The Budd-Chiari syndrome revisited

Major advances in diagnosis and treatment of the Budd-Chiari syndrome over the past decade have made it worthwhile to revisit this intriguing condition. During this time it has become evident that an underlying predisposing cause, in particular a hematological, thrombogenic disorder, is present in up to 70% of patients presenting with hepatic venous outflow obstruction. Doppler ultrasound has proven extremely valuable as the initial imaging modality to establish the presence or absence of normal hepatic veins and phasic flow. Angiography with vena caval pressure measurements and liver biopsy remain essential in decision making with regard to surgical and nonsurgical management. Nonsurgical management has expanded to include angioplasty of stenoses and webs, although the placement of transjugular intrahepatic portosystemic shunts offers a useful alternative to operative shunts in selected circumstances. In many situations, however, decompressive side-to-side type shunts have a definitive role in patients with preserved liver function and structure. Other patients with severe necrosis, fibrosis, or cirrhosis may be offered a life-saving alternative only by orthotopic liver transplantation.