Non-cirrhotic portal hypertension: an imaging review

  • S. Rajesh
  • Amar Mukund
  • Binit Sureka
  • Kalpana Bansal
  • Maxime Ronot
  • Ankur Arora
Invited article
  • 84 Downloads

Abstract

Non-cirrhotic portal hypertension (NCPH) comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver. It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver. The natural course of NCPH is largely indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed and regular surveillance is carried out. However, due to the numerous and variable etiological factors and lack of standardized diagnostic criteria, this entity is under-recognized clinically as well as pathologically and often gets mislabeled as cryptogenic cirrhosis. Therefore, it is imperative for radiologists to be aware of the imaging features of various entities that constitute NCPH to facilitate early recognition of this disorder. The present review shall comprehensively discuss the role of imaging in the diagnosis of obliterative portal venopathy, which is a common and one of the most poorly understood disorders leading to NCPH. The other radiologically relevant entities will also be briefly appraised.

Keywords

Non-cirrhotic Portal Idiopathic Obliterative Venopathy 

Notes

Acknowledgements

The present review is based on the electronic poster presented as part of ESGAR 2015 entitled “Not all varices mean cirrhosis!: What every radiologist should know about Non-cirrhotic Portal Hypertension (NCPH)” by Arora A, Rajesh S, Sarin S K.

Compliance with ethical standards

Conflict of interest

All authors state that there are no conflicts of interests.

References

  1. 1.
    Sarin SK, Khanna R (2014) Non-cirrhotic portal hypertension. Clin Liver Dis 18(2):451–476CrossRefPubMedGoogle Scholar
  2. 2.
    Khanna R, Sarin SK (2014) Non-cirrhotic portal hypertension - diagnosis and management. J Hepatol. 60(2):421–441CrossRefPubMedGoogle Scholar
  3. 3.
    Rajesh S, Mukund A, Arora A (2015) Imaging diagnosis of splanchnic venous thrombosis. Gastroenterol Res Pract. 101029.Google Scholar
  4. 4.
    Ponziani FR, Zocco MA, Campanale C, et al. (2010) Portal vein thrombosis: insight into physiopathology, diagnosis, and treatment. World J Gastroenterol 16(2):143–155CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Arora A, Sarin SK (2015) Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know. Br J Radiol 88(1052):20150008CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Pargewar SS, Desai SN, Rajesh S, et al. (2016) Imaging and radiological interventions in extra-hepatic portal vein obstruction. World J Radiol 8(6):556–570CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Weiss B, Shteyer E, Vivante A, et al. (2010) Etiology and long-term outcome of extrahepatic portal vein obstruction in children. World J Gastroenterol 16(39):4968–4972CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Condat B, Vilgrain V, Asselah T, et al. (2003) Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology 37(6):1302–1308CrossRefPubMedGoogle Scholar
  9. 9.
    Shin SM, Kim S, Lee JW, et al. (2007) Biliary abnormalities associated with portal biliopathy: evaluation on MR cholangiography. AJR Am J Roentgenol 188(4):W341–W347CrossRefPubMedGoogle Scholar
  10. 10.
    Walser EM, Runyan BR, Heckman MG, et al. (2011) Extrahepatic portal biliopathy: proposed etiology on the basis of anatomic and clinical features. Radiology 258(1):146–153CrossRefPubMedGoogle Scholar
  11. 11.
    Arora A, Rajesh S, Sarin SK (2015) Not all varices mean cirrhosis!: what every radiologist should know about non-cirrhotic portal hypertension (NCPH). Electron Post Present Part ESGAR.  https://doi.org/10.5444/esgar2015/EE-123 Google Scholar
  12. 12.
    Liu Q, Song Y, Xu X, et al. (2014) Management of bleeding gastric varices in patients with sinistral portal hypertension. Dig Dis Sci 59(7):1625–1629CrossRefPubMedGoogle Scholar
  13. 13.
    Wang L, Liu GJ, Chen YX, Dong HP, Wang LX (2012) Sinistral portal hypertension: clinical features and surgical treatment of chronic splenic vein occlusion. Med Princ Pract 21(1):20–23CrossRefPubMedGoogle Scholar
  14. 14.
    Kokabi N, Lee E, Echevarria C, Loh C, Kee S (2010) Sinistral portal hypertension: presentation, radiological findings, and treatment options: a case report. J Radiol Case Rep 4(10):14–20PubMedPubMedCentralGoogle Scholar
  15. 15.
    Saad WE (2012) Arterioportal fistulas in liver transplant recipients. Semin Intervent Radiol 29(2):105–110CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Zhang DY, Weng SQ, Dong L, Shen XZ, Qu XD (2015) Portal hypertension induced by congenital hepatic arterioportal fistula: report of four clinical cases and review of the literature. World J Gastroenterol 21(7):2229–2235CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Hernández M, González I, Llop E, et al. (2016) Arterioportal fistula, a rare cause of presinusoidal portal hypertension. Gastroenterol Hepatol 39(1):43–45CrossRefPubMedGoogle Scholar
  18. 18.
    Dumortier J, Pilleul F, Adham M, et al. (2007) Severe portal hypertension secondary to arterio-portal fistula: salvage surgical treatment. Liver Int 27(6):865–868CrossRefPubMedGoogle Scholar
  19. 19.
    Wanless IR, Peterson P, Das A, et al. (1990) Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. Hepatology 12(5):1166–1174CrossRefPubMedGoogle Scholar
  20. 20.
    Toros AB, Gokcay S, Cetin G, et al. (2013) Portal hypertension and myeloproliferative neoplasms: a relationship revealed. ISRN Hematol 16:673781Google Scholar
  21. 21.
    Abu-Hilal M, Tawaker J (2009) Portal hypertension secondary to myelofibrosis with myeloid metaplasia: a study of 13 cases. World J Gastroenterol 15(25):3128–3133CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Lindor K, Rakela J, Perrault J, Van Heerden J (1987) Noncirrhotic portal hypertension due to lymphoma. Reversal following splenectomy. Dig Dis Sci 32(9):1056–1058CrossRefPubMedGoogle Scholar
  23. 23.
    Arora A, Sarin SK (2015) Multimodality imaging of obliterative portal venopathy: what every radiologist should know. Br J Radiol 88(1046):20140653CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Aggarwal S, Fiel MI, Schiano TD (2013) Obliterative portal venopathy: a clinical and histopathological review. Dig Dis Sci 58(10):2767–2776CrossRefPubMedGoogle Scholar
  25. 25.
    Cazals-Hatem D, Hillaire S, Rudler M, et al. (2011) Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 54(3):455–461CrossRefPubMedGoogle Scholar
  26. 26.
    Guido M, Sarcognato S, Sonzogni A, et al. (2016) Obliterative portal venopathy without portal hypertension: an underestimated condition. Liver Int 36(3):454–460CrossRefPubMedGoogle Scholar
  27. 27.
    Franchi-Abella S, Fabre M, Mselati E, et al. (2014) Obliterative portal venopathy: a study of 48 children. J Pediatr 165(1):190–193CrossRefPubMedGoogle Scholar
  28. 28.
    Zuo C, Chumbalkar V, Ells PF, Bonville DJ, Lee H (2017) Prevalence of histological features of idiopathic noncirrhotic portal hypertension in general population: a retrospective study of incidental liver biopsies. Hepatol Int. 11(5):452–460CrossRefPubMedGoogle Scholar
  29. 29.
    Okuda K, Nakashima T, Okudaira M, et al. (1982) Liver pathology of idio-pathic portal hypertension: comparison with non-cirrhotic portal fibrosis of India. The Japan idiopathic portal hypertension study. Liver Int 2(16):176–192Google Scholar
  30. 30.
    Schouten JN, Verheij J, Seijo S (2015) Idiopathic non-cirrhotic portal hypertension: a review. Orphanet J Rare Dis 10(17):67CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Sarin SK, Kumar A, Chawla YK, et al. (2007) Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int 1(18):398–413CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Krishnasamy VP, Hagar MJ, Chun AK, Levy E (2015) Noncirrhotic portal hypertension: imaging, hemodynamics, and endovascular therapy. Clin Liver Dis 6:67–71CrossRefGoogle Scholar
  33. 33.
    Maruyama H, Okugawa H, Kobayashi S, et al. (2012) Non-invasive portography: a microbubble-induced three-dimensional sonogram for discriminating idiopathic portal hypertension from cirrhosis. Br J Radiol 85(1013):587–595CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Maruyama H, Shimada T, Ishibashi H, et al. (2012) Delayed periportal enhancement: a characteristic finding on contrast ultrasound in idiopathic portal hypertension. Hepatol Int 6(2):511–519CrossRefPubMedGoogle Scholar
  35. 35.
    Furuichi Y, Moriyasu F, Taira J, et al. (2013) Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography. J Gastroenterol 48(20):1061–1068CrossRefPubMedGoogle Scholar
  36. 36.
    Seijo S, Reverter E, Miquel R, et al. (2012) Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. Dig Liver Dis 44(10):855–860CrossRefPubMedGoogle Scholar
  37. 37.
    Glatard AS, Hillaire S, d’Assignies G, et al. (2012) Obliterative portal venopathy: findings at CT imaging. Radiology 263(21):741–750CrossRefPubMedGoogle Scholar
  38. 38.
    Vilgrain V, Lagadec M, Ronot M (2016) Pitfalls in liver imaging. Radiology 278(1):34–51CrossRefPubMedGoogle Scholar
  39. 39.
    Krishnan P, Fiel MI, Rosenkrantz AB, et al. (2012) Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation. AJR Am J Roentgenol 198(23):370–376CrossRefPubMedGoogle Scholar
  40. 40.
    Waguri N, Suda T, Kamura T, Aoyagi Y (2002) Heterogeneous hepatic enhancement on CT angiography in idiopathic portal hypertension. Liver 22(22):276–280CrossRefPubMedGoogle Scholar
  41. 41.
    Santiago I, Vilgrain V, Cipriano MA, et al. (2012) Case 183: obliterative portal venopathy. Radiology 264(1):297–302CrossRefPubMedGoogle Scholar
  42. 42.
    Futagawa S, Fukazawa M, Horisawa M, et al. (1980) Portographic liver changes in idiopathic noncirrhotic portal hypertension. AJR Am J Roentgenol 134:917–923CrossRefPubMedGoogle Scholar
  43. 43.
    Poddar U, Thapa BR, Vashishta RK, Girish CS, Singh K (1999) Congenital hepatic fibrosis in Indian children. J Gastroenterol Hepatol 14(12):1192–1196CrossRefPubMedGoogle Scholar
  44. 44.
    Zeitoun D, Brancatelli G, Colombat M, et al. (2004) Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 231:109–116CrossRefPubMedGoogle Scholar
  45. 45.
    Davies CH, Stringer DA, Whyte H, Daneman A, Mancer K (1986) Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidney diseases. Pediatr Radiol 16:302–305CrossRefPubMedGoogle Scholar
  46. 46.
    Akhan O, Karaosmanoğlu AD, Ergen B (2007) Imaging findings in congenital hepatic fibrosis. Eur J Radiol 61(1):18–24CrossRefPubMedGoogle Scholar
  47. 47.
    Lehmuskallio E, Hannuksela M, Halme H (1977) The liver in sarcoidosis. Acta Med Scand 202:289–293CrossRefPubMedGoogle Scholar
  48. 48.
    Lynch JP III (1998) Extrapulmonary sarcoid. Semin Respir Infect 13:229–254PubMedGoogle Scholar
  49. 49.
    Fetzer DT, Rees MA, Dasyam AK, Tublin ME (2016) Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution. Eur Radiol 26(9):3129–3137CrossRefPubMedGoogle Scholar
  50. 50.
    Ebert EC, Kierson M, Hagspiel KD (2008) Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol 103(12):3184–3192CrossRefPubMedGoogle Scholar
  51. 51.
    Karaosmanoğlu AD, Onur MR, Saini S, Taberi A, Karcaaltincaba M (2015) Imaging of hepatobiliary involvement in sarcoidosis. Abdom Imaging 40(8):3330–3337CrossRefPubMedGoogle Scholar
  52. 52.
    Warshauer DM, Joseph K, Lee T (2004) Imaging manifestations of abdominal sarcoidosis. Am J Roentgenol 182:15–28CrossRefGoogle Scholar
  53. 53.
    Warshauer DM, Molina PL, Hamman SM, et al. (1995) Nodular sarcoidosis of the liver and spleen: analysis of 32 cases. Radiology 195:757–762CrossRefPubMedGoogle Scholar
  54. 54.
    Kessler A, Mitchell DG, Israel HL, Goldberg BB (1993) Hepatic and splenic sarcoidosis: ultrasound and MR imaging. Abdom Imaging 18:159–163CrossRefPubMedGoogle Scholar
  55. 55.
    Elbaz T, Esmat G (2013) Hepatic and intestinal schistosomiasis: review. J Adv Res 4(5):445–452CrossRefPubMedPubMedCentralGoogle Scholar
  56. 56.
    Bächler P, Baladron MJ, Menias C, et al. (2016) Multimodality Imaging of Liver Infections: differential Diagnosis and Potential Pitfalls. Radiographics 36(4):1001–1023CrossRefPubMedGoogle Scholar
  57. 57.
    Mortelé KJ, Segatto E, Ros PR (2004) The infected liver: radiologic-pathologic correlation. Radiographics 24(4):937–955CrossRefPubMedGoogle Scholar
  58. 58.
    Patel SA, Castillo DF, Hibbeln JF, Watkins JL (1993) Magnetic resonance imaging appearance of hepatic schistosomiasis, with ultrasound and computed tomography correlation. Am J Gastroenterol 88:113–116PubMedGoogle Scholar
  59. 59.
    Yeh MJ, Kim SY, Jhaveri KS, et al. (2017) Imaging of autoimmune biliary disease. Abdom Radiol 42(1):3–18CrossRefGoogle Scholar
  60. 60.
    Bressler B, Pinto R, El-Ashry D, Heathcote EJ (2005) Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic screening for oesophageal varices detection? Gut 54(3):407–410CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Blachar A, Federle MP, Brancatelli G (2001) Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology 220(2):329–336CrossRefPubMedGoogle Scholar
  62. 62.
    Dodd GD III, Baron RL, Oliver JH III, et al. (1997) Enlarged abdominal lymph nodes in end-stage cirrhosis: CT-histologic correlation in 507 patients. Radiology 203:127–130CrossRefPubMedGoogle Scholar
  63. 63.
    Wenzel JS, Donohoe A, Ford KL 3rd, et al. (2001) Primary biliary cirrhosis: MR imaging findings and description of MR imaging periportal halo sign. AJR Am J Roentgenol 176(4):885–889CrossRefPubMedGoogle Scholar
  64. 64.
    Haliloglu N, Erden A, Erden I (2009) Primary biliary cirrhosis: evaluation with T2-weighted MR imaging and MR cholangiopancreatography. Eur J Radiol 69(3):523–527CrossRefPubMedGoogle Scholar
  65. 65.
    Elsayes KM, Shaaban AM, Rothan SM, et al. (2017) A comprehensive approach to hepatic vascular disease. Radiographics 37(3):813–836CrossRefPubMedGoogle Scholar
  66. 66.
    Fan CQ, Crawford JM (2014) Sinusoidal obstruction syndrome (hepatic veno-occlusive disease). J Clin Exp Hepatol 4(4):332–346CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Zhou H, Wang YX, Lou HY, Xu XJ, Zhang MM (2014) Hepatic sinusoidal obstruction syndrome caused by herbal medicine: CT and MRI features. Korean J Radiol 15(2):218–225CrossRefPubMedPubMedCentralGoogle Scholar
  68. 68.
    Han NY, Park BJ, Kim MJ, Sung DJ, Cho SB (2015) Hepatic parenchymal heterogeneity on contrast-enhanced CT scans following oxaliplatin-based chemotherapy: natural history and association with clinical evidence of sinusoidal obstruction syndrome. Radiology 276(3):766–774CrossRefPubMedGoogle Scholar
  69. 69.
    Cayet S, Pasco J, Dujardin F, Besson M, et al. (2017) Diagnostic performance of contrast-enhanced CT-scan in sinusoidal obstruction syndrome induced by chemotherapy of colorectal liver metastases: radio-pathological correlation. Eur J Radiol 94:180–190CrossRefPubMedGoogle Scholar
  70. 70.
    Kan X, Ye J, Rong X, Lu Z, et al. (2016) Diagnostic performance of contrast-enhanced CT in pyrrolizidine alkaloids-induced hepatic sinusoidal obstructive syndrome. Sci Rep 6:37998CrossRefPubMedPubMedCentralGoogle Scholar
  71. 71.
    Li X, Yang X, Xu D, et al. (2017) Magnetic resonance imaging findings in patients with pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome. Clin Gastroenterol Hepatol 15(6):955–957CrossRefPubMedGoogle Scholar
  72. 72.
    Shin NY, Kim MJ, Lim JS, et al. (2012) Accuracy of gadoxetic acid-enhanced magnetic resonance imaging for the diagnosis of sinusoidal obstruction syndrome in patients with chemotherapy-treated colorectal liver metastases. Eur Radiol 22(4):864–871CrossRefPubMedGoogle Scholar
  73. 73.
    Faraoun SA, Boudjella Mel A, Debzi N, et al. (2016) Budd–Chiari syndrome: an update on imaging features. Clin Imaging 40(4):637–646CrossRefPubMedGoogle Scholar
  74. 74.
    Cura M, Haskal Z, Lopera J (2009) Diagnostic and interventional radiology for Budd–Chiari syndrome. Radiographics 29(3):669–681CrossRefPubMedGoogle Scholar
  75. 75.
    Erden A (2007) Budd–Chiari syndrome: a review of imaging findings. Eur J Radiol 61(1):44–56CrossRefPubMedGoogle Scholar
  76. 76.
    Wells ML, Venkatesh SK (2017) Congestive hepatopathy. Abdom Radiol (NY). 2017 Nov 17.  https://doi.org/10.1007/s00261-017-1387-x. [Epub ahead of print] PubMed PMID: 29147765
  77. 77.
    Fouad YM, Yehia R (2014) Hepato-cardiac disorders. World. J Hepatol 6(1):41–54Google Scholar
  78. 78.
    Correale M, Tarantino N, Petrucci R, et al. (2017) Liver disease and heart failure: back and forth. Eur J Intern Med.  https://doi.org/10.1016/j.ejim.2017.10.016 Google Scholar
  79. 79.
    Møller S, Bernardi M (2013) Interactions of the heart and the liver. Eur Heart J 34(36):2804–2811CrossRefPubMedGoogle Scholar

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Authors and Affiliations

  • S. Rajesh
    • 1
  • Amar Mukund
    • 2
  • Binit Sureka
    • 3
  • Kalpana Bansal
    • 3
  • Maxime Ronot
    • 4
  • Ankur Arora
    • 5
  1. 1.The Liver Unit, Cochin Gastroenterology Group, Department of Diagnostic and Interventional RadiologyErnakulam Medical CentreKochiIndia
  2. 2.Department of Interventional RadiologyInstitute of Liver and Biliary SciencesNew DelhiIndia
  3. 3.Department of Diagnostic RadiologyInstitute of Liver and Biliary SciencesNew DelhiIndia
  4. 4.Service de RadiologieAP-HP, Hôpitaux Universitaires Paris Nord Val de SeineClichyFrance
  5. 5.Department of RadiologyWorthing Hospital, Western Sussex NHS Foundation TrustWorthingUK

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