Portal biliopathy, portal ductopathy or portal cholangiopathy refers to biliary obstruction that is associated with cavernous transformation of the portal vein due to portal vein thrombosis ^1,2,8.

Clinical presentation

Portal biliopathy may present as jaundice, cholangitis due to bile duct obstruction, or rarely as hemobilia.

Pathology

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 ⁵.

There are also underlying inflammatory and ischemic changes resulting in peribiliary fibrosis, which can also result or be amplified by repeated episodes of cholangitis, a condition to which portal biliopathy predisposes ^5,6.

Classification

Portal biliopathy may be classified as ⁴: 

• varicoid: obstruction by large collaterals

• fibrotic: from intramural (epicholedochal) collaterals seen as thickened and densely enhancing bile ducts. It may rarely present as mass-like thickening of CBD ⁹

• mixed

Radiographic features

Ultrasound

Shows both the cavernous transformation of the portal vein and the dilatation of bile ducts ⁸.

MRI

MRCP features of portal biliopathy in order of frequency are as follows:

• biliary stenosis

• wavy appearance of the bile ducts ³

• angulation of the CBD

• upstream dilatation of the bile ducts

Facilitated diffusion and smooth narrowing of CBD can suggest benign portal cavernoma cholangiopathy presented as mass-like fibrotic type ⁹.

Differential diagnosis

Possible differential considerations include:

• cholangiocarcinoma

• sclerosing cholangitis

• choledocholithiasis

• IgG4-related sclerosing cholangitis ⁹

• CBD lymphoma ⁹