CASE OF THE DAY ANSWER

CASE OF THE DAY ANSWER

This infant is suffering from cleft lip and palate. Clefts are highest amongst the Asians and lowest amongst the blacks. Clefts of the lip and palate are more common in males. 

Cleft lip may be incomplete or complete (complete separate up to the floor of nose). Clefts may be unilateral or bilateral. They are relatively more common on left side. Deformities of teeth, alveolar ridge and premaxilla may be associated with it.

Isolated cleft palate occurs in midline and may be restricted to uvula only or may involve the soft and hard palate unilaterally or bilaterally exposing the nasal cavities.

Children with cleft palate are more vulnerable to suffer from recurrent middle ear infections that substantially increase the chances of conductive hearing loss in these children.

Cleft lip and palate are usually inherited in a multifactorial manner. Multifactorially determined disorders are the ones that are product of multiple genetic and environmental factors.

Risk of recurrence of unilateral cleft lip and palate is 4% if one child is affected but it increases to 9% if two children are affected.

1-2% of children with cleft lip or palate may have Van der Woude syndrome. It may have different presentations with varying combinations of cleft lip, cleft palate, hypodontia and characteristic lip pits on the lower lip. These lip pits have openings of accessory salivary glands with visible saliva coming out. It is inherited in a dominant way. So, one must examine parents carefully to exclude it as recurrence risk is 50% in this situation.

Management

Taking care of appropriate feeding in these children is the first priority at birth.

Surgical closure of the lip is usually done at 3 months of age. Z-plasty is the most commonly used technique. Preoperatively infant should be free of oral, respiratory or systemic infection and must have appropriate weight (Rule of 10 suggests 10 weeks age, 10 pounds weight and 10 grams hemoglobin).

Initial repair may be revised at 4 or 5 years of age and deformity of nose can be corrected by adolescence.

Timing of surgery of cleft palate is individualized because of many variables in its presentation. Usually it is done around 1 year age to enhance the chances of normal speech development. Some children may require speech therapy for normal speech development. Displacement of maxillary arches and malformed teeth often require orthodontic correction.