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.
Aronoff
D M
DM
Department of Internal Medicine, Vanderbilt University Medical Center, Nashville, Tenn, USA.
Roshon
M
M
D000328:Adult; D001780:Blood Coagulation Tests; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000175:diagnosis*; D004598:Electrosurgery; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000097:blood / Q000175:diagnosis*; D019106:Postoperative Hemorrhage / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D012008:Recurrence; D013921:Thrombocytopenia / Q000097:blood / Q000175:diagnosis*; D002578:Uterine Cervical Dysplasia / Q000097:blood / Q000601:surgery*