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World J Gastroenterol. Sep 21, 2016; 22(35): 7973-7982
Published online Sep 21, 2016. doi: 10.3748/wjg.v22.i35.7973
Portal biliopathy
Mohammad S Khuroo, Ajaz A Rather, Naira S Khuroo, Mehnaaz S Khuroo
Mohammad S Khuroo, Sher-I-Kashmir Institute of Medical Sciences, Srinagar Kashmir 190010, India
Mohammad S Khuroo, Digestive Diseases Centre, Dr. Khuroo’s medical Clinic, Srinagar, Kashmir 190010, India
Ajaz A Rather, Department of Surgery, SKIMS Medical College and Hospital, Bemina, Srinagar, Kashmir 190010, India
Naira S Khuroo, Consultant Radiology, Digestive Diseases Centre, Dr. Khuroo’s Medical Clinic, Srinagar, Kashmir 190010, India
Mehnaaz S Khuroo, Department of Pathology, Govt. Medical College, Srinagar, Kashmir 190001, India
Author contributions: All authors contributed equally; Rather AA, Khuroo NS and Khuroo MS made literature search; Khuroo NS conducted the radiological studies and critically reviewed the images; Khuroo MS and Khuroo NS wrote the paper; all authors read the paper and made necessary corrections.
Supported by Dr. Khuroo’s Medical Trust, a nonprofit organization which supports academic activities, disseminates medical education and helps poor patients for medical treatment.
Conflict-of-interest statement: The authors report no conflict of interest.
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: Mohammad S Khuroo, Director, Digestive Diseases Centre, Dr. Khuroo’s Medical Clinic, Sector 1, SK Colony, Qamarwari, Srinagar, Kashmir 190010, India. khuroo@yahoo.com
Telephone: +91-194-2492398 Fax: +91-194-2491190
Received: March 28, 2016
Peer-review started: March 29, 2016
First decision: May 12, 2016
Revised: June 22, 2016
Accepted: August 1, 2016
Article in press: August 1, 2016
Published online: September 21, 2016
Processing time: 170 Days and 14.8 Hours
Abstract

Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures (hepaticojejunostomy or choledechoduodenostomy).

Keywords: Biliary disease; Extrahepatic portal venous obstruction; Portal cavernoma; Bile duct strictures; Bile duct calculi

Core tip: Extrahepatic portal vein obstruction is often encountered in children in India. It is caused by long standing thrombosis of portal vein and leads to cavernous transformation of the bridging venous collaterals. Cholangiographic abnormalities occur in majority of such patients, however, the entity stays asymptomatic in early stages. Biliopathy is a progressive disease and patients surviving to adulthood develop more severe biliary abnormalities and present with clinical disease. Now, portal biliopathy is an important clinical entity faced by hepatologists in India. Since the disease was described in early 90’s, there have been important developments in definition, pathogenesis, diagnostic modalities and therapeutic interventions of portal biliopathy.