Parkes Weber syndrome: possible

Case contributed by Dr Laughlin Dawes

Presentation

Assessment of leg length discrepancy and cutaneous “capillary” vascular malformation.

Patient Data

Age: 10 years
Gender: Male
MRI

Dilated vascular structures in the right lower limb involving subcutaneous and multiple muscle compartments. Flow voids and pulsation artefact (particularly laterally) suggest a high flow component consistent with arteriovenous malformation. 

Case Discussion

The provisional diagnosis was Klippel-Trenaunay syndrome but these findings favour Parkes Weber syndrome over Klippel-Trenaunay syndrome. There was no high output cardiac failure in this case.

Vascular malformations can be divided into high flow (predominantly AV malformations) and low flow (capillary, venous, lymphatic and mixed). Klippel-Trenaunay syndrome usually involves an entire limb and is of the mixed, slow-flow type. Parkes Weber syndrome also typically involves an entire limb, but the malformation contains high-flow components.

Note that Parkes Weber is one man, of Sturge-Weber, Klippel-Trenaunay-Weber, Osler-Weber-Rendu, Pfeifer-Weber-Christian, and Weber-Cockayne fame.

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Case information

rID: 7685
Case created: 18th Nov 2009
Last edited: 7th Oct 2015
Inclusion in quiz mode: Excluded

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