Budd–Chiari syndrome (BCS) is the disease which represented by an obstruction in the hepatic venous outflow from small hepatic veins to the inferior vena cava (IVC) caused by thrombosis or fibrous sequelae. BCS is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year whose etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of IVC, and environmental conditions. The treatment for BCS contains multi-step management. Initially anticoagulation therapy has tended to be chosen. In patients without any recovery of symptom after anticoagulation therapy, interventional revascularization and the transjugular intrahepatic portosystemic shunt procedure are indicated, whereas IVC plasty with a patch graft and cavo-right atrium, portosystemic shunt is indicated for obstruction of the IVC. The patency of IVC plasty and bypass has been reported as satisfactory. Long-term outcomes after surgical procedures are sustainable but the risk of hepatocellular carcinoma occurrence has been a problem in long-term follow-up. Liver transplantation is usually indicated as a treatment in patients who develop liver failure despite undergoing various treatments.
KeywordsPortosystemic shunt Thrombectomy Radical open endovenectomy Autologous patch graft Liver transplantation
Percutaneous transluminal angioplasty
Transjugular intrahepatic portosystemic shunt
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