Hereditary hemorrhagic telangiectasia (liver manifestations)
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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.
- 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
- heterogeneous attenuation enhancement pattern of the liver parenchyma, which tends to be more diffuse (nonfocal) and ill-defined 3
- transient hepatic attenuation differences (THAD) may be present
- 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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