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A rare disorder caused by obstruction of the hepatic venous outflow tract resulting in hepatomegaly, portal hypertension (HTN), right upper quadrant (RUQ) abdominal pain, and ascites
- ~80% of all Budd-Chiari syndrome (BCS) cases occur in women.
- Average age of BCS patients is 30 years.
- ~85% of BCS patients have at least one prothrombotic risk factor; ~25% have two or more.
- ~40% of BCS patients also have a primary myeloproliferative disorder.
~1/100,000 worldwide per year (1)
~1.4/100,000 worldwide per year (1)
Etiology and Pathophysiology
- Reduced or obstructed hepatic venous outflow causes increased sinusoidal pressure, resulting in hepatic congestion, RUQ abdominal pain, and ascites.
- Outflow obstruction can occur anywhere from small hepatic veins to the atriocaval junction.
- Often classified as (i) primary: obstruction arising from within the venous lumen or (ii) secondary: obstruction from outside the venous lumen
- Intraluminal obstructions are typically asynchronous and progressive (1).
- Thrombosis of hepatic vein due to inherited thrombophilia or hypercoagulable states
- Myeloproliferative diseases (often V617F JAK2 mutation)
- Polycythemia vera
- Pregnancy or postpartum state
- Use of oral contraceptives
- Paroxysmal nocturnal hemoglobinuria
- Hepatocellular carcinoma (HCC)
- Antiphospholipid syndrome; prothrombin mutation
- Factor V Leiden mutation
- Protein C and S deficiencies
- Antithrombin III deficiency
- Inflammatory bowel disease
- Behçet syndrome
- Vena caval webs: more common in Asians
- Idiopathic membranous stenosis of inferior vena cava (IVC) or hepatic veins (1)
- Compression of the hepatic veins:
- Abscess or cysts; sepsis
- Status postomphalocele or gastroschisis repair
- Bush teas (pyrrolizidine alkaloids)
- Blunt abdominal trauma