Schizencephaly

Schizencephaly is a rare birth defect.

It causes slits or clefts in the cerebral hemispheres of your brain. These clefts may appear on one or both sides of your brain. They may be filled with cerebrospinal fluid.

a) sine qua non: cleft that communicates with the ventricle (communication may be confirmed with CT cisternogram if necessary)

b) cleft lined with cortical grey matter (often abnormal, may have polymicrogyria). This distinguishes it from porencephaly, a cystic lesion lined with connective or glial tissue that may communicate with the ventricular system, often caused by vascular infarcts or following intracerebral hemorrhage or penetrating trauma (including repeated ventricular punctures)

c) two forms: ● open lipped: large cleft to the ventricle. Very severe forms may mimic hydranencephaly

● close-lipped (walls fused): look for a dimple in the lateral wall of the lateral ventricle immediately under the cortical cleft (the appearance of which may mimic an enlarged sulcus)