Subependymal grey matter heterotopia with partial corpus callosum dysgenesis, open lip schizencephaly, pachygyria and Dandy Walker variant

Case contributed by Keshaw Kumar
Diagnosis certain


Developmental delay

Patient Data

Age: 10 years
Gender: Male

There are multiple small nodular lesions along the ependymal surface of bilateral lateral ventricle, following grey matter signal intensity on all sequences - subependymal nodular grey matter heterotopia.

There is abnormal communication between left lateral ventricle and interhemispheric fissure, which is lined by grey matter- open lip schizencephaly.

The isthmus and splenium of corpus callosum are not visualized. The rostrum, genu and body of corpus callosum are well formed - Partial corpus callosum dysgenesis.

There is abnormal thin connection between 4th ventricle and cisterna magna associated with inferior vermis hypoplasia --- Dandy-Walker variant.

Mild cortical thickening with sparse overlying sulci in the bilateral parieto-occipito-temporal lobes with mild dilatation of temporal horn of bilateral lateral ventricles - Pachygyria.

Note is made of fluid signal intensity in bilateral mastoid air cells, likely represent mastoiditis.

Case Discussion

Findings are suggestive of subependymal grey matter heterotopia associated with partial dysgenesis of corpus callosum, open lip schizencephaly, pachygyria and Dandy-Walker variant.

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