is severely deformed. compress application to the affected side is recommended.
Complications
THERMOGRAPHY
1. The rare serious complications associated with arthro-
graphy include joint sepsis, allergic reaction to the It is also called thermal imaging or infrared imaging.
iodinated contrast medium and hemarthrosis. Thermography is a non-invasive diagnostic imaging pro-
2. Pain during and after the procedure, extravasation of the cedure that detects, records, and produces an image (ther-
contrast medium, disk perforation and transient facial mogram) of a patient’s skin surface temperatures and/or
paralysis are less serious complications of arthrography. thermal patterns (Figures 88 and 89). The procedure uses
3. The radiation exposure to the patient can be signifi- equipment that can provide both qualitative and quantita-
cant, depending on the duration of fluoroscopy and tive information on the normal and abnormal functioning
the number of tomographic exposures made. of the sensory and sympathetic nervous systems, vascular
4. The most frequent complication of the technique is the system, musculoskeletal system, and local inflammatory
extravasation of contrast medium into the capsule and processes.
soft tissues around the joint, causing pain. Nonionic There are presently two recognized techniques of thermal
contrast media will be the agents of choice to minimize imaging, namely, electronic infrared telethermography and
this discomfort. liquid crystal thermography.
5. Parotitis has been reported following arthrography with
large needles and cannulas.
Liquid Crystal Thermography
6. Some patients experience a vagal reaction, as a result
of increased anxiety during the procedure, this can be Liquid crystal thermography (LCT) utilizes a range of inter-
managed by administering 0.6 mg of atropine intra- changeable ‘screens’ impregnated with cholesteric methy-
venously. lester derivatives that change color as a function of their
7. Intravasation of contrast material infrequently occurs. temperature. The ‘screens’ are placed on the anatomic sur-
Epinephrine in a dose of 0.03 ml (1:1000) per 3 ml of face for development. A 35 mm or polaroid picture of the
contrast material is recommended because there is a image is taken for later analysis and archiving.
risk of an acute hypotensive episode with intravasa-
tion of higher doses.
Electronic Infrared Telethermography
8. Transient facial paralysis may result from a rapid infil-
tration of lidocaine. Some patients experience a mod- Infrared telethermography (IRT) equipment incorporates
erate degree of pain as the needle is placed on the single or multiple infrared detectors that survey the region
Figure 88
37.0
36.2
35.4
34.6
33.8
33.0
32.2
31.4
30.6
29.8
29.0
°C
Two examples of infrared images of the lateral aspect of the face. Courtesy: Prof Francis Ring, Head of Group,
Thermography, University of Glamorgan, UK
796
Figure 89
38.5° C
38
36
34
32
30
28
26
25.5° C
Infrared and visual image of a subject with an elevated body temperature. The color alarm clearly shows
the parts of the head with a temperature higher than 38C
to be studied in two directions simultaneously. The process must be free from drafts. Windows and doors should be
does not involve any contact with the surface of the skin. adequately sealed to prevent airflow in the area where the
patient is positioned. Incandescent lighting should not be
used during the examination due to the amount of infra-
Patient Preparation red radiation produced. Standard fluorescent lighting is
adequate. The temperature range in the room should be
❍ The patient should be instructed not to use lotions,
maintained between 18 and 23C. Room temperature tran-
creams, powders, makeup, deodorants or antiperspi-
sitions during the course of an examination must be grad-
rants on the body area to be imaged on the day of the
ual so that steady state physiology is maintained and all
examination.
parts of the body can adjust uniformly.
❍ The body areas included in the image should not be
The temperature of the room should not vary more than
shaved within 4 hours of the examination.
1C during the course of a study. The humidity of the room
❍ No physical therapy, ultrasound treatment, acupunc-
must also be controlled such that there is no air moisture
ture or hot/cold pack should be used 24 hours before
build up on the skin, perspiration, or vapor levels that can
the examination.
interact with radiant infrared energy.
❍ No exercise should be done 4 hours before the exami-
nation.
❍ Patient should not bathe for an hour before the exam- Indications
ination.
❍ The area to be imaged should remain completely uncov- Merla et al (2004) assessed the use of functional infrared
ered of clothing or jewelry. imaging in the diagnosis of the myofascial pain. They
concluded that functional infrared imaging seemed to dis-
tinguish healthy subjects from the patients suffering myo-
Procedural Requirements fascial pain.
Gratt and Anbar (1998) summarized the following clin-
The room should be of adequate size to maintain a uni- ical applications for thermography in dentistry:
form temperature. A room approximately 8 10 feet size
is adequate to meet these requirements. During the exam- 1. Evaluation of atypical odontalgia
ination, the patient should be positioned relatively equi- 2. Diagnosis of chronic orofacial pain
distant and adequately spaced from each wall. The room 3. Assessment of TMJ disorders (internal derangement of
should be carpeted. Curtains may be used to prevent out- TMJ)
side infrared radiation from entering the room. The room 4. Assessment of inferior alveolar nerve deficit.
797

SECTION Processing of
XI Radiographs and
Radiographic
Interpretation
29 Latent Image Formation 801

30 Processing of Radiographic Films 803
31 Radiographic Faults 812
799
Chapter-29.indd 799 10/3/2012 10:59:18 AM


CHAPTER
Latent Image Formation

Ravikiran Ongole, Praveen BN 29
Formation of a visible radiographic image is a three-stage 2. Interaction of the information carrying X-ray beam
process: with the photosensitive silver halide crystals in the
film (latent image formation).
1. Interaction of X-ray beams with the object of interest. 3. Chemical action of developing solutions which convert
the latent into visible image.
Figure 1
Bromide ion Sensitivity site
Silver ion
X-ray photon
Silver bromide
e-
e-
X-ray film
Interstitial
silver ions
e- e- -
e e-
e-
e-
e-
e- -
e
e-
Latent image formation. Courtesy: Dr Jaideep Shekhar
801
Section XI – Processing of Radiographs and Radiographic Interpretation
FORMATION OF LATENT IMAGE 3. As a result of Compton and photoelectric effects, elec-

trons are displaced from the bromide ions producing
To understand the formation of latent image, one needs to high-speed electrons (recoil electron) and scattered
know the composition of an intraoral film. A dental film X-ray photons.
comprises of an emulsion suspended in gelatinous vehicle 4. These recoil electrons travel within the silver halide
that is coated onto a plastic supporting base. crystal creating additional bromine atoms, scattered
The emulsion is composed mainly of photosensitive sil- photons and secondary recoil electrons until their
ver bromide crystals and to a lesser extent silver iodide energy is dissipated.
crystals and very small amounts of free silver ions inter- 5. These recoil electrons get trapped in the latent image
spersed in the crystal lattice (interstitial silver ions), iodide site and impart a negative charge to these sites.
ions (produce the physical irregularity in the array of silver 6. The free interstitial silver ions which are positively
and bromide ions) and traces of sulfur compounds, which charged are drawn toward the negatively charged
bind to the surface of the crystals. These sulfur compounds latent image sites.
along with the physical irregularities on the surface of the 7. Within these sites the silver ion is neutralized result-
silver halide crystal form the latent image sites. ing in an atom of sliver that gets deposited.
This process repeats multiple times at every single latent
site leading to the further deposition of silver atoms which
Steps in Latent Image Formation (Figure 1)
ultimately gives rise to the ‘latent image’.
1. X-ray photons pass through the object of interest and Once these radiographs are placed in the developer
carry information to the film. solution, the silver atoms in the latent image sites are con-
2. X-ray photons strike the film and interact with the verted to black metallic silver grains and the unexposed
bromide ions primarily (bromide ions are about one- silver halide crystals are removed by the clearing agent in
and-a-half times larger in diameter when compared to the fixing solution resulting in the formation of a ‘visible
silver ions). image’.
802
CHAPTER
Processing of Radiographic
Films
Ravikiran Ongole
30
Techniques of Film Processing Daylight Processor
➧ Manual Processing of Films Self-developing Film
Darkroom ➧ Automatic Processing
Visual Method Working Mechanism
Time–Temperature Controlled Method Composition of Processing Solutions
Following exposure to radiation, the latent image formed Ideal requirements

on the film is converted to a visible image by a technique
1. Darkroom should be spacious enough to permit at least
referred to as film processing. Radiographic films have
one person to work comfortably and should measure
been traditionally processed in a darkroom. However, over
at least 4 ⫻ 5 feet in dimension (Figure 1).
the years, techniques such as daylight processing, auto-
2. An ideal darkroom should have a doorless maze pat-
matic processing and self-developing films have eliminated
tern (Figure 2A) or a conch shell design (Figure 2B) to
the need to use a darkroom for processing films.
prevent entry of light into working area.
Techniques of Film Processing

1. Manual processing Figure 1
❍ Time–temperature method (darkroom technique)
❍ Visual method (darkroom technique)
❍ Daylight processing (darkroom not required)
❍ Self-developing films (darkroom not required)
2. Automatic processing.
MANUAL PROCESSING OF FILMS
Traditionally radiographic films are processed in a dark-

room. Before one learns the art of radiographic film pro-
cessing, it is essential to be familiar with the designing of
the darkroom and its contents.
Darkroom
Radiographic films are sensitive to X-rays and visible
light. In order to prevent exposure of the films to visible
light it is necessary to process the exposed radiograph in a
Photograph of a compact darkroom of adequate size
room specially designed to keep away visible light.
803
Figure 2
A B Conch shell
design
Working
area
Doorless maze pattern

Working area
(A) Illustration showing the doorless maze pattern of a darkroom. (B) Conch shell design for the darkroom.
Figure 3 Figure 4
Photograph showing the contents of the darkroom

such as the processing tanks, wash basin, drying racks,
film holders and safe light
Darkroom infrastructure (Figure 4)

An ideal darkroom should contain the following:
1. Processing tanks (master tank with developer and
Light tight door with a locking mechanism for the
fixer tanks)
darkroom 2. Safe light
3. Visible light source (tube lights)
4. Working area (to load extraoral film cassettes and
3. In the event of having a door it should be light tight and unwrap films)
should have provisions for locking to prevent acci- 5. Dryer
dental opening of the door, which might unnecessarily 6. Thermometer and stop clock
expose the image receptors to visible light (Figure 3). 7. Storage facility (to store unexposed films)
804
Chapter 30 – Processing of Radiographic Films
Figure 5
A B
(A) Master tank with water and containing the developer and fixer solution tanks.
(B) Master tank covered with a lid when not in use
8. Exhaust and appropriate ventilation the films. However, the visible lights may be switched on
9. Drying racks once the films are placed in the fixer tank.
10. Processing solution stirrer and film retrievers. The safe light should ideally be 15 W bulb in the orange-
red-yellow spectrum of light (Figure 5B) as these colors
Processing tank have the longest wavelength and low penetrating power
(X-rays are highly sensitive to blue and green colors).
The film processing tank is commercially available and
A red GBX-2 filter may be placed over the safe light.
made of inert plastic (RinnTM) or can be made custom-
One or two safe lights may be used based on the size of
made out of stainless steel or bricks and mortar (to prevent
the darkroom. One safe light over the processing tanks (ide-
reaction with the chemicals of the processing solutions).
ally on the right hand side or the fixer solution side of the
The processing tank should be placed at a convenient
tank) and one over the working area (place where the films
height and away from the working area where films are
are loaded). The safe lights should ideally be placed 4 feet
unwrapped or loaded into cassettes.
above the working area and processing tanks (Figure 6B).
Commercially available tanks comprise of a large mas-
ter tank and two smaller removable inserts (for the devel-
Floating thermometer and stop clock
oper and fixer solution) which are placed within the master
tank that contains water (Figure 5A). Many of the com- A floating thermometer (Figure 7A) and stop clock are
mercially available master tanks measure 8 ⫻ 10 inches used for the time–temperature method of processing films.
and the smaller tanks hold about 9 liters of the fixer and The thermometer helps to assess the temperature of the
developer solution. processing solutions so that the duration for which the
The master tank holds either running water or water film should be developed is known. The thermometer is
stored at room temperature. The temperature of the water left floating in the water in the master tank prior to the
in the master tank will help regulate the temperature of processing (Figure 7B). Alcohol containing thermometer is
the developer and fixer. The water contained in the master preferred as mercury containing thermometers may con-
tank is also used for rinsing the film during processing. A taminate the processing solutions in the event of a break-
lid should always be placed over the tank to minimize the age. The stop clock helps to set the exact time for the
oxidation of the processing solutions by atmospheric oxy- developing procedure.
gen and evaporation (Figure 5B).
Drying racks and film hangers
Safe light and visible light illumination
Drying racks should strategically be located above the
Visible light illumination can be in the form of ceiling or washing area in the darkroom. Once the films are removed
wall mounted light source (Figure 6A). However, care should from film hangers after drying, the empty hangers should
be taken to ensure that these tube lights are switched off be thoroughly rinsed and left to hang dry over the drying
during loading the films into cassettes or while processing racks (Figure 8).
805
Figure 6
A B
(A) Photograph showing the visible light mounted on the ceiling. (B) 15 W safe light in the orange-red spectrum of light
Figure 7
A B
(A) Floating thermometer. (B) Photograph showing the floating thermometer in the master tank containing water
Presence of any remnant fixer solution remaining on

Figure 8
the hangers may result in faulty radiographs in the form
of light spots. Water droplets on the hangers may produce
watermarks on the films, which might obscure diagnostic
details in the radiographic image.
Dryer cabinet
Wet films following the processing can be dried effectively
using cabinet driers, which help in circulating warm air
(produced by heaters) around the films thereby hastening
the drying process (Figure 9).
However, films should not be placed close to the heat
source in the cabinet drier as they can get distorted. Alter-
natively, wall mounted fans over the drying racks can be
used. Cabinet driers may also be placed outside the darkroom.
Processing technique
Time–temperature method of processing films is the most
Film hangers shown drying after use
ideal method of processing films manually. However,
806
Figure 9 Table 1 Time–temperature reference chart
Temperature of the processing Development time

solutions (Fahrenheit) (minute)
68 5
70 4.5
72 4
76 3
80 2.5
3. Use a separate stirrer for the developer and fixing

solutions and stir the solutions each day in the morn-
ing. Stirring will help even distribution of the con-
tents of the solutions and may help in maintaining a
constant temperature throughout the solutions.
4. Generally the metal insert containing the developer
solution is placed toward the left hand side of the
Cabinet film dryer master tank and the fixing solution is placed on the
right hand side of the master tank (with the personnel
facing the master tank). However, as the inserts are
generally not labeled it is wise to check the solutions
when the facility of temperature controlled processing is
before processing the films. This can be done by dip-
not available, the visual method can be employed.
ping a finger into one of the tanks and gently rubbing
the fingertips. The developer solution will feel soapy
Visual Method as it is alkaline in nature.
5. Once the solutions are stirred evenly, the temperature
In this method, there is no predetermined time for which of the solutions is assessed by placing the floating
the film is placed in the developer solution. The exposed thermometer in the water bath. For intraoral films the
film is placed in the developer solution and taken out at manufacturer specified duration of developing can be
regular intervals and examined under safe light for the most used. The following time–temperature reference chart
calcified/radiopaque structures to be evident. Appearance may be used (Table 1).
of radiopaque structures (like the enamel cap, restorations, 6. Film hanger is selected and ensured that it is dry and
artificial crowns) indicates the end point of developing does not contain any trace of water or processing
time. At this juncture, films are taken out of the developer solutions. The film packet is opened in the darkroom
solution, rinsed in water and placed in the fixer solution. and the film is clipped onto the hanger. The film thus
Disadvantage Quality of images is not reproducible as clipped is drawn out of the film packet thereby ensur-
the technique is very subjective. ing that the film does not come in contact with the
fingers of the operator, which might obscure diagnos-
tic details on the resultant radiographic image.
Time–Temperature Controlled Method 7. Once the film is placed in the developing solution, the
stop clock is started. Initially the hanger is gently agi-
The time–temperature method for processing films ensures
tated for a few seconds to eliminate any air bubbles
optimal film quality.
on the film. Later the hanger is placed in the devel-
oper tank for the predetermined time based on the
Steps in processing the film
temperature of the processing solutions. After the pre-
1. Ensure that the levels in the developer and fixer tank set time, the hanger holding the film is removed gen-
are adequate (the entire film should submerge into the tly and all excess developer solution clinging onto the
solutions). film is let to drain away into the master tank by hold-
2. Make sure that the solutions are not too old. Depleted ing the hanger over the master tank containing water
developer solution appears brownish black. On an aver- for a couple of seconds.
age developer and fixing solutions are changed every 8. The film is then placed into the master tank containing
15 days (taking into account that approximately 30 water for about 30 seconds. The hanger holding the
intraoral and 5 extraoral films are processed per day). film should be agitated gently so that all the excess
807
developer solution is removed from the film (this halts a single film holder and processed traditionally by dipping
the developing process) and the fixer solution is not the film first into the developer, water and finally the fix-
contaminated by the developer solution (contamina- ing solution. Once processed, the film can be washed out-
tion of the fixer solution causes the acidic fixer solution side the housing under running water and subsequently
to be neutralized by the alkaline developer solution). dried.
9. Once the films are rinsed, they are placed in the fixer
solution for about 4 minutes. The hanger holding the
film is agitated at regular intervals to bring the fixer Self-developing Film
solution to evenly contact the film. These have also been referred to as rapid autodeveloping
10. After the films are taken out of the fixer solution they films. It is essentially a one-piece soft plastic (polyvinyl
are held under running water for about 5 minutes chloride) pouch designed such that the film is located at
ensuring that all the surfaces of the film come under one end and a monobath of developer and fixer is located
the running water and remnants of the processing at the other end. Both the ends are connected via a narrow
solutions are totally washed away. The film is gently corridor that helps in carrying the processing solutions to
shaken to remove water drops clinging onto the sur- the film (Figure 11).
face of the film. During the exposure, the film end is positioned in the
11. The hanger holding films are then dried using a fan or mouth with the other end hanging outside the mouth. Once
a cabinet dryer. the exposure is made, the pouch is first washed to remove
12. Dried films are placed in a film mount before inter- the patient’s saliva. Then the film is developed by holding the
preting the films using an illuminated viewer box. monobath end of the pouch upright and rolling it down
until the processing chamber ruptures and empties into the
corridor. Continuing to roll the pouch ensures that all of
Daylight Processor
the monobath solution travels toward the film end.
It is essentially a plastic housing with a 15 W bulb. The The user continues to hold the pouch upright and mas-
housing is just large enough to cover three plastic bowls sages the monobath around in the film end of the pouch
that contain developer, water and fixer solutions. It is like
a make-shift arrangement for a darkroom especially for
dental clinics and mobile dental clinics. Figure 11
The operator can slide his/her hands via two sleeves pro-
vided at the sides of the plastic housing and then unwrap
the film. A plastic, tinted see-through lid at the top of the
housing enables the operator to see within the plastic case
without causing unnecessary light exposure (Figure 10A, B).
Under safe light conditions, provided by the 15 W
Self-developing film
orange-red bulb, the film can be unwrapped, helped with
Figure 10
A B
(A) Daylight processing unit. (B) Small cups filled with processing solutions within the daylight processor
808
for 50 seconds. When development is complete, the pouch Circulation-filtration system (Figure 15)
is turned upside down and the monobath is massaged back
The roller-transport system squeezes the chemicals into
to its original location at the opposite end. The film end of
and out of film emulsion, providing an agitating action,
the pouch is then opened by pulling apart the tabs and the
which promotes even processing and increases the speed
film is removed. The film is rinsed under water to remove
the remaining monobath solution.
Figure 13
AUTOMATIC PROCESSING
Automatic film processing refers to the processing of

radiographs using machines specifically designed for that
purpose. Most automatic processors are not universal
(there are separate machines to process extraoral and
intraoral films). These processors have inherent limitations
with regard to the need for regular maintenance and cost
(Figure 12).
Working Mechanism Roller mechanism within the automatic processor

The automatic processor is made up of five basic systems—
the transport system, the circulation and filtration system, the
replenishment system, the tempering system, and the dryer Figure 14
system.
Roller-transport system (Figure 13)

The roller-transport system is composed of a feed tray, a
main drive, and a number of rollers called crossovers and
racks.
As the film is placed in the feed tray, two feed rollers
draw the film into the machine (Figure 14). The film moves
circularly through a crossover and vertically down in the
developer by means of a series of rollers. It moves the
same way through the rest of the chemicals.
Figure 12 Film placement slots within the automatic processor
Figure 15
Processing solutions and the roller mechanism

Intraoral film automatic processor within the automatic processor
809
of reactions. A circulation-filtration system is used to helps in converting the silver ions into metallic silver at
boost this action. The circulation pump recirculates the the latent image sites. However, in this process phenidone
solutions through filters, keeping the chemicals properly gets oxidized and becomes inactive. Hydroquinone in the
mixed and clean as well as in a state of agitation. developer solution reduces the oxidized phenidone thereby
helping in its reactivation.
Replenishment system
As each film passes through the automatic processor, the Activator
chemicals are changed slightly. To offset the resulting Function Activators help in maintaining the alkaline pH
deficiencies, new developer and fixer in measured amounts of the developer solution and cause the gelatin of the film
are pumped into the solutions via the replenishment tanks to swell thereby helping the diffusion of the developing
attached to the processor. These tanks should be checked agents into the emulsion and reach the silver halide crys-
weekly and refilled periodically. tals. Developer solutions are active at an alkaline pH
(approximately 10).
Tempering system This alkaline pH is achieved and maintained by the
addition of alkaline agents such as sodium or potassium
To maintain the desired temperature of the developer and
hydrozide and buffers like sodium carbonate, sodium
fixer, a heating device and automatic thermostat are used.
hydroxide and sodium metaborate or tetraborate.
Water is passed through a mixing valve, so that it is 4⬚F or
5⬚F below the desired temperature, and then heated to the
desired temperature by the heating element in the machine. Preservative
The wash water temperature is controlled by a mixing Function It prevents the oxidation of the developer solu-
valve, which mixes the hot and cold water. A thermometer tion by atmospheric oxygen. It also combines with the
gauge is located between the mixing valve and the wash oxidized developer solution (appears brown) and forms a
tank near the mixing valve. colorless soluble compound. The preservative is an anti-
oxidant and is usually sodium sulfite.
Air-dryer system
To dry the film, there is a heater to heat the air and a Restrainer
blower to direct the air. An efficient exhaust system ensures Function It acts as an antifog agent. It minimizes/
that the warm, moist air is removed and only hot, dry air restrains the development of unexposed silver halide grains.
is directed over the films as they move through the roller- Potassium bromide or sodium bromide is used as the
transport system. restrainer in the developer solution.
Fixing solution
Composition of Processing Solutions
Functions Fixing solution helps in removal of the
Processing solutions are composed of a developer and undeveloped silver halide grains from the emulsion. If the
fixer solution. unexposed silver halide grains remain on the film the radio-
graphic image will appear black and will result in a non-
Developer solution diagnostic image.
Function To reduce the silver ions in the exposed crystals Composition The fixer solution contains four constitu-
of silver halide (latent image) to specks of black metallic ents, which are dissolved in water. They are:
silver (diagnostic, visible image).
❍ Clearing agent
Composition Developer solution contains four constitu- ❍ Acidifier
ents, which are dissolved in water. They are: ❍ Preservative
❍ Developer ❍ Hardener.
❍ Activator
❍ Preservative Clearing agent
❍ Restrainer.
Function Dissolves and removes the unexposed silver
halide crystals from the emulsion of the film. The removal
Developer
of the unexposed crystals takes place at a controlled slow
Function It converts the exposed silver halide grains to pace, however fixing for a prolonged time can lead to a
black metallic silver. It is made up of phenidone and gradual loss of film density (silver grains dissolve in acetic
hydroquinone. Phenidone acts as an electron donor and acid of the fixing solution).
810
Aqueous solution of ammonium thiosulfate is used as limiting water absorption, which helps in drying the film
the clearing agent. faster. Aluminum salts are used as hardeners.
Acidifier
Comparison between manual and automatic processing
Function The acidifier has two important functions. It
helps in the diffusion of ammonium thiosulfate into the Manual processing Automatic processing
emulsion and the exit of silver thiosulfate complex from Darkroom is required Use of darkroom eliminated
the emulsion. It inactivates remnants of developer solution Technique sensitive Non-technique sensitive as the
that is present over the film thereby halting the developing films are processed automatically
process of any unexposed silver halide crystals during the Direct exposure to hazardous No direct exposure to hazardous
process of fixing. The acidifier used is acetic acid buffer processing chemicals chemicals
system (pH of 4–4.5). Films need to be manually dried Films are dried automatically
Processing solutions need to be Processing solutions are available
Preservative
manually prepared readymade
Function It prevents the oxidation of the clearing agent. Longer time for processing Short processing time
It also combines with the oxidized developer solution, Negligible maintenance—need to Regular maintenance—need to
which is carried over to the fixing solution and thereby clean the tanks and replace fresh clean rollers and servicing gear
preventing staining of the film. The preservative used is solutions every 2–3 weeks mechanism
sodium sulfite or ammonium sulfite. Relatively inexpensive (not Expensive
considering the construction
Hardener of a darkroom)
Function The hardening agents combine with the gelatin Universal for intraoral and Separate processors are required
and prevent damage to the gelatin during handling of the extraoral radiographs (provided for extraoral and intraoral
the tanks are large enough) radiographs
film. It also reduces the swelling of the emulsion thereby
Penny Test
Penny test or coin test is used to assess the safe light condition in the darkroom. It is a known fact that radiographic
film is sensitive to visible light and X-rays. Excessive exposure to safe light or an improper safe light condition can
lead to exposure of the film resulting in film fog.
Procedure
1. In the darkroom, an exposed film is removed from the film packet and placed on the working bench in an area
where films are usually unwrapped.
2. A coin is placed on the film and left in place for a few minutes (usually the time it takes for a film to be unwrapped
and clipped onto a film holder for processing.
3. Process the film as usual. If the image of the coin is seen on the processed film then the darkroom does not have
optimum safe lighting.
Test to Determine the Quality of the Processing Solutions

Over a period of time the processing solutions deteriorate and need to be changed. On an average the processing solu-
tions need to be changed every 2 weeks (depending on the number of films processed per day).
A practical method of evaluating the solutions can be done by exposing a dental radiograph and using freshly
prepared solution to process this film. The film can then be used as a reference film. All successive films processed
can be compared with this film for contrast and density. Loss of image contrast and density is an indicator for solution
change.
811
CHAPTER
31 Radiographic Faults
➧ Errors in Film Storage and Handling ➧ Errors in Exposure Parameters and Processing
Film Fog Technique
Emulsion Peel and Scratched Film Blank Radiograph
Dark Spots or Lines Dark Radiograph
Static Electricity Artifact Light Radiographs
Nail Marks or Kink Marks Film Fog
➧ Errors in Film Placement and Projection Insufficient Contrast
Technique Yellow or Brown Stains
Herring Bone Effect, Tyre Mark Pattern, Raised Partial Image
Diamond Markings/Knurled Effect Blisters on the Film
Cone Cut White Spots
Slanting of the Occlusal Plane Dark Spots on the Radiograph
Apical Ends of the Teeth Not Imaged (Partial Light Spots on the Radiograph
Image) Emulsion Wash Away
Crown Portion of Teeth Not Imaged (Partial Reticulation of Emulsion
Image) Hyporetention
Overlapped Image Dyschroic Fog
Fore-shortened Image No Image/Blank Radiograph
Elongated Image (Total) ➧ Artifacts
Elongated Image (Partial)
Blurred Image
A radiograph is considered ideal when it is dimensionally ERRORS IN FILM STORAGE AND HANDLING
accurate, covers the area of interest completely and has
optimum density and contrast. When the above criteria are These can occur prior to the radiographic exposure and
not met the radiograph is termed faulty. A faulty radiograph after the processing especially before the film is com-
is non-diagnostic and necessitates retaking the radiograph pletely dry.
which leads to unnecessary patient exposure.
Causes of faulty radiograph Film Fog

Radiographic faults can occur at any stage in the radio- The overall appearance of the radiograph is dark. The
graphic process, right from handling and storing of films radiographic image will show decreased image contrast and
to the processing. The causes for radiographic faults can detail and as some authors believe they appear as though
be categorized as: the radiographs are being viewed through a fog thereby
obscuring the image (Figure 1).
❍ Errors in film storage and handling
❍ Errors in film placement and projection technique
Causes
❍ Errors in exposure parameters and processing technique
❍ Artifacts. ❍ Films stored at high temperatures and humidity
812
Chapter 31 – Radiographic Faults
❍ Outdated films However, during the developing process, the emulsion is

❍ Films exposed to extraneous radiation. rendered soft to aid in the rapid diffusion of the developing
agents into the emulsion and reacts with the silver halide
Ideal film storage temperature is between 50⬚F and 70⬚F
grains. However, the hardeners in the fixing solution shrink
(10–20⬚C) and between 30% and 50% relative humidity.
and harden the gelatin to prevent its damage. White lines
Any increase is known to cause increased sensitization of
appear when the soft film emulsion is removed from the
the film emulsion. The expiry date of the film packets have
film base by sharp objects.
to be checked and it is also recommended that the radiol-
ogy unit stocks only sufficient number of film packets that
Causes
can be used within 2 months’ time.
Extraoral films should be stored in a vertical orientation ❍ Wet film in contact with finger nails or other sharp
to prevent pressure artifacts. objects such as film clips (Figure 2A, B).
❍ Placing wet films on unclean surfaces.
Emulsion Peel and Scratched Film ❍ After processing, films must not come into contact
with each other until completely dry as the wet emul-
Emulsion is coated onto the plastic supportive base of the sions can stick together and peel off the emulsions
film and it adheres to the base via a coat of adhesive agent. when they are separated.
Care must be taken when placing the film holder in the
Figure 1 processing solutions and drier. Avoid contact with other
film hangers and with the tank walls.
Dark Spots or Lines

Cause
Finger print contamination.
❍ Dark finger marks: from improper handling of the film
(holding the film with greasy fingers on the surface of
the film, especially when the fingers are contaminated
with developer solution).
❍ Clear finger marks or white fingerprint marks can be
caused by fixer solution or oily substances on the fin-
gers that prevent the emulsion from developing.
Fogged radiograph
Films should be held by the edges only.
Figure 2
A B
(A) Emulsion peel. (B) Scratched film
813
Figure 3 Figure 4
Static electricity artifact on a panoramic film
Crescent shaped nail mark

Static Electricity Artifact
Causes
Figure 5
Static electricity artifacts are formed due to the build-up
of electrons in the emulsion. They are frequently caused A
by low humidity/dry weather or static producing objects
such as synthetic materials used in uniforms. Moreover,
when films are removed from the film packet or the cas-
sette too rapidly in a dry atmosphere, a small charge of
electricity can be released producing these artifacts.
Static artifacts have two common appearances: tree like
and smudges. Tree like artifacts (Figure 3) are produced
when films are unwrapped rapidly and appear as black
lines running across the film and smudge static electricity
is produced by polyester clothing and appear as black
smudges on the film.
Nail Marks or Kink Marks

B
Appear as a crescent shaped radiolucent finger nail marks
(Figure 4).
Cause
Excessive bending of the film.
ERRORS IN FILM PLACEMENT AND

PROJECTION TECHNIQUE
(A) Radiograph showing the tyre mark or raised diamond
Herring Bone Effect, Tyre Mark Pattern, pattern. (B) Knurled pattern on a nut
Raised Diamond Markings/Knurled Effect
A lead foil is placed within every intraoral radiographic
film packet to prevent unnecessary exposure to surround- Though the term ‘herring bone pattern’ is widely used,
ing structures from the residual X-ray beam and also pro- many of the new film packets do not display this pattern
tects the film from back scatter or secondary radiation that on the lead foil, rather the tyre mark (Figure 5A) or knurled
may result in film fog. pattern (Figure 5B) are common.
814
Cause Slanting of the Occlusal Plane

Wrong side (opposite side) of the film exposed to radiation. The radiographic film should ideally be held in the mouth
using an appropriate film holder which stabilizes the
film during exposure. Slanting of the occlusal plane is
Cone Cut usually seen when the patient attempts to stabilize the film
A part of the radiographic image appears blank on the with a finger. Ideally when the film is placed in the mouth
radiograph (Figure 6), which confirms to the circular shape the edge of the film should be parallel to the occlusal
of the position indicating device (PID). plane.
Causes Cause
❍ Improper placement of film Improper placement of the film.

❍ Improper placement of the PID (film partially outside
the area covered by the PID).
Apical Ends of the Teeth Not Imaged
(Partial Image)
Ideally the tooth to be imaged should be positioned in the
Figure 6 center of the film. Also care should taken during film
placement that at least 2 mm all around the tooth of inter-
est should be visible in the resultant radiograph.
Causes
❍ Insufficient vertical angulation
❍ Film not placed sufficiently deep into the palatal vault
(Figure 7A) or lingual vestibule
❍ Patient opens mouth just short of the exposure
(Figure 7B).
Crown Portion of Teeth Not Imaged

(Partial Image)
When the film is placed against the tooth in bisecting
Cone cut
angle technique, it should be ensured that the edge of the
Figure 7
A B
(A) Partial image due to improper film placement resulting in the apices of premolars cut-off. (B) Partial image caused due to
opening of the patient’s mouth during exposure
815
Figure 8 Figure 9
Film placed too apically resulting in crowns not imaged Overlapped proximal aspects of teeth due to improper
horizontal angulation
film should be placed about 2 mm beyond the cuspal tips

or incisal edges of teeth.
Figure 10
Cause
Improper placement of film (Figure 8).
Overlapped Image
For all practical purposes, the central beam of X-rays should
be directed perpendicular to the film and tooth (in paral-
leling technique) and to the imaginary bisector bisecting
the long axis of tooth and long axis of film (in bisecting
angle technique) thereby passing through the interproximal
contacts between teeth.
Causes
❍ Improper horizontal angulation (Figure 9) Overlapped images due to double exposure
❍ Double exposure.
If an already exposed film is re-used by mistake, then the
resultant radiograph will show two overlapped images Figure 11
(Figure 10).
Fore-shortened Image
The radiograph exhibits unusually short images (Figure 11).
Causes
❍ Increased vertical angulation (bisecting angle tech-
nique)
❍ Film not placed parallel to the long axis of the tooth
(paralleling technique).
Elongated Image (Total)

Fore-shortened images
Unusually long radiographic images are seen (Figure 12).
816
Causes placed in the mouth due to the curvature of the palate or

lingual arch such as canines, third molars or mandibular
❍ Decreased vertical angulation (bisecting angle technique)
anteriors. Receptors can be flexed but should never be
❍ Film not placed parallel to the long axis of the tooth
bent as that causes creasing of the emulsion, which in turn
(paralleling technique).
compromises the quality of the image.
Total blurring occurs either due to the movement of
Elongated Image (Partial) the patient or the X-ray tube head (Figure 14A). It is wise
to wait for a few seconds after adjusting the X-ray tube
Only part of the image appears elongated. head before making an exposure. Also, patients should
be instructed to remain still during the exposure.
Cause
Excessive bending of the film (Figure 13). Causes
❍ Movement of the film or patient during the exposure
(image totally blurred)
Blurred Image
❍ Excessive bending of the film (image partially blurred,
Blurred images can either be total or partial. Partial blur- Figure 14B).
ring occurs when the film gets bent excessively when
Figure 13
Figure 12
Partial elongation due to bending of film

Elongated images during film placement
Figure 14
A B
(A) Total blurring of image. (B) Partial blurring of image
817
Figure 15 Figure 17
Partially blank radiograph

Light radiograph
Figure 16
b. Processing errors
❍ Developer temperature too high
❍ Film developed for a longer time
❍ Concentration of the developer too high
❍ Accidental exposure to light
❍ Improper safe lighting.
Light Radiographs (Figure 17)

Causes
a. Exposure errors
❍ Insufficient mA, insufficient kVp, insufficient
exposure time
Dark radiograph
❍ Film packet placed with the wrong side facing the
X-ray source
❍ Increased film source distance
ERRORS IN EXPOSURE PARAMETERS AND b. Processing errors
PROCESSING TECHNIQUE ❍ Placed in the developer solution for a short dura-
tion of time
Blank Radiograph ❍ Temperature of the developer solution too low
❍ Depleted developer solution
Causes
❍ Diluted or contaminated developer solution
❍ Unexposed film (Figure 15) ❍ Prolonged fixation.
❍ Exposed film dipped into the fixer solution before it
was placed into the developer solution.
Film Fog
Causes
Dark Radiograph (Figure 16)
❍ Improper wattage of the safe light
Causes
❍ Prolonged exposure of the film to safe light
a. Exposure errors ❍ Safe light not at a proper distance from the working
❍ Excessive milliampere (mA), excessive kilovolt peak place
(kVp), excessive exposure time ❍ Light leaks from cracked safe light filters/light from
❍ Insufficient film–X-ray source distance doors and ventilators.
818
Figure 18 Figure 19
Yellow/brown stains Blisters on the film
Insufficient Contrast
Figure 20
Causes
❍ Underexposed to radiation
❍ Insufficient developing time.
Yellow or Brown Stains (Figure 18)

Causes
❍ Film inadequately fixed
❍ Depleted developer solution
❍ Depleted fixer solution
❍ Film inadequately washed in water White spots
❍ Contaminated processing solutions.
Partial Image ❍ These can be avoided by gently agitating the film

holders in the processing solutions.
Cause
Part of the film not immersed into the developer solution.
Dark Spots on the Radiograph
Blisters on the Film Causes
Causes ❍ Excessive bending of film, fingerprints

❍ Film contaminated with the developer solution before
❍ Air bubbles on the film while developing the actual processing
❍ Temperature difference between the developer and fixer ❍ Film in contact with another film or tank walls during
solutions (Figure 19) the fixing procedure.
❍ Increased acidity of the developer solution.
Light Spots on the Radiograph (Figure 21)

White Spots (Figure 20)
❍ Film contaminated with the fixer solution before the
Causes
actual processing (remnants of fixing solution present
❍ Air trapped on film surface after the film is placed on the hangers for processing will cause this artifact).
in the processing solutions, air bubbles prevent the ❍ Film in contact with the tank wall or another film dur-
chemicals from affecting the emulsion in that area. ing the developing process.
819
Figure 21 Figure 23
Light spots on the radiograph Yellow stain in the film due to

hyporetention
Figure 22
Causes
❍ Inadequate washing
❍ Remaining thiosulfate from fixer solution.
Dyschroic Fog
Fogging of the radiograph, characterized by the appear-
ance of a pink surface when the film is viewed by trans-
mitted light and a green surface when the film is seen by
reflected light.
Cause
Exhaustion of the acid content of the fixing solution
Reticulation of the emulsion (incomplete fixation).
No Image/Blank Radiograph
Emulsion Wash Away
Since none of the X-rays reach the film to form the latent
Radiograph with part of the emulsion lost due to a pro- image, all the unexposed silver halide grains are removed
longed washing. by the clearing agent resulting in a blank radiograph
(Figure 24).
Reticulation of Emulsion Causes
Cause ❍ Mechanical problems such as electrical failure, failure
Reticulation of emulsion results when a film is subjected to turn on the machine
to a sudden temperature change between the developer ❍ Improper alignment of the PID or the film.
solution and the water bath (Figure 22).
ARTIFACTS
Hyporetention
It appears as a yellowish stain on the radiograph that is An artifact is a structure or radiographic appearance that
processed (Figure 23). is normally not present in the radiograph and is produced
820
by artificial means. It might be considered as foreign body

Figure 24
image on the radiograph.
The common artifacts are metallic objects in the path
of the X-ray beam such as jewelry (Figure 25A, B), dental
appliances (Figure 25C, D), etc. or placement of the finger
between the X-ray tube and the film (such as using the
finger to stabilize the film in the mouth) resulting in a
phalangioma (Figure 25E).
Prior to taking a radiograph, patients should be instructed
to remove all removable metallic objects such as jewelry,
prosthodontics appliances (removable partial and complete
dentures), spectacles, hair clips, etc. that are in the area to
be imaged. These foreign objects may superimpose over
image and obscure the findings. Thyroid collars or lead
Blank radiograph
aprons may also be imaged on radiographs.
Figure 25
A B C
D E
(A) Nose stud artifact. (B) Hair clip, nose stud and earrings artifacts. (C) Intraoral appliance.
(D) Cast partial denture. (E) Phalangioma
821

SECTION Radiographic
XII Landmarks
32 Intraoral Radiographic Anatomical Landmarks 825

33 Extraoral Radiographic Landmarks 837
34 Site Selection, Evaluation and Imaging for 842
Dental Implants
823
Chapter-32.indd 823 10/3/2012 3:34:30 PM


CHAPTER
Intraoral Radiographic
Anatomical Landmarks
Ravikiran Ongole
32
➧ Landmarks Common to Both the Maxillary and ➧ Landmarks Unique to the Maxillary Intraoral
Mandibular Radiographs Periapical Radiograph
Teeth ➧ Landmarks Unique to the Mandibular Intraoral
Periodontal Ligament Space Periapical Radiograph
Alveolar Bone
During the process of interpreting a radiograph the radi- Teeth

ologist is expected to identify normal radiographic anat-
omy, thereby distinguishing normal features from Enamel cap, dentin, cementum, pulp.
pathology. These normal radiographical anatomical land- Enamel appears almost snow white and is seen extend-
marks are unique to each area of the maxilla or mandible ing from the neck of the tooth, i.e. the cementoenamel
and appear either radiopaque or radiolucent. They may be junction from one side to the other covering the tooth like
used to identify a specific area of the jaw. For example, the a cap (enamel cap). It is the most mineralized structure on
intermaxillary suture is unique to the radiographs of the the radiograph (90% mineralized) and appears more radi-
maxillary central incisors, mental foramen is typically opaque than other structures (Figure 1).
associated with the radiographs of the mandibular premo- Dentin is seen as homogenous radiopacity (less radi-
lars etc. On occasions, these landmarks may exhibit some opaque than enamel as it is 75% mineralized) and radio-
minor variations (such as a bifid mandibular canal) or may graphically has the density of bone.
not be very evident on a faulty radiograph.
The landmarks discussed here are those that are seen in
intraoral periapical radiographs. Figure 1
Intraoral radiographic landmarks

1. Landmarks common to both the maxillary and man-
dibular radiographs
2. Landmarks unique to the maxillary radiograph
3. Landmarks unique to the mandibular radiograph.
LANDMARKS COMMON TO BOTH

THE MAXILLARY AND MANDIBULAR
RADIOGRAPHS
❍ Teeth—enamel cap, dentin, cementum, pulp

❍ Periodontal ligament space
Radiograph showing enamel, dentin and pulp. Enamel is the
❍ Alveolar bone—lamina dura, alveolar crestal bone,
most radiopaque and pulp is radiolucent
marrow spaces and trabecular bone.
825
Section XII – Radiographic Landmarks
Figure 2 Figure 3
Dental papilla enclosed by the radiopaque bony crypt in Radiololucent periodontal ligament space confined between
a root yet to be completely formed the radiopaque surface of the root and the lamina dura
Cementum is not appreciated radiographically because

Figure 4
the contrast between it and dentin is minimal. Cementum
is 50% mineralized.
Pulp appears radiolucent. The coronal pulp (pulp cham-
ber) extends inferiorly into the roots, which is referred to
as radicular portion of pulp.
In a developing tooth, the pulp canal diverges and the
walls of the root taper to a knife edge. A small round area
of radiolucency is seen at the root tip which is surrounded
by a thin layer of hyperostotic bone which is the dental
papilla enclosed by the bony crypt (Figure 2).
Periodontal Ligament Space

The periodontal ligament space is seen as a radiolucent
area between the root surface and the lamina dura. It is
Radiograph showing the radiopaque lamina dura
seen all around the root extending from the alveolar crest
surrounding the roots of the teeth
on one side to the other (Figure 3). The width of the peri-
odontal ligament space varies from 0.15 to 0.36 mm; it is
generally thinner in the middle third of the root and wider
near the alveolar crest and the apical region of the root. seen as a well-defined radiopaque line that surrounds the
However due to projection errors, the periodontal ligament roots of teeth in health (Figure 4). The appearance of lam-
space may not be discernible on some radiographs. ina dura on a radiograph is because of the attenuation of
the X-ray beam as it passes through the thin layer of bone
tangentially.
Alveolar Bone Usually lamina dura is well-defined. However, on occa-
sions, even in a healthy tooth lamina dura may appear
Lamina dura, alveolar crestal bone, cancellous trabecular
indistinct and diffuse because of an obliquely directed
bone.
X-ray beam. Lamina dura is generally more radiodense
and thick around the roots of teeth under heavy occlusal
Lamina dura
forces. A double lamina dura is seen when the surfaces of
Lamina dura is a radiographic term used to describe alveo- the mesial and distal root are in the path of the central
lar bone proper (cortical bone), which forms the sockets of beam of the X-ray. Loss of lamina dura either partially or
teeth. It is also considered to be a ‘specialized’ continua- generalized may indicate the presence of a local periapical
tion of the cortical plate. Radiographically, lamina dura is pathology or an underlying systemic disturbance.
826
Chapter 32 – Intraoral Radiographic Anatomical Landmarks
Figure 5 Figure 6
Pointed radiopaque crest of the interdental alveolar bone in Flat topped radiopaque interdental bone in the posterior
the anterior teeth teeth that runs parallel to the cementoenamel junction
Figure 7
A B
(A) Trabeculae are fine and dense in the maxillary anterior region. (B) Trabeculae are oriented irregularly and
bone marrow spaces are relatively
Alveolar crest up of a network of radiopaque trabeculae that enclose radio-

lucent bone marrow spaces.
Crest of the alveolar bone is a radiopaque structure. It is
In the maxillary anterior region, the trabeculae are
seen as a continuation of the lamina dura (cortical bone).
fine and dense and in the posterior region the bone mar-
The junction between the alveolar crest and lamina dura is
row spaces are relatively larger and oriented irregularly
seen as a sharp well-defined angle. Generally the crest of
(Figure 7A). In the anterior region of the mandible, the
the alveolar ridge is 1.5 mm apical to the cementoenamel
trabeculae are fewer in number and oriented horizontally.
junction. In the anterior teeth, the alveolar crest termi-
In the posterior region of the mandible the bone marrow
nates as a pointed projection and in the posterior teeth it
spaces are larger (Figure 7B).
appears flat and parallel to the cementoenamel junction
(Figures 5 and 6).
LANDMARKS UNIQUE TO THE MAXILLARY
Cancellous bone INTRAORAL PERIAPICAL RADIOGRAPH
It is present between the buccal and lingual cortical plates
in the maxilla and the mandible and accounts for most of ❍ Intermaxillary suture
the bulk of the alveolar bone. The cancellous bone is made ❍ Anterior nasal spine
827
Figure 8
A B
(A) Radiograph of the mandibular anterior region showing less number of trabeculae that are oriented horizontally.
(B) Radiograph of the mandibular posterior region showing large bone marrow spaces
❍ Nasal fossae and floor of the nasal fossae

Figure 9
❍ Nasal septum
❍ Incisive foramen
❍ Tip of the nose shadow
❍ Lateral fossa
❍ Canine fossa
❍ Nasopalatine canal
❍ Superior foramina of the nasopalatine canal
❍ Nasolacrimal canal
❍ Nasolabial fold
❍ Inverted Y line of Innes
❍ Maxillary sinus
❍ Zygomatic process and zygomatic bone
❍ Maxillary tuberosity
❍ Pterygoid plates
❍ Hamular process
❍ Coronoid process of the mandible. Radiograph showing the intermaxillary suture as a linear
radiolucent line extending from the crest of the alveolar bone
between the maxillary central incisors to the anterior nasal spine
Intermaxillary suture/median suture
Anatomical location/projection The intermaxillary suture
runs from the alveolar crest between the maxillary central
fossa. When an IOPAR is taken for the upper central inci-
incisors to the posterior aspect of the hard palate. It is seen
sors it is located between and roughly 2 cm beyond the
when an intraoral periapical radiograph (IOPAR) is taken
periapices of the central incisors.
for the upper central incisors.
Radiographic appearance (Figure 8B) It is a V-shaped
Radiographic appearance (Figure 8A) It is seen as a lin-
radiopaque structure.
ear radiolucency bounded by radiopaque lines extending
from the crest of the alveolar bone between the maxillary
Nasal fossae and floor of the nasal fossa
central incisors to the anterior nasal spine on an IOPAR.
Occasionally it is seen as a V-shaped enlargement at the Anatomical location/projection The inferior portion and
alveolar crest. floor of the nasal fossae are seen on an IOPAR beyond the
periapical regions of the maxillary anterior teeth, when an
Anterior nasal spine IOPAR is taken for the upper central and lateral incisors.
Anatomical location/projection Seen on an IOPAR at Radiographic appearance (Figure 9) The floor of the
the junction of the nasal septum and the floor of the nasal nasal fossae appear as well-defined radiopaque lines that
828
Figure 10 Figure 11
Anterior nasal spine seen as a V-shaped radiopaque Radiograph showing floors of the nasal fossae as well-defined
structure at the junction of the nasal septum and radiopaque lines extending bilaterally on either side of the
the floor of the nasal fossa anterior nasal spine and the inferior portion of the nasal
fossae are seen as radiolucent areas above the nasal floor
extend bilaterally on either side of the anterior nasal spine.

The radiolucent region above the floor of the nasal fossae Figure 12
is the inferior portion of the nasal fossa.
Nasal septum
Anatomical location/projection It is seen on periapical
radiographs taken in relation to maxillary central incisors.
It is a midline structure and seen extending superiorly
from the anterior nasal spine.
Radiographic appearance (Figure 10) The nasal septum
is a sharply defined linear radiopacity, which may gener-
ally appear to be deviated from the midline. However, on
occasions the image of septal cartilage and the vomer bone
may be superimposed over the nasal septum.
Incisive foramen Radiograph depicting the nasal septum as a sharply defined

linear radiopacity extending superiorly from the anterior nasal
Anatomical location/projection Incisive foramen is spine and dividing the nasal fossae
seen in relation to the middle and apical one-third of the
roots of the maxillary central incisors. It is seen on peri-
apical radiographs taken in relation to maxillary central
nose tip. The shadow of the tip of the nose is seen when a
incisors.
radiograph is taken for the maxillary central incisors.
Radiographic appearance (Figure 11) It is radiolucent and
Radiographic appearance (Figure 12) It appears as a
may have various appearances ranging from a smoothly
homogenous cup-shaped radiopacity with a definite outline.
symmetric outline to irregular or ill-defined border and usu-
ally smaller than 1 cm in diameter.
Lateral fossa/incisive fossa
Tip of the nose Anatomical location/projection It is seen in relation to
the apical region of the maxillary lateral incisor. Radiographs
Anatomical location/projection The image of the tip
of the lateral incisors will reveal this anatomic landmark.
of the nose is superimposed over the apical one-third of
the roots of the maxillary central and lateral incisors. It is Radiographic appearance (Figure 13) It appears as a
usually seen in individuals with a prominent and bulbous diffuse radiolucency.
829
Figure 13 Figure 15
Incisive foramen seen as a well-defined radiolucent Lateral fossa seen as diffuse radioluceny in relation to the
area in relation to the middle and apical one-third of the apical region of the maxillary lateral incisor
roots of the maxillary central incisors
Figure 14 of maxillary central incisors, when taken with an increased

vertical angulation.
Radiographic appearance (Figure 15) They appear as
well-defined oval radiolucencies on either side of the nasal
septum (superimposed close to the floor of the nasal fossa).
Nasolacrimal canal
Anatomical location/projection Rarely visualized on a
periapical radiograph. When an increased vertical angula-
tion is used, the nasolacrimal canal may be seen in the
periapical region of the canine. In maxillary occlusal
radiographs, they are seen in relation to the apices of the
molars.
Radiographic appearance (Figure 16) Well-defined ovoid/
The tip of nose shadow is seen as a homogenous cup shaped round radiolucencies.
radiopacity superimposed over the apical one-third of the
roots of the maxillary central and lateral incisors Nasolabial fold
Anatomical location/projection The nasolabial fold is seen
extending across the middle and apical one-third of the
Nasopalatine canal
roots of the canine and first premolar. It is seen on IOPAR
Anatomical location/projection The nasopalatine canal taken on the maxillary canine.
is rarely seen on periapical radiographs. However, they may
Radiographic appearance (Figure 17) The nasolabial
be evident on radiographs taken in relation to the maxil-
fold appears as a linear radiopaque shadow. Occasionally,
lary central incisors.
a zone of increased radiopacity is seen above this shadow
Radiographic appearance (Figure 14) The lateral walls which is caused by the superimposition of the buccal
of the nasopalatine canal appear radiopaque and extend mucosa and alveolus.
from the incisive foramen to the floor of the nasal fossa.
Inverted Y line of Innes
Superior foramina of the nasopalatine canal
Anatomical location/projection The inverted Y line or Y
Anatomical location/projection Generally seen on max- line of Innes is seen when radiographs are taken in the
illary occlusal radiographs or on a periapical radiograph maxillary canine region.
830
Figure 16 Figure 18
Radiograph showing the nasaopalatine canal at the Maxillary occlusal radiograph showing nasolacrimal
periapical region of the maxillary lateral incisor canals as well-defined ovoid radiolucencies in relation to
the apices of the molars
Figure 17 Figure 19
IOPAR of the maxillary premolar region showing the

nasolabial fold as a linear radiopaque shadow extending
across the middle and apical one third of the roots of the
Radiograph reveals superior foramina of the nasopalatine
canine and first premolar
canal as well-defined oval radiolucencies, superimposed
close to the floor of the nasal fossa
Maxillary sinus
Anatomical location/projection The floor and a minimal
Radiographic appearance In relation to the periapex of
portion of the inferior aspect of the maxillary sinus are seen
the canine, the floor of the maxillary sinus and the floor of
on periapical radiographs taken in relation to the maxillary
the nasal fossa cross one another forming an inverted Y,
premolar and molar teeth.
which is referred to as Y line of Innes (Figure 18).
Radiographic appearance The maxillary sinus appears as
Canine fossa a uniformly radiolucent structure bounded by well-defined
thin radiopaque line.
Anatomical location/projection Seen at the periapical
The roots of the maxillary molars ‘appear’ to project
regions of the maxillary canine (Figure 19).
into the maxillary antrum as a result of the angulation of
Radiographic appearance Seen as a diffuse radiolucency. the X-ray beam (Figure 20).
831
Figure 20 Figure 22
Radiograph showing the inverted Y line of Innes in relation to Radiograph of the maxillary first molar reveals the scalloped
the maxillary canine-first premolar region well-defined radiopaque floor of the maxillary sinus and the
radiolucent maxillary sinus
Figure 21
the maxillary sinus in relation to the apices of the molar
teeth. It is seen as a U-shaped or V-shaped radiopaque line.
The zygomatic bone is seen as a homogenous radiopacity
over the apices of the second and third molars, generally
obscuring the periapical region.
Pterygoid plates and hamular process

Anatomical location/projection The pterygoid plates and
hamular process are seen distal to the maxillary tuberosity.
They are generally seen on intraoral periapical radiographs
taken in relation to the third molars. However, in most
cases they may not be evident.
Radiographic appearance (Figure 22) The pterygoid
IOPAR showing the canine fossa as a diffuse radiolucency at plates are seen as a radiopaque shadow distal to the max-
the periapical region of the maxillary canine illary tuberosity and the hamular process is evident as a
single radiopaque linear structure extending from the
inferior aspect of the medial pterygoid plate.
In edentulous spaces, the floor of the sinus dips down Coronoid process
and lies close to the alveolar ridge (pneumatization of
Anatomical location/projection The coronoid process
the sinus). Occasionally thin radiolucent lines are seen to
of the mandible is usually seen superimposed over the
traverse the sinus walls. These radiolucent tracks represent
periapical regions of the second and third maxillary molars
the neural and vascular supply of the sinus.
and sometimes over the maxillary tuberosity region. The
position of the image of the coronoid process on the radio-
Zygomatic process and zygomatic bone
graph depends on the extent to which the mouth was
Anatomical location/projection The zygomatic bone and opened during the radiographic projection. It is seen
the zygomatic process of the maxilla are seen in the peri- when an IOPAR is taken for the upper second and third
apical regions of the second and third molars. Zygomatic molars.
bone is usually seen when an excessive vertical angulation
Radiographic appearance (Figure 23) The coronoid
is used.
process appears as a homogenous triangular shaped radi-
Radiographic appearance (Figure 21) Zygomatic pro- opacity with the tip facing the occlusal aspects of the
cess of the maxilla is seen above the level of the floor of teeth.
832
Figure 23 Figure 25
IOPAR of the maxillary molars reveals a V-shaped radiopaque IOPAR of the maxillary third molar showing the mandibular
structure seen above the level of the floor of the maxillary coronoid process as a homogenous triangular shaped
sinus projecting downward toward the apices of molar teeth radiopacity with the tip facing the occlusal aspects of
the upper teeth
Figure 24
Symphysis
Anatomical location/projection Seen only in the first year
of life. It is seen in the region corresponding to the midline
of the mandible. It is seen on the radiographs of the decid-
uous mandibular central incisors.
Radiographic appearance Symphysis appears as a linear
radiolucency between the deciduous mandibular central
incisors and extending inferiorly to involve the lower bor-
der of the mandible (Figure 24).
Genial tubercles (mental spine) and lingual

foramen
Radiograph showing the pterygoid plates as a radiopaque Anatomical location/projection They are generally evi-
shadow distal to the maxillary tuberosity and the hamular dent on the periapical radiographs of mandibular central
process as a radiopaque linear structure extending from the incisors and mandibular occlusal radiographs.
inferior aspect of the medial pterygoid plate
Radiographic appearance Genial tubercles are seen on
mandibular occlusal radiographs as discrete radiopaque
LANDMARKS UNIQUE TO THE MANDIBULAR structures measuring about 4 mm in diameter. They are
INTRAORAL PERIAPICAL RADIOGRAPH present beneath the apices of the mandibular central inci-
sors in the midline.
In periapical radiographs, they are seen as a well-defined
❍ Symphysis
radiopaque mass enclosing a circular radiolucent area which
❍ Genial tubercles
is referred to as the lingual foramen (incisal branches of
❍ Mental ridge
mental nerve exit out of the lingual foramen to innervate
❍ Mental fossa
the incisor teeth) (Figure 25).
❍ Mental foramen
❍ Mandibular canal
Mental ridge
❍ Mylohyoid ridge
❍ Submandibular gland fossa Anatomical location/projection Mental ridge is seen
❍ External oblique ridge superimposed over the apical one-third of the roots of the
❍ Inferior border of mandible. mandibular central and lateral incisors. It appears to extend
833
Figure 26 Figure 27
IOPAR showing well-defined radiopaque mass Radiograph of the mandibular incisor region showing two
(genial tubercles) enclosing a circular radiolucent well-defined radiopaque lines extending bilaterally from the
lingual foramen premolar region toward the midline
from the premolar region up to the central incisors. These

are seen in IOPAR taken for the mandibular incisors. Figure 28
Radiographic appearance Seen as two well-defined radi-
opaque lines extending bilaterally from the premolar region
toward the midline.
Mental fossa
Anatomical location/projection The mental fossa is an
anatomical depression present on the labial aspect of the
mandible extending bilaterally from the midline to the lat-
eral incisor and occasionally up to the canine.
Radiographic appearance It is seen as a diffuse radio-
lucent area, which is superimposed over the roots of the
mandibular anterior teeth. The mental fossa is bounded
superiorly by the alveolar ridge and inferiorly by the men-
tal ridge (Figure 26). Mental fossa seen as a diffuse radiolucency superimposed
over the roots of the mandibular anterior teeth
Mental foramen
Anatomical location/projection The mental foramen is
usually seen at the periapical regions of mandibular pre- Radiographic appearance (Figure 28) It is seen as linear
molars. It is usually present equidistant from the both the radiolucent canal running along the apices of the molar
lower border of the mandible and the alveolar ridge. teeth. The course of the canal is generally lined by thin
radiopaque lamellae of bone.
Radiographic appearance It appears radiolucent and has a
round, ovoid or an elongated shape. It may or may not have
Mylohyoid ridge or internal oblique ridge
a well-defined radiopaque corticated border (Figure 27).
Anatomical location/projection The mylohyoid ridge is
Mandibular canal seen extending from the third molar region to the premo-
lars usually along the apices of these teeth. Usually seen
Anatomical location/projection The mandibular canal is
when radiographs are taken for the mandibular molars.
usually seen on periapical projection of mandibular molars.
The apices of the molar teeth may some times lie close to Radiographic appearance (Figure 29) It is generally seen
the canal. as a well-demarcated radiopaque line below the level of
834
Figure 29 Figure 31
IOPAR of the mandibular premolar region showing a Radiograph showing the mylohyoid ridge as a
well-defined radiolucent area at the periapical region of the well-demarcated radiopaque line running along the
2nd premolar apical thirds of the mandibular molar teeth
Figure 30
Figure 32
Radiograph of the mandibular molar region showing the IOPAR showing the external oblique ridge as a linear
mandibular canal as a linear radiolucent canal running along radiopacity which continues as the ascending ramus
the apices of the molar teeth superiorly
the external oblique ridge that runs along the apical thirds
of these teeth. Submandibular gland fossa
Anatomical location/projection The submandibular gland
External oblique ridge
fossa is an anatomical depression that houses the sub-
Anatomical location/projection It is seen on IOPARs of mandibular gland. It is seen in the periapical region of
mandibular posterior teeth. The external oblique ridge is molar teeth, below the level of the mylohyoid ridge. It is
seen above and parallel to the mylohyoid ridge. extends up to the premolar region anteriorly and to the
ascending ramus posteriorly. It is seen when a radiograph
Radiographic appearance (Figure 30) The external
is taken for the lower molar teeth especially second and
oblique ridge appears as a linear radiopacity which merges
third molars.
with the alveolar bone as it traverses toward the premolar
region and superiorly it continues as the ascending ramus Radiographic appearance (Figure 32) It appears as a
of the mandible. diffuse radiolucency.
835
Figure 33 Inferior border of the mandible

Anatomical location/projection May be seen in any of
the projections of the mandibular teeth. It is usually seen
when the film is placed deep within the lingual sulcus and
the vertical angulation is increased.
Radiographic appearance (Figure 33) The inferior bor-

der of the mandible is rarely seen on intraoral periapical
radiographs. It is evident as a radiopaque strip of uniform
thickness (cortical portion) of bone along the inferior edge
of the mandible.
Radiopaque anatomical landmarks
Maxilla Mandible
IOPAR of the mandibular third molar region showing the
Anterior nasal spine Genial tubercles
submandibular gland fossa as a diffuse radiolucency beneath
Floor of nasal fossa Mental ridge
the level of the mylohyoid ridge and mandibular canal
Tip of nose, nasolabial fold Mylohyoid ridge
Floor of maxillary sinus External oblique ridge
Zygomatic process of maxilla Inferior border of the mandible
Figure 34 Zygomatic bone
Maxillary tuberosity
Pterygoid plates
Hamular process
Coronoid process
Radiolucent anatomical landmarks
Maxilla Mandible
Intermaxillary suture Symphysis
Nasal fossa Lingual foramen
Incisive foramen Mental foramen
Superior foramina of nasopalatine Mandibular canal
canal Submandibular gland fossa
Lateral fossa/incisive fossa
IOPAR showing the inferior border of the mandible as a Canine fossa
well-defined linear radiopaque strip of uniform thickness of Nasolacrimal canal
bone along the inferior edge of the mandible Maxillary sinus
836
CHAPTER
Extraoral Radiographic
Landmarks
Praveen BN
33
Figure 1
1. External auditory meatus

23
2. Hyoid bone
3. Dorsum of tongue
4. Hard palate
5. Anterior nasal spine
6. Soft palate
20 7. Floor of orbit
22 12 8. Frontal process of zygoma
21
10 9. Nasal bone
24 9 10. Nasion
16 11. Inferior rim of orbit
17 8 12. Ethmoid air cells
1 13. Posterior wall of maxillary sinus
19 14. Posterior arch of atlas
11 15. Occipital condyle
16. Mastoid air cells
13 17. Condyle
18 18. Articular eminence
5
19. Base of skull
15 6 20. Sella turcica
7 21. Sphenoid sinus
22. Posterior clinoid process
14 4
23. Roof of orbit
24. Clivus
3
2
Lateral cephalogram
837
Figure 2
1
2
8
3 9
4
10
5
6
11
7
12
13
14
1. Supraorbital canal 8. Frontal sinus

2. Nasal septum 9. Zygomaticofrontal suture
3. Infraorbital margin 10. Innominate line
4. Ala of nose 11. Inferior orbital fissure
5. Zygomatic bone 12. Sphenoid sinus
6. Maxillary sinus 13. Foramen rotundum
7. Lateral wall of maxillary sinus 14. Mid-palatal suture
Paranasal sinus radiograph
Figure 3
1. Nasal septum
2. Mid-palatal suture
3. Sphenoid sinuses
4. Coronoid process of mandible
2 5. Ramus
3 6. Condyle of mandible
7. Clivus
4 8. Posterior pharyngeal
9. Anterior arch of atlas
3
5 10. Dens
11. Occipital condyle
14 7 12. Ossicles
15
8 13. External auditory meatus
6
16 9 14. Foramen spinosum
15. Foramen ovale
10 16. Hyoid bone
13 11
12
Submentovertex (base of skull) view
838
Chapter 33 – Extraoral Radiographic Landmarks
Figure 4
2
2
1
3 1. Lateral pole of condyle
2. Superior surface of condyle
3. Medial pole of condyle 1 3
4. Coronoid process
4
Closed mouth Open mouth
Temporomandibular joint (posteroanterior) view—closed and open mouth position
Figure 5
19
1. Superior rim of orbit
18 2. Lesser wing of sphenoid
1 3. Innominate line
17 2 4. Mastoid process
5. Foramen rotundum
3 6. Pterygoid process
16 4 7. Base of skull
8. Maxillary antrum
9. Zygomatic process of maxilla
10. Inferior turbinate
5 11. Intermaxillary suture
12. Nasal septum
15 6 13. Articulation between atlas and axis
14. Coronoid process
8 15. Infraorbital canal
16. Superior border of petrous
17. Superior orbital fissure
9 18. Ethmoid air cells
14 19. Frontal sinus
11 10
13 12
7
Posteroanterior view of the skull
839
Figure 6
1. Condyle head
2. Articular fossa
3. Articular eminence
2
3
Open mouth Closed mouth
Temporomandibular joint (lateral) view—closed and open mouth position
Figure 7
13
1
2
3
12 1. Foramen magnum
2. Posterior arch of atlas
3. Dorsum sellae
4 4. Frontal process of zygoma
5. Posterior wall of maxillary sinus
6. Inferior orbital fissure
7. Zygoma
8. Inferior rim of orbit
9. Nasal septum
5 10. Coronoid process of mandible
11 11. Condyle of mandible
12. Internal auditory meatus
13. Articular eminence
10
7
9
8
Reverse Townes’ view
840
Chapter 33 – Extraoral Radiographic Landmarks
Figure 8
15
16
10 9 13
18
6 12 14
2
5
17 11
3 7
4
8 19
20
1. Mandibular canal 8. Mental foramen 15. Condylar fossa

2. Inferior border of zygomatic arch 9. Soft tissue of inferior turbinate 16. Articular eminence
3. Posterior wall of maxillary sinus 10. Pterygomaxillary fissure 17. Real image of hard palate
4. Floor of maxillary sinus 11. Coronoid process 18. Common meatus of nose
5. Anterior wall of maxillary sinus 12. Condyle 19. Ghost image of inferior edge
6. Inferior orbital canal 13. Inferior orbital rim of contralateral ramus
7. Nasal septum 14. External auditory meatus 20. Body of hyoid
Orthopantomogram
Figure 9
27
28
22 22
21
25
21 21 21 21
21
24
26 29
23
30
21. Outline of base and dorsum of tongue 25. Nasopharyngeal air space 29. Angle of mandible
22. Air-space above the tongue 26. External oblique ridge 30. Inferior border of mandible
23. Hypoglossal air space 27. Ghost image of left palate
24. Oropharyngeal air space 28. Sigmoid notch
Orthopantomogram
841
CHAPTER
Site Selection, Evaluation
34 and Imaging for Dental

Implants
Muralidhar Mupparapu
Historical Perspectives Identification of the Implant Site

Indications for Dental Implants Selection of the Implant Site
Patient Management with Dental Implants Imaging of the Implant Site
Dental implants are prosthetic devices implanted into the anatomy of the maxillofacial region and the anatomical
oral tissues either beneath the mucoperiosteal layer or in variations that might be encountered. Secondly, they need
the bone to provide retention and support for the fixed to grasp the biomechanics of the dental implant material.
or removable prosthesis. Although fabrication of dental Fortunately, now, the science behind the dental implantol-
implants dates back to many decades, the advent of dental ogy is strong, backed with research and evidence-based
root form implants (DRFIs) have paved way for universal trials. The fruits of the decades of clinical trials using vari-
acceptance of implants as replacement for lost teeth. The ous dental implant materials along with numerous loading
dental implant restorations not only enhance the esthetics protocols have refined the predictability of dental implants
for the patients, but also improve the oral function for for the maxillofacial region.
speech and mastication. Hence, the dental root form implants
(Figure 1) gained immense popularity among the dental
practitioners as well as the patients in the dental profes- Historical Perspectives
sion. Nothing is more important in the dental implantology The treatment options that are available for replacement
than the precise evaluation and placement of the implant of single or multiple teeth in the oral cavity are removable
structures in the maxilla and mandible. In order to do this partial denture (RPD), fixed partial denture (FPD) and
effectively, one needs to precisely understand the surgical implant prosthesis (IP). The RPDs which were not helpful
in the maintenance of existing bone, compromise the
Figure 1 esthetic result, need more bulk for cross-arch stabilization,
easily trap food debris and plaque, and interfere with the
speech and function. Similarly, the FPDs especially pro-
mote caries of the abutment teeth but if not well fabri-
cated, lead to plaque retention in the pontic increasing
periodontal risk, involve damage to adjacent teeth, frac-
Upper lip turing of the porcelain, and have esthetic concerns for the
anterior regions. The FPDs are contraindicated when there
is poor abutment support, inadequate hard and soft tissues
in the esthetic regions and in young patients with large
Implant pulp horns. The dental implants would be an ideal alterna-
fixture
tive for replacement of teeth.
Crown Historically, there were three distinct forms of dental
prosthesis implants, namely, the subperiosteal (epiosteal), the transos-
seous (staple bone or transmandibular) and the endosteal
(blade or plate, ramus–frame and root form) types. Dental
Diagrammatic representation of the typical implant fixture and
practitioners should be familiar with the radiographic
the crown properly placed within the maxillary alveolus
appearances of older dental implant systems although many
842
Chapter 34 – Site Selection, Evaluation and Imaging for Dental Implants
CAD/CAM fabrication of the framework, eliminating the

Figure 2
need for impressions via surgery. Subperiosteal implants
are no longer preferred after the success of endosteal
Posts will remain
outside the gingiva
implants although one can occasionally see them in clini-
as anchors for the cal practice with varying degrees of presentation (Beddis
new prosthesis et al, 2012).
Transosseous implants
Subperiosteal metal
framework The transosseous implants, also known as staple bone
implants or transmandibular implants are restricted to the
anterior region of the mandible. They penetrate both corti-
cal plates and pass through the entire thickness of the bone.
Mandible Endosteal implants
Gingiva covering the
implant framework There are three forms of endosteal implants: the root form,
the ramus frame and the plate or blade form implants.
Diagrammatic representation of subperiosteal implant
framework and the posts in the edentulous mandible
Blade implants are rectangular in shape similar to a razor
blade. These are used over horizontal column of bone. One
or more posts usually extend into the oral cavity to permit
fixation of the prosthesis. The only blade implants that are
of them are rarely used after the advent of the dental root seen clinically at present are the previously placed implants
form implants. that either failed or fractured and the patient reported for
treatment. One such example where the blade implant fail-
Subperiosteal implants ure led to the osteomyelitis in the mandible is shown in
Subperiosteal implants are metallic meshes that are custom- Figure 3. The root form implants can be either cylinder-
built to fit over the alveolar processes and are located type, screw root type or a combination of both. These
underneath the periosteum (Mupparapu and Beideman, implants use the vertical column of bone unlike the blade
2000). Direct bone impressions via surgery would lead implants.
to the fabrication of mesh in a laboratory that fits under
Types of dental implants available
the mucoperiosteum (Figure 2). The framework normally
(according to the material used)
rests on the mandible with no penetration into the bone.
Two surgeries were involved in the fabrication of these The following are the commonly used implants according
implants. First surgery is for the impression and the sec- to the type of material used for fabrication:
ond surgery is for the placement of the mesh. The first
1. Metallic implants
subperiosteal dental implant in the world was placed by
a. Titanium
George Dahl in Sweden. The first American subperiosteal
b. Cobalt–chromium–molybdenum alloy with titanium,
implants were developed in 1947. Gershkoff and Goldberg
aluminum, vanadium
placed the first subperiosteal complete denture implant
c. Cobalt–chromium–molybdenum
manufactured with Vitallium (Moore and Hansen, 2004).
d. Stainless steel
Dr Leonard Linkow of New York made significant changes
e. Zirconium
to the original design by incorporating fenestrations into
f. Tantalum
the buccal peripheral struts which made it possible for the
g. Gold
mucoperiosteum to reattach the bone in between these
h. Platinum
fenestrations. He also holds 35 patents on various designs
of subperiosteal and oral implants. Multiple metallic posts 2. Non-metallic implants
extend from the mesh into the oral cavity crossing the a. Ceramics
mucoperiosteal barrier to support the prosthesis. Mandibu- b. Carbon.
lar subperiosteal implants have been shown to be success-
The most commonly used implants are:
ful in many clinical studies and some of the implants
reviewed were present in the mouth for more than 10 years. 1. Commercially pure (CP) titanium This is light weight,
The success rate for these implants varied in many studies biocompatible and corrosion resistant. The material is many
with higher success rate for 10-year periods and lower times stronger than compact bone and the modulus of
success rates for longer periods (Moore and Hansen, 2004). elasticity is five times greater than that of compact bone.
Computed tomography (CT) scans were also used to allow This equals the mechanical stress transfer of compact bone.
843
Figure 3
Failing blade implants in the mandibular molar region as seen from the panoramic reformatted image from a
Denta-Scan® CT study. On the right is a picture of a premolar blade implant manufactured by Oraltronics, Russia
Figure 4
Facial profile changes after loss of teeth including the collapse of the perioral soft tissues and loss of vertical dimension
2. Titanium–aluminum–vanadium alloy (Ti–6Al–4V) wear dentures for psychological reasons are all candidates
This is stronger and used for small diameter implants. for dental implants. In patients wearing complete den-
tures, bite force is decreased from about 200 to 50 psi
with decreased masticatory efficiency. Food selection is
Indications for Dental Implants
limited and healthy food intake is decreased among these
Implants are indicated in many situations in patients with patients. Hence the dental implants will greatly help them
partial or full edentulism. They are indicated in patients in getting back the normal occlusal function added with
with a history of difficulty in wearing a removable partial the enhanced esthetics. Finally, in patients with tooth
denture or have a long span fixed partial denture. They are agenesis (congenitally missing teeth), the implants are
also indicated in patients with severe bony changes sec- ideally suited.
ondary to prolonged use of complete or partial dentures. Patients who lost the entire dentition will show signifi-
The implants are ideal for patients with poor oral muscular cant changes to the facial profile after loss of teeth chiefly
coordination and in those with parafunctional habits com- due to the alveolar resorption and the loss of vertical dimen-
promising the stability of the prosthesis. Patients with sion (Figure 4). Restoration of function and esthetics would
hyperactive gag reflex, unfavorable number and location then become the goal for any prosthesis without compro-
of abutments, single tooth loss and those who refuse to mising on the masticatory efficiency.
844
Patient Management with Dental Implants Since the success of implant therapy is tied to the osseoin-
tegration, this is a crucial step in the overall implant ther-
There are three aspects of patient management that the apy. Several medical conditions that would jeopardize the
practitioner should remember when planning treatment success of an implant should be considered during the
for dental implants: screening process. The list (Hwang and Wang, 2006) includes
1. Identification of the appropriate site for implant place- the following absolute contraindications and relative con-
ment via preimplant assessment This requires patient traindications:
assessment via thorough medical and dental history, labo-
ratory findings and radiographic evaluation of the exist- Absolute contraindications
ing teeth and edentulous ridges.
1. Recent myocardial infarction or cerebrovascular accident
2. Selection of appropriate dental implant system (DIS) and Due to the increased risk of complications following a
fabrication of necessary guides for surgery This involves myocardial infarction (MI) or a cerebrovascular accident
selection of appropriate implant material and imaging soft- (CVA), the dental practitioner must wait until preliminary
ware to display the cone beam computed tomographic vol- stabilization. It is recommended that elective dental care is
umes and a good communication with the laboratory. deferred for at least 6 months status post MI or CVA and
the patient is medically stable.
3. Planning and designing appropriate abutment This
could be easily accomplished via virtual implant selection 2. Valvular prosthesis placement It is essential that the
and placement. The identification of appropriate length and dental practitioner defers placement of implants in these
width of the implant fixture would be determined at this patients for 6 months to 1 year after the cardiac surgery.
stage taking into consideration, the proximity of the nerve Anticoagulants would be used depending on whether the
canal and/or sinus cavity. valve is bioprosthetic or mechanical in which case, caution
should be exercised.
Identification of the Implant Site 3. Prior history of bleeding Since uncontrolled bleeding
stems from a multitude of systemic conditions, patients who
Selection of the patient are on oral anticoagulant therapy should be carefully eval-
Patient selection is a key factor in going forward with the uated and the bleeding time, international normalized ratio
identification of future implant site(s). This essentially boils (INR) must be assessed. An INR of 2.2 or lower is recom-
down to the assessment of the medical and dental status of mended for surgical procedures by Fazio and Fang (2003).
the patient. If the patient’s medical and dental history pre-
4. Immunosuppression If the WBC count falls below
cludes a dental implant placement and a good prognosis,
1,500–3,000 cells/mm3 the patient is not considered a good
then that should be communicated to the patient ahead of
candidate to receive implants as the ability to combat infec-
time. Systemic health of the patient plays a vital role in the
tions, repair or regeneration would be compromised. With
overall selection of the patient. Any medical condition that
a near normal WBC count and a grossly abnormal neutrophil
delays or alters wound healing should be identified first.
count (less than 2,000 cells/mm3), the normal range being
A good example for this is ‘diabetes mellitus’. Uncontrolled
3,500–7,000 cells/mm3, a medical consultation is necessary
type 1/type 2 diabetes or untreated diabetes usually results
before planning dental implants. It is generally considered
in delayed wound healing. Diabetes (type 1 and type 2
that when the patient’s CD4 T-cell count measures below
combined) affects approximately 25.8 million people in the
500 cells/mm3, he/she is considered immunosuppressed and
Unites States alone. There are at least 79 million people
appropriate care should be exercised.
who are considered prediabetics (National Diabetes Fact
Sheet, 2011, Centers for Disease Control and Prevention 5. Active radiation and/or chemotherapy Both ionizing
[http://www.cdc.gov/diabetes]). Worldwide, as per 2011 radiation and chemotherapy disrupt hematopoiesis, and
World Health Organization estimates, around 346 million implantation should be deferred in such situations as wound
people have diabetes (http://www.who.int/mediacentre/ healing is delayed. In addition, if the salivary glands are
factsheets/fs312/en/index.html). involved in the line of fire, there would be substantial
Wound healing occurs as a cellular response to injury xerostomia which in turn, would contribute to poor oral
and involves activation of keratinocytes, fibroblasts, endo- hygiene and increase in dental caries secondary to xero-
thelial cells, macrophages and platelets. Many growth fac- stomia. Studies have shown that in about 3–35% of patients,
tors and cytokines released by these cell types are essential spontaneous or traumatic osteoradionecrosis would be a
for coordination and maintenance of healing process complication (Marx and Johnson, 1987). When the patient
(Brem and Tomic-Canic, 2007). Deficiency of any of the is on cytotoxic anticancer drugs, granulocytopenia fol-
above factors would lead to improper and sometimes non- lowed by thrombocytopenia are expected which might
healing of the extraction site as well as the implanted site. lead to infection, hemorrhage, mucositis and pain. Implant
845
therapy should be deferred until after the patient is com- the case, an implant-supported overdenture is more likely
pletely off the cytotoxic medication. to provide greater lip support in such cases, giving more
acceptable esthetic result (Wismeijer et al, 2010).
6. Psychiatric disorders If a patient is unable to compre-
hend and anticipate dental treatment logically, then it is Relative contraindications
advised not to place implants. Several conditions includ-
ing psychotic disorders like schizophrenia, severe character There are situations where the condition lends to a milder,
associated conditions like hysteria, borderline personality often local complications or loss of implants in a patient
disorders, dysmorphophobia, cerebral lesions, presenile (Hwang and Wang, 2007). The following list, although
dementia as well as alcohol and drug abuse. Although comprehensive may not include every single situation and
there are no biological reasons for patients with most of or condition that might predispose local loss of bone and
the above disorders to lose implants, there are reported eventual failure of the implant:
cases of removal of osseointegrated fixtures based on psy- 1. Age of the patient Bone growth in the maxilla and
chiatric factors (Hwang and Wang, 2006). mandible is dynamic in growing children and adolescents.
7. Intravenous bisphosphonate treatment There is enough The implants simulate an ankylosed tooth in the growing
evidence to date linking bisphosphonate use to either spon- alveolus and might not be able to catch up with the verti-
taneous or traumatic induction of osteonecrosis of the jaw. cal growth of the alveolar process that happens naturally
Bisphosphonates inhibit bone resorption and hence are with the other permanent teeth. This might lead to infra-
used for treatment of osteoporosis, hypercalcemia of malig- occlusion or displacement with time with respect to the
nancy and Paget’s disease. Bisphosphonates may inhibit natural dentition. It is considered prudent to wait until the
osteoclast precursors and cholesterol synthesis as well as completion of skeletal maturation before placement of
promote osteoclast apoptosis and osteoblast proliferation. implants in the younger patients to avoid imperfect fixture
In a large study, among all the different forms of bisphos- positioning and/or stunt osseous expansion. The elderly
phonates that were used in cancer patients, pamidronate tend to have greater prevalence of local conditions like the
(Aredia®, Novartis, Basel, Switzerland) and zoledronic acid ridge resorption, nerve proximity, xerostomia as well as
(Zometa®, Novartis, Basel, Switzerland) stood out as the systemic conditions like osteoporosis, hypertension and dia-
agents that were associated with most cases of osteonecro- betes. Although old age does not seem to be a major con-
sis, majority of them being mandibular necrosis with recent tributing factor in the long-term survival of the implants,
dentoalveolar procedures performed on them (Ruggiero et al, the associated factors might lead to certain complications
2004). So far, there are no published studies on the risk (Hwang and Wang, 2007).
stratification of osteonecrosis of the jaws after drug discon- 2. Osteoporosis Osteoporosis is a condition that is preva-
tinuation. Hence, the risk is omnipresent once the bisphos- lent in the fourth or fifth decade in both men and women
phonates have been used and even if they are discontinued. in which, there is universal reduction in bone mass with no
Professional organizations like the American Dental Asso- other abnormality. The resorption overtakes deposition of
ciation, the American Association of Oral and Maxillofacial bone. Although it is generally thought to be a risk factor for
Surgeons and the American Academy of Oral Medicine have failure of osseointegration, the clinical studies that were
issued guidelines regarding the identification and manage- done revealed little effect of this condition on implant suc-
ment of bisphosphonate-related osteonecrosis of the jaw. cess, at least in the lower jaw (Minsk and Polson, 1998).
8. Acute infection The presence of acute infection is an 3. Smoking Studies have shown that persistent tobacco
absolute contraindication for oral implant therapy. The use following implantation lessened the ability of bone or
soft tissues and bone should be completely devoid of any other periodontal tissues to adapt over time and contribut-
infection before the implant therapy can be instituted. ing to the failure of treatment after fixture uncovering
(Lambert et al, 2000). The authors suggested smoking ces-
9. Morphology of the edentulous bone crest and vestibule
sation for all future implant receiving patients. More recent
Intraoral inspection and palpation may reveal information
studies that have examined the effect of smoking in patients
about the type of ridge, a suspected thin ridge or a flabby
with treated periodontal disease show mixed results and it
ridge, shape of the ridge, muscle insertions, mandibular tori
is suggested that since smoking appears to reduce implant
and the floor of the mouth. A radiographic evaluation of
success especially in the maxilla, smoking cessation before
the crestal bone is mandatory before treatment planning.
implant rehabilitation might lead to better results (Hwang
A knife-edged ridge often needs correction before insert-
and Wang, 2007).
ing implants. A bony plateau must be created that is wide
enough to insert implants with an adequate width. This 4. Diabetes Patients with both type 1 (previously called the
could mean reducing the existing crest by a few millimeters. insulin-dependent diabetes mellitus or the juvenile onset
The depth of vestibule is an important factor to consider. diabetes) and the type 2 (previously called the non-insulin
Bone resorption often leads to shallow vestibule. If that is dependent diabetes mellitus) diabetes mellitus are at risk
846
for implant failure due to the pathologic changes that are

Figure 5
brought about by the disease in the cardiovascular, renal
and circulatory systems which in turn lead to changes at
local level. The American Diabetic Association recommends
a hemoglobin A1C (HbA1C) level less than 7.0% in patients
with type 2 diabetes. With appropriate glycemic control,
diabetes does not compromise implant success (Hwang
et al, 2007).
5. Cardiovascular disease All forms of cardiovascular
diseases including the hypertension, coronary artery dis-
ease, vascular stenosis, atherosclerosis, congestive heart
failure may impair the healing process after implant place-
ment. Although cardiovascular disease leads to physiolog- Diagrammatic representation of the Misch classification using
mandibular bone. The short arrow with long arrow head
ical alterations, generally, it does not seem to affect the
points to the dense cortical bone in D1. The long arrow with
clinical implant success (Hwang et al, 2007).
short arrow head in D4 points to the generalized fine
6. HIV disease Acquired immunodeficiency syndrome trabecular bone with absence of cortical bone
remains one of the leading causes of death in the world
even though the mortality rate from HIV infection has
substantially declined in the past decade due to advent of for multiple sites. Historically, the most frequently used and
highly active antiretroviral therapies (HAART). This has available intraoral and extraoral radiographs were used to
become a chronic, manageable disease. In patients with evaluate the sites receiving the dental implant. Misch clas-
CD4 count of more than 200 cells/mm3, successful osseo- sified the bone density to four different groups based on
integration and function after 4 years was noticed and in the observed density of the bone (Misch, 1990).
patients with CD4 count less than 200 cells/mm3, a regimen
❍ D1 bone (1,250 Hounsfield unit [HU]) bone is com-
of the antibiotic amoxicillin was suggested post-operatively
posed of almost all cortical bone mass located primar-
for a successful outcome (Hwang et al, 2007).
ily in the anterior mandible.
7. Hypothyroidism Thyroid disorders generally affect ❍ D2 bone (850–1,250 HU) bone is composed of a thick
the bone metabolism. Both T4 and T3 regulate several crestal layer of cortical bone and coarse trabecular bone
homeostatic processes. Wound healing in both soft tissue underneath the cortical bone. This type of bone can
and bone is controlled by these hormones. Hypothyroidism mostly be found in the anterior maxilla and mandible
decreases bone cell recruitment, maturation and activity, and in the posterior mandible.
possibly by reducing the circulatory levels of insulin-like ❍ D3 bone (350–850 HU) is composed of a porous crestal
growth factor-1; this ultimately suppresses the bone forma- layer of cortical bone and fine trabecular bone under-
tion as well as resorption. Implant sites healing and osseo- neath the cortical bone. This type of bone can mostly
integration would be potentially delayed. Even though in be found in the anterior and posterior maxilla but also
theory, the hypothyroid state might lead to potential fail- in the posterior mandible. It is also seen after osteo-
ure in implant osseointegration, the studies that were done plasty of D2 bone.
in this area did not present data that corroborate the the- ❍ D4 bone (150–350 HU) bone is composed of primarily
ory. Hence, it can be concluded that in a well-controlled fine trabecular bone and often the absence of cortical
patient, hypothyroidism has no effect on the survival of bone. This type of bone can mostly be found in the
the dental implants. posterior maxilla and poses the greatest challenge in
implant placement (Figure 5). Implants usually placed
8. Past history of periodontal disease The risks related to
in D1 or D2 bone stand a very good chance of under-
implant loss in patients who lost their teeth to periodontal
going osseointegration, while the implants placed in
disease are much higher than the patients who lost their
D3 or D4 bone either undergo fibrointegration or fail
teeth to caries or trauma. The patients who lost their teeth
to integrate at all. There are many factors that affect
as a result of periodontal disease run a higher risk of
the long-term success or failure of an implant.
developing peri-implant infection even if they are com-
pletely edentulous (Heitz-Mayfield, 2008). Misch classification is a subjective grading of bone density
and the site in question might not always fall under a cer-
tain category as the bony anatomy is more complex than
Selection of the Implant Site
what appears visually. The visual radiographic analysis has
Imaging plays a key role in the selection of the site for man- to be augmented with other parameters of bone quality
dible or maxilla, whether the implant is for a single site or analysis. This might include factors such as the total
847
overall availability of bone and the health of the alveolar

Figure 6
bone and number of implants to be placed in the area.
They include correct initial placement of the fixture, suc-
cessful osseointegration of the fixture, and the ability of
the surrounding bone to withstand the occlusal and mas-
ticatory forces. A properly placed implant fixture can also
fail if the occlusal forces are excessive, leading to a break-
down of the bone around the fixture. Hence, it is important
to identify the correct occlusal scheme for the patient even
before restoring the implant. Three-dimensional and cross-
sectional reformatted images of the patient’s jaw through
the areas of interest demonstrate the spatial relationships
of the opposing arches. The cross-sectional images are
very helpful in assessing the direction of various force
vectors acting on an implant placed and gives the clini-
cian clues for preimplantation bone augmentation surgery
(Delbalso et al, 1994). Edentulous maxillary areas showing alveolar extensions of
The widely available panoramic radiography and the the maxillary sinus and one of the periapical images of
right maxillary molar region showing the presence of a
linear tomography have a very limited value in the assess-
compound odontoma in the right maxillary sinus
ment of bone quality. Preoperative assessment of bone
density is now performed primarily by radiographic means
that includes the cross-sectional data. More recently dental
CT has become more acceptable standard procedure.
Standard periapical radiographs
Although standard periapical radiographs have value in
Imaging of the Implant Site
the evaluation of single implant sites for the availability
Diagnostic imaging is an essential and integral compo- of bone in the superior-inferior direction (Figure 6), the
nent of the implant treatment planning. Until the late evaluation of bucco-lingual width of the bone is not pos-
1980s, conventional radiographic techniques, namely, intra- sible with this two-dimensional (2D) imaging technique.
oral, panoramic, cephalometric radiographs have been the The periapical radiographs can be used to identify the areas
accepted standard (Harris et al, 2002). Developments in for possible implant placement and select the appropriate
the cross-sectional imaging techniques such as complex advanced imaging procedure. However, the periapical radio-
motion tomography, reformatted CT have become avail- graphs can be used for post-placement evaluation of the
able and were found to be useful compared with the previ- implants for angulations and evaluation of suspected peri-
ously accepted imaging techniques. Additionally, available implantitis (Figure 7).
proprietary and third party software paved way for the
visualization of the third dimension and virtual position-
Panoramic radiography
ing of the implant in the radiographic volumes before the
implant surgery. The European Association for Osseointe- Panoramic radiographs were used in the past for identifica-
gration (EAO) recommended that even for single implant tion of the implant sites via radiographic markers but lacked
site, cross-sectional imaging may be beneficial and allows the third dimension (Figure 8). The uneven and unreliable
for more precise treatment planning. For all posterior magnification of the image as well as the projection arti-
maxillary and mandibular sites that are partially or fully facts made it impossible to be accurate especially when an
edentulous, a cross-sectional imaging will provide sufficient implant is being placed close to the mental foramen, man-
information regarding the bone volume and the proximity dibular canal or the maxillary sinus floor. This technique
to the inferior alveolar nerve canal and/or the maxillary became largely outdated after the advent of the cross-
sinus. Cross-sectional images will also help in the predict- sectional imaging. The technique still has value for post-
ability of prosthetic results and transferring this informa- operative assessment of the implant fixture similar to the
tion to guide the clinician or the implantologist in implant periapical radiographs.
positioning. Special techniques such as zygomatic implants
may also dictate the need for additional imaging. Post-
Conventional tomography
operative monitoring cross-sectional imaging is not a part
(linear or complex motion cross-sectional tomography)
of the routine protocol unless there is a need for such an
imaging due to an infection or suspected endangerment of Quint Sectograph was one of the first linear tomographic
the neighboring vital structures. machines that were available in the United States market
848
Figure 7 Figure 9
Periapical radiograph of maxillary left first molar region

showing the grafted bone in the maxillary sinus and the
root form implant in place. The implant appears to be
stable with no evidence of peri-implantitis.
Tomax ultrascan® complex motion tomographic
Courtesy: Dale Rosenbach, DMD
machine
Figure 8
Dental computed tomography
Multidetector computed tomography (MDCT) is an imag-
ing technique that uses reconstruction mathematics to
create images based on a series of radiographs. The initial
CT concepts were developed by Alan Cormack and later
brought to fruition by a British engineer Godfrey Hounsfield
in 1972. The technique was first used to produce cross-
sectional images of the brain. The radiographic densities
of the imaged structures were compared along a scale with
the standard densities of water, air and the bone. The units
came to be known as Hounsfield units (HU) or CT num-
bers. The HU has been used for measurement of radioden-
sity of structures being examined. The numerical scale
Cropped panoramic radiograph showing the edentulous ranges from air (1,000) to water (0) and to bone (1,000)
maxilla and partially edentulous mandible. The two linear and upward of 1,000 for even denser structures. The appli-
opacities above the crestal level in the region of the cation of HU measurement can decrease the observer bias
left mandibular molars are the radiopaque markers inherent in the interpretation of plain films. Overall, the
(gutta percha) that were used with imaging HUs are important in an objective determination of bone
density. Dental CT and the concepts of curved plane
tomography were first published by Schwarz and coworkers
when linear tomography became available as a modality in 1987 (Schwarz et al, 1987), which were utilized in the
in the early 1930s. They were eventually used worldwide. dental CT studies for reformatting the multiplanar and
The complex motion tomographic machines were intro- cross-sectional views. In addition, the dental CT programs
duced to dental imaging market in the 1990s. The machines like the ‘DentaScan’ were able to produce the ‘panoramic
like Tomax® (Figure 9) and Commcat® were used success- reformation’ from the conventional CT datasets (Figure 10).
fully for TMJ imaging as well as implant related cross- This gained acceptance by the dental practitioners. Even
sectional imaging. A digital acquisition charge coupled though the CT radiation dose was higher compared to all
device camera was available with the Tomax ultrascan but the previous radiographic modalities, the benefit of accu-
the technology was soon outdated by the introduction of rate placement of implants outweighed any potential
dental CT and eventually by the CBCT. risks.
849
Figure 10
DentaScan® software generated CT images. Courtesy: General Electric Company, USA
and communications in medicine (DICOM) format. These

Cone beam computed tomography (CBCT)
DICOM sets (with a file extension of .dcm) are used for
Introduced in the mid 90s, this dental specific CT scanning reconstruction and display using appropriate software.
technology became a well-liked imaging technology, mostly The acquisition of CBCT volumes and the resultant
due to the smaller footprint of the machines that can be anatomy is dictated by what is known as the FOV. The
purchased and installed in dental offices. Secondly, the scanner’s FOV determines how much of the patient’s anat-
radiation doses were much lower compared to the MDCT omy one can visualize. Scanners using flat panel detectors
scanners, which were located mostly in the hospitals. CBCT describe the dimensions of their cylindrical FOVs as height
is a relatively new technique, which shares some of the by width (H W). Scanners that use image intensifiers and
basic principles with MDCT. CBCT uses a cone beam instead charge-coupled devices (CCDs) as their detectors identify
of a fan shaped beam and all the basis images are acquired their spherical FOVs as cm3. Figure 12 shows the grouping
in one single pass instead of multiple passes as in MDCT. of the FOVs based on their largest unstitched FOVs. A scan-
The acquisition speed and relatively low dose in CBCT lead ner with large FOV (at least 16 18 cm) will demonstrate
to increased noise and lower image resolution when com- at least the roof of the orbits superiorly and the hyoid
pared to MDCT. Hence, utilization of HUs derived from bone inferiorly. The large FOV scans are used for orth-
CBCT imaging has come under criticism due to the differ- odontic, maxillofacial prosthodontic or oral surgical pur-
ences in acquisition of CBCT volumes as compared to the poses commonly but can also be used in other situations
MDCT. The CBCT volumes are acquired via a 360-degree where there is a need for such a volume (Figure 13). The
rotation of the X-ray source using a flat panel detector. voxel sizes vary from scanner to scanner and the range is
The raw data as shown in the Figure 11 will be transmitted from 0.075 to 0.4 mm. Medium FOV (at least 8 14 cm)
first back to the workstation. When the technologist con- scanner capture typically from the infraorbital area supe-
firms the field of view (FOV), the actual acquisition will be riorly down to lower border of the mandible inferiorly and
completed, and the entire study is stored as digital imaging from condyle to condyle horizontally. These views are
850
Figure 11 Figure 13
Appearance of a raw CBCT scan volume Showing the CBCT machine, Kodak 9500® that has multiple
FOV settings. Both medium and large FOV settings are
available in this machine
Figure 12
volumes practical for storage and transmission, optimal
voxel sizes are employed.
In a recent consensus of the International Congress of
Oral Iimplantologists, the use of CBCT implant placement
Large volume was discussed and the proceedings were recently published
Height: >16 cm
Width: >18 cm
(Benavides et al, 2012). It is recommended that the CBCT
along with all other radiographic examinations must be
justified on an individual need basis for implant planning.
The benefits of these examinations must clearly outweigh
the potential radiation related risks. CBCT should be used
as an imaging alternative in cases where the projected
implant receptor site or the bone augmentation sites are
Small volume (Limited)
suspects for adequacy of bone. When used, the smallest
Height: >4 cm possible volume should be imaged and the entire volume
Width: >4 cm
should be interpreted.
Medium volume
Height: >8 cm Implant manufacturers have developed specific designs
Width: >14 cm for their implant fixtures taking into consideration the
anatomy, retentive capacity and osseointegration ability
of each implant. The implant interface that will face the
Diagrammatic representation of large, medium and
small volume CBCT field of view (FOV)
bone is designed to promote osseointegration with the bone
after implants are placed. Hence, the biocompatibility of
implant material is very important for the success and
long-term prognosis of the implant fixture.
used commonly for preimplant assessment in maxilla and
mandible and the assessment of maxillary sinuses and the
Radiographic appearance of screw-type and
TMJs bilaterally. The range of minimum voxel sizes vary
cylinder-type implants
from 0.080 to 0.13 mm and the maximum could be as
large as 0.4 mm. Small FOV (at least 4 4 cm) scanners are As shown in Figure 14, the implant fixtures are unique to
used for capturing a small area within the mandible or each manufacturer and the type of implant can perhaps be
maxilla, usually for preimplant assessment or periodontal recognized by the radiographic appearance of the fixture.
or endodontic needs. The minimum voxel sizes here vary The figure shows various Nobel Biocare’s root form implants.
from 0.076 to 0.15 mm. Generally, smaller voxel sizes are In Figure 15, the cylinder-type implants are noted (IMZ
used in small FOV scanners and larger voxel sizes are used dental implants). The radiographic appearances will be
in medium and large FOV scanners. The data volume of useful in post-operative radiographic identification of the
the CBCT study depends on the voxel size and to keep the type of implants.
851
Figure 14
Nobel Biocare Nobel Biocare Nobel Biocare Nobel Biocare Nobel Biocare
Replace Select Nobel Speedy Nobel Speedy Nobel Perfect Nobel Perfect
Straight RP Groovy RP Shorty WP (View A) (View B)
[4.3 × 11.5 mm] [4.0 × 13 mm] [6.0 × 7 mm] [4.3 × 10 mm] [4.3 × 10 mm]
Nobel Biocare Nobel Biocare Nobel Biocare Nobel Biocare Nobel Biocare
Immediate (Branemark) (Branemark) (Branemark) (Branemark)
Provisional Implant Self-tapping Conical Self-tapping Branemark MK I Branemark MK I
[3.75 mm] [3.75 × 10 mm] [3.75 × 10 mm] [3.75 × 13 mm]
Radiographic appearance of screw-type dental implants manufactured by Nobel Biocare.

Courtesy: www.whatimplantisthat.com
Figure 15
IMZ (Attachments Int.) IMZ (Attachments Int.) IMZ (Attachments Int.) IMZ (Attachments Int.)
High Cyclinder High Cyclinder High Cyclinder Hex Head
[4.25 × 11 mm] [4.25 × 11 mm] [3.3 × 10 mm] [4.0 mm]
Cylinder-type IMZ’s dental implants. Courtesy: www.whatimplantisthat.com
Placement of implants, software manipulation and

and the width of the remaining bone are measured. This
assessment of osseointegration
knowledge will help the clinician choose the implant with
The anatomy of the site should be thoroughly checked appropriate dimensions. In order to make sure that the
before attempting to measure the area. Typically, the height implant size and placement angulation are predictable, the
852
clinician uses software that are either provided by the via a process called ‘paint the canal’ using pre-identified
manufacturer or by utilizing the third party software sepa- coordinates. If a radiographic template/guide (Figure 16)
rately via importing the data volume to the software. The was used before the radiographic examination, then that
implant is ‘virtually’ placed within the volume. Before template can be converted to a surgical template by the
placing the implant, the mandibular canal is mapped out laboratory using the information gained from the CT scans.
Figure 16 Figure 17
The existing complete denture can be used for

fabrication of a radiographic guide and a surgical guide. Lab prepared surgical guide in the patient’s mouth before
Courtesy: Hani Braidy, DMD implant placement. Courtesy: Hani Braidy, DMD
Figure 18
CBCT showing the anterior extension of the mental foramen (arrows) in the edentulous mandible. The CBCT-based
cross-sections near the midline demonstrate the ‘lingual foramen’ which would be a blind sac that derives the
neurovascular bundle from the lingual nerve and artery
853
The surgical template so formed will have the prefabri- The surgeon or the implantologist places the implant
cated implant guides. A typical surgical guide that has fixture using a surgical guide and based on whether the
been fabricated using an existing denture is shown in the system is one-stage or two-stage, healing abutments would
Figure 17. be placed before closure of the wound. Single-stage implants
Figure 19
CBCT cross-sectional views (parasagittal views) of the left mandible showing the ‘dumbbell-shaped’ anatomy and
the clear delineation of the mandibular canal and the mental foramen (arrow)
Figure 20
Another instance of preimplant CBCT scan showing the reformatted panoramic view and cross-sectional views of the
left molar area. Note the measurements that are directly digitally obtained from the sections
854
Figure 21
CBCT reformatted panoramic view and maxillary anterior cross-sections showing knife-edge shaped ridge
Figure 22
CBCT reformatted panoramic view and maxillary posterior cross-sections showing severely resorbed ridges bilaterally
855
Figure 23
Bone grafting completed in the maxilla bilaterally via sinus lift procedure as shown in the
panoramic reconstruction of CBCT and left cross-sectional views of the maxilla
can be loaded immediately and the clinician can make that by the display and enhancement software (Figure 20).
determination based on the type of implant, anatomy of The measurements are usually considered 1:1 without any
the implant site and other pertinent factors. significant magnification or distortion. Hence, the measure-
The inferior alveolar nerve canal is typically very dis- ments can be translated directly to the selection of appro-
tinct bilaterally up to the mental foramen. From mental priate implant sizes. Ridge resorption is noted commonly
foramen onward medially toward the midline, there are in the anterior maxilla as shown in Figure 21. Once a knife-
instances where the smaller branches of the mandibular edge like ridge is noted, then bone grafting becomes nec-
canal continue even after the mental foramen, Figure 18 essary to be able to place root form implants in that region.
demonstrates one such example. Trauma to this foramen Posteriorly, if the ridge is severely resorbed (Figure 22)
leads to excessive bleeding from the lingual vessels. A nerve bone grafting can be done via ‘sinus-lift’ procedure and
injury in this area might lead to numbness of the sublin- the appropriate implants can be placed using the newly
gual area close to the genial tubercles. Hence, care should gained bone volume (Figure 23). This has become increas-
be exercised while planning implants in the anterior man- ingly common and complications are relatively rare with
dible between the two canines. In the posterior areas of the improvements in both the technique and the materials
the mandible, the location of the mental foramen and the used for grafting. If appropriate imaging is not performed
mandibular canal are important landmarks for identifica- before the implant placement, the implant fixtures could
tion and measurement of the residual alveolar bone. The be disastrously placed sometimes leading to immediate loss
bone height and width are carefully measured and the of implants (Figure 24). Adequacy of bone and the quality
bony anatomy of the ridge and the basal bone is observed of the bone is paramount to the success of the root form
for anatomical variations. One such variation could be a implant. The effective radiation doses are an important
‘dumbbell-shaped mandible’ as shown in Figure 19. If the consideration before selecting any radiographic procedure.
bone width and alveolar bone direction is misjudged in CBCT examinations should be prescribed only after a thor-
such situations, it leads to selection of the wrong type of ough patient examination preceded by collection of an
implant and possibly an unsuccessful outcome. equally thorough medical and dental history as was dis-
During preimplant assessment of the site, the measurements cussed in the beginning of this chapter. The effective radi-
are digitally obtained using the measuring tool provided ation doses for CBCT-based procedures are in the range
856
Figure 24
A SimPlant® study of a patient showing the disastrous outcome of the implant fixtures. Poor planning and
lack of preimplant imaging usually leads to such results. Note the displacement of an implant
into the left maxillary sinus. Courtesy: Barry E Zweig, DDS
of 30–400 Sv as compared to a panoramic radiograph bone healing around the fixture. Three-dimensional imag-
(2.7–23 Sv) and maxilla-mandibular CT (180–2,100 Sv). ing is recommended only when complications arise from
These measurements are based on effective radiation dose the placement. A good example would be to evaluate the
compilation from various studies. The dose comparisons nerve canal proximity if the patient is symptomatic for
can be found online at the European CBCT guidelines site nerve injury. In such situations, a limited volume CBCT
for dental and maxillofacial radiology (www.sedentext.eu). would be ideal to evaluate the area. Dental implantology
and implant imaging are rapidly expanding areas of den-
tistry. The information presented in this chapter should
Post-operative evaluation of implants and
provide some basic concepts regarding implant selection,
future directions
imaging and placement strategies for both doctoral and
Imaging modalities used in the post-operative evaluation graduate level dental students. However, the reader is
of the dental implant patient include intraoral periapical encouraged to follow the literature on the subject for a
radiography and panoramic radiography for assessment of more up-to-date knowledge.
857

SECTION Appendices
XIII
Appendix 1 Terminologies 861

Appendix 2 Summary of Radiographic Pathology 867
Appendix 3 Characteristics of Ideal Radiograph 871
Appendix 4 Indications for Intraoral Radiography 872
Appendix 5 Patient Position for Extraoral Radiography 873
859
Appendices.indd 859 10/3/2012 3:15:07 PM


APPENDIX
Terminologies
Ravikiran Ongole 1
Abrasion Pathologic wearing away of tooth substance Anomaly An irregularity or deviation from normal; an
due to an abnormal mechanical process, e.g. faulty tooth- abnormal structure.
brushing technique.
Anosmia Inability to detect odors.
Abscess Localized collection of pus. Antibody An immunoglobulin molecule that reacts with
Absorbed dose ‘It is a measure of the total energy a specific antigen that induced its synthesis. Synthesized
absorbed by any type of ionizing radiation per unit mass by B lymphocytes that have been activated by the binding
of any type of matter’. It is given by D ⫽ d␧/dm, where d␧ of an antigen to a cell surface receptor.
is the mean energy imparted to matter of mass (dm) by Antigen Any substance, almost always a protein, not
ionizing radiation. The SI unit for absorbed dose is gray normally present in the body which when introduced to
(Gy). 1 Gy ⫽ 1 joule per kilogram (J/kg). The traditional unit the body stimulates a specific immune response and the
of absorbed dose is rad (radiation absorbed dose). production of antibodies.
Acantholysis Dissolution of the intercellular bridges of Apoptosis (Greek—falling off) It is defined as programed
the prickle cell layer of the epithelium. cell death. The cell gets fragmented into membrane-bound
particles that are subsequently eliminated by phagocytosis.
Acanthosis Hypertrophy or thickening of the prickle cell
layer of the skin. Atopy A clinical hypersensitivity state which is subject
to hereditary influences, e.g. asthma, eczema.
Acute Sudden onset of signs and a short course.
Atresia Absence or closure of a normal body orifice or
Agenesis Absence or failure of formation of any part or passage.
organ.
Atrophy A decrease in the size of a normally developed
Ageusia A total lack of taste perception. organ or tissue.
Agnosia Inability to classify or contrast odors, although Attrition It is the physiological wearing away of tooth
able to detect odors. substance as a result of tooth-to-tooth contact, e.g. age-
related wearing away of the occlusal/incisal surfaces.
ALARA ‘As Low As Reasonably Achievable’, means that
exposure to ionizing radiation should be kept as low as Bacteremia Presence of bacteria in blood.
reasonably achievable, economic and social factors being Becquerel (Bq) It is the international unit of radioactivity.
taken into consideration. It is defined as the number of radioactive decays (disinte-
Analgesia A drug that dulls the sense of pain. It relieves grations)/unit of time. 1 Bq ⫽ 60 disintegrations/minute or
pain without loss of consciousness. 1 Bq ⫽ 1 disintegration/second.
Anaphylaxis The immediate immunologic (allergic) Blanching (French—white) It refers to change in color
reaction initiated by the combination of antigen (allergen) of a tissue to a paler and lighter shade when pressure is
with mast cell cytophilic antibody (chiefly IgE). applied.
Bulla Elevated blister containing clear fluid and greater
Anaplasia A condition in tumor cells in which there is
than 1 cm in diameter, e.g. pemphigus, pemphigoid.
loss of normal differentiation, organization and specific
function. (Adult cells that have changed irreversibly Cachexia This refers to a state of decreased tissue or organ
toward more primitive cell types. These changes are often mass resulting from a state of malnutrition or underlying
malignant.) severe debilitating disease.
861
Section XIII – Appendices
Carcinoma in situ The most severe stage of epithelial Diagnosis, final It is the diagnosis that is arrived at after
dysplasia, involving the entire thickness of the epithelium, all the necessary data history, clinical examination and
with the epithelial basement membrane remaining intact. investigations have been collected, analyzed and subjected
to a logical thought process.
Cellulitis Acute inflammation which spreads rapidly
through tissue spaces and along tissue planes. It is usually Disintegration A spontaneous nuclear transformation.
suppurative in nature.
Disorder, congenital It is the disorder which occurs dur-
Chancre Painless ulceration formed during the primary ing the development process (intrauterine life). The mani-
stage of syphilis. This infectious lesion forms approximately festation of which is seen either at birth or later in life.
21 days after the initial exposure to Treponema pallidum.
Disorder, developmental It is the abnormality that occurs
Cheilitis Inflammation of the lips. during the formative stage of an organ or tissue.
Cheilosis Condition characterized by fissuring and dry Disorder, genetic It is a disorder caused by abnormality
scaling of the vermilion border of lips (usually seen as a in the gene or an abnormality in mutation.
feature in riboflavin deficiency).
Disorder, inherited It is the disorder, which is transmitted
Chloroma Malignant green color tumor occurring any- through genes to the offspring of the next generation or to
where on the body and originating from myeloid tissue. subsequent generations.
These are associated with myelogenous leukemia.
Dose It is the amount of energy absorbed per unit of mass
Choristoma Microscopically normal cells that are present at a site of interest. Expressed in units of rad or gray.
in abnormal location.
Dose equivalent (DE) Absorbed dose multiplied by cer-
Chronic Slow onset, mild but continuous manifestations tain modifying factors. The principle factor is the quality
and long-lasting, often progressive effects. factor (QF), which corrects absorbed dose for relative bio-
Congenital Conditions that are present at birth, regard- logical damage for quality factors for:
less of their causation. Beta, gamma and X-rays ⫽ 1
Alpha ⫽ 20
Contusion A bruise; an injury of a part without a break in
1 rad ⫽ 1 rem for beta, gamma and X-ray.
the skin, characterized by swelling, discoloration, and pain.
Various types of radiation, expressed in units of rem or
Consanguinity In genetics, the term is generally used to sievert.
describe mating or marriages among close relatives.
a. Deep dose equivalent—applies to the external whole
Controlled area It is a limited access area in which the occu- body exposure in terms of dose equivalent at a tissue
pational exposure of personnel to radiation is under the super- depth of 1 cm (1,000 mg/cm2).
vision of an individual incharge of radiation protection. The b. Shallow dose equivalent—applies to the skin at a max.
reduced exposure in that area is to be ⬍26 mC/kg/week. Tissue depth of 0.007 cm (7 mg/cm2).
Curie (Ci) The number of radioactive decays (disintegra- c. Effective dose equivalent—is the sum of the product of
tions)/unit of time. the dose equivalents each weighted by a factor for the
1 Ci ⫽ 2.2 ⫻ 1012 disintegrations/minute tissue organ in question.
1 Ci ⫽ 3.7 ⫻ 1010 disintegrations/second. Dose, loading A larger than normal dose administered as
Cyst It is a pathological cavity filled with a fluid, semi- the first in a series of doses, the others of which are smaller
fluid or gaseous material but not by pus, which may fre- than loading dose but equal to each other. The loading
quently but not always lined by epithelium. dose is administered in order to achieve a therapeutic
amount in the body more rapidly than would occur only
Decay Radioactive decay or the disintegration of the by accumulation of the repeated smaller doses.
nucleus of an unstable atom by spontaneous emission of
energy in the form of high speed particles or electromag- Dose, maintenance The smaller doses which are given
netic waves. after the loading dose are called ‘maintenance’ doses.
Diagnosis, clinical/provisional/tentative It is the diagnosis Drug, bioavailability The percentage of dose entering
that is made after thorough case history taking and clinical the systemic circulation after administration of a given
examination but before any investigations are performed. dosage form.
Diagnosis, differential Identification of a particular dis- Drug, first pass effect All drugs that are absorbed from
ease by differentiating it from all other disease processes the intestine enter the hepatic portal vein and pass through
that may have similar symptoms and signs. the liver before they are distributed systemically.
862
Appendix 1 – Terminologies
Drug, half-life The period of time required for the con- Exudate Fluid with a high concentration of protein and
centration or amount of drug in the body to be reduced to cellular debris which has escaped from blood vessels and
exactly one-half of a given concentration or amount. has been deposited in tissues, or on tissue surfaces, usually
as a result of inflammation. (Specific gravity can be 1.020
Dysgeusia An altered taste sensation.
or higher and high protein content can be 1–6 g/dl.)
Dysosmia Distorted identification of smell.
Fascial spaces Potential tissue spaces through which
Dysphagia Painful swallowing or difficulty in swal- infection spreads easily, e.g. submandibular space, buccal
lowing. space, pterygomandibular space.
Dysplasia An abnormality of development characterized Final diagnosis It is the diagnosis arrived at after all the
by the loss of normal cellular architecture. data has been collected, analyzed and subjected to logical
thought.
Ecchymosis Larger purpuric lesions.
Fistula It is an abnormal communicating tract between
Edema Accumulation of excess fluid in the intercellular two epithelial surfaces or organs. A fistulous tract is gen-
or interstitial tissue spaces or body cavities. erally lined with granulation tissue but over a period of
Effective dose (E) It is a radiation quantity used to esti- time may become epithelialized.
mate the risk in humans. Glossodynia Pain in the tongue.
It is the tissue-weighted sum of the equivalent doses in
all specified tissues and organs of the body, given by the Glossopyrosis Burning sensation in the tongue.
expression: Granuloma Tumor-like mass of inflammatory tissue con-
E ⫽ ⌺T WT ⌺RWRDT, R or E ⫽ ⌺WT X HT sisting of a central collection of macrophages, often with
multinucleated giant cells, surrounded by lymphocytes.
where HT or WR DT, R is the equivalent dose in a tissue or
organ T, and WT is the tissue weighting factor. The unit for Granulomatous Pertaining to a well-defined area that
the effective dose is the same as for absorbed dose, J kg⫺1, has developed as a reaction to the presence of living
and its special name is sievert (Sv). organisms or a foreign body. The tissue consists primarily
of histiocytes.
Epiphora Tearing from the eye.
Gray (Gy) The international unit of absorbed dose.
Epistaxis Bleeding from the nose. 1 Gy ⫽ 100 rad; 1 Gy ⫽ 1 joule/kilogram (J/kg).
Equivalent dose (HT) It is a radiation quantity used to Hamartoma Abnormal proliferation of tissues of struc-
compare the biological effects of different types of radia- tures native to the part.
tion on a tissue or organ.
The dose in a tissue or organ T is given by: Hematoma Tumor-like mass produced by coagulation of
extravasated blood into tissues from ruptured blood vessels.
HT ⫽ ⌺WRDT, R
Hemiparesis Weakness on one side of the body.
where DT, R is the mean absorbed dose from radiation R in
a tissue or organ T and WR is the radiation weighting fac- Hemiplegia Paralysis of one side of the body.
tor. Since WR is dimensionless, the unit for the equivalent Hereditary A condition that may be genetically trans-
dose is sievert (Sv). 1 Sv ⫽ 1 Gy. Traditional unit of equiva- mitted from parent to offspring.
lent dose is the rem (Roentgen equivalent man).
High radiation area It is defined as an area, accessible to
Erosion A shallow defect in the oral mucosa representing individuals, in which radiation levels from radiation
a loss of covering epithelium down to, but not involving sources, external to the body could result in an individual
the stratum germinatum, e.g. erosive lichen planus. receiving a dose equivalent in excess of 0.1 rem (100 mrem)
Erosion (wasting disease) It is the loss of tooth sub- in 1 hour at 30 cm from the radiation source or from any
stance by a chemical process that does not involve known surface that the radiation penetrates.
bacteria, e.g. excessive consumption of citrus fruits. Hyperemia Presence of an increased amount of blood in
a part or an organ.
Exposure A special radiation quantity of ionization in air
from X-rays or gamma rays. It is measured as the amount Hyperplasia Enlargement caused by an increase in the
of charge per mass of air, which is coulombs/kg. Units are number of normal cells.
expressed in roentgen (R). 1 R ⫽ 2.58 ⫻ 10⫺4 C/kg.
Hypertelorism Abnormal increased distance between two
External exposure Radiation exposure from a source organs or parts. Ocular hypertelorism refers to increased
outside the body. interorbital distance.
863
Hypertrophy Increase in the size or volume of a tissue or Neoplasm Abnormal mass of tissue, the growth of which
organ as a result of enlargement of the cell. exceeds and is uncoordinated with that of the normal tis-
sues and persists in the same excessive manner after ces-
Hypogeusia Substantial loss of all taste sensation.
sation of the stimuli which evoked the change.
Hypoplasia Incomplete development of a tissue or organ;
Nevus A small flat elevated pigmented or non-pigmented
a tissue reduced in size because of a decreased number of
lesion of congenital origin involving the skin or mucous
constituent cells.
membrane.
Hyposmia Decreased ability to detect odors.
Nodule Solid, elevated lesion varying in size from 5 mm
Iatrogenic Adverse condition in a patient resulting from to 2 cm, e.g. fibroma.
treatment by a physician or surgeon.
Oral diagnosis It is the art of using scientific knowledge
Id reaction Hypersensitivity reaction to candidal antigen, to identify oral disease process and to distinguish one dis-
manifests as vesicular and papular rash on skin of patients ease process from the others.
with chronic candidiasis.
Oral medicine It is the specialty of dentistry that is con-
Idiopathic Occurring without known cause. cerned with the oral healthcare of medically compromised
Induration Abnormally hard portion of a tissue with patients and with the diagnosis and non-surgical manage-
respect to the surrounding similar tissue; often used to ment of medically related disorders or conditions affecting
describe the feel of locally invasive malignant tissue on the oral and maxillofacial region. Oral medicine specialists
palpation. are concerned with the non-surgical medical aspects of den-
tistry. These specialists are involved in the primary diag-
Ischemia A local deficiency of blood due in part to func- nosis and treatment of oral diseases that do not respond to
tional constriction or actual mechanical obstruction of a conventional dental or maxillofacial surgical procedures.
blood vessel. The practice of oral medicine will provide optimal health to
Kerma (K) It is the quotient of the sum of the kinetic ener- all people through the diagnosis and management of oral
gies, dEtr, of all charged particles liberated by uncharged diseases.
particles in a mass, dm, of material. Papule Small circumscribed solid elevated lesion varying
K ⫽ dEtr/dm in size from a pinhead to 5 mm, e.g. Fordyce’s granules.
Kerma is a non-stochastic quantity and dEtr is the expec- Paraparesis Weakness affecting the lower extremities.
tation value of the sum of the kinetic energies. The unit for Paraplegia Paralysis of the lower limbs.
kerma is joule per kilogram (J/kg) and its special name is
gray (Gy). Paresis Slight or partial paralysis.
Lesion A morphological alteration of the tissue with Paresthesia It is an altered sensation or abnormal sensa-
objective signs of disease. tion (tingling sensation).
Linear energy transfer (L or LET) It is the average linear Parosmia Altered perception of smell in the presence of
rate of energy loss of charged particle radiation in a medium, an odor, usually unpleasant.
i.e. the radiation energy lost per unit length of path through Paroxysmal Recurring ‘sudden attacks’ of symptoms.
a material. That is, the quotient of dE by dl where dE is the
mean energy lost by a charged particle owing to collisions Parulis A sessile nodule on the gingiva at the site where
with electrons in traversing a distance dl in matter. a draining sinus tract reaches the surface.
L ⫽ dE/dl Petechiae Pinpoint purpuric spots, 1–2 mm in diameter.
The unit of L is Jm⫺1, often given in keV ␮m⫺1. Phantosmia Perception of smell without an odor present.
Macule It is a circumscribed non-raised area of altered Plaque A circumscribed solid elevated lesion greater than
coloration varying in size from a pinhead to several centi- 5 mm in diameter, e.g. leukoplakia.
meters in diameter, e.g. petechiae, melanin pigmentation
Precancerous condition Generalized state associated with
in Addison’s disease.
a significantly increased risk of cancer, e.g. Plummer–
Metastasis The transport of neoplastic cells to parts of Vinson syndrome, oral submucous fibrosis.
the body remote from the primary tumor and the estab-
Precancerous lesion A morphologically altered tissue in
lishment of new tumors in those sites.
which cancer is more likely to occur than its normal coun-
Necrosis Death of a cell as a result of disease or injury. terpart, e.g. erythroplakia, leukoplakia.
864
Appendix 1 – Terminologies
Prognosis It is the prediction of the course, duration and It accounts for the different degrees of damage pro-
termination of a disease and the likelihood of its response duced by equal doses of different radiations.
to treatment. Example: Radiation weighing factor (Wr): for X-rays,
gamma rays, beta particles ⫽ 1
Ptosis Drooping of the upper eyelid.
Neutrons (range) ⫽ 2–20; alpha particles ⫽ 20.
Purpura Reddish to purple flat lesions caused by blood
leaking into the subcutaneous tissue. They do not blanch Sarcoma It is a malignant tumor arising from connective
on pressure. Depending on their size they are termed as tissue.
purpura or petechiae. Larger petechiae are purpura. Septicemia Presence of bacterial toxins in blood.
Pustule It is a vesicular type of lesion containing pus, Sessile The term describes the base of the lesion which is
e.g. pyostomatitis. flat or broad.
Quadriplegic Paralyzed in all four limbs. Sievert (Sv) The special name for the SI unit of equiva-
Quality factor (QF) A linear energy transfer dependent lent dose, effective dose, and operational dose quantities.
factor by which absorbed dose is multiplied to obtain a The unit is joule per kilogram (J kg⫺1).
dose quantity, dose equivalent, to indicate the biological 1 Sv ⫽ 100 rem; 1 Sv ⫽ 1 Gy ⫻ Wr.
effectiveness of absorbed dose. Sign Any change in the body or its function, which is per-
Radiation The emission and propagation of energy ceptible to a trained observer and may indicate a disease.
through space. Sinus It is a blind tract that connects a cavity lined by
Radiation absorbed dose (rad) The energy absorbed per granulation tissue to the epithelial surface. This lining
gram of material. It represents the amount of energy that may subsequently become epithelialized.
is absorbed by the material of interest, e.g. person, organ, Stochastic effect It is the sub-lethal changes in the DNA
tissue, cells. 1 rad ⫽ 100 erg/g. of an individual cell and the heritable effects for which the
Radiation area It is an area, accessible to individuals, in probability of an effect occurs, but not its severity. It is
which radiation levels could result in an individual receiv- regarded as a function of dose without threshold.
ing a dose equivalent in excess of 0.005 rem (5 mrem) in Swelling It is a non-specific term used to describe any
1 hour at 30 cm from the radiation source or from any enlargement or protuberance in the body.
surface where the radiation penetrates.
Symptom Any change in the body or its function, which
Radiation weighting factor (WR) A dimensionless factor is perceptible to the patient and may indicate a disease.
by which the organ or tissue absorbed dose is multiplied to
Teratoma A true neoplasm made up of a number of dif-
reflect the higher biological effectiveness of high-LET
ferent types of tissues, none of which is native to the area
radiations compared with low-LET radiations. It is used to
in which it occurs.
derive the equivalent dose from the absorbed dose aver-
aged over a tissue or organ. Therapy, curative Treatment directed toward eradication
of one or more of the agencies etiologic to the patient’s
Red lesion A non-specific term used to describe any area
condition, e.g. use of antibiotics such as penicillin.
on the oral mucosa that on clinical examination appears
more red than the surrounding tissues and is usually vel- Therapy, palliative or symptomatic Treatment directed
vety or granular or smooth in texture. only toward relief of the patient’s symptoms. The natural
course of the disease is unaffected.
Restricted area An area, access to which is limited by the
licensee for the purpose of protecting individuals against Therapy, restorative Therapy directed at rapid restoration
undue risks from exposure to radiation and radioactive of health, usually regardless of the nature of the original
materials. disease; restorative therapy is most frequently given during
convalescence, e.g. vitamin supplements.
Roentgen (R) It is a unit of radiation exposure in air.
It is defined as the amount of X-ray or gamma radiation Therapy, substitutive or replacement Treatment directed
that will generate 2.58 ⫻ 10⫺4 coulombs (a measure of elec- toward supplying a material normally present in the body,
trical charge) per kilogram of air at standard temperature but absent in a specific patient because of disease, injury,
and pressure. This can also be defined as the amount of congenital deficiencies, etc., e.g. use of hormones.
energy absorbed in air. This unit is applied for only X-rays Therapy, supportive Treatment directed toward main-
and gamma rays. taining the patient’s physiological or functional integrity
Roentgen equivalent man (rem) The product of the until more definitive treatment can be carried out, or until
amount of energy absorbed (rad) times the coefficient of the patient’s recuperative powers function to obviate the
radiation in producing damage. rem ⫽ rad ⫻ (Wr) need for further treatment.
865
Tissue weighting factor (WT) The factor by which the Uncontrolled area It is an area where access is neither con-
equivalent dose in a tissue or organ T is weighted to rep- trolled nor restricted. In an uncontrolled area the shielding
resent the relative contribution of that tissue or organ to should reduce the exposure rate to ⬍ 2.6 mC/kg/week.
the total health detriment resulting from uniform irradia-
Vesicle Elevated blisters containing clear fluid and less
tion of the body. It is weighted such that:
than 1 cm in diameter, e.g. herpes simplex infection,
WT ⫽ 1. herpangina, herpes zoster.
Tumor Any enlargement, especially one due to a patho- Viremia Presence of viruses in blood.
logical overgrowth of tissue.
White lesion Non-specific term used to describe any
Ulcer Breach in the continuity of the surface epithelium area of the oral mucosa that on clinical examination
of the skin or mucous membrane to involve the underly- appears whiter than the surrounding tissues and is usually
ing connective tissue as a result of micromolecular cell raised, roughened or otherwise of a different texture than
death of the surface epithelium or its traumatic removal. the adjacent normal tissue.
866
APPENDIX
Summary of Radiographic
Pathology
Medha Babshet
2
Table 1 Radiolucent lesions affecting the jaws
Unilocular
Associated with teeth Not associated with

teeth
Pericoronal Periapical Others
• Inflammatory paradental cyst • Periapical granuloma • Traumatic bone cysts • Primordial cyst
• Dentigerous cyst • Periapical cyst • Globulomaxillary cyst • Stafne’s bone cyst
• Mural ameloblastoma • Dentoalveolar abscess • Incisive canal cyst • Neurilemmoma
• Keratocystic odontogenic tumor (OKC) • Periapical scar • Lateral periodontal cyst • Traumatic neuroma
• Adenomatoid odontogenic tumor • Periapical cemento-osseous • Gingival cyst of adult • Post-surgical maxillary
(early stage) dysplasia (lytic stage) • Cemento-ossifying fibroma (early stage) cyst
• Ameloblastic fibroma • Residual cysts
• Squamous odontogenic tumor
Multilocular
Associated with teeth Not associated with teeth

• Ameloblastoma • Central giant cell granuloma
• Keratocystic odontogenic tumor (OKC) • Brown tumor of hyperparathyroidism
• Botryoid odontogenic cyst • Cherubism
• Odontogenic myxoma • Aneurysmal bone cyst
• Glandular odontogenic cyst • Central hemangioma
• Central odontogenic fibroma • Arteriovenous malformation
• Desmoplastic fibroma
• Metastatic tumors of jaw
• Focal osteoporotic bone marrow defects
867
Table 2 Radiopaque lesions affecting the jaws
Focal Generalized

• Condensing osteitis • Tori • Florid cemento-osseous dysplasia
• Idiopathic osteosclerosis • Exostosis • Paget’s disease
• Garrey’s osteomyelitis • Osteomas • Osteopetrosis
• Hypercementosis • Foreign bodies • Albright’s syndrome
• Periapical cemento-osseous dysplasia • Mucosal cysts of maxillary sinus • Gardner’s syndrome
• Focal cemento-osseous dysplasia • Ectopic calcification • Caffe’s disease (infantile cortical hyperostosis)
• Cemento-ossifying fibroma • Sialolith • Familial gigantiform cementoma
• Cementoblastoma • Rhinolith
• Complex odontoma • Calcified lymph node
• Phlebolith
• Arterial calcification
Table 3 Mixed radiolucent-radiopaque lesions of the jaws
Periapical Pericoronal
• Rarefying and condensing osteitis • Odontoma (intermediate stage) • Chronic osteomyelitis
• Periapical cemento-osseous dysplasia (intermediate stage) • Adenomatoid odontogenic tumor • Osteoradionecrosis
• Cemento-ossifying fibroma • Calcifying epithelial odontogenic cyst • Focal cemento-osseous dysplasia
• Calcifying epithelial odontogenic tumor • Florid cemento-osseous dysplasia
• Ameloblastic fibro-odontoma • Fibrous dysplasia
• Ameloblastic odontoma • Paget’s disease (intermediate stage)
• Ameloblastic fibrodentinoma • Cemento-ossifying fibroma
• Dentinoma • Osteogenic sarcoma
• Desmoplastic ameloblastoma
• Osteoblatic metastatic carcinoma
• Chondroma
• Chondrosarcoma
868
Appendix 2 – Summary of Radiographic Pathology
Table 4 Characteristic radiographic findings of jaw pathologies
Characteristic radiographic finding Jaw lesions

Cotton wool • Paget’s disease
• Diffuse sclerosing osteomyelitis
• Florid osseous dysplasia
• Sclerotic cemental masses
Ground glass • Hyperparathyroidism
• Fibrous dysplasia
Orange peel • Fibrous dysplasia
Soap bubble appearance • Hemangioma
• Aneurysmal bone cyst
• Cherubism
• Ameloblastoma
• Giant cell lesion of hyperparathyroidism
• Central giant cell granuloma
• Odontogenic cysts like odontogenic keratocyst, follicular and residual
• Arteriovenous malformation
Honeycomb appearance • Ameloblastoma
• Central hemangioma of bone
Tennis racket appearance • Odontogenic myxoma
Moth eaten • Burkitt’s lymphoma
• Chronic suppurative osteomyelitis
• Lytic variety of osteosarcoma
• Osteoradionecrosis
• Osteosarcoma (lytic type)
Driven snow • Pindborg’s tumor (CEOT)
Codman’s triangle • Osteosarcoma
Cumulus cloud formation
Onion peel appearance • Garre’s osteomyelitis
• Calcifying subperiosteal hematoma
• Ewing’s sarcoma
• Osteogenic sarcoma
• Leukemia
• Syphilis
• Hypervitaminoses A
• Caffey’s disease
• Metastatic neuroblastoma
• Fracture callus
Sunray (sun burst) appearance • Osteosarcoma
• Ewing’s sarcoma
• Hemangioma (central)
• Chondrosarcoma
• Odontoma (rarely)
• Burkitt’s lymphoma
Hair-on-end • Sickle cell anemia
• Thalassemia
• Chronic iron deficiency anemia
Stepladder appearance • Thalassemia
• Sickle cell anemia
Chicken wire appearance of enlarged marrow spaces • Thalassemia
(Contd.)
869
Table 4 Continued
Characteristic radiographic finding Jaw lesions

Beaten silver • Craniofacial synostosis & dysostosis
• Calcifying subperiosteal hematoma
• Leukemia
• Caffey’s disease
• Metastatic neuroblastoma
• Hypervitaminoses A
• Syphilis
Punched out • Multiple myeloma
• Histiocytosis X
• Eosinophilic granuloma
Salt and pepper • Metastatic lesions
• Hyperparathyroidism
• Fibrous dysplasia
Floating teeth appearance • Histiocytosis X
• Squamous cell carcinoma of gingiva
• Papillon–Lefevre syndrome
• Advanced periodontitis
• Cherubism
Ballooning expansion • Follicular cyst
• Aneurysmal bone cyst
Candle stick appearance • Progressive systemic sclerosis
• Pyknodysostosis
Chalk like appearance • Osteopetrosis
• Hyperparathyroidism
• Pyknodysostosis
Egg shell appearance • Multilocular cyst
• Ameloblastoma
Heart-shaped radiolucency • Nasopalatine cyst
Pear-shaped radiolucency • Globulomaxillary cyst
Other characteristic radiographic findings
Bull’s eye appearance Dilaceration of roots

Moth eaten appearance of crowns of teeth Amelogenesis imperfecta especially the hypocalcified type
Shell teeth Dentinogenesis imperfecta
Thistle tube-shaped pulp chambers in anteriors Type II/coronal dentin dysplasia
Flame-shaped pulp chambers in molars
Ghost-like teeth Regional odontodysplasia
Trap door sign Blow-out fracture involving the orbit
Hanging drop/tear drop sign Herniation of orbital contents into the maxillary sinus
Joint mice Small calcifications (Loose bodies) within the temporomandibular joint space
870
APPENDIX
Characteristics of
Ideal Radiograph
3
CHARACTERISTICS OF IDEAL RADIOGRAPH a short gray scale. A low contrast radiograph displays many
shades of gray and thus exhibits a long gray scale. For all
Ideal radiograph is one with optimum density and contrast practical purposes, an ideal radiograph is a compromise
showing all the details which are well defined with mini- between high and low contrast.
mum or negligible distortion.
An ideal radiograph should have optimum visual char- Sharpness
acteristics (density and contrast), geometric characteristics
(sharpness, minimal or no magnification and distortion), Sharpness is also referred to as detail, resolution or defini-
record anatomical accuracy of radiographic images and tion. It refers to the capability of X-rays to reproduce dis-
exhibit adequate coverage of anatomic region of interest. tinct outlines of an object or to reproduce the smallest
A radiograph should record the complete area of inter- details of an object.
est. For an intraoral periapical radiograph (IOPAR) the full Intraoral periapical radiographs have better resolution
length of root and at least 2 mm of periapical bone should than panoramic radiograph and computed tomographic
be visible. scans. Resolution is described as line pairs per mm.
Density Magnification
Density is the degree of overall blackness or darkness of a Radiographic image that appears larger than the object it
dental radiograph. represents, because of the divergent path of X-rays.
The primary factors that affect density are kilovoltage
peak (kVp), milliamperage (mA), exposure time and the
Distortion
source to film distance. Some of the secondary factors the
affect density are development characteristics (dark and Distortion is the variation in the true size and shape of the
light radiographs), subject thickness, speed of film, film object being recorded. It results from unequal magnification
latitude, use of screens and grids and extent of filtration. of different parts of the object.
Contrast
Characteristic Curve
Contrast is difference in the degree of blackness (densities)
Characteristic curve is a graphic plot of relationship
between adjacent areas on a dental radiograph.
between film density and exposure. It is also called H
A high contrast radiograph is one with very dark and
and D curve after Hunter and Driffield.
very light areas. Such a radiograph is considered to exhibit
871
APPENDIX
Indications for
4 Intraoral Radiography
Indications for Intraoral Periapical Radiography Indications of Bitewing Radiography

❍ Assessment of developmental disturbances affecting ❍ Proximal regions of three maxillary and three
the root morphology for diagnosis, exodontia and mandibular teeth can be assessed in a single radio-
endodontic treatment graph
❍ Assessment of dental caries and periapical pathology ❍ Detection of incipient proximal caries (however, if the
(including status of periodontal ligament space, integ- carious lesions is deep, IOPAR should be taken to
rity of lamina dura and bone changes at the periapical assess any periapical change)
region of teeth) ❍ Detection of alveolar crestal bone loss
❍ Assessment of the periodontal ligament space and ❍ Assessment of ill-fitting/overcontoured crowns
interdental bone in periodontal diseases ❍ Assessment of overhanging restorations
❍ Detection of dento-alveolar fractures ❍ Detection of secondary caries
❍ Assessment of the quality of the bone prior to place-
ment of dental implants
❍ Detection of small cysts and tumors affecting the jaw
Indications of Occlusal Radiography
bones
❍ Detection of the presence and position of the impacted ❍ Provides a bird’s eye view of the dental arches
teeth (supernumerary, odontomes, etc.) ❍ Detection of cortical plate changes caused by bony
❍ Detection of secondary caries beneath restorations pathology (cysts, tumors) in a buccolingual direction
and crowns (such as expansion, resorption, pathological fractures,
❍ Detection of foreign material embedded within the laminar bone formation etc.)
soft tissue such as the lip, buccal mucosa ❍ Location of impacted teeth (buccal/lingual position)
❍ Detection of the status of eruption of primary/perma- ❍ Detection of sialoliths (calculi in the ducts of the sub-
nent teeth mandibular salivary gland and sublingual gland)
❍ Assessment of endodontic treatment ❍ Detection of symphyseal, parasymphyseal mandibular
❍ Assessment of alveolar bone grafts fractures
872
APPENDIX
Patient Position for
Extraoral Radiography
Suman Jai Sanghar, Jai Sanghar N
5
Figure 1 Figure 2
Posteroanterior view Posteroanterior Calwell view
OML: orbitomeatal line, CR: central ray.
873
Figure 3 Figure 5
Posteroanterior mandible 30⬚ occipitomental view
Figure 4 Figure 6
Standard occipitomental view Waters’ view
874
Appendix 5 – Patient Position for Extraoral Radiography
Figure 7
Figure 9
Open mouth Waters’ view Patient’s head position for submentovertex view
Figure 8 Figure 10
Patient’s head positioning for reverse Waters’ view Patient’s head position for lateral view projection
875
Figure 11
Figure 13
Patient’s head position for lateral oblique view of

Patient’s head position for Townes’ view the ramus
Figure 12 Figure 14
Patient’s head position for reverse Townes’ view Patient’s head position for transcranial view
876
Appendix 5 – Patient Position for Extraoral Radiography
Figure 15 Figure 16
Patient’s head position for transorbital view
Patient’s head position for transpharyngeal view
877

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884
Index
A Agnathia, 30 Anorexia, 287

ABCDE warning signs, 369 Agranulocytic angina, 491 Antemortem radiographs, 657
Abdominal ultrasonography, 397 Agranulocytosis, 455 Anterior median lingual cyst, 323
Abfraction, 458, 464 Ankyloglossia, 23 Antidiuretic hormone, 538
Abrasion, 452, 460 classification, 23 Antihypertensive therapy, 477
Acanthomatous ameloblastoma, 335 ALARA principle, 704, 728 Anti-mongoloid eyelids, 646
Acanthosis nigricans, 227 Albers–Schönberg disease, 578 Antinuclear antibody (ANA), 599
Accessory cusps, 44 Alcohol, 139, 384 Anti-Saccharomyces cerevisiae antibodies, 624
Acid-enzyme theory, 407 Alkaptonuria, 76 Antiseptic mouthrinses, 508
Acinic cell adenocarcinoma, 297 Allergic rhinitis, 506 Antistreptolysin O titers, 480
Ackerman’s tumor, 366 Allergic salute, 507 Antoni type B cells, 356
Acquired immune deficiency syndrome Allergic shiners, 507 Antral polyps, 318
(AIDS), 99 Allodynia, 111 Antrocystectomy, 330
Acquired immunity, 590 Allowable occupational dose, 704 AP axial projection, 752
Acquired melanocytic nevus, 348 Amalgam tattoo, 63 APECED, 600
Acrodermatitis enteropathica, 228 Ameloblastic Aphthous stomatitis, 211, 591
Acrodynia, 63 carcinoma, 363 Aphthous ulcers, 104, 210, 211, 520, 591
Acromegaly, 536 fibrodentinoma, 342 Apical periodontal cyst, see Periapical cyst
Acrosclerosis, 601 fibroma, 342 Aplastic anemia, 448
Actinomycosis, 86 fibro-odontoma, 342 Apnea, 512
Activated partial thromboplastin time, 498 fibrosarcoma, 366 Apple-green birefringence, 642
Acute adrenal insufficiency, 547 sarcoma, 366 Areca nut, 383, 463
Acute dental caries, 413 Ameloblastoma, 33 Argyll Robertson pupil, 627
Acute intermittent porphyria, 642, 643 Amelogenesis imperfecta, 53, 54, 77 Argyria, 64
Acute leukemia, 492 Amine odor test, 632 Aromatic hydrocarbons, 383
Acute lymphocytic leukemia, 386, 492 Amino acids, 635 Arrested caries, 412
Acute lymphonodular pharyngitis, 90 Amlodipine, 446 Arsenic, 63
Acute monocytic leukemia, 492 Amorphous calcium phosphate, 464 Arteriovenous malformations, 359
Acute necrotizing ulcerative gingivitis Amputation caries, 401 Arthrography, 795–796
(ANUG), 204, 452 Amyloid, 642 Articular capsule, 239
Acute pain, 115 Amyloidosis, 642 Articular disk, 239, 240, 246
Acute pseudomembranous candidiasis, Anaphylaxis, 481 Articular eminence, 239
135, 154 Anatomical variations, 842 Artifacts, 820
Acute radiation syndrome, 699 Androgens, 445 Aryl hydrocarbon hydroxylase, 383
Acute sinusitis, 105 Andy Gump facies, 255 Ascher’s syndrome, 27
Acute suppurative osteomyelitis, 433 Anemia, 456, 485 Aschoffs nodules, 480
Added filtration, 694 Aneurysmal bone cyst, 322 Ascorbate, 639
Addison’s disease, 66, 546 Angina pectoris, 478 Ascorbic acid, 499, 639
Adenocarcinoma, 295, 297 Angiogenesis, 385, 401 Ash leaf macules, 233
Adenoid cystic carcinoma, 298 Angiolipoma, 355 Ashboe–Hansen sign, 212
Adenomatoid odontogenic tumor, 338 Angiosarcoma, 377 Aspergillosis, 109, 207, 615
Adherence and adhesions, 247 Angle’s classification of malocclusion, 255 Aspiration biopsy, 393
Adult periodontitis, 442 Angular cheilitis (perleche), 155, 156, 487 Aspirin, 483, 500
Aerodontalgia, 118 Anhidrotic ectodermal dysplasia, 222 Asteroid bodies, 290, 619
African Kaposi’s sarcoma, 377 Ankyloglossia, 23 Asthma, 508, 510
Agammaglobulinemia, 456 Ankylosis, 253 Atherosclerosis, 478
Age estimation methods, 669 Ann Arbor classification, 495 Atrophic glossitis, 487
Agenesis, 266 Annual dose limits, 710 Atrophic lichen planus, 148, 150
Aggressive periodontitis, 442 Anodontia, 45 Attached gingiva, 210, 312, 346
Aglossia, 22 Anorexia nervosa, 524 Attrition, 459, 670
885
Index
Atypical facial pain, 126 Bisphosphonates, 588 C

Atypical odontalgia, 125 Bite mark procedures, 672 Café-au-lait macules, 536
AUPD cells, 552 Black beard sign, 588 pigmentations, 66
Aura, 530 Black box, 522 spots, 234, 576
Auriculocondylar syndrome, 33 Black hairy tongue, 18 Caffey’s disease, 583
Auspitz’s sign, 221, 234 Black tarry stools, 523 Caffey–Silverman syndrome, 583
Autoimmune hepatitis, 527 Blade of grass lesion, 588 Calcifying epithelial odontogenic tumor, 337
Autoimmune hypothyroidism atrophic Blank radiograph, 820 Calcifying odontogenic cyst, 312, 339
thyroiditis, 539 Blastomycosis, 107, 613 Calcium, 477
Hashimoto’s thyroiditis, 539 Bleeding time, 498 channel blockers, 446
Autoimmune polyendocrinopathy-candidiasis- Bleomycin, 402 hydroxide, 463
ectodermal dystrophy, 551, 600 Blepharosis moniliformis, 646 oxide, 463
Autoimmune theory, 407 Bloch–Sulzberger syndrome, 231 Calculus, 451
Autoimmunity, 590 Blood dyscrasias, 440 Cameron ulcers, 523
Avulsion, 673 Blood pressure, 475 Canalicular adenoma, 293
Azotemia, 514 Blood studies, 397 Cancrum oris, see Noma
Aδ fibers, 112 Bloody crusted appearance, 191 Candida leukoplakia, see Chronic hyperplastic
Blue bloaters, 510 candidiasis
B Blue colored sclera, 577 Candidiasis, 101, 140, 153
Bacille Calmette–Guérin (BCG) vaccination, Blue dye, 394 classification, 154
610 Blue, purple or gray tinted sclera, Canine space, 429
Baclofen, 122 577 Canker sores, 591
Bacterial pharyngitis, 508 Blurred image, 817 Capillary fragility test, 498
Bacterial sialadenitis, 282 Bone scanning, 788 Capillary malformations, 359
Baelz’s disease, 283 Bohn’s nodules, 316 Caplan’s syndrome, 594
Barodontalgia, 118 Bollinger’s granules, 88 Capsulitis, 249
Barosinusitis, 119 Botryoid odontogenic cyst, 311 Carbamazepine, 122
Barrel-shaped rib cage, 577 Botryomycosis, 88 Carbohydrates, 634
Bartholin’s duct, 267 Bouin’s solution, 219 Carbon monoxide, 383, 531
Basal cell adenoma, 293 Boundary lubrication, 247 Carbonic acid, 463
Basal cell carcinoma, 367 Brachytherapy, 400 Carboxymethylcellulose, 401
Basal cell nevus syndrome, 342 Bradykinesia, 531 Carcinogens, 384
Battle’s sign, 263 Brazilian pemphigus, 596 Carcinoma ex ameloblastoma, 364
Bay cyst, 320 Breathlessness, 475, 505 Carious lesions, 410, 425
B-cell immunity, 590 Bremsstrahlung radiation, 688, 689 Carotenoids, 637
Beam limiting devices, 706 Breschet–Gorham syndrome, 585 Carotid sheaths, 393
Bean-shaped cyst, 347 Brim sign, 588 Carpal tunnel syndrome, 537, 540
Bean-shaped radiopacities, 646 Brittle bone disease, 576 Cascading waterfall appearance, 58
Bearded infant appearance, 584 Broad saddle nose, 644 Casein phosphopeptide, 464
Beckwith–Wiedemann syndrome, 27 Bronchodilators, 510 Caseous necrosis, 608, 609
Beedis, 381, 382, 383 Bronchogenic carcinomas, 511 Cat-scratch disease, 89
Behçet’s disease or syndrome, 31, 216 Bronze diabetes, 67 Causalgia, 311
Bejel, 626 Brown tumors, 516, 543 Caviar lesions, 16
Bell’s palsy, 529 Bruck syndrome, 578 Celiac disease, 53, 214
Bence Jones proteins, 497 Brueghel’s syndrome, 532 Cell mediated immunity, 157, 590
Benign chondroblastoma, 351 Brush biopsy, 395, 398 Cell rests of Serres, 305
Benign lymphoepithelial lesion, 277, 591 Bruxism, 459 Cemental tears, 452
Benign migratory glossitis, 24 Buccal space, 427, 429 Cementicles, 470
Benign mucosal cysts, 317 Bulimia, 287 Cementifying fibroma, 571
Benign mucous membrane pemphigoid, 188, Bulimia-related sialadenosis, see Anorexia Cementoblastoma, 343
212 Bulimia nervosa, 524 Cemento-ossifying fibroma, 571
Benign osteoblastoma, 351 Bull neck appearance, 431 Cephalometric radiography, 725
Benzopyrene, 383 Bulla spreading sign, see Ashboe–Hansen Cervical burn out, 461
Benzydamine hydrochloride, 25, 401 sign Cervical enamel extensions, 43
Beriberi, 640 Bulldog jaw, 628 Cervical esophagus, 393
Bernard–Soulier syndrome, 500 Bull’s eye lesion, 190 Cervical lymphadenopathy, 508
Betel quid chewers, 463 Bullous impetigo, 84, 193 Cetuximab, 402
Betel quid lichenoid lesion, 153 Bullous pemphigoid, 185, 212, 596 Chalky, ground glass, granular and salt and
Bifid condyles, 33 Burkitt’s lymphoma, 496 pepper appearance of bone, 516
Bifid tongue, 24 Burning feet, 514 Chancre, 206, 612, 626
Bilaminar zone, 240 Burning mouth sensation, 487 Characteristic radiation, 688, 689
Bilirubin, 61 Burning mouth syndrome, 127 Charcot’s joints, 627
Biliverdin, 62 Burning tongue, 272 Chediak–Higashi syndrome, 453, 491
Biological indicators, 671 Burtonian line, 64 Cheilitis glandularis, 283
Bird face deformity, 255 Busse–Buschke disease, 109 Cheilitis glandularis apostematosa, 283
Bismuth grip, 63 Butterfly rash, 84, 598 Cheilitis granulomatosa, 289, 620
886
Index
Chelation, 406 Clay modeling, 667 Coxa vara, 577

Chemical burn, 135 Clear or gargoyle cells, 644 Cowdry type A, 240
Chemical indicator, 706 Cleft lip, 27 CPP-ACP, 464
Chemo-osteonecrosis, 588 Cleft palate, 27 Cracked tooth syndrome, 466
Chemotherapeutic agents, 401 Cleft tongue, 24 Cracker sign, 268
Chemotherapy, 104, 401, 845 Cleidocranial dysplasia and Maroteaux-Lamy Cranial arteritis, 591
Cherubism, 580 syndrome, 307 Cranial suture pattern, 664
Chest pain, 475, 505 Clinical forms of leukoplakia, 140 Craniotabes, 638
Chicken pox, 178, 201 Closed lock, 248 Crazing, 660
Chicken-wire appearance, 490 Clotting mechanism, 497 C-reactive protein, 253, 624
Chicken-wire pattern, 351 Clutton’s joints, 628 Crescent-shaped lesions, 464
Chinese script writing, 575 Cobblestone appearance, 622 CREST syndrome, 603
Chlamydial infections, 631 Cod fish vertebrae, 578 Cretinism, 539
Chloasma, 64 Codman’s triangle appearance, 371 Cricoid cartilage, 393
Chloasma gravidarum, 551 Codman’s tumor, see Benign chondroblastoma Crohn’s disease, 520, 521, 622
Chloromas, 493 Coffee ground vomitus, 523 Croup, 505
Chloroquine, 64, 65 Cogan’s lid twitch, 604 Cryptococcosis, 109, 207
Cholesterol clefts, 320 Coherent scatter, 695 Cullen’s sign, 90
Cholesterol crystals, 318, 326 Cold test, 422 Cushing’s syndrome, 545
Chondroma, 350 Collagenases, 385 Cusp of Carabelli, 44
Chondrosarcomas, 261, 372 Collarette, 193 Cyanosis, 68, 475
Christmas disease, 503 Collimators, 694 Cyclic neutropenia, 455, 492
Christmas tree pattern, 226 functions, 694 Cyclosporine, 446
Christ–Siemens–Touraine syndrome, see types, 694 Cylinder–type implants, 851
Hypohidrolic ectodermal dysplasia Colloid bodies, 694 radiographic appearance, 851
Chromogenic bacteria, 73 clvatte, 694 Cystic fibrosis, 511
Chronic atrophic candidiasis, 155 hyaline, 694 Cystic hygroma, 326, 792
Chronic bronchitis, 505, 509 cytoid, 694 Cystic PIOC, 364
Chronic dental caries, 413 Color changes in burned teeth, 661 Cysticercosis, 327
Chronic diffuse sclerosing osteomyelitis, 437 Colpitis macularis, 631
Chronic fatigue syndrome, 90 Comb sign, 522 D
Chronic focal sclerosing osteomyelitis, 436 Commissural lip pits, 26 Dane particle, 526
Chronic myelocytic leukemia, 493 Complex odontome, 341 Dapsone, 611
Chronic hyperplastic candidiasis, 154 Composite odontome, 341 Dark-field microscopic examination, 613
Chronic irreversible pulpitis, 414, 418 Compound hemangiomas, 358 Darkroom infrastructure, 804
Chronic leukemia, 493 Compound nevus, 348 Dark spots, 819
Chronic lymphocytic leukemia, 493 Compton effect, 696 Daylight processor, 808
Chronic lymphoid leukemia, 493 Compton scatter, 695 De Musset’s sign, 627
Chronic mucocutaneous candidiasis Computed tomography (CT), 760–762 De novo ameloblastic carcinoma, 364
(CMC), 156 Concept of referred pain, 114 Dead tracts, 468
Chronic obstructive airway disease, 509 Concrescence, 36 Debulking, 399
Chronic obstructive pulmonary disease, 509 Condensing osteitis, 414 Decortication, 436
Chronic osteomyelitis with proliferative Condylar aplasia, 33 Deep cervical nodes, 12
periostitis, see Periostitis ossificans Condylar hyperplasia, 33 Deep fascia, 426
Chronic pain, 115 Condylar neck fractures, 262 Deep fungal infections, 613
Chronic periapical abscess, 415 Condyloma acuminatum, 347, 630 Deep hemangiomas, 207
Chronic renal failure, 513 Cone-beam computed tomography (CBCT), 762 Degenerative joint diseases 358
Chronic sclerosing sialadenitis, 285 Cone cuts, 741 Demirjian’s method, 250
Chronic sinusitis, 105 Congenital heart disease, 479 Dennie–Morgan lines, 670
Chronic suppurative osteomyelitis, 434 hemoglobinopathies, 490 Dens evaginatus, 41
Chronological hypoplasias, 52 lip pits, 26 Dens in dente, 40
Chvostek sign, 544 rubella syndrome, 91 Dens invaginatus, 40, 41
Chylothorax, 586 syphilis, 628 Dental attrition, 670
Cicatricial pemphigoid, 186, 213, 596 teeth, 46 Dental caries, 405
Cigarettes, 139, 381, 383 Congenital aplasia, see Agenesis classification, 410
Cigars, 381 Congenital epulis of newborn, 355 Dental computed tomography, 849
Ciprofloxacin, 79 Congo red staining, 642 Dental floss, 417, 460
Circumferential caries, 401 Conn’s syndrome, 549 Dental fluorosis, 55, 79
Circumscribed morphea, 601 Contact stomatitis, 209 Dental identification, 654
Circumvallate papillae, 16 Cooper type scissors, 659 procedure, 655
Cisplatin, 402 Coronary artery disease, 847 Dental implants, 842–845
Civatte, hyaline, cytoid bodies, 150 Coronary heart disease, 478 forms, 842
Classification and staging system for oral Coronoid hyperplasia, 34 historical perspectives, 842
leukoplakia, 138 Corrosion, see Erosion indications, 844
Classification of ankyloglossia, 23 Cotton-wool appearance, 438, 588 patient management, 845
Classification system for Kaposi’s sarcoma, 377 Cough, 504 post-operative evaluation, 857
887
Index
Dental implants (Contd.) Dyskeratosis congenita, 166, 225 Erythema nodosum, 619
treatment options, 842 Dysostosis multiplex, 645 Erythematous candidiasis, see Chronic atropic
types, 843 Dyspnea, see Breathlessness candidiasis
Dental plaque, 406, 440, 442 Dystonia, 532 Erythrodontia, 76, 643
Dental records, 657 Dystrophic calcification, 16 Erythroleukoplakia, 141, 142
Dental transposition, 47 Dystrophic epidermolysis bullosa, 194 Erythroplakia, 168
Dentifrices, 460 Esophageal reflux disease, 213, 519
Dentigerous cyst, 306 E Esophageal webs, 487
Dentin dysplasia, 58, 78 Eagle’s syndrome, 122 Estrogens, 445
Dentinal caries, 414 Eating disorders, 524 Ethyl alcohol, 384
Dentinal hypersensitivity, 464 Echelon lymph nodes, 393 Eumelanin, 18
Dentinogenesis imperfecta, 55, 56, 77 Ectodermal dysplasia, 222 Eustachian tube, 390
Denture marking systems, 663 Edrophonium test, 604 Evaginatus odontomas, 41
Dermatitis herpetiformis, 180 EDTA, 463 Ewing’s sarcoma, 376
Dermoid cysts, 327 Ehlers–Danlos syndrome, 223, 453, 499 Examination of temporomandibular joint, 243
Desmoglein-I, 181 Ekman–Lobstein syndrome, 576 Exanthematous diseases/infections, 52
Desmoplastic fibroma, 344 Electrical insulating oil, 693 Exertional dyspnea, 475
Developmental cysts, 304 Electric pulp tester, 423 Exfoliative cytology, 397, 395
Diabetes, 384, 454, 846 Electromagnetic spectrum, 687, 688 Exocrine glands, 532
insipidus, 538 ELISA test, 102 Exophytic growth, 390
mellitus, 288 Elongated image, 816 Exostoses, 19, 35
mellitus-associated gingivitis, 440 Ely cyst, 251 Exposure parameters and processing
mellitus type I insulin dependent Emphysema, 509 technique, 818
(IDDM), 600 Enamel caries, 414 errors, 818
Diabetic ketoacidoses, 108 Enamel hypoplasia, 511, 516 blank radiograph, 818
Diascopy test, 68 Enamel pearls, 42 dark radiograph, 818
Digastric muscle, 244 Enameloma, 42 film fog, 818
Digital radiography, 707 Enchondromas, 350 light radiographs, 818
Dilaceration, 38 Endarteritis obliterans, 612 External resorption, 466
Dimple wart, see Molluscum contagiosum Endemic Kaposi’s sarcoma, see African Extraoral films, 721
Diphtheria, 83 Kaposi’s sarcoma Extraoral radiographic techniques, 752
Diphtheritic membrane, 83 Endobone, 579 axiolateral oblique projection, 753
Diplopia, 390 Endocrine glands, 532 lesser known/forgotten, 752
Direct digital receptors, 732 Endocrinopathic pigmentation, 546 acanthioparietal projection reverse
Direct target theory, 399 Energy requirement, 633 Waters’ method, 754
Discal ligaments, 241 Enostosis, 584 May method, 752
Discoid lupus erythematosus, 170 Entropion, 195 modified parietoacanthial
Disinfection, 104 Enucleation, 330 projection, 754
Disk displacement, 248 Environmental enamel hypoplasia, 51 Townes’ method, 752
with reduction, 248 Enzymatic theory, 407 Extraoral radiography, 743
without reduction, 248 Eosinophilic granuloma, 647 grids, 743
Disk thinning and prerforation, 246 Epidermal growth factor receptor, 402 image receptors, 743
Dislocation, 248 Epidermoid cysts, 327 intensifying screens, 743
types, 249 Epidermolysis bullosa (EB), 193 Extraskeletal chondroma, 350
Displacement of disk–condyle complex, 248 types, 193 Extrinsic asthma, 508
hypermobility, 248 Epidermolysis bullosa acquisita, 596 Extrinsic cysts, 316, 318
Disseminated form of histoplasmosis, 107 Epilepsy, 530 Extrinsic discoloration, 71
Disseminated intravascular coagulation Epiloia, see Tuberous sclerosis complex Extrinsic stains, 72
(DIC), 498 Epiphora, 390 Eye to heaven appearance, 581
Distodens, 46 Epistaxis, 499 Eyelid beading, 646
Distomolar, 46 Epithelial dysplasias, 143 Eyelid twitch response, 604
Doppler ultrasound, 791 Epithelial rests of Malassez, 318
Dose limits, 704 Epithelioid macrophages, 605 F
Double lip, 26 Epithelioma contagiosum, see Faces pain scale, 116, 117
Down syndrome, 22, 35, 43, 326 Molluscum contagiosum Facet, 459
Driven snow patterns, 337 Epithelioma cuniculatum, 367 Facial approximation, 667
Drug influenced enlargements, 440 Epsilon-aminocaproic acid (EACA), 502 Facial hirsutism, 643
Drug influenced gingival diseases, 440 Epstein pearls, 316 Facial neuropathies, 628
Drug-induced pigmentation, 64 Epstein–Barr virus (EBV), 385, 450 Facies leprosa, 611
Dry beriberi, 640 Epulis fissurata, 354 Factitial injury, 452
Dry mouth, 223, 269, 272 Erb’s palsy, 584 Falling snowflakes, 328
Duchenne muscular dystrophy, 532 Erosion, 461 Familial fibrous dysplasia, see Cherubism
Dwarfism, 535 Erosive lichen planus, 147 Fanconi’s anemia, 488
Dyschroic fog, 820 Eruption sequestrum, 47 Fascia, 428, 429
Dysesthesia, 387 Erysipelas, 84 Fascial spaces, 426
Dysgeusia, 518 Erythema multiforme, 177, 189 Fat soluble vitamin, 636
888
Index
Fatty acids, 635 Fogo selvagem, see Brazilian pemphigus Glands of Blandin and Nuhn, 267
Fatty degeneration, 401 Folate, 385 Glands of von Ebner, 267
Fellatio syndrome, 625 Foliate papillitis, 168 Glanzmann’s thrombasthenia, 500
Fergusson, 659 Folic acid deficiency, 487 Glenoid fossa, 239
Fever, 598 Follicular cyst, see Dentigerous cyst Glomeruloid appearance, 365
Fibroma, 345 Forchheimer spots, 91 Glomerulonephritis, 617
Fibromatoses, 361 Fordyce granules, 11, 29 Glossitis, 628, 487
Fibromatosis gingivae, 29 Foreign body granulomas, 616 Glossocele, 323
Fibromyalgia, 259 Forensic dentistry, 654–678 Glossopharyngeal neuralgia, 122
Fibro-osseous lesions, 568 Fore-shortened image, 816 Glucocerebroside, 648
Fibro-osseous metaplasia, 675 Fothergill’s disease, 120 Glucose-6-phosphate dehydrogenase
Fibrosarcoma, 370 Fournier’s molars, 52 deficiency, 488
Fibrous dysplasia, 573 Fractionated irradiation, 400 Goldenhaar’s syndrome, 33
Fibrous hyperplasia, 354 Framed vertebrae, 588 Golgi tendon organs, 241
Filiform papillae, 19, 24 Free radicals, 384 Goltz–Gorlin syndrome, 228
Film badge monitoring, 713 Free-floating desmosomes, 646 Gomori methenamine silver stain, 108
Film badges, 713 Frictional traumatic keratosis, 135 Gonococcal arthritis of TMJ, 631
Film placement and projection technique, 814 Frontal bossae of parrot, 628 Gonorrhea, 630
errors, 814 Frontal sinus configuration, 664 Gorham’s disease, 585
Film processing, 803 Full axial projection, 748 Gorham’s osteolysis, 585
techniques, 803 Fulminant hepatitis, 528 Gorham–Stout syndrome, 585
automatic, 809 Functional capacity, 475 Gorlin’s sign, 224
manual, 803 Fusion, 28, 36 Gout, 253
steps in processing, 807 Graft versus host disease, 234
visual method, 807 G Graham-Little syndrome, 149
Film receptors, 706 GABHS, 507, 508 Grand mal seizures, 530
Film storage, 707 Gag reflex, 844 Granulocytes, 490
Film storage and handling, 812 Galacturonic acid, 463 Granuloma, 504, 605
errors, 812 Galvanizing, 464 Granuloma pyogenicum, 353
dark spots or lines, 812 Gamma rays, 687 Granulomatous diseases, 511, 605
emulsion peel, 812 Gangrenous, see Noma Graves’ disease, 541
film fog, 812 GAPO, 48 Grids, 708, 722
nail marks or kink marks, 814 Gardner’s syndrome, 341, 349 composition, 722
scratched film, 812 Gargoylism, 644 functions, 723
static electricity artifact, 814 Garrè’s osteomyelitis, 437 types, 723
Films and processing of films, 687 Gastroesophageal reflux disease (GERD), 519 Grinspan syndrome, 149
Filters, 694 Gastrointestinal syndrome, 700 Grocott methenamine silver stain, 621
Finger clubbing, 475, 519 Gate control theory, 114 Grocott-Gomori methenamine silver, 208
Fish scale appearance, 577 Gaucher cells, 648 Ground glass appearance, 517, 574
Fishy scale odor, 515 Gaucher disease, 648 Growth hormone, 535
Fissured tongue, 13, 24 Gemination, 36 in children, 535
Fite method, 611 Generalized seizures, 530 Guillain–Barre syndrome, 599
Fitz–Hugh–Curtis syndrome, 630 Geniculate neuralgia, 124 Gumma, 207, 613
Flapping tremors, 525 Genital wart, see Condyloma acuminatum Gunther’s disease, 643
Floating in air apperance teeth, 647 Geographic tongue, 24 Gutkha, 381
Floating in space, 395 German measles, 91
Floating thermometer, 805 Gestant odontome, 40 H
Floating tooth syndrome, 582 Ghost cells, 365 Hailey–Hailey disease, 229
Florid cemento-osseous dysplasia, 569 Ghost images, 759 Hair-on-end radiographic appearance,
Florid osseous dysplasia, 570 Ghost-like appearance, 60 489, 490
Fluid deprivation test, 538 Giant cell arteritis, 591 Hairy leukoplakia, 102, 516
Fluid level appearance, 105, 106 Giant cell fibroma, 360 Hairy tongue, 18
Fluid-fluid levels, 323 Giant cell granuloma, 352 Half value layer, 694
Fluorescent treponemal antibody-absorbed Giant cell reparative granuloma, 352 Halitosis, 561
(FTA-ABS) test, 613 Giant cell tumors, 542 Hallopeau type pemphigus vegetans, 596
Fluorosis, 52 Giant osteoid osteoma, 352 Halo-shadow, 438
FNAC, 396 Gigantiform cementoma, 570 Hand, foot and mouth (HFM) disease,
Foamy macrophages, 648 Gigantism, 656 179, 204
Focal cemento-osseous dysplasia, 611 Gilchrist disease, 107, 207 Hand–Schuller–Christian disease, 647
Focal epithelial hyperplasia, 165, 347 Gingival cyst of infants, 305 Hangar-Rose skin test, 89
Focal reversible pulpitis, 439 Gingival diseases modified by Hanging teeth, 390
Focal sclerosing osteomyelitis, 414 medications, 440 Hansen’s disease, 610
Focal sialadenitis, 271 Gingival hyperplasia, 483, 644 Heart failure, 482
Focal trough, 759 Gingival recession, 425 Heartburn, 519
concept, 759 Ginglymodiarthroidal joints, 239 Heat test, 423
effect of patient positioning, 759 Glanders like disease, 85 Hebra nose, 88
889
Index
Heck’s disease, see Focal epithelial Host modulation, 444 Immune granulomas, 605
hyperplasia Hot potato voice, 508 Immune-mediated granulomatous
Heerfordt syndrome, 619 Howship’s lacunae, 579 inflammation, 605
Heinz bodies, 488 Hoyeraal–Hreidarsson syndrome, 225 Immunity, 157
Heister mouth gags, 659 HPA axis, 535 Immunomodulators, 184
Helicobacter pylori, 519 Human herpes virus-8, 175, 376 Impacted teeth, 49
Hemangiomas, 68, 357 Human immunodeficiency virus (HIV), Impetigo, 84
Hemarthrosis, 501 92, 285, 385, Implant site identification, 845
Hematological diseases, 489 oral manifestations, 101 absolute contraindications, 845
Hematomas, 69 structure of HIV-1 virion, 92 conventional tomography, 848
Hematopoietic syndrome, 699 transmission, 97 imaging, 848
Hemifacial atrophy, 32 Human papilloma viruses, 385 panoramic radiography, 848
Hemifacial hyperplasia, 31 Humoral immunity, 590 relative contraindications, 846
Hemifacial hypertrophy, 31 Hunter syndrome, 644 selection of the implant site, 847
Hemihyperplasia, 35 Hurler syndrome, 644 selection of the patient, 845
Hemochromatosis, 67 Hutchinson teeth, 613 standard periapical radiographs, 848
Hemodialysis, 481, 517 Hutchinson’s incisor, 52 Incinerated teeth, 660
Hemoglobinopathies, 489, 490 Hutchinson’s triad, 628 Incineration, 659
Hemophilia A, B and C, 502, 503 Hyalinosis cutis et mucosae, 646 Incipient caries, 411
Hemoptysis, 505 Hydatid disease, 328 Incontinentia pigmenti, 231
Hemorrhage, 662 Hydrostatic enlargement of cysts, 305 Indirect target theory, 399
Hemosiderin, 62 Hydroxyl radicals, 384 Indwelling catheters and stents, 481
Heparin therapy, 484 Hyperacusis, 529 Infantile cortical hyperostosis, 583
Hepatitis, 526 Hyperalgesia, 111 Infantile osteomyelitis, 436
A, 526 Hyperbaric oxygen (HBO), 436 Infectious arthritis, 252
B, 526 Hypercalcemia, 497, 542 Infectious mononucleosis, 90
B surface antigen, 528 Hypercarotenemia, 637 Inflammatory bowel disease, 519
C, 527 Hypercementosis, 470 Inflammatory cysts, 304
D, 527 Hyperdontia, 45 Infrabony defects, 456
G virus, 527 Hyperglycemia, 600 Infrared telethermography (IRT), 796
Hereditary benign intraepithelial Hypermobility, 224, 248 Infratemporal fossa space, 429
dyskeratosis, 165 Hyperostosis, 584 Inherent filtration, 694
Hereditary corpoporphyria, 643 Hyperparathyroidism, 542 Inherited coagulation disorders, 502
Hereditary gingival fibromatosis, 449 Hyperpigmentation, 400 Intensifying screens, 707, 722
Hereditary fibrous dysplasia, Hyperplasia of minor salivary glands, 266 Intensity modulated radiation therapy
see Cherubism Hypersensitivity, 459 (IMRT), 400
Hereditary opalescent dentin, 57 Hypertension, 476, 549 Internal replacement, 467
Hermann’s syndrome, 341 Hyperthyroidism, 540 Internal resorption, 466, 467
Herpangina, 180, 203 Hyperuricemia, 253 International normalized ratio (INR), 484
Herpes labialis, 201 Hyperventilation, 544 Interstitial glossitis, 613
Herpes simplex viruses (HSV), 175, 385 Hypodontia, 45 Intradermal injections, 611
type 1, 175 Hypoesthesia, 111 Intramuscular injections, 502
type 2, 175 Hypoglycemia, 540 Intraoral films, 719, 720
Herpes viruses, 175 Hypohidrotic ectodermal dysplasia, see composition, 720
Herpes zoster, 178, 203 Anhidrotic ectodermal dysplasia dimensions, 721
Herpetic gingivostomatitis, 449 Hypomineralized enamel, 50 uses, 719
Herpetiform ulcers, 211 Hypoparathyroidism, 544 Intraoral herpes, 201
Herring bone pattern, 814 Hypopharynx, 392 Intraoral radiography, 725
Hiatal hernia, 522 Hypophosphatasia, 453 common technical errors, 740
High performance laser spectroscopy-laser Hypoplastic enamel, 50 preliminary procedures, 729
induced fluorescence (HPLC-LIF), 395 Hypoplastic teeth, 640 procedures, 733
High voltage transformer, 693 Hypopnea, 512 bitewing radiography, 735
Highly differentiated squamous Hyposalivation, 267, 268 occlusal radiography, 736
cellcarcinoma, 398 Hypovolemia, 523 periapical radiography, 733
Higoumenakis sign, 235 selection criteria guidelines, 729
Hilar lymphadenopathy, 607 I techniques, 730
Histiocytosis X, 647 Ice pack test, 604 bisecting angle, 730
Histoplasmosis, 106, 207, 614 Icterus, see Jaundice common technique errors, 739
Hodgkin’s disease, 495 Idiopathic hypertension, see Hypertension paralleling, 730
Homogeneous leukoplakia, 141 Idiopathic osteolysis, 586 Intraoral X-ray machine, 691, 693, 726
Honeycomb like appearance, 243, 374 Idiopathic osteosclerosis, 584 components, 691
Honeycomb pattern, 351 Idiopathic thrombocytopenic dissipation of heat from, 693
Honiton lace, 219 purpura, 500 working mechanism of, 693
Horizontal overlap, 741 IgA, 195, 409 Intraoral X-ray units, 690
Hormone resistance, 535 IgG, 409 mobile intraoral radiographic
Hormones, 533 Image guided radiotherapy (IGRT), 400 units, 690
890
Index
Intraosseous mucoepidermoid carcinoma, Lagophthalmos, 610 Lipoma, 355

296, 365 Lamina dura, 825 Liposarcoma, 373
Intravenous bisphosphonate treatment, 846 Langhans type giant cells, 605 Lipschütz bodies, 240
Intrinsic cysts, 316 Latent bone cyst, 323 Lipstick sign, 268
Intrinsic discoloration, 81 Latent image formation, 802 Liquid crystal thermography (LCT), 796
Inverted pear-shaped radiolucency, 315 steps, 802 Liver disease, 68, 503, 525
Inverted Y line of Innes, 830 Latent syphilis, 627 functions, 525
Ionization chambers, 712 Lateral oblique view, 750 Liver metastasis, 397
Ionizing radiation, 384 Lateral periodontal cyst, 311 Localized microdontia, 35
Iris lesion, 190 Lateral pharyngeal space, 430 Lockjaw, 85
Iron deficiency anemia, 210, 486 Lateral pterygoid muscle, 242 Löfgren syndrome, 619
Iron, 210 Lateral radicular cyst, 319 Log wheel rigidity, 531
Ischemic heart disease, 478 Lazy leukocyte syndrome, 453, 456 Loose bodies, see Joint mice
Isotope tracer probe, 394 Lead aprons, 709, 711, 715 Loss of taste, 401
Ivory-white papules, 645 Left shift, 492 Lowenstein tumor, 367
Ivy bleeding time, 498 Legionnaire’s disease (legionellosis), 504 Lower respiratory tract infections, 504, 508
Leiomyoma, 357 LT ratio, 396
J Leiomyosarcoma, 377 Ludwig’s angina, 428
Jadassohn–Lewandowsky syndrome, 225 Lemierre’s syndrome, 90 Lues, 626
Jaffe–Lichtenstein syndrome, 574 Leong’s premolar, see Dens evaginatus Lues maligna, 207, 627
Jaundice, 68, 525 Leontiasis ossea, 31 Luminescence, 713
Jaw resection, 659 Lepra cells, 611 Lumpy jaw, 86
Jewels sign, 195 Lepromatous leprosy, 610, 611 Lupus erythematosus (LE), 170, 600
Jigsaw puzzle pattern, 588 Lepromin test, 611 Luxation, 779
Jod–Basedow phenomenon, 540 Leprosy, 610 Lyell’s syndrome, 189
Joint mice, 251 Letterer–Siwe disease, 648 Lymph nodes, 495
Jugulo-digastric lymph nodes, 393 Leucovorin, 402 Lymphatic malformations, 359
Junctional epidermolysis bullosa, 194 Leukemia, 447, 455 Lymphatics, 392
Junctional nevus, 348 Leukemia associated gingivitis, 440, 447 Lymphoid nodules, 595
Juvenile idiopathic arthritis, 251 Leukemoid reaction, 492 Lymphomas, 495
Juvenile ossifying fibroma, 348, 572 Leukocyte disorders, 455 Lymphoproliferative syndrome, 491
Juxta articular chondromas, 350 Leukocytosis, 492 Lyonization effect/Lyon hypothesis, 54
Leukoedema, 13 Lysosomal storage diseases, 641
K Leukopenia, 491 Lysosomes, 641
Kaposi’s sarcoma, 69, 376 Leukoplakia, 137, 225
classification system, 377 classification and staging system, 138 M
Kassowitz’s law, 628 clinical features, 140 Macqueen-dell technique, 751
Kawasaki disease, 232 medical management, 144 Macrodontia, 35
Keratin pearls, 399 Leutic glossitis, 628 Macroglossia, 22
Keratoacanthoma, 347 Levine’s ionic see-saw theory, 407 Macrognathia, 30
Keratoconjunctivitis sicca, 270 Levine’s sign, 478 Macrophages, 605
Keratocystic odontogenic tumor, 304, Levy–Hollister syndrome, 266 Maculopapular lesions, 627
307, 339 Lichen planopilaris, 147 Maculopapular rash, 612
Khaini, 381 Lichen planus, 172, 189, 218 MAGIC syndrome, 215
Kidney shaped cyst, 316 Lichenoid drug reaction, 152 Magnetic resonance imaging (MRI), 781
Kilovoltage, 709 Lichenoid mucosal reactions, 521 advantages, 787
Kissing disease, see Infectious mononucleosis Lichenoid stomatitis, 483 common artifacts, 787
Klestadt’s cyst, 315 Light spots, 819 due to motion and use of
Klinefelter syndrome, 326 Lincoln’s highway, 430 denture, 787
Klippel–Trenaunay syndrome, 358 Lincoln’s sign, 588 flow effect and movement
Knurled effect, 814 Linea alba buccalis, 134 artifacts, 787
Koebner’s phenomenon, 147 Linear ‘en coup de sabre’, 601 contrast agents, 786
Koenen’s tumors, 233 Linear accelerators, 400 disadvantages, 787
Kohlschutter-Tonz syndrome, 56 Linear enamel caries, 411 historical perspective, 781
Koiloncychia, 486 Linear gingival erythema (LGE), 448 image interpretation, 787
Koplik’s spots, 91 Linear IgA bullous dermatosis, 195 methods for obtaining spatial
Kuttner tumor, see Chronic sclerosing sialadenitis Linear IgA disease, 195 resolution, 786
Kvaal’s radiographic method, 672 Lines of Zahn, 16 principles, 781
Kveim test, 620 Lingual frenum, 626 MR scanner, 781
Kwashiorkor, 635 Lingual pits 26 nuclear magnetic dipole moment, 781
Lingual thyroid, 25 proton magnetization, 782
L Lingual tonsils, 12 relaxation, 783
Labial salivary gland biopsy, 275 Lingual varices, 15, 69 resonance, 783
Lacrimal sac, 390 Lip print, 678 spin-echo phenomenon, 784
Lacrimo-auriculo-dento-digital (LADD) Lipids, 635 uses, 787
syndrome, 266 Lipoid proteinosis, 646 Maillard pigments, 79
891
Index
Main d’ accoucheur sign, 544 Microbial plaque, 442 Muscle splinting, 256
Major aphthous ulcers, 211 Microdontia, 35 Muscles of mastication, 244
Malignant ameloblastoma, 363 Microfracture, 464 Muscular dystrophy, 532
Malignant epithelial odontogenic Microglossia, 22 Muscular hypertrophy, 258
ghost cell tumor, 365 Micrognathia, 30 Musculoskeletal disorders, 115
Malignant hypertension, 477 Midline cysts, 324 Mushroom fracture, 254
Malignant melanoma, 368 Migratory glossitis, 168 Mushroom-shaped skull, 577
Malignant schwannoma, 378 Mikulicz cells, 89 Myasthenia gravis, 532, 603
Malignant tumors, 261, 380 Mikulicz’s disease, 277, 591 Mycobacterium tuberculosis, 606
Mandibular advancement devices, 512 Mikulicz ulcer, 211 Myocardial infarction, 479
Mandibular buccal bifurcation cyst, 321 Milia, 194 Myofascial pain, 119, 257
Mandibular condyle, 239 Miliary tuberculosis, 607 Myofibroblastoma, 362
Mandibular infected buccal cyst, 321 Mineralocorticoids, 549 Myositis, 256
Man-made radiation, 702 Minocycline, 64, 78, 79 Myospasm, 257
Manual and automatic processing, 811 Minor aphthous ulcers, 211 Myotomy, 659
comparisons, 811 Mishri, 381
Marasmus, 635 Mobility, 497 N
Marble bone disease, 579 Moderately differentiated squamous cell Nanocomplex, 464
Marfan syndrome, 249 carcinoma, 398 Nasal obstruction, 390
Marijuana, 383 Modified Waters’ view, see Skull Nasopalatine duct cyst, 314
Maroteaux–Lamy syndrome, 645 radiography Nasopharyngeal cysts, 323
Marsupialization, 349 Molar-incisor hypomineralization, 77 Natal and neonatal teeth, 46
Mask-like appearance of face, 602 Molluscum contagiosum, 629 Natal teeth, 46
Masseter, 244 Monocytes, 490 Neck dissection, 399
Masseter muscle, 241, 258 Monoglandular fever, see Infectious Necrotic pulp, 466
Massive osteolysis, 586 mononucleosis Necrotizing fasciitis, 428
Masson-Fontana silver stain, 110 Monomorphic adenoma, 293 Necrotizing sialometaplasia, 215, 284
Masticatory abrasion, 460 Mononucleosis, 450 Necrotizing stomatitis, see Noma
Masticatory space, 429 Monounsaturated fatty acids, 635 Neisseria gonorrhoeae, 254
Matrix metalloproteinases, 444 Moon face, 546, 635 Neonatal line, 52
Maxillary tuberosity, 831 Moon’s molars, 52 Neonatal lupus erythematosus, 598
Maximum permissable dose, 703 Moorrees’ method, 670 Neonatal teeth, 46
May–Hegglin anomaly, 500 Morquio syndrome, 33 Neonatal tetanus, 86
Mayer mucicarmine stain, 110 Morsicatio buccarum, 134 Neospinothalamic tract, 113
McGill pain questionnaire, 116 Morsicatio labiorum, 135 Nervus intermedius neuralgia, 124
McGregor line, 578 Morsicatio linguarum, 135 Neuralgia, 120
Measles, 91 Mosaic pattern, 588 Neurofibroma, 356
Measles-mumps-rubella (MMR) vaccine, 91 Moth-eaten appearance, 395, 435 Neurofibrosarcoma, 378, 379
Meckel’s diverticulum, 523 Mottled enamel, 52 Neurogenic sarcoma, see Neurofibrosarcoma
Medial pterygoid muscle, 242, 258 Mouse-like facies, 602 Neurohypophysis, 538
Median mandibular cysts, 316 Mouth mirror test, 268 Neuromelanin, 18
Median rhomboid glossitis, 155, 168 Mucic acid, 463 Neurosyphilis, 628
Mediastinal group lymph nodes, 393 Mucocele(s), 278 Neurovascular syndrome, 700
Melanin, 62, 18 Mucocutaneous lymph node syndrome, 232 Neutropenia, 455, 491
Melanin incontinence, 29 Mucoepidermoid carcinoma, 295 Nevoid basal cell carcinoma syndrome
Melanoacanthosis, see Oral melanoacanthoma Mucopolysaccharidoses, 454, 644 (NBCCS), 304
Melanocarcinoma, see Malignant melanoma Mucormycosis, 108, 207, 615 Nevus cells, 348
Melanocytic nevi, 62 Mucosa-associated lymphoid tissue (MALT) Niacin deficiency, 455
Melanomas, 369 lymphoma, 277 Nicotinamide adenine dinucleotide phosphate
Melanotic macule 69 Mucositis, 700 (NADPH) oxidase, 384
Melioidosis, 84 Mucous membrane pemphigoid, 186 Nicotine, 382
Melkersson–Rosenthal syndrome, 13, Mucous patches, 206, 627 Nicotine stomatitis, 136
289, 521, 620 Mucous retention cyst, 316 Niemann–Pick disease, 649
Menaquinones, 638 Mulberry molars, 52, 613 Nifedipine, 447, 483
Menstrual cycle-associated gingivitis, 445 Multidrug-resistant tuberculosis, 609 Nikolsky’s sign, 182, 235
Mercury, 661 Multifocal eosinophilic granuloma, 647 Nitrosamine, see Nicotine
Mercury poisoning, 63 Multinucleated giant cells, 582 Nitrosodiethanolamine, 382
Merkel cell carcinoma, 552 Multiple endocrine neoplasia (MEN), 552 Nitrosoproline, 382
Mesh-like pattern, 577 MEN I, 552 Nociception, 111
Mesiodens, 46 MEN II, 552 Nociceptors, 11, 112
Metabolic disorders, 52, 641 MEN III, 552 Noma, 87
Metachromatic dye, 397 Multiple mucosal neuroma syndrome, 552 Noma neonatorum, 87
Metastases, 392 Multiple myeloma, 496 Noma pudendi, 87
Methenamine silver stains, 108 Multiple sclerosis, 531 Non-Hodgkin’s lymphoma (NHL), 495
Methotrexate, 402 Munro’s abscesses, 222 Non-homogeneous leukoplakia, 141
Michel’s solution, 219 Mural growth theory, 304 Non-lipid reticuloendothelioses, 647
892
Index
Non-odontogenic cysts, 314 Osteogenic sarcoma, 573 Peg lateral, 35

Non-specific sialadenitis, 281 Osteoma, 349 Pel–Ebstein fever, 495
Non-starch polysaccharides, 634 Osteomalacia, 455, 638 Pellagra, see Niacin deficiency
Non-suppurative osteomyelitis, 433 Osteomyelitis, 431 Pelvic inflammatory disease, 630
Noonan syndrome, 326 microorganisms responsible, 432 Pemphigoid, 212, 596, 597
North American blastomycosis, 207 Osteopetrosis, 578 Pemphigus, 212, 596
Nuclear medicine, 788 congenita, 579 Pemphigus erythematosus, 596
types, 788 tarda, 579 Pemphigus foliaceous, 181, 596
NUG, 449 Osteoporosis, 846 Pemphigus vegetans, 181, 596
NUP, 449 Osteoporosis circumscripta, 588 Pemphigus vulgaris, 181, 596
Nursing bottle caries, 412 Osteoradionecrosis, 401, 701 Penny test, 811
Nutrients, 633 Osteosarcoma, 373, 374 Peptic ulcer disease, 523
Nutriology, 633 Otalgia, 387 Percussion test, 466
Overlapped image, 816 Perforations, 795
O Oxyphilic adenoma, 295 Periadenitis mucosa necrotica recurrents,
Obstructive sleep apnea syndrome, 512 Oxytocin, 538 see Major aphthous ulcers
Occipital neuralgia, 125 Periapical cemento-osseous dysplasia, 569
Occlusal enamel pearl, see Dens evaginatus P Periapical cyst, 318, 416
Occlusal radiographs, 395, 721 Paan, 381, 382 Periapical granuloma, 416, 419
Occupational exposure, 703 Pacemakers, 484 Periapical pocket cyst, 320
Occupational hazards, 390 Pachyonychia congenita, 225 Periodontal disease, 101, 440, 450, 592
Odontecrexis, 119 Paget’s disease, 586 Periodontal pain, 118
Odontoameloblastoma, 336 Palatal cancer, 590 Periostitis ossificans, 437
Odontogenic carcinomas, 363 Palatal rugae, 15, 663 Peripheral edema, 475
Odontogenic carcinosarcoma, 366 Palatal torus, 19, 20 Peripheral giant cell granuloma, 352
Odontogenic fibroma, 344 Paleospinothalamic tract, 113 Peripheral ossifying fibroma, 348
Odontogenic keratocyst, 307 Pancreatin, 511 Pernicious anemia, 488
Odontogenic myxomas, 344 Pancytopenia, 226, 488 Personal protective equipment (PPE), 728
Odontogenic sarcomas, 365 Panhypopituitarism, 536 Personnel dosimeter, 713
Odontogenic tumors, 332, 344 Pannus, 252 Personnel shielding, 711
Olfactory reference syndrome, 561 Panoramic radiograph, 759 Petechiae, 69
Oligodontia, 45 disadvantages and limitations, 760 Petit mal seizures, 530
Olympian row, 628 indications, 759 Peutz-Jeghers syndrome, 66
Omohyoid muscle, 399 Panoramic radiology, 756 PFAPA syndrome, 215
Oncocytoma, 295 concepts, 756 Phantom bone disease, 585
Oncogenes, 386 technique and imaging principles, 756 Pharyngitis, 507
Opalescent brittle teeth, 577 working principle, 757 Pharyngotonsillitis, 508
Open apices method, 671 origin, 756 Phenytoin-induced gingival enlargement, 446
Open lock, see Dislocation Pansinusitis, 105 Pheochromocytoma, 549
Open mouth Waters’ view, see Skull Papillary cystadenoma lymphomatosum, 294 Pheomelanin, 18
radiography Papillon–Lefevre syndrome, 453 Phleboliths, 16
OPG, 306, 320 Paracoccidioidomycosis, 208 Phoenix abscess, 415
Oral contraceptive, 447 Parade ground fracture, 262 Phosphate, 488
Oral hairy leukoplakia, 100, 516 Paradental cyst, 320 Phosphopeptide, 464
Oral melanoacanthoma, 347 Paramedian lip pits, 26 Photoelectric effect, 696
Oral submucous fibrosis, 159 Paramolar, 46, 47 Photographic effect, 713
Oral warts, 346 Paraneoplastic pemphigus, 184, 185, 596 Photopheresis, 184
Orofacial digital syndrome, 24 Parapharyngeal space, 427 Photostimulable phosphor plates, 733
Orofacial granulomatosis, 289, 620 Parasitic cysts, 327 Phycomycosis, 615
Orofacial pain, 115 Parathyroid glands, 542 Pilocarpine, 288
Oromandibular dystonia, 532 Paratracheal lymphadenopathy, 607 Pink disease, 63
Oromandibular limb hypogenesis syndrome, 22 Parinaud oculoglandular syndrome, 89 Pink form, 655
Oropharyngeal candidiasis, 509 Parkinson’s disease, 278, 531 Pink panters, 510
Oropharyngeal trichomonal infection, 631 Parma projection, 751 Pink puffers, 510
Orthokeratinized odontogenic cyst, 307 Parotid enlargement, 290 Pink tooth, 466, 467
Orthopnea, 475 Parotid papillae, 17 Pink tooth of Mummery, 80
Orthostatic hypotension, 563 Paroxysmal nocturnal dyspnea, 475 Pinta, 626
OSMF, 159, 160, 161 Partial seizures, 530 Pinto’s ligament, 241
Ossifying fibroma, 348, 571 Pastia’s lines, 82, 508 Pit and fissure caries, 410
Osteitis fibrosa generalisata, 543 Patch test, 153 Pituitary dwarfs, 536
Osteoarthritis, 251 Paterson–Kelly syndrome, 487 Pityriasis rosea, 226
Osteoarthrosis, 250 Pathological fracture, 395, 396 Planck’s constant, 687
Osteoclast, 543, 578 Pathological wear, 464 Plasmapheresis, 600
Osteodentin, 640 Patient shielding, 711 Platelet count, 500
Osteodystrophia deformans, 587 Paul-Bunnell test, 90 Platelet disorders, 499
Osteogenesis imperfecta, 576 Pediatric medicines, 462 Platelets, 497
893
Index
Platyspondyly, 578 Pseudo macroglossia, 22 Radioallergosorbent test, 507

Pleomorphic adenoma, 291, 792 Pseudomicrodontia, 35 Radicular cyst, see Periapical cyst
Pleomorphism, 398 Psoriasis, 165, 221 Radiculomegaly, 35
Plumbism, 64 Psychosomatic factors, 455 Radiographic evaluation of carious lesions, 425
Plummer–Vinson syndrome, 487 Pterygomandibular space, 427, 430 Radiographic faults, 812
Pocket dosimeter, 713 Ptyalism, 63, 278 causes, 812
Podophyllin, 103, 630 Puberty-associated gingivitis, 445 Radiographic film, 731
Polonium, 382 Pulmonary tuberculosis, 608 Radiographic image formation, 801
Polyarthritides, 252 Pulp calcifications, 468 Radiographic infection control, 728
Polycyclic aromatic hydrocarbon (tars), 382 Pulp canals, 42, 459 Radiographic landmarks, 83, 825
Polycythemia, 485 Pulp hyperemia, see Focal reversible pulpitis common to both the maxillary and
Polycythemia rubra vera, 485 Pulpal pain, 116 mandibular radiographs, 825
Polyunsaturated fatty acids, 635 Pulse oximetry, 424 extraoral, 837
Pontiac fever, 512 Pulse rate, 476 radiolucent anatomical, 836
Poorly differentiated squamous cell Pulse rhythm, 476 radiopaque anatomical, 836
carcinoma, 399 Punch biopsy, 398 unique to the mandibular radiograph,
Popcorn calcifications, 578 Punched-out radiolucencies, 497 825, 833
Porphyria, 642 Purified protein derivative, 609 unique to the maxillary radiograph, 825, 827
Porphyria cutanea tarda, 643 Pyoderma gangrenosum, 520 Radiographic techniques, 724–797
Portable handheld X-ray unit, 691 Pyogenic granuloma, 353 conventional imaging, 724, 725
Position indicating device (PID), 694, 706, 709 Pyostomatitis vegetans, 623 extraoral radiography, 724
Positive identification, 657, 678 intraoral radiography, 724
Positron emission tomography, 294 Q specialized imaging, 724, 760
Possible identification, 657, 678 Qualitative disorders, 491 arthrography, 725
Posterior auricular nodes, 91 Qualitative neutrophil defects, 488 computed tomography (CT), 724
Posterior cervical muscles, 245 Quantitative defects, 485 dentomaxillofacial cone-beam
Postmortem dental examination, 655 computed tomography (CBCT), 724
Postmortem identification, 655 R magnetic resonance imaging, 725
Postmortem pink teeth, 661 Rabbit fever, 83 nuclear medicine, 725
Postmortem radiographs, 657 Racial pigmentation, 17 sialography, 725
Postnasal drip, 105 Radiation, 701 thermography (thermal imaging or
Pregnancy, 445, 462, 550 sources, 701 infrared imaging), 725
Pregnancy gingivitis, 551 cosmic sources, 702 ultrasonography, 725
Pregnancy tumor, 445 natural radiation, 702 Radiography, 703
Pregnancy-associated gingivitis, 445 terrestrial sources, 702 dose limits, 703
Prenatal diagnosis of clefts, 28 Radiation biology, 690, 697 patient exposure, 703
Prevotella intermedia, 454 deterministic effects, 699 reducing dental exposure, 703
Prickle cells, 398, 399 of total body radiation, 699 Radioisotope scan, 394
Primary herpetic gingivostomatitis, 176, 200 on tissues and organs, 699 Radionuclide imaging, 294, 541, 588
Primary HPT, 542 effects on living systems, 697 Radiotherapy, 363, 364, 399
Primary intraosseous squamous cell bystander effect, 699 Radiotracer, 395, 584
carcinoma, 364 cell cycle effects, 698 Raman spectroscopy, 395
Primary hypertension, see Hypertension direct effect, 697 Rampant caries, 411, 426
Prinzmetal’s angina, 478 effects in the developing embryo Ramsay Hunt syndrome, 124, 125, 179, 529
Probable identification, 657 and fetus, 700 Ranula, 279
Probe, 394, 395 on oral tissues, 700 Rapid plasma reagin (RPR), 613
Processing solutions, 810, 811 film exposure and processing, 714 Ras protein, 386
composition, 810 stochastic effects, 699 Raspberry like papillomas, 228
developer solution, 810 Radiation caries, 401, 412, 426, 701 Rathke’s pouch, 324
fixing solution, 810 types, 701 Raw-ham colored lesions, 627
test to determine the quality, 811 Radiation dose, 399 Ray phenomenon, 87
Progesterone, 550 Radiation hazards, 685 RBC disorders, 210
Proliferative verrucous leukoplakia, 142 Radiation-induced cancer, 700 Reactive leukocytosis, 492
Proptosis, 390 Radiation-induced heritable diseases, 700 Receptor placement, 740
Prostaglandins, 112 Radiation monitoring devices, 713 Recommended dietary allowance (RDA), 633
Protective co-contraction, 256 Radiation physics, 687 Recurrent caries, 412, 425
Protein energy malnutrition (PEM), 635 fundamentals, 687 Recurrent erythema multiforme, 190
Proteins, 635 electromagnetic radiation, 687 Red lesion, 134
Proteolysis-chelation theory, 406 ionizing and non-ionizing Red strawberry tongue, 83
Proteolytic theory, 406 radiation, 688 Referral pattern for myofascial pain, 258
Prothrombin time, 498 particulate radiation, 687 Regional odontodysplasia, 59
Proton pump inhibitors, 269, 523 production of X-rays, 688 Reiter’s syndrome, 215
Protostylid, 44, 45 radiation, 687 Renal failure, 513
Protruded tongue, 662 Radiation protection survey, 711 Renal osteodystrophy, 516, 542
Proximal surface caries, 417 Radiation safety and protection, 701, 728 Renal rickets, 516
Psammomatoid ossicles, 573 historical events in, 701 Rendu-Osler-Weber syndrome, 69
894
Index
Reparative dentin, 468 Saucerization, 436 Silent thyroiditis, 539, 540

Residual cyst, 320 Scarlet fever, 82 Simian gait, 587
Respiratory infections, 504 Schaumann bodies, 290, 619 Simmond’s disease, 536
Respiratory tract diseases, 506 Scheie syndrome, 644 Sinusitis, 104
Resting and reversal lines, 588 Schirmer’s test, 271, 272 Sistrunk procedure, 325
Rests of Malassez, 318, 320, 337, 363 Schuller method, 748 Situs inversus, 22
Reticular atrophy of pulp, 468 Schwannoma, 356 Sjögren’s syndrome, 791
Reticular forms, 148 Scintigraphy, 271, 496, 543 Skin prick test, 507
Retrocuspid papilla, 14 Scintillation, 713 Skull-photograph superimposition, 666
Retrodiscitis, 249 Sclerotic cemental masses, 570 Skull radiography, 743
Retrograde parotitis, 515 SCOFF questionnaire, 525 landmarks for positioning, 743
Retropharyngeal space, 427, 430 Scooped dentin, 464 patient preparation, 744
Retrozygomatic space, see Infratemporal Scratch test, 81 planes for positioning of the skull, 743
fossa space Screw-driver appearance, 52 positioning errors, 744
Reverse smoking, 383, 390 Screwdriver edge-shaped central incisors, 628 positioning terminology, 744
Reverse Townes’ view, 750 Screwdriver-shaped incisors, 613 radiation protection, 744
Reverse Waters’ method, see Skull Screw-type implants, 851 techniques, 744
radiography radiographic appearance, 851 acanthoparietal projection, 747
Rhabdomyoma, 357 Scrofula, 608 AP axial projection, 749
RHD, 480 Scrotal tongue, 13 bimolar projection, 751
Rheumatic fever, 480 Scurvy, 499 lateral cephalometry, 749
Rheumatoid arthritis, 252, 592, 595 Secondary aldosteronism, 549 lateral oblique of the ramus of the
Rheumatoid factor, 253, 600 Secondary dentin, 468 mandible, 751
Rheumatoid synovial fluid, 594 Secondary hypertension, 476 lateral view, 748
Rhinocerebral zygomycosis, 616 Secondary pulmonary tuberculosis, 607, 608 modified parietoacanthial
Rhinoscleroma, 88 Selenium, 144, 385 projection, 747
Rhinoviruses, 506 Self-developing film, 808 30° occipitomental, 746
Rhizomegaly, 35 Self-healing carcinoma, 347 PA cephalometric, 745
Rhizomicry, 35 Senear–Usher syndrome, 596 PA mandible, 745
Rhodopsin, 636 Sentinel node, 394 parietoacanthial view, 747
Riboflavin deficiency (riboflavinosis), 455 Septic arthritis, 252 posteroanterior projection, 745
Rickets, 52, 638 Sequelae of pulpitis, 413, 416 rotated PA view, 746
Rickety rosary, 638 Sequestrated bone, 401 standard occipitomental view, 746
Riga–Fede disease, 47, 197 Sequestrectomy, 436 submentovertex view, 748
Rigor mortis, 659 Serous non-secretory cyst, 317 Slaked lime, 139, 381
Risus sardonicus, 85 Serum alkaline phosphatase levels, 575, 584 Slow virus, 587
Rodent ulcer, 367 Serum bilirubin, 525, 526, 528 SLUDGE syndrome, 604
Root caries, 425 Sex hormones, 454 Sly syndrome, 645
Root dwarfism, 35 Sexually transmitted diseases, 625 Smoker’s melanosis, 65
Root end cyst, see Periapical cyst Shagreen patch, 233 Smoker’s palate, 136
Root surface caries, 410, 417, 461 Shell teeth, 57 Smoking, 452
Rootless teeth, 58 Shepherd’s crook deformity, 575 Smooth surface caries, 410
Rose bengal staining, 272 Shingles, 178, 201 Snail track ulcers, 207, 613, 627
Row of tombstones, 183 Shovel-shaped incisors, 41 Snarling face, 603
Rubella, 91 Sialadenitis, 281 Snuff, 139, 382
Rubeola, 91 Sialadenosis, 287 Snuff dipper’s cancer, 366
Rugger-jersey sign, 579 Sialography, 793, 795 Soap bubble appearance, 322, 334, 362
Rushton’s hyaline bodies, 319 contraindications, 795 Social profiling, 662
Russel bodies, 89 indications, 793 Sodium valproate, 446, 566
injection of the contrast medium, 794 Solid PIOC, 364
S phases, 794 Somatostatin analog, 537
Sabouraud’s agar, 108, 109, 157 procedure, 793 Source to image receptor distance (SID), 708
Saddle nose, 617, 628 armamentarium, 793 South American blastomycosis,
Safe light, 805 preimaging assessment, 793 see Paracoccidiodomycosis
Saliva, 267 preimaging instructions to the Spatula test, 86
Saliva swab, 676 patient, 793 Speckled leukoplakia, 219
Salivary calculi, 280 technique, 793 Sphenomandibular ligament, 241
Salivary dysfunction, 75, 400 Sialolithiasis, 280 Spindle-shaped fibroblasts, 582
Salivary flow rate, 408 Sialometry, 274, 290 Splendore-Hoeppli phenomenon, 88
Salivary gland scans, 789 Sialorrhea, 278 Splenus capitis, 245
clinical applications, 789 Sialosis, 287 Split papules, 206, 627
procedure, 789 Sicca complex, 277 Spoon-shaped nails, see Koilonychia
Sandpaper rash, 82 Sicca syndrome, 271 Squamous cell carcinoma, 364, 398, 399
Sandpaper-like exanthema, 508 Sickle cell anemia, 489 Squamous odontogenic tumor, 337
Sanfilippo syndrome, 645 Sickle cell trait, 489 Squamous papilloma, 345
Sarcoidosis, 289, 618 Siga, 409, 462 St. Anthony’s fire, 84
895
Index
Stable angina, 478 Systemic lupus erythematosus Tongue hemorrhage, 662

Stafne’s bone cyst, 323 (SLE), 172, 597 Tonsillar pillars, 91, 110, 390
Static bone cyst, 323 Systemic sclerosis, 601 Tonsillitis, 83, 507
Stellate-shaped fibroblasts, 570 Topographical occlusal technique, 736
Stensen’s duct, 17, 267, 285 T Tori, 19
Step down transformer, 693 Tabes dorsalis, 612, 627 Tornwaldt’s bursa, 324
Sterilization, 104 Talon cusp, 39, 40 Torus mandibularis, 19, 20
Sternocleidomastoid, 245 Tam O’Shanter skull, 578 Torus palatinus, 20
Steroids, 186, 509, 533, 580 Tanaka’s ligament, 241 Total filtration, 694
Stevens–Johnson syndrome (SJS), 191, 216 Target or double halo appearance, 522 Tourniquet test, 498
management guidelines, 512 Target lesion, 190 Townes’ method, 749
Still’s disease, 254 Tars, 382–383 Toxic epidermal necrolysis (TEN), 191
Stomatitis medicamentosa, 209 Taurodontism, 42, 43 management guidelines, 512
Stomatitis venenata, 209 T-cell immunity, 590 Traditional CT, 397
Strawberry cervix, 631 Technetium scan, 394 Tram line calcifications, 68
Strawberry gingivitis, 108, 617, 618 Teletherapy, 400 Transcutaneous electric nerve stimulation
Strawberry tongue, 83, 232, 508 Telomeres, 225, 385 (TENS), 258, 484
Stress reflux syndrome, 461 Temporal space, 429 Transient neonatal MG, 603
Stretch test, 14 Temporalis muscle, 241 Transmigration, 48
Stridor, 428, 505 Temporomandibular joint (TMJ), 239, Trapezius, 245
String of beads sign, 195 241, 243, 593, 840 Traumatic arthritis, 252
Stunted roots, 401, 578 arterial supply, 243 Traumatic bone cyst, 321
Sturge–Weber syndrome, 40, 45, 68 examination, 243 Traumatic neuroma, 357
Stylomandibular ligament, 241 radiography, 751 Traumatic ulcers, 197, 199
Subacute necrotizing sialadenitis, 283 sensory innervation, 243 Treacher Collins syndrome, 27, 31, 33
Subchondral bone cysts, 251 transcranial view, 751 Treponema pallidum, 200, 206, 612, 626
Subcutaneous injections, 580 transorbital view, 752 Triangular shaped face, 577
Subglottic larynx, 393 transpharyngeal view, 751 Trichiasis, 195
Sublingual space, 427, 429, 431 venous drainage, 243 Trichinosis, 329
Subluxation, 248, 249 Temporomandibular joint imaging, 849 Trifid condyles, 34
Submandibular nodes, 389 Teratoid cysts, 327 Trifacial neuralgia, 120
Submandibular space, 427, 429, 431 Tertiary syphilis, 627, 628 Trigeminal neuralgia, 120–123
Submental nodes, 393, 394, 429 Test cavity, 422 Trigeminal neuropathy, 101, 628
Submental space, 429 Tetanolysin, 85 Trigger point therapy, 258
Submerged teeth, 49 Tetanospasmin, 85, 86 Trigger points, 120, 125
Succinic acid, 463 Tetanus, 85 Trismus, 85, 428
Sucrose chelation theory, 406 Tetracycline, 78, 444 Trisomy, 22, 43
Sugar substitutes, 634 Thalassemia, 490 Trousseau’s sign, 544
Sulcus terminalis, 16 Thermal burns, 135 True cysts, 303, 304
Sulfur colloid, 394, 788 Thermography, 796 True macroglossia, 22
Sulfur granules, 86, 87 indications, 797 TSH, 533, 535, 540
Sunray appearance, 362, 371, 374, 375 patient preparation, 797 Tuberculoid leprosy, 610, 611
Superficial fascia, 426 procedural requirements, 797 Tuberculosis, 101, 205, 606–611
Superficial hemangiomas, 358 techniques, 796 Tuberculous lymphadenitis, 608
Superior joint space, 240, 247 Thermoluminescent dosimetry (TLD) Tuberculous meningitis, 607
Supernumerary teeth, see Hyperdontia badges, 714 Tuberculous osteomyelitis, 205, 608
Supine hypotensive syndrome, 551 Thiamin deficiency, 455 Tuberous sclerosis complex, 232
Supplemental teeth, 46, 47 Thinning of articular disk, 246 Tularemia, 83–84
Suppurative osteomyelitis, 433, 434 Thiocyanate, 383 Tumor markers, see Blood studies
Supraclavicular nodes, 393, 608 Third molars in age estimation, 671 Tumor suppressor genes, 143, 386
Supraomohyoid triangle, 394 Thistle-tube-shaped pulp chamber, 59 Tunneling technique, 659
Suprasternal notch, 325, 393 Thorny radiation, 585 Turner syndrome, 45, 326, 550
Sutton’s ulcers, see Major aphthous ulcers Thrombocytopenia, 488, 500 Turner’s hypoplasia, 51
Sweet criteria, 121 Thrombocytopenic purpura, 456, 500 Turner’s tooth, 51
Swift disease, 63 Thrombotic thrombocytopenic purpura, 500 Type I collagen, 543, 576, 592
Swimmer’s erosion, 464 Thyroid collars, 709, 711, 715 Types of dislocation, 249
Swiss cheese pattern, 298 Thyroid gland, 538, 539, 542 Tzanck cells, 176, 177
Symblepharon, 187, 195, 227 Thyroid storm, 541 Tzanck smear, 201, 648
Synovial chondromatosis, 261, 262 Thyrotoxic crisis, 541 Tzanck test, 183
Synovial chondrosarcoma, 261 Thyrotoxicosis, 540, 591
Synovial fluid, 240 Thyroxin, 538, 540 U
Synovial sarcomas, 261, 372 Tic douloureux, see Trigeminal neuralgia Ugly duckling sign, 369
Synovitis, 249 Tissue biopsy, 395, 614 Ulcerative colitis, 214, 520
Syphilis, 384, 612, 626 TNM staging, 391 Ulceronodular disease, 207, 627
Syphilitic aortitis, 627 Tobacco, 138, 381 Ultrasonography, 789–792
Syphilitic osteomyelitis, 628 Toluidine blue staining, 142, 397 Unicystic ameloblastoma, 336
896
Index
Unifocal eosinophilic granuloma, 647 B1-thiamine, 640 White spots, 819

Universal precautions, 104 B2-riboflavin, 640 White strawberry tongue, 83
Unstable angina, 478 C, 455, 639 Wickham’s striae, 147, 219, 236
Upper respiratory tract infections, 506 D, 455, 637 Wilms’ tumor, 31
Urbach–Wiethe disease, 646 E, 145, 455 Winchester syndrome, 586
Uremia, 514, 515 K, 502, 638 Witkop’s disease, see Hereditary benign
Uremic fetor, 514 Vitamin K deficiency, 503 intraepithelial dyskeratosis
Uremic frost, 515 Volatile sulfur compounds, 444 Wood-hard tongue, 646
Uremic gastroenteritis, 514 Von Ebner’s glands, 17 Wormian bones, 540, 578
Uremic stomatitis, 518 Von Recklinghausen disease of bone, 542
Uremic syndrome, 514 Von Willebrand factor, 497 X
Uremic toxin, 514, 517 Von Willebrand’s disease, 503 Xeroderma pigmentosum, 226
Usher syndrome, 56, 596 Vrolik syndrome, 576 Xerophthalmia, 271, 636
Uto-Aztecan premolar, 44 Xerosis, 636
W Xerostomia, 267–270, 700
V Waddling, 587 X-rays, 685, 687, 689
Vanishing bone disease, 585 Wallerian degeneration, 529 properties, 689
Varicella zoster infection, 178, 201 Wall mounted intraoral radiographic unit, publications, 685
Variegate porphyria, 643 690 X-ray beam angulation and
Vascular disorders, 474, 476, 498, 499 Warfarin, 484, 504 alignment, 727
Vascular malformations, 68, 357, 358 Warthin–Starry method, 89, 613 X-ray equipment, 706, 710, 712
Velscope and Vizilite plus, 395 Warthin’s tumor, 294, 295 X-ray photons, 695
Venereal disease research laboratory (VDRL) Water brash, 278, 519 X-ray tube, 692
tests, 613, 628 Water-clear cells, 310 schematic diagram, 692
Venereal diseases, 625 Water lily sign, 328 Xylitol, 276, 634
Venereal wart, see Condyloma acuminatum Water soluble vitamins, 636, 639
Verapamil, 446 Waters’ view, see Skull radiography Y
Verocay bodies, 356 WBC disorders, 210 Yaws, 612, 626
Verruca vulgaris, 346 Weber and Fechner’s law, 111 Yellow form, 657
Verrucous carcinoma, 366–367 Weber’s glands, 267 Yunis–Varon syndrome, 48
Verrucous hyperplasia, 346 Weeping lubrication, 247
Verrucous leukoplakia, 138, 141 Wegener’s granulomatosis, 617, 618 Z
Vertical angulation, 740 Well-differentiated squamous cell Zahn lines, 16
Vietnamese time-bomb, 85 carcinoma, 398 Ziehl–Neelsen stain, 606, 609
Viral pharyngitis, 507 Wet beriberi, 640 Zinc sulfate, 229, 401
Visual analog scale (VAS), 116 Wharton’s duct, 267, 280 Zinsser–Engman-Cole syndrome, 225
Vital signs, 482, 550 Wheezing, 505 Zollinger–Ellison syndrome, 523
Vital staining, 395, 397 Whiff test, 631 Zoster ophthalmicus, 179, 235
Vitality tests, 468, 522 White blood cells, 490 Zoster sine herpete, 124, 179
Vitamin(s), 636–641 White coat hypertension, 477 Zygomatic bone, 832
A (retinol), 636 White lesion, 133 Zygomatic process, 832
B complex, 455, 640 White sponge nevus, 166 Zygomycosis, 615
897

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Oral Medicine, Oral Diagnosis

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Part I—Oral Medicine and Oral Diagnosis

SECTION I INTRODUCTION AND APPROACH TO DIAGNOSIS

Chapter 1 History and Scope of Oral Medicine and Oral Radiology 5

SECTION II ORAL AND MAXILLOFACIAL DISTURBANCES

Chapter 2 Developmental Disturbances 11

Chapter 3 Orofacial Pigmentation Disorders 61

Chapter 4 Bacterial, Viral and Fungal Infections 82

Chapter 5 Orofacial Pain 111

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SECTION III MUCOCUTANEOUS DISORDERS

Chapter 6 Red and White Lesions 133

Chapter 7 Vesiculobullous Disorders 174

Chapter 8 Oral Ulcerative Diseases 196

Chapter 9 Dermatological Diseases 218

SECTION IV DISEASES OF SPECIFIC STRUCTURES

Chapter 10 Temporomandibular Disorders 239

Chapter 11 Diseases of Salivary Glands 265

SECTION V CYSTS AND TUMORS OF OROFACIAL REGION

Chapter 12 Cysts of Orofacial Region 303

Chapter 13 Tumors of Orofacial Region 331

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Chapter 14 Oral Cancer 380

SECTION VI TEETH AND PERIODONTIUM

Chapter 15 Dental Caries, Pulp and Periapical Lesions 405

Chapter 16 Gingival and Periodontal Diseases 440