Budd-Chiari Syndrome: Pathology

  • Kamal G. Ishak


Budd-Chiari syndrome (BCS) is a rare condition resulting from obstruction to the outflow of blood from the liver. It is characterized clinically by hepatomegaly, splenomegaly, right upper quadrant (RUQ) abdominal pain, and ascites.1–16 Most patients have an acute onset, but occasionally the disorder appears insidiously. BCS is caused by many different conditions and diseases, including membranous webs, veno-occlusive disease (VOD), and hepatic vein thrombosis. Almost one third of cases of hepatic vein thrombosis are idiopathic.1, 5 Well-recognized underlying conditions associated with hepatic vein thrombosis include hematologic disorders, such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria, lupus anticoagulant,6 antithrombosis 3 deficiency,7 and myeloproliferative disorders, as well as the use of oral contraceptives.8 Worldwide, the most common cause of hepatic outflow obstruction is membranous obstruction of the inferior vena cava.9 Cases related to oral contraceptives are discussed by Lewis et al.10 and Maddrey11. In a case-control study from France, the relative risk of hepatic vein thrombosis among recent oral contraceptive users was 2.37, compared with nonusers.12 The duration of oral contraception prior to development of the syndrome is variable, with a mean of approximately 30 months.10 The etiopathogenesis of hepatic vein thrombosis in women on oral contraceptives remains undetermined. It has been suggested that oral contraceptives may increase the risk of thrombosis associated with primary myeloproliferative disorders, which was found to be a major cause of hepatic vein thrombosis in young women in one study.13


Oral Contraceptive Hepatic Vein Alcoholic Liver Disease Paroxysmal Nocturnal Hemoglobinuria Epithelioid Hemangioendothelioma 
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  1. 1.
    Parker RGF: Occlusion of the hepatic veins in man. Medicine (Baltimore) 38:369–401, 1959.Google Scholar
  2. 2.
    Clain D, Freston J, Kreel L, et al: Clinical diagnosis of the Budd-Chiari syndrome. Am J Med 43:544–554, 1967.PubMedCrossRefGoogle Scholar
  3. 3.
    Tavill AS, Wood EJ, Kreel L, et al: The Budd-Chiari syndrome: Correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology 68:509–518, 1975.PubMedGoogle Scholar
  4. 4.
    Powell-Jackson PR, Melia W, Canalese J, et al: Budd-Chiari syndrome: Clinical pattern and therapy. Q J Med 51:79–88, 1982.PubMedGoogle Scholar
  5. 5.
    Mitchell MC, Boitnott JK, Kaufman S, et al: Budd-Chiari syndrome: Etiology, diagnosis and management. Medicine (Baltimore) 61:199–218, 1982.Google Scholar
  6. 6.
    Van Steenbergen W, Beyls J, Vermylen J, et al: “Lupus” anticoagulant and thrombosis of the hepatic veins (Budd-Chiari syndrome). Report of three patients and review of the literature. J Hepatol 3:87–94, 1986.PubMedCrossRefGoogle Scholar
  7. 7.
    Das M, Carroll SF: Antithrombin III deficiency: An etiology of Budd-Chiari syndrome. Surgery 97:242–245, 1985.PubMedGoogle Scholar
  8. 8.
    Maddrey WC: Hepatic vein thrombosis (Budd-Chiari syndrome). Hepatology 4:44S–46S, 1984.PubMedCrossRefGoogle Scholar
  9. 9.
    Rector WG, Yu Y, Goldstein L, et al: Membranous obstruction of the inferior vena cava in the United States. Medicine (Baltimore) 64:134–143, 1985.Google Scholar
  10. 10.
    Lewis JH, Tice HL, Zimmerman HJ: Budd-Chiari syndrome associated with oral contraceptive steroids: Review and treatment of 47 cases. Dig Dis Sci 28:673–683, 1985.CrossRefGoogle Scholar
  11. 11.
    Maddrey WC: Hepatic vein thrombosis (Budd-Chiari syndrome): Possible association with the use of oral contraceptives. Semin Liver Dis 7:32–39, 1987.PubMedCrossRefGoogle Scholar
  12. 12.
    Valla D, Monique GL, Poynard T, et al: Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. Gastroenterology 90:807–811, 1986.PubMedGoogle Scholar
  13. 13.
    Valla D, Casadevall N, Lacombe C, et al: Primary myeloproliferative disorder and hepatic vein thrombosis. Ann Intern Med 103:329–334, 1985.PubMedGoogle Scholar
  14. 14.
    Thomas M, Caroli J: Polycythemie et syndrome de Budd-Chiari: A propos de 17 observations. Ann Intern Med 122:1175–1186, 1971.Google Scholar
  15. 15.
    Levy VG, Ruskone A, Baillou C, et al: Polycythemia and the Budd-Chiari syndrome: Study of the serum erythropoietin and bone marrow erythroid progenitors. Hepatology 5:858–861, 1985.PubMedCrossRefGoogle Scholar
  16. 16.
    Bras G, Brandt KH: Vascular disorders, in MacSween RNM, Anthony PP, Scheuer PJ (eds): Pathology of the Liver. Churchill-Livingstone, Edinburgh, 1970, pp. 407–502.Google Scholar
  17. 17.
    Ingold JA, Reed GB, Kaplan HS, et al: Radiation hepatitis. AJR 93:200–208, 1965.Google Scholar
  18. 18.
    Reed GB, Cox AJ: The human liver after radiation injury: A form of veno-occlusive disease. Am J Pathol 48:597–612, 1966.PubMedGoogle Scholar
  19. 19.
    Fajardo LF, Berthrong M: Radiation injury in surgical pathology. Am J Surg Pathol 2:159–199, 1978.PubMedCrossRefGoogle Scholar
  20. 20.
    Fajardo LF, Colby TV: Pathogenesis of veno-occlusive disease after radiation. Arch Pathol Lab Med 104:584–588, 1980.PubMedGoogle Scholar
  21. 21.
    Rowland R, Pieterse AS, Kimber RJ, et al: Radiation veno-occlusive disease. Aust NZ J Med 11:534–538, 1981.CrossRefGoogle Scholar
  22. 22.
    Dejaard A, Krogsgaard K, Jacobsen M: Veno-occlusive disease and peliosis of the liver after Thorotrast administration. Virchows Arch Pathol Anat 403:87–94, 1984.CrossRefGoogle Scholar
  23. 23.
    Feigen M: Fatal veno-occlusive disease of the liver associated with herbal tea consumption and radiation. Aust NZ J Med 14:61–62, 1984.CrossRefGoogle Scholar
  24. 24.
    Krishnamachari KAVR, Bhat RV, Nagarajan V, et al: Hepatitis due to aflatoxicosis: An outbreak in Western India. Lancet 1:1061–1063, 1975.PubMedCrossRefGoogle Scholar
  25. 25.
    Tandon BN, Krishnamurthy L, Koshy A, et al: Study of an epidemic of jaundice, presumably due to toxic hepatitis, in Northwest India. Gastroenterology 72:488–494, 1977.PubMedGoogle Scholar
  26. 26.
    Russell RM, Boyer JL, Bagheri SA, et al: Hepatic injury from chronic hypervitaminosis A resulting in portal hypertension and ascites. N Engl J Med 291:435–440, 1974.PubMedCrossRefGoogle Scholar
  27. 27.
    Meacham GL, Tillotson FW, Heinle RW: Liver damage after prolonged urethane therapy. Am J Clin Pathol 22:22–27, 1952.PubMedGoogle Scholar
  28. 28.
    Brodsky I, Johnson H, Killman SA, et al: Fibrosis of central and hepatic veins, and perisinusoidal spaces of the liver following prolonged administration of urethane. Am J Med 29:976–980, 1961.CrossRefGoogle Scholar
  29. 29.
    Satti MB, Weinbren K, Gordon-Smith EC: 6-Thioguanine as a cause of toxic veno-occlusive disease of the liver. J Clin Pathol 35:1086–1091, 1982.PubMedCrossRefGoogle Scholar
  30. 30.
    D’Cruz CA, Wimmer, RS, Harcke HT, et al: Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia. Cancer 52:1803–1807, 1983.PubMedCrossRefGoogle Scholar
  31. 31.
    Wasser JS, Coleman M: Leukemia chemotherapy and centrilobular hepatic necrosis. Ann Intern Med 86:508–509, 1977.Google Scholar
  32. 32.
    Griner PF, Elbadawi A, Packman CH: Veno-occlusive disease of the liver after chemotherapy of acute leukemia: Report of two cases. Ann Intern Med 85:578–582, 1976.PubMedGoogle Scholar
  33. 33.
    Gill RA, Onstad GR, Cardamone JM, et al: Hepatic veno-occlusive disease caused by 6-thioguanine. Ann Intern Med 96:58–60, 1982.PubMedGoogle Scholar
  34. 34.
    Burkhardt A, Kloppel G: Unusual obliterative disease of the hepatic veins in an infant. Virchows Arch Pathol Anat 375:225–232, 1977.PubMedGoogle Scholar
  35. 35.
    Clark PA, Hsia Ye, Huntsman RG: Toxic complications of treatment with 6-mercaptopurine: Two cases with hepatic necrosis and intestinal ulceration. Br Med J 1:393–395, 1960.PubMedCrossRefGoogle Scholar
  36. 36.
    Houghton AN, Shafi N, Rickles FR: Acute hepatic vein thrombosis occurring during therapy for Hodgkin’s disease: A case report. Cancer 44:2324–2329, 1979.PubMedCrossRefGoogle Scholar
  37. 37.
    Asbury RF, Rosenthal SN, Decalzi ME, et al: Hepatic veno-occlusive disease due to DTIC. Cancer 45:2670–2674, 1980.PubMedCrossRefGoogle Scholar
  38. 38.
    Greenstone MA, Dowd PM, Mikhailidis DP, et al: Hepatic vascular lesions associated with dacarbazine treatment. Br Med J 282:1744–1745, 1981.CrossRefGoogle Scholar
  39. 39.
    Letendre L, Kovach JS, Ludwig J, et al: Hepatic veno-occlusive disease in patients with acute leukemia treated with indocine N-oxide. Proc Am Soc Clin Oncol Annu Mtg 22:488, 1981 (abst).Google Scholar
  40. 40.
    Jacobs P, Miller JL, Uys CJ, et al: Fatal veno-occlusive disease of the liver after chemotherapy, whole-body irradiation and bone marrow transplantation for refractory acute leukemia. S Afr Med J 55:5–10, 1979.PubMedGoogle Scholar
  41. 41.
    Schulman HM, McDonald GB, Mathews D, et al: An analysis of hepatic veno-occlusive disease and centrilobular hepatic degeneration following bone marrow transplantation. Gastroenterology 79:1178–1191, 1980.Google Scholar
  42. 42.
    Woods WG, Dehner LP, Nesbit ME, et al: Fatal veno-occlusive disease of the liver following high dose chemotherapy, irradiation and bone marrow transplantation. Am J Med 68:285–290, 1980.PubMedCrossRefGoogle Scholar
  43. 43.
    Beschorner WE, Pino J, Boitnott JK, et al: Pathology of the liver with bone marrow transplantation: Effects of busulfan, carmustine, acute graft-versus-host disease, and cytomegalovirus infection. Am J Pathol 99:369–386, 1980.PubMedGoogle Scholar
  44. 44.
    Lazarus HM, Gottfried MR, Herzig RH, et al: Veno-occlusive disease of the liver after high doses mitomycin C therapy and autologous bone marrow transplantation. Cancer 49:1789–1795, 1982.PubMedCrossRefGoogle Scholar
  45. 45.
    Gottfried MR, Sudilovsky O: Hepatic veno-occlusive disease after high-dose mitomycin C and autologous bone marrow transplantation therapy.Hum Pathol 13:646–650, 1982.PubMedCrossRefGoogle Scholar
  46. 46.
    Farthing MJG, Clark ML, Sloane JP, et al: Liver disease after bone marrow transplantation. Gut 23:465–474, 1982.PubMedCrossRefGoogle Scholar
  47. 47.
    McDonald GB, Sharma P, Mathews D, et al: Veno-occlusive disease of the liver after bone marrow transplantation: Diagnosis, incidence, and predisposing factors. Hepatology 4:123–130, 1984.CrossRefGoogle Scholar
  48. 48.
    McDonald GB, Shulman HM, Sullivan KM, et al: Intestinal and hepatic complications of human bone marrow transplantation. Part I. Gastroenterology 90:460–477, 1986.PubMedGoogle Scholar
  49. 49.
    Marubbio AT, Danielson B: Hepatic veno-occlusive disease in a renal transplant patient receiving azathioprine. Gastroenterology 69:739–743, 1975.PubMedGoogle Scholar
  50. 50.
    Eisenhauer T, Hartmann H, Rumpf KW, et al: Favorable outcome of hepatic veno-occlusive disease in a renal transplant patient receiving azathioprine, treated by portacaval shunt: Report of a case and review of the literature. Digestion 30:185–190, 1984.PubMedCrossRefGoogle Scholar
  51. 51.
    Weitz H, Gokel JM, Loeschka K, et al: Veno-occlusive disease of the liver in patients receiving immunosuppressive therapy. Virchows Arch Pathol Anat 395:245–256, 1982.PubMedGoogle Scholar
  52. 52.
    Katzka DA, Saul SH, Jorkasky D, et al: Azathioprine and hepatic venocclusive disease in renal transplant patients. Gastroenterology 90:446–454, 1986.PubMedGoogle Scholar
  53. 53.
    McLean C, Bras G, McLean AEM: Venous occlusions in the liver following dimethylnitrosamine. Br J Exp Pathol 3:367–369, 1965.Google Scholar
  54. 54.
    Unigar H: Veno-occlusive disease of the liver and phlebectatic peliosis in the golden hamster exposed to dimethylnitrosamine. Pathol Res Pract 181:180–187, 1986.CrossRefGoogle Scholar
  55. 55.
    Setchell KDR, Gosselin SJ, Welsh MB, et al: Dietary estrogens as a probable cause of infertility and liver disease in captive cheetahs. Gastroenterology 93:225–233, 1987.PubMedGoogle Scholar
  56. 56.
    Mellis C, Bale PM: Familial veno-occlusive disease with probable immune deficiency. J Pediatr 88:236–242, 1976.PubMedCrossRefGoogle Scholar
  57. 57.
    Goodman ZD, Ishak KG: Occlusive venous lesions in alcoholic liver disease: A study of 200 cases. Gastroenterology 83:786–796, 1982.PubMedGoogle Scholar
  58. 58.
    Nakanuma Y, Ohta G, Doishita K: Quantitation and serial section observations of focal veno-occlusive lesions of the hepatic veins in liver cirrhosis. Virchows Arch Pathol Anat 405:429–438, 1985.CrossRefGoogle Scholar
  59. 59.
    Burt AD, MacSween RNM: Hepatic vein lesions in alcoholic liver disease: Retrospective biopsy and necropsy study. J Clin Pathol 39:63–67, 1986.PubMedCrossRefGoogle Scholar
  60. 60.
    Baert AL, Fevery J, Marchai G, et al: Early diagnosis of Budd-Chiari syndrome by computed tomography and ultrasonography: Report of five cases. Gastroenterology 84:587–595, 1983.PubMedGoogle Scholar
  61. 61.
    Stark DD, Hahn PF, Trey C, et al: MRI of the Budd-Chiari syndrome. AJR 146:1141–1148, 1986.PubMedGoogle Scholar
  62. 62.
    Murphy FB, Steinberg HV, Shires GT III, et al: The Budd-Chiari syndrome: A review. AJR 147:9–15,1986.PubMedGoogle Scholar
  63. 63.
    Friedman AC, Ramchandari P, Black M, et al: Magnetic resonance imaging diagnosis of Budd-Chiari syndrome. Gastroenterology 91:1289–1295, 1986.PubMedGoogle Scholar
  64. 64.
    Vogelzang RL, Anschuetz SL, Gore RM: Budd-Chiari syndrome: CT observations. Radiology 163:329–333, 1987.PubMedGoogle Scholar
  65. 65.
    Sanfey H, Boitnott JK, Cameron JL: Surgical management of patients with the Budd-Chiari syndrome. World J Surg 8:706–715, 1984.PubMedCrossRefGoogle Scholar
  66. 66.
    McDermott WV, Stone MD, Bothe A, et al: Budd-Chiari syndrome. Historical and clinical review with an analysis of surgical corrective procedures. Am J Surg 147:463–467, 1984.PubMedCrossRefGoogle Scholar
  67. 67.
    McCarthy PM, van Heerden JA, Adson MA, et al: The Budd-Chiari syndrome. Medical and surgical management of 30 patients. Arch Surg 120:657–662, 1985.PubMedCrossRefGoogle Scholar
  68. 68.
    Gupta S, Blumgart LH, Hodgson HJF: Budd-Chiari syndrome: Long-term survival and factors affecting mortality. J Med 60:781–791, 1986.Google Scholar
  69. 69.
    Simson IW: Budd-Chiari syndrome and veno-occlusive disease, in Peters RL, Craig JR (eds): Liver Pathology. Churchill Livingstone, New York, 1986, pp. 299–314.Google Scholar
  70. 70.
    Nakamura T, Nakamura S, Aikawa T, et al: Obstruction of the inferior vena cava in the hepatic portion and the hepatic veins. Angiology 19:479–498, 1968.PubMedCrossRefGoogle Scholar
  71. 71.
    Simson IW: Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology 82:171–178, 1982.PubMedGoogle Scholar
  72. 72.
    Ishak KG, Sesterhenn IA, Goodman ZD, et al: Epithelioid hemangioendothelioma of the liver: A clinicopathologic and follow-up study of 32 cases. Hum Pathol 15:839–852, 1984.PubMedCrossRefGoogle Scholar
  73. 73.
    Taylor RW, Sylwesrtowicz T, Kossakowska AE, et al: Leiomyosarcoma of the inferior vena cava presenting as Budd-Chiara syndrome. Liver 7:201–205, 1987.PubMedGoogle Scholar
  74. 74.
    Ishak KG: Malignant mesenchymal tumors of the liver, in Okuda K, Ishak KG (eds): Neoplasms of the Liver. Springer-Verlag, Tokyo, 1987, pp. 159–176.Google Scholar
  75. 75.
    Nakashima T, Kojiro M: Hepatocellular Carcinoma. Springer-Verlag, Tokyo, 1987.Google Scholar

Copyright information

© Plenum Publishing Corporation 1989

Authors and Affiliations

  • Kamal G. Ishak
    • 1
  1. 1.Department of Hepatic PathologyArmed Forces Institute of PathologyUSA

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