Budd-Chiari syndrome surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Surgery
Interventional therapy
- The choice of interventional procedures in the treatment of BCS is based on the type of venous occlusion.
- Aspiration thrombectomy followed by predilatation with a small diameter catheter. This is associated with a decreased risk of pulmonary embolism and allows the introduction of a thrombolytic catheter for administration of a thrombolytic agent.
- Endovascular treatment is indicated in patients with thrombosed IVC. Recanalization of the occluded IVC results in rapid clinical improvement.
- Angioplasty is recommended for patients with occluded hepatic vein and patent inferior vena cava.
- Stenting is preferred if the hepatic vein has a straight course and is of sufficient diameter (≥7 mm).
- The hepatic vein is usually accessed through the internal jugular vein. Other options include percutaneous transhepatic approach or access through the femoral vein. In acute thrombosis, thrombolytic therapy alone has good results.
- Interventional treatment with stenting has a long-term patency of 97% in IVC and 91% in HV, but angioplasty alone has a high rate of restenosis.
- Anticoagulant therapy that has to be maintained for at least 6 months after the intervention. Restenosis occurs after stenting, is usually due to inadequate anticoagulant therapy.
- Transjugular portosystemic shunt (TIPS) is recommended in patients who have developed liver cirrhosis. transjugular
portosystemic shunt (TIPS) decreases porto-systemic gradient hence reducing the risk of variceal bleeding.
- The use of a TIPS stent covered with polytetrafluoroethylene (ePTFE) or TIPS stent-graft improves longterm patency rates compared to bare stents.
Liver Transplantation
- Liver transplantation is the last rescue treatment when conservative and interventional therapy does not prevent the development of liver cirrhosis and progressive liver failure in chronic BCS.
- It is generally reserved for patients with fulminant hepatic failure, failure of shunts, or progression of cirrhosis that reduces the life expectancy to 1 year.
- Requires careful evaluation of indication for liver transplantation. patients presenting with fulminant hepatic failure, it is an urgent indication.
- Patients after liver transplantation require life-long immunosuppressive therapy and long-term anticoagulation therapy.
- The most common complications of transplant include rejection, arterial or venous thromboses, and bleeding due to anticoagulation. Up to 10% of patients may have a recurrence of Budd-Chiari syndrome after the transplant.