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:

• 25% - no cause identified
• 75% -
• myeloproliferative disorders - commonest single cause, around 20% of cases
• antiphospholipid syndrome
• deficiencies of proteins C,S or antithrombin III
• factor V Leiden mutation
• oral contraceptive use
• pregnancy
• paroxysmal noctural haemoglobinuria
• metastatic invasion of hepatic vein/IVC

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

References
Aydinil, M. and Bayraktar, Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007; 13(19): 2693-6.
Cura, M., Haskal, Z. and Lopera, J. Diagnostic and interventional radiology for Budd-Chiari syndrome. Radiographics. 2009; 29(3): 669-681.