Teaching Case

Right lower limb venous insufficiency, hemosiderin pigmentation and a varicose ulcer at the malleolar region. At clinical examination, his right thigh was swollen and painful with numerous and tortuous varicosities, particularly in the region of the saphenous vein. There was a thrill on palpation and a murmur on auscultation of the right thigh. A phlebography of the right lower limb was performed and showed no opacification of the deep venous system. Subsequently an arteriography was carried out. A scanogram, not shown, revealed a lengthening of the right lower limb

A 30-year-old man was admitted to the hospital with right lower limb venous insufficiency, hemosiderin pigmentation and a varicose ulcer at the malleolar region. At clinical examination, his right thigh was swollen and painful with numerous and tortuous varicosities, particularly in the region of the saphenous vein. There was a thrill on palpation and a murmur on auscultation of the right thigh. A phlebography of the right lower limb was performed and showed no opacification of the deep venous system. Subsequently an arteriography was carried out. A scanogram, not shown, revealed a lengthening of the right lower limb

Klippel-Trenaunay Syndrome (KTS) is an uncommon disease (300 cases published). KTS is an association of soft tisssue and bone hypertrophy, cutaneous hemangiomas and superficial varicosities. Klippel-Trenaunay-Wever Syndrome (KTWS) is the association of KTS with arterio-venous fistulae. KTS and KTWS are due to a congenital malformation of the deep venous system with agenesis, hypoplasia or segmental atresia. The etiology is not clear but the syndrome is most likely the result of a diffuse mesodermal abnormality. KTS and KTWS are not hereditary but show some female predominance. Usually KTS and KTWS affect only lower SWOP-Coated extremities, are sometimes bilateral of may also involve the upper extremities. Soft tissue and bone hypertrophy associated with portwine hemangiomas suggest the diagnosis at birth. Varicosities appear later in the disease course. Phlebography and arteriography are necessary to define the extension of the abnormalities and to demonstrate the presence or absence of arterio-venous fistulae. Arterio-venous fistulae worsen the prognosis of the disease.