Skip to main content
SpringerLink
Log in

Hepatic Venous Outflow Obstruction

Budd-Chiari Syndrome and Veno-Occlusive Disease (Sinusoidal Obstruction Syndrome)

  • Chapter
Portal Hypertension

Part of the book series: Clinical Gastroenterology ((CG))

  • 1269 Accesses

Abstract

The liver is very susceptible to increases in venous pressure because of its unique architecture, in contrast to most organs, the liver has large endothelial pores and lacks a basement membrane. Thus, any increase in venous pressure leads to the rapid movement of large volumes of fluid from the vascular space into the interstitial space. The ability of the hepatic lymphatics to remove this excess fluid is limited and, therefore, the excess fluid enters the peritoneal cavity. Thus, obstruction to the venous outflow of the liver [Budd-Chiari syndrome (BCS)] presents clinically with signs of congestion of the liver including ascites, hepatomegaly, and right upper quadrant abdominal pain. It is perhaps this typical presentation that has led clinicians over the years to lump together all vascular obstruction into one general classification regardless of the vessels involved. In this chapter, we will make use of recent classifications of hepatic venous obstruction that allow for a better understanding of how each disease causing the BCS is different in its presentation and management. The term BCS will include hepatic vein occlusion, including combined occlusion of the hepatic veins and the vena cava. Hepatic veno-occlusive disease (VOD) also termed sinusoidal occlusion syndrome (SOS) will be considered separately. Cardiac causes of hepatic outflow obstruction will not be considered.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 299.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Budd G. On diseases of the liver. John Churchill, London, UK, 1845.

    Google Scholar 

  2. Reuben A. Illustrious, industrious, and perhaps notorious. Hepatology 2003;38:1065–1069.

    Article  PubMed  Google Scholar 

  3. Chiari H. Phlebitis obliterans der Haupstamme der Venae hepaticae als Todersursache. Beitrage zur pathologischen Anatomie und zur allgemeinen. Pathologic 1899;26:1–17.

    Google Scholar 

  4. Parker R. Occlusion of the hepatic veins in man. Medicine 1959;38:369–402.

    PubMed  CAS  Google Scholar 

  5. Tavill AS, Wood EJ, Kreel L, Jones EA, Gregory M, Sherlock S. The Budd-Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology 1975;68:509–518.

    PubMed  CAS  Google Scholar 

  6. Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38:364–371.

    Article  PubMed  Google Scholar 

  7. Ludwig J, Hashimoto E, McGill DB, van Heerden JA. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clinic Proc 1990;65:51–55.

    CAS  Google Scholar 

  8. Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 1998;28:1191–1198.

    Article  PubMed  CAS  Google Scholar 

  9. Cazals-Hatem D, Vilgrain V, Genin P, et al. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers. Hepatology 2003;37:510–519.

    Article  PubMed  Google Scholar 

  10. Valla D, Dhumeaux D, Babany G, et al. Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology 1987;93:569–575.

    PubMed  CAS  Google Scholar 

  11. Valla D, Casadevall N, Lacombe C, et al. Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. Ann Intern Med 1985;103:329–334.

    PubMed  CAS  Google Scholar 

  12. Janssen HL, Meinardi JR, Vleggaar FP, et al. 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 [comment]. Blood 2000;96:2364–2368.

    PubMed  CAS  Google Scholar 

  13. Denninger MH, Helley D, Valla D, Guillin MC. Prospective evaluation of the prevalence of factor V Leiden mutation in portal or hepatic vein thrombosis. [comment]. Thrornb Haemost 1997;78:1297, 1298.

    CAS  Google Scholar 

  14. Mohanty D, Shetty S, Narayanan TS, Abraham P. Factor V leiden mutation and Budd-Chiari syndrome. Blood 1998;92:1838,1839.

    PubMed  CAS  Google Scholar 

  15. Dayal S, Pati IIP, Pande GK, Sharma MP, Saraya AK. Multilineage hemopoietic stem cell defects in Budd Chiari syndrome. J Hepatol 1997;26:293–297.

    Article  PubMed  CAS  Google Scholar 

  16. Dayal S, Pati HP, Sharma MP. Tissue plasminogen activator and plasminogen activator inhibitor status in Budd-Chiari syndrome. Haemostasis 1996;26:284–287.

    PubMed  CAS  Google Scholar 

  17. Okuda H, Yamagata H, Obata H, et al. Epidemiological and clinical features of Budd-Chiari syndrome in Japan. J Hepatol 1995;22:1–9.

    Article  PubMed  CAS  Google Scholar 

  18. Okuda K. Obstructive disease of the inferior vena cava—an idiopathic type of Budd-Chiari syndrome. Trop Gastroenterol 1997;18:91,92.

    PubMed  CAS  Google Scholar 

  19. Chan P, Lee CP, Yang CY, Hung JS. Complete membranous obstruction of the inferior vena cava: clinical characteristics of Chinese patients. J Int Med 1993;234:501–505.

    Article  CAS  Google Scholar 

  20. Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology 2003;38:793–803.

    PubMed  Google Scholar 

  21. Ganguli SC, Ramzan NN, McKusick MA, Andrews JC, Phyliky RL, Kamath PS. Budd-Chiari syndrome in patients with hematological disease: a therapeutic challenge. Hepatology 1998;27:1157–1161.

    Article  PubMed  CAS  Google Scholar 

  22. Spritzer CE. Vascular diseases and MR angiography of the liver. Mag Reson Imag Clin N Am 1997;5:377–396.

    CAS  Google Scholar 

  23. Murphy FB, Steinberg HV, Shires GT, 3rd, Martin LG, Bernardino ME. The Budd-Chiari syndrome: a review. (AJR) Am J Roentgenol 1986;147:9–15.

    CAS  Google Scholar 

  24. Bolondi L, Gaiani S, Li Bassi S, et al. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology 1991;100:1324–1331.

    PubMed  CAS  Google Scholar 

  25. Millener P, Grant EG, Rose S, et al. Color Doppler imaging findings in patients with Budd-Chiari syndrome: correlation with venographic findings. (AJR) Am J Roentgenol 1993;161:307–312.

    CAS  Google Scholar 

  26. Miller WJ, Federle MP, Straub WH, Davis PL. Budd-Chiari syndrome: imaging with pathologic correlation. Abdom Imag 1993;18:329–335.

    Article  CAS  Google Scholar 

  27. Henderson JM, Warren WD, Millikan WJ Jr, et al. Surgical options, hematologic evaluation, and pathologic changes in Budd-Chiari syndrome. Am J Surg 1990;159:41–48; discussion 48–50.

    Article  PubMed  CAS  Google Scholar 

  28. Tang TJ, Batts KP, de Groen PC, et al. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol 2001;35:338–343.

    Article  PubMed  CAS  Google Scholar 

  29. Tanaka M, Wanless IR. 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 1998;27:488–496.

    Article  PubMed  CAS  Google Scholar 

  30. Klein AS, Molmenti EP. Surgical treatment of Budd-Chiari syndrome. Liver Transpl 2003;9:891–896.

    Article  PubMed  Google Scholar 

  31. Min AD, Atillasoy EO, Schwartz ME, Thiim M, Miller CM, Bodenheimer HC Jr. Reassessing the role of medical therapy in the management of hepatic vein thrombosis. [comment]. Liver Transplant Surg 1997;3:423–429.

    Article  CAS  Google Scholar 

  32. Perello A, Garcia-Pagan JC, Gilabert R, et al. TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. [comment]. Hepatology 2002;35:132–139.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  34. Ilan Y, Oren R, Shouval D. Postpartum Budd-Chiari syndrome with prolonged hypercoagulability state. Am J Obstet Gynecol 1990;162:1164,1165.

    PubMed  CAS  Google Scholar 

  35. Suzuoki M, Kondo S, Ambo Y, et al. Treatment of Budd-Chiari syndrome with percutaneous trans-luminal angioplasty: report of a case. Surg Today 2002;32:559–562.

    Article  PubMed  Google Scholar 

  36. Frank JW, Kamath PS, Stanson AW. Budd-Chiari syndrome: early intervention with angioplasty and thrombolytic therapy. Mayo Clinic Proc 1994;69:877–881.

    CAS  Google Scholar 

  37. Fisher NC, McCafferty I, Dolapci M, et al. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut 1999;44:568–574.

    PubMed  CAS  Google Scholar 

  38. Kohli V, Pande GK, Dev V, Reddy KS, Kaul U, Nundy S. Management of hepatic venous outflow obstruction. Lancet 1993;342:718–722.

    Article  PubMed  CAS  Google Scholar 

  39. Blum U, Rossle M, Haag K, et al. Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology 1995;197:805–811.

    PubMed  CAS  Google Scholar 

  40. Ochs A, Sellinger M, Haag K, et al. Transjugular intrahepatic portosystemic stent-shunt (TIPS) in the treatment of Budd-Chiari syndrome. [comment]. J Hepatol 1993;18:217–225.

    Article  PubMed  CAS  Google Scholar 

  41. Shrestha R, Durham JD, Wachs M, et al. Use of transjugular intrahepatic portosystemic shunt as a bridge to transplantation in fulminant hepatic failure due to Budd-Chiari syndrome. Am J Gastroenterol 1997;92:2304–2306.

    PubMed  CAS  Google Scholar 

  42. Mancuso A, Fung K, Mela M, et al. TIPS for acute and chronic Budd-Chiari syndrome: a single-center experience. J Hepatol 2003;38:751–754.

    Article  PubMed  Google Scholar 

  43. Orloff MJ, Daily PC), Orloff SL, Girard B, Orloff MS. A 27-year experience with surgical treatment of Budd-Chiari syndrome. Ann Surg 2000;232:340–352.

    Article  PubMed  CAS  Google Scholar 

  44. Millikan WJ Jr. Henderson JM, Sewell CW, et al. Approach to the spectrum of Budd-Chiari syndrome: which patients require portal decompression? Am J Surg 1985;149:167–176.

    Article  PubMed  Google Scholar 

  45. Bismuth H, Sherlock DJ. Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg 1991;214:581–589.

    Article  PubMed  CAS  Google Scholar 

  46. Shaked A, Goldstein RM, Klintmalm GB, Drazan K, Husberg B, Busuttil RW. Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. Surg Gynecol Obstet 1992;174:453–459.

    PubMed  CAS  Google Scholar 

  47. Zeitoun G, Escolano S, Hadengue A, et al. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatol 1999;30:84–89.

    Article  CAS  Google Scholar 

  48. Singh V, Sinha SK, Nam CK, et al. Budd-Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol 2000;15:550–554.

    Article  PubMed  CAS  Google Scholar 

  49. Sauvanet A, Panis Y, Valla D, Vilgrain V, Belghiti J. Budd-Chiari syndrome with extensive portal thrombosis: treatment with Senning’s procedure. Hepato-Gastroenterol 1994;41:174–176.

    CAS  Google Scholar 

  50. Putnam CW, Porter KA, Weil R 3rd, Reid HA, Starzl TE. Liver transplantation of Budd-Chiari syndrome. JAMA 1976;236:1142–1143.

    Article  PubMed  CAS  Google Scholar 

  51. Seltman HJ, Dekker A, Van Thiel DH, Boggs DR, Starzl TE. Budd-Chiari syndrome recurring in a transplanted liver. Gastroenterology 1983;84:640–643.

    PubMed  CAS  Google Scholar 

  52. Halff G, Todo S, Tzakis AG, Gordon RD, Starzl TE. Liver transplantation for the Budd-Chiari syndrome. Ann Surg 1990;211:43–49.

    Article  PubMed  CAS  Google Scholar 

  53. Jamieson NV, Williams R, Calne RY. Liver transplantation for Budd-Chiari syndrome, 1976–1990. Annal Chirurg 1991;45:362–365.

    CAS  Google Scholar 

  54. Ringe B, Lang H, Oldhafer KJ, et al. Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Hepatology 1995;21:1337–1344.

    PubMed  CAS  Google Scholar 

  55. Gordon RD. Liver transplantation and venous disorders of the liver. Liver Transplant Surg 1997;3:S41–S51.

    CAS  Google Scholar 

  56. Srinivasan P, Rela M, Prachalias A, et al. Liver transplantation for Budd-Chiari syndrome. Transplantation 2002;73:973–977.

    Article  PubMed  Google Scholar 

  57. Sakai Y, Wall WJ. Liver transplantation for Budd-Chiari syndrome: a retrospective study. Surg Today 1994;24:49–53.

    Article  PubMed  CAS  Google Scholar 

  58. Yasutomi M, Egawa H, Kobayashi Y, Oike F, Tanaka K. Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and associated antiphospholipid antibody syndrome. J Pediatric Surg 2001;36:659–662.

    Article  CAS  Google Scholar 

  59. Langlet P, Escolano S, Valla D, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496–501.

    Article  PubMed  Google Scholar 

  60. DeLeve LD, Shulman HM, McDonald GB. Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease). Semin Liver Dis 2002;22:27–42.

    Article  PubMed  Google Scholar 

  61. Tandon BN, Tandon HD, Tandon RK, Narndranathan M, Joshi YK. An epidemic of veno-occlusive disease of liver in central India. Lancet 1976;2:271,272.

    Article  PubMed  CAS  Google Scholar 

  62. Mohabbat O, Younos MS, Merzad AA, et al. An outbreak of hepatic veno-occlusive disease in north-western Afghanistan. Lancet 1976;2:269–271.

    Article  PubMed  CAS  Google Scholar 

  63. Ridker PM, McDermott WV. Comfrey herb tea and hepatic veno-occlusive disease. Lancet 1989;1:657,658.

    Article  PubMed  CAS  Google Scholar 

  64. Steenkamp V, Stewart MJ, Zuckerman M. Clinical and analytical aspects of pyrrolizidine poisoning caused by South African traditional medicines. Therapeut Drug Monitor 2000;22:302–306.

    Article  CAS  Google Scholar 

  65. McDonald GB, Sharma P, Matthews DE, Shulman HM, Thomas ED. Venocclusive disease of the liver after bone marrow transplantation: diagnosis, incidence, and predisposing factors. Hepatology 1984;4:116–122.

    PubMed  CAS  Google Scholar 

  66. Berk PD, Popper II, Krueger GR, Decter J, Herzig G, Graw RG Jr. Veno-occlusive disease of the liver after allogeneic bone marrow transplantation: possible association with graft-versus-host disease. Ann Intern Med 1979;90:158–164.

    PubMed  CAS  Google Scholar 

  67. Shulman HM, McDonald GB, Matthews D, et al. An analysis of hepatic venocclusive disease and centrilobular hepatic degeneration following bone marrow transplantation. Gastroenterology 1980;79:1178–1191.

    PubMed  CAS  Google Scholar 

  68. Read AE, Wiesner RH, LaBrecque DR, et al. Hepatic veno-occlusive disease associated with renal transplantation and azathioprine therapy. Ann Intern Med 1986;104:651–655.

    PubMed  CAS  Google Scholar 

  69. Merino JM, Casanova F, Saez-Royuela F, Velasco A, Gonzalez JB. Veno-occlusive disease of the liver associated with thiopurines in a child with acute lymphoblastic leukemia. Pediat Hematol Oncol 2000;17:429–431.

    Article  CAS  Google Scholar 

  70. Kao NL, Rosenblate HJ. 6-Thioguanine therapy for psoriasis causing toxic hepatic venoocclusive disease. J Am Acad Dermatol 1993;28:1017,1018.

    PubMed  CAS  Google Scholar 

  71. Paschke R, Heine M. Pathophysiological aspects of dacarbazine-induced human liver damage. Hepato-gastroenterology 1985;32:273–275.

    PubMed  CAS  Google Scholar 

  72. Hanel M, Kroger N, Sonnenberg S, et al. Busulfan, cyclophosphamide, and etoposide as high-dose conditioning regimen in patients with malignant lymphoma. Ann Hematol 2002;81:96–102.

    Article  PubMed  CAS  Google Scholar 

  73. Giles FJ, Kantarjian HM, Kornblau SM, et al. Mylotarg (gemtuzumab ozogamicin) therapy is associated with hepatic venoocclusive disease in patients who have not received stem cell transplantation. Cancer 2001;92:406–413.

    Article  PubMed  CAS  Google Scholar 

  74. Giles F, Garcia-Manero G, O’Brien S, Estey E, Kantarjian H. Fatal hepatic veno-occlusive disease in a phase I study of mylotarg and troxatyl in patients with refractory acute myeloid leukemia or myelodysplastic syndrome. Acta Haematolog 2002;108:164–167.

    Article  Google Scholar 

  75. Bisogno G, de Kraker J, Weirich A, et al. Veno-occlusive disease of the liver in children treated for Wilms tumor. Med Pediat Oncol 1997;29:245–251.

    Article  CAS  Google Scholar 

  76. Czauderna P, Katski K, Kowalczyk J, et al. Venoocclusive liver disease (VOD) as a complication of Wilms’ tumour management in the series of consecutive 206 patients. Europ J Pediat Surg 2000;10:300–303.

    CAS  Google Scholar 

  77. Hazar V, Kutluk T, Akyuz C, Varan A, Yaris N, Buyukpamukcu M. Veno-occlusive disease-like hepatotoxicity in two children receiving chemotherapy for Wilms’ tumor and clear cell sarcoma of kidney. Pediat Hematol Oncol 1998;15:85–89.

    Article  CAS  Google Scholar 

  78. Nakazawa Y, Chisuwa H, Mita A, et al. Life-threatening veno-occlusive disease after living-related liver transplantation. Transplantation 2003;75:727–730.

    Article  PubMed  Google Scholar 

  79. Sebagh M, Debette M, Samuel D, et al. “Silent” presentation of veno-occlusive disease after liver transplantation as part of the process of cellular rejection with endothelial predilection. Hepatology 1999;30:1144–1150.

    Article  PubMed  CAS  Google Scholar 

  80. Shulman HM, Fisher LB, Schoch HG, Henne KW, McDonald GB. Veno-occlusive disease of the liver after marrow transplantation: histological correlates of clinical signs and symptoms. Hepatology 1994;19:1171–1181.

    PubMed  CAS  Google Scholar 

  81. Shulman HM, Hinterberger W. Hepatic veno-occlusive disease—liver toxicity syndrome after bone marrow transplantation. Bone Marrow Transplant 1992;10:197–214.

    PubMed  CAS  Google Scholar 

  82. Sato Y, Asada Y, Hara S, et al. Hepatic stellate cells (Ito cells) in veno-occlusive disease of the liver after allogeneic bone marrow transplantation. Histopathology 1999;34:66–70.

    Article  PubMed  CAS  Google Scholar 

  83. Korte W. Veno-occlusive disease of the liver after bone marrow transplantation: is hypercoagulability really part of the problem? Blood Coagulat Fibrinolysis 1997;8:367–381.

    Article  CAS  Google Scholar 

  84. DeLeve LD, McCuskey RS, Wang X, et al. Characterization of a reproducible rat model of hepatic veno-occlusive disease. Hepatology 1999;29:1779–1791.

    Article  PubMed  CAS  Google Scholar 

  85. Wang X, Kanel GC, DeLeve LD. Support of sinusoidal endothelial cell glutathione prevents hepatic veno-occlusive disease in the rat. Hepatology 2000;31:428–434.

    Article  PubMed  CAS  Google Scholar 

  86. DeLeve LD, Wang X, Kanel GC, et al. Decreased hepatic nitric oxide production contributes to the development of rat sinusoidal obstruction syndrome. Hepatology 2003;38:900–908.

    PubMed  CAS  Google Scholar 

  87. Eberhardt W, Beeg T, Beck KF, et al. Nitric oxide modulates expression of matrix metalloproteinase-9 in rat mesangial cells. Kidney Int 2000;57:59–69.

    Article  PubMed  CAS  Google Scholar 

  88. Salat C, Holler E, Kolb HJ, et al. Plasminogen activator inhibitor-1 confirms the diagnosis of hepatic veno-occlusive disease in patients with hyperbilirubinemia after bone marrow transplantation. Blood 1997;89:2184–2188.

    PubMed  CAS  Google Scholar 

  89. Bearman SI. The syndrome of hepatic veno-occlusive disease after marrow transplantation. Blood 1995;85:3005–3020.

    PubMed  CAS  Google Scholar 

  90. Carreras L. Veno-occlusive disease of the liver after hemopoietic cell transplantation. Europ J Haematol 2000;64:281–291.

    Article  CAS  Google Scholar 

  91. McDonald GB, Shulman HM, Sullivan KM, Spencer GD. Intestinal and hepatic complications of human bone marrow transplantation. Part I. Gastroenterology 1986;90:460–477.

    PubMed  CAS  Google Scholar 

  92. McDonald GB, Shulman HM, Sullivan KM, Spencer GD. Intestinal and hepatic complications of human bone marrow transplantation. Part II. Gastroenterology 1986;90:770–784.

    PubMed  CAS  Google Scholar 

  93. McDonald GB, Shulman HM, Wolford JL, Spencer GD. Liver disease after human marrow transplantation. Semin Liver Dis 1987;7:210–229.

    PubMed  CAS  Google Scholar 

  94. Kumar S, DeLeve LD, Kamath PS, Tefferi A. Hepatic veno-occlusive disease (sinusoidal obstruction syndrome) after hematopoietic stem cell transplantation. Mayo Clinic Proc 2003;78:589–598.

    Article  Google Scholar 

  95. Lee JL, Gooley T, Bensinger W, Schiffman K, McDonald GB. Veno-occlusive disease of the liver after busulfan, melphalan, and thiotepa conditioning therapy: incidence, risk factors, and outcome. Biol Blood Marrow Transplant 1999;5:306–315.

    Article  PubMed  CAS  Google Scholar 

  96. Bearman SI. Veno-occlusive disease of the liver. Curr Opin Oncol 2000;12:103–109.

    Article  PubMed  CAS  Google Scholar 

  97. Rubenfeld GD, Crawford SW. Withdrawing life support from mechanically ventilated recipients of bone marrow transplants: a case for evidence-based guidelines. Ann Intern Med 1996;125:625–633.

    PubMed  CAS  Google Scholar 

  98. McDonald GB, Hinds MS, Fisher LD, et al. Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients. Ann Int Med 1993;118:255–267.

    PubMed  CAS  Google Scholar 

  99. Lee JH, Lee KH, Kim S, et al. Relevance of proteins C and S, antithrombin III, von Willebrand factor, and factor VITI for the development of hepatic veno-occlusive disease in patients undergoing allogeneic bone marrow transplantation: a prospective study. Bone Marrow Transplant 1998;22:883–888.

    Article  PubMed  CAS  Google Scholar 

  100. Salat C, Holler E, Kolb HJ, et al. The relevance of plasminogen activator inhibitor 1 (PAI-1) as a marker for the diagnosis of hepatic veno-occlusive disease in patients after bone marrow transplantation. Leuk Lymph 1999;33:25–32.

    CAS  Google Scholar 

  101. Kaleelrahman M, Eaton JD, Leeming D, et al. Role of plasminogen activator inhibitor-1 (PAI-1) levels in the diagnosis of BMT-associated hepatic veno-occlusive disease and monitoring of subsequent therapy with defibrotide (DF). Hematology 2003;8:91–95.

    Article  PubMed  CAS  Google Scholar 

  102. Lee JH, Lee KH, Kim S, et al. Plasminogen activator inhibitor-1 is an independent diagnostic marker as well as severity predictor of hepatic veno-occlusive disease after allogeneic bone marrow transplantation in adults conditioned with busulphan and cyclophosphamide. Br J Haematol 2002;118:1087–1094.

    Article  PubMed  CAS  Google Scholar 

  103. Fried MW, Duncan A, Soroka S, et al. Serum hyaluronic acid in patients with veno-occlusive disease following bone marrow transplantation. Bone Marrow Transplant 2001;27:635–639.

    Article  PubMed  CAS  Google Scholar 

  104. Shulman HM, Gooley T, Dudley MD, et al. Utility of transvenous liver biopsies and wedged hepatic venous pressure measurements in sixty marrow transplant recipients. Transplantation 1995;59:1015–1022.

    Article  PubMed  CAS  Google Scholar 

  105. Carreras E, Granena A, Navasa M, et al. Transjugular liver biopsy in BMT. Bone Marrow Transplant 1993;11:21–26.

    PubMed  CAS  Google Scholar 

  106. Carreras E, Garcia-Pagan JC, Bosch J, Rozman C. Transvenous liver biopsies in marrow transplant recipients. Transplantation 1995;60:1375.

    PubMed  CAS  Google Scholar 

  107. Hommeyer SC, Teefey SA, Jacobson AF, et al. Venocclusive disease of the liver: prospective study of US evaluation. Radiology 1992;184:683–686.

    PubMed  CAS  Google Scholar 

  108. Lassau N, Leclere J, Auperin A, et al. Hepatic veno-occlusive disease after myeloablative treatment and bone marrow transplantation: value of gray-scale and Doppler US in 100 patients. Radiology 1997;204:545–552.

    PubMed  CAS  Google Scholar 

  109. Lassau N, Auperin A, Leclere J, Bennaceur A, Valteau-Couanet D, Hartmann O. Prognostic value of doppler-ultrasonography in hepatic veno-occlusive disease. Transplantation 2002;74:60–66.

    Article  PubMed  Google Scholar 

  110. van den Bosch MA, van Hoe L. MR imaging findings in two patients with hepatic veno-occlusive disease following bone marrow transplantation. Europ Radiol 2000;10:1290–1293.

    Article  Google Scholar 

  111. Mortele KJ, Van Vlierberghe H, Wiesner W, Ros PR. Hepatic veno-occlusive disease: MRI findings. Abdom Imag 2002;27:523–526.

    Article  CAS  Google Scholar 

  112. Jones RJ, Lee KS, Beschorner WE, et al. Venoocclusive disease of the liver following bone marrow transplantation. Transplantation 1987;44:778–783.

    Article  PubMed  CAS  Google Scholar 

  113. Carreras E, Granena A, Navasa M, et al. On the reliability of clinical criteria for the diagnosis of hepatic veno-occlusive disease. Ann Hematol 1993;66:77–80.

    Article  PubMed  CAS  Google Scholar 

  114. Bearman SI. Avoiding hepatic veno-occlusive disease: what do we know and where are we going? Bone Marrow Transplantation 2001;27:1113–1120.

    Article  PubMed  CAS  Google Scholar 

  115. Attal M, Huguet F, Rubie H, et al. Prevention of hepatic veno-occlusive disease after bone marrow transplantation by continuous infusion of low-dose heparin: a prospective, randomized trial. Blood 1992;79:2834–2840.

    PubMed  CAS  Google Scholar 

  116. Bearman ST, Hinds MS, Wolford JL, et al. A pilot study of continuous infusion heparin for the prevention of hepatic veno-occlusive disease after bone marrow transplantation. Bone Marrow Transplant 1990;5:407–411.

    PubMed  CAS  Google Scholar 

  117. Simon M, Hahn T, Ford LA, et al. Retrospective multivariate analysis of hepatic veno-occlusive disease after blood or marrow transplantation: possible beneficial use of low molecular weight heparin. Bone Marrow Transplant 2001;27:627–633.

    Article  PubMed  CAS  Google Scholar 

  118. Or R, Nagler A, Shpilberg O, et al. Low molecular weight heparin for the prevention of veno-occlusive disease of the liver in bone marrow transplantation patients. Transplantation 1996;61:1067–1071.

    Article  PubMed  CAS  Google Scholar 

  119. DeLeve LD, Ito Y, Bethea NW, McCuskey MK, Wang X, McCuskey RS. Embolization by sinusoidal lining cells obstructs the microcirculation in rat sinusoidal obstruction syndrome. Am J Physiol Gastrointest Liver Physiol 2003;284:G1045–G1052.

    PubMed  CAS  Google Scholar 

  120. Goringe AP, Brown S, O’Callaghan I J, et al. Glutamine and vitamin E in the treatment of hepatic veno-occlusive disease following high-dose chemotherapy. Bone Marrow Transplant 1998;21:829–832.

    Article  PubMed  CAS  Google Scholar 

  121. Shulman HM, Luk K, Deeg HJ, Shuman WB, Storb R. Induction of hepatic veno-occlusive disease in dogs. Am J Pathol 1987;126:114–125.

    PubMed  CAS  Google Scholar 

  122. Essell JH, Schroeder MT, Harman GS, et al. Ursodiol prophylaxis against hepatic complications of allogeneic bone marrow transplantation. A randomized, double-blind, placebo-controlled trial. Ann Int Med 1998;128:975–981.

    PubMed  CAS  Google Scholar 

  123. Ohashi K, Tanabe J, Watanabe R, et al. The Japanese multicenter open randomized trial of ursodeoxy-cholic acid prophylaxis for hepatic veno-occlusive disease after stem cell transplantation. Am J Hematol 2000;64:32–38.

    Article  PubMed  CAS  Google Scholar 

  124. Clift RA, Bianco JA, Appelbaum FR, et al. A randomized controlled trial of pentoxifylline for the prevention of regimen-related toxicities in patients undergoing allogeneic marrow transplantation. Blood 1993;82:2025–2030.

    PubMed  CAS  Google Scholar 

  125. Ferra C, de Sanjose S, Lastra CF, et al. Pentoxifylline, ciprofloxacin and prednisone failed to prevent transplant-related toxicities in bone marrow transplant recipients and were associated with an increased incidence of infectious complications. Bone Marrow Transplant 1997;20:1075–1080.

    Article  PubMed  CAS  Google Scholar 

  126. Bearman SI, Anderson GL, Mori M, Hinds MS, Shulman HM, McDonald GB. Venoocclusive disease of the liver: development of a model for predicting fatal outcome after marrow transplantation. J Clin Oncol 1993;11:1729–1736.

    PubMed  CAS  Google Scholar 

  127. Bearman SI, Lee JL, Baron AE, McDonald GB. Treatment of hepatic venocclusive disease with recombinant human tissue plasminogen activator and heparin in 42 marrow transplant patients. Blood 1997;89:1501–1506.

    PubMed  CAS  Google Scholar 

  128. Richardson PG, Elias AD, Krishnan A, et al. Treatment of severe veno-occlusive disease with defibrotide: compassionate use results in response without significant toxicity in a high-risk population. Blood 1998;92:737–744.

    PubMed  CAS  Google Scholar 

  129. Chopra R, Eaton JD, Grassi A, et al. Defibrotide for the treatment of hepatic veno-occlusive disease: results of the European compassionate-use study. Br J Haematol 2000;111:1122–1129.

    Article  PubMed  CAS  Google Scholar 

  130. Richardson PG, Murakami C, Jin Z, et al. Multi-institutional use of defibrotide in 88 patients after stem cell transplantation with severe veno-occlusive disease and multisystem organ failure: response without significant toxicity in a high-risk population and factors predictive of outcome. Blood 2002;100:4337–4343.

    Article  PubMed  CAS  Google Scholar 

  131. Ringden O, Remberger M, Lehmann S, et al. N-acetylcysteine for hepatic veno-occlusive disease after allogeneic stem cell transplantation. Bone Marrow Transplant 2000;25:993–996.

    Article  PubMed  CAS  Google Scholar 

  132. Morris JD, Harris RE, Hashmi R, et al. Antithrombin-III for the treatment of chemotherapy-induced organ dysfunction following bone marrow transplantation. Bone Marrow Transplant 1997;20:871–878.

    Article  PubMed  CAS  Google Scholar 

  133. Mertens R, Brost H, Granzen B, Nowak-Gottl U. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer. Europ J Pediat 1999;158:S154–S158.

    Article  PubMed  CAS  Google Scholar 

  134. Tefferi A, Kumar S, Wolf RC, et al. Charcoal hemofiltration for hepatic veno-occlusive disease after hematopoietic stem cell transplantation. Bone Marrow Transplant 2001;28:997–999.

    Article  PubMed  CAS  Google Scholar 

  135. Azoulay D, Castaing D, Lemoine A, Hargreaves GM, Bismuth H. Transjugular intrahepatic portosystemic shunt (TIPS) for severe veno-occlusive disease of the liver following bone marrow transplantation. Bone Marrow Transplant 2000;25:987–992.

    Article  PubMed  CAS  Google Scholar 

  136. de la Rubia J, Carral A, Montes H, Urquijo JJ, Sanz GF, Sanz MA. Successful treatment of hepatic veno-occlusive disease in a peripheral blood progenitor cell transplant patient with a transjugular intrahepatic portosystemic stent-shunt (TIPS). Haematologica 1996;81:536–539.

    PubMed  Google Scholar 

  137. Zenz T, Rossle M, Bertz H, Siegerstetter V, Ochs A, Finke J. Severe veno-occlusive disease after allogeneic bone marrow or peripheral stem cell transplantation—role of transjugular intrahepatic portosystemic shunt (TIPS). Liver 2001;21:31–36.

    Article  PubMed  CAS  Google Scholar 

  138. Rosen HR, Martin P, Schiller GJ, et al. Orthotopic liver transplantation for bone-marrow transplant-associated veno-occlusive disease and graft-versus-host disease of the liver. Liver Transplant Surg 1996;2:225–232.

    Article  CAS  Google Scholar 

  139. Salat C, Holler E, Gohring P, et al. Protein C, protein S and antithrombin III levels in the course of bone marrow and subsequent liver transplantation due to veno-occlusive disease. Europ J Med Res 1996;1:571–574.

    CAS  Google Scholar 

  140. Kim ID, Egawa H, Marui Y, et al. A successful liver transplantation for refractory hepatic veno-occlusive disease originating from cord blood transplantation. Am J Transplant 2002;2:796–800.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Mayo Clinic, Division of Transplantation Medicine, Scottsdale, AZ

    Hugo E. Vargas MD

  2. Liver Research Institute, University of Arizona, Tuscon, AZ

    Thomas D. Boyer MD

Authors
  1. Hugo E. Vargas MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Thomas D. Boyer MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Division of Gastroenterology, Virginia Commonwealth Medical Center, Richmond, VA

    Arun J. Sanyal MBBS, MD

  2. GI Research Unit, Mayo Clinic, Rochester, MN

    Vijay H. Shah MD

Rights and permissions

Reprints and permissions

Copyright information

© 2005 Humana Press Inc., Totowa, NJ

About this chapter

Cite this chapter

Vargas, H.E., Boyer, T.D. (2005). Hepatic Venous Outflow Obstruction. In: Sanyal, A.J., Shah, V.H. (eds) Portal Hypertension. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-59259-885-4_26

Download citation

  • DOI: https://doi.org/10.1007/978-1-59259-885-4_26

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-386-2

  • Online ISBN: 978-1-59259-885-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation