Dandy-Walker malformation associated with Klippel-Feil syndrome

Case contributed by Dr Bob Zhang

Presentation

Fall with headstrike

Patient Data

Age: 50
Gender: Male
CT

Non-enhanced CT brain and cervical spine

Enlarged retrocerebellar CSF space with torcula-lambdoid inversion, cerebellar vermis hypoplasia and dilatation of the fourth ventricle and torcula-lambdoid inversion is in keeping with a Dandy-Walker malformation. There is also associated dysgenesis of the corpus callosum.

A right parietal approach VP shunt is in-situ, associated with diffuse calvarial thickening and dural calcification including the falx and tentorium, which can be seen in chronic VP shunting.

In addition, in the cervical spine there is failure of segmentation from C6-T1, compatible with Klippel-Feil syndrome, which can be associated with Dandy-Walker malformations. There is also evidence of prior bilateral C3-C6 decompressive laminectomy.

Case Discussion

Dandy-Walker malformations can be associated with Klippel-Feil syndrome, as seen in this patient. There are also calvarial and dural changes associated with chronic VP shunting.

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Case information

rID: 78601
Published: 7th Jun 2020
Last edited: 27th Jun 2020
Inclusion in quiz mode: Excluded
Institution: Alfred Health

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