Cleft palate

Submucous cleft palate occurs when the palate has mucosal continuity but the underlying levator palatini muscle is discontinuous across the midline and longitudinally oriented, similar to the muscle anatomy in overt clefts of the palate ( Fig. 12.1 ) .

Calnan's classic triad is diagnostic of this condition:

• A midline clear zone (zona pellucida).

• A bifid uvula.

• A palpable notch in the posterior hard palate.

• With contraction of velar musculature, a distinct midline muscle diastasis may be seen (see Fig. 12.1 ).

The significance of a submucous cleft may be difficult to assess clinically; the child with submucous cleft palate is often undiagnosed in infancy.

It has been reported that 45–55% of patients with isolated submucous cleft palate are symptomatic with regard to speech, serous otitis media, or hearing loss.

However, an infant identified with submucous cleft palate need not routinely undergo repair because a significant number of individuals with submucous cleft palate will not develop velopharyngeal insufficiency.

Rather, these patients should be closely monitored with serial speech evaluations and audiometric surveillance.

Patients who present with velopharyngeal insufficiency and submucous cleft palate on examination require full evaluation, including speech evaluation and endoscopy.

Even in the absence of obvious findings on clinical examination, anatomic abnormalities are found in most patients (>90%) at the time of surgery.

Corrective surgical technique for submucous cleft palate is focused on anatomic correction of the velar muscle diastasis.

Although pharyngeal flaps and sphincter pharyngoplasty have been proposed as primary means of treatment, most surgeons focus on repair of the abnormal levator muscle position.

The Furlow double-opposing Z-plasty (see below) is an ideal procedure for these patients because there is no width discrepancy to overcome.

Pierre Robin described the triad of micrognathia, glossoptosis, and respiratory distress.

60–90% of patients diagnosed with Pierre Robin sequence have cleft palate.

Infants with Pierre Robin sequence also have increased incidence of associated anomalies, particularly cardiac and renal problems.

Newborns with Pierre Robin sequence may have severe respiratory and feeding difficulty because of the posterior displacement of the tongue.

Initial treatment consists of placing the child prone and use of gastric lavage feeding tubes to push the tongue forward.

Nasal airways have been used for the same purpose with reported success rates of 80–90%.

If these conservative measures fail, surgical management of the airway may be required.

A tongue–lip adhesion has been used as an alternative to tracheostomy and is generally effective.

More recently, mandibular distraction osteogenesis has been used in neonates with success in averting tracheostomy.

Palatoplasty in children with Pierre Robin sequence must be carefully timed with growth of the child, particularly the mandible, as closure of the palate narrows the effective area for respiration and can lead to respiratory distress.

• If the mandible attains reasonable size in the first year of life, palate repair can still be performed safely before 1 year of age.

• In the rare patient who has previously undergone tracheostomy, the palate should be repaired before decannulation.

• The risk of airway compromise after palatoplasty reaches 25%, with an emergent tracheostomy or reintubation rate of 11% at one institution.

Cleft palate without associated cleft lip has been reported to be associated with a syndrome in as many as 50% of cases, while cleft lip and palate together have an incidence of syndromes of about 30%.

Van der Woude syndrome is associated with a mutation in the interferon regulatory factor 6 ( IRF6 ) gene; this is an autosomal dominant syndrome associated with lower lip sinus tracts (“lip pits”), and has variable penetrance including the full range of cleft lips as well as palates.

Velocardiofacial syndrome is associated with a 22q chromosomal deletion, detected by fluorescent immunohybridization. These children have a characteristic “birdlike” facial appearance, soft palate dysfunction, developmental delay, and various cardiac conditions.

The same deletion gives rise to DiGeorge syndrome with associated B-cell and immune dysfunction.

Infants with profound developmental delay and severely shortened life span projection should have surgical intervention delayed or should undergo palatoplasty under special circumstances only.

Palate repair in severely disabled children can lead to altered airway status and obstructed upper airway in those with neuromuscular delay.

At birth, the average weight is the same for cleft and unaffected newborns. However, cleft infants have been shown to exhibit poor weight gain in early infancy.

After repair of the palate, average growth returns to normal compared with unaffected children by the age of 4 years.

Children with orofacial clefting stabilize and continue normal growth to at least 6 years of age, with no statistically significant differences in height and weight when compared to unaffected children.

In later childhood, however, the average weight and height of children with cleft palate appears to diminish compared with those of control subjects.