Dandy-Walker malformation

Case contributed by Huda B. Gharbia
Diagnosis almost certain

Presentation

Large head, with posterior swelling.

Patient Data

Age: 3 months
Gender: Male
mri

Large posterior fossa, with cystic dilatation of the 4th ventricle, of CSF signal intensity, extending into posterior fontanelle forming subcutaneous bulging.

Hypoplasia of cerebellar vermis, cephalad rotation of vermian remnant.

Torcula-lambdoid inversion, abnormal high tentorium.

Case Discussion

Dandy-Walker malformation is a cerebellar anomaly, in which cystic balloon-like dilatation of the fourth ventricle, large posterior fossa, usually associated with supratentorial hydrocephalus, in such case open posterior fontanelle.

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