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Journal of the Korean Society of Neonatology 1998;5(1):86-89.
Published online January 1, 2001.
A Case of Open - lip Schizencephaly.
Woo Jin Kwon, Sun Kim Lee, Seung Keun Lim, Jae Ho Hyun, Mun Young Lee, Jin Park, Man Kyu Yang
Abstract
Schizencephaly, first described by Yakovlev and Wadsworth in 1946, is a rare congenital anomaly characterized by gray matter-lined clefts that extend through the entire cerebral hemisphere, from the ependymal lining of the lateral ventricle to the subarachnoid space. Clinically, patients with schizencephaly present motor dysfunction such as hemiparesis, seizures, and variable developmental delay. Absence of septum pellucidum, gray matter heterotopia, polymicrogyria, and hypoplasia of optic nerves were also frequently found to be associated with schizencephaly. We experienced a case of open-lip schizencephaly in a one-day-old male infant with wide, tense fontanel, which was confirmed by MRI.
Key Words: Schizencephaly, Gray matter-lined cleft


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