Treatment of a Giant Haemangioma of the Liver With Kasabach‐Merritt Syndrome by Orthotopic Liver Transplant

Abstract

We describe a case of giant cavernous haemangioma of the liver with disseminated intravascular coagulopathy (Kasabach-Merritt syndrome) which was cured by orthotopic liver transplant.A 47 year old man presented with bleeding and tender massive hepatomegaly after tooth extraction.Investigations showed disseminated intravascular coagulopathy and a giant hepatic haemangioma involving both lobes of the liver. Initial treatment failed to resolve the coagulopathy and liver resection was attempted. At laparotomy the turnout was unresectable and the only option for cure was to offer a liver transplantation. The orthotopic liver transplant was performed 20 days after initial laparotomy. Subsequently, all coagulation parameters returned to normal and the patient remains well after 12 months. Orthotopic liver transplant can be considered for giant hepatic haemangioma with Kasabach-Merritt syndrome when resection is necessary and a partial hepatectomy is not technically feasible.

Giant Haemangioma of the Liver: Observation or Resection?

“…16 patients underwent intervention including 8 resections, 5 arterial embolisations, 2 transjugular intrahepatic portosystemic shunts, 1 right hepatic artery ligation, and 1 liver transplantation. Liver transplantation has been used by a number of groups for unresectable disease, and for Kasabach-Merritt syndrome [24]. Groups have also reported extracorporeal resection for ‘unresectable’ giant haemangiomata [25].…”

Hemangioma hepático gigante asociado a síndrome de Kasabach-Merrit

“…En pacientes con sín-tomas sugestivos de compresión extrínseca se debe considerar la intervención quirúrgica, siendo más indicada la enucleación frente a la resección, debido a que presenta menor sangrado en el postoperatorio (13,14). El trasplante hepático se llevará a cabo en pacientes sintomáticos con lesiones irresecables o en caso de un hemangioma gigante con hemangiomatosis y asociados al síndrome de Kasabach-Merrit (15)(16)(17). En determinados casos se ha probado la eficacia de la embolización transarterial de los hemangiomas con mejoría rápida de la coagulopatía de consumo (18).…”

Resektion und Transplantation bei Lebertumoren

“…Eine Lebertransplantation ist bei benignen Lebertumoren nur extrem selten indiziert, etwa beim Kasabach-Merritt-Syndrom, bei dem es durch Spontanruptur eines Hämangioms zu einer disseminierten intravasalen Koagulopathie mit Fibrinolyse und Thrombozytopenie kommt [34,49]. Eine Transplantationsindikation kann zudem bei einer Adenomatose oder bei multiplen HCA auf dem Boden einer Glykogenose bestehen.…”