INIENCEPHALY - NEURORADIOLOGICAL AND SURGICAL FEATURES - CASE-REPORT AND REVIEW OF THE LITERATURE

Iniencephaly is a rare congenital anomaly characterized by spina bifid a of the cervical vertebrae, fixed retroflexion of the head on the cer vical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that we re surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retar dation. The neuroradiological and surgical findings of the case sugges ted that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.