Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

Update on the classification, assessment of prognosis and therapy of Budd–Chiari syndrome

Nature Clinical Practice Gastroenterology & Hepatology volume 2, pages 182–190 (2005)Cite this article

Abstract

Budd–Chiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS—as fulminant, acute or chronic—is not prognostically useful. This makes assessing the benefit of therapy difficult, especially as there is no evidence from randomized studies. This article highlights advances in the prognosis and therapy of BCS. Identification of the site of venous obstruction has a major effect on prognosis. Portal-vein thrombosis occurs in 20–30% of cases, and acute presentation of BCS reflects an acute or chronic syndrome in 60% of BCS cases. BCS can be diagnosed and treated on a single occasion in the setting of the radiology department, with hepatic venography, transjugular liver biopsy, retrograde CO2 portography and inferior vena cava pressure measurements performed simultaneously with therapies such as dilation or stenting of webs in the inferior vena cava or hepatic veins, and placement of transjugular intrahepatic portosystemic shunts. Disruption of a portal vein thrombus can also be done during the same session. Surgical shunts have been superseded by the use of transjugular intrahepatic portosystemic shunts. Liver transplantation is reserved for fulminant and progressive chronic forms of BCS. Anticoagulation therapy must be used routinely, before and after specific therapy, regardless of whether a thrombophilic disorder is diagnosed.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Algorithm for diagnostic and therapeutic management of Budd–Chiari syndrome.

Similar content being viewed by others

References

  1. Okuda K et al. (1998) Proposal of a new nomenclature for Budd–Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 28: 1191–1198

    Article  CAS  Google Scholar 

  2. Valla DC (2003) The diagnosis and management of the Budd–Chiari syndrome: consensus and controversies. Hepatology 38: 793–803

    Article  Google Scholar 

  3. Tanaka M and Wanless IR (1998) Pathology of the liver in Budd–Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. Hepatology 27: 488–496

    Article  CAS  Google Scholar 

  4. Gonzalez-Flecha B et al. (1993) Oxidative stress produced by suprahepatic occlusion and reperfusion. Hepatology 18: 881–889

    Article  CAS  Google Scholar 

  5. DeLeve LD et al. (2002) Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease). Semin Liver Dis 22: 27–42

    Article  Google Scholar 

  6. Kiuchi T et al. (2003) Small-for-size graft in living donor liver transplantation: how far should we go? Liver Transpl 9: S29–S35

    Article  Google Scholar 

  7. Valla DC (2002) Hepatic vein thrombosis (Budd–Chiari syndrome). Semin Liver Dis 22: 5–14

    Article  Google Scholar 

  8. Gupta S et al. (1987) Comparison of ultrasonography, computed tomography and 99mTc liver scan in diagnosis of Budd–Chiari syndrome. Gut 28: 242–247

    Article  CAS  Google Scholar 

  9. Klein AS et al. (2003) Surgical treatment of Budd–Chiari syndrome. Liver Transpl 9: 891–896

    Article  Google Scholar 

  10. Mahmoud AE et al. (1997) Poor prognosis and limited therapeutic options in patients with Budd–Chiari syndrome and portal venous system thrombosis. Eur J Gastroenterol Hepatol 9: 485–489

    Article  CAS  Google Scholar 

  11. Mancuso A et al. (2003) Budd–Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins. Gut 52: 438

    Article  CAS  Google Scholar 

  12. Okuda H et al. (1995) Epidemiological and clinical features of Budd–Chiari syndrome in Japan. J Hepatol 22: 1–9

    Article  CAS  Google Scholar 

  13. Okuda K (2002) Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy). Semin Liver Dis 22: 15–26

    Article  Google Scholar 

  14. Ohta M et al. (1994) Analysis of hepatic vein waveform by Doppler ultrasonography in 100 patients with portal hypertension. Am J Gastroenterol 89: 170–175

    CAS  PubMed  Google Scholar 

  15. Erden A et al. (2002) Budd–Chiari syndrome: evaluation with multiphase contrast-enhanced three-dimensional MR angiography. AJR Am J Roentgenol 179: 1287–1292

    Article  Google Scholar 

  16. Vlachogiannakos J et al. (2000) Carbon-dioxide portography: an expanding role? Lancet 355: 987–988

    Article  CAS  Google Scholar 

  17. Debernardi-Venon W et al. (2000) CO(2) wedged hepatic venography in the evaluation of portal hypertension. Gut 46: 856–860

    Article  CAS  Google Scholar 

  18. Martinez-Cuesta A et al. (2000) CO(2) wedged hepatic venography: technical considerations and comparison with direct and indirect portography with iodinated contrast. Abdom Imaging 25: 576–582

    Article  CAS  Google Scholar 

  19. Janssen HL et al. (2003) Budd–Chiari syndrome: a review by an expert panel. J Hepatol 38: 364–371

    Article  Google Scholar 

  20. Hadengue A et al. (1994) The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 106: 1042–1047

    Article  CAS  Google Scholar 

  21. Murad SD et al. (2004) Determinants of survival and the effect of portosystemic shunting in patients with Budd–Chiari syndrome. Hepatology 39: 500–508

    Article  Google Scholar 

  22. Langlet P et al. (2003) Clinicopathological forms and prognostic index in Budd–Chiari syndrome. J Hepatol 39: 496–501

    Article  Google Scholar 

  23. Tang TJ et al. (2001) The prognostic value of histology in the assessment of patients with Budd–Chiari syndrome. J Hepatol 35: 338–343

    Article  CAS  Google Scholar 

  24. Singh V et al. (2000) Budd–Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol 15: 550–554

    Article  CAS  Google Scholar 

  25. Ibarrola C et al. (2004) Focal hyperplastic hepatocellular nodules in hepatic venous outflow obstruction: a clinicopathological study of four patients and 24 nodules. Histopathology 44: 172–179

    Article  CAS  Google Scholar 

  26. Shrestha SM et al. (1996) Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal. J Gastroenterol Hepatol 11: 170–179

    Article  CAS  Google Scholar 

  27. Denninger MH et al. (2000) Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 31: 587–591

    Article  CAS  Google Scholar 

  28. Janssen HL et al. (2000) Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd–Chiari syndrome and portal vein thrombosis: results of a case-control study. Blood 96: 2364–2368

    CAS  Google Scholar 

  29. Hirshberg B et al. (2000) Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd–Chiari syndrome. J Hepatol 32: 574–578

    Article  CAS  Google Scholar 

  30. Acharya J et al. (1995) Identification of latent myeloproliferative disease in patients with Budd–Chiari syndrome using X-chromosome inactivation patterns and in vitro erythroid colony formation. Eur J Haematol 55: 315–321

    Article  CAS  Google Scholar 

  31. Mohanty D et al. (2001) Hereditary thrombophilia as a cause of Budd–Chiari syndrome: a study from Western India. Hepatology 34 (Pt 1): 666–670

    Article  Google Scholar 

  32. Minnema MC et al. (2000) Budd–Chiari syndrome: combination of genetic defects and the use of oral contraceptives leading to hypercoagulability. J Hepatol 33: 509–512

    Article  CAS  Google Scholar 

  33. Salooja N and Perry DJ (2001) Thrombelastography. Blood Coagul Fibrinolysis 12: 327–337

    Article  CAS  Google Scholar 

  34. Zeitoun G et al. (1999) Outcome of Budd–Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology 30: 84–89

    Article  CAS  Google Scholar 

  35. Henderson JM et al. (1990) Surgical options, hematologic evaluation, and pathologic changes in Budd–Chiari syndrome. Am J Surg 159: 41–50

    Article  CAS  Google Scholar 

  36. Dilawari JB et al. (1994) Hepatic outflow obstruction (Budd–Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine (Baltimore) 73: 21–36

    Article  CAS  Google Scholar 

  37. McCarthy PM et al. (1985) The Budd–Chiari syndrome. Medical and surgical management of 30 patients. Arch Surg 120: 657–662

    Article  CAS  Google Scholar 

  38. Ringe B et al. (1995) Which is the best surgery for Budd–Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Hepatology 21: 1337–1344

    Article  CAS  Google Scholar 

  39. Frank JW et al. (1994) Budd–Chiari syndrome: early intervention with angioplasty and thrombolytic therapy. Mayo Clin Proc 69: 877–881

    Article  CAS  Google Scholar 

  40. Raju GS et al. (1996) Thrombolysis for acute Budd–Chiari syndrome: case report and literature review. Am J Gastroenterol 91: 1262–1263

    CAS  PubMed  Google Scholar 

  41. Sharma S et al. (2004) Pharmacological thrombolysis in Budd–Chiari syndrome: a single centre experience and review of the literature. J Hepatol 40: 172–180

    Article  CAS  Google Scholar 

  42. Bilbao JI et al. (1997) Interventional therapeutic techniques in Budd–Chiari syndrome. Cardiovasc Intervent Radiol 20: 112–119

    Article  CAS  Google Scholar 

  43. Fisher NC et al. (1999) Managing Budd–Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut 44: 568–574

    Article  CAS  Google Scholar 

  44. Zhang CQ et al. (2003) Long-term effect of stent placement in 115 patients with Budd–Chiari syndrome. World J Gastroenterol 9: 2587–2591

    Article  CAS  Google Scholar 

  45. Orloff MJ et al. (2000) A 27-year experience with surgical treatment of Budd–Chiari syndrome. Ann Surg 232: 340–352

    Article  CAS  Google Scholar 

  46. Slakey DP et al. (2001) Budd–Chiari syndrome: current management options. Ann Surg 233: 522–527

    Article  CAS  Google Scholar 

  47. Gillams A et al. (1991) Dilatation of the inferior vena cava using an expandable metal stent in Budd–Chiari syndrome. J Hepatol 13: 149–151

    Article  CAS  Google Scholar 

  48. Behera A et al. (2002) Treatment of Budd–Chiari syndrome with inferior vena caval occlusion by mesoatrial shunt. Eur J Surg 168: 355–359

    Article  Google Scholar 

  49. Bachet JB et al. (2002) Long term survival after portosystemic shunting for Budd–Chiari syndrome: shunt patency is determinant. Hepatology 36: 416A

    Google Scholar 

  50. Senning A (1983) Transcaval posterocranial resection of the liver as treatment of the Budd–Chiari syndrome. World J Surg 7: 632–640

    Article  CAS  Google Scholar 

  51. Mancuso A et al. (2003) TIPS for acute and chronic Budd–Chiari syndrome: a single-centre experience. J Hepatol 38: 751–754

    Article  Google Scholar 

  52. Perello A et al. (2002) TIPS is a useful long-term derivative therapy for patients with Budd–Chiari syndrome uncontrolled by medical therapy. Hepatology 35: 132–139

    Article  Google Scholar 

  53. Rossle M et al. (2004) The Budd–Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery 135: 394–403

    Article  Google Scholar 

  54. Hernandez-Guerra M et al. (2004) PTFE-covered stents improve TIPS patency in Budd–Chiari syndrome. Hepatology 40: 1197–1202

    Article  Google Scholar 

  55. Attwell A et al. (2004) Treatment of Budd–Chiari syndrome in a liver transplant unit, the role of transjugular intrahepatic porto-systemic shunt and liver transplantation. Aliment Pharmacol Ther 20: 867–873

    Article  CAS  Google Scholar 

  56. Thompson NP et al. (1994) Emergency rescue hepatic transplantation following shunt surgery for Budd–Chiari syndrome. Eur J Gastroenterol Hepatol 6: 835–837

    Article  Google Scholar 

  57. Halff G et al. (1990) Liver transplantation for the Budd–Chiari syndrome. Ann Surg 211: 43–49

    Article  CAS  Google Scholar 

  58. Jamieson NV et al. (1991) Liver transplantation for Budd–Chiari syndrome, 1976-1990. Ann Chir 45: 362–365

    CAS  PubMed  Google Scholar 

  59. Srinivasan P et al. (2002) Liver transplantation for Budd–Chiari syndrome. Transplantation 73: 973–977

    Article  Google Scholar 

  60. Ulrich F et al. (2002) Liver transplantation in patients with advanced Budd–Chiari syndrome. Transplant Proc 34: 2278

    Article  CAS  Google Scholar 

  61. Shaked A et al. (1992) Portosystemic shunt versus orthotopic liver transplantation for the Budd–Chiari syndrome. Surg Gynecol Obstet 174: 453–459

    CAS  PubMed  Google Scholar 

  62. Knoop M et al. (1994) Liver transplantation for Budd–Chiari syndrome. Transplant Proc 26: 3577–3578

    CAS  PubMed  Google Scholar 

  63. Melear JM et al. (2002) Hematologic aspects of liver transplantation for Budd–Chiari syndrome with special reference to myeloproliferative disorders. Transplantation 74: 1090–1095

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Clinical Research Fellows Liver Unit at the Royal Free Hospital, London, UK

    Marco Senzolo & Evangelos C Cholongitas

  2. Consultant Physicians and Hepatologists, in the Liver Unit at the Royal Free Hospital, London, UK

    David Patch & Andrew K Burroughs

Authors
  1. Marco Senzolo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Evangelos C Cholongitas
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. David Patch
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Andrew K Burroughs
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrew K Burroughs.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Glossary

HEPATIC VENOGRAPHY

Direct imaging by contrast injection into the hepatic veins by transjugular or transfemoral route

CO2 PORTOGRAPHY

Retrograde injection of CO2 via a hepatic vein, occluded proximally by a balloon catheter or wedging of the catheter, to outline the portal vein without contrast agents

Rights and permissions

Reprints and permissions

About this article

Cite this article

Senzolo, M., Cholongitas, E., Patch, D. et al. Update on the classification, assessment of prognosis and therapy of Budd–Chiari syndrome. Nat Rev Gastroenterol Hepatol 2, 182–190 (2005). https://doi.org/10.1038/ncpgasthep0143

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpgasthep0143

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing