Budd-Chiari syndrome : reassessment of a step-wise treatment strategy

Background and study aims : The Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction. A step-wise management was recently proposed. The aim of this study is to reassess our treatment approach and long-term outcome. Patients and methods : The data of 37 Budd-Chiari patients, seen in our unit, were critically analyzed and compared with the ENVIE (European Network For Vascular Disorders of the Liver) data. Results : Most patients had multiple prothrombotic conditions (41%), of which an underlying myeloproliferative neoplasm was the most frequent (59%). The JAK2V617F mutation was associat- ed with more complete occlusion of all hepatic veins (JAK2 muta- tion + : 70% vs JAK2 mutation - : 23% and a higher severity score. The step-wise treatment algorithm used in our unit, in function of the severity of the liver impairment and the number and the exten- sion of hepatic veins occluded, resulted in the following treatments : only anticoagulation (n = 7.21%), recanalization procedure (n = 4.21%), portosystemic shunts (n = 9.26%) and liver transplanta- tion (n = 14.44%). This resulted in a 10 year survival rate of 90%. Treatment of the underlying hemostatic disorder offered a low re- currence rate. None of the 21 patients with a myeloproliferative neoplasm died in relation to the hematologic disorder.