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Cleft Lip & Palate

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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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(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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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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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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(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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Two shelflike 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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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.

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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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Normal Lip & Palate

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Cleft Lip & Palate

Clefts of the lip, alveolus, hard and soft palate are the most common congenital abnormalities of the orofacial structures.

They frequently occur as isolated deformities but can be associated with other medical conditions particularly congenital heart disease.

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Cont….

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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Incidence

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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Cont….

Typical distribution of cleft types is:

• cleft lip alone 15 %

• cleft lip & palate 45 %

• isolated cleft palate 40 % Cleft lip/palate predominate in males Cleft palate alone appears more

common in females

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Etiology

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 nonsyndromic 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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Cont…

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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Associated Syndromes

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)

Down’s syndrome Apert’s syndrome

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Pierre Robin’s Syndrome

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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Cont…

• 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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Cont…

Respiratory obstruction due to poor control of the tongue & with suckling require careful neonatal management

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Cleft palate 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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Cleft lip

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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Classification of cleft lip

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The characteristics of cleft lip and cleft palate 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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Bilateral cleft lip

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complications

feeding difficultiesFeeding 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 lossEar 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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Cont…

speech and language delayDue 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 problemsAs a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required

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Cont…

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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Principles of Cleft Surgery

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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Time for surgery

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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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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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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Before and after

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After cleft palate repair

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Secondary Management

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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Cont…

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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Dental: 1. Delayed development2. Delayed eruption3. Morphological abnormalities4. Number of teeth could be increased or

decreased Most commonly seen in maxillary

lateral incisor tooth.

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Cont…

Orthodontic management: because of malocclusion.

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Thank you