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Articles » Central nervous system » Schizencephaly

2006-05-31-14 Schizencephaly © Cuillier


Cuillier F*, Lemaire P**, Deshayes M**, Fossati P***

* Department of Gynecology, Félix Guyon’Hospital ** Sonographer, Moufia’street  *** Department of Radiology, Hôpital Félix Guyon - Ile de la Réunion - France

Definition: An old definition of schizencephaly is “a form of porencephaly characterized by a cyst or cavity in the cerebral hemispheres”. In fact, schizencephaly is a congenital defect due to an abnormal development of the fetal brain. Schizencephaly is certainly an uncommon disorder of neuronal migration, characterized by a cerebrum-spinal fluid-filled cleft, lined by gray matter. The clefts may extend through the entire hemisphere from the ependymal lining of the lateral ventricles to the periphery (pial surface) covering the cortex of some part of the brain. The cleft can be unilateral or bilateral and are commonly located near the pre- or post-central gyri. The gray matter lining can be dysplastic. There are two types of schizencephaly:

  • Type I: The clefts can be unilateral or bilateral and may be closed (fused lips). In closed-lip (type I), the cleft walls are in apposition, causing obliteration of the CSF space within the cleft.
  • Type II: The clefts can be unilateral or bilateral and may be separated (open lips). In open-lips (type II), the clefts walls are separated. The CSF fills the cleft from the lateral ventricles to the subarachnoid space that surrounds the hemispheres.


Case report: This is a 30-year-old-woman, G2P1, referred to our antenatal unit due to a hydrocephaly. The triple test and the nuchal translucency were normal. At 21 weeks, a bilateral hydrocephaly was observed. The spine was normal. The cerebellum was visualized. There was no Arnold-Chiari syndrome. The couple was counseled. They refused to perform an MRI or interruption of pregnancy. The serial scans showed a progressive evolution of the brain lesion. The cortical brain seemed present but very thin.


At 36 weeks, a cesarean section was p