SCHIZENCEPHALY

Schizencephaly is a rare anomaly of neuronal migration characterized by clefts extending from the ventricular margin to the cortical surface. Schizencephaly results when the derangement occurs very early, and there is an interruption in the migration of specific neuroblasts along prescribed glial pathways. The result is a pial-ependymal cleft, lined by gray matter (either totally or in part) which extends from the ventricular surface (the ependyma) to the periphery of the brain (pial surface).

Two types are present:

• Type I - cleft lips are fused within a pia-ependymal seam that is continuous with the ependyma of the lateral ventricle.
• Type II - cleft lips are open allowing direct communication between the Sylvian subarachnoid space and the lateral ventricle.

1. Pathogenesis

2. Ultrasound

3. Differential Diagnosis

4. Prognosis