Budd Chiari Syndrome

Exam Cram

• Hepatic vein obstruction, usually from thrombosis

• Presentation: RUQ pain, ascites & hepatomegaly with unremarkable LFTs

• Diagnosis largely on imaging (ultrasound)

• Management: senior consideration of anticoagulation & stenting

Outline

Background 

Thrombosis of the hepatic vein is associated with hypercoagulable states. These include: 

• Myeloproliferative disorders (PV, ET), 

• Malignancy (HCC), 

• Local liver abscesses, 

• Pregnancy or COCP 

• Thrombophilias: e.g. factor V Leiden

• Idiopathic

Typically occurs in males around age 40. 

The occlusion of the hepatic vein causes venous congestion within the liver, stretching the liver capsule. This can be painful and cause ascites, ultimately ending with portal hypertension & hepatomegaly. The caudate liver lobe is most commonly affected. On biopsy this can show congestion and necrosis. 

Assessment

History:

• Progressive RUQ pain & ascites 

• Timescale: acute (couple weeks), subacute (3m) or chronic 

Examination (triad):

• RUQ pain

• Ascites

• Hepatomegaly

• Splenomegaly, jaundice, peripheral oedema

Investigations

• Relatively normal liver function tests

• Imaging ?hepatic vein obstruction

  • • • • USS 90% sensitivity and specificity

        • MRI > 90% sensitivity & specificity

• Ascitic fluid: transudate, raised SAAG

• Liver biopsy (usually not required): necrosis & congestion

Diagnosis

Diagnostic criteria: 

• Ultimately clinical diagnosis but helped heavily by imaging: 

• Hepatic vein outlet obstruction on imaging (US, CT, MRI) with

• Ascites, RUQ pain & hepatomegaly

Differentials

• Cholecystitis: typically nil ascites/ liver failure signs

• Hepatitis: deranged LFTs

Management

Consultant consideration of:

1.  Anticoagulation

2.  Hepatic vein stenting

Gastro Reg Notes

Definition of Budd Chiari

Budd Chiari syndrome actually refers hepatic vein outlet obstruction (1). There are two types- primary and secondary. Primary Budd Chiari syndrome refers to a hepatic vein thrombosis and secondary refers to another cause of hepatic obstruction, for example malignancy. 

Management II

? If the hepatic vein thrombosis is part of cirrhotic liver disease it doesn't require treatment. 

References

1. Hitawala AA, Gupta V. Budd-Chiari Syndrome. [Updated 2023 Jan 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK558941/ 

2. https://britishlivertrust.org.uk/information-and-support/liver-conditions/cirrhosis/