Hemimegalencephaly

Case contributed by Naqibullah Foladi
Diagnosis certain

Presentation

Developmental delay

Patient Data

Age: 6 months
Gender: Male
ct

Significant enlargement of the left frontal, parietal and occipital lobes with significant cortical thickening and indistinct grey-white matter differentiation. The cortical surfaces are appearing smooth with a paucity of gyri and sulci. The significant mass effect over the right cerebral hemisphere and shift of the flax cerebri to the right side.

The left ventricle appears entrapped within the enlarged left frontal and parietal lobes. There is nipple like outpouching at the ependymal surface along the left parental bone which is likely extending up to the left lateral ventricle likely representing closed-lip schizencephaly.

Increased density is identified in the atrioventricular area on the left side, representing accelerated myelination. 

The is relative sparing of left temporal and occipital lobes.

The right cerebral hemisphere appears grossly unremarkable.

 

Case Discussion

The features are consistent with left side hemimegalencephaly.

The right cerebral hemisphere appears grossly unremarkable, proper evaluation with MRI can be considered.

 

 

This case is also contributed by Dr. Naila Nadeem.

 

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