Budd-Chiari Syndrome

Rare disorder caused by hepatic venous outflow obstruction with a wide wide range of aetiologies

Aetiology

  • Haematological - including thrombophilic conditions (e.g. protein C or S deficiency), antiphospholipid syndrome
  • Reduced blood flow - including vena caval abnormalities (e.g. congenital wevs)
  • Obstetric
  • Drugs - including combined oral contraceptives
  • Chronic infections and chronic inflammatory conditions
  • Malignancy
  • Trauma, surgery

Clinical presentation

  • Typically presents with abdominal pain, jaundice, and ascites without frank liver failure
  • May also evolve more rapidly to present with acute liver failure
  • Can cause acute liver disease and cirrhosis

Investigations

  • LFTs mildly elevated
  • Imaging - USS, MRI

Management

  • Recanalization or TIPPS