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Unilateral Closed Lip Schizencephaly with Septo-Optic Dysplasia

Case Findings: CSF isointense cystic lesion in the left anterior temporal region with evidence of operative shunt in place.  Gray matter lined cleft extennding from right lateral ventricle to the pial surface suggesting closed lip schizencephaly. Interventricular spetum is absent (associated septo-optic dysplasia can be considered). Usually in SOD associated with schizencephaly optic apparatus less severely affected and is sometimes referred to as septo-optic dysplasia plus. 

Discussion : 
  • Schizencephaly is a malformation of cortical development characterized by gray matter lined clefts in the cerebral cortex. 
  • Both genetic and non-genetic etiologies like prenatal infections or ischemia have been postulated. 
  • Clinical manifestations most often include varying degrees of developmental delay, motor impairment and seizures. 
  • It can be associated with septo-optic dysplasia (SOD), optic nerve hypoplasia and absence of septum pellucidum, pachygyria, polymicrogyria, heterotopia and arachnoid cysts. 

Unilateral Closed Lip Schizencephaly with Septo-Optic Dysplasia Reviewed by Sumer Sethi on Monday, February 06, 2017 Rating: 5

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