SEVERE HEMORRHAGE COMPLICATING THE KLIPPEL-TRENAUNAY-WEBER-SYNDROME

The Klippel-Trenaunay-Weber (KTW) syndrome is a congenital disorder of angiogenesis characterized by macular nevus, skeletal and soft tissue hypertrophy, venous varicosities, and arteriovenous fistulas. Dissemi nated intravascular coagulation (DIC) and the Kasabach-Merritt syndrom e, a consumptive coagulopathy with thrombocytopenia, are both associat ed with the KTW syndrome. We describe a 30-year-old woman with KTW syn drome and Kasabach-Merritt syndrome who had DIC with severe hemorrhage after a routine gynecologic procedure, The bleeding was controlled wi th the use of intravenous low-dose heparin and antithrombin III.