Treatment of Kasabach-Merritt syndrome by embolisation of a giant liver hemangioma

Abstract

We report the case of a 14-month-old child with Kasabach-Merritt Syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.

Transarterial embolization of giant liver hemangiomas associated with kasabach-merritt syndrome: a case report

“…If treated with corticosteroids, anticoagulants, antiplatelets, antifibrinolytics, interferon, radiation, or new antiangiogenic factors, the time to reach an acceptable hematological response has been reported to range from a few days to months, definitely longer than if compared to embolization, and not without complications (11,12,14). In our two patients, platelets and fibrin values rose to safe levels within 24 hours after embolization, similar to that reported in other published case reports (3,19). In addition, embolization provides a minimally invasive treatment option, since the severe coagulation disorders in this condition render these patients poor surgical candidates.…”

“…Although embolization may be considered a nonradical treatment (possibility for revascularization), it invariably corrects coagulation disorders without remissions, as shown by our and previously published data (3,5,19). These results are also concordant with the surgical literature, showing that even debulking of the vascular anomalies alleviates the coagulopathy associated with Kasabach-Merritt syndrome (10).…”

“…Kasabach-Merritt syndrome (KMS) is a rare and severe coagulation disorder caused by vascular malformations within or outside the liver (3,5,7). Its incidence is not known, since published papers only present case reports.…”

“…Transarterial embolization (TAE) has been applied at several centers as a minimally invasive treatment option for hemangiomas causing KMS (3,17). Noninvasive control of this severe, life-threatening coagulation disorder is essential, since these patients are poor surgical candidates.…”

“…Noninvasive control of this severe, life-threatening coagulation disorder is essential, since these patients are poor surgical candidates. Most reports on embolization treatment for KMS include various causative vascular anomalies, notably superficial angiomatous lesions with arteriovenous shunting in both infancy and adulthood, with few long-term results (3,17,19).…”