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GOOD MORNING

CLEFT LIP & CLEFT


PALATE

CONTENTS

INTRODUCTION
EMBRYOLOGY
ETIOLOGY
EPIDEMIOLOGY
CLASSIFICATION
CLINICAL FEATURES
MANAGEMENT

INTRODUCTION
Cleft lip and palate are congenital
deformities that are manifested at birth.
The birth of a child with this deformity is
a shocking event to the parents.

Cleft lip results in a uni or bilateral


opening in the upper lip between the
mouth and the nose.
Cleft palate is characterized by an
opening in the roof of the mouth

Development of lip and palate


Development of the Face
5 facial primordia

Frontonasal prominence

Paired maxillary prominences

Paired mandibular prominences

PRIMARY PALATE FORMATION:

2 Median nasal process


merge at the deeper levels to
form the intermaxillary
segment.

IMS has
Labial component- philtrum
Maxilla component-alveolus
Palatal component primary
palate

SECONDARY PALATE FORMATION:

The maxillary processes


give rise to two palatal
shelves.(6th week)

Grow Inferiorly in
vertical direction along
both sides of the tongue.

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Tongue contracts, moves


anteriorly & inferiorly
(8th week).
Palatal shelves flip in
superior direction
They elongate & move
medially fusing to form
secondary palate, soft
palate & uvula

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COMPLETION OF PALATE:

The secondary palate &


posterior portion of the
primary palate fuses
together forming the final
palate. (12th week).

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Cleft lip when one or both max processess


do not fuse with MNP
Oblique facial cleft non fusion of the max
process & LNP
Lateral facial cleft when max process &
mand process donot fuse
Cleft palate failure of palatal shelves to
fuse with each other & with pri palate

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Mechanism of cleft
formation

Deficient mesoderm

Insufficient migration potential

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Anterior Cleft Anomalies

Cleft lip with or without cleft of alveolar


part of the maxilla
Complete ant cleft separates ant & post
part of palate

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Cleft lip

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CL with alveolus

deficiency of
mesenchyme in the
maxillary
prominanace and the
intermaxillary
segment

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Posterior Cleft Anomalies

Clefts extending
through both soft and
hard (bony) palate
post to the incisive
fossa
Caused by interference
with
Shelf elevation
Attachment and
fusion
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Complete Cleft Palate

Maximum clefting
anomaly
Cause : failure of
mesenchymal masses
in the lateral palatine
processes to meet and
fuse with
mesenchyme in the
primary palate and
with each other and
with nasal septum

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Etiology
Hereditary
Nutritional disturbance
Stress
Increased maternal age
Defective vascular supply
Infections Rubella viruses
Mechanical disturbance size of tongue
Radiation
Maternal smoking
Lack of inherent developmental forces
Chronic alcoholism & certain drugs
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EPIDEMIOLOGY

App. 45% of cases are CL + CP


30% isolated CP
20% isolated CL

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CL CP
Whites 1 in 700 to 1000 births
Asians > 1.5 times
Blacks 0.4 per 1000
Americans 3.6 per 1000 births

Isolated CP 0.4 per 1000 births

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classification

Morphologic Davis &Ritchie


Veau lip, palate

Embryologic Fogh & Andersons


International
confederation
of plastic and reconstructive surgery

Symbolic kernahn & Stark

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Davis and Ritchie classification(1922)


Based on location of cleft relative to alveolar
process
Group I: pre alveolar clefts only lip
unilateral, median, or bilateral
Group II: Postalveolar clefts
CP alone, soft palate alone, soft palate and
hard palate, or submucous cleft
Group III : Alveolar clefts palate, alveolar
ridge & lip
Unilateral, median, or bilateral
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Modification of Davis & Ritchie


classification
Prof. Balakrishnan
Group I : cleft lip alone
complete, incomplete, unilateral, bilateral
Group Ia : cleft lip and alveolus
unilateral , bilateral

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Veaus classification of cleft lip

Class I a unilateral notching of vermilion


not extending into the lip
Class II a unilateral notching of
vermilion with cleft extending into the lip
but not including the floor of nose

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Class III a unilateral cleft of vermilion


extending into the floor of the nose
Class IV any bilateral clefting of the lip
either complete or incomplete.

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Veau classification for cleft palate


Class I

: clefts of SP only

Class II : SP, HP extending upto


incisive foramen
Class III : complete unilateral
clefts SP, HP & alveolus
Class IV : Complete bilateral
clefts SP, HP& alveolus

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Fogh and Andersons


Group I cleft lip (single or double)
Group II cleft lip & cleft palate
Group III cleft palate

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International confederation of plastic and


reconstructive surgery classification

Group I : Defects of the lip or alveolus


Group II : Clefts of the secondary palate (HP,
SP ,both)
Group III : Any combination of clefts involving
the pri & sec palates

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Kernahan and starks symbolic classification

Upper arm alveolus hard


palate upto incisive
foramen
Lower arm hard and soft
palate

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Millards modification of the Kernahans stripped y


classification

Elsahys modification of the


kernahans stripped y classification

American cleft palate associations


classification-1962

1.clefts of pre palate


2.clefts of palate
3. cleft of hard and soft palates
4.clefts of pre palate and palate

Iowa Classification
Group I

Group II

Clefts of lip only

Clefts of palate only

Group III

Group IV

Clefts of lip,
alveolus, palate

Clefts of lip and


alveolus (primary
cleft palate and
lip)

Group V
Miscellaneous
Clinical Aspects of Cleft Lip/Palate Reconstruction

Clinical Features

CL with or without palate - common in males


Isolated cleft palate - common in females
Unilat 80% of clefts (70% on left)
Bilat 20%

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Clefts - Nonsyndromic
Syndromic forms

Syndromic - patient has more than one


malformation involving more than one
developmental field

Nonsyndromic - affected individual has no


other physical or developmental anomalies

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Syndromes associated (5%)


Autosomal dominant

Vander woude syndrome


EEC (Ectrodactyly, ectodermal dysplasia, cleft)
Autosomal Recessive

Meckel syndrome( sloping forehead, poly


dactyly, polycystic kidney)

Orofacial digital syndrome type II ( poly dactyly,


brachydactyly, craino facial anomalies)

Roberts syndrome

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X linked
Oro facial digital syndrome type I( syndactyly,
craniofacial anomalies, mental retardation)

Isolated cleft palate with ankyloglossia


Chromosomal

Trisomy 13 ( mental retardation, polydactyly,


abnormalities of heart & genetalia)

Trisomy 18(micrognathia, low set ears,short


digits)
Non mendelian

pierre robin syndrome

clefting with congenital heart disease


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Cleft lip

Line of cleft - always


starts on the lateral
part of the upper lip
and continues
through the philtrum
to the alveolus
between the lateral
incisors and canines.

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Complete
extends upwards
into the nostril ,
Incomplete cleft
lip does not
involve the nostril

42

Cleft lip - Unilateral


Bilateral

Most severe form bilat cleft lip and


alveolus

separate the philtrum


of the upper lip and
premaxilla from the
rest of the maxillary
arch
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CL with palate

Cleft lip continues from


the incisive foramen
further through the
palatal suture in the
middle of the palate

Unilateral
Bilateral

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Isolated cleft palate

Clefting post. to incisive


foramen - cleft of secondary
palate

Cleft uvula - minimal form


More common than CP
Cleft of SP - severe form
Complete cleft palate - cleft of
the HP, SP, uvula

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Submucosal palatal cleft


( Roux 1825)
Triad

Bifid uvula

Notch - along post


margin of HP

Muscular diastasis of
SP with an intact
mucosa

Bluish midline
discoloration
Identified by palpation

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Clinical appearance

Cleft of lip and alveolus


associated with the deformities of
the nose.
The ala of the cleft side is hypo
plastic, flared and at a lower level
than on the non cleft side.
The nasal septum is deviated to the
non cleft side.
The maxilla on the cleft side is
hypoplastic, both vertically and
horizontally.
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Cleft of the palate


considerable underdevelopment of
mandible
tongue prolapsing into the oropharynx
and interfering with breathing in new
born child

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Median maxillary anterior


alveolar cleft

Common
Precocious limitation of the growth on
either side of the midline at the primary
palate or
Failure to fuse
No clinical manifestations
Syndrome median cleft face (Miller &
co)
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Problems associated with CLP


Classified into
1. Dental
2. Esthetic
3. Speech & hearing
4. Psychologic
Dental congenitally missing teeth
Supernumerary teeth
Microdontia, macrodontia
Spacing, crowding
Deepbite, protruding premaxilla

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Aesthetics - results in facial disfigurement


Hearing & speech
CLP associated with disorders of the
middle ear & affect hearing
Hearing problems difficulty in speech
Psychological
Pts are under a lot of psychological stress
due to facial app

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Insufficient suction to pull milk


Excessive air intake during feeding
Choking
Nasal regurgitation
Excessive time required for nourishment
Respiratory tract infections
Ear infection

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Treatment

Challenging, lengthy, costly, and


dependent on the skills and experience of
a medical team
Pediatrician, pedodontist, orthodontist,
prostho, oral sur, plastic, ENT, social
worker, genetist, psychiatrist & speech
therapist.
Severity of defect
Closure of the CL - 3 mon of life followed
by repair of palate
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Early dental management


1. Intraoral maxillary obturator therapy
2. Appliance for pre maxillary retraction
3. Management of dental problems if any

Orthognathic procedures to improve func


& cosmetic app

Genetic counselling for pt & family

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Pediatricians follow a rule of 'three 10s


(i.e. 10 Ib, 10 mg/dL of Hb & 10 weeks)

Cleft palate repair


Primary bone grafting (< 2yrs)
Early secondary bone grafting (2- 4 yrs)
Secondary bone grafting (6 15 yrs)
Late secondary bone grafting (in adults)

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conclusion
CLP are the most common
developmental disturbances and are
extremely disfiguring and affect to a
considerable extent.
our understanding of the biology
of clefting is also increasing at a dramatic
rate, and we will soon be at the time
when we can take measures to prevent
the clefts.

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REFERENCES

Oral & Maxillofacial Pathology-Second Edition


Neville, Damm, Allen and Bouquot

SHAFERS TEXT BOOK OF ORALPATHOLOGY 5TH


Edition, R Rajendran, B Sivapathasundharam.

Text book of pediatric dentistry, S.G Damle, 3rd


edition

57

Oral Anatomy,Histology and Embryology,


B.K.B.Berkovitz, G.R.Holland, B.J.Moxham, 3 rd
Edition.

The developing human. Moore persaud. 7 th edition.

A New Approach to Classify Cleft Lip and Palate.


M.R. ORTIZ-POSADAS, L. VEGA-ALVARADO, J. MAYABEHAR. Cleft PalateCraniofacial Journal, November
2001, Vol. 38 No. 6

Biological Mechanisms in Palatogenesis and Cleft


Palate.L. Meng,Z. Bian, R. Torensma, and J.W. Von
den Hoff. J Dent Res 88(1):22-33, January, 2009
58

Syndromes associated with cleft lip and palate


Chromosomal

Trisomy 13

Trisomy 18

Velocardiofacial syndrome (22q11 deletion)


Non-Mendelian

Pierre Robin sequence

CHARGE association

Goldenhar syndrome
(cleft journal-2005,vol 1)

Mendelian disorders

Ectrodactyly-ectodermal dysplasia-clefting
syndrome (AD)
Gorlin syndrome (AD)
Oto-palato-digital syndrome (XL)
Oral-facial-digital syndrome (XL)
Smith-Lemli-Opitz syndrome (AR)
Stickler syndrome (AD)
Treacher Collins syndrome (AD)
Van der Woude syndrome (AD)

Unknown
de Lange syndrome
Kabuki syndrome
Teratogenic
Fetal alcohol syndrome
Fetal phenytoin syndrome
Fetal valproate syndrome

THANK
YOU
62

Lahshal classification-Okreins-1987

LAH-R

Effect of clefts on teeth

rotation and crowding of the maxillary anterior


teeth
congenitally missing lateral and central incisors
extremely high incidence of enamel hypoplasia in
the incisors of both dentitions
premolars and canines on the cleft side of the
upper jaw erupt later than the corresponding
teeth on the noncleft side of the upper jaw.
the crown-to-root formation - asymmetric

AJO, Vol 1986 Jul (11 - 18) Tooth formation in children with cleft lip/palate

The teeth of the cleft group were


significantly smaller
The normal sex differences in tooth
dimensions were to some extent reversed
in the cleft palate subjects and several of
the dimensions were significantly larger
for the females than for the males.

Cleft palate - signs

Median part of the palatal vault is absent


Large defect in the roof of the palate, with
a direct opening into the nasal cavity
lateral displacement of the maxillary
segments due to a nonfunctioning soft palate
Palatal fibromucosa is reduced
vomer is reduced

BCLP results in an anteriorly progressive


constriction of the upper dental arch
The BCLP group has a significantly longer
maxillary dental arch, which is attributed to
the premaxillary anterior projection.

AJO-DO), Volume 1998 Aug- Influence of cleft type on mandibular growth,

Transition of palatal shelves


from vertical to horizontal

Biochemical transformations in the physical


consistency of the connective tissue matrix of the
shelves
Variation in vasculature and blood flow to these
structures
A sudden increase in their tissue resilience
Rapid differential mitotic growth
An intrinsic shelf elevating force generated by
accumulation and hydration of hyaluronic acid. The
alignment of mesenchymal cells within the palatal
shelves may serve to direct the elevating forces
Muscular movements
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DOUBLE LIP

Is an anomaly
characterized by a
horizontal fold of
redundant mucosal
tissue located on the
inner aspect of upp lip
Congenital or acquired
Congenital arise
during 2nd or 3rd month
of gestation
Acquired component
of ascher syndrome or
trauma or oral habits

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C/F

1.
2.
3.

Unnoticeable lips at
rest
Visible pt smiles or
lips tensed
Ascher syndrome
triad of
Doublelip
Blepharochalasis
Non toxic thyroid
enlargement
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Cleft of mandible

Rare
Appear along the midline
Because of pressure from the adjacent
enlarged heart, which begins beating
before the mandibles midline fusion
The early constriction did not disappear
and later resulted in a separation of the
mandibles halves.
Orofacial digital syndrome
71

Drug that causing CLP

Phenytoin

Bacterial infection causing CLP


syphilis

72

Nerve supply to Philtrum of the


upperlip
2 differing accounts
1) Maxillary processes overgrow the medial
nasal process to meet in the midline and
thus contribute all the tissue for the
upper lip. This is based upon an
appreciation of the innervation of the
fully formed upper lip ( i.e. infraorbital
branch of the maxillary division of the
trigeminal nerve) the max process being
supplied by the max n & the the
frontonasal process by opthalmicN

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2) alternatively it has been suggested that the


maxillary process meet the medial nasal
processes without such overgrowth, the middle
third of the upper lip being derived from the
frontonasal process
While histological evidence favours the latter
explanation, at present little is known about the
behavior of the mesenchyme of the facial
processes after the initial fusion , there by not
excluding the possibility of subsequent
migration of tissue derived from the maxillary
processes towards the midline.
74

Abnormalities reported in
patients with CLP

Congenital heart disease


Polydactylisim
Syndactylism
Hydrocephalus
Microcephalous
Club foot

75

Supernumerary ear
Hypospadias
Spinabifida Hypertelorism
Mental deficiency

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Definitions

Congenital disease : is one which is present at


or before birth but is not necessarily inherited
Hereditary condition pertain to a condition or
a disease transmitted from parent to offspring
which may be apparent at birth or may
manifest after a no of years after birth
Familial :pertaining to a characteristic,
condition or disease that is present in some
families and not others or that occurs in more
family members than would be expected by
chance. It is usually but not always hereditary
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Acquired diseases originate after birth and


caused not by hereditary or developmental
factors but by a reaction to environmental
influences out side of the organisms.
syndrome: is a complex of signs and
symptoms resulting from a common cause
or appearing in combination to present a
clinical picture of a disease or inherited
abnormality.
Anomalad a group of morphological
defects that stem from a single localized
structural anomaly which resulted in a
cascade of consequent defects
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