Schizencephaly - closed lip

Case contributed by Ammar Haouimi
Diagnosis almost certain



Patient Data

Age: 15 years
Gender: Female

The MRI sequences demonstrate a left frontal parenchymal defect lined by heterotopic grey matter, extending from the cortex surface to the body of left lateral ventricle with separated walls near the entrance of the lateral ventricle. The lateral ventricle is enlarged with a small triangular ependymal defect.  

Areas of polymicrogyria are noted along the borders of the defect as well as left frontal lobe surface. The septum pellucidum is absent. The corpus callosum appears normal.

Case Discussion

MRI features are characteristic of a closed lip schizencephaly (type I) associated with absent septum pellucidum and polymicrogyria

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