A rare cause of hepatic arteriovenous fistula: Hereditary hemorrhagic telangiectasia

A 71-year-old man, who provided consent for this publication, experiencing frequent nosebleeds was diagnosed with anemia 10 years ago. Physical examination revealed the presence of dilated capillaries in the nasal mucosa. Laboratory tests indicated a hemoglobin level of 63 g/L and a prolonged prothrombin time of 14.3 seconds. Maximum projection density on abdominal computed tomography angiography revealed a significant thickening of the celiac trunk, common hepatic artery, and innominate hepatic artery, with a tortuous and malformed course (A). Cinematic rendering and volume rendering revealed a notable thickening of the inferior vena cava. The portal vein was markedly thickened and its course was tortuous and malformed (B/Cover). Digital subtraction angiography revealed a marked thickening of the hepatic arteries and the formation of a fistula between the left hepatic artery and vein. Chest computed tomography scan also showed a pulmonary arteriovenous malformation in the patient's left lung. The patient was treated with spring coil interventional embolization of the left hepatic artery-left hepatic vein fistula (C). The patient symptoms were relieved after interventional treatment. Owing to recurrent epistaxis, skin and mucosal telangiectasia, and multiple hepatic venous malformations, the diagnostic criteria for hereditary hemorrhagic telangiectasia, a rare vascular disorder caused by multiple genetic abnormalities, were determined.1Kritharis A. Al-Samkari H. Kuter D.J. Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist's perspective.Haematologica. 2018; 103: 1433-1443Crossref PubMed Scopus (125) Google Scholar,2Chiang C.M. Chiang H.C. Chiu H.C. An uncommon cause of hemobilia.Gastroenterology. 2024; PubMed Google Scholar Treatment depends on the organs involved and the severity of the symptoms; affected patients may have involvement of the lungs, brain, gastrointestinal, and hepatic systems. Interventional embolization is effective against visceral vascular malformations and bleeding in patients with hereditary hemorrhagic telangiectasia. The interventional therapy aimed to prevent bleeding and right-sided heart failure, and decrease cardiac output, decreases clinical symptoms such as weakness and fatigue in patients. None.