Portal Biliopathy

Portal biliopathy is bile duct and gallbladder abnormalities caused by compression or ischaemia from collateral veins of portal cavernous transformation.

• Reported in 80 to 100% of patients with extrahepatic portal vein obstruction on cholangiography, but symptomatic in 5 to 30%.
• Mechanism: paracholedochal and epicholedochal venous plexuses form collaterals, compress the common bile duct; ischaemic stricturing follows.
• Findings: bile duct wall irregularity, extrinsic indentations, strictures, stones (secondary), upstream dilatation.
• Pattern classification (Chandra): types I to III by involvement of extrahepatic and intrahepatic ducts. Left hepatic duct involvement is common.
• Complications: cholestasis, choledocholithiasis, recurrent cholangitis, secondary biliary cirrhosis.

• Most are asymptomatic and found on imaging.
• Jaundice, biliary pain, cholangitis, pruritus, pancreatitis.
• Features of portal hypertension: variceal bleeding, splenomegaly.
• Symptoms often appear after many years (mean 10 to 20 years of portal vein obstruction).

• Liver tests: raised alkaline phosphatase and GGT, bilirubin in symptomatic cases.
• MRCP with MR portography is the investigation of choice: wall irregularity, strictures, dilatation, filling defects from stones, collaterals.
• Endoscopic ultrasound: paracholedochal varices, wall thickening.
• Doppler and CT venography to document cavernoma.
• ERCP is reserved for therapy because of risk of haemorrhage from varices; diagnostic cholangiography shows pseudo cholangiocarcinoma sign.
• Exclude cholangiocarcinoma, primary sclerosing cholangitis, choledocholithiasis from other causes, chronic pancreatitis.

• Asymptomatic: observation.
• Symptomatic: ursodeoxycholic acid 13 to 15 mg/kg daily; antibiotics for cholangitis (ceftriaxone 2 g daily with metronidazole 500 mg three times daily, adjusted to cultures).
• Endoscopic therapy: ERCP with sphincterotomy, stone extraction, balloon dilatation and biliary stenting (multiple plastic stents with exchange every 3 months, or fully covered metal stents) for strictures. Bleeding risk is lower with endoscopic papillary balloon dilatation.
• Portal decompression: portosystemic shunts (surgical or TIPS) can relieve compression and improve biliary changes in 50 to 70%, particularly early in the course.
• Biliary drainage surgery (choledochojejunostomy, hepaticojejunostomy) for refractory strictures in non shuntable patients, with high morbidity because of collaterals.
• Treat portal hypertension (varices) as for cavernoma.
• Long term surveillance for recurrent cholangitis and stones.