Schizencephaly Schizencephaly refers to a type of migrational anomaly in which a cleft a extends from the ependyma of the ventricle to the pial surface. The clefts are lined by gray matter and are filled with cerebrospinal fluid. Open lip schizencephaly refers to clefts where the walls are separated an is associated with a worse prognosis than closed lip schizencephaly in which the walls are in apposition.
Patients typically present with intractable seizures and developmental delay.
The generally accepted pathogenesis of schizencephaly is via early in-utero vascular insult. Associations include other migrational anomalies, dysgenesis of the corpus callosum and septo-optic dysplasia. One unique feature of this case is the type of callosal dysgenesis seen here. Usually, when there is partial absence of the corpus, it is the splenium and posterior body which is missing. This is due to the fact that the corpus callosum develops from the genu posterior to the splenium.