Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular syndrome involving bone and soft tissue hypertrophy of the involved limb and vascular malformations of the lymphatic, capillary, and venous systems. It is often confused with Parkes-Weber syndrome (PWS). KTS is characterized by a triad of capillary malformation in the form of port wine stains, bone or limb hypertrophy, and varicose veins. The vein of Servelle, also known as the lateral marginal vein, is one of the two persisting embryonic veins of the leg, the persistent sciatic vein being the other. Truncal vascular malformation can be a complication of failure of obliteration of these veins. We present a case of a 24-year-old male of KTS who had varicose veins in his right lower limbs since five years of age and macrodactyly with a synchronous presentation of the vein of Servelle.
Keywords: klippel-trenaunay syndrome; limb hypertrophy; parkes weber syndrome; short saphenous vein; vascular malformation; vein of servelle.
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