Hepatic Hereditary Hemorrhagic Telangiectasia
Authors: Daniel Y. Chung, Michael P. Federle, John P. Barrett, David A. Sass
Journal: Clinical Gastroenterology and Hepatology (2006)

A 31-year-old woman underwent an exploratory laparoscopy for evaluation of chronic pelvic pain. She was found to have mild endometriosis; however, an incidental finding was that intraoperatively the liver was noted to be abnormal, with nodularity and discrete telangiectasias noted throughout the entire surface (Figure A). A triphasic computed tomography scan of the abdomen showed heterogeneous enhancement of hepatic parenchyma, premature opacification of hepatic veins (striped arrows), and dilated tortuous arterial branches (solid arrow) in the arterial phase (Figure B). There was also marked enlargement of the hepatic artery (Figure C). The patient related a history of frequent episodes of epistaxis and was diagnosed with hereditary hemorrhagic telangiectasia (HHT) with hepatic involvement.View Large Image Figure ViewerDownload (PPT)View Large Image Figure ViewerDownload (PPT)HHT, also known as Osler-Weber-Rendu disease, is characterized by angiodysplastic lesions affecting the skin, lungs, brain, and gastrointestinal tract.1Garcia-Tsao G. Korzenik J.R. Young L. et al.Liver disease in patients with hereditary hemorrhagic telangiectasia.N Engl J Med. 2000; 343: 931-936Crossref PubMed Scopus (310) Google Scholar Up to one third of patients with HHT have hepatic involvement. Shunting from the hepatic artery to the portal vein might lead to portal hypertension, whereas hepatic artery to hepatic vein shunting might lead to high-output right-sided heart failure and hypoperfusion of the liver parenchyma and biliary tree.2Larson A.M. Liver disease in hereditary hemorrhagic telangiectasia.J Clin Gastroenterol. 2003; 36: 149-158Crossref PubMed Scopus (54) Google Scholar Treatment is generally supportive, although severe cases of hepatic HHT might require liver transplantation. As in this case, 30%–50% of patients with liver involvement remain asymptomatic. A 31-year-old woman underwent an exploratory laparoscopy for evaluation of chronic pelvic pain. She was found to have mild endometriosis; however, an incidental finding was that intraoperatively the liver was noted to be abnormal, with nodularity and discrete telangiectasias noted throughout the entire surface (Figure A). A triphasic computed tomography scan of the abdomen showed heterogeneous enhancement of hepatic parenchyma, premature opacification of hepatic veins (striped arrows), and dilated tortuous arterial branches (solid arrow) in the arterial phase (Figure B). There was also marked enlargement of the hepatic artery (Figure C). The patient related a history of frequent episodes of epistaxis and was diagnosed with hereditary hemorrhagic telangiectasia (HHT) with hepatic involvement. HHT, also known as Osler-Weber-Rendu disease, is characterized by angiodysplastic lesions affecting the skin, lungs, brain, and gastrointestinal tract.1Garcia-Tsao G. Korzenik J.R. Young L. et al.Liver disease in patients with hereditary hemorrhagic telangiectasia.N Engl J Med. 2000; 343: 931-936Crossref PubMed Scopus (310) Google Scholar Up to one third of patients with HHT have hepatic involvement. Shunting from the hepatic artery to the portal vein might lead to portal hypertension, whereas hepatic artery to hepatic vein shunting might lead to high-output right-sided heart failure and hypoperfusion of the liver parenchyma and biliary tree.2Larson A.M. Liver disease in hereditary hemorrhagic telangiectasia.J Clin Gastroenterol. 2003; 36: 149-158Crossref PubMed Scopus (54) Google Scholar Treatment is generally supportive, although severe cases of hepatic HHT might require liver transplantation. As in this case, 30%–50% of patients with liver involvement remain asymptomatic.