Budd Chiari Syndrome (BCS)

What Is Budd Chiari Syndrome?

  • Collection of anatomic and physiological changes brought about by reduction of hepatic venous outflow anywhere from right atrium to small hepatic venules.
  • Classical BCS is near complete obstruction to flow of hepatic veins into the inferior vena cava or obstruction of the flow in IVC.
  • Venoocclusive disease (VOD) and right heart failure are distinct subgroups with different management.

Discuss the causes of BCS?

Causes may be found in 80%

  • Hypercoagulable states- myeloproliferative disorders, Factor V Leiden, Protein C or Protein S deficiency etc. Myeloproliferative disease is the commonest cause (between 20-50%)
  • Infections(amoebic liver abscess, TB, Hydatid cyst, Schistosomiasis, Liver abscess, aspergillosis)
  • Cancers(HCC, adrenal, Renal cell, Lung cancer, Leukaemia)
  • Membranous obstruction of vena cava (MOVC) which can account for up to 40% in Asia.
  • Gynaecological: OCP, PCOD, Pregnancy and OCP
  • Others: Sarcoid, Crohn’s, Coeliac

Discuss the clinical features of BCS?


  • Median age at presentation is 34 yrs.
  • 67% female
  • Ascites in 84% and Hepatomegaly in 75%
  • Hepatic vein occlusion in 62 % and IVC occlusion in 7 %. 31% has both.
  • Associated Portal vein thrombosis in 34%

Fulminant: Generally pregnancy associated presentation. Severe pain, hepatomegaly, jaundice and ascites. May have hepatic coma. Prognosis is poor.

Acute: Generally present over weeks(less than 6 months). Hepatic coma usually not the presenting symptom.  Hepatitic picture more common.

Chronic: Commonest form of presentation. May be already cirrhotic. Often has large firm liver. Enzymes and bilirubin are less markedly elevated in subacute and chronic forms. Also caudate lobe hypertrophy is seen as blood supply is different.

Discuss the diagnosis of BCS?

  • Ultrasound Doppler is the investigation of choice. CT and MRI can be done as well. Look for inability to visualise the hepatic veins to connect to IVC. In CT non or delayed filling of the hepatic veins and rapid clearance of the caudate lobe can be seen.
  • IVC or percutaneous transhepatic hepatovenography to confirm in doubtful cases where suspicion is high and Doppler or MRI is negative.
  • Arteriography to plan surgical intervention.
  • Liver biopsy in doubtful cases and to assess for cirrhosis as cirrhotics will be candidates for transplant.

Discuss the management of BCS?

  • Anticoagulation- Use in well compensated acute or chronic patients particularly if they are not for other form of therapies. Monitor for varices.
  • Thrombolysis- can be used in acute presentations (less than 3-4 weeks) with good hepatic reserve. It does not work in extensive intrahepatic clot.
  • Radiological intervention: Angioplasty, Stent and TIPS are the options. These are generally used in recent onset BCS as salvage procedures before transplant in fulminant cases.
  • Shunts- Side to side splenorenal, mesocaval and portocaval shunts. It need patent IVC with not too much pressure difference between supra and infrahepatic portions. Shunts are used for clinically stable acute presentations and chronic presentation with good hepatic reserve.
  • Liver transplantation- for patients with decompensated cirrhosis and fulminant hepatic failure.
  • Treat the underlying cause.

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