KASABACH-MERRITT COAGULOPATHY COMPLICATING KLIPPEL-TRENAUNAY-WEBER-SYNDROME IN PREGNANCY

Background: Klippel-Trenaunay-Weber syndrome is a sporadic genetic syn drome characterized by localized hemangiomas, venous varicosities, and asymmetric osseous hypertrophy of the ipsilateral extremities. Most c ommonly seen in association with hemangiomas, Kasabach-Merritt syndrom e is defined by the presence of thrombocytopenia and a consumptive coa gulopathy. Case: A 22-year-old primigravida with a prior diagnosis of Klippel-Trenaunay-Weber syndrome presented for genetic counseling and delivery management at 37 weeks' gestation. Large varicosities of the vulva required cesarean delivery. Multiple hemangiomas in the right lo wer quadrant of the abdomen necessitated the use of a left paramedian cutaneous incision. The patient subsequently developed Kasabach-Merrit t syndrome and required the transfusion of blood products as well as h eparin and aminocaproic acid therapy for her postoperative management. Conclusions: Klippel-Trenaunay-Weber syndrome in pregnancy is rare. T he potential for a refractory coagulopathy presenting as Kasabach-Merr itt syndrome should be considered in any patient who presents with ext ensive hemangiomas.