title: Intrahepatic portal cavernoma as an indication for liver transplantation.
abstract: Cavernous transformation of the portal vein (portal cavernoma) consists of a periportal or/and intrahepatic venous collateral network, developed as a result of acute or long-standing portal vein thrombosis. Better control of hemorrhagic and thrombotic complications in the patients with portal cavernoma substantially improves their life span and the clinical outcome. However, biliary complications that occur in the late stages of this disease have been recently recognized as challenging management issues because they recur and are difficult to treat. Because of the relatively small number of the patients with cholangiopathy due to portal cavernoma, there is no current standardized treatment approach. We report the case of a predominantly intrahepatic portal cavernoma occurring in a patient with chronic idiopathic portal vein thrombosis, which led to severe cholangiopathy that mimicked primary sclerosing cholangitis and cholangiocarcinoma, was unresponsive to endoscopic stent placement, and finally required liver transplantation.
authors.affiliation: Department of Pathology, New York University School of Medicine, New York, NY 10016, USA.
authors.forename: Cristina H
authors.initials: CH
authors.lastname: Hajdu
authors.forename: Takahiro
authors.initials: T
authors.lastname: Murakami
authors.forename: Thomas
authors.initials: T
authors.lastname: Diflo
authors.forename: Bachir
authors.initials: B
authors.lastname: Taouli
authors.forename: Jordan
authors.initials: J
authors.lastname: Laser
authors.forename: Lewis
authors.initials: L
authors.lastname: Teperman
authors.forename: Lydia M
authors.initials: LM
authors.lastname: Petrovic
mesh_terms: D000328:Adult; D006392:Hemangioma, Cavernous / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D011169:Portal Vein / Q000473:pathology / Q000601:surgery*; D015607:Stents; D016896:Treatment Outcome