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Classically described by Yakovlev and Wadsworth1 in 1946, schizencephaly is a rare congenital brain malformation characterized by clefts of the cerebral mantle extending from the pial surface to lateral ventricles lined by heterotopic gray matter. Observed associations between schizencephaly and in utero infections, trauma, fetal exposure to teratogens, and death of a monozygotic twin support the hypothesis that schizencephaly is the consequence of fetal vascular disruption during the late first or early secondary trimester of pregnancy.2

COL4A1 and fetal vascular origins of schizencephaly