Cleft lip and palate

1. Dr. Simon
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2. Cont…
 Clefts of the lip and palate are the most common
serious congenital anomalies to affect the orofaciaI
region.
 The initial appearance of clefts may be grotesque.
 Because clefts are deformities that can be seen,
felt, and heard, they constitute a serious affliction to
those who have them
 EMBRYOLOGY
 During the fifth week, two fast-growing ridges, the lateral and medial
nasal swellings, surround the nasal vestige.
 The lateral swellings will form the alae of the nose; the medial swellings
will give rise to four areas:
 (1) the middle portion of the nose,
 (2) the middle portion of the upper lip,
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4.  (3) the middle portion of the maxilla,
 (4) the entire primary palate. Simultaneously the maxillary
swellings will approach the medial and lateral nasal swellings
but remain separated from them by well marked grooves
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5. During the next 2 weeks, the appearance of the
face changes considerably.
The maxillary swellings continue to grow in a
medial direction and compress the medial nasal
swellings toward the midline.
Subsequently these swellings simultaneously
merge with each other and with the maxillary
swellings laterally.
Hence the upper lip is formed by the two medial
nasal swellings and the two maxillary swellings.
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6.  The two medial swellings merge not only at the surface but also
at the deeper level.
 The structures formed by the two merged swellings are known
together as the intermaxillary segament which is comprised of
three components:
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7.  (1) a labial component, which forms the philtrum of the upper lip;
 (2) an upper jaw component, which carries the- four incisor teeth;
and
 (3) a palatal component, which forms the triangular primary
palate.
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8. Two shelf like outgrowths from the maxillary
.swellings form the secondary palate.
 These palatine shelves appear in the sixth week
of development and are directed obliquely
downward on either side of the tongue.
 In the seventh week, however, the palatine
shelves ascend to attain a horizontal position
above the tongue and fuse with each other,
thereby forming the secondary palate.
Anteriorly the shelves fuse with the triangular
primary palate, and the incisive foramen is formed
at this junction.
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11.  Clefts of the primary palate result from a failure of mesoderm to
penetrate into the grooves between the medial nasal and maxillary
processes, which prohibits their merging with one another.
 Clefts of the secondary palate are caused by a failure of the palatine
shelves to fuse with one another.
 The causes for this are Speculative and include failure of the tongue
to descend into the oral cavity.
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13.  Clefts of the lip, alveolus, hard and soft palate are the most common
congenital abnormalities of the orofacial structures.
 after clubfoot)
 They frequently occur as isolated deformities but can be associated
with other medical conditions particularly congenital heart disease.
 They are also an associated features in over 300 recognized
syndromes.
 All children born with a cleft lip and palate need careful pediatric
assessment to exclude other congenital abnormalities.
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14.  Incidence of cleft lip/palate is 1:600 live births.
 1:1000 live births for isolated cleft palate
 The highest incidence occurs in native Americans and occurs less
frequently in African Americans
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15.  Typical distribution of cleft types is:
• cleft lip alone 15 %
• cleft lip & palate 45 %
• isolated cleft palate 40 %
..Clefts of the lip occur more commonly in males than
in female
 Cleft lip/palate predominate in males
 Cleft palate alone appears more common in females
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16.  Cleft lip/palate and isolated cleft palate have a genetic
predisposition and environmental component
 Studies have been able to implicate genetics in only
20% to 30% of cleft lip or palate patients
 The majority of non syndromic clefts appear to be
caused by an interaction between the individual's
genes (i.e., genetic predisposition) and certain factors
In the environment that may or may not be specifically
identified
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17.  Environmental causes
1.Maternal epilepsy & Drugs
(steroids,diazepam,phenytoin)
2. Folic acid deficiency antenatally
 Most clefts of the lip and palate occurs as an
isolated deformity, the Pierre Robin sequence
remains the most common syndrome
 Isolated cleft palate is more commonly associated
with a syndrome
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18.  Clefts have been identified as a feature in over 300 syndromes,
most of which are rare. The most syndromes
 Strickler syndrome (ophthalmic and musculoskeletal
abnormalities)
 Van dour wood syndrome
 Down’s syndrome
 Apert’s syndrome
 Velocardio-facial syndrome, goldenhar syndrome & treacher-
collins syndromes
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19. It is characterized by:
1. Micrognathia
2. Glossoptosis
3. Cleft palate
 the overall impression is of bird face
Etiology:
•It does not appear to be an inherited
syndrome and it has been thought that
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20. • extreme flexion of the neck during fetal life may contribute to the
disorder
• the tongue is thrust upwards between the palatal shelves which
fail to fuse so causing the cleft palate to develop
 Recent work on gene function suggests that deficiencies of
TGFB3 is responsible
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21.  Respiratory obstruction due to poor control of the tongue & with
suckling require careful neonatal management
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22.  Cleft palate occurs when the roof of the mouth does not
completely close, leaving an opening that can extend into the
nasal cavity.
 The cleft may involve:
1. either side of the palate
2. extend from the front of the mouth (hard palate) to the throat
(soft palate).
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23.  The degree of the cleft lip can vary greatly, from mild (notching of
the lip) to severe (large opening from the lip up through the nose).
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28. The symptoms of these abnormalities are
visible during the first examination after birth.
prenatal ultrasounds can detect a cleft lip or
palate prior a child's birth.
By detecting the cleft abnormality during a
pregnancy, the expecting parents can have a
prenatal consultation with a plastic surgeon.
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29.  feeding difficulties
Feeding difficulties occur more with cleft palate
abnormalities. The infant may be unable to suck
properly because the roof of the mouth is not
formed completely.
 ear infections and hearing loss
Ear infections are often due to a dysfunction of the
tube that connects the middle ear and the throat.
Recurrent infections can then lead to hearing loss.
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30. speech and language delay
Due to the opening of the roof of the mouth
and the lip, muscle function may be
decreased, which can lead to a delay in
speech or abnormal speech. (hypernasal speech)
due to Velopharyngeal incompetence.
dental problems
As a result of the abnormalities, teeth may not
erupt normally and orthodontic treatment is
usually required
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31.  Airway :
• Major respiratory obstruction is uncommon
• Hypoxic episodes during sleep and feeding
are life threatening
• intermittent airway obstruction is more
frequent and managed by nursing the baby
prone
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32.  goal
a patient with normal appearance of lips, nose and face, whose
speech is normal and whose dentition and facial growth fall within
the range of normal development
 Aim is to restore normal anatomy
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33. Cleft lip repair is commonly performed
between 3-6 months of age (rule of ten 10g/dl,
10 pounds, 10 weeks
Cleft palate repair is frequently performed
between 12-18 months (after walk and before
talk) .
Grafting of alveolar cleft between 9-11 years
before eruption of max canine
Orthognathic surgery at 16--18 years after
completion of skeletal growth
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34.  The six advantages for early closure of palatal defects
are
 (1) better palatal and pharyngeal muscle development
once repaired,
 (2) ease of feeding,
 (3) better development of phonation skills
 (4) better auditory tube function,
 (5) better hygiene when the oral and nasal partition is
competent, and
 (6) improved psychologic state for parents and baby.
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35.  The disadvantages of closing palatal clefts early in life are also
several: The two most important are
 (1) surgical correction is more difficult in younger children with
small structures, and
 (2) scar formation resulting from the surgery causes maxillary
growth restriction.
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43.  Hearing: all children should undergo assessment before 12
months for sensorineural and conductive hearing loss
 Speech: initial assessment at 18 months and should be
repeated regularly
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44. Common speech problems
1. Nasal quality of speech
2. Articulation problems
Management:
1. Speech and language therapy
2. Palatoplasty
3. Speech training device
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45.  Dental:
1. Delayed development
2. Delayed eruption
3. Morphological abnormalities
4. Number of teeth could be increased or decreased
 Most commonly seen in maxillary lateral incisor tooth.
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46.  Orthodontic management: because of malocclusion.
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47. Thank
you
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