PMID: 9187882May 1, 1997Paper

Poor prognosis and limited therapeutic options in patients with Budd-Chiari syndrome and portal venous system thrombosis

European Journal of Gastroenterology & Hepatology
A E MahmoudE Elias

Abstract

Therapeutic options in Budd-Chiari syndrome (BCS) are highly 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. 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 patients were female and three male with a mean age at presentation of 42 years (range 32-67). An underlying haematological aetiology was identified in 10 of the 13 patients. Only four patients (31%) were anticoagulated 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. The presentation was acute in three patients, subacute in six and chronic in four with a high incidence of encephalopathy (6/13; 46%). Treatment included liver transplantation (four), mesoatrial shunt (one) and balloon dilatation of hepatic veins (two). Six patients were treated medically as all o...Continue Reading

Citations

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