Computed Tomography Imaging Findings of Hepatic Hereditary Haemorrhagic Telangiectasia

Abstract

Hereditary Haemorrhagic Telangiectasia (HHT) also known as Osler Weber Rendu disease is a rare, under diagnosed, autosomal dominant multiorgan angio dysplasia. We present a case of 61-year-old female with complaints of abdominal distension and recurrent epistaxis. Contrast Enhanced Computed Tomography (CECT) abdomen revealed arterio portal shunt, disseminated intrahepatic telangiectasias, dilated hepatic arteries, and hepatic haemangioma. Furthermore presence of cirrhosis with focal left lobar Intra Hepatic Biliary Radical Dilatation (IHBRD) suggested advanced disease. On further investigation pulmonary involvement was also identified. HHT being clinicoradiological diagnosis, knowledge of its feature is essential for its timely correct diagnosis and to obviate inadvertent biopsy.

Keywords

• arterio portal fistula
• intrahepatic
• osler weber rendu disease