Schizencephaly

Schizencephaly is a rare cortical malformation characterized by a full-thickness gray matter-lined cleft extending from the pial surface to the ventricular wall. This defect is frequently associated with other intracranial abnormalities, including absence of the septum pellucidum, optic nerve hypoplasia, corpus callosum dysplasia, hydrocephalus, arachnoid cysts, and cerebellar malformations. While increasingly diagnosed antenatally, many patients present later in life with seizures, intellectual disability, or psychomotor delay. Clinical severity is closely related to the size and location of the cleft. Management is symptom-based and may include medical treatment for seizures, shunt placement for hydrocephalus, and supportive therapies for developmental and motor impairments.