Otocephaly - A case report

Abstract

Otocephaly is a rare congenital malformation of the head and neck caused by maldevelopment of the 1st and 2nd pharyngeal arches. It is characterized by absence of the mandible and approximation of the ears in the midline region normally occupied by the mandible. Pathomorphological features of an infant with otocephaly born during 33rd week of gestation, which died immediately after birth because of the oral cavity atresia, are described. The infant was born from the first (poorly controlled) pregnancy in healthy parents. The putative causative factors of this malformation are briefly dicussed.