Iniencephaly: A Case Report

Iniencephaly is an unusual malformation of variable incidence with involvement of the occiput (inion) and the brain. The features are: (1) Imperfect formation of the base of skull; (2) Rachischisis; (3) Retroflexion of the spine. We present a case report that illustrates the morphological features of iniencephaly. A multipara with 22 weeks gestation and history of gestational diabetes had a twin gestation with intrauterine death of both fetuses detected by ultrasound. An autopsy performed revealed one twin had features of iniencephaly while the other was morphologically normal. Iniencephaly is incompatible with life and its etiology is unknown requiring further experimental studies.