Mass-Forming Portal Biliopathy Presenting as Extreme Wall-Thickening of the Common Bile Duct

Portal biliopathy refers to biliary tree abnormalities in patients with peribiliary collateral vessels and non-neoplastic extrahepatic portal vein occlusion. These biliary abnormalities are caused by vascular compression and ischemic damage of the biliary tree, which can result in bile duct compression, stenosis, fibrotic strictures, bile duct dilation, and thickening of the bile duct wall. Portal biliopathy is difficult to distinguish from cholangiocarcinoma, IgG4-related disease, and sclerosing cholangitis. Although most patients are asymptomatic, portal biliopathy can lead to serious complications, such as recurrent cholangitis. This case illustrates the importance of including portal biliopathy in the differential diagnosis at an early stage, especially in patients with portal hypertension. With early recognition, the need for additional invasive diagnostic procedures such as biopsies is minimized. Pathogenesis, clinical presentation, diagnostics, and treatment options of portal biliopathy are described in the article.

Endoscopic Biliary Stenting for Portal Biliopathy Perforating Paracholedochal Collateral: A Rare Complication

Extrahepatic portal venous obstruction (EHPVO) usually presents with upper gastrointestinal bleed in the first decade of life. Symptomatic portal hypertensive biliopathy is seen in a minority of patients with EHPVO. With use of endoscopic intervention, biliary drainage is maintained in these patients. Various procedural complications have been linked while performing endoscopic retrograde cholangiography and stenting; however, these are managed conservatively. Here, we are highlighting a case of EHPVO with symptomatic portal biliopathy in which the patient bled from paracholedochal collateral after biliary stenting and was managed successfully with a multidisciplinary approach.

Surgical Management of Portal Biliopathy – A Prospective Analysis.

Background: Portal biliopathy denotes intrahepatic and extra hepatic biliary ductal abnormalities in portal hypertension. It is usually associated with extra hepatic portal vein obstruction (EHPVO). These patients are also prone to develop obstructive jaundice as a result of strictures and/or choledocholithiasis. Surgical management of obstructive jaundice in such patients becomes difficult in the presence of these collaterals. The aim of the study is to prospectively analyze the approach to management of patients with Symptomatic portal biliopathy. Subjects and Methods: The study was conducted at Narayana Medical College & Hospital, Chintareddy Palem, Nellore, Andhra Pradesh on surgical management of the patients of EHPVO with portal biliopathy presenting to the surgical clinic of this tertiary referral center between November 2016 and October 2017. The data was analyzed for presentation, clinical features, imaging and results of surgical management. Results: During the study period, total of 44 patients of EHPVO were referred for surgical management. Of these 14 patients (9 males, mean age 34.6 years) were diagnosed to have portal biliopathy. Ten patients had prior history of variceal bleed which was managed endoscopically. Jaundice was the most common symptom followed by right upper quadrant pain and recurrent cholangitis. Four patients had prior unsuccessful endoscopic management. ERCP/ MRCP was used for delineation of the biliary tree, which showed irregularity (14 patients), dominant strictures (8 patients), filling defects (5 patients), and intrahepatic biliary dilatation (7 patients). Proximal splenorenal shunt (PSRS) was performed in 13 patients. While in 1 patient peroperatively liver was found to be grossly nodular, hence gastro-esophageal devascularization with simultaneous biliary drainage was done. Of the 13 patients who underwent PSRS, all patients were intensively followed for 4-6 weeks with history and liver function tests. After 6 weeks, five patients showed clinical as well as biochemical improvement and they are being followed up regularly. Eight patients had persistent symptoms and abnormal liver function tests. These were the patients with dominant stricture and choledocholithiasis. Of these, 6 patients underwent Roux-en-Y hepaticojejunostomy. The average blood transfusion requirement at second surgery was 1 unit. Postoperative complications were minimal with no mortality. One patient and was lost to follow up and the remaining one is awaiting second surgery (RYHJ). Over a follow up of 3-28 months the patients are asymptomatic and well. Conclusion: Portal biliopathy with symptomatic biliary obstruction needs intervention. Surgical decompressive shunt followed by biliary drainage is the best possible treatment. While for most of the early biliary changes shunt alone is effective, patients with dominant stricture will need a biliary diversion which can be safely performed following Porto systemic shunt without increase in morbidity or mortality.