Congenital Malformations Associated With Iniencephaly

Abstract

INTRODUCTION: Iniencephaly is a rare birth defect having retroflexion of the head combined with severe distortion of the spine. Based on the presence or absence of encephalocele, Lewis [1, 9] has classified iniencephaly into two groups-iniencephaly apertus and iniencephaly clausus. Cardiovascular disorders, diaphragmatic hernias, and gastrointestinal malformations are other additional defects seen in these cases. Most of iniencephalic babies are stillborn or die soon after birth (2, 9); however the milder forms of iniencephaly cases are not fatal. Here we present a rare case of iniencephaly clausus with review of literature.