title: Hepatic involvement in hereditary hemorrhagic telangiectasia: helical computed tomography features in 24 consecutive patients.
abstract: OBJECTIVE
  Among the various organs that may be affected by hereditary hemorrhagic telangiectasia (HHT), the liver can show various degrees of vascular and parenchymal involvement. The purpose of this prospective study comprising a large series of patients was to reassess the computed tomography (CT) features of hepatic involvement in HHT using helical CT.
  
  
  METHODS
  Twenty-four consecutive patients with HHT had prospective helical CT of the liver, including noncontrast, arterial-dominant, and portal-dominant phases. The CT images were analyzed by 2 readers in consensus to determine the presence of vascular and parenchymal abnormalities. The diameter of the proper hepatic artery in these 24 patients was compared with that in 24 healthy subjects (Student t test).
  
  
  RESULTS
  Helical CT was normal in 5 patients (21%) and abnormal in 19 patients (79%). Vascular abnormalities were found in 16 patients (67%), consisting of marked dilatation of the hepatic artery (n = 16), intrahepatic telangiectases (n = 12), arteriovenous shunting (n = 5), and arterioportal shunting (n=3). The diameter of the proper hepatic artery was greater in the patients with HHT than in control subjects (6.12 +/- 2.52 mm vs. 3.29 +/- 0.65 mm, respectively; P < 0.05). Helical CT showed nodular hyperplasia in 1 patient with vascular and parenchymal abnormalities, cavernous hemangiomas in 2 patients (1 in a patient with an enlarged hepatic artery, intrahepatic telangiectases, and arteriovenous shunting and 1 in a patient with an isolated enlarged hepatic artery), and biliary cysts in 3 patients (2 biliary cysts were present in 2 patients with an enlarged hepatic artery and intrahepatic telangiectases, and 1 biliary cyst was present without any manifestations in the third patient).
  
  
  CONCLUSIONS
  Liver involvement in HHT is associated with a constellation of findings on helical CT, including significant dilatation of the proper hepatic artery, telangiectases, arteriovenous shunting, and focal liver lesions. Familiarity with these findings will result in more accurate diagnosis and allows better therapeutic options if necessary.
authors.affiliation: Department of Radiology, Hopital Lariboisière-AP-HP, Paris, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Ravard
authors.forename: Philippe
authors.initials: P
authors.lastname: Soyer
authors.forename: Mourad
authors.initials: M
authors.lastname: Boudiaf
authors.forename: Carine
authors.initials: C
authors.lastname: Terem
authors.forename: Martine
authors.initials: M
authors.lastname: Abitbol
authors.forename: Jian Fang
authors.initials: JF
authors.lastname: Yeh
authors.forename: Rémi
authors.initials: R
authors.lastname: Brouard
authors.forename: Lounis
authors.initials: L
authors.lastname: Hamzi
authors.forename: Roland
authors.initials: R
authors.lastname: Rymer
mesh_terms: D000328:Adult; D000368:Aged; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging; D015529:Choledochal Cyst / Q000000981:diagnostic imaging; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging; D005260:Female; D006392:Hemangioma, Cavernous / Q000000981:diagnostic imaging; D006499:Hepatic Artery / Q000002:abnormalities / Q000000981:diagnostic imaging; D006801:Humans; D006965:Hyperplasia; D007091:Image Processing, Computer-Assisted / Q000379:methods; D008107:Liver Diseases / Q000000981:diagnostic imaging*; D008113:Liver Neoplasms / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D011169:Portal Vein / Q000002:abnormalities; D011172:Portography; D011446:Prospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000000981:diagnostic imaging*; D036542:Tomography, Spiral Computed