Hemihydranencephaly: Case report and literature review

Hydranencephaly is a severe brain condition characterized by complete or al most complete absence of cerebral cortex with preservation of meninges, bas al ganglia, pons, medulla, cerebellum, and falx. It has been ascribed to di fferent causes (infections, irradiations, fetal anoxia, medications, twin-t win transfusion), all leading to vascular disruption. Hemihydranencephaly i s an extremely rare condition in which the vascular anomaly is unilateral. We report on a patient who was suspected to have hydrocephalus in utero; a brain magnetic resonance imaging scan showed left-sided hydranencephaly wit h preservation of basal ganglia. The patient developed signs of right hemip aresis but notably has only mild language delay. The available literature o n hemihydranencephaly is reviewed.