Portal biliopathy (PB) is a rare disorder defined as abnormalities of the extrahepatic and intrahepatic bile ducts (usually obstruction), gallbladder and cystic duct, observed in patients with portal hypertension. ¹

The abnormalities consist mainly of bile duct compression, stenoses, fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts, as well as gallbladder varices

Pathology ¹

When portal vein thrombosis occurs multiple venous collaterals develop to bypass the obstruction resulting in portal cavernoma and peribiliary collateral vessels. The peribiliary collateral vessels cause extrinsic compression of the intrahepatic and extrahepatic bile ducts.

There are also underlying inflammatory and ischemic changes resulting in peribiliary fibrosis.

 Portal biliopathy may be classified as varicoid (obstruction by large collaterals), fibrotic (from  intramural (epicholedochal) collaterals seen as thickened and densely enhancing bile ducts) and mixed type.

Clinical presentation

Right upper quadrant pain, jaundice, pruritus and cholangitis

Differential diagnosis

• cholangiocarcinoma
• sclerosing cholangitis
• choledocholithiasis

Synonyms

Pseudosclerosing cholangitis, Non cirrhotic portal fibrosis, Extrahepatic portal venous obstruction, Proximal lienorenal shunt, Biliary obstruction, Portal cavernoma