Dandy-Walker malformation

Case contributed by Dr Hidayatullah Hamidi

Presentation

Enlarged head, developmental delay.

Patient Data

Age: 6 months
Gender: Male
CT

The classic triad of the disease is evident:

1. severely hypoplastic vermis with cephalad rotation of the vermian remnant

2. cystic dilatation of the fourth ventricle extending posteriorly to an enlarged posterior fossa

3. significantly enlarged posterior fossa with torcular-lambdoid inversion and open posterior fontanel and thinning of adjacent bones

Marked communicating chronic hydrocephalus.

Case Discussion

Dandy-Walker malformation is the most common posterior fossa malformation (about 1 per 30,000). It can be an isolated entity or associated with other anomalies.

The imaging differential diagnosis can be mega cisterna magna and large arachnoid cyst.

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Case information

rID: 56022
Case created: 5th Oct 2017
Last edited: 12th Oct 2017
System: Paediatrics
Inclusion in quiz mode: Included
Institution: French Medical Institute for Mothers and Children

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