Portal biliopathy

Portal biliopathy or portal ductopathy refers to biliary obstruction that is associated with cavernous transformation of the portal vein due to portal vein thrombosis 1.2.

It may present as jaundice, cholangitis due to bile duct obstruction, or rarely as haemobilia.

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.6

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.6,7

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.6

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

  • biliary stenosis
  • wavy appearance of the bile ducts 3
  • angulation of the CBD
  • upstream dilatation of the bile ducts

Possible differential considerations include

 

 

 

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Article information

rID: 18650
Tags: stub, cases
Synonyms or Alternate Spellings:
  • Portal biliopathies
  • Non cirrhotic portal fibrosis
  • Pseudosclerosing cholangitis
  • Extrahepatic portal venous obstruction
  • Proximal lienorenal shunt
  • Biliary obstruction
  • Portal cavernoma
  • Portal ductopathy

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Cases and figures

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    Cavernomatous tra...
    Case 1: portal biliopathy
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