Intrahepatic Osler's disease: report of two cases and review of the literature
Arfa, M.N.; Bouzaiene, H.; Ben Farhat, L.; Gharbi, L.; Ghariani, B.; Mestiri, H.; Hendaoui, L.; Khalfallah, M.T.
Hepato-Gastroenterology 50 Suppl. 2: Ccx-Ccxiii
2003
ISSN/ISBN: 0172-6390 PMID: 15244181 Document Number: 560064
Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.