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


METHODS
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.


CONCLUSIONS
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.
Vagefi
Parsia A
PA
Division of Transplant Surgery, University of California, San Francisco, San Francisco, CA 94143, USA. parsia.vagefi@ucsfmedctr.org
Klein
Ingo
I
Gelb
Bruce
B
Hameed
Bilal
B
Moff
Stephen L
SL
Simko
Jeff P
JP
Fix
Oren K
OK
Eilers
Helge
H
Feiner
John R
JR
Ascher
Nancy L
NL
Freise
Chris E
CE
Bass
Nathan M
NM
D034841:Abdominal Cavity; D000328:Adult; D004630:Emergencies; D005260:Female; D006392:Hemangioma, Cavernous / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006470:Hemorrhage / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000175:diagnosis; D016031:Liver Transplantation / Q000379:methods*; D014057:Tomography, X-Ray Computed