NONLETHAL CASE OF OTOCEPHALY AND ITS IMPLICATIONS FOR TREATMENT

Otocephaly is a craniofacial syndrome with a constellation of findings consistent with symmetrically deficient development of the first bran chial arch. Characteristically, those affected have ventrally displace d ears, microstomia, and an atrophic to absent mandible. Secondary to oropharyngeal incompetence, ventilatory difficulties typically lead to the imminent death of these infants shortly after birth. Here we pres ent a patient who survived well beyond the perinatal period with the s upport of pediatric intensivists and advanced technology available for the diagnosis and treatment of such a complex crajniofacial syndrome.