HEPATIC VASCULAR MALFORMATIONS IN HEREDITARY HEMORRHAGIC TELANGIECTASIA - IMAGING FINDINGS

Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disea se is an autosomal dominant disorder characterized by telangiectases a nd arteriovenous malformations (AVM) of skin, mucosa, and potentially every organ. The reported prevalence of the disorder is 1/100,000-2/10 0,000; hepatic involvement occurs in 8-31% of cases (almost always def ined by clinical criteria). Hepatic involvement is shown by examinatio n of biopsy and necropsy specimens [1] and by angiography [2]. More re cently, hepatic vascular malformations have been detected by using son ography [3], Doppler sonography [4, 5], CT [6], and MR imaging [7]. In almost all the cases described, the malformations were detected in a late stage of hepatic vascular derangement. This pictorial essay illus trates the broad spectrum of abnormalities of hepatic vessels and coll aterals in HHT that are detectable by imaging techniques even in the e arly or clinically silent stages of the disease.