DISORDER OF DEVELOPMENT OF

THE TEETH AND RELATED TISSUES.

Dr. M. Wachira, BDS, OMS.

Requirements for development of an ideal dentition;

Formation of a full complement of teeth. i.e. formation
of the full dental formula.
Normal development of the dental tissues.
Eruption of each group of teeth at the appropriate
time into an adequate space.
Normal development of jaw size and relationship.
Eruption of teeth into correct relationship to occlude
with their opposite numbers.

Disorders of development of
teeth;
Abnormalities in number;-anodontia or
hypodontia, additional teeth
(hyperdontia)
Disorder in eruption.
Defects of structure;- enamel defects,
dentine defects.
Developmental anomalies of several
dental tissues;- odontomas.

Developmental disorders of
teeth
Hereditary Factors
All congenital diseases are
developmental.
Not all developmental diseases are
congenital(can be acquired).

Hereditary Factors

Genes occur in pairs(from each parent).

Genes forming a pair are called alleles.
Genes are composed of DNA (deoxyribonucleic acid)
The genome is the complete set of genes.
Genotype is the genetic makeup of an individual.
Phenotype is the visible trait that results from a
particular genotype.
If two alleles are similar - homozygous
If two alleles are dissimilar - heterozygous.
A dominant gene produces its effect both in
heterozygote/homozygote.
A recessive gene produces it effect only in the
homozygous condition.

Classification of hereditary
diseases
Genetic defects can be
Inherited /Acquired
Classification of hereditary
diseases
Chromosomal abnormalities
Single gene defect
Multifactorial inheritance

Chromosomal abnormalities
Can be classified into those that involve
Autosomes
Sex chromosomes
Each can be subdivided into
Numerical anomalies
(trisomy/monosomy)
Structural anomalies
(translocation/deletion)

Single gene abnormalities

(Single gene defect, unigenic,
unifactorial).
Anomalies may affect the genes
located on the autosomes (autosomal
linked diseases), or affect the genes
located on the sex chromosomes (Xlinked diseases).

Multifactorial inheritance
It has been suggested that such traits
are caused by many genes (polygenic)
plus the effect of environment, so called
multifactorial inheritance. E.g.
Psychiatric disorders - schizophrenia,
manic depressive psychosis,
Diseases of modern society hypertension, diabetes mellitus,
rheumatoid arthritis, peptic ulcer,
ischemic heart disease

Environmental Factors
1. Teratogens
A teratogen causes malformation if exposure occurs
when the embryo is sensitive to its effects.
2. Drugs and chemicals
Alcohol: growth retardation.
Phenytoin: Cleft lip and or cleft palate.
Tetracycline: Teeth pigmentation
Fluorides: Mottled enamel
3. Infections
Syphilis: Dental anomalies
4. Radiation
Almost all types or radiation can cause developmental
malformations.

Abnormalities in Number
(Hyperdontia)

These are the effects of excessive but organized growth of the dental lamina of unknown
cause. Additional teeth create areas of stagnation and greater susceptibility to caries, gingivitis
and periodontitis. A supernumerary tooth may prevent a normal tooth from erupting.
Supernumerary Teeth (Not similar morphologically to normal teeth). Most frequently form in
the incisor or molar region and very occasionally in the midline (mesiodens)
Small and conical in shape. E.g. Mesiodens (between upper centrals)/Paramolar/
Distodens(Distal to third molars)
Supplemental Teeth(Similar morphologically to normal teeth).
Common sites: are Upper lateral incisors/Lower premolar region/Distal to the lower third molar
Common in Cleidocranial dysplasia.
Pre-deciduous Dentition
Present at birth - natal teeth.
Erupt during the first month - Neonatal teeth.
Post-permanent Dentition
formed after the permanent dentition.

Syndromes associated with hyperdontia:Cleidocranial dysplasia-many additional teeth develop but fail to erupt.

Decrease in Number of Teeth

(Hypodontia, Oligodontia,
Anodontia)
1. Absence of single tooth.
- Maxillary lateral incisors tooth.
- Lower premolars.
- Third molars
2. Absence of group of teeth.
3. Absence of all teeth (total
anodontia)

Cleidocranial Dysplasia
1. Etiology
Autosomal Dominant
2. Clinically:
Partial or complete absence of the clavicles
Delayed shedding of deciduous teeth.
Unerupted supernumerary or supplemental teeth.
Hooked roots.
Enamel hypoplasia.
Teeth, conical shape.
Gemination
Absence of cellular cementum.

Hereditary Ectodermal
Dysplasia

Etiology
1. X-linked anhidrotic, most common form.
2. Autosomal recessive.
3. Autosomal dominant.
X-linked Anhidrotic Ectodermal Dysplasia (EDA)
Males
Absent or thin patchy hair.
Absence of sweat glands (anhidrosis), cannot tolerate
hot weather.
Lack of sebaceous glands (dry skin).
Dental manifestations include partial or total anodontia
with malformation of any teeth that may be present
(small/peg shaped).

Abnormalities in Size
Macrodonita
Means large teeth.
Macrodonita may be true or relative.
Relative macrodontia results from disproportion between size
of teeth and size of jaws/skull.
True macrodontia may involve all teeth, group of teeth or
single tooth.
Most commonly affected, maxillary central incisors, canines.
Microdontia
Means small teeth.
Microdontia may be true or relative.
Relative microdontia results from disproportion between size,
teeth and size of jaws or skull.
Most commonly affected maxillary lateral incisors, third
molars.

Abnormalities in Shape 1
1. Gemination
One tooth appears as two teeth joined together.
Results from partial division of the enamel organ.
Single root canal.
The number of teeth is normal.

2. Fusion

Two teeth are joined together.

Results from the union of two adjacent enamel organs.

Usually two root canals.

Fusion must involve dentine.

The number of teeth is reduced by one.

3. Concrescence
Two teeth are joined along their roots by
cementum.
Trauma or crowding of teeth can cause
concrescence.

Abnormalities in Shape 2
4. Dilaceration

Sharp bend along the long axis of a tooth.

Due to trauma during tooth development which displaces the calcified

portion of the tooth while the rest of the tooth develops in an abnormal
angle.

5.Taurodontism
Enlargement of the body and the pulp chamber of a multirooted tooth with
apical displacement of the pulpal floor and bifurcation of the roots.

6.Dens in dente(Dens Invaginatus)

Is the deep surface invagination of the crown or root that is lined with enamel.

Coronal dens in dente is seen more frequently.

In order of decreasing frequency, the teeth that are affected most include permanent
lateral incisor, central incisors, premolars, canines & molars. Maxillary predominance
is seen.

The depth of the invagination varies to a slight enlargement of the cingulum pit to a
deep infolding that extends to the apex.

During eruption, the lumen of the invagination is filled with soft tissue that becomes
necrotic on eruption. Classifications;
Type I-invagination confined to the crown.
Type II-extends below the CEJ and ends in a blind sac that may or may not
communicate with the adjacent dental pulp.
Type III-extends through the root and terminates in the apical areas and has no
communication with the pulp.

Appears radiographically as a tooth within a tooth

Abnormalities in Shape 3
7.Dens Evaginatus (central tubercle)

A cusplike elevation of enamel located in the central groove or the lingual ridge of
the buccal cusp of the permanent premolar or molar teeth.

Usually bilateral on premolars mostly and shows mandibular predominance.

8.Enamel Pearl

Globule of enamel usually found on the bifurcation.

Arise from localized bulging of the odontoblastic layer which may provide
prolonged contact between Hertwigs root sheath and the developing dentin
triggering enamel formation.

9.Talon Cusp (Dens Evaginatus of

anterior teeth)

Extra cusp on the lingual surface of the cingulum area.

Usually affects upper teeth.

Abnormalities in Shape 4
10.Supernumerary Roots

Refers to the development of more roots on a tooth than is

described in dental anatomy.
Found on both primary and secondary dentitions.
Most commonly affects molars (3rd molars)

11.Supernumerary Cusps
Most common is the Carabelli cusp
located on the palatal surface of the
mesiolingual cusp of a maxillary molar. Its
normal.

12.Congenital Syphilis
(a) Hutchinsons teeth

(b)

Moons molars

(c)
(d)

Mulberry molars
Enamel hypoplasia

Abnormalities in Structure

Etiology
1. Acquired causes
A. Local factors
Infection
Trauma
B. Systemic factors
Cytotoxic drugs/Tetracycline/Fluorides
Dietary deficiencies - calcium/phosphorus/vitamins (A,
C, D).
Hormonal disturbances (hypoparathyroidism)
Irradiation
Systemic Infections - Syphilis
2. Hereditary causes

Differences between Hereditary and Acquired Defects

Hereditary Defects

Acquired Defects

Affect enamel or dentine only

Affect enamel and dentine

Affect both dentitions

Affects one dentition usually the

permanent

Usually affect all teeth

Usually affect single or group of

teeth

Produce vertically or randomly

oriented pits or defects

Produce horizontally oriented pits

or defects.

Classification of Abnormalities
in Structure

1.

Acquired defects
A Acquired focal enamel hypoplasia (Turners
tooth).
B Acquired generalized enamel hypoplasia.
C Dental fluorosis
D Abnormal discoloration

2.

Hereditary defects
A Hereditary defects of enamel
1.Amelogenesis imperfecta
B Hereditary defects of dentine
1. Dentinogenesis imperfecta.
2. Dentinal dysplasia

Acquired Abnormalities in
Structure
1. Acquired Localized Enamel
Hypoplasia
Affects only one or two permanent
teeth
Turners tooth.
Localized trauma or localized infection
of a deciduous tooth which impinges
upon its developing permanent
successor.
Shows pits, or grooves with brown

Acquired Abnormalities in
Structure
2. Acquired Generalized Enamel
Hypoplasia
Enamel hypoplasia affecting group of
teeth.
Systemic factors involved.
Clinically as a horizontal line of small
pits, grooves with brown pigmentation.
Involve teeth which are formed during
the first_after birth.
Permanent incisors, canines and first
molars.
3. Dental Fluorosis (Mottled Enamel)
1.5 ppm or more.

Abnormal Tooth Discoloration

Discoloration by exogenous
pigments or chemicals
Diet
Coffee and tea
Tobacco
Discoloration by endogenous
pigments or chemicals
Fluorine
Tetracycline

Hereditary Defects of Enamel

Amelogenesis Imperfecta

A group of developmental hereditary defect of enamel. Inheritance can be autosomal dominant,

recessive or X-linked but the most common types have an autosomal inheritance and are thought to
be caused by mutations in the AMEL-X gene, which codes for ameloblastin (C4), enamelin (C4) or
tuftelin (C1). In the case of the autosomal dominant type of amelogenesis imperfecta, the locus of the
defective gene is on chromosome 4q21 to which enamelin maps. X-linked types are caused by a
variety of defects in the amelogenesis genes.

Genetic factors act throughout the whole duration of amelogenesis, therefore all teeth are affected
and both dentitions are also affected.

Clinical types of amelogenesis imperfecta

1. Hypoplastic type (AD, AR, XD)

2. Hypomineralized type (AD, AR)

3. Hypomaturation type (AD, AR, XD, XR)

4. Hypoplastic/hypomaturation type (AD)

AD: Autosomal dominant, AR: Autosomal recessive, XD: X-linked dominant, XR: X-linked recessive.

Genes involved

1. Amelogenin (AMELEX) (AMGX)

2. Ameloblastin

3. Enamelin (ENAM)

Hypoplastic amelogenesis imperfecta;Inadequate formation of the matrix.

Enamel is randomly pitted, grooved or very thin but hard and translucent.
The defects tend to be stained but the teeth are not esp. susceptible to caries unless the enamel is
scanty and easily damaged.
Main patterns of inheritance are autosomal dominant and recessive, X-linked and (a generic rarity) an Xlinked dominant type in which there is almost complete failure of enamel formation in affected males,
while in females the enamel is vertically ridged.

Hypomaturation amelogenesis imperfecta;

The enamel is normal in form on eruption but opaque, white to brownish-yellow.
Teeth appear similar to mottled fluoride effects but theyre soft and vulnerable to
attrition though not as severely as the hypocalcified type.

Hypocalcified amelogenesis imperfecta;Enamel matrix is formed in normal quantity but poorly calcified.
When newly erupted the enamel is normal in thickness and form but weak and
opaque or chalky in appearance.
The teeth tend to become stained and relatively rapidly worn away.

The upper incisors may acquire a shouldered form due to the chipping away of the
thin, soft enamel of the incisal edge.

Hereditary Defects of Dentine

Dentinogenesis Imperfecta ( odontogenesis
imperfecta );A group of developmental hereditary defect of dentine. Is a defect
of collagen formation that is transmitted as an autosomal dominant
trait.
Defects in the genes COL1A1 and COL1A2 for the procollagen
alpha helix, prevents polymerization into normal type 1 collagen.
Closely related to osteogenesis imperfecta particularly type IV.
Classification
1. Dentinogenesis imperfecta type I (associated with osteogenesis
imperfecta).
2. Dentinogenesis imperfecta type II (not associated with
osteogenesis imperfecta) (brown opalescent dentine) (Shields
type).
3. Dentinogenesis imperfecta type III (shell tooth) (Brandywine
type)-dental development delayed in 20%
4. Dentinal Dysplasia (Rootless Teeth)
Affects both deciduous and permanent dentitions.
Normal coronal morphology and color.
Very short and conical tapered roots.

Clinical features:Enamel appears normal but uniformly brownish or purplish and abnormally
translucent.
The form of the teeth is essentially normal but the crowns of the molars tend
to be bulbous and the roots are usually short.
Enamel is weakly attached and tends to chip away from the dentine
abnormally easily.
In severe cases, the teeth become rapidly worn down to the gingivae.
Radiographically, the main features are obliterated pulp chambers and
stunted roots.

Pathology:The earliest dentine formed at the amelodentional junction is normal

but the deeper layers are defective in that tubules become few,
calcification is incomplete and the matrix is imperfectly formed. The
pulp chamber becomes obliterated and odontloblasts degenerate but
cellular inclusions in the dentine are common. Scalloping of the
amelodentional junction is stms absent. The enamel tends to split
from the dentine.

SHELL TEETH ( Dentinogenesis Imperfecta type III)-Only a thin

shell of hart dental tissue surrounds overlarge pulp chambers. Theres
normal but thin mantle dentine which covers irregular dentine. The pulp
lacks a normal odontoblast layer and consists of coarse connective
tissue which becomes incorporated into the deep surface of the dentine.

Dentinal dysplasia (rootless teeth)- short and conical root. The

pulp chambers are obliterated by multiple nodules of poorly
organized dentine containing sheaves of tubules. Tooth lost early
in life.
Regional odontodysplasia (ghost teeth)-disorder of development
that affects a group of teeth in which there are severe
abnormalities of enamel, dentine and pulp. Affected teeth have
very thin enamel and dentine surrounding a greatly enlarged pulp
chamber. In radiographs, the teeth appear crumpled and
abnormally radiolucent or hazy, due to the paucity of dental hard
tissues. Histologically, the enamel thickness is highly irregular
and lacks well defined prismatic structure. The dentine which has
a disorganized tubular structure contains clefts and interglobular
dentine mixed with amorphous tissue. The surrounding follicular
tissue may contain small calcifications.

Segmental odontomaxillary
dysplasia;
Causes unilateral expansion of the alveolar process of the maxillary
in a child in the stage of the primary or mixed dentition.
The premolar and molar regions are the most affected.
Enlargement is due to fibrous enlargement of the gingiva and
expansion of the alveolar bone.
Eruption of teeth in the affected areas is delayed and theyre
hypoplastic to varying degrees often with enlargement of the pulps,
an irregular pulp/dentine interface and many pseudoinclusions and
pulp stones.
Radiographically, theres a zone of sclerosis with a coarse
trabecular pattern and loss of the cortex with missing and distorted
teeth.
Histologically, the sclerotic zone consists of woven bone trabeculae
in bland fibrous tissue and appears similar to the regressing stage
of fibrous dysplasia.

Epidermolysis bullosa.
Blistering dzz of skin and mucosae.
Dental;- fine or coarse pitting defects or thin and uneven enamel which may
also lack prismatic structure. Amelodentinal junction may be smooth.

INFECTION;
Congenital Syphilis
Incisors ( Hutchinsons incisors) are small, barrel-shaped & taper towards
the tip.

The incisal edge stms shows a crescentic notch or deep fissure which forms
before eruption and can be seen radiographically.
The anterior open bite is characteristic.
The 1st molars are dome-shaped ( Moons molars) or may have a rough
pitted occlusal surface with compressed nodules instead of cusps (mulberry
molars)
PATHOLOGY: due to infection of the dental follicle by Treponema pallidum
which causes chronic inflammation, fibrosis of the tooth sac, compression of
the developing tooth and distortion of the ameloblast layer. The micro-org
causes proliferation of the odontogenic epithelium which bulges into the
dentine papilla causing the characteristic central notch.

Disturbances in Eruption

A. Early Eruption
Show genetic and racial predilection.
Usually deciduous dentition.
Is of no clinical significance.
B. Delayed Eruption
Local Causes
1. Lack of space or overcrowding of teeth.
2. Presence of supplemental and supernumerary
teeth.
3. Over Retention of deciduous teeth.
4. Eruption cyst/Dentigerous cyst.
Systemic Causes
1. Rickets/Cretinism/Cherubism
2. Hereditary gingival fibromatosis.

Complications affecting
unerupted or partially erupted
teeth
1. Often no clinical effects.
2. Pericoronitis
3. Cyst formation.
4. Resorption of adjacent teeth.
5. Root resorption of the affected
tooth.
6. Hypercementosis of the affected
tooth.

Multisystem Disorders Affecting

Teeth
Ehlers-Danlos Syndrome (Floppy Joint).
Collagen disorder chxd by hypermobile joints, loose hyper extensible skin,
fragile oral mucosa and in type VIII, early onset periodontitis & TMJ
symptoms such as recurrent dislocation.

Main dental abnormalities are small teeth with short roots and multiple pulp
stones.

Gardeners Syndrome (Familial

Adenomatous Polyposis).
Chxd by multiple osteomas esp. of the jaws, colonic polys and skin tumors.
Dental abnormalities include; impacted teeth other than 3rd molars,
supernumerary or missing teeth and abnormal root formation.
Caused by mutation in the APC tumor suppressor gene.

Metabolic disturbances
General disturbances of metabolism affecting the development of the
teeth may be caused by the childhood fevers or severe infantile
gastroenteritis.
Measles causes hypoplasia of the enamel only on a restricted area.
Rickets can cause hypocalcification of the teeth in severe cases.
Early-onset idiopathic hypoparathyroidism causes teeth to be
hypoplastic with ridged enamel, short blunt roots and persistent open
apices.
Hypophosphatasia is a rare genetic disorder which has severe
skeletal effects as a result of failure of development of mature bone.
Failure of cementum formation may occur causing loosening or early
loss of teeth.

Drugs;
Tetracycline pigmentation
The drug is taken in by calcifying tissues and the band of
tetracycline-stained bone or tooth substance fluoresces bright yellow
under UV light.
The teeth only become stained only when the drug is given during
their development and it can cross the placenta to stain the
developing teeth of the fetus.
The drug is deposited along the incremental lines of the dentine and
to a lesser extent of the enamel.
The teeth are at 1st bright yellow but become a dirty brown to grey.
Cytotoxic chemotherapy
Teeth developing during treatment may have short roots, hypoplasia
of the crowns and enamel defects.
Microscopically, incremental lines may be more prominent
corresponding with the period of chemotherapy but in extreme cases,
tooth formation may be aborted.

Fluorosis
Distinctive features;
Geographical distribution, endemic in areas where water-borne fluoride
exceed about 2 parts per million.
Neighbouring communities with fluoride-free water do not suffer from the
disorder.
Only those who have lived in a high-fluoride area during dental
development show mottling.
Permanent teeth are affected; mottling of deciduous teeth is rare.
Mottled teeth are less susceptible to caries that normal teeth from lowfluoride areas.
A typical effect is paper-white enamel opacities.
Brown staining of these patches may be acquired after eruption.
Grading of mottled enamel;
Very mild- small paper-white areas involve less than 25% the surface.
Mild- opaque areas involve more that 50% of the surface.
Moderate- the whole of the enamel surface may be affected with paperwhite or brownish areas or both.
Severe- the enamel is grossly defective, opaque, pitted, stained brown
and brittle.

Pathology of fluorosis;
Fluoride combines to form calcium fluorapitite in place of part of the
hydroxyapatite. Damage to ameloblasts leading to defective matrix
formation is only seen in very high concentrations of fluorides. At
intermediate level (2-6ppm) the matrix is normal in structure and
quantity. The form of the tooth is unaffected but patches of
incomplete calcification beneath the surface layer are found. In high
conc. ( over 6ppm) the enamel is pitted and brittle with severe and
widespread staining.
Deciduous teeth are rarely mottled coz excess fluoride is taken up by
the maternal skeleton but at levels of over 8ppm like in India the teeth
can mottle.

Other acquired developmental

anomalies;
Dilaceration;
Trauma to a developing tooth can cause the root to form at an angle
to the normal axis of the tooth.
The sudden disturbance of odontogenesis may cause the formation
of highly irregular dentine in the coronal part of the chamber but the
angulated root may consist of normal tubular dentine.
Fetal alcohol syndrome;
Dental development may be delayed and there may be enamel
defects such as mottled opacities in the enamel near the incisal
margins.

Odontomas
They result from aberrant development of the dental lamina e.g.
exaggerated cingulum or extra roots or cusps.
Dens invaginatus;- an exaggeration of the process of formation of a
cingular pit. Dentine and enamel-forming tissue invaginate the whole
length of a tooth to appear radiographically as a tooth with a tooth.
Dens evaginatus;- an enamel and dentine covered spur* from the
occlusal surface of a premolar or molar.
Germinated teeth;- common in the maxillary incisor region. The pulp
chambers may be entirely separate, joined in the middle of the tooth,
or branched, with the pulp chambers of separated crowns sharing a
common root canal. The crowns maybe entirely separate or divided
only by a shallow groove. The roots may be single or double.
Enamel pearls;- formed by ameloblasts displaced below the
amelocemental junction and often form at the bifurcation of upper
permanent molars.

Attrition

Abrasion

Abrasion

Abrasion

Tooth wear
Attrition;Loss of tooth structure due to tooth-tooth contact during mastication and
occlusion.
Some degree of attrition is physiologic but when it begins to affect esthetic
and function, its considered pathologic.
Tooth destruction can be accelerated by:

Poor quality or absent enamel( enviromental or hereditary enamel

hypoplasia, dentinogenesis imperfecta, fluorosis)

Premature contacts (edge-edge occlusion)

Intraoral abrasives, erosion and grinding habits

Abrasion;Pathologic loss of tooth structure or restoration secondary to an external

agent.
Tooth brushing is the most common cause of abrasion.
Other causes; tooth picks, pencils, hair pins, pipe stems, chewing tobacco,
inappropriate use of dental floss and biting thread.

Demastication-tooth wear accelerated by chewing an abrasive substance

Internal and external resorption

Internal resorption is done by cells located in the dental pulp while external resorption is
done by cells in the PDL.
Internal resorption occurs after injury to pulpal tissues e.g. physical trauma or caries
related pulpitis. The resorption can continues so long as vital pulp remains and may
result in the communication of the pulp with the PDL.
I.R is divided into inflammatory resorption and replacement or metaplastic resorption.
Inflammatory. R, inflamed dentin is replaced by inflamed granulation tissue and usually
occurs on traumatized teeth that have recently undergone orthodontic or periodontal
therapy.
External resorption is more common of the two and its potential is inherent with the
periodontal tissue of the pt.