[Budd-Chiari syndrome and Behçet's disease. A case treated by mesenterico-atrial prosthesis]

A 36 year-old North African man, with Behçet's syndrome complicated by an inferior vena caval thrombosis, developed a chronic Budd-Chiari syndrome associated with bleeding esophageal varices. He was treated by an emergency mesoatrial shunt. Results at 2 years were good. Analysis of this case and the 13 other similar cases with associated Budd-Chiari syndrome and Behçet's syndrome found in the literature showed that hepatic veins thrombosis: a) is often due to inferior vena caval thrombosis or membranous obstruction; b) has a high spontaneous mortality rate by acute liver failure; c) remains a potential indication for porto-systemic shunt, as are other causes of Budd-Chiari syndrome.