Skip to main content

Advertisement

Log in

Systemic thrombolysis for acute, severe Budd-Chiari syndrome

  • Published:
Journal of Thrombosis and Thrombolysis Aims and scope Submit manuscript

Abstract

Budd-Chiari syndrome (BCS) or hepatic venous outflow obstruction, is a rare and complex clinical entity, with diverse aetiology and a wide range of presentations. Its rarity and heterogeneity create significant challenges for evidence-based clinical decision-making, particularly in the even more infrequent setting of acute BCS presentation. Here we present the case of a young woman with acute, severe BCS, where treatment alternatives were limited due to a significant IVC thrombus burden. A clearly defined acute onset allowed the prompt use of systemic thrombolysis, conducted in consultation with a multi-disciplinary team of clinicians, with close monitoring for both treatment response and complications. Her treatment resulted in resolution of hepatic vein occlusion and prevention of the potentially significant clinical sequelae. We review the literature and discuss current opinion in the management of BCS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Janssen HLA, Garcia-Pagan J-C, Elias E, Mentha G, Hadengue A, Valla D-C (2003) Budd-Chiari syndrome: a review by an expert panel. J Hepatol 38(3):364–371. doi:10.1016/s0168-8278(02)00434-8

    Article  PubMed  Google Scholar 

  2. Denninger M-H, Chaït Y, Casadevall N, Hillaire S, Guillin M-C, Bezeaud A, Erlinger S, Briere J, Valla D (2000) Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 31(3):587–591. doi:10.1002/hep510310307

    Article  PubMed  CAS  Google Scholar 

  3. Pelletier S, Landi B, Piette J-C, Ekert P, Coutellier A, Desmoulins C, Fadlallah J-P, Herson S, Valla D (1994) Antiphospholipid syndrome as the second cause of non-tumorous Budd-Chiari syndrome. J Hepatol 21(1):76–80. doi:10.1016/s0168-8278(94)80140-1

    Article  PubMed  CAS  Google Scholar 

  4. Lim W, Eikelboom JW, Ginsberg JS (2007) Inherited thrombophilia and pregnancy associated venous thromboembolism. BMJ 334(7607):1318–1321. doi:10.1136/bmj.39205.484572.55

    Article  PubMed  Google Scholar 

  5. Janssen HLA, Meinardi JR, Vleggaar FP, van Uum SHM, Haagsma EB, van der Meer FJM, van Hattum J, Chamuleau RAFM, Adang RP, Vandenbroucke JP, van Hoek B, Rosendaal FR (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(7):2364–2368

    PubMed  CAS  Google Scholar 

  6. Deltenre P, Denninger M-H, Hillaire S, Guillin M-C, Casadevall N, Brière J, Erlinger S, Valla D-C (2001) Factor V Leiden related Budd-Chiari syndrome. Gut 48(2):264–268. doi:10.1136/gut.48.2.264

    Article  PubMed  CAS  Google Scholar 

  7. Bhattacharyya M, Makharia G, Kannan M, Ahmed RPH, Gupta PK, Saxena R (2004) Inherited prothrombotic defects in Budd-Chiari syndrome and portal vein thrombosis. Am J Clin Pathol 121(6):844–847. doi:10.1309/f2u1xbv4rxyuayg0

    Article  PubMed  Google Scholar 

  8. Valla DCN, Huisse MG, Tulliez M, Grange JD, Muller O, Binda T, Varet B, Rueff B, Benhamou JP (1988) Etiology of portal vein thrombosis in adults. A prospective evaluation of primary myeloproliferative disorders. Gastroenterology 94(4):1063–1069

    PubMed  CAS  Google Scholar 

  9. Patel RK, Lea NC, Heneghan MA, Westwood NB, Milojkovic D, Thanigaikumar M, Yallop D, Arya R, Pagliuca A, Gäken J, Wendon J, Heaton ND, Mufti GJ (2006) Prevalence of the activating JAK2 Tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology 130(7):2031–2038. doi:10.1053/j.gastro.2006.04.008

    Article  PubMed  CAS  Google Scholar 

  10. Valla D, Dhumeaux D, Babany G, Hillon P, Rueff B, Rochant H, Benhamou JP (1987) Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology 93(3):569–575

    PubMed  CAS  Google Scholar 

  11. Pomeroy C, Knodell RG, Swaim WR, Arneson P, Mahowald ML (1984) Budd-Chiari syndrome in a patient with the lupus anticoagulant. Gastroenterology 86(1):158–161

    PubMed  CAS  Google Scholar 

  12. Mackworth-Young CG, Melia WM, Harris EN, Gharavi AE, Sivathondan Y, Derue G, Sherlock S, Hughes GRV (1986) The Budd-Chiari syndrome: possible pathogenetic role of anti-phospholipid antibodies. J Hepatol 3(1):83–86. doi:10.1016/s0168-8278(86)80150-7

    Article  PubMed  CAS  Google Scholar 

  13. Murad SD, Valla D-C, de Groen PC, Zeitoun G, Hopmans JAM, Haagsma EB, van Hoek B, Hansen BE, Rosendaal FR, Janssen HLA (2004) Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 39(2):500–508. doi:10.1002/hep20064

    Article  Google Scholar 

  14. Khuroo MS, Datta DV (1980) Budd-Chiari syndrome following pregnancy. Report of 16 cases, with roentgenologic, hemodynamic and histologic studies of the hepatic outflow tract. Am J Med 68(1):113–121

    Article  PubMed  CAS  Google Scholar 

  15. Rautou P-E, Angermayr B, Garcia-Pagan J-C, Moucari R, Peck-Radosavljevic M, Raffa S, Bernuau J, Condat B, Levardon M, Yver C, Ducarme G, Luton D, Denninger M-H, Valla D, Plessier A (2009) Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes. J Hepatol 51(1):47–54. doi:10.1016/jjhep200902028

    Article  PubMed  Google Scholar 

  16. Krass I (1968) Budd-Chiari syndrome after oral contraceptives. BMJ 1(5593):708. doi:10.1136/bmj.1.5593.708-d

    Article  PubMed  CAS  Google Scholar 

  17. Valla D, Le MG, Poynard T, Zucman N, Rueff B, Benhamou JP (1986) Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. Gastroenterology 90(4):807–811

    PubMed  CAS  Google Scholar 

  18. Kume H, Kameyama S, Kasuya Y, Tajima A, Kawabe K (1999) Surgical treatment of renal cell carcinoma associated with Budd-Chiari syndrome: report of four cases and review of the literature. Eur J Surg Oncol (EJSO) 25(1):71–75. doi:10.1053/ejso.1998.0603

    Article  CAS  Google Scholar 

  19. Takeuchi J, Takada A, Hasumura Y, Matsuda Y, Ikegami F (1971) Budd-Chiari syndrome associated with obstruction of the inferior vena cava: a report of seven cases. Am J Med 51(1):11–20. doi:10.1016/0002-9343(71)90319-6

    Article  PubMed  CAS  Google Scholar 

  20. McDermott WV, Stone MD, Bothe A Jr, Trey C (1984) Budd-Chiari syndrome: historical and clinical review with an analysis of surgical corrective procedures. Am J Surg 147(4):463–467. doi:10.1016/0002-9610(84)90006-0

    Article  PubMed  CAS  Google Scholar 

  21. Schaffner F, Gadboys HL, Safran AP, Baron MG, Aufses AH Jr (1967) Budd-Chiari syndrome caused by a web in the inferior vena cava. Am J Med 42(5):838–843. doi:10.1016/0002-9343(67)90100-3

    Article  PubMed  CAS  Google Scholar 

  22. Markert DJ, Shanmuganathan K, Mirvis SE, Nakajima Y, Hayakawa M (1997) Budd-Chiari syndrome resulting from intrahepatic IVC compression secondary to blunt hepatic trauma. Clin Radiol 52(5):384–387. doi:10.1016/s0009-9260(97)80135-3

    Article  PubMed  CAS  Google Scholar 

  23. Whiteford M, Moritz M, Ferber A, Fry R (1999) Budd-Chiari syndrome complicating restorative proctocolectomy for ulcerative colitis. Dis Colon Rectum 42(9):1220–1224. doi:10.1007/bf02238579

    Article  PubMed  CAS  Google Scholar 

  24. Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M (1997) Budd-Chiari syndrome: a common complication of Behcet’s disease. Am J Gastroenterol 92(5):858–862

    PubMed  CAS  Google Scholar 

  25. Wilkey D, Yocum DE, Oberley TD, Sundstrom WR, Karl L (1983) Budd-Chiari syndrome and renal failure in Behcet disease: report of a case and review of the literature. Am J Med 75(3):541–550. doi:10.1016/0002-9343(83)90364-9

    Article  PubMed  CAS  Google Scholar 

  26. Maccini DM, Berg JC, Bell GA (1989) Budd-Chiari syndrome and Crohn’s disease. Dig Dis Sci 34(12):1933–1936. doi:10.1007/bf01536715

    Article  PubMed  CAS  Google Scholar 

  27. Chesner IM, Muller S, Newman J (1986) Ulcerative colitis complicated by Budd-Chiari syndrome. Gut 27(9):1096–1100. doi:10.1136/gut.27.9.1096

    Article  PubMed  CAS  Google Scholar 

  28. Deniz K, Ward SC, Rosen A, Grewal P, Xu R (2008) Budd-Chiari syndrome in sarcoidosis involving liver. Liver Int 28(4):580–581. doi:10.1111/j.1478-3231.2007.01633.x

    Article  PubMed  Google Scholar 

  29. Murad SD, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, Trebicka J, Morard I, Lasser L, Heller J, Hadengue A, Langlet P, Miranda H, Primignani M, Elias E, Leebeek FW, Rosendaal FR, Garcia-Pagan J-C, Valla DC, Janssen HLA, Vief EN (2009) Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Int Med 151(3):167–175. doi:10.1059/0003-4819-151-3-200908040-00004

    Google Scholar 

  30. Valla D, Casadevall N, Lacombe C, Varet B, Goldwasser E, Franco D, Maillard J-N, Pariente EA, Leporrier M, Rueff B (1985) Primary myeloproliferative disorder and hepatic vein thrombosis. Ann Int Med 103(3):329–334

    PubMed  CAS  Google Scholar 

  31. Horton JD, San Miguel FL, Ortiz JA (2008) Budd-Chiari syndrome: illustrated review of current management. Liver Int 28(4):455–466. doi:10.1111/j.1478-3231.2008.01684.x

    Article  PubMed  Google Scholar 

  32. Menon KS, Shah V, Kamath PS (2004) The Budd–Chiari syndrome. New Engl J Med 350(6):578–585. doi:10.1056/NEJMra020282

    Article  PubMed  CAS  Google Scholar 

  33. Zeitoun G, Escolano S, Hadengue A, Azar N, Younsi ME, Mallet A, Boudet M-J, Hay J-M, Erlinger S, Benhamou J-P, Belghiti J, Valla D (1999) Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology 30(1):84–89. doi:10.1002/hep510300125

    Article  PubMed  CAS  Google Scholar 

  34. Segev DL, Nguyen GC, Locke JE, Simpkins CE, Montgomery RA, Maley WR, Thuluvath PJ (2007) Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl 13(9):1285–1294. doi:10.1002/lt.21220

    Article  PubMed  Google Scholar 

  35. Plessier A, Valla DC (2008) Budd-Chiari syndrome. Semin Liver Dis 28(03):259. doi:10.1055/s-0028-1085094

    Article  PubMed  Google Scholar 

  36. Sharma S, Texeira A, Texeira P, Elias E, Wilde J, Olliff SP (2004) Pharmacological thrombolysis in Budd-Chiari syndrome: a single centre experience and review of the literature. J Hepatol 40(1):172–180. doi:10.1016/jjhep200309028

    Article  PubMed  CAS  Google Scholar 

  37. Valla D-C (2004) Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol 19:S204–S211. doi:10.1111/j.1440-1746.2004.03642.x

    Article  Google Scholar 

  38. Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger M-H, Condat B, Farges O, Chagneau C, de Ledinghen V, Francoz C, Sauvanet A, Vilgrain V, Belghiti J, Durand F, Valla D (2006) Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 44(5):1308–1316. doi:10.1002/hep21354

    Article  PubMed  Google Scholar 

  39. Garcia-Pagán JC, Heydtmann M, Raffa S, Plessier A, Murad S, Fabris F, Vizzini G, Abraldes JG, Olliff S, Nicolini A, Luca A, Primignani M, Janssen HLA, Valla D, Elias E, Bosch J (2008) TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology 135(3):808–815. doi:10.1053/j.gastro.2008.05.051

    Article  PubMed  Google Scholar 

  40. Mentha G, Giostra E, Majno PE, Bechstein WO, Neuhaus P, O’Grady J, Praseedom RK, Burroughs AK, Treut YPL, Kirkegaard P, Rogiers X, Ericzon B-G, Hockerstedt K, Adam R, Klempnauer J (2006) Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol 44(3):520–528. doi:10.1016/jjhep200512002

    Article  PubMed  Google Scholar 

  41. Warren RL, Schlant RC Wenger NK (1972) Treatment of Budd-Chiari syndrome with streptokinase. Gastroenterology 62 (200):(abstract)

    Google Scholar 

  42. Greenwood L, Yrizarry J, Hallett J, Scoville G (1983) Urokinase treatment of Budd-Chiari syndrome. Am J Roentgenol 141(5):1057–1059

    CAS  Google Scholar 

  43. McMullin MF, Hillmen P, Jackson J, Ganly P, Luzzatto L (1994) Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria. J Intern Med 235(1):85–89. doi:10.1111/j.1365-2796.1994.tb01037.x

    Article  PubMed  CAS  Google Scholar 

  44. Raju GS, Felver M, Olin JW, Satti SD (1996) Thrombolysis for acute Budd-Chiari syndrome: case report and literature review. Am J Gastroenterol 91(6):1262–1263

    PubMed  CAS  Google Scholar 

  45. Hoekstra J, Guimarães AHC, Leebeek FWG, Darwish Murad S, Malfliet JJMC, Plessier A, Hernandez-Guerra M, Langlet P, Elias E, Trebicka J, Primignani M, Garcia-Pagan J-C, Valla DC, Rijken DC, Janssen HL, European Network for Vascular disorders of the Liver (2010) Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome. Blood 115 (2):388–395. doi:10.1182/blood-2009-03-211557

    Google Scholar 

  46. Guerin JM, Meyer P (1988) Need for early thrombolysis in Budd-Chiari syndrome. Gastroenterology 94(4):1109

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Paul J. Clark.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Clark, P.J., Slaughter, R.E. & Radford, D.J. Systemic thrombolysis for acute, severe Budd-Chiari syndrome. J Thromb Thrombolysis 34, 410–415 (2012). https://doi.org/10.1007/s11239-012-0760-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11239-012-0760-4

Keywords

Navigation