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

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

LONG-TERM RESULTS AFTER LIVER-TRANSPLANTATION IN ADULTS

The prospects for patients who survive the first year after liver transplantation are discussed. The 10-year survival for these patients is more than 80%. The quality of life is good, as measured on self-assessment scales. Pregnancy is possible. The main side-effects of drugs concern osteoporosis (corticosteroids), deterioration of renal function (cyclosporine), and de novo malignancies. Recurrence of original disease is at present of clinical importance for Budd-Chiari syndrome and hepatitis B and D. Recurrence of primary biliary cirrhosis is still under discussion

Primary sclerosing cholangitis and liver transplantation

Primary sclerosing cholangitis is a chronic disease, strongly associated with ulcerative colitis and cholangiocarcinoma. Ulcerative colitis itself does not influence the liver transplant results. However; intensified screening after liver transplantation for carcinoma of the colon may be necessary. Cholangiocarcinoma, although incidentally found in hepatectomy specimens, has a bad prognosis; Initial reports in the literature indicate a far lower survival when liver transplantation is performed for PSC, in comparison to the results achieved in other transplant indications. This might have been due to surgical interventions which nowadays are avoided. Later reports show a better prognosis than the initial ones. We report on the results of liver transplantation for PSC, as indicated in the literature and on the results of the Groningen transplant centre