Treating Budd-Chiari syndrome - Making rational choices from a myriad of options

Budd-Chiari syndrome represents a spectrum of disorders characterized by obstruction to hepatic venous drainage. Originally described as an "obliterating endophlebitis of the hepatic veins," this condition has come to refer to the manifestations of hepatic venous outflow obstruction anywhere above the level of: the hepatic venulae regardless of the etiology, position, or severity of the obstruction or of the clinical course. Depending on the nature and anatomy of the obstruction, the disease presents acutely, with a rapidly progressive course, or insidiously, with gradual development of symptoms. The optimal management strategy for a given patient with Budd-Chiari syndrome depends on the anatomy of the obstruction, its physiologic consequences, and the natural history of the specific lesion. The specific treatments available and their use in the treatment of Budd-Chiari syndrome are reviewed.