Budd-Chiari syndrome: Current management options

Objective To assess the outcomes of current treatment strategies for Budd-C hiari syndrome. Summary Background Data Budd-Chiari syndrome, occlusion or obstruction of h epatic venous outflow, is a disease traditionally managed by portal or mese nteric-systemic shunting. The development of other treatment options, such as catheter-directed thrombolysis, transjugular portosystemic shunting (TIP S), and liver transplantation, has expanded the therapeutic algorithm. Methods The authors reviewed the medical records of all patients diagnosed with Budd-Chiari syndrome at the Johns Hopkins Hospital during the past 20 years. Results A total of 54 patients were identified: 13 (24%) male patients and 41 (76%) female patients, ranging in age from 2 to 76 years (median 33 year s). Twenty-one (39%) had polycythemia vera, 3 (5.6%) used estrogens, 11 (20 %) had a myeloproliferative or coagulation disorder, and in 7 (13%) the cau se remained unknown. Forty-three patients were treated with surgical shunti ng, 24 mesocaval and 19 mesoatrial. Actuarial survival rates at 1, 3, and 5 years after shunting were 83%, 78%, and 75%, respectively. Of 33 patients surviving more than 4 years, 28 (85%) had relief of clinical symptoms. Five patients required shunt revision and eight had radiologic procedures to ma intain shunt patency. Primary and secondary shunt patency rates were 46% an d 69% respectively for mesoatrial shunts and 70% and 85% respectively for m esocaval shunts. Clot lysis was successful as primary treatment in seven pa tients. TIPS was performed in three patients, one after a failed mesocaval shunt. During an average of 4 years of follow-up, these patients required m ultiple procedures to maintain TIPS patency. Six patients underwent liver t ransplantation. Of these, three had previous shunt procedures. Five of the transplant recipients are alive with follow-up of 2 to 9 years (median 6). Conclusions Both shunting and transplantation can result in a 5-year surviv al rate of at least 75%, and other treatment modalities may be appropriate for highly selected patients. Optimal management requires that treatment be directed by the predominant clinical symptom (liver failure or portal hype rtension) and anatomical considerations and be tempered by careful assessme nt of surgical risk.