All issues > Volume 16(12); 1973

Case Report

J Korean Pediatr Soc. 1973;16(12):960-964. Published online December 31, 1973.

A Case of Klippel-Trenaunay-Weber Syndrome

Jin Yung Ro¹, Eui Hyun Jo¹, Sang Man Shin¹, Sang Jhoo Lee¹, Tai Yun Yoo²

¹Department of Pediatrics,College of Medicine,Kyung Hee University Seoul,Korea
²Department of Dermatology, College of Medicine,Kyung Hee University Seoul,Korea

Abstract

A case of Klippel-Trenaunay-Weber syndrome with macrodactylia in a 11-year-old boy is presented who was admitted to our Kyung Hee Medical Center with Chief complaints of nevus flammeus, swelling of the left lower leg and foot for 3 years duration. This patient was born with an extensive nevus flammeus of left leg, left upper arm, abdomen and right lumbar region. Additionaly he has had varicose veins of left leg and left arm with hypertrophy of left leg since at age of 8. There is no history of similar illness in the family. Physical examination revealed nevus flammeus of left side of leg, abdomen, left upper arm and right flank. There was hypertrophy on the left leg and foot with macrodactylia of great toes on both feet and right middle finger. Radiologic stuides: chest X-ray showed no abnormalitis, X-ray of the both hands revealed enlargement of all phalangeal bones in right middle finger. Venography of left lower leg revealed unusually dilated lower leg veins with marked engorgement suggestive of venous malformation. Laboratory data including Hb., Ht., C.B.C., total cholesterol, alkaline phosphatase, serum protein, S.G.O.T., S.G.P.T. calcium phosphorus, urinalysis were within normal limit except sed. rate (Wintrobe) 50 mm/hr, 2+ C.R.P. globulin 3. 8 gm% and albumin 3. 7 gm%. Patient was treated with elastic bandaging of the left leg to reduce edema and prevent stasis dermatitis.

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