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Hepatobiliary and Pancreatic: Giant hepatomegaly from hepatic hemagiomatosis
Author(s) -
Costa JM,
Costa D,
Vieira F,
Rolanda C
Publication year - 2017
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/jgh.13769
Subject(s) - medicine , government (linguistics) , library science , philosophy , linguistics , computer science
A healthy 29-year-old man was presented with a 6-month history of abdominal discomfort. The patient had no personal or familiar prior history of relevant diseases or drug consumption. Physical examination was unremarkable, except for a massive hepatomegaly. Blood test revealed only a slight elevation of γ-glutamil transpeptidase (111UI/L; normal range: 15-85UI/L). Abdominal computed tomography (CT) was the initial imaging assessment and showed multiple nodules throughout the liver parenchyma. An abdominal magnetic resonance imaging with contrast, T2 and T1-weighed images, was then performed for further evaluation (Fig. 1). Abdominal magnetic resonance imaging depicted a giant hepatomegaly (cranial-caudal diameter: 26.7 cm) (Fig.1a), countless hepatic confluent nodular lesions with high T2-weighted signal intensity and centripetal filling in post-contrast T1-weighted images (Fig. 1b,c) suggestive of hemangiomas. The hepatic lesions also caused extrinsic compression of the stomach, hepatic and portal veins. Technetium-99 m-labelled Red Blood Cell scan (Tc99m RBC scan) and the fusion imaging of single-photon emission CT/CT (Fig. 2) confirmed hepatic hemangiomatosis and excluded extrahepatic hemangiomas. Hepatic hemangiomatosis is characterized by diffuse replacement of hepatic parenchyma with hemangiomatous lesions, possibly associated with systemic hemangiomas. It is extremely rare in adults and usually seen in neonates. Liver failure, consumptive coagulopathy, and high output heart failure are the most frequent symptoms, contributing to a poor clinical outcome. Because of its general rareness and the few cases reported of long-term adult survival, the underlying etiology, clinical course and management remain unclear. Previous reports have suggested the role of estrogen and metoclopramide medications in the development diffuse hepatic hemangiomatosis. Although, this association is not yet confirmed. After multidisciplinary discussion, our patient was referred for hepatic transplantation.