Budd-Chiari syndrome surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
Therapeutic paracentesis can be used for symptomatic treatment of ascites. Paracentesis can be associated with complications such as bacterial peritonitis. The benefits of therapeutic paracentesis must be carefully weighed against its risks. 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 inferior vena cava. Recanalization of the occluded inferior vena cava 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). Transjugular Intrahepatic portosystemic shunt (TIPS) is recommended in patients who have developed liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) decreases porto-systemic gradient hence reducing the risk of variceal bleeding. 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.
Surgery
Surgical treatment options available for patients of Budd-Chiari syndrome include:[1][2][3]
Paracentesis
Therapeutic paracentesis can be used for symptomatic treatment of ascites. Paracentesis can be associated with complications such as bacterial peritonitis. The benefits of therapeutic paracentesis must be carefully weighed against its risks.
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 inferior vena cava. Recanalization of the occluded inferior vena cava 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 inferior vena cava and 91% in hepatic vein , 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 Intrahepatic 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.
References
- ↑ Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B (2015). "Diagnosis and management of Budd Chiari syndrome: an update". Cardiovasc Intervent Radiol. 38 (1): 1–12. doi:10.1007/s00270-014-0919-9. PMID 24923240.
- ↑ Segev DL, Nguyen GC, Locke JE, Simpkins CE, Montgomery RA, Maley WR, Thuluvath PJ (2007). "Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis". Liver Transpl. 13 (9): 1285–94. doi:10.1002/lt.21220. PMID 17763380.
- ↑ Fitsiori K, Tsitskari M, Kelekis A, Filippiadis D, Triantafyllou K, Brountzos E (2014). "Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome patients: results from a single center". Cardiovasc Intervent Radiol. 37 (3): 691–7. doi:10.1007/s00270-013-0697-9. PMID 23860938.