Skip to main content
SpringerLink
Log in

Idiopathic Portal Hypertension (Hepatoportal Sclerosis)

  • Chapter
Portal Hypertension

Abstract

Toward the end of the last century, Band [1] called attention to a disorder characterized by anemia and splenomegaly not associated with any known hematological disease. He emphasized the histological changes in the spleen which he called “fibroadenia”, and proposed that the disease presenting this morphologic feature be called “morbus Banti”. He divided the natural history of this disease into (a) the anemia phase with splenomegaly, (b) a transitional phase with urobilinuria, discoloration of the skin, and increasing gastrointestinal bleeding, and (c) and ascitic phase with atrophy of the liver terminating in death from hemorrhage or hepatic failure. According to him, the spleen was the primary seat of the disease, and anemia, hepatic cirrhosis, and sclerosing endophlebitis of the splenic and portal veins were the consequences. However, it soon became clear that fibroadenia of the spleen was not a specific change, being seen in various other diseases. In 1905, Naunyn [2] suggested that Banti’s disease represented liver cirrhosis with secondary splenomegaly and bleeding diathesis, and suggested that this disorder be called “splenomegalic liver cirrhosis”. This concept was supported by Eppinger [3], McNee [4], and others. The other theory put forward by Dock and Warthin [5] was a vascular and circulatory one which maintained that splenomegaly in patients who fulfilled the clinical criteria of Banti’s disease was a consequence of circulatory disturbances, particularly in patients with thrombophlebitic changes along the portal axis. This theory had advantages over the primary splenic disease concept when it was recognized that the histology of Banti spleen was mainly congestive, and that intrasplenic pressure was markedly elevated. Eppinger [3] among others advised that the term Banti’s disease be abandoned.

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 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight 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. Banti G (1898) Splenomegalie mit Leberizirrhose. Beitr Pathol Anat Allg Pathol 24: 21–33

    Google Scholar 

  2. Naunyn G (1905) Leberzirrhose. Verh Dtsch Ges Pathol 8: 57–75

    Google Scholar 

  3. Eppinger H (1920) Die Hepato-lienalen Erkrankungen. Springer, London, Berlin, Heidelberg, New York, Paris, Tokyo

    Book  Google Scholar 

  4. McNee JW (1932) Liver and spleen: Their clinical and pathological association. Br Med J 1: 1111–1116

    Article  PubMed  CAS  Google Scholar 

  5. Dock G, Warthin AS (1904) A clinical and pathological study on two cases of splenic anemia, with early and late stages of cirrhosis. Am J Med Sci 127: 24–55

    Article  Google Scholar 

  6. Rousselot LM (1936) The role of congestive (portal) hypertension in so-called Banti’s syndrome. JAMA 107: 1788–1793

    Article  Google Scholar 

  7. Ravenna P (1940) Banti’s syndrome (fibrocongestive splenomegaly). Definition, classification and pathogenesis. Arch Intern Med 66: 879–892

    Article  Google Scholar 

  8. Whipple AO (1945) The spleen of portal hypertension in relation to the hepatosplenopathies. Ann surg 122: 449–475

    Article  PubMed  CAS  Google Scholar 

  9. Osler W (1900) On splenic anemia. Am J Med Sci 119: 54–73

    Google Scholar 

  10. Tisdale WA, Katskin G, Glenn WWL (1959) Portal hypertension and bleeding esophageal varices. Their occurrence in the absence of both intrahepatic and extrahepatic obstruction of the portal vein. N Engl J Med 261: 209–214

    Article  PubMed  CAS  Google Scholar 

  11. Imanaga H, Yamamoto S, Kuroyanahgi Y (1962) Surgical treatment of portal hypertension according to state of intrahepatic circulation. Ann Surg 155: 43–50

    Article  Google Scholar 

  12. Ramalingaswami B, Wig HL, Sama SK (1962) Cirrhosis of the liver in northern India. A clinico-pathological study. Arch Intern Med 110: 350–358

    Article  PubMed  CAS  Google Scholar 

  13. Sama SK, Bhargava S, Gopi Nath N, Talwar JP, Nayak NC, Tandon BN, Wig KL (1971) Non-cirrhotic portal fibrosis. Am J Med 51: 160–169

    Article  PubMed  CAS  Google Scholar 

  14. Basu AK, Boyer J, Bhattacharya R, Basu Mallik KC, Sen Gupta LP (1967) Non-cirrhotic portal fibrosis with portal hypertension: a new syndrome. I. Clinical and function studies and results of operations. II. Histopathological studies. Ind J Med Res 55: 336–339

    CAS  Google Scholar 

  15. Datta DU (1976) Non-cirrhotic portal fibrosis (idiopathic portal hypertension in India). J Assoc Physicians India 24: 511–527

    PubMed  CAS  Google Scholar 

  16. Basu AK, Guharay BN (1978) Non-cirrhotic portal fibrosis with portal hypertension, Academic, Calcutta

    Google Scholar 

  17. Mikkelsen WP, Edmondson HA, Peters RL, Redeker AG, Reynolds TB (1965) Extra-and intra-hepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg 162: 602–620

    Article  PubMed  CAS  Google Scholar 

  18. Boyer JL, Hales MR, Klatskin G (1974) ‘Idiopathic’ portal hypertension due to occlusion of intrahepatic portal veins by organized thrombi. Medicine (Baltimore) 53: 87–91

    Google Scholar 

  19. Gray HK, Whitesell FB Jr (1950) An hemorrhage from esophageal varices: surgical management. Ann Surg 132: 798–810

    Article  PubMed  CAS  Google Scholar 

  20. Garrett N Jr, Gall EA (1953) Esophageal varices without hepatic cirrhosis. Arch Pathol Lab Med 55: 196–202

    CAS  Google Scholar 

  21. Taylor FW (1957) Experimental portal hypertension. Ann Surg 146: 683–690

    Article  PubMed  CAS  Google Scholar 

  22. Hallenbeck GA, Adson MA (1961) Esophagogastric varices without hepatic cirrhosis. A clinical study of treatment of their bleeding in 72 cases. Arch Surg 83: 370–383

    Article  PubMed  CAS  Google Scholar 

  23. Leather HM (1961) Portal hypertension and gross splenomegaly in Uganda. Br Med J 1: 15–18

    Article  PubMed  CAS  Google Scholar 

  24. Polish E, Christie J, Cohen A, Sullivan B Jr (1962) Idiopathic presinusoidal portal hypertension (Banti’s syndrome) Ann Intern Med 56: 624–627

    Article  PubMed  CAS  Google Scholar 

  25. Miller MC, Brandt JL (1962) Poral hypertension in the absence of both liver disease and vascular obstruction. Am J Dig Dis 7: 442–448

    Article  PubMed  CAS  Google Scholar 

  26. Cook J, MacFadzean AJS, Todd D (1963) Splenectomy in cryptogenetic splenomegaly. Br Med J 2: 337–344

    Article  PubMed  CAS  Google Scholar 

  27. Siderys H, Vellios F (1964) Portal hypertension without cirrhosis or extrahepatic obstruction. Report of a case. Am J Surg 108: 785–786

    CAS  Google Scholar 

  28. Ueda J, Kitani K, Kameda H, Takeda T, Chiba K, Nagatani M, Yamada H, I M (1971) Splenic blood flow in idiopathic portal hypertension in Japan measured by 85Kr clearance method. Acta Hepatosplenol 18: 28–40

    PubMed  CAS  Google Scholar 

  29. McMichael J (1934) The pathology of hepatolienal fibrosis. J Pathol Bacteriol 39: 481–502

    Article  Google Scholar 

  30. Okuda K, Nakashima T, Okudaira M, Kage M, Aida Y, Omata M, Sugiura M, Kameda H, Inokuchi K, Bhusnurmath SR, Aikat BA (1982) Liver pathology idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India. Liver 2: 176–192

    Article  PubMed  CAS  Google Scholar 

  31. Okuda K, Omata M (1983) Idiopathic portal hypertension. University of Tokyo Press, Tokyo

    Google Scholar 

  32. Eckhauser FE, Appleman HD, Knol JA, Strodel WE, Coran AG, Turcotte JG (1983) Non-cirrhotic portal hypertension: differing patterns of disease in children and adults. Surgery 94: 721–728

    PubMed  CAS  Google Scholar 

  33. Kingham JGC, Levison DA, Stansfeld AG, Dawson AM (1981) Non-cirrhotic intrahepatic portal hypertension: a long term follow-up study. Q J Med 50: 259–268

    PubMed  CAS  Google Scholar 

  34. Kuley M, Ulagay I, Vardar A, Uenerman M, Oran M (1972) Idiopatik portal hipertensiyon. Turk Tip Cemiyeti Mecmuasi Ayri Baski 38: 497–502

    CAS  Google Scholar 

  35. Villeneuve JP, Huet PM, Joly JG, Marleau M, Cote J, Legare A, Lafortune M, Lavoie P, Viallet A (1976) Idiopathic portal hypertension. Am J Med 61: 459–464

    Article  PubMed  CAS  Google Scholar 

  36. Nundy S, Nayak NC (1983) The relationship between non-cirrhotic portal fibrosis and extra-hepatic portal-splenic vein obstruction: A New Delhi study. In: Okuda K, Omata M (eds) Idiopathic portal hypertension. University of Tokyo Press, Tokyo, pp 197–205

    Google Scholar 

  37. Okuda K, Nakashima T, kameda H, Sugiura M, Ohnishi K, Kobayashi M, Japan Ministry of Health and Welfare Research Committee on Idiopathic Portal Hypertension (1985) Idiopathic noncirrhotic portal hypertension: A national study. In: Brunner H, Thaler H (eds) Hepatology: A Festschrift for Hans Popper. Raven, New YOrk, pp 95–108

    Google Scholar 

  38. Japan Mimistry of Health and Welafre Research Committee on Portal Hemodynamics Abnormalities (1988) Survey on therapeutic results in idiopathic portal hypertension and extrahepatic portal obstruction, Tokyo

    Google Scholar 

  39. Nayak NC, Ramalingaswami B (1969) Obliterative portal venopathy of the liver. Arch Pathol Lab Med 87: 359–369

    CAS  Google Scholar 

  40. Takashi M, Igarashi M, Hino S, Goto N, Okuda K (1984) Chronic portal-systemic enceppalopathy with normal portal vein pressure possibly due to noncirrhotic portal fibrosis. Dig Dis Sci 29: 669–673

    Article  PubMed  CAS  Google Scholar 

  41. Okuda K (1986) Idiopathic portal hypertension. In: Thomas HC, Jones EA (eds). Recent advances in heptology. Churchill Livingstone, Edinburgh, pp 93-108

    Google Scholar 

  42. Sherlock S, Feldman DA, Moran G, Scheuer PJ (1966) Partial nodular transformation of the liver with portal hypertension. Am J Med 40: 195–203

    Article  PubMed  CAS  Google Scholar 

  43. Aikat BK, Bhusnurmath SR, Chuttani PN, Mitra SK, Dutta DV (1979) The pathology of noncirrhotic portal fibrosis. Hum Pathol 10: 405–418

    Article  PubMed  CAS  Google Scholar 

  44. Levison DA, Kingham JGC, Dawson AM, Stansfeld AG (1982) Slow cirrhosis—or no cirrhosis? A lesion causing benign intrahepatic portal hypertension. J Pathol 137: 253–272

    Article  PubMed  CAS  Google Scholar 

  45. Nakagawa S (1983) Pathology of the spleen in idiopathic portal hypertension. In: Okuda K, Omata M (eds) Idiopathic portal hypertension. University of Tokyo Press, Tokyo, pp 207–217

    Google Scholar 

  46. Thomas LB, Popper H, Berk PD, Selikoff I, Falk H (1975) Vinyl-chloride-induced liver disease. From idiopathic portal hypertension (Banti’s syndrome) to angiosarcoma. N Engl J Med 292: 17–22

    Article  PubMed  CAS  Google Scholar 

  47. Morris JS, Schmid M, Newman S, Scheuer PJ, Path MRC, Sherlock S (1974) Arsenic and noncirrhotic portal hypertension. Gastroenterology 64: 86–94

    Google Scholar 

  48. Pimental JC, Menezes AP (1977) Liver disease in vineyard sprayers. Gastroenterology 72: 275–283

    Google Scholar 

  49. Ungar H (1984) Primary portal venopathy in the golden hamsters treated with low doses of dimethylnitrosamine. Liver 4: 244–254

    Article  PubMed  CAS  Google Scholar 

  50. Kameda H, Yamazaki K, Imai F, Sugiura M, Nakashima T, Okuda K and the Japan Ministry of Health and Welfare Research Committee on Idiopathic Portal Hypertension (1986) Obliterative portal venopathy: A comparative study of 184 cases of extrahepatic portal obstruction and 469 cases of idiopathic portal hypertension. J Gastroenterol Hepatol 1: 139–149

    Article  Google Scholar 

  51. Okuda K and Idiopathic Hypertension Research Committee, Japan Ministry of Health and Welfare (1983) Epidemiology of idiopathic portal hypertension. In: Okuda K, Omata (eds). Idiopathic portal hypertension. University of Tokyo Press, Tokyo pp 3–12

    Google Scholar 

  52. Kern F Jr (1976) Hepatobiliary disorders in inflammatory bowel disease. In: Popper H, Schaffner F (eds) Progress in liver diseases. Grune and Stratton, New York, pp 575–589

    Google Scholar 

  53. Kono K, Ohnishi K, Omata M, Saito M, Nakayama T, Hatano H, Nakajima Y, Sugita S, Okuda K (1988) Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Eschrichia coli in rabbits. Gastroenterology 94: 787–796

    PubMed  CAS  Google Scholar 

  54. Sugita S, Ohnishi K, Iida S, Nomura F, Okuda K (1988) Histological changes in the liver and portal hypertension subsequent to repeated intraportal injections of killed E. coli in the dog. Liver 8: 1–9

    Article  PubMed  CAS  Google Scholar 

  55. Sugita S, Ohnishi K, Saito M, Okuda K (1987) Splanchnic hemodynamics in portal hypertensive dogs with portal fibrosis. Am J Physiol 252: G748–G754

    PubMed  CAS  Google Scholar 

  56. Burgener FA, Gutierrez OH, Logsdon GA (1982) Angiographic, hemodynamic and histologic evaluation of portal hypertension and periportal fibrosis induced in the dog by intraportal polyvinyl alcohol injection. Radiology 143: 379–385

    PubMed  CAS  Google Scholar 

  57. Moriyasu F, Nishida O, Ban N, Nakayamura T, Miura K, Sakai M, Miyake T, Uchino H (1986) Measurement of portal vascular resistance in patients with portal hypertension. Gastroenterology 90: 710–717

    PubMed  CAS  Google Scholar 

  58. Obata H (1988) Immunologie study of IPH (in Japanese). In: Kameda H (ed) Portal hemodynamics abnormalities. Recent progress. Japan Ministry of Health and Welfare Research Committee on Portal Hemodynamics Abnormalities, pp 71-79

    Google Scholar 

  59. Hofman SL, Piessens WF, Ratiwayanto S, Hussein PR, Kurniawan L, Piessens PW, Campbell J, Marwoto HA (1984) Reduction of suppressor T lymphocytes in the tropical splenomegaly syndrome. N Engl J Med 310: 337–341

    Article  Google Scholar 

  60. Williams R, Parsonson A, Somers K, Hamilton PJS (1966) Portal hypertension in idiopathic tropical splenomegaly. Lancet I: 329–333

    Article  Google Scholar 

  61. Suzuki T (1964) Morbus Banti as a definite morbid entity. Nippon Ketsueki Gakkai Zasshi 26: 820–831

    Google Scholar 

  62. Okabayashi A (1983) Experimental production of non-cirrhotic portal hypertension and splenomegaly with prolonged stimulation. In: Okuda K, Omata M (eds) Idiopathic portal hypertension. University of Tokyo Press, Tokyo, pp 197–205

    Google Scholar 

  63. Iuchi M, Kitani K (1983) Rapid development of gross splenomegaly in the course of chronic schistosomiasis japonica. In: Okuda K, Omata M (eds) Idiopathic portal hypertension. University of Tokyo Press, Tokyo, pp 241–252

    Google Scholar 

  64. Donovan AJ, Reynolds TB, Mikkelsen WP, Peters RL (1969) Systemic-portal arteriovenous fistulas: pathological and hemodynamic observations in two patients. Surgery 94: 721–728

    Google Scholar 

  65. Fogarty International Center (1976) Diseases of the liver and biliary tract. Standardization of nomenclature, diagnostic criteria, and diagnostic methodology, Fogarty International Center, Washington, DC

    Google Scholar 

  66. Okuda K, Kono K, Ohnishi K, Kimura K, Omata M, Koen H, Nakajima Y, Musha H, Hirashima T, Takashi M, Takayasu K (1984) Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 86: 600–610

    PubMed  CAS  Google Scholar 

  67. James SP, Elison CO, Waggoner JG, Jones EA (1980) Edficiency of the autologous mixed lymphocyte reaction in patients with primary biliary cirrhosis. J Clin Invest 66: 1305–1310

    Article  PubMed  CAS  Google Scholar 

  68. Kurihara T, Yamauchi K, Nakanishi T, Obata H (1987) Failure of autologous lymphocyte reaction in patients with auto-immune chronic active hepatitis. J Gastroenterol Hepatol 2: 19–25

    Article  Google Scholar 

  69. Smith JB, Knowlton RP (1979) Activation of suppressor T cells in human autologous mixed lymphocyte culture. J Immunol 123: 419–422

    PubMed  CAS  Google Scholar 

  70. Fujimoto Y (1982) Immunological studies of idiopathic portal hypertension with special reference to analysis of suppressor T cell in peripheral lymphocytes. J Osaka City Med Coll 31: 139–154

    Google Scholar 

  71. Shikata T, Uzawa Y, Yamazake S (1976) Causal genesis of liver fibrosis accompanied with portal hypertension. Acta Hepatol Jpn 17: 417–424

    Article  Google Scholar 

  72. Ito Y, Omata M, Uchiumi K, Terao K, Okuda K (1983) Ultrastructural and morphometric study of the liver in idiopathic portal hypertension. In: Okuda K, Omata M (eds) Idiopthic portal hypertension. University of Tokyo Press, Tokyo, pp 143–153

    Google Scholar 

  73. Ohnishi K, Saito M, Sato S, Terabayashi H, Iida S, Nomura F, Nakano M, Okuda K (1987) Portal hemodynamics in idiopathic portal hypertension (Banti’s syndrome). Comparison with chronic persistent hepatitis and normal subjects. Gastroenterology 92: 751–758

    PubMed  CAS  Google Scholar 

  74. Futagawa S, Fukazawa M, Horisawa M, Musha H, Sugiura M, Kameda H, Okuda K (1980) Portogarphic liver changes in idiopathic portal hypertension. AJR 34: 917–923

    Article  Google Scholar 

  75. Futagawa S, Fukazawa M, Musha H, Isomatsu T, Koyama K, Ito T, Horisawa M, Nakayama S, sugiura M, Kameda H, Okuda K (1981) Hepatic venography in non-cirrhotic idiopathic portal hypertension. Comparison with cirrhosis of the liver. Radiology 141: 303–309

    PubMed  CAS  Google Scholar 

  76. Okuda K, Nakashima T, Okudaira M, Kage M, Aida Y, Omata M, Musha H, Futagawa S, Sugiura M, Kameda H (1981) Anatomical basis of hepatic venographic alterations in idiopathic portal hypertension. Liver 1: 255–263

    Article  PubMed  CAS  Google Scholar 

  77. Rosenbaum DL, Murphy CW, Swisher SN (1966) Hemodynamic studies of the portal circulation in myeloid metaplasia. Am J Med 41: 360–369

    Article  PubMed  CAS  Google Scholar 

  78. Shaldon S, Sherlock S (1962) Portal hypertension in the myeloproliferative syndrome and reticulosis. Am J Med 32: 758–764

    Article  PubMed  CAS  Google Scholar 

  79. Blendis LM, Ansell ID, Lloyd-Jones K, Hamilton E, Williams R (1970) Liver in Felty’s syndrome. Br Med J 1: 131–135

    Article  PubMed  CAS  Google Scholar 

  80. Reisman T, Levi JU, Zeppa R, Clark R, Morton R, Schiff ER (1977) Noncirrhotic portal hypertension in Felty’s syndrome. A J Dig Dis 22: 145–148

    Article  CAS  Google Scholar 

  81. Mosimann F, Mange B (1980) Portal hypertension as a complication of idiopathic retroperitoneal fibrosis. Br J Surg 67: 804

    Article  PubMed  CAS  Google Scholar 

  82. Guarda LA, Jales MR (1981) Nodular regenerative hyperplasia of the liver: Report of two cases and review of the literature. J Clin Gastroenterol 3: 157–164

    Article  PubMed  CAS  Google Scholar 

  83. Nataf C, Feldmann G, Lebrec D, Descamps JM, Rueff B, Benhamou JP (1979) Idiopathic portal hypertension (perisinusoidal fibrosis) after renal transplantation. Gut 20: 531–537

    Article  PubMed  CAS  Google Scholar 

  84. Wanless IR (1987) The pathophysiology of noncirrhotic portal hypertension: A pathologist’s perspective. In: Boyer JL, Bianchi L (eds). Liver cirrhosis. MTP Press, Lancaster, pp 293–311

    Google Scholar 

  85. Boyer JL, Jales MR, Klatskin G (1974) “Idiopathic” portal hypertension due to occlusion of intrahepatic portal veins by organized thrombi. A study bases on postmorten vinylite-injection corrosion and dissection of the intrahepatic vas-culature in 4 cases. Medicine 53: 77–91

    Article  PubMed  CAS  Google Scholar 

  86. Wanless IR, Bernier V, Seger M (1982) Intrahepatic portal vein sclerosis in patients without a history of liver disease. An autopsy study. Am J Pathol 106: 63–70

    PubMed  CAS  Google Scholar 

  87. Nakanuma Y, Ohta G, Kurumaya H, Doishita K, Takayanagi N, Rin S (1984) Pathological study on livers with noncirrhotic portal hypertension and portal venous thromboembolic occlusion: Report of seven autopsy cases. Am J Gastroenterol 79: 782–789

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine, Chiba University School of Medicine, Chiba, 280, Japan

    Kunio Okuda

  2. Institute of Gastroenterology, Tokyo Women’s Medical College, Tokyo, 162, Japan

    Hiroshi Obata

Authors
  1. Kunio Okuda
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hiroshi Obata
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Medicine, Chiba University Hospital, Inohana 1-8-1, Chiba, 280, Japan

    Kunio Okuda M.D., Ph.D. (Professor) (Professor)

  2. Department of Hepatology, Hopital Beaujon, 92118, Clichy, France

    Jean-Pierre Benhamou M.D. (Professor) (Professor)

Rights and permissions

Reprints and permissions

Copyright information

© 1991 Springer Japan

About this chapter

Cite this chapter

Okuda, K., Obata, H. (1991). Idiopathic Portal Hypertension (Hepatoportal Sclerosis). In: Okuda, K., Benhamou, JP. (eds) Portal Hypertension. Springer, Tokyo. https://doi.org/10.1007/978-4-431-68361-2_21

Download citation

  • DOI: https://doi.org/10.1007/978-4-431-68361-2_21

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-68363-6

  • Online ISBN: 978-4-431-68361-2

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation