Budd-Chiari Syndrome

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Basics

Description

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

Epidemiology

  • ~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.

Incidence
~1/100,000 worldwide per year (1)

Prevalence
~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
    • Hyperhomocysteinemia
    • 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:
    • HCC
    • Abscess or cysts; sepsis
    • Status postomphalocele or gastroschisis repair
  • Bush teas (pyrrolizidine alkaloids)
  • Blunt abdominal trauma

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