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Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Dec 7, 2016; 22(45): 9909-9920
Published online Dec 7, 2016. doi: 10.3748/wjg.v22.i45.9909
Therapeutic approaches for portal biliopathy: A systematic review
Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo
Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy
Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Multivisceral Transplant Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy
Author contributions: Franceschet I and Senzolo M designed and drafted the manuscript; Franceschet I, Zanetto A and Ferrarese A contributed to the literature search; Burra P and Senzolo M revised the final manuscript.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Marco Senzolo, MD, PhD, Multivisceral Transplant Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, Via Giustiniani 2, 35128 Padua, Italy. marcosenzolo@hotmail.com
Telephone: +39-4-98218726 Fax: +39-4-98760820
Received: August 26, 2016
Peer-review started: August 29, 2016
First decision: September 20, 2016
Revised: October 12, 2016
Accepted: November 14, 2016
Article in press: November 16, 2016
Published online: December 7, 2016
Processing time: 102 Days and 12.8 Hours
Abstract

Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.

Keywords: Portal biliopathy; Portal cavernoma; Magnetic resonance cholangiopancreatography; Endoscopic retrograde cholangiopancreatography; Porto-systemic shunt

Core tip: Portal biliopathy in patients with portal vein thrombosis and portal cavernoma can be symptomatic in about 5%-38% of patients. Therapy includes endoscopic and surgical approaches aimed to improve both portal hypertension and biliary alterations and clinical manifestation. Usually, multiple and combined treatments are required to resolve portal biliopathy.