Portal Hypertension

Living reference work entry


Portal hypertension (PHT) in children may be caused by a wide range of etiologies, including extrahepatic portal vein obstruction, biliary atresia, alpha-1-antitrypsin deficiency, and autoimmune hepatitis. PHT presents with acute gastrointestinal bleeding and/or splenomegaly or with symptoms and signs of chronic liver disease. Gastroesophageal varices and ascites are two main complications of PHT responsible for morbidity and mortality. This chapter describes the pathogenesis and management of PHT in children.



This chapter has been adapted by permission from Springer Nature: Springer-Verlag, Berlin. Pediatric Surgery. Diagnosis and Management. Portal hypertension, Stringer MD, 2009.


  1. Allaire M, Walter A, Sutter O et al. TIPS for management of portal-hypertension-related complications in patients with cirrhosis. Clin Res Hepatol Gastroenterol. 2019. pii: S2210-7401(19)30217-7.Google Scholar
  2. Bernabeu JQ, Juampérez J, Mercadal-Hally M, et al. Endoscopic variceal ligation as primary prophylaxis for upper GI bleeding in children. Gastrointest Endosc. 2020;pii: S0016-5107(20)30211-XGoogle Scholar
  3. Bertocchini A, Falappa P, Grimaldi C, Bolla G, Monti L, de Ville de Goyet J. Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance. J Pediatr Surg. 2014;49:1268–75.PubMedCrossRefPubMedCentralGoogle Scholar
  4. Botha JF, Campos BD, Grant WJ, Horslen SP, Sudan DL, Shaw BW Jr, Langnas AN. Portosystemic shunts in children: a 15-year experience. J Am Coll Surg. 2004;199:179–85.PubMedCrossRefPubMedCentralGoogle Scholar
  5. Chen VT, Wei J, Liu YC. A new procedure for management of extrahepatic portal obstruction. Proximal splenic-left intrahepatic portal shunt. Arch Surg. 1992;127:1358–60.PubMedCrossRefPubMedCentralGoogle Scholar
  6. Chiou FK, Ong C, Low Y, et al. Non-invasive predictors for the first variceal hemorrhage in children with biliary atresia after Kasai Portoenterostomy. J Clin Exp Hepatol. 2019;9(5):581–7.PubMedCrossRefPubMedCentralGoogle Scholar
  7. Cwikiel W, Keussen I, Larsson L, Solvig J, Kullendorff CM. Interventional treatment of children with portal hypertension secondary to portal vein occlusion. Eur J Pediatr Surg. 2003;13:312–8.PubMedCrossRefPubMedCentralGoogle Scholar
  8. de Ville de Goyet J, Clapuyt P, Otte JB. Extrahilar mesenterico-left portal shunt to relieve extrahepatic portal hypertension after partial liver transplant. Transplantation. 1992;53:231–2.Google Scholar
  9. de Ville de Goyet J, Alberti D, Clapuyt P, et al. Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension. J Pediatr Surg. 1998;33:597–601.CrossRefGoogle Scholar
  10. di Francesco F, Grimaldi C, de Ville de Goyet J. Meso-Rex bypass – a procedure to cure prehepatic portal hypertension: the insight and the inside. J Am Coll Surg. 2014;218:e23–36.PubMedCrossRefPubMedCentralGoogle Scholar
  11. Dignan FL, Wynn RF, Hadzic N, et al. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. Br J Haematol. 2013;163:444–57.PubMedCrossRefPubMedCentralGoogle Scholar
  12. Gattini D, Cifuentes LI, Torres-Robles R, Gana JC. Sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev. 2020;3:CD011573Google Scholar
  13. Gauthier F, De Dreuzy O, Valayer J, Montupet P. H-type shunt with an autologous venous graft for treatment of portal hypertension in children. J Pediatr Surg. 1989;24:1041–3.PubMedCrossRefPubMedCentralGoogle Scholar
  14. Gharehbaghi MM, Nemati M, Hosseinpour SS, Taei R, Ghargharechi R. Umbilical vascular catheter associated portal vein thrombosis detected by ultrasound. Indian J Pediatr. 2011;78:161–4.PubMedCrossRefPubMedCentralGoogle Scholar
  15. Gibelli NE, Tannuri AC, Pinho-Apezzato ML, Maksoud-Filho JG, Tannuri U. Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt? J Pediatr Surg. 2011;46:214–6.PubMedCrossRefPubMedCentralGoogle Scholar
  16. Guérin F, Porras J, Fabre M, Guettier C, Pariente D, Bernard O, Gauthier F. Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg. 2009;44:1337–43.PubMedCrossRefPubMedCentralGoogle Scholar
  17. Guérin F, Bidault V, Gonzales E, Franchi-Abella S, De Lambert G, Branchereau S. Meso-Rex bypass for extrahepatic portal vein obstruction in children. Br J Surg. 2013;100:1606–13.PubMedCrossRefPubMedCentralGoogle Scholar
  18. Ha TY, Kim KM, Ko GY, Oh SH, Kwon TW, Cho YP, Lee SG. Variant meso-Rex bypass with transposition of abdominal autogenous vein for the management of idiopathic extrahepatic portal vein obstruction: a retrospective observational study. BMC Surg. 2015;15:116.PubMedPubMedCentralCrossRefGoogle Scholar
  19. Koconis KG, Singh H, Soares G. Partial splenic embolization in the treatment of patients with portal hypertension: a review of the English language literature. J Vasc Interv Radiol. 2007;18:463–81.PubMedCrossRefPubMedCentralGoogle Scholar
  20. Lautz TB, Sundaram SS, Whitington PF, Keys L, Superina RA. Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass. J Pediatr Surg. 2009;44:2067–70.PubMedCrossRefPubMedCentralGoogle Scholar
  21. Long L, Jinshan Z, Zhen C, et al. Portal-to-right portal vein bypass for extrahepatic portal vein obstruction. J Pediatr Surg. 2018;53:1403–1407Google Scholar
  22. Mack CL, Superina RA, Whitington PF. Surgical restoration of portal flow corrects procoagulant and anticoagulant deficiencies associated with extrahepatic portal vein thrombosis. J Pediatr. 2003;142:197–9.PubMedCrossRefGoogle Scholar
  23. Maksoud-Filho JG, Gonçalves ME, Cardoso SR, Gibelli NE, Tannuri U. Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality. J Pediatr Surg. 2009;44:1877–83.PubMedCrossRefPubMedCentralGoogle Scholar
  24. Nio M, Hayashi Y, Sano N, Ishii T, Sasaki H, Ohi R. Long-term efficacy of partial splenic embolization in children. J Pediatr Surg. 2003;38:1760–2.PubMedCrossRefPubMedCentralGoogle Scholar
  25. Nobre S, Khanna R, Bab N, et al. Primary Budd-Chiari syndrome in children: King’s College Hospital experience. J Pediatr Gastroenterol Nutr. 2017;65:93–6.PubMedCrossRefPubMedCentralGoogle Scholar
  26. Paternostro R, Reiberger T, Bucsics T. Elastography-based screening for esophageal varices in patients with advanced chronic liver disease. World J Gastroenterol. 2019;25(3):308–29.PubMedPubMedCentralCrossRefGoogle Scholar
  27. Pimenta JR, Ferreira AR, Fagundes ED, et al. Evaluation of endoscopic secondary prophylaxis in children and adolescents with esophageal varices. Arq Gastroenterol. 2017;54(1):21–6.PubMedCrossRefPubMedCentralGoogle Scholar
  28. Pinto RB, Silveira TR, Bandinelli E, Röhsig L. Portal vein thrombosis in children and adolescents: the low prevalence of hereditary thrombophilic disorders. J Pediatr Surg. 2004;39:1356–61.PubMedCrossRefPubMedCentralGoogle Scholar
  29. Ramachandran P, Shanmugam NP, Vij M, Rela M. Surgical management of hepatic arterioportal fistula in a neonate. Pediatr Surg Int. 2014;30:557–9.PubMedCrossRefPubMedCentralGoogle Scholar
  30. Sari S, Oguz D, Sucak T, Dalgic B, Atasever T. Hepatopulmonary syndrome in children with cirrhotic and non-cirrhotic portal hypertension: a single-center experience. Dig Dis Sci. 2012;57:175–81.PubMedCrossRefPubMedCentralGoogle Scholar
  31. Sharif K, McKiernan P, de Ville de Goyet J. Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most! J Pediatr Surg. 2010;45:272–6.PubMedCrossRefPubMedCentralGoogle Scholar
  32. Sharma VK, Ranade PR, Marar S, Nabi F, Nagral A. Long-term clinical outcome of Budd-Chiari syndrome in children after radiological intervention. Eur J Gastroenterol Hepatol. 2016;28:567–75.PubMedCrossRefPubMedCentralGoogle Scholar
  33. Shilyansky J, Roberts EA, Superina RA. Distal splenorenal shunts for the treatment of severe thrombocytopenia from portal hypertension in children. J Gastrointest Surg. 1999;3:167–72.PubMedCrossRefPubMedCentralGoogle Scholar
  34. Shneider BL. Tacit consensus. J Pediatr Gastroenterol Nutr. 2017;64:497.PubMedCrossRefPubMedCentralGoogle Scholar
  35. Shneider BL, de Ville de Goyet J, Leung DH, et al. Primary prophylaxis of variceal bleeding in children and the role of MesoRex bypass: summary of the Baveno VI Pediatric Satellite symposium. Hepatology. 2016;63:1368–80.PubMedCrossRefPubMedCentralGoogle Scholar
  36. Srivastava A, Yadav SK, Lal R, et al. Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry. J Pediatr Gastroenterol Nutr. 2010;51:766–72.PubMedCrossRefPubMedCentralGoogle Scholar
  37. Stringer MD. Esophageal varices. In: Najmaldin AS, Rothenberg S, Crabbe DCG, Beasley S, editors. Operative endoscopy and endoscopic surgery in infants and children. London: Hodder Arnold; 2005. p. 141–8.CrossRefGoogle Scholar
  38. Stringer MD. Portal hypertension – endoscopic treatment. In: Stringer MD, Oldham KT, Mouriquand PDE, editors. Pediatric surgery and urology: long term outcomes. 2nd ed. Cambridge University Press: New York; 2006. p. 502–9.CrossRefGoogle Scholar
  39. Stringer MD. Improved body mass index after mesenterico-portal bypass. Pediatr Surg Int. 2007;23:539–43.PubMedCrossRefPubMedCentralGoogle Scholar
  40. Stringer MD, Howard ER. Surgical disorders of the liver and Bile ducts and portal hypertension. In: Kelly DA, editor. Diseases of the liver and biliary system in children. Oxford: Blackwell; 2004. p. 345.Google Scholar
  41. Stringer MD, Heaton ND, Karani J, Olliff S, Howard ER. Patterns of portal vein occlusion and their aetiological significance. Br J Surg. 1994;81:1328–31.PubMedCrossRefPubMedCentralGoogle Scholar
  42. Sutcliffe R, Mieli-Vergani G, Dhawan A, Corbally M, Karani J, Heaton N. A novel treatment of congenital hepatoportal arteriovenous fistula. J Pediatr Surg. 2008;43:571–3.PubMedCrossRefPubMedCentralGoogle Scholar
  43. Tingo J, Rosenzweig EB, Lobritto S, Krishnan US. Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease. Pulm Circ. 2017;7:712–8.PubMedPubMedCentralCrossRefGoogle Scholar
  44. Uchiyama M, Iwafuchi M, Ohsawa Y, Naito S, Naito M, Yagi M, Tsukada K. Long-term results after nonshunt operations for esophageal varices in children. J Pediatr Surg. 1994;29:1429–33.PubMedCrossRefPubMedCentralGoogle Scholar
  45. Valayer J, Branchereau S. Portal hypertension: portosystemic shunts. In: Stringer MD, Oldham KT, Mouriquand PDE, et al., editors. Pediatric surgery and urology: long-term outcomes. Philadelphia: Saunders; 1998. p. 439–46.Google Scholar
  46. Vittorio J, Orellana K, Martinez M, et al. Partial splenic embolization is a safe and effective alternative in the management of portal hypertension in children. J Pediatr Gastroenterol Nutr. 2019;68(6):793–8.PubMedCrossRefPubMedCentralGoogle Scholar
  47. Yoon H, Shin HJ, Kim MJ, et al. Predicting gastroesophageal varices through spleen magnetic resonance elastography in pediatric liver fibrosis. World J Gastroenterol. 2019;25(3):367–77.PubMedPubMedCentralCrossRefGoogle Scholar
  48. Zargar SA, Javid G, Khan BA, Yattoo GN, et al. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology. 2002;36:666–72.PubMedCrossRefPubMedCentralGoogle Scholar

Further Reading

  1. de Ville de Goyet J, D’Ambrosio G, Grimaldi C. Surgical management of portal hypertension in children. Semin Pediatr Surg. 2012;21:219–32.CrossRefGoogle Scholar
  2. Stringer MD, Oldham KT, PDE M. Pediatric surgery and urology: long term outcomes, Chapters on portal hypertension. 2nd ed. New York: Cambridge University Press; 2006. p. 491–509.CrossRefGoogle Scholar

Authors and Affiliations

  1. 1.Department of Paediatric Surgery, Wellington Children’s Hospital and Department of Paeddiatrics and Child HealthWellington School of Medicine, University of OtagoWellingtonNew Zealand

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