Klippel-Trenaunay-Weber syndrome - CNS manifestations

Case contributed by Dr Henry Knipe


Headaches and seizures.

Patient Data

Age: 20 years
Gender: Female

Diffuse predominantly right-sided vascular anomalies, with asymmetrically prominent right sided arterial branches, and prominent right-sided veins. The right sided medullary, internal cerebral and superficial veins are particularly prominent, together with the vein of Galen, straight sinus and right transverse sinus.

The right hemisphere is slightly more T2 hyperintense than the left, with more prominent perivascular spaces. No definite hemihypertrophy or atrophy.

Venous dysplasias are again noted in the right pons and cerebellum, also arterial flow signal in the pontine lesion also suggests the presence of AV shunting. Focus of hemorrhage in the right mid corona radiata.

Case Discussion

Klippel-Trénaunay-Weber syndrome is a combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. This patient had a known history of the syndrome. 

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Case information

rID: 52122
Published: 22nd Mar 2017
Last edited: 24th Apr 2019
Inclusion in quiz mode: Included

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