Severe hemorrhage complicating the Klippel-Trénaunay-Weber syndrome

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