Abstract

Schizencephaly is a developmental anomaly that causes unilateral or bilateral clefts of the cerebral hemisphere as a result of disordered neuronal migration. The clefts are lined by gray matter that can extend medially from the subarachnoid cerebrospinal fluid space into the lateral ventricle as part of a disease spectrum that includes porencephaly. Closed-lip schizencephaly (Type 1) occurs when the gray-matter lined walls are in contact with each other (1). By contrast, the open lip variety (Type 2) has separated lips with a cleft of cerebrospinal fluid that extends to the ventricles (2). Barkovich and co-workers (3) have reported that the clinical manifestations of affected infants are related to the size and location of the cleft defect. Late prenatal schizencephaly has been well documented. We now report well-documented case that demonstrates diagnostic challenges and pitfalls associated with its early diagnosis by 2D/3D ultrasonography and magnetic resonance imaging (MR).

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