Dandy-Walker malformation associated with Klippel-Feil syndrome

Case contributed by Bob Zhang
Diagnosis certain

Presentation

Fall with headstrike

Patient Data

Age: 50 years
Gender: Male

Non-enhanced CTB & Cx spine

ct

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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