Internal Medicine

Budd–Chiari syndrome

Occlusion of the hepatic veins and/ or the suprahepatic inferior vena cava.


Occlusion of the hepatic veins and/ or the suprahepatic inferior vena cava.

  • Classical triad:
    • Abdominal pain
    • Ascites
    • Hepatomegaly


Primary Budd–Chiari syndrome (75% cases)

  • Thrombosis of the hepatic vein (Decreasing order of frequency):
    • Polycythemia vera
    • Pregnancy
    • Postpartum state
    • Use of oral contraceptives
    • Paroxysmal nocturnal hemoglobinuria
    • Hepatocellular carcinoma
    • Lupus anticoagulants

Secondary Budd–Chiari syndrome (25% cases)

  • Compression of the hepatic vein by an outside structure (e.g. a tumor, abscess, cysts)

Clinical features

Chronic form (common)

  • Hepatomegaly
  • Abdominal distension
  • Portal hypertension

Acute disorder (uncommon)

  • Abdominal pain
  • Ascites
  • Hepatomegaly
  • Rapidly progressive hepatic failure

Inferior vena cava block:

  • Back veins become prominent, dilated and tortuous with flow from below upwards


  • Doppler ultrasound
  • Venography
Budd–Chiari syndrome secondary to cancer, note clot in the inferior vena cava and the metastasis in the liver | James Heilman, MD – CC BY-SA 3.0,


Restoring the patency of hepatic vein/inferior vena cava

Radiological management:

  • Angioplasty
  • Stenting
  • Trans-jugular intrahepatic portosystemic shunt (TIPS)

Surgical management:

  • Mesoatrial shunt
  • Mesocaval shunt

Orthotopic liver transplant

End-stage liver disease

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