Dandy Walker malformation with callosal dysgenesis and subependymal heterotopia
Diagnosis almost certain
Presentation
Seizures.
Patient Data
Age: 17 months
Gender: Male
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- dilated 4th ventricle communicating with an enlarged posterior pericerebellar space through vermian hypoplasia with a moderate anterolateral displacement of the cerebellar hemispheres in keeping with Dandy-Walker malformation
-
agenesis of the corpus callosum
- parallel lateral ventricles "racing car sign" and dilatation of the occipital horns (colpocephaly)
- narrow frontal horns, high-riding 3rd ventricle, giving a "moose head appearance" on coronal T2
- sunray appearance of the gyri on sagittal T1 sequence
- widened mid-portion of the interhemispheric fissure
- absence of the cingulate sulcus
- subependymal grey matter heterotopia mainly along the ventricular wall of the temporal horns and trigone of the lateral ventricles
Case Discussion
MRI features of Dandy-Walker malformation with associated callosal dysgenesis and subependymal grey matter heterotopia.