Klippel-Trenaunay-Weber syndrome: CNS manifestations
Headaches and seizures.
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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 haemorrhage in the right mid corona radiata.
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.