Transcatheter embolization as a rescue therapy for refractory pelvic kaposiform hemangioendothelioma with...

Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy in children with kaposiform hemangioendothelioma (KHE) or tufted angioma. Until now, there has been no well-accepted treatment regimen in the literature. A pelvic KHE is very rare and its deep anatomical location and invasive growth pattern toward adjacent vital organs pose significant management challenges. Here, we report a 10-month-old girl presenting with KMP secondary to a pelvic KHE. Initial systemic therapy with sirolimus and corticosteroids failed to control her KMP. Transcatheter arterial embolization was performed using polyvinyl alcohol particles and coils. After embolization, the tumor's blood supply decreased significantly and the patient's platelet counts and coagulation markers returned to normal in 3 days. At the 2-year follow-up, her coagulation function remained normal. The perineal cutaneous rash resolved and the tumor's volume decreased substantially. This case demonstrates that transarterial embolization can be an effective therapeutic choice when systemic treatment has failed.