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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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. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition.
Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.
However, jaundice and common bile duct CBD compression associated with portal hypertension had been, in fact, described by Fraser et al[ 2 ] in and by Gibson et al[ 3 ] in Hunt[ 4 ], also in , described the treatment of CBD obstruction secondary to distended venous collaterals.
The condition has also been described in patients who have non-cirrhotic portal fibrosis NCPF and cirrhosis, albeit in smaller numbers. Portosystemic shunting as a decompressive treatment for portal biliopathy was first described in by Choudhuri et al[ 11 ]. However, subsequent studies have shown that in a subset of patients the biliary obstruction is not relieved by portosystemic shunts alone and requires an additional biliary drainage procedure.
The characteristics of patients in whom the biliary obstruction is not reversed after a portosystemic shunt and the role of endoscopic management of the condition is still not clear.
In this article, we review the current data on portal biliopathy and outline the various controversies associated with its effective treatment. The anatomical basis for the condition was suggested by the works of Petren[ 12 ] and Saint[ 13 ] who described the venous anatomy of the bile ducts in and respectively.
An epicholedochal plexus of Saint forms a reticular network of veins maximum size 1 mm on the outer surface of the bile ducts.
The paracholedochal network of Petren courses parallel to the CBD and is connected to the gastric, pancreaticoduodenal and portal veins below, and to the liver above. Compression theory The first cholangiographic evidence of CBD varices compressing the bile duct was published by Williams et al[ 14 ] in In EHPVO, long standing obstruction of the portal vein leads to replacement of the portal vein by large collaterals along the CBD - the so-called cavernomatous transformation of the portal vein.
Also, with increased duration of portal thrombosis, there is vascular neogenesis and formation of tumor-like connective tissue, which can encase the CBD or cause angulation of the bile ducts[ 9 ].
In a study by Dilawari et al[ 5 ], 18 out of 20 patients had indentations suggestive of external compressions on ERCP. The reversibility of biliary tract changes after portal decompressive surgery[ 11 , 15 - 17 ] or transjugular intrahepatic portosystemic shunts TIPS [ 18 , 19 ], shown in various studies, further corroborates this mechanism. Ischaemic theory According to this theory, longstanding portal thrombosis leads to sclerosis of the veins draining the bile ducts, which in turn can lead to damage to the capillaries and arterioles.
This interruption of the vascular supply can, in turn, lead to the development of ischaemic strictures in the bile duct which are not reversed after a portosystemic shunt or TIPS. In a study by Khuroo et al[ 7 ], strictures in the CBD, both short segment and long confluent, were the most common findings seen on ERCP suggestive of an ischemic pathology.
Dhiman et al[ 20 ] studied bile duct changes after shunt surgery in 5 patients by performing ERCP pre- and post-surgery and reported complete reversal in one patient, partial reversal in three and no reversal in one patient, postulating that ischemia or scarring may be the etiology behind persistence of bile duct changes. Infective theory Infection or cholangitis was postulated by some authors in earlier studies to be the cause of jaundice in patients with portal vein thrombosis[ 15 , 21 , 22 ].
However, later cholangiographic studies have shown that changes in the biliary tract are seen even in asymptomatic patients, and cholangitis occurs late in its natural history.
Cholangitis, once present, may lead to inflammation, neogenesis and deposition of fibrous tissue, along with persistence of strictures following shunt surgeries. All the above mentioned mechanisms may be present simultaneously, resulting in the characteristic changes of portal biliopathy.
The natural history of portal biliopathy is not known. However, patients with symptomatic portal biliopathy are normally older than patients presenting with EHPVO[ 7 , 10 ], which is suggestive of long term obstruction. No evidence of malignant potential on long term follow-up exists in the literature. These symptoms may be secondary to bile duct obstruction like jaundice and pruritus, or to ductal stones like fever with chills and biliary colic.
All symptomatic patients in his study were adults and almost a decade older than the patients presenting with variceal bleeding. Sezgin et al[ 10 ] studied 10 patients with portal biliopathy who presented with jaundice, cholangitis, pruritus and abdominal pain and their mean age at presentation was 36 years, whereas other patients with EHPVO generally presented with bleeding or splenomegaly during childhood.
Their studies also suggest that portal biliopathy is a progressive condition which develops late in the course of portal hypertension and may progress to secondary biliary cirrhosis characterised by decreased serum albumin levels, ascites and a deranged coagulation time[ 1 , 9 ].
Liver function tests are the best initial investigations to identify patients who might benefit from imaging studies. A raised serum bilirubin level with a predominant increase in its direct component and an elevated serum alkaline phosphatase is an indication for performing biliary imaging.
Serum albumin levels and prothrombin time abnormalities become abnormal only after prolonged biliary obstruction when secondary biliary cirrhosis develops. Endoscopic retrograde cholangiopancreatography ERCP has been used by various authors to define portal biliopathy.
The changes seen in the bile ducts include single or multiple smooth strictures of varying length and degree, saccular dilatations, indentations, dilated intrahepatic bile duct radicles, displacement of bile ducts, clustering and pruning of intrahepatic ducts, and filling defects in the CBD which may be due to stones or varices[ 5 - 10 , 25 ]. These changes occur most commonly in the CBD and the left hepatic ducts. The differential diagnoses on cholangiography include sclerosing cholangitis, recurrent pyogenic cholangitis, CBD stones with stricture, and biliary ascariasis.
ERCP also has a therapeutic role in portal biliopathy. This includes removal of CBD stones, relief of cholangitis, and dilatation of dominant strictures with stenting. The latter is indicated only in patients not fit for surgery, or in whom shunt surgery is not feasible or has not reversed the biliopathy. Presently, ERCP is indicated only if a therapeutic intervention is required and not for diagnosis. Magnetic resonance cholangiopancreatography Due to the invasive nature of ERCP and its attendant risks, magnetic resonance cholangiopancreatography MRCP with or without magnetic resonance MR portography has become the investigation of choice for portal biliopathy.
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. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases.