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 features

• 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

• Bloods: LFTs mildly elevated
• Imaging: USS, MRI

Management

• Recanalization or TIPPS