Hereditary hemorrhagic telangiectasia (liver manifestations)
Updates to Article Attributes
Hepatic manifestations of Hereditary haemorrhagic 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 Hereditaryhereditary haemorrhagic telangiectasia (HHT).
Epidemiology
Although it has been historically estimated in about 30% 4-5,5, new reports of the hepatic involvement in HHThereditary haemorrhagic telangiectasia indicate a higher incidence, on the order of ~ 80~80% 3.
Clinical presentation
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:
Radiographic features
Liver involvement in HHThereditary haemorrhagic telangiectasia is best assessed with multiphase CT images.
- telangiectasis
- most commonly found hepatic lesion 3
- sub centimetre 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
-<p><strong>Hepatic manifestations of Hereditary haemorrhagic telangiectasia (HHT)</strong>, which is also known as <strong>Osler-Weber-Rendu syndrome</strong>, 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 <a href="/articles/high-output-cardiac-failure">high-output cardiac failure</a>.</p><p>For a general discussion of the underlying condition, please refer to the article on <a href="/articles/hereditary-haemorrhagic-telangiectasia">Hereditary haemorrhagic telangiectasia (HHT)</a>. </p><h4>Epidemiology</h4><p>Although it has been historically estimated in about 30% <sup>4-5</sup>, new reports of the hepatic involvement in HHT indicate a higher incidence, on the order of ~ 80% <sup>3</sup>. </p><h4>Clinical presentation</h4><p>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 <sup>4</sup>:</p><ul>- +<p><strong>Hepatic manifestations of Hereditary haemorrhagic telangiectasia (HHT)</strong>, which is also known as <strong>Osler-Weber-Rendu syndrome</strong>, 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 <a href="/articles/high-output-cardiac-failure">high-output cardiac failure</a>.</p><p>For a general discussion of the underlying condition, please refer to the article on <a href="/articles/hereditary-haemorrhagic-telangiectasia">hereditary haemorrhagic telangiectasia (HHT)</a>. </p><h4>Epidemiology</h4><p>Although it has been historically estimated in about 30% <sup>4,5</sup>, new reports of the hepatic involvement in hereditary haemorrhagic telangiectasia indicate a higher incidence, on the order of ~80% <sup>3</sup>. </p><h4>Clinical presentation</h4><p>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 <sup>4</sup>:</p><ul>
-</ul><h4>Radiographic features</h4><p>Liver involvement in HHT is best assessed with multiphase CT images. </p><ul>- +</ul><h4>Radiographic features</h4><p>Liver involvement in hereditary haemorrhagic telangiectasia is best assessed with multiphase CT images. </p><ul>
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