Budd Chiari Syndrome

Himali Meshram
• Medical management consists of anticoagulation. In patients with ascites, sodium restriction, diuretic therapy and paracentesis may be required.
• In the acute conditions, Heparin can be used to achieve anticoagulation while Warfarin can be used for long term treatment.
• The management depends on the local expertise, type of presentation, site and number of occlusions.

• When presentation is acute, catheter-directed thrombolytic therapy, angioplasty and stent placement can be effective.
• Angioplasty has high reocclusion rates. Hence, placement of stents in the IVC or hepatic veins has been recommended. But the small size of vessels in pediatric age group and complications of stent such as migration and thrombosis should be considered before the stent placement.
• Transjugular intrahepatic portosystemic shunt surgery (TIPSS) may be employed in the acute or chronic setting. However, it is still used rarely in children.
• Liver transplantation – In cases of BCS with acute fulminant liver failure or decompensated cirrhosis (albumin <3g/dl, prothrombin time 3s greater than control, and conjugated bilirubin >3mg/dl), liver transplantation is the treatment of choice.

Algorithm for management
• Step 1 Anticoagulation
• Step 2 Recanalization procedure (percutaneous angioplasty with stenting or thrombolysis)
• Step 3 TIPSS (or surgical shunt)
• Step 4 Liver transplantation

• The overall five-year survival rate is nearly 90%
• Medical management alone has varying results.
• Success with angioplasty was seen in 43% of cases, with hepatic vein stenting in 66%, whereas with TIPSS in 72% of cases(Eur J Gastroenterol Hepatol. 2016 May)
• Mortality was 3/18 following angioplasty and 8/18 following surgical shunt(Gut 1999;44:568–574)

Budd Chiari Syndrome Budd Chiari Syndrome 09/05/2018
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