Total knee arthroplasty in Klippel-Trenaunay syndrome.

A 35-year-old man had suffered pain in his left knee for several years. He found walking extremely difficult. Nonsurgical treatment did not alleviate the pain. On examination, he had an enlarged left lower extremity. There was an abundance of hemangiomas and varicose veins throughout the affected extremity. There was a varus alignment. He had a fixed flexion deformity with extension to only 30° and flexion to only 80°. True leg-length discrepancies were difficult to measure because of the flexion contracture. The neurologic findings were normal. The dorsalis pedis and posterior tibial arteries were difficult to palpate. However, the extremity was warm, had good capillary refill and was well perfused. Radiographs (Fig. 1) revealed severe degenerative arthritis in the left knee with bone loss from the medial plateau. The femoral condyles were large and squared. There was subchondral sclerosis with osteophytic formation. There was generalized osteoporosis. Angiography revealed that none of the cutaneous hemangiomas communicated with the deep venous system. The patient’s coagulation indices were normal. A tourniquet was used during surgery. Many hemangiomas and varicosities were encountered, but hemostasis was obtained. The synovium had a grey-black appearance. The remaining articular cartilage had an orange tinge. Total knee arthroplasty was performed without complication. A cemented prosthesis with modular tibial components was implanted. The patella was well preserved and not resurfaced. Patellar osteophytes were removed. His postoperative course was uncomplicated. He did not require transfusion of any blood products. Low molecular weight heparin was administered prophylactically. He had no signs or symptoms of pulmonary embolus or deep vein thrombosis (DVT). Duplex ultrasonography of both lower extremities was performed on postoperative day 5. The scan was technically difficult but showed no evidence of DVT. Histologic examination of excised knee tissue showed that the synovium contained labyrinths of wide vascular