SUCCESSFUL MANAGEMENT OF VISCERAL KLIPPEL-TRENAUNAY-WEBER-SYNDROME WITH THE ANTIFIBRINOLYTIC AGENT TRANEXAMIC ACID (CYCLOCAPRON) - A CASE-REPORT

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital disorder characterized by vascular nevus formation, deep venous thrombosis, va ricosities, and hypertrophy of affected tissues. A patient with known thrombosis of his splanchnic circulation and visceral KTWS presented w ith life-threatening hemorrhage from rectosigmoid varices. Portosystem ic shunting was not feasible. Endoscopic sclerosis, variceal ligation, and proctocolectomy were not possible due to the size and number of t he varices. Previous treatment with epsilon-aminocaproic acid had been unsuccessful and complicated by thrombophlebitis. Conservative treatm ent with blood transfusions, cryoprecipitate, fresh frozen plasma, vit amin K, propanolol, and somatostatin analog failed to stop the bleedin g. The patient was given the antifibrinolytic agent, tranexamic acid, with cessation of his hemorrhage. Serial thromboelastograms confirmed improved reaction time, coagulation time, clot formation rate, and max imum amplitude. We conclude that tranexamic acid may be a useful adjun ct in the medical treatment of high-risk patients with KTWS and other vascular nevi complicated by coagulopathy.