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24th International Meeting Abstracts: POSTER SESSIONS: Liver: Miscellaneous

Diffuse Liver Hemangiomatosis: An Atypical Liver Transplantation Indication in Adult

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Garcia, S.1; Caso, O.1; Calvo, J.1; Fakih, N.1; Justo, I.1; Garcia-Sesma, A.1; Alvaro, E.1; Alegre, C.1; Abradelo, M.1; Manrique, A.1; Garcia, M.1; Cambra, F.1; Loinaz, C.1; Olivares, S. P.1; Sanabria, R.1; Moreno, E.1; Jimenez, C.1

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Introduction: Patients with diffuse liver hemangiomatosis (DLH) showed symptoms of abdominal pain and a palpable abdominal mass. The natural history and its etiology are uncertain, although in the literature have been cited the role of steroids and metoclopramide. DLH usually occurs in neonates characterized by hemangiomas of the skin and involvement of at least two visceral organs. DLH without extrahepatic lesions is extremely rare in adults. The prognosis is still unclear because it occurs so rarely and has variable clinical courses in affected patients. When patients became symptomatic, liver transplantation is a good choice of treatment if surgical resection can not be performed.

Material and methods: We present 2 patients who underwent orthotopic liver transplantation (OLT) for diffuse liver hemangiomatosis. The first one is a 50 years old woman, who showed a 2-year history of progressive abdomen distention and abdominal pain. Firstly, she was diagnosed of a giant liver hemangioma. Left lobe hepatectomy was performed without incidences. Later, the patient developed a DLH with compressive symptoms, so liver transplantation from brain death donor was performed. Postoperative course was uneventful, and after 8 years there has been no incidences. The second case is a 41 year old patient who presented painless hepatomegaly, coagulopathy and thrombocytopenia. DLH with portal vein thrombosis was diagnosed in imaging tests, and the patient is currently on the waiting list for liver transplantation.

Conclusions: Difusse liver hemangiomatosis without extrahepatic lesions is extremely rare in adults. The presence of symptoms suggesting extrinsic compression of adjacent structures should be considered for surgical resection. Liver transplantation must be indicated in patients with symptomatic and unresectable disease.

© 2012 by Lippincott Williams & Wilkins