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CLEFT LIP

AND PALATE

It is congenital abnormal space in upper lip,

alveolus and palate.

INCIDENCE
Common in south asians 1 in 500
Less frequent in africans 1 in 200
Prevalance in pakistan 1 in 523
Boys are more affected than girls 3:2
75% are unilateral
Left side is more affected than right

side

EMBRYOLOGY

DEVELOPMENT
OF PALATE

CLASSIFICATION
Generally classified as
Isolated cleft of lip/palate
Combined CL+CP
Unilateral or bilateral
complete (if it crosses nasal philtrum) or
incomplete (if it does not cross philtrum)

Veau classification
Kernehans stripped Y
Millard modification of kernehan stripped Y
Kriens LAHSHAL

VEAU CLASSIFICATION
GROUP 1
Cleft of the soft palate

GROUP 2
Cleft of hard and soft palate up to

incisive foramen
GROUP 3
Complete unilateral cleft lip and palate
GROUP 4
Complete bilateral cleft lip and palate

LAHSHAL
CLASSIFICATION
L=lip (right)
A=alveolus (right)
Capital letter = complete
H=hard palate (right)cleft
Lower case letter =
S=soft palate
incomplete cleft
(median)
. or , = normal

H=hard palate (left)


A=alveolus (left)
L=lip (left)

ETIOLOGY
Genetics, inherited from one or both

parents
Environmental factors
Drugs, phenytoin, retinoids
Infection, rubella
Smoking
Alcohol
Hypoxia
Vitamin A defeciency
Folic acid defeciency

DIGANOSIS
Cleft can be diagnosed prenatally
Can be diagnosed by using 3-D imaging in

ultrasonography and genetic tests for


screening of birth defects.
In second trimester
Premaxillary protrusion is an important
clue to the presence of cleft lip and palate.

ADVANTAGE
Prenatal education
Psychological preperation of parents
Helps to asses and investigate other

associated anomalies

NON SYNDROMIC CLEFT


PIERRE ROBIN

SEQUENCE
Cleft palate
Micrognathia
Glossoptosis (retrarction of

tongue)

SYNDROMIC CLEFT
STICKLERS SYNDROME
Flttened face
PRS
Ear infections
Occular HTN
Retinal detachment
VELOCARDIOFACIAL

SYNDROME
Cardiac abnormality
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia/hypoparathyroidism

VAN-DER-WOUDES

SYNDROME
Genetic disorder
Combination of lower lip pits+CL

with or without cleft palate and


CP alone.
Hypodontia
Congenital heart disease
Cerebral abnormalities
Syndactyly

PROBLEMS
Feeding
Breathing
Speech problems
Hearing
Esthetic problems
Nasal deformity
Dental problems

FEEDING
Infant cannot suck
May cause milk to be

accidently taken in nasal


cavity
Unable to create negative
pressure
Frequent regurgitation
Upper respiratory tract
infections

Mead-Johnson
cleft palate nurser

Haberman feeder

Pigeon nipple

EAR, NOSE AND THROAT


EVALUATION
Audiology assessment is recommended soon

after birth
High frequency of otitis media
Myringotomy is performed to relese otitis
media fffusion

DENTAL PROBLEMS

Missing teeth
Hypodontia
Hyperdontia
Natal teeth
Microdontia/macrodontia
Enamel hypoplasia
Poor PDL support
Early loss of teeth
Class III tendency
Spacing
Crowding
Xbite

NASAL DEFORMITY
Poor nasal shape
Scar mark due to surgery

ESTHETIC PROBLEMS
Poor dental alignment
Poor smile
Poor lip function during speech

EAR PROBLEM
Middle ear infection
Hearing loss

SPEECH PROBLEMS
Velopharyngeal insufficiency (VPI)
Poor pronounciation of bilabial,

labiodental, linguoalveolar sounds

MANAGEMENT

TEAM APPROACH
Genetic scientist
Peditrician
ENT surgeon
Paedodontist
Oral surgeon
Prosthodontist
Plastic surgeon
Speech therapist
Pshyciatrist
Social worker

TIMING OF TREATMENT IN CLEFT


PATIENT

PRENATAL
Diagnosis and parental counseling
0-6 MONTHS
General assessment for associated anomalies
ENT evaluation- breathing, feeding, swallowing and
hearing
Pre surgical orthopedics (0-3 months)
Primary lip repair (3-4 months)
6 MONTHS-2 YEARS
Speech and oral sensory motor assessment
Grommets/ear tubes (as needed)
Primary palate repair (9-12 months)
PRESCHOOL: 3-5 YEARS
Dental care
Speech assessment and therapy

CHILDHOOD: 6-12 YEARS


Correction of velo-pharyngeal dysfunction (as needed)
Orthodontic treatment-phase I
Alveolar cleft repair (8-11 years)

ADOLESCENCE: 13-18 YEARS


Orthodontic treatment-phase II
Orthognathic surgery (if needed)-14-16 years (female), 16-18

years (male)
Revision chielo-rhinoplasty
Replacement of missing teeth (as needed)

PRESURGICAL
ORTHOPEDICS
Gentle application of pre surgical orthopedic force

allow the molding of alveolar segments and the


nostrils within 0-3 months of birth and provide
benefit in correction of the nasal deformity in
childern with bilateral CLP and wide unilateral
clefts.
Nasoalveolar molding
increases the surface area of nasal mucosal
lining
Helps to elongate columella
Make columella upright
Allows suturing of alar cartilages without
tension

Two categories of appliances are used


Passive
active.

ACTIVE APPLIANCE
Active

appliances
They are fixed

intra orally
apply traction
through
mechanical means
such as elastic
chains, screws,
and plates.

Passive

appliances

maintain the distance between

the 2 maxillary segments while


external force is applied to the
primarily to reposition it
posteriorly.
The naso alveolar molding
device commonly used passive
device
it consists of an intraoral
acrylic plate held in place with
extraoral elastics and tape.
In later phases of treatment,
wire are added to apply force
to the vestibule of the nares,
lengthening the columella

OTHER METHODS
External force can be

applied by
external taping of the lip
a head cap with elastic

straps
or a surgical lip adhesion.

CLEFT LIP REPAIR


Goal is to reconstruct a functional lip, with

normal appearnce
Timining is between 3-6 months
A RULE OF TEN is followed to ensure the
infant is fit for surgery.
Infant should be at least 10 weeks of age
Weigh 10lbs
Haemoglobin level 10g/100ml

UNILATERAL CLEFT LIP


ANATOMY
Asymmetric deformity of lip and nose
Nasal deformity severity of cleft
Due to abnormal position of
orbicularis oris muscle because they
terminate medially beneath the base
of clumella and laterally beneath alar
base and periosreum of piriform rim
Distortion of nose
Deflection of ANS
Deflect nasal septum

TECHNIQUE
Blair, Le Mesurier, Tennison and Randall contributed

to evolution of cleft lip repair.


Popular is Millard Technique who described
ROTATION-ADVANCEMENT concept.
Medial flap is rotated downward to achieve length
Lateral is advanced.

Can be modified at the time of operation


ADVANTAGE:
Suture lies on the recreated philtrum
Incision allows easy access for primary rhinoplasty to

reposition nasal septum, lateral cartilage and alar base.

PRINCIPLES
1. ROTATION OR LENGTHENING OF SHORTENED
2.
3.
4.
5.
6.

VERTICAL HEIGHT OF LIP


ADVANCEMENT OF FLAP FROM LABTERAL TO
MEDIAL
RETAINING CUPIDS BOW AND CREATE
PHILTRAL COLUMN
MUSCLE RECONSTRUCTION
RESTORATION OF ALVEOLAR CONTINUITY
PRIMARY REPAIR OF DISTORTED NASAL
ANATOMY

BILATERAL CLEFT LIP

ANATOMY

Difficult to repair
Absence of muscle in

prolabial segment
Lack of philtrum dimple
Lack of Philtral columns
Absent Roll margins
Premaxillary segment
protrudes making tension
free margins of muscle
repair difficult.
Columella short
Wide nasal tip
Flare alar base

PRINCIPLES
ESTABLISH SYMMETRY
DESIGNIGNING A PROLABIAL FLAP OF

APPROPRIATE WIDTH
FORMING THE CUPIDS BOW AND MEDIAN
TUBERCLE FROM LATERAL LIP ELEMENTS
ESTABLISH MUSCLE CONTINUITY
RECONSTRUCTION OF NASAL TIP AND
COLUMELLA
REPOSITIONING THE ALAR BASE
MANAGEMENT OF PREMAXILLA

REPAIR OF CLEFT PALATE


GOALS:
To normalize speech
Align the aberrant attachment of palatal

muscles
Seal communication between the oral and nasal
cavities

TIMING OF REPAIR:
Historically cleft palate repair of the hard

palate was delayed to minimize


impairment of maxillofacial growth
Nowadays, literature shows that speech
outcomes are better when soft and hard
palate repair is completed before speech
development
It is around 9-12 months
Most prefer to repair one stage before 12
months of age
Some prefer two-stage repair soft palate
repair at 3-6 months and hard palate at
12-15 months

ANATOMY:
Minor submucous cleft:
affect soft palate only
Bifid uvula
Notching of posterior nasal
spine
Translucency in mid palatine
region of soft palate due to
lack of muscle
Abnormal orientation and
attachment of the muscles,
primarily
Levator palatini
Tensor palatine
Musculus uvula
Muscle bundle insert
longitudinally into the posterior

PRINCIPLE
Main principle is to detach and retropose the

abnormal insertion of levator palatini and join


the in midline
In hard palate reflect mucoperiosteal flap
based on greater palatine arteries

TWO-FLAP PALATOPLASTY
Commonly used
For complete unilateral and bilateral cleft

of palate
Edges of cleft are incised from alveolus to
base of uvula
Bilateral full thickness muco periosteal flap
is raised
Levator palatini muscles are released and
dissected
Reposition horizontally and sutured
Bilateral releasing incision are made to

VON LANGENBECK REPAIR


For secondary

palate cleft
Bilateral releasing
incisions are made
muco periosteum is
elevated
Complete stripping
of nasal mucosa
Then close nasal
layer, muscle and
oral mucosa in
layers

FURLOW Z-PLASTY REPAIR


Uses two reversed

Z-plasties of oral
and nasal mucosa
to repair the cleft
ADVANTAGE:
Restore the
normal anatomic
position of levator
palatini in midline
Increases soft
palate length

KILNER (V-Y
PUSHBACK) REPAIR
was the commonest technique
V-Y procedure is performed
mucoperiosteal flap and the soft palate are retroposed

and the palate is lengthened.


it leaves an extensive raw area anteriorly and laterally
along the alveolar margin with exposed bare
membranous bone
heals with secondary intention.
This causes shortening of the palate and
velopharyngeal incompetence.
alveolar arch deformity and
dental malalignment.

SPEECH DYSFUNCTION
Resonance
Articulation
Phonation
Language delay

CAUSE
Velo pharyngael insufficiency
Oro nasal fistula
Weak lip pressure
Abnormal tongue pressure
Malposed teeth
Abnormal jaw relationship
Neuromuscular dysfunction
Hearing loss

VELOPHARYNGEAL
INSUFFICIENCY(VPI)
It is as a failure of the separation

between nose and mouth, because of


an anatomical dysfunction of thesoft
palate, the lateral or posterior wall of
the pharynx.
leads to functional problems with
speech (hypernasality), eating and
breathing

DIAGNOSIS OF VPI
SPEECH ANALYSIS:
The main symptom is hypernasality of the voice. The

patient is unable to create normal resonance because of


nasal air emission.
NASOMETRY:
It is a test which calculates a ratio between the nasal
and oral sound emissions. Ratios are compared with a
normal ratio. Preoperative ratios are compared with
postoperative ratios.
NASOENDOSCOPY:
provides an overview of the anatomy of the velopharynx
during phonation
Cannot be used in kids
With nasal obstruction
VIDEOFLUROSCOPY:
a radiographic technique, mostly to demonstrate the
lateral and posterior wall of the pharynx.

TREATMENT (SURGICAL)

FLAP PHARYNGOPLASTY:
When the pharyngeal flap is used, a flap of

the posterior wall is attached to the


posterior border of thesoft palate.
The flap consists of mucosa and the
superior pharyngeal constrictor muscle.
The muscle stays attached to the
pharyngeal wall at the upper side (superior
flap) or at the lower side (inferior flap).
The function of the muscle is to obstruct
the pharyngeal port at the moment that
the pharyngeal lateral walls move towards
each other

NON SURGICAL
used for nonsurgical closure.
There are two types of prostheses.
speech bulb: The speech bulb is

an acrylic body that can be


placed in the velopharyngeal port
and can achieve obstruction
palatal lift prosthesis: is
comparable with the speech
bulb, but with a metal skeleton
attached to the acrylic body to
obstruct the velopharyngeal port.

ALVEOLAR CLEFT
REPAIR
Goal is to maintain bony continuity of alveolar

ridge
Provide bone support for the teeth adjacent ti
cleft
Seal nasal and oral cavity
Facilitate eruption and orthodontic movement
of teeth mostly canine
Maintain healthy periodontium
Provide alar base support
Improve nasal symmetry

TIMING OF REPAIR
Secondary bone grafting at the age of 7-11

years is done
This coincides with development of one half to
two thirds of root of canine or lateral incisor if
present.
Graft material is corticocancellous bone from
iliac crest, rib, symphysis, calvarium and tibia.
Before grafting of bone maxillary expansion
should be completed to provide acess to the
defect.

PRINCIPLES
Closure of nasal floor
Removal of any supernumerary teeth in the

cleft
Fill with bone
Approximate oral mucosa
Orthodontic movement can be initiated in 3-4
months if required
Palatal retainer should be placed after surgery
to prevent loss of maxillary width

ORTHODONTIC
MANAGEMENT
2-10 weeks:
Pre surgical orthopedics

6-10 years:
Phase I orthodontics:

Maxillary expansion for alveolar bone graft


Maxillary protraction with face mask if required

10-14 years:
Maintain maxillary expansion and alignment of teeth
Monitor facial growth and eruption of teeth

14-18 years:
Phase II orthodontics:

Orthodontic treatment with full fixed appliance


Prepare orthognathic surgery if required
Extract teeth if mandibular arch is crowded
Decide to replace absent teeth

REPLACEMENT OF TEETH
Most common teeth is maxillary lateral incisor
Managed by closing the space and substitute the adjacent

canine in its position


Or by opening the space and replace it with a fixed or
removal prosthesis
End osseous implant can be placed when adequate bone
space and bone quantity is present
Alveolar ridge augmentation with cortical onlay can be
performed 4 months before placement of implant
Removable prosthesis can be given if there is
Loss of pre maxilla
Severe deficiency of bone height
Deficient bone height
Scarring of overlying soft tissue
Lack of lip support

CORRECTION OF
MAXILLARY
HYPOPLASIA
Maxillary hypoplasia is due to restriction of

midfacial growth
It can be attributed partly due to
congenital malformation
Scar contracture following primary palate

surgery

Can be done by:


Orthodontic management with dental
compensation
Surgical maxillary advancement

SECONDARY LIP AND


NOSE DEFORMITY
Final phase for the cleft patient
Involve correction of residual lip and nasal

deformity
Lip and nose revision is done during late
adolescence after correction of maxillary
hypoplasia
Done to achieve balanced facial esthetics

SECONDARY LIP
DEFORMITY
Residual deformity in unilateral cleft

include
Mismatch of cutaneous vermillion border
realignment and small triangular flap
or Z-plasty procedure
Vertical shortening of lip full thickness
revision of skin, muscle and mucosa
Hypertrophic scar

Residual deformity in bilateral cleft

include
Tight upper lip Abbe flap
Poor philtral column
Poor cupids bow
Poor vermillion show in midline
Scar
Shallow labial sulcus perform Z-plasty

CLEFT NASAL DEFORMITY


Correction is difficult because
Soft tissue distortion
Scarring
Nasal stenosis
Uneven bony foundation
Done by open rhinoplasty under direct vision

An esthetic smile and normal speech are

two important requirements to make an


identity and self esteem
Ability to restore lip and palate function
sre key to management of cleft patient

N
A
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