POOR-PROGNOSIS AND LIMITED THERAPEUTIC OPTIONS IN PATIENTS WITH BUDD-CHIARI SYNDROME AND PORTAL VENOUS SYSTEM THROMBOSIS

Objective: Therapeutic options in Budd-Chiari syndrome (BCS) are highl y dependent on the site and extent of hepatic vein thrombosis. The aim of this study was to evaluate the effect of additional portal venous system thrombosis on the clinical presentation, treatment and outcome in patients with BCS. Patients: Clinical notes of 51 patients with BCS admitted to our centre were evaluated. We identified 13 patients (25% ) with BCS and additional portal venous system thrombosis. Ten patient s were female and three male with a mean age at presentation of 42 yea rs (range 32-67). Results: An underlying haematological aetiology was identified in 10 of the 13 patients. Only four patients (31%) were ant icoagulated before referral to our centre. In addition to hepatic vein thrombosis seven patients had portal vein thrombosis (PVT), three had PVT, splenic vein (SV) and superior mesenteric vein (SMV) thrombosis and three had either SV, SMV or inferior vean cava (IVC) thrombosis. T he presentation was acute in three patients, subacute in six and chron ic in four with a high incidence of encephalopathy (6/13; 46%). Treatm ent included liver transplantation (four), mesoatrial shunt (one) and balloon dilatation of hepatic Veins (two). Six patients were treated m edically as all other options were considered too risky or technically impossible. Nine of 13 patients (70%) died either after surgery or be fore any treatment could be instituted (median survival 1 month), comp ared to 14/38 (37%) in patients with isolated hepatic vein thrombosis (median survival 6.3 years). Conclusion: We conclude that patients wit h BCS and portal venous system thrombosis constitute a unique group wi th limited therapeutic options and poor prognosis. The importance of e arly recognition and anticoagulation of patients with BCS is emphasize d.