Extrahepatic portal hypertension

Authors

Keywords:

Non-cirrhotic portal hypertension, portal vein thrombosis, acute portal vein thrombosis, chronic portal vein thrombosis

Abstract

Extrahepatic portal venous obstruction is an important cause of non-cirrhotic portal hypertension, especially in third world countries. Material and methods. Contemporary literature analysis focused on the main databases: PubMed, Hinari, SpringerLink and Scopus (Elsevier) was performed. Inclusion criteria served clinical practice guidelines, scientific articles on current knowledge addressed: causes, diagnostic criteria and differential diagnosis of extrahepatic portal venous obstruction. Exclusion criteria were articles that did not meet the current requirements and information provided for patients. Results. The term non-cirrhotic portal hypertension refers to a heterogeneous group of liver diseases affecting the hepatic vasculature that are anatomically classified based on blood flow resistance as pre-hepatic, hepatic (pre-sinusoidal, sinusoidal or post-sinusoidal) and post-hepatic in the absence of liver cirrhosis or known risk factors responsible for portal hipertension. Multiple hematological, immune system-related, infectious, hereditary and metabolic risk factors have been associated with this disorder. However, the exact etiopathogenesis is largely unknown. Conclusions. The diagnosis of this pathology requires a multidisciplinary approach, through evaluation by a gastroenterologist, hematologist, cardiologist, and requires an increase in the degree of awareness of this entity by highlighting the complex pathophysiology and clinical-pathological associations.

References

1. Shah S.K., Butt J.A., Awan A. Profile of extrahepatic portal venous obstruction (EHPVO) in a tertiary care hospital in Pakistan. In: Pak J Med Sci. October - December 2007, (Part-I), vol. 23, No. 5, pp. 677-680.

2. Sarin S.K., Kumar A. Noncirrhotic portal hypertension. In: Clin Liver Dis. 2006, vol. 10, pp. 627-51. https://doi.org/10.1016/j.cld.2006.08.021

3. Gioia S., Nardelli S., Ridola L., Riggio O. Causes and Management of Non-cirrhotic Portal Hypertension. In: Curr Gastroenterol Rep. 2020, vol. 22(12), p. 56. https://doi.org/10.1007/s11894-020-00792-0

4. Kmeid M., Liu X., Ballentine S., Lee H. Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data. In: Gastroenterology Res. 2021, vol 14(2), pp. 49-65. https://doi.org/10.14740/gr1376

5. De Gottardi A. et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. In: Lancet Gastroenterol Hepatol. 2019, vol. 4(5), pp. 399-411. https://doi.org/10.1016/S2468-1253(19)30047-0

6. Cazals-Hatem D., Hillaire S., Rudler M. et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. In: J Hepatol. 2011, 54(3), pp. 455-461. https://doi.org/10.1016/j.jhep.2010.07.038

7. Siramolpiwat S., Seijo S., Miquel R. et al. Idiopathic portal hypertension: natural history and long-term outcome. In: Hepatology. 2014, 59(6), pp. 2276-2285. https://doi.org/10.1002/hep.26904

8. Gioia S., Nardelli S., Pasquale C. et al. Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis. In: Dig Liver Dis. 2018, 50(8), pp. 839-844. https://doi.org/10.1016/j.dld.2018.01.132

9. Seijo S., Reverter E., Miquel R. et al. Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. In: Dig Liver Dis. 2012, 44(10), pp. 855-860. https://doi.org/10.1016/j.dld.2012.05.005

10. Siramolpiwat S., Seijo S., Miquel R. et al. Idiopathic portal hypertension: natural history and long-term outcome. In: Hepatology. 2014, 59(6), pp. 2276-2285. https://doi.org/10.1002/hep.26904

11. Schouten J.N., Garcia-Pagan J.C., Valla D.C., Janssen H.L. Idiopathic noncirrhotic portal hypertension. In: Hepatology. 2011, vol. 54(3), pp. 1071-1081. https://doi.org/10.1002/hep.24422

12. Siramolpiwat S., Seijo S., Miquel R. et al. Idiopathic portal hypertension: natural history and long-term outcome. In: Hepatology. 2014, vol. 59(6), pp. 2276-2285. https://doi.org/10.1002/hep.26904

13. Riggio O., Gioia S., Pentassuglio I. et al. Idiopathic noncirrhotic portal hypertension: current perspectives. In: Hepat Med. 2016, 8, pp. 81-88. https://doi.org/10.2147/HMER.S85544

14. Glatard A.S., Hillaire S., D'Assignies G. et al. Obliterative portal venopathy: findings at CT imaging. In: Radiology. 2012;263(3):741-750. https://doi.org/10.1148/radiol.12111785

15. Krishnan P., Fiel M.I., Rosenkrantz A.B. et al. Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation. In: AJR Am J Roentgenol. 2012;198(2):370-376. doi: 10.2214/AJR.11.6855. https://doi.org/10.2214/AJR.11.6855

16. Vuppalanchi R., Mathur K., Pyko M. et al. Liver Stiffness Measurements in Patients with Noncirrhotic Portal Hypertension-The Devil Is in the Details. In: Hepatology. 2018, vol. 68(6), pp. 2438-2440. https://doi.org/10.1002/hep.30167

17. Gioia S., Nardelli S., Riggio O. Letter to the Editor: Liver Stiffness in Noncirrhotic Portal Hypertension: The Devil Is in the Diagnosis. In: Hepatology. 2019;70(1):444-445. https://doi.org/10.1002/hep.30367

18. De Gottardi A., Rautou P.E., Schouten J. Porto-sinusoidal vascular disease: proposal and description of a novel entity. Lancet Gastroenterol Hepatol. 2019, vol. 4(5), pp. 399-411. https://doi.org/10.1016/S2468-1253(19)30047-0

19. EUROPEAN ASSOCIATION FOR THE STUDY OF THE LIVER. EASL Clinical Practice Guidelines: Vascular diseases of the liver. In: J Hepatol. 2016, vol. 64(1), pp. 179-202. https://doi.org/10.1016/j.jhep.2015.07.040

20. De Franchis R. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. In: J Hepatol. 2015, vol. 63(3), pp. 743-752. https://doi.org/10.1016/j.jhep.2015.05.022

21. Valla D.C., Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. In: J Hepatol. 2000, vol. 32(5), pp. 865-871. https://doi.org/10.1016/S0168-8278(00)80259-7

22. Dhiman R.K., Saraswat V.A., Valla D.C. et al. Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver. In: J Clin Exp Hepatol. 2014, vol. 4(Suppl 1), pp. S2-S14. https://doi.org/10.1016/j.jceh.2014.02.003

23. Plessier A., Darwish-Murad S., Hernandez-Guerra M. et al. Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. In: Hepatology. 2010, vol. 51(1), pp. 210-218. https://doi.org/10.1002/hep.23259

24. Orr D.W., Harrison P.M., Devlin J. et al. Chronic mesenteric venous thrombosis: evaluation and determinants of survival during long-term follow-up. In: Clin Gastroenterol Hepatol. 2007, vol. 5(1), pp. 80-86. https://doi.org/10.1016/j.cgh.2006.09.030

25. Gioia S., Nardelli S., Riggio O. Letter to the Editor: Liver Stiffness in Noncirrhotic Portal Hypertension: The Devil Is in the Diagnosis. In: Hepatology. 2019, vol. 70(1), pp. 444-445. https://doi.org/10.1002/hep.30367

26. De Gottardi A., Rautou P.E., Schouten J. et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. In: Lancet Gastroenterol Hepatol. 2019, vol. 4(5), pp. 399-411. https://doi.org/10.1016/S2468-1253(19)30047-0

27. Gioia S., Nardelli S., Pasquale C. et al. Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis. In: Dig Liver Dis. 2018, vol. 50(8), pp. 839-844. https://doi.org/10.1016/j.dld.2018.01.132

28. Marot A. et al. A new classification of chronic portal vein occlusion for assessing the feasibility of recanalization in non-cirrhotic patients. Journal of Hepatology. 2017. Vol. 66. pp. S133. https://doi.org/10.1016/S0168-8278(17)30535-4

29. De Gottardi A., Trebicka J., Klinger C. et al. Antithrombotic treatment with direct-acting oral anticoagulants in patients with splanchnic vein thrombosis and cirrhosis. In: Liver Int. 2017, vol. 37(5), pp. 694-699. https://doi.org/10.1111/liv.13285

30. Priyanka P., Kupec J.T., Krafft M. et al. Newer Oral Anticoagulants in the Treatment of Acute Portal Vein Thrombosis in Patients with and without Cirrhosis. In: Int J Hepatol. 2018, vol. 2018, pp. 8432781. https://doi.org/10.1155/2018/8432781

31. Gioia, S., Nardelli S., Ridola L., Riggio O. Causes and Management of Non-cirrhotic Portal Hypertension. In: Curr Gastroenterol Rep. 2020, vol. 22(12), pp. 56. https://doi.org/10.1007/s11894-020-00792-0

32. Bissonnette J., Garcia-Pagán J.C., Albillos A. et al. Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension. In: Hepatology. 2016, vol. 64(1), pp. 224-231. https://doi.org/10.1002/hep.28547

33. Etzion O., Koh C., Heller T. Noncirrhotic portal hypertension: An overview. In: Clin Liver Dis (Hoboken). 2015, vol. 6(3), pp. 72-74. doi: 10.1002/cld.497. https://doi.org/10.1002/cld.497

Published

2026-04-17

How to Cite

[1]
Taran, N. et al. 2026. Extrahepatic portal hypertension. Public Health, Economy and Management in Medicine. 4(97) (Apr. 2026), 48–54.

Most read articles by the same author(s)

Similar Articles

1-10 of 85

You may also start an advanced similarity search for this article.