Skip to main content

Advertisement

SpringerLink
Log in

Idiopathic portal hypertension and extrahepatic portal venous obstruction

  • Special Issue - Portal Hypertension
  • Published:
Hepatology International Aims and scope Submit manuscript

Abstract

Background

Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations.

Aim

The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO.

Results

IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO.

Conclusion

This review gives a detailed summary of these two vascular conditions of liver—IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Khanna R, Sarin SK. Non-cirrhotic portal hypertension. J Hepatol 2014;60:421–441

    Article  PubMed  Google Scholar 

  2. Garcia-Pagan JC, Hernandez-Guerra M, Bosch J. Extrahepatic portal vein thrombosis. Semin Liver Dis 2008;28(3):282–292

    Article  PubMed  Google Scholar 

  3. Aoki H, Hasumi A, Yoshida K. A Questionnaire Study on Treatment of Idiopathic Portal Hypertension and Extrahepatic Portal Obstruction. In Kameda H, editor. Annual Report on Portal Portal Hemodynamics Abnormalities (in Japanese). Tokyo: Japan Ministry of Health and Welfare; 1988. p 179–189

    Google Scholar 

  4. Vakili C, Farahvash MJ, Bynum TE. ‘Endemic’ idiopathic portal hypertension. Report on 32 patients with noncirrhotic portal fibrosis. World J Surg 1992;16:118–125

    Article  CAS  PubMed  Google Scholar 

  5. 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

    Article  PubMed  Google Scholar 

  6. Pande C, Kumar A, Sarin SK. Non-cirrhotic portal fibrosis: a clinical profile of 366 patients. Am J Gastroenterol 2006;101:S2:191 (abstract No.439)

  7. Madhu K, Avinash B, Ramakrishna B, Eapen CE, Shyamkumar NK, Zachariah U, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital. Indian J Gastroenterol 2009;28(3):83–87

    Article  PubMed  Google Scholar 

  8. Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, Valla D, Degott C. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002;51(2):275–280

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 2011;54(3):455–461

    Article  PubMed  Google Scholar 

  10. Schouten JNL, Nevens F, Hansen B, Laleman W, van den Born M, Komuta M, et al. Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther 2012;35(12):1424–1433

    Article  CAS  PubMed  Google Scholar 

  11. Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, et al. Idiopathic portal hypertension: natural history and long-term outcome. Hepatology 2014;59:2276–2285

    Article  PubMed  Google Scholar 

  12. Vispo E, Moreno A, Maida I, Barreiro P, Cuevas A, Albertos S, Soriano V. Noncirrhotic portal hypertension in HIV-infected patients: unique clinical and pathological findings. AIDS 2010;24:1171–1176

    Article  PubMed  Google Scholar 

  13. Schouten JN, Van der Ende ME, Koëter T, Rossing HH, Komuta M, Verheij J, et al. Risk factors and outcome of HIV-associated idiopathic noncirrhotic portal hypertension. Aliment Pharmacol Ther 2012;36(9):875–885

    Article  CAS  PubMed  Google Scholar 

  14. Chang PE, Miquel R, Blanco JL, Laguno M, Bruguera M, Abraldes JG, et al. Idiopathic portal hypertension in patients with HIV infection treated with highly active antiretroviral therapy. Am J Gastroenterol 2009;104:1707–1714

    Article  CAS  PubMed  Google Scholar 

  15. Franchi-Abella S, Fabre M, Mselati E, De Marsillac ME, Bayari M, Pariente D, Jacquemin E, Bernard O. Obliterative portal venopathy: a study of 48 children. J Pediatr 2014;165(1):190.e2–193.e2

    Article  Google Scholar 

  16. Sood V, Lal BB, Khanna R, Rawat D, Bihari C, Alam S. Noncirrhotic portal fibrosis in pediatric population. J Pediatr Gastroenterol Nutr 2017;64(5):748–753

    Article  PubMed  Google Scholar 

  17. Sarin SK, Kumar A, Chawla YK, Baijal SS, Dhiman RK, Jafri W, et al. Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int 2007;1:398–413

    Article  PubMed  PubMed Central  Google Scholar 

  18. Krasinskas AM, Eghtesad B, Kamath PS, Demetris AJ, Abraham SC. Liver transplantation for severe intrahepatic noncirrhotic portal hypertension. Liver Transpl 2005;11(6):627–634

    Article  PubMed  Google Scholar 

  19. Goel A, Madhu K, Zachariah U, Sajith KG, Ramachandran J, Ramakrishna B, et al. A study of aetiology of portal hypertension in adults (including the elderly) at a tertiary centre in southern India. Indian J Med Res 2013;137(5):922–927

    PubMed  PubMed Central  Google Scholar 

  20. Omanwar S, Rizvi MR, Kathayat R, Sharma BK, Pandey GK, Alam MA, et al. A rabbit model of non-cirrhotic portal hypertension by repeated injections of E. coli through indwelling cannulation of the gastrosplenic vein. Hepatobiliary Pancreat Dis Int 2004;3:417–422

    PubMed  Google Scholar 

  21. Sato Y, Nakanuma Y. Role of endothelial-mesenchymal transition in idiopathic portal hypertension. Histol Histopathol 2013;28(2):145–154

    PubMed  Google Scholar 

  22. Sato Y, Ren XS, Harada K, Sasaki M, Morikawa H, Shiomi S, et al. Induction of elastin expression in vascular endothelial cells relates to hepatoportal sclerosis in idiopathic portal hypertension: possible link to serum anti-endothelial cell antibodies. Clin Exp Immunol 2012;167(3):532–542

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Guido M, Sarcognato S, Sonzogni A, Lucà MG, Senzolo M, Fagiuoli S, et al. Obliterative portal venopathy without portal hypertension: an underestimated condition. Liver Int 2016;36(3):454–460

    Article  PubMed  Google Scholar 

  24. Okudaria M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis 2002;22:59–71

    Article  Google Scholar 

  25. Klein S, Hinüber C, Hittatiya K, Schierwagen R, Uschner FE, Strassburg CP, et al. Novel rat model of repetitive portal venous embolization mimicking human non-cirrhotic idiopathic portal hypertension. PLoS One 2016;11(9):e0162144

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  26. Mackie I, Eapen CE, Neil D, Lawrie AS, Chitolie A, Shaw JC, Elias E. Idiopathic noncirrhotic intrahepatic portal hypertension is associated with sustained ADAMTS13 deficiency. Dig Dis Sci 2011;56(8):2456–2465

    Article  CAS  PubMed  Google Scholar 

  27. Eapen CE, Nightingale P, Hubscher SG, Lane PJ, Plant T, Velissaris D, Elias E. Non-cirrhotic intrahepatic portal hypertension: associated gut diseases and prognostic factors. Dig Dis Sci 2011;56(1):227–235

    Article  CAS  PubMed  Google Scholar 

  28. Maiwall R, Goel A, Pulimood AB, Babji S, Sophia J, Prasad C, et al. Investigation into celiac disease in Indian patients with portal hypertension. Indian J Gastroenterol 2014;33(6):517–523

    Article  PubMed  Google Scholar 

  29. Pulvirenti F, Pentassuglio I, Milito C, Valente M, De Santis A, Conti V, et al. Idiopathic non cirrhotic portal hypertension and spleno-portal axis abnormalities in patients with severe primary antibody deficiencies. J Immunol Res 2014;2014:672458

    Article  PubMed  PubMed Central  Google Scholar 

  30. Santra A, Das Gupta J, De BK, Roy B, Guha Mazumder DN. Hepatic manifestations in chronic arsenic toxicity. Indian J Gastroenterol 1999;18(4):152–155

    CAS  PubMed  Google Scholar 

  31. Sarin SK, Mehra NK, Agarwal A, Malhotra V, Anand BS, Taneja V. Familial aggregation in noncirrhotic portal fibrosis: a report of four families. Am J Gastroenterol 1987;82:1130–1133

    CAS  PubMed  Google Scholar 

  32. Koot BG, Alders M, Verheij J, Beuers U, Cobben JM. A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhoticportal hypertension. J Hepatol 2016;64(4):974–977

    Article  CAS  PubMed  Google Scholar 

  33. Rangari M, Sinha S, Kapoor D, Mohan JC, Sarin SK. Prevalence of autonomic dysfunction in cirrhotic and non-cirrhotic portal hypertension. Am J Gastroenterol 2002;97(3):707–713

    Article  PubMed  Google Scholar 

  34. Bajaj JS, Bhattacharjee J, Sarin SK. Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non-cirrhotic portal fibrosis. J Gastroenterol Hepatol 2001;16:641–646

    Article  CAS  PubMed  Google Scholar 

  35. Goel A, Ramakrishna B, Muliyil J, Madhu K, Sajith KG, Zachariah U, Ramachandran J, Keshava SN, Selvakumar R, Chandy GM, Elias E, Eapen CE. Use of serum vitamin B12 level as a marker to differentiate idiopathic noncirrhotic intrahepatic portal hypertension from cryptogenic cirrhosis. Dig Dis Sci 2013;58(1):179–187

    CAS  PubMed  Google Scholar 

  36. Seijo S, Reverter E, Miquel R, Berzigotti A, Abraldes JG, Bosch J, et al. Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. Dig Liver Dis 2012;44(10):855–860

    Article  PubMed  Google Scholar 

  37. Sarin SK, Lahoti D, Saxena SP, Murthy NS, Makwana UK. Prevalence, classification and natural history of gastric varices: a long-term follow up study in 568 portal hypertension patients. Hepatology 1992;16:1343–1349

    Article  CAS  PubMed  Google Scholar 

  38. Sarin SK, Shahi HM, Jain M, et al. The natural history of portal hypertensive gastropathy: influence of variceal eradication. Am J Gastroenterol 2000;95:2888–2893

    Article  CAS  PubMed  Google Scholar 

  39. Chawla Y, Dilawari JB. Anorectal varices—their frequency in cirrhotic and non-cirrhotic portal hypertension. Gut 1991;32:309–311

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Ganguly S, Sarin SK, Bhatia V, Lahoti D. The prevalence and spectrum of colonic lesions in patients with cirrhosis and noncirrhotic portal hypertension. Hepatology 1995;21:1226–1231

    CAS  PubMed  Google Scholar 

  41. Maruyama H, Shimada T, Ishibashi H, Takahashi M, Kamesaki H, Yokosuka O. Delayed periportal enhancement: a characteristic finding on contrast ultrasound in idiopathic portal hypertension. Hepatol Int 2012;6(2):511–519

    Article  PubMed  Google Scholar 

  42. Arora A, Sarin SK. Multimodality imaging of obliterative portal venopathy: what every radiologist should know. Br J Radiol 2015;88:20140653

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Glatard AS, Hillaire S, d’Assignies G, Cazals-Hatem D, Plessier A, Valla DC, Vilgrain V. Obliterative portal venopathy: findings at CT imaging. Radiology 2012;263(3):741–750

    Article  PubMed  Google Scholar 

  44. Qureshi H, Kamal S, Khan RA, et al. Differentiation of cirrhotic vs idiopathic portal hypertension using 99mTc-Sn colloid dynamic and static scintigraphy. J Pak Med Assoc 1991;41:126–129

    CAS  PubMed  Google Scholar 

  45. Furuichi Y, Moriyasu F, Taira J, Sugimoto K, Sano T, Ichimura S, et al. Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography. J Gastroenterol 2013;48(9):1061–1068

    Article  PubMed  Google Scholar 

  46. Seijo S, Lozano JJ, Alonso C, Reverter E, Miquel R, Abraldes JG, et al. Metabolomics discloses potential biomarkers for the noninvasive diagnosis of idiopathic portal hypertension. Am J Gastroenterol 2013;108(6):926–932

    Article  CAS  PubMed  Google Scholar 

  47. Seijo S, Lozano JJ, Alonso C, Miquel R, Berzigotti A, Reverter E, et al. Metabolomics as a diagnostic tool for idiopathic non-cirrhotic portal hypertension. Liver Int 2016;36(7):1051–1058

    Article  CAS  PubMed  Google Scholar 

  48. Kotani K, Kawabe J, Morikawa H, Akahoshi T, Hashizume M, Shiomi S. Comprehensive screening of gene function and networks by DNA microarray analysis in Japanese patients with idiopathic portal hypertension. Med Inflamm. 2015;2015:349215

    Article  CAS  Google Scholar 

  49. De Franchis R, Baveno VI Faculty. Expanding consensus in portal hypertension report of the Baveno VI consensus workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol 2015;63:743–752

    Article  PubMed  Google Scholar 

  50. Pal S, Radhakrishna P, Sahni P, Pande GK, Nundy S, Chattopadhyay TK. Prophylactic surgery in non-cirrhotic portal fibrosis: is it worthwhile? Indian J Gastroenterol 2005;24(6):239–242

  51. Sawada S, Sato Y, Aoyama H, Harada K, Nakanuma Y. Pathological study of idiopathic portal hypertension with an emphasis on cause of death based on records of Annuals of Pathological Autopsy Cases in Japan. J Gastroenterol Hepatol 2007;22(2):204–209

    Article  PubMed  Google Scholar 

  52. Murai Y, Ohfuji S, Fukushima W, Tamakoshi A, Yamaguchi S, Hashizume M, et al. Prognostic factors in patients with idiopathic portal hypertension: two Japanese nationwide epidemiological surveys in 1999 and 2005. Hepatol Res 2012;42(12):1211–1220

    Article  PubMed  Google Scholar 

  53. Poddar U, Thapa BR, Rao KL, Singh K. Etiological spectrum of esphageal varices due to portal hypertension in Indian children: is it different from the West? J Gastroenterol Hepatol 2008;23:1354–1357

    Article  PubMed  Google Scholar 

  54. Fagundes ED, Ferreira AR, Roquete ML, Penna FJ, Goulart EM, Figueiredo Filho PP, Bittencourt PF, Carvalho SD, Albuquerque W. Clinical and laboratory predictors of esophageal varices in children and adolescents with portal hypertension syndrome. J Pediatr Gastroenterol Nutr 2008;46(2):178–183

    Article  PubMed  Google Scholar 

  55. Duché M, Ducot B, Ackermann O, Guérin F, Jacquemin E, Bernard O. Portalhypertension in children: high-risk varices, primary prophylaxis and consequencesof bleeding. J Hepatol 2017;66(2):320–327

    Article  PubMed  Google Scholar 

  56. Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, et al. Members of the APASL working party on portal hypertension. Consensus on extra-hepatic portal vein obstruction. Liver Int 2006;26(5):512–519

    Article  PubMed  Google Scholar 

  57. Denninger MH, Chaït Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000;31(3):587–591

    Article  CAS  PubMed  Google Scholar 

  58. Janssen HL, Wijnhoud A, Haagsma EB, van Uum SH, van Nieuwkerk CM, Adang RP, Chamuleau RA, van Hattum J, Vleggaar FP, Hansen BE, Rosendaal FR, van Hoek B. Extrahepatic portal vein thrombosis: aetiology and determinants of survival. Gut 2001;49(5):720–724

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Bhattacharyya M, Makharia G, Kannan M, Ahmed RP, Gupta PK, Saxena R. Inherited prothrombotic defects in Budd-Chiari syndrome and portal vein thrombosis: a study from north India. Am J Clin Pathol 2004;121(6):844–847

    Article  PubMed  Google Scholar 

  60. Primignani M, Barosi G, Bergamaschi G, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology 2006;44(6):1528–1534

    Article  CAS  PubMed  Google Scholar 

  61. Amitrano L, Guardascione MA, Scaglione M, et al. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol 2007;102(11):2464–2470

    Article  PubMed  Google Scholar 

  62. Sogaard KK, Astrup LB, Vilstrup H, Gronbaek H. Portal vein thrombosis; risk factors, clinical presentation and treatment. BMC Gastroenterol 2007;7:34

    Article  PubMed  PubMed Central  Google Scholar 

  63. El-Karaksy H, El-Koofy N, El-Hawary M, et al. Prevalence of factor V Leiden mutation and other hereditary thrombophilic factors in Egyptian children with portal vein thrombosis: results of a single-center case–control study. Ann Hematol 2004;83:712–715

    Article  CAS  PubMed  Google Scholar 

  64. El-Karaksy HM, El-Koofy N, Mohsen N, Helmy H, Nabil N, El-Shabrawi M. Extrahepatic portal vein obstruction in Egyptian children. J Pediatr Gastroenterol Nutr 2015;60(1):105–109

    Article  PubMed  Google Scholar 

  65. Abd El-Hamid N, Taylor RM, Marinello D, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King’s College Hospital experience. J Pediatr Gastroenterol Nutr 2008;47:630–634

    Article  PubMed  Google Scholar 

  66. Pietrobattista A, Luciani M, Abraldes JG, Candusso M, Pancotti S, Soldati M, Monti L, Torre G, Nobili V. Extrahepatic portal vein thrombosis in children and adolescents: Influence of genetic thrombophilic disorders. World J Gastroenterol 2010;16(48):6123–6127

    Article  PubMed  PubMed Central  Google Scholar 

  67. Weiss B, Shteyer E, Vivante A, et al. Etiology and long-term outcome of extrahepatic portal vein obstruction in children. World J Gastroenterol 2010;16:4968–4972

    Article  PubMed  PubMed Central  Google Scholar 

  68. Ferri PM, Rodrigues Ferreira A, Fagundes ED, Xavier SG, Dias Ribeiro D, et al. Evaluation of the presence of hereditary and acquired thrombophilias in brazilian children and adolescents with diagnoses of portal vein thrombosis. J Pediatr Gastroenterol Nutr 2012;55(5):599–604

    Article  PubMed  Google Scholar 

  69. Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood 2012;120(25):4921–4928

    Article  CAS  PubMed  Google Scholar 

  70. Nowak-Göttl U, Junker R, Kreuz W, von Eckardstein A, Kosch A, Nohe N, Schobess R, Ehrenforth S, Childhood Thrombophilia Study Group. Risk of recurrent venous thrombosis in children with combined prothrombotic risk factors. Blood 2001;97(4):858–862

    Article  PubMed  Google Scholar 

  71. Odièvre M, Pigé G, Alagille D. Congenital abnormalities associated with extrahepatic portal hypertension. Arch Dis Child 1977;52(5):383–385

    Article  PubMed  PubMed Central  Google Scholar 

  72. Yadav S, Dutta AK, Sarin SK. Do umbilical vein catheterization and sepsis lead to portal vein thrombosis? A prospective, clinical and sonographic evaluation. J Pediatr Gastroenterol Nutr 1993;17:392–396

    Article  CAS  PubMed  Google Scholar 

  73. Stringer MD, Heaton ND, Karani J, et al. Patterns of portal vein occlusion and their aetiological significance. Br J Surg 1994;81:1328–1331

    Article  CAS  PubMed  Google Scholar 

  74. Sarin SK, Philips CA, Kamath PS, Choudhury A, Maruyama H, Nery FG, Valla DC. Toward a comprehensive new classification of portal vein thrombosis in patients with cirrhosis. Gastroenterology 2016;151(4):574–577.e3

  75. Shneider BL, de Ville de Goyet J, Leung DH, Srivastava A, Ling SC, Duché M, et al. Primary prophylaxis of variceal bleeding in children and the role of Meso-Rex bypass: summary of the Baveno VI pediatric satellite symposium. Hepatology 2016;63(4):1368–1380

    Article  PubMed  Google Scholar 

  76. Alberti D, Colusso M, Cheli M, Ravelli P, Indriolo A, Signorelli S, Fagiuoli S, D’Antiga L. Results of a stepwise approach to extrahepatic portal vein obstruction in children. J Pediatr Gastroenterol Nutr 2013;57(5):619–626

    Article  PubMed  Google Scholar 

  77. Zargar SA, Yattoo GN, Javid G, Khan BA, Shah AH, Shah NA, et al. Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction. J Gastroenterol Hepatol 2004;19:139–145

    Article  PubMed  Google Scholar 

  78. Thomas V, Jose T, Kumar S. Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction: a 20-year follow-up. Indian J Gastroenterol 2009;28:206–211

    Article  PubMed  Google Scholar 

  79. Noronha Ferreira C, Seijo S, Plessier A, Silva-Junior G, Turon F, Rautou PE, et al. Natural history and management of esophagogastric varices in chronic noncirrhotic, nontumoral portal vein thrombosis. Hepatology 2016;63(5):1640–1650

    Article  PubMed  Google Scholar 

  80. Rangari M, Gupta R, Jain M, Malhotra V, Sarin SK. Hepatic dysfunction in patients with extrahepatic portal venous obstruction. Liver Int 2003;23(6):434–439

    Article  CAS  PubMed  Google Scholar 

  81. Jha SK, Kumar A, Sharma BC, Sarin SK. Systemic and pulmonary hemodynamics in patients with extrahepatic portal vein obstruction is similar to compensated cirrhotic patients. Hepatol Int 2009;3:384–391

    Article  PubMed  Google Scholar 

  82. Itha S, Yachha SK. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr 2006;42(2):196–200

    Article  PubMed  Google Scholar 

  83. Yachha SK, Dhiman RK, Gupta R, Ghoshal UC. Endosonographic evaluation of the rectum in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr 1996;23(4):438–441

    Article  CAS  PubMed  Google Scholar 

  84. Sharma P, Mishra SR, Kumar M, Sharma BC, Sarin SK. Liver and spleen stiffness in patients with extrahepatic portal vein obstruction. Radiology 2012;263(3):893–899

    Article  PubMed  Google Scholar 

  85. Sarin SK, Bansal A, Sasan S, Nigam A. Portal-vein obstruction in children leads to growth retardation. Hepatology 1992;15:229–233

    Article  CAS  PubMed  Google Scholar 

  86. Mehrotra RN, Bhatia V, Dabadghao P, Yachha SK. Extrahepatic portal vein obstruction in children: anthropometry, growth hormone and insulin-like growth factor I. J Pediatri Gastroenterol Nutr 1997;25:520–523

    Article  CAS  Google Scholar 

  87. Krishna YR, Yachha SK, Srivastava A, Negi D, Lal R, Poddar U. Quality of life in children managed for extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr 2010;50(5):531–536

    PubMed  Google Scholar 

  88. Chandra R, Kapoor D, Tharakan A, Chaudhary A, Sarin SK. Portal biliopathy. J Gastroenterol Hepatol 2001;16:1086–1092

    Article  CAS  PubMed  Google Scholar 

  89. Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, et al. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology 1993;17:807–813

    Article  CAS  PubMed  Google Scholar 

  90. Shin SM, Kim S, Lee JW, Kim CW, Lee TH, Lee SH, Kim GH. Biliary abnormalities associated with portal biliopathy: evaluation on MR cholangiography. AJR Am J Roentgenol 2007;188(4):W341–W3417

    Article  PubMed  Google Scholar 

  91. Chattopadhyay S, Govindasamy M, Singla P, Varma V, Mehta N, Kumaran V, Nundy S. Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome? HPB (Oxford) 2012;14(7):441–447

    Article  Google Scholar 

  92. Jabeen S, Robbani I, Choh NA, Ashraf O, Shaheen F, Gojwari T, et al. Spectrum of biliary abnormalities in portal cavernoma cholangiopathy (PCC) secondary toidiopathic extrahepatic portal vein obstruction (EHPVO)—a prospective magnetic resonance cholangiopancreaticography (MRCP) based study. Br J Radiol 2016;89(1068):20160636

    Article  PubMed  PubMed Central  Google Scholar 

  93. Rai GP, Nijhawan S, Madhu MP, Sharma SS, Pokharna R. Comparative evaluation of magnetic resonance cholangiopancreatography/magnetic resonance splenoportovenography and endoscopic ultrasound in the diagnosis of portal cavernoma cholangiopathy. Indian J Gastroenterol 2015;34(6):442–447

    Article  PubMed  Google Scholar 

  94. Sharma P, Sharma BC, Puri V, Sarin SK. Minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol 2008;103(6):1406–1412

    Article  PubMed  Google Scholar 

  95. Yadav SK, Srivastava A, Srivastava A, Thomas MA, Agarwal J, Pandey CM, et al. Encephalopathy assessment in children with extra-hepatic portal vein obstruction with MR, psychometry and critical flicker frequency. J Hepatol 2010;52:348–354

    Article  PubMed  Google Scholar 

  96. Srivastava A, Yadav SK, Yachha SK, Thomas MA, Saraswat VA, Gupta RK. Pro-inflammatory cytokines are raised in extrahepatic portal venous obstruction with minimal hepatic encephalopathy. J Gastroenterol Hepatol 2011;26(6):979–986

    Article  CAS  PubMed  Google Scholar 

  97. Sharma P, Sharma BC, Puri V, Sarin SK. Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol 2009;104(4):885–890

    Article  PubMed  Google Scholar 

  98. Sarin SK, Govil A, Jain AK, Guptan RC, Issar SK, Jain M, et al. Prospective randomized trial of endoscopic sclerotherapy versus variceal band ligation for esophageal varices: influence on gastropathy, gastric varices and variceal recurrence. J Hepatol 1997;26(4):826–832

    Article  CAS  PubMed  Google Scholar 

  99. Zargar SA, Javid G, Khan BA, Yattoo GN, Shah AH, Gulzar GM, et al. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology 2002;36:666–672

    Article  PubMed  Google Scholar 

  100. Poddar U, Bhatnagar S, Yachha SK. Endoscopic band ligation followed by sclerotherapy: is it superior to sclerotherapy in children with extrahepatic portal venous obstruction? J Gastroenterol Hepatol 2011;26:255–259

    Article  PubMed  Google Scholar 

  101. Sarin SK, Gupta N, Jha SK, Agrawal A, Mishra SR, Sharma BC, Kumar A. Equal efficacy of endoscopic variceal ligation and propranolol in preventing variceal bleeding in patients with noncirrhotic portal hypertension. Gastroenterology 2010;139(4):1238–1245

    Article  CAS  PubMed  Google Scholar 

  102. Superina R, Bambini DA, Lokar J, Rigsby C, Whittington PF. Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass. Ann Surg 2006;243:515–521

    Article  PubMed  PubMed Central  Google Scholar 

  103. Lautz TB, Keys LA, Melvin JC, Ito J, Superina RA. Advantages of the meso-Rex bypass compared with portosystemic shunts in the management of extrahepatic portal vein obstruction in children. J Am Coll Surg 2013;216(1):83–89

    Article  PubMed  Google Scholar 

  104. Mack CL, Zelko FA, Lokar J, Superina R, Alonso EM, Blei AT, Whitington PF. Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability. Pediatrics 2006;117(3):e405–e4012

    Article  PubMed  Google Scholar 

  105. Prasad AS, Gupta S, Kohli V, Pande GK, Sahni P, Nundy S. Proximal splenorenal shunts for extrahepatic portal venous obstruction in children. Ann Surg 1994;219:193–196

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  106. Sharma BC, Singh RP, Chawla YK, Narasimhan KL, Rao KL, Mitra SK, Dilawari JB. Effect of shunt surgery on spleen size, portal pressure and oesophageal varices in patients with non-cirrhotic portal hypertension. J Gastroenterol Hepatol 1997;12(8):582–584

    Article  CAS  PubMed  Google Scholar 

  107. Sharma N, Bajpai M, Kumar A, Paul S, Jana M. Portal hypertension: a critical appraisal of shunt procedures with emphasis on distal splenorenal shunt in children. J Indian Assoc Pediatr Surg 2014;19(2):80–84

    Article  PubMed  PubMed Central  Google Scholar 

  108. Mishra PK, Patil NS, Saluja S, Narang P, Solanki N, Varshney V. High patency of proximal splenorenal shunt: A myth or reality?—a prospective cohort study. Int J Surg 2016;27:82–87

    Article  PubMed  Google Scholar 

  109. Wani AH, Shah OJ, Zargar SA. Management of variceal hemorrhage in children with extrahepatic portal venous obstruction—Shunt surgery versus endoscopic sclerotherapy. Indian J Surg 2011;73(6):409–413

  110. Saigal S, Nayak NC, Jain D, Kumaran V, Mohanka R, Saraf N, et al. Non-cirrhotic portal fibrosis related end stage liver disease in adults: evaluation from a study on living donor liver transplant recipients. Hepatol Int 2011;5(4):882–889

    Article  PubMed  Google Scholar 

  111. Bissonnette J, Garcia-Pagán JC, Albillos A, Turon F, Ferreira C, Tellez L, et al. Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension. Hepatology 2016;64(1):224–231

    Article  PubMed  Google Scholar 

  112. Pargewar SS, Desai SN, Rajesh S, Singh VP, Arora A, Mukund A. Imaging and radiological interventions in extra-hepatic portal vein obstruction. World J Radiol 2016;8(6):556–570

    Article  PubMed  PubMed Central  Google Scholar 

  113. Ozturk O, Eldem G, Peynircioglu B, Kav T, Görmez A, Cil BE, et al. Outcomes of partial splenic embolization in patients with massive splenomegaly due to idiopathic portal hypertension. World J Gastroenterol 2016;22(43):9623–9630

    Article  PubMed  PubMed Central  Google Scholar 

  114. De Gottardi A, Trebicka J, Klinger C, Plessier A, Seijo S, Terziroli B, VALDIG Investigators, et al. Antithrombotic treatment with direct-acting oral anticoagulants in patients with splanchnic vein thrombosis and cirrhosis. Liver Int 2017;37(5):694–699

    Article  PubMed  CAS  Google Scholar 

  115. Chaudhary A, Dhar P, Sarin SK, Sachdev A, Agarwal AK, Vij JC, et al. Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management. Br J Surg 1998;85:326–329

    Article  CAS  PubMed  Google Scholar 

  116. Agarwal AK, Sharma S, Singh S, Agarwal S, Girish SP. Portal biliopathy: a study of 39 surgically treated patients. HPB 2011;13:33–39

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India

    Rajeev Khanna

  2. Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India

    Shiv Kumar Sarin

Authors
  1. Rajeev Khanna
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shiv Kumar Sarin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shiv Kumar Sarin.

Ethics declarations

Conflict of interest

Rajeev Khanna and Shiv Kumar Sarin declare that they have no conflicts of interest.

Ethical approval

The study involves a review of literature on idiopathic portal hypertension and extrahepatic venous obstruction and is in accordance with the ethical standards of the Institutional Ethical Committee. The article does mention about animal studies performed earlier by one of the authors which again were performed with ethical approval.

Informed Consent

The study doesn’t involve patients but provides a review of existing literature on the concerned subject.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Khanna, R., Sarin, S.K. Idiopathic portal hypertension and extrahepatic portal venous obstruction. Hepatol Int 12 (Suppl 1), 148–167 (2018). https://doi.org/10.1007/s12072-018-9844-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12072-018-9844-3

Keywords

Search

Navigation