Hemobilia due to hepatic artery aneurysm as the presenting sign of fibro-muscular dysplasia

Abstract

Fibro-muscular dysplasia (FMD) is a rare but well documented disease with multiple arterial aneurysms. The patients, usually women, present with various clinical manifestations according to the specific arteries that are affected. Typical findings are aneurysmatic dilatations of medium-sized arteries. The renal and the internal carotid arteries are most frequently affected, but other anatomical sites might be affected too. The typical angiographic picture is that of a "string of beads". Common histological features are additionally described. Here we present a case of a 47-year-old woman, who was hospitalized due to intractable abdominal pain. A routine work-up revealed a liver mass near the portal vein. Before a definite diagnosis was reached, the patient developed massive upper gastrointestinal bleeding. In order to control the hemorrhage, celiac angiography was performed revealing features of FMD in several arteries, including large aneurysms of the hepatic artery. Active bleeding from one of these aneurysms into the biliary tree indicated selective embolization of the hepatic artery. The immediate results were satisfactory, and the 5 years follow-up revealed absence of any clinical symptoms.