Hereditary hemorrhagic telangiectasia (liver manifestations)
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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 Hereditary haemorrhagic telangiectasia (HHT).
Epidemiology
Although it has been historically estimated in about 30% 4-5, new reports of the hepatic involvement in HHT indicate a higher incidence, on the order of ~ 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 HHT 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>- +<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>
- +<li><a href="/articles/high-output-cardiac-failure">high-output cardiac failure</a></li>
- +<li><a href="/articles/portal-hypertension">portal hypertension</a></li>
- +<li>
- +<a href="/articles/hepatic-encephalopathy">portosystemic encephalopathy</a> </li>
- +<li><a href="/articles/cirrhosis">cirrhosis</a></li>
- +</ul><h4>Radiographic features</h4><p>Liver involvement in HHT is best assessed with multiphase CT images. </p><ul>
- +<li>telangiectasis<ul>
- +<li>most commonly found hepatic lesion <sup>3</sup>
- +</li>
- +<li>sub centimetre hypervascular focal lesions resembling an asterisk </li>
- +<li>
- +<a href="/articles/maximum-intensity-projection">MIP reformats</a> from CT arterial phase images are extremely helpful in demonstrating them </li>
- +<li>large confluent vascular masses: term described to refer to multiple coalescing telangiectases that form vascular mass-like lesions <sup>3</sup>
- +</li>
- +</ul>
- +</li>
- +<li>hepatic perfusion abnormalities<ul>
- +<li>heterogeneous attenuation enhancement pattern of the liver parenchyma, which tends to be more diffuse (nonfocal) and ill-defined <sup>3</sup>
- +</li>
- +<li>
- +<a href="/articles/transient-hepatic-attenuation-differences">transient hepatic attenuation differences (THAD)</a> may be present</li>
- +</ul>
- +</li>
- +<li>arteriovenous shunts: communication between the <a href="/articles/hepatic-artery-proper">hepatic artery</a> and <a href="/articles/hepatic-veins">hepatic vein</a><ul>
- +<li>hepatic vein filling with contrast on arterial phase</li>
- +<li>enlarged hepatic artery and hepatic veins due to high-output shunting</li>
- +<li>associated with telangiectasis <sup>3</sup>
- +</li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/intrahepatic-arterioportal-shunt">arterioportal shunts</a>: communication between the hepatic artery and <a href="/articles/portal-vein">portal vein</a> <ul>
- +<li>portal vein opacification on arterial phase </li>
- +<li>associated with THAD</li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/congenital-portosystemic-shunt">portovenous shunts</a>: communication between the portal and hepatic veins<ul>
- +<li>rarely seen in HHT <sup>3</sup>
- +</li>
- +<li>dilated portal vein branch bridging to a hepatic vein</li>
- +<li>best seen on portal venous phase </li>
- +<li>occult at angiography <sup>3</sup>
- +</li>
- +</ul>
- +</li>
- +</ul>
References changed:
- 4. M. Memeo, A. A. Stabile Ianora, A. Scardapane, P. Buonamico, C. Sabbà, G. Angelelli. Hepatic involvement in hereditary hemorrhagic telangiectasia:. (2004) Abdominal Imaging. 29 (2): 211. <a href="https://doi.org/10.1007/s00261-003-0101-3">doi:10.1007/s00261-003-0101-3</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15290948">Pubmed</a> <span class="ref_v4"></span>
- 5. P Bhargava, S Vaidya, O Kolokythas, D S Katz, M Dighe. Hepatic vascular shunts: embryology and imaging appearances. (2014) The British Journal of Radiology. 84 (1008): 1142-52. <a href="https://doi.org/10.1259/bjr/82649468">doi:10.1259/bjr/82649468</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22101582">Pubmed</a> <span class="ref_v4"></span>
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