Liver Findings in Patients with Hereditary Hemorrhagic Telangiectasia.

Abstract

BackgroundHereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular dysplasia.ObjectivesThe aim is to characterize the liver involvement of HHT by multi-detector helical computed tomography.Patients and MethodsThrough the use of multi-detector helical computed tomography, scan data derived from 12 cases of HHT were retrospectively studied, and an abdominal scan was performed on all of the patients.Results(i) Three types of shunting were found in the livers, including arteriovenous (hepatic artery to hepatic vein) in 6 cases, arterioportal (hepatic artery to portal vein) in 2 cases, and portal venous (portal vein to hepatic vein) in 4 cases, (ii) Biliary diseases were detected in 8 cases, including 3 cases of bilomas accompanied by increases of alkaline phosphatase (mean 271 units, ranging from 152 to 479 units) and arteriovenous shunts, and the other 5 cases revealed slight biliary dilation with no increase of alkaline phosphates, but were instead accompanied by arteriovenous shunts (3 cases) and portal venous shunts (2 cases). (iii) Vascular anatomic variants were observed in 4 cases, including 3 with accompanying arteriovenous shunts and 1 with accompanying arterioportal shunting.ConclusionThe involved liver with HHT typically shows vascular shunting and biliary diseases. Also, arteriovenous shunts may be vulnerable to biliary diseases.