Infantile Hypertrophic Pyloric Stenosis

  • Takao FujimotoEmail author
Living reference work entry


Infantile hypertrophic pyloric stenosis (IHPS) is a common surgical condition encountered in early infancy. Despite the extensive research, the etiology of IHPS remains still obscure. Genetic, environmental, and hormonal factors have been implicated in the pathogenesis of this condition. In terms of the pathophysiology, abnormalities of smooth muscle cells, growth factors, extracellular matrix, nerve and supporting systems, neurotransmitter, and intestinal cell of Cajal have been also reported. The incidence is approximately 1–5:1,000 with a peak incidence of 2–8 weeks. Also, epidemiologically, wide variations of incidence have been reported with geographic location, season, and ethnic origin. Although extramucosal pyloromyotomy is the procedure of choice, the best way to approach the pylorus is debated; conventional right upper quadrant open procedure, transumbilical approach, and more recently laparoscopic procedure. This chapter outlines the new insights into the etiology and pathogenesis and also the important elements necessary to care for the patients in safe and effective manner.


Hypertrophic pyloric stenosis Epidemiology Etiology Genetics Environmental and extrinsic factors Diagnosis Preoperative care Surgical technique Postoperative care 


  1. Adibe OO, Nichol PF, Lim FY, et al. Ad libitum feeds after laparoscopic pyloromyotomy: a retrospective comparison with standardized feeding regimen in 227 infants. J Laparoendosc Adv Surg Tech A. 2007;17(2):235–7.CrossRefPubMedGoogle Scholar
  2. Alain JL, Grousseau D, Terrier G. Extramucosal pyloromyotomy by laparoscopy. Surg Endosc. 1991;5(4):174–5.CrossRefPubMedGoogle Scholar
  3. Aspelund G, Langer JC. Current management of hypertrophic pyloric stenosis. Semin Pediatr Surg. 2007;16(1):27–33.CrossRefPubMedGoogle Scholar
  4. Capon F, Reece A, Ravindarajah R, et al. Linkage of monogenic infantile hypertrophic pyloric stenosis to chromosome 16p12-p13 and evidence of genetic heterogeneity. Am J Hum Genet. 2006;79(2):378–82.CrossRefPubMedPubMedCentralGoogle Scholar
  5. Cooper WO, Ray WA. Prenatal prescription of macrolide antibiotics and infantile hypertrophied pyloric stenosis. Obstet Gynecol. 2002;100(1):101–6.PubMedGoogle Scholar
  6. Everett K, Chioza BA, Georgoula C, et al. Linkage of monogenic infantile hypertrophic pyloric stenosis to chromosome 16p24. Eur J Hum Genet. 2008a;16(9):1151–4.CrossRefPubMedGoogle Scholar
  7. Everett KV, Chioza BA, Georgroula C, et al. Genome-wide high-density SNP-based linkage analysis of infantile hypertrophic pyloric stenosis identifies loci on chromosomes 11q14-q22 and Xq23. Am J Hum Genet. 2008b;82(3):756–62.CrossRefPubMedPubMedCentralGoogle Scholar
  8. Garza JJ, Morash D, Dzakovic A, et al. Ad libitum feeding decreases hospital stay for neonates after pyloromyotomy. J Pediatr Surg. 2002;37(3):493–5.CrossRefPubMedGoogle Scholar
  9. Georgeson KE, Corbin TJ, Griffen JW, et al. An analysis of feeding regimens after pyloromyotomy for hypertrophic pyloric stenosis. J Pediatr Surg. 1993;28(11):1478–80.CrossRefPubMedGoogle Scholar
  10. Hall NJ, Van Der Zee J, Tan HL, et al. Meta-analysis of laparoscopic versus open pyloromyotomy. Ann Surg. 2004;240(5):774–8.CrossRefPubMedCentralGoogle Scholar
  11. Hernaz-Schulman M. Pyloric stenosis: role of imaging. Pediatr Radiol. 2009;39(Suppl 2):S134–9.CrossRefGoogle Scholar
  12. Huang PL, Dawson TM, Bredt DS, et al. Targeted disruption of neuronal nitric oxide gene. Cell. 1993;75:1273–86.CrossRefPubMedGoogle Scholar
  13. Indiran V, Selvaraj V. The cervix sign and other sonographic signs of hypertrophic pyloric stenosis. Abdom Radiol (NY). 2016;41(10):2085–6.CrossRefGoogle Scholar
  14. Iqbal CW, Rivard DC, Mortellao VE, et al. Evaluation of ultrasonographic parameters in the diagnosis of pyloric stenosis relative to patient age and size. J Pediatr Surg. 2012;47(8):1542–7.CrossRefPubMedGoogle Scholar
  15. Katz MS, Schwartz MZ, Moront ML, et al. Prophylactic antibiotics do not decrease the incidence of wound infections after laparoscopic pyloromyotomy. J Pediatr Surg. 2011;46(6):1086–8.CrossRefPubMedGoogle Scholar
  16. Kawai M, Peretta S, Burckhardt O, et al. Endoscopic pyloromyotomy: a new concept of minimally invasive surgery for pyloric stenosis. Endoscopy. 2012;44:169–73.CrossRefPubMedGoogle Scholar
  17. Kim SS, Lau ST, Lee SL, et al. Pyloromyotomy: a comparison of laparoscopic, circumumbilical and right upper quadrant operative techniques. J Am Coll Surg. 2005;201(1):66–70.CrossRefPubMedGoogle Scholar
  18. Koike Y, Uchihara K, Nakazawa M, et al. Predictive factors of negative outcome in initial atropine therapy for infantile pyloric stenosis. Pediatr Int. 2013;55(5):619–23. doi: 10.1111/ped.12137.CrossRefPubMedGoogle Scholar
  19. Krogh C, Fischer TK, Skotte L, et al. Familial aggregation and heritability of pyloric stenosis. JAMA. 2011;303(23):2393–9.CrossRefGoogle Scholar
  20. Krohg C, Biggar RJ, Fischer TK, et al. Bottle-feeding and the risk of pyloric stenosis. Pediatrics. 2012;130:e943–9.CrossRefGoogle Scholar
  21. Kusafuka T, Puri P. Altered messenger RNA expression of neuronal nitric oxide synthase gene in infantile hypertrophic pyloric stenosis. Pediatr Surg Int. 1997;12:576–9.CrossRefPubMedGoogle Scholar
  22. Ladd AP, Nemeth SA, Grosfeld JL, et al. Supraumbilical pyloromyotomy: a unique indication for antimicrobial prophylaxis. J Pediatr Surg. 2005;40(6):974–7.CrossRefPubMedGoogle Scholar
  23. Laffoklie J, Turial S, Heckmann M, et al. Decline in infantile hypertrophic pyloric stenosis in Germany in 2000–2008. Pediatrics. 2012;129(4):e901–6.CrossRefGoogle Scholar
  24. Leaphart CL, Borland K, Kane TD, et al. Hypertrophic pyloric stenosis in newborns younger than 21 days: remodeling the path of surgical intervention. J Pediatr Surg. 2008;43(6):998–1001.CrossRefPubMedGoogle Scholar
  25. Leclair MD, Plattner V, Mirallie E, et al. Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a perspective, randomized controlled trial. J Pediatr Surg. 2007;42(4):692–8.CrossRefPubMedGoogle Scholar
  26. Lowe LH, Banks WJ, Shyr Y, et al. Pyloric ratio: efficacy in the diagnosis of hypertrophic pyloric stenosis. J Ultrasound Med. 1999;18(11):773–7.CrossRefPubMedGoogle Scholar
  27. Macdessi J, Oates RK. Clinical diagnosis of pyloric stenosis: a declining art. BMJ. 1993;306(6877):553–5.CrossRefPubMedPubMedCentralGoogle Scholar
  28. MacMahon B. The continuing enigma of pyloric stenosis of infancy: a review. Epidemiology. 2006;17(2):195–201.CrossRefPubMedGoogle Scholar
  29. Mahon BE, Rosenman MB, Kleiman MB. Maternal and infant use of erythromycin and other macrolide antibiotics as risk factors for infantile hypertrophic pyloric stenosis. J Pediatr. 2001;139(3):380–4.CrossRefPubMedGoogle Scholar
  30. Malcom 3rd GE, Raio CC, Del Rios M, et al. Feasibility of emergency physician diagnosis of hypertrophic pyloric stenosis using point-of-case ultrasound: a multi-center cases series. J Emerg Med. 2009;37(39):283–6.CrossRefPubMedGoogle Scholar
  31. Markel TA, Scott MR, Stokes SM, Ladd AP. A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. J Pediatr Surg. 2016. doi: 10.1016/j.jpedsurg.2016.09.069. pii: S0022–3468(16)30457–2. [Epub ahead of print].
  32. Murchison L, De Coppi P, Eaton S. Post-natal erythromycin exposure and risk of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. Pediatr Surg Int. 2016;32(12):1147–52.CrossRefPubMedPubMedCentralGoogle Scholar
  33. National Birth defect Prevention Network. Selected birth defects data from population based birth defects surveillance programs in United States, 2003–2007. Birth Defects Res A Clin Mol Teratol. 2010;88(12):1062–174.CrossRefGoogle Scholar
  34. NIedzielski J, Kobielski A, Sokal J, et al. Accuracy of sonographic criteria in the decision for surgical treatment in infantile hypertrophic pyloric stenosis. Arch Med Sci. 2011;7(3):508–11.CrossRefPubMedPubMedCentralGoogle Scholar
  35. Nour S, Mackinnon AE, Dickson JA, et al. Antibiotics prophylaxis for infantile pyloromyotomy. J R Coll Surg Edinb. 1996;41(3):178–80.PubMedGoogle Scholar
  36. Oomen MW, Hoekstra LT, Bakx R, et al. Open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a systemic review and meta-analysis focusing on major complications. Surg Endosc. 2012;26(8):2104–10.CrossRefPubMedPubMedCentralGoogle Scholar
  37. Panteli C. New insights into the pathogenesis of infantile pyloric stenosis. Pediatr Surg Int. 2009;25(12):1043–52.CrossRefPubMedGoogle Scholar
  38. Pedersen RN, Garne E, Loane M, EUROCAT Working Group, et al. Infantile hypertrophic pyloric stenosis: a comparative study of incidence and other epidemiological characteristics in seven European regions. J Matern Fetal Neonatal Med. 2008;21(9):599–604.CrossRefPubMedGoogle Scholar
  39. Persson S, Ekbom A, Granath F, et al. Parallel incidence of sudden infant death syndrome and infantile hypertrophic pyloric stenosis: a common cause? Pediatrics. 2001;108(4):E70.CrossRefPubMedGoogle Scholar
  40. Purapong D, Kahng D, Ko A, et al. Ad libitum feeding; safely improving the cost effectiveness of pyloromyotomy. J Pediatr Surg. 2002;37(12):1667–8.CrossRefGoogle Scholar
  41. Ranells JD, Carver JD, Kirby RS. Infantile hypertrophic pyloric stenosis: epidemiology, genetics and clinical update. Adv Pediatr Infect Dis. 2011;58(1):195–206.Google Scholar
  42. Schari AF, Leditschke JF. Gastric motility after pyloromyotomy in infants: a reappraisal of postoperative feeding. Surgery. 1968;64(6):1133–7.Google Scholar
  43. Serra A, Schuchardt K, Genuneit J, et al. The role of ret genomic variants in infantile hypertrophic pyloric stenosis. Eur J Pediatr Surg. 2011;21(6):389–94.CrossRefPubMedGoogle Scholar
  44. Shinohara K, Shimizu T. Correlation of prostaglandin E2 production and gastric acid secretion in infants with hypertrophic pyloric stenosis. J Pediatr Surg. 1998;33(10):1483–5.CrossRefPubMedGoogle Scholar
  45. Sola JE, Neville HL. Laparoscopic vs open pyloromyotomy: a systematic review and meta-analysis. J Pediatr Surg. 2009;44(8):1631–7.CrossRefPubMedGoogle Scholar
  46. Sorensen HT, Skriver MV. Risk of infantile hypertrophic pyloric stenosis after maternal postnatal use of macrolides. Scand J Infect Dis. 2003;35(2):104–6.CrossRefPubMedGoogle Scholar
  47. St Peter SD, Osstli DJ. Pyloric stenosis: from a retrospective analysis to a prospective clinical trial-the impact on surgical outcomes. Curr Opin Pediatr. 2008;20(3):311–4.CrossRefPubMedGoogle Scholar
  48. St Peter SD, Holcomb 3rd GW, Calkins CM, et al. Open versus laparoscopic pyloromyotomy for pyloric stenosis: a prospective, randomized trial. Ann Surg. 2006;244(3):363–7.PubMedPubMedCentralGoogle Scholar
  49. Sullivan KJ, Chan E, Vincent J, Canadian Association of Paediatric Surgeons Evidence-Based Resource, et al. Feeding post-pyloromyotomy: a meta-analysis. Pediatrics. 2016;137(1):1–11.CrossRefGoogle Scholar
  50. Svenningsson A, Soderhall C, Persson S, et al. Genome-wide linkage analysis in families with infantile hypertrophic pyloric stenosis indicates novel susceptibility loci. J Hum Genet. 2012;57(2):115–21.CrossRefPubMedGoogle Scholar
  51. Takeuchi M, Yasunaga H, Horiguchi H, et al. Pyloromyotomy versus i.v. atropine therapy for the treatment of infantile hypertrophic pyloric stenosis: nationwide hospital discharge database analysis. Pediatr Int. 2013;55(4):488–91.CrossRefPubMedGoogle Scholar
  52. Tan KC, Bianchi A. Circumumbilical incision for pyloromyotomy. Br J Surg. 1986;73(5):399.CrossRefPubMedGoogle Scholar
  53. Turnock RR, Rangercroft L. Comparison of pyloromyotomy feeding regimens in infantile hypertrophic pyloric stenosis. J R Coll Surg Edinb. 1991;36(3):164–5.PubMedGoogle Scholar
  54. Wang J, Waller DK, Hwang LY, et al. Prevalence of infantile hypertrophic stenosis in Texas.1999–2002. Birth Defects Res A Clin Mol Teratol. 2008;82(11):763–7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of Paediatric SurgeryImperial Gift Foundation, Aiiku Maternal and Children’s Medical CenterTokyoJapan

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