Portal hypertensive biliopathy

Case contributed by Dr Dalia Ibrahim

Presentation

Recurrent attacks of obstructive jaundice. History of portal vein thrombosis.

Patient Data

Age: 25-year-old
Gender: Male
MRI

The portal vein was not visualized separately. The portal vein showed cavernous transformation that encircled the CBD with subsequent proximal intra and extra-hepatic biliary dilation.

Concentric low-signal intensity areas surrounding the bile ducts and the gallbladder showing post contrast enhancement, suggesting epicholedochal veins and fibrosis.

The CBD is collapsed distally and surrounded by multiple collaterals.

MRCP images biliary stenosis involving the proximal CBD with proximal bile duct dilation.

Splenomegaly.

Multiple peripancreatic venous collaterals.

Normal MRI signal of the pancreas, adrenal and both kidneys.

Annotated image

The portal vein was not visualized separately. The portal vein showed cavernous transformation with multiple collaterals (blue arrows) encircled the CBD causing externsic compression and stenosis (circle on MRCP) with subsequent proximal intra and extra-hepatic biliary dilation.

Concentric low-signal intensity areas surrounding the bile ducts and the gallbladder showing post contrast enhancement, suggesting epicholedochal veins and fibrosis. (yellow arrows).

Case Discussion

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

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Case information

rID: 49938
Case created: 10th Dec 2016
Last edited: 11th Dec 2016
Inclusion in quiz mode: Included

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