Kasabach-Merritt coagulopathy complicating Klippel-Trenaunay-Weber syndrome in pregnancy

Klippel-Trenaunay-Weber syndrome is a sporadic genetic syndrome characterized by localized hemangiomas, venous varicosities, and asymmetric osseous hypertrophy of the ipsilateral extremities. Most commonly seen in association with hemangiomas, Kasabach-Merritt syndrome is defined by the presence of thrombocytopenia and a consumptive coagulopathy.

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 lower quadrant of the abdomen necessitated the use of a left paramedian cutaneous incision. The patient subsequently developed Kasabach-Merritt syndrome and required the transfusion of blood products as well as heparin and aminocaproic acid therapy for her postoperative management.

Klippel-Trenaunay-Weber syndrome in pregnancy is rare. The potential for a refractory coagulopathy presenting as Kasabach-Merritt syndrome should be considered in any patient who presents with extensive hemangiomas.