Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease)

Hepatic abnormalities include enlarged hepatic arteries, hepatic artery aneurysms, telangiectasia and arteriovenous fistulae. Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder. In the arterial phase, the liver shows a heterogeneous mosaic-like perfusion pattern due to multiple arteriovenous shunts that show different attenuations and telangiectasias.Telangiectasias are rounded hypervascular nodules more frequent in the periphery. The vascular supply of the bile ducts depends on the branches of the hepatic artery, so shunts by subtracting the arterial flow can cause ischemic cholangitis. The bile ducts dilatation it may also due to the compression of enlarged vascular structures.

Case courtesy: Dr. Luca Giampaolo

During the arterial phases, liver shows heterogeneous perfusion pattern with millimetric hypervascular images disseminated throughout the hepatic parenchyma, referring to telangiectases and arteriovenous shunts that are no longer evident during the venous phase. The early venous drainage with the simultaneous opacification of the dilated hepatic veins and the hepatic artery is evident during arterial phase. These finds are no longer evident during the venous phase. Hepatic artery enlargement: 10 mm (i.e. diameter > 6.5 mm) and portal vein enlargement: 17 mm (i.e. diameter > 13 mm). The liver and splenic size are normal. Some telangiectasias are present in the pancreas. There’s modest bile ducts dilatation.