Emergent Orthotopic Liver Transplantation for Hemorrhage from a Giant Cavernous Hepatic Hemangioma: Case Report and Review

Abstract

IntroductionCavernous hemangiomas represent the most common benign primary hepatic neoplasm, often being incidentally detected. Although the majority of hepatic hemangiomas remain asymptomatic, symptomatic hepatic hemangiomas can present with abdominal pain, hemorrhage, biliary compression, or a consumptive coagulopathy. The optimal surgical management of symptomatic hepatic hemangiomas remains controversial, with resection, enucleation, and both deceased donor and living donor liver transplantation having been reported.Case ReportWe report the case of a patient found to have a unique syndrome of multiorgan cavernous hemangiomatosis involving the liver, lung, omentum, and spleen without cutaneous involvement. Sixteen years following her initial diagnosis, the patient suffered from intra-abdominal hemorrhage due to her giant cavernous hepatic hemangioma. Evidence of continued bleeding, in the setting of Kasabach-Merritt Syndrome and worsening abdominal compartment syndrome, prompted MELD exemption listing. The patient subsequently underwent emergent liver transplantation without complication.ConclusionAlthough cavernous hemangiomas represent the most common benign primary hepatic neoplasm, hepatic hemangioma rupture remains a rare presentation in these patients. Management at a center with expertise in liver transplantation is warranted for those patients presenting with worsening DIC or hemorrhage, given the potential for rapid clinical decompensation.

Giant Hepatic Hemangioma Presenting as Gastric Outlet Obstruction

“…However, in some patients the location and size of the lesion can cause various signs and findings. According to a previous study, 4 although 40% of hemangioma about 4 cm in diameter are symptomatic, 90% of those approximately 10 cm in diameter are symptomatic. The most frequently seen symptom is pain in the right upper quadrant.…”

“…[1][2][3]6 Hemangioma are often seen between the ages of 30 and 50 years and 5 to 6 times more often in women than in men. 4,6,7,9,10 Therefore, it has been suggested that there may be a relationship between the disease and the use of exogenous steroids or female-specific hormones, although the relationship has not been clearly shown. 2,3,10 Because most hepatic hemangioma are asymptomatic, they are often recognized incidentally during radiologic tests for other reasons.…”

“…Most lesions are less than 3 cm and a significant proportion of patients are asymptomatic. [1][2][3][4] In symptomatic patients, symptoms are associated with the size and location of the lesion, such as nausea, vomiting, abdominal pain, palpable mass, obstructive jaundice, gastric outlet obstruction and hemorrhage, consumption coagulopathy, and thrombocytopenia, which are characteristic of Kasabach-Merritt syndrome. 1,2,5 Although there is no general consensus on size, hemangioma .4 cm are referred to as giant.…”

Therapeutic Options of Giant Liver Hemangioma

“…Surgery includes liver resection, enucleation and transplantation [29]. A rare cases of liver transplantation performed for diffuse hepatic hemangiomatosis, lesions anatomically unresectable by conventional approaches and in patients with Kasabach-Merritt syndrome, have been reported [7][8][9][10]. However there is a shortage of liver graft and the need of a long-term anti-immunologic treatment in transplanted patients.…”

“…A hemangioma can be removed by enucleation or liver resection however, most authors advocated enucleation procedure. In very rare and exceptional condition, Liver transplantation can be considered as a treatment option for hemangioma [7][8][9][10]. Other therapeutic options as Radiofrequency ablation (RFA) and transcatheter arterial embolization (TAE) can be considered in some situations.…”