These changes are believed to be caused by the pressure of the venous collaterals on both the intrahepatic and extrahepatic bile ducts and the gallbladder. However, once symptoms ensue, the course of illness becomes tenacious, with the progressive worsening necessitating biliary decompression, shunt surgery, or even liver transplantation in terminal stages. This is a preview of subscription content, log in to check access. References 1.
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Supported by Dr. Published by Baishideng Publishing Group Inc. All rights reserved. This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. This article has been cited by other articles in PMC. 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. Biliary abnormalities do occur in patients with cirrhosis and idiopathic portal hypertension.
These are primarily in the intrahepatic bile ducts and are caused by hepatic nodularity and fibrosis rather than portal cavernoma[ 4 ]. Some investigators have suggested that such cholangiographic abnormalities should also be defined as portal biliopathy.
However, there is a general consensus that portal biliopathy should be restricted to those cholangiographic abnormalities which are caused by portal cavernoma. Other biliary diseases which may superficially resemble portal biliopathy and need to be excluded include choledocholithiasis, primary sclerosing cholangitis, biliary parasitosis, AIDS cholangiopathy, oriental-cholangio-hepatitis and cholangiocarcinoma etc.
In , we were confronted with biliary disease in two patients of portal cavernoma[ 5 ]. Both patients exsanguinated and died at elective gall bladder surgery. We searched the literature around that time and could not find well-documented reports of biliary disease with extrahepatic portal venous obstruction EHPVO.
From December to November , we[ 3 ] prospectively studied 21 consecutive such patients for evidence of biliary tract disease. Cholangiographic abnormalities were detected in 17 Three adult patients had clinically manifest biliary disease. The pathogenesis of these changes and their relationship with portal cavernoma was critically evaluated. Another study reported on bile duct changes in 20 patients with portal cavernoma[ 6 ].
Portal biliopathy has been described by a multitude of names in literature. Dhiman et al[ 16 ] named it portal hypertensive biliopathy, while others have used terms extrahepatic portal biliopathy[ 23 ], vascular biliopathy[ 24 ], portal ductopathy, portal cholangiopathy[ 25 ] and portal cavernoma cholangiopathy[ 1 ] to describe this entity.
However, authors believe that portal biliopathy suggested by Sarin et al[ 7 ] is appropriate term to reflect the cholangiographic abnormalities in this entity. Table 1 Reported studies of portal biliopathy Ref.
Author information Article notes Copyright and License information Disclaimer Somnath Chattopadhyay, Samiran Nundy, Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi , India Author contributions: Chattopadhyay S contributed to the conception and design of the editorial, along with the writing of the manuscript; Nundy S contributed to the conception and design of the editorial, along with critically revising it for important intellectual content and gave final approval of the version to be published. Correspondence to: Dr. All rights reserved. This article has been cited by other articles in PMC.