Hereditary hemorrhagic telangiectasia (liver manifestations)
Citation, DOI, disclosures and article data
At the time the article was created Bruno Di Muzio had no recorded disclosures.View Bruno Di Muzio's current disclosures
At the time the article was last revised Jeremy Jones had no recorded disclosures.View Jeremy Jones's current disclosures
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