Dandy Walker malformation with callosal dysgenesis and subependymal heterotopia

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Seizures.

Patient Data

Age: 17 months
Gender: Male
  • 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.

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