Emergent orthotopic liver transplantation for hemorrhage from a giant cavernous hepatic hemangioma: case report and review

Abstract

Introduction: Cavernous 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 report: We 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.

Conclusion: Although 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.

Fig. 1

a Initial abdominal CT at presentation in 2003 showing the largest lesion (18.1 × 15.9 cm) measured at level of the right portal vein and the second largest lesion was 4.3 × 3.5 cm. b Initial chest CT in 2003 revealing innumerable pulmonary nodules. c Video-assisted thoracoscopic surgery (VATS). Diffuse hemorrhagic, purple/red, raised nodules are seen on the surface of the lung. Biopsy of these lesions revealed benign cavernous hemangiomas. d Photomicrograph of an H&E-stained section of cavernous hemangioma in a lung biopsy specimen (40×). The lung lesions were small and well-circumscribed with benign endothelial cells and thin vessel walls and septa composed predominantly of fibrous tissue.

Fig. 2

a Intra-operative photo prior to start of liver transplantation demonstrating the patient's massive abdominal distension. b Giant cavernous hepatic hemangioma occupying the entire abdominal cavity. c Posterior aspect of gross liver specimen following resection and complete decompression (the white ruler on the specimen measures 15 cm in length and is on top of the left lobe). d Intra-operative photo at the completion of liver transplantation demonstrating the patient's abdomen.