Budd-Chiari Syndrome

• Clotting of the hepatic vein, the major vein that leaves the liver
• It occurs in 1 out of 100000 individuals and is more common in females.
  
• Some 10-20% also have obstruction of the portal vein.
• Most patients have an underlying condition that predisposes to blood clotting
1. 10% have polycythemia vera
2. 10% of patients with Budd-Chiari syndrome take birth control pills
• Polycythemia vera and myelodysplastic syndrome are the most frequent cause.
• Thrombophilic conditions
• Antiphospholipid syndrome .
• Paroxysmal nocturnal hemoglobinuria in about 12 % of patients; PNH can also induce a VOD-like picture.
• Rarer causes include Behçets disease - often with involvement of the inferior vena cava , granulomatous idiopathic venulitis and hypereosinophilic syndrome . The latter two conditions may be steroid responsive.
• Occasionaly tumors such as atrial myxoma, Wilms tumor and cystic or alveolar echinococcosis can mimick BCS.
• Most Common Features is ascites, classical triad of ascites, abdominal pain and hepatomegaly.

• Anticoagulation starting at the time of diagnosis, initially using low-molecular heparin (conventional heparin being used only when rapid reversal of anticoagulation was anticipated) and switching to oral agents after an average of 2 weeks.
• Interventional radiological recanalization of the hepatic veins with angioplasty or stenting for short and long stenoses, respectively.
• If this failed, TIPS insertion via a hepatic vein stump or by transcaval approach
• OLT if TIPS failed or was not technically feasible.

• In general, nearly 2/3 of patients with Budd-Chiari survive 10 years.
• Important negative prognostic indicators include ascites, encephalopathy, elevated Child-Pugh scores, elevated prothrombin time, and altered serum levels of various substances (sodium, creatinine, albumin, and bilirubin).

Category: Gastroenterology Notes