HEPATIC VASCULAR MALFORMATIONS IN HEREDITARY HEMORRHAGIC TELANGIECTASIA - IMAGING FINDINGS

Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disease is an autosomal dominant disorder characterized by telangiectases and arteriovenous malformations (AVM) of skin, mucosa, and potentially every organ. The reported prevalence of the disorder is 1/100,000-2/100,000; hepatic involvement occurs in 8-31% of cases (almost always defined by clinical criteria). Hepatic involvement is shown by examination of biopsy and necropsy specimens [1] and by angiography [2]. More recently, hepatic vascular malformations have been detected by using sonography [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 illustrates the broad spectrum of abnormalities of hepatic vessels and collaterals in HHT that are detectable by imaging techniques even in the early or clinically silent stages of the disease.