Budd Chiari Syndrome

Budd-Chiari syndrome is a group of disorders characterised by hepatic venous outflow obstruction.  The obstruction may occur anywhere from the hepatic venules to the right atrium.

As a result of this obstruction there is increased hepatic sinusoidal pressure, leading to:
  • portal hypertension
  • liver congestion
  • decreased liver perfusion

For clinical disease to occur, 2 veins must be blocked. Clinical symptoms/signs include:
  • RUQ pain
  • ascites
  • hepatomegaly

Causes of Budd-Chiari syndrome:

Investigation
  • doppler ultrasound - assessment of hepatic venous flow - sensitivity/specificity >85% - first line
  • MR - second line
  • CT

Management
  • medical
    • anticoagulant therapy
    • manage ascites - spironolactone
  • surgical
    • portosystemic shunt - typically a transjugular intrahepatic portosystemic shunt (TIPS)
    • liver transplant



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