Hereditary hemorrhagic telangiectasia (liver manifestations)

Last revised by Jeremy Jones on 20 Sep 2021

Hepatic manifestations of Hereditary hemorrhagic telangiectasia (HHT), which is also known as Osler-Weber-Rendu syndrome, are typically seen on imaging as multiple telangiectasias and arteriovenous malformations (i.e. arteriovenous and portovenous shunts). These multiple shunts lead to a hyperdynamic circulation and therefore, possible high-output cardiac failure.

For a general discussion of the underlying condition, please refer to the article on hereditary hemorrhagic telangiectasia (HHT)

Although it has been historically estimated in about 30% 4,5, new reports of the hepatic involvement in hereditary hemorrhagic telangiectasia indicate a higher incidence, on the order of ~80% 3

Slightly over half of the patients are asymptomatic. Symptoms tend to develop when there are extensive vascular shunts causing a hyperdynamic circulation; these may include symptoms due to secondary 4:

Liver involvement in hereditary hemorrhagic telangiectasia is best assessed with multiphase CT images. 

  • telangiectasis
    • most commonly found hepatic lesion 3
    • sub centimeter hypervascular focal lesions resembling an asterisk 
    • MIP reformats from CT arterial phase images are extremely helpful in demonstrating them  
    • large confluent vascular masses: term described to refer to multiple coalescing telangiectases that form vascular mass-like lesions 3
  • hepatic perfusion abnormalities
  • arteriovenous shunts: communication between the hepatic artery and hepatic vein
    • hepatic vein filling with contrast on arterial phase
    • enlarged hepatic artery and hepatic veins due to high-output shunting
    • associated with telangiectasis 3
  • arterioportal shunts: communication between the hepatic artery and portal vein 
    • portal vein opacification on arterial phase 
    • associated with THAD
  • portovenous shunts: communication between the portal and hepatic veins
    • rarely seen in HHT 3
    • dilated portal vein branch bridging to a hepatic vein
    • best seen on portal venous phase 
    • occult at angiography 3

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