The Klippel-Trenaunay Syndrome: Clinical and Radiological Aspect

Abstract

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine hemangiomas, deep venous system abnormalities, superficial varicosities, and bony and soft-tissue hypertrophy. When associated with an arteriovenous fistula, it has been termed Klippel-Trenaunay-Parkes-Weber syndrome. It is imperative that both the radiologist and surgeon be aware of this entity, as incomplete evaluation and inappropriate surgery may be devastating. Radiological workup includes phlebography, angiography, and conventional radiography of the involved extremities. Surgery should be performed only to relieve deep venous obstruction (if present) or to correct inequality in the lengths of legs. Removal of superficial varicosities is contraindicated because it will worsen existing symptoms. Five cases of KTS are presented and the literature reviewed.