Management of complex patients with Budd-Chiari syndrome

Therapy for patients with Budd-Chiari syndrome is well established. For those with commonly seen localized lesions, percutaneous transluminal angioplasty or stenting is the first-line treatment. Treatment methods for severely ill patients in whom intervention has failed, or those in a poor general condition, are worth exploring. From February 2002 to July 2008, 31 patients were referred to us. Eighteen patients had a failed intervention, 4 had undergone surgery, and 10 had conservative therapy. All had intractable ascites or/and hematemesis. The procedures carried out in this series included mesocavoatrial shunt in 10 patients, radical correction in 9, mesocavojugular shunt in 7 (including 2 mesojugular shunts), mesocaval shunt in 2, cavoatrial shunt in 2 (including a revision of cavoatrial shunt), and cavojugular shunt in 1. Surgical mortality and postoperative complications were both 3.2%. Twenty-eight patients had a mean follow-up of 40 months. Outcome of follow-up was measured as excellent, good, fair, poor, and death (28.6%, 53.6%, 10.7%, 3.6%, and 3.6%, respectively). The total mortality of the group is 6.5%. After appropriate preoperative evaluation and preparation, active and cautious treatment individualized to the underlying disease may help severely ill patients with Budd-Chiari syndrome.