Surgery for Portal Hypertension in Children



Management of children with portal hypertension has evolved considerably over the past decades. Development of physiologic shunts (meso-Rex bypass) and successful liver transplant has changed the paradigm of portal hypertension surgery. Children with pre-hepatic portal hypertension are investigated and, if suitable, candidates are offered the mesenteric-to-left portal vein bypass (meso-Rex) preemptively, before development of symptoms of portal hypertension. Aggressive medical management, endoscopic ligation of bleeding varices, and radiologically placed intrahepatic stents have greatly reduced the need for emergent surgical procedures. A larger number of surgical options offer a permanent solution for children with portal hypertension in the setting of well-compensated liver function. Portal hypertension in the setting of decompensated liver disease is managed medically (via endoscopy) or radiologically (via transjugular intrahepatic portosystemic shunt) with the aim to offer liver transplant as a permanent solution.


Portal hypertension Children Shunts Meso-caval shunt Meso-Rex bypass Selective shunts Nonselective shunts Splenectomy Sugiura procedure 



Conflicts of interest: S. Scholz—none; K. Sharif—none.


Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 1.
    Whitington P. Portal hypertension in children. Pediatr Ann. 1985;14:494–9.PubMedGoogle Scholar
  2. 2.
    Bismuth H, Franco D. Portal diversion for portal hypertension in early childhood. Ann Surg. 1976;183:439–46.PubMedCrossRefGoogle Scholar
  3. 3.
    Superina RA, Alonso EM. Medical and surgical management of portal hypertension in children. Curr Treat Options Gastroenterol. 2006;9:432–43.PubMedCrossRefGoogle Scholar
  4. 4.
    Grace ND, Conn HO, Resnick RH, et al. Distal splenorenal vs portal-systemic shunts after hemorrhage from varices: a randomized controlled trial. Hepatology. 1988;8:1475–81.PubMedCrossRefGoogle Scholar
  5. 5.
    Botha JF, Campos BD, Grant WJ, et al. Portosystemic shunts in children: a 15-year experience. J Am Coll Surg. 2004;199:179–85.PubMedCrossRefGoogle Scholar
  6. 6.
    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.PubMedCrossRefGoogle Scholar
  7. 7.
    Warren WD, Zeppa R, Fomon JJ. Selective trans-splenic decompression of gastroesophageal varices by distal splenorenal shunt. Ann Surg. 1967;166:437–55.PubMedCrossRefGoogle Scholar
  8. 8.
    Maksoud JG, Gonçalves ME. Treatment of portal hypertension in children. World J Surg. 1994;18:251–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Brems JJ, Hiatt JR, Klein AS, et al. Effect of a prior portasystemic shunt on subsequent liver transplantation. Ann Surg. 1989;209:51–6.PubMedCrossRefGoogle Scholar
  10. 10.
    Hasegawa T, Tamada H, Fukui Y, et al. Distal splenorenal shunt with splenopancreatic disconnection for portal hypertension in biliary atresia. Pediatr Surg Int. 1999;15:92–6.PubMedCrossRefGoogle Scholar
  11. 11.
    Spina GP, Santambrogio R, Opocher E, et al. Early hemodynamic changes following selective distal splenorenal shunt for portal hypertension: comparison of surgical techniques. World J Surg. 1990;14:115–21.PubMedCrossRefGoogle Scholar
  12. 12.
    Eizaguirre I, Tovar JA, Orcolaga R, et al. Warren's shunt in the treatment of portal hypertension in children. Cir Pediatr. 1991;4:134–9.PubMedGoogle Scholar
  13. 13.
    Mitra SK, Mukherjee B, Prasad GR, et al. Extrahepatic portal obstruction in children–experience with side-to-side lienorenal shunt without splenectomy. Indian J Pediatr. 1983;50:55–60.PubMedCrossRefGoogle Scholar
  14. 14.
    Mitra SK, Rao KL, Narasimhan KL, et al. Side-to-side lienorenal shunt without splenectomy in noncirrhotic portal hypertension in children. J Pediatr Surg. 1993;28:398–401.PubMedCrossRefGoogle Scholar
  15. 15.
    Auvert J. Portal hypertension in children. Long-term results of porto-caval interventions for extra-hepatic blockage. Chir Pédiatr. 1982;23:234–6.PubMedGoogle Scholar
  16. 16.
    Ahn SS, Yellin A, Sheng FC, et al. Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg. 1987;5:28–37.PubMedCrossRefGoogle Scholar
  17. 17.
    Descottes B, Lachachi F, Maisonnette F, et al. Long-term results of mesocaval shunts with polytetrafluoroethylene grafts. Int Surg. 2008;93:268–73.PubMedGoogle Scholar
  18. 18.
    Altman RP. Portal decompression by interposition mesocaval shunt in patients with biliary atresia. J Pediatr Surg. 1976;11:809–14.PubMedCrossRefGoogle Scholar
  19. 19.
    Gauthier F, De Dreuzy O, Valayer J, et al. H-type shunt with an autologous venous graft for treatment of portal hypertension in children. J Pediatr Surg. 1989;24:1041–3.PubMedCrossRefGoogle Scholar
  20. 20.
    Collins JC, Rypins EB, Sarfeh IJ. Narrow-diameter portacaval shunts for management of variceal bleeding. World J Surg. 1994;18:211–5.PubMedCrossRefGoogle Scholar
  21. 21.
    Rypins EB, Sarfeh IJ. Partial portal decompression: two approaches to the management of portal hypertension. Hepatology. 1990;12:370–2.PubMedCrossRefGoogle Scholar
  22. 22.
    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.PubMedCrossRefGoogle Scholar
  23. 23.
    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.PubMedGoogle Scholar
  24. 24.
    de Ville de Goyet J, Alberti D, Falchetti D, et al. Treatment of extrahepatic portal hypertension in children with mesenteric-to-left portal vein bypass: a new physiological procedure. Eur J Surg. 1999;165:777–81.PubMedCrossRefGoogle Scholar
  25. 25.
    de Ville de Goyet J, Gibbs P, Clapuyt P, et al. Original extrahilar approach for hepatic portal revascularization and relief of extrahepatic portal hypertension related to later portal vein thrombosis after pediatric liver transplantation. Long term results. Transplantation. 1996;62:71–5.PubMedCrossRefGoogle Scholar
  26. 26.
    Dasgupta R, Roberts E, Superina RA, et al. Effectiveness of Rex shunt in the treatment of portal hypertension. J Pediatr Surg. 2006;41:108–12.PubMedCrossRefGoogle Scholar
  27. 27.
    Krebs-Schmitt D, Briem-Richter A, Grabhorn E, et al. Effectiveness of Rex shunt in children with portal hypertension following liver transplantation or with primary portal hypertension. Pediatr Transplant. 2009;13:540–4.PubMedCrossRefGoogle Scholar
  28. 28.
    • Sharif K, McKiernan P, de Ville de Goyet J, et al.: Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most! J Pediatr Surg. 2010; 45:272–6. This paper reports an experience of meso-portal bypass showing good results. It highlights the fact that meso-rex with jugular vein has the best result, the spleen decrease in size but returns to normal for age in 30% of children. The return of flow in the cavernoma needs further evaluation.PubMedCrossRefGoogle Scholar
  29. 29.
    • Superina R, Shneider B, Emre S, et al.: Surgical guidelines for the management of extra-hepatic portal vein obstruction. Pediatr Transplant. 2006; 10:908–13. This fine article gives guidelines for the management of portal hypertension in children.PubMedCrossRefGoogle Scholar
  30. 30.
    Chiu B, Pillai SB, Sandler AD, et al. Experience with alternate sources of venous inflow in the meso-Rex bypass operation: the coronary and splenic veins. J Pediatr Surg. 2007;42:1199–202.PubMedCrossRefGoogle Scholar
  31. 31.
    Ateş O, Hakgüder G, Olguner M, et al. Extrahepatic portal hypertension treated by anastomosing inferior mesenteric vein to left portal vein at Rex recessus. J Pediatr Surg. 2003;38:E10–1.PubMedGoogle Scholar
  32. 32.
    Chardot C, Darani A, Dubois R, et al. Modified technique of meso-Rex shunt in case of insufficient length of the jugular vein graft. J Pediatr Surg. 2009;44:e9–12.PubMedCrossRefGoogle Scholar
  33. 33.
    Chin AC, Thow F, Superina RA. Previous portal hypertension surgery negatively affects results of mesenteric to left portal vein bypass. J Pediatr Surg. 2008;43:114–9.PubMedCrossRefGoogle Scholar
  34. 34.
    Sugiura M, Futagawa S. A new technique for treating esophageal varices. J Thorac Cardiovasc Surg. 1973;66:677–85.PubMedGoogle Scholar
  35. 35.
    Sharma D, Agrawal S, Saxena A, et al. A modified technique of devascularization for surgical management of portal hypertension in children. Trop Doct. 2001;31:93–5.PubMedGoogle Scholar
  36. 36.
    Superina RA, Weber JL, Shandling B. A modified Sugiura operation for bleeding varices in children. J Pediatr Surg. 1983;18:794–9.PubMedCrossRefGoogle Scholar
  37. 37.
    Uchiyama M, Iwafuchi M, Ohsawa Y, et al. Long-term results after nonshunt operations for esophageal varices in children. J Pediatr Surg. 1994;29:1429–33.PubMedCrossRefGoogle Scholar
  38. 38.
    Abouna GM, Baissony H, Al-Nakib BM, et al. The place of Sugiura operation for portal hypertension and bleeding esophageal varices. Surgery. 1987;101:91–8.PubMedGoogle Scholar
  39. 39.
    Shah R, Mahour GH, Ford EG, et al. Partial splenic embolization: an effective alternative to splenectomy for hypersplenism. Am Surg. 1990;56:774–7.PubMedGoogle Scholar
  40. 40.
    Kheradpir MH. Late results of a modified new surgical procedure for the treatment of portal hypertension. Eur J Pediatr Surg. 2007;17:17–22.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Paediatric HPB Surgery and Transplantation, Liver UnitBirmingham Children’s HospitalBirminghamUK

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