Otocephaly-dysgnathia complex: a new case report

Abstract

Otocephaly-dysgnathia complex is a very rare malformation characterized by microstomia, hypo or aglossia, agnathia and low inserted ears. The affection results from an anomaly of development of the first and second branchial archs. Affected patients have poor prognosis and death occurs generally shortly after birth because of respiratory problems. We report the case of a male premature infant born at 32 weeks, presenting with severe respiratory distress at birth. Morphological features were consistent with the diagnosis of otocephaly. Death occurred shortly after birth. Autopsy didn't find any associated malformation.