Budd-Chiari syndrome

Budd-Chiari syndrome refers to the clinical picture that occurs when there is obstruction of the hepatic veins

Budd-Chiari syndrome is rare. A Japanese study estimated the prevalence to be in the region of 2.4 cases/million 4.  In Western populations, the most common cause is thrombosis. Membranous webs have been increasingly described in Asian patients as a cause of obstruction.

The classic acute presentation is with the clinical triad of asciteshepatomegaly and abdominal pain, although this is non-specific. The presentation may be acute or chronic:

  • acute: results from an acute thrombosis of the main hepatic veins or the IVC; patients may present with rapid onset ascites
  • chronic: the chronic form is related to fibrosis of the intrahepatic veins, presumably related to inflammation

The aetiology is mixed and varied. The majority of cases result from thrombosis within the hepatic veins. However, 25% arise from external compression that results in obstruction:

Associations
Ultrasound
  • acute
  • chronic
    • hypertrophied caudate lobe
    • peripheral atrophy
    • regenerative nodules
  • gallbladder wall thickening
  • ascites
Colour Doppler
  • part of or the entire right hepatic vein with no flow or inappropriately directed flow

  • discontinuity between the main hepatic vein and the IVC

  • reversed flow in hepatic veins and intra and extrahepatic collaterals
  • portal vein changes such as hepatopetal or hepatofugal flow
  • low or no flow in the IVC or balanced bidirectional flow
  • thrombus or tumour within the IVC
  • increased resistive index within the hepatic artery: >0.75
CT
  • early enhancement of the caudate lobe and central liver around the IVC
  • delayed enhancement of the peripheral liver with accompanying central low density (flip-flop appearance)
  • inhomogeneous mottled liver (nutmeg liver)
  • peripheral zones of the liver may appear hypoattenuating because of reversed portal venous blood flow
  • inability to identify hepatic veins
  • in the chronic phase, there is caudate lobe enlargement 
Angiography

On hepatic venography: 

  • complete occlusion of hepatic veins
  • (focal) stenosis in intrahepatic IVC may or may not be associated
  • spider web appearance and intrahepatic collateral veins

Initially described by Budd in 1845 before Chiari lent his first pathological description of "obliterating endophlebitis of the hepatic veins" in 1899.

If left untreated, progression to liver failure from fibrosis and death occurs. Management options include:

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Article Information

rID: 1023
Sections: Pathology, Syndromes
Synonyms or Alternate Spellings:
  • Budd Chiari syndrome
  • Hepatic venous outflow obstruction

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Cases and Figures

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    Case 1
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    Spider web and in...
    Case 2: with spider web and intrahepatic collaterals
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    Case 3
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    Case 4: membranous
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    Case 5: hepatic vein thrombosis
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