Liver Involvement in Hereditary Hemorrhagic Telangiectasia: CT and Clinical Findings Do Not Correlate in Symptomatic Patients

Abstract

Patients with symptomatic HHT liver disease have diffuse hepatic telangiectases, a dilated common hepatic artery, and a high incidence of biliary abnormalities. Multiphasic CT is useful in diagnosing liver involvement due to HHT; however, no strong correlation was seen between CT findings and the clinical subtype of HHT liver disease.

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

“…Portal vein to hepatic vein shunts predispose to hepatic encephalopathy manifested by confusion and asterixis [41,42]. CT scanning generally identifies these vascular abnormalities, but does not specifically predict the predominant clinical manifestations in individual patients [44]. Although most patients initially manifest one symptom type, clinical overlap can occur with the development of biliary ischaemia or hepatic encephalopathy in heart failure patients [41].…”

Adults with genetic syndromes and cardiovascular abnormalities: clinical history and management

“…Fortunately, few of these lesions become symptomatic, 171 though depending upon the nature of the aberrant connections, some patients develop hepatic encephalopathy from reduced effective portal flow through the liver, whereas others develop high-output cardiac failure. 172 Embolization of hepatic vascular malformations is rarely an option because of the risk of fulminant hepatic infarction.…”

Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients

“…Intrahepatic shunts included arteriovenous shunts between hepatic artery and hepatic veins (which were considered to be present in case of early opacification of the hepatic veins during the arterial phase) [9,16], arterioportal shunts between hepatic artery and portal vein (which were considered to be present in case of early opacification of the branches of the portal vein during the arterialdominant phase) [6,9], and portovenous shunts between portal vein and hepatic vein (which were considered to be present in case of dilated portal branch communicating with an enlarged hepatic vein during the portal phase) [9,16]. The presence of areas of intrahepatic telangiectasia (which were considered to be present in case of star-shaped appearance of the distal intraparenchymal vessels and/or multiple extravascular intraparenchymal foci of hyperenhancement with a diameter <10 mm and predominantly in a peripheral location) was noted [6,7,9,14].…”

“…Liver involvement in HHT includes suggestive and non-specific abnormalities. Suggestive hepatic abnormalities include enlarged hepatic arteries, hepatic artery aneurysms, and telangiectasia and arteriovenous fistulae [5][6][7][8]. Non-specific lesions include indirect signs of portal hypertension, perfusion disorders, cirrhosis, fibrosis, bile duct dilatation and focal nodular parenchymal lesions [6,[9][10][11].…”

“…In addition, substantial improvements in helical technology have been achieved since the most recent studies reporting helical CT appearance of hepatic and pancreatic involvement by HHT were initiated [6,7,12,14,16]. In this regard, helical CT systems allow now the abdomen and the pelvis to be imaged at a resolution of less than 1 mm so that resulting voxels are submillimetre and isotropic [17].…”