Pathology of Non-cirrhotic Liver Disease

  • Masayoshi KageEmail author
  • Reiichirou Kondou
  • Jun Akiba


The pathology of non-cirrhotic liver diseases causing portal hypertension such as extrahepatic portal obstruction, idiopathic portal hypertension, nodular regenerative hyperplasia, veno-occlusive disease, and Budd-Chiari syndrome is mainly explained.

The interrupted region of the portal circulation and hepatic blood flow pattern vary depending on disease, and several factors are combined in the mechanism of portal pressure elevation in many cases. Accordingly, the pathology varies among diseases and cases, showing diversity. Understanding the pathology of the liver and vascular system in patients with non-cirrhotic liver diseases causing portal hypertension is important to accurately clarify the diverse pathology of those diseases and useful in making a diagnosis and prognosis.


Portal hypertension Liver pathology Extrahepatic portal obstruction (EHO) Idiopathic portal hypertension (IPH) Budd-Chiari syndrome (BCS) 


  1. 1.
    Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, Jafri W, Kumar A, Kudo M, Lesmana LA, Sharma BC, Shiba SG, de Silva HJ, Members of the APASL Working Party on Portal Hypertension. Consensus on extra-hepatic portal vein obstruction. Liver Int. 2006;26:512–9.CrossRefGoogle Scholar
  2. 2.
    Kunio O, Aoki H. Extrahepatic portal vein obstruction. In: Okuda K, Benhamou JP, editors. Portal hypertension. Tokyo: Springer; 1991. p. 365–79.Google Scholar
  3. 3.
    Ohnishi K, Okuda K, Ohtsuki T, et al. Formation of hilar collaterals or cavernous transformation after portal vein obstruction by hepatocellular carcinoma. Observations in ten patients. Gastroenterology. 1984;87:1150–3.PubMedGoogle Scholar
  4. 4.
    Cho KJ, Lunderquist A. The peribiliary vascular plexus: the microvascular architecture of the bile duct in the rabbit and in clinical cases. Radiology. 1983;147:357–69.CrossRefGoogle Scholar
  5. 5.
    Okuda K, Nakashima T, Okudaira K, et al. Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India. Liver. 1982;2:176–92.CrossRefGoogle Scholar
  6. 6.
    Dhiman RK, Chawla Y, Vasishta RK, et al. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol. 2002;17:6–16.CrossRefGoogle Scholar
  7. 7.
    Siramolpiwat S, et al. Idiopathic portal hypertension: natural history and long-term outcome. Hepatology. 2014;59:2276–85.CrossRefGoogle Scholar
  8. 8.
    Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis. 2002;22:59–71.CrossRefGoogle Scholar
  9. 9.
    Nakanuma Y, Sato Y, Kiktao A. Pathology and pathogenesis of portal venopathy in idiopathic portal hypertension: hints from systemic sclerosis. Hepatol Res. 2009;39(10):1023–31. Scholar
  10. 10.
    Kitao A, Sato Y, Sawada-Kitamura S, et al. Endothelial to mesenchymal transition via transforming growth factor-beta1/Smad activation is associated with portal venous stenosis in idiopathic portal hypertension. Am J Pathol. 2009;175(2):616–26. Scholar
  11. 11.
    Ohbu M, Okudaira M, Watanabe K, et al. Histopathological study of intrahepatic aberrant vessels in cases of noncirrhotic portal hypertension. Acta Hepatol. 1994;20:302–8.CrossRefGoogle Scholar
  12. 12.
    Steiner PE. Nodular regenerative hyperplasia of the liver. Am J Pathol. 1959;35:943–53.PubMedPubMedCentralGoogle Scholar
  13. 13.
    Wanless IR. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2500 autopsies and a new classification of benign hepatocellular nodules. Hepatology. 1990;11:787–97.CrossRefGoogle Scholar
  14. 14.
    Stromeyer FW, Ishak KG. Nodular transformation (nodular regenerative hyperplasia) of the liver-a clinico-pathologic study of 30 cases. Hum Pathol. 1981;12:60–71.CrossRefGoogle Scholar
  15. 15.
    Nakanuma Y. Nodular regenerative hyperplasia of the liver: retrospective survey in autopsy series. J Clin Gastroenterol. 1990;12:460–5.CrossRefGoogle Scholar
  16. 16.
    Wanless IR, Goldwin TA, Allen F, et al. Nodular regenerative hyperplasia of hematologic disorders: a possible response to obliterative portal venopathy-a morphometric study of nine cases with hypothesis on the pathogenesis. Medicine. 1980;59:367–79.CrossRefGoogle Scholar
  17. 17.
    Shulman HM, Fisher LB, Schoch HG, et al. Veno-occlusive disease of the liver after marrow transplantation: histological correlates of clinical signs and symptoms. Hepatology. 1994;19:1171–81.CrossRefGoogle Scholar
  18. 18.
    DeLeve LD, Shulman HM, McDonald GB. Characterization of a reproducible rat model of hepatic veno-occlusive disease. Hepatology. 1999;29:1779–91.CrossRefGoogle Scholar
  19. 19.
    McDonald GB, Sharma P, Matthews D, et al. Venoocclusive disease of the liver after bone marrow transplantation: diagnosis, incidence, and predisposing factors. Hepatology. 1984;4:116–22.CrossRefGoogle Scholar
  20. 20.
    Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology. 2003;38:793–803.CrossRefGoogle Scholar
  21. 21.
    Tanaka M, Wanless I. Pathology of the liver in Budd-Chiarisyndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodule. Hepatology. 1998;27:488–96.CrossRefGoogle Scholar
  22. 22.
    Okuda K. Membranous obstruction of the hepatic portion of the inferior vena cava. Etiology and relation to hepatocellular carcinoma. Gastroenterology. 1982;82:376–9.PubMedGoogle Scholar
  23. 23.
    Kew MC, McKnight A, Hodkinson J, et al. The role of membranous obstruction of the inferior vena cava in the etiology of hepatocellular carcinoma in Southern African blacks. Hepatology. 1989;9:121–5.CrossRefGoogle Scholar
  24. 24.
    Shrestha SM, Okuda K, Uchida T, et al. Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal. J Gastroenterol Hepatol. 1996;11:170–9.CrossRefGoogle Scholar
  25. 25.
    Kage M, Arakawa M, Kojiro M, et al. Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome. Gastroenterology. 1992;102:2081–90.CrossRefGoogle Scholar
  26. 26.
    Nakamura T, Nakamura S, Aikawa T, et al. Obstruction of the inferior vena cava in the hepatic portion and the hepatic veins. Angiology. 1968;19:479–98.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Kurume University Research Center for Innovative Cancer TherapyKurumeJapan
  2. 2.Department of PathologyKurume University, School of MedicineKurumeJapan
  3. 3.Department of Diagnostic PathologyKurume University HospitalKurumeJapan

Personalised recommendations