Pediatric Surgery International

, Volume 34, Issue 1, pp 21–27 | Cite as

Pyloric stenosis: an enigma more than a century after the first successful treatment

  • Yousef El-Gohary
  • Abdelhafeez Abdelhafeez
  • Elizabeth Paton
  • Ankush Gosain
  • Andrew J. Murphy
Review Article

Abstract

Despite hypertrophic pyloric stenosis (HPS) being one of the most frequently treated pediatric surgical conditions, its etiology remains incompletely understood. We review the diagnosis and treatment of this condition with an emphasis on the evolution of surgical techniques that led to laparoscopic pyloromyotomy, the most frequently performed technique for HPS today. In addition, we review key developments in the understanding of HPS etiology and treatment, including the postulated etiology of work-induced hypertrophy of the pylorus, its association with prokinetic macrolide antibiotics, and the emerging role of atropine sulfate as a medical treatment for HPS or a rescue treatment for incomplete myotomy.

Keywords

Pyloric stenosis Pyloromyotomy Laparoscopic pyloromyotomy Macrolide 

Notes

Acknowledgements

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Ethical approval

This article does not contain any studies with human participants performed by any of the authors.

Conflict of interest

The authors declare no conflict of interest.

Supplementary material

Supplementary material 1 (WMV 18474 KB)

References

  1. 1.
    Ramstedt C (1912) Zur operation der angeborenen pylorus stenose. Med Klin 8:1702–1705Google Scholar
  2. 2.
    To T, Wajja A, Wales PW, Langer JC (2005) Population demographic indicators associated with incidence of pyloric stenosis. Arch Pediatr Adolesc Med 159:520–525CrossRefPubMedGoogle Scholar
  3. 3.
    Hirschsprung H (1888) Falle von angeborener pylorus stenose, beobachtet bej sauglingen. Jahrb der Kinderh 28:61–68Google Scholar
  4. 4.
    Taylor S (1959) Pyloric stenosis before and after Ramstedt. Arch Dis Child 34:20–23CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Gardner W (1889) A case of Loreta’s operation for dilatation of the pyloric orifice of the stomach. Br Med J 2:1322–1323CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Nicoll JH (1904) Congenital hypertrophic stenosis of the pylorus. From an experience of fifteen cases. Br Med J 2:1148–1149Google Scholar
  7. 7.
    MacKinlay G, Barhnart D (2008) Laparoscopic pyloromyotomy. In: Bax NMA (ed) Endoscopic surgery in infants and children. Springer, Berlin, pp 281–286CrossRefGoogle Scholar
  8. 8.
    Graham RR (1937) The treatment of perforated duodenal ulcers. Surg Gynecol Obstet 64:235–238Google Scholar
  9. 9.
    Alain JL, Grousseau D, Terrier G (1991) Extramucosal pyloromyotomy by laparoscopy. Surg Endosc 5:174–175CrossRefPubMedGoogle Scholar
  10. 10.
    Bickler SW (2000) Incidence of appendicitis and hypertrophic pyloric stenosis in a sub-Saharan African country. J Pediatr Gastroenterol Nutr 31:S39CrossRefGoogle Scholar
  11. 11.
    Bickler SW (2006) Tropical enteropathy protects against Western diseases in environments of poor sanitation. Med Hypotheses 67:146–150CrossRefPubMedGoogle Scholar
  12. 12.
    El-Gohary Y, Yeap BH, Hempel G, Gillick J (2010) A 9-year single center experience with circumumbilical Ramstedt’s pyloromyotomy. Eur J Pediatr Surg 20:387–390CrossRefPubMedGoogle Scholar
  13. 13.
    Schechter R, Torfs CP, Bateson TF (1997) The epidemiology of infantile hypertrophic pyloric stenosis. Paediatr Perinat Epidemiol 11:407–427CrossRefPubMedGoogle Scholar
  14. 14.
    Lippert MM (1986) Pyloric stenosis presenting as severe prolonged jaundice. A case report. S Afr Med J 69:446–447PubMedGoogle Scholar
  15. 15.
    Trioche P, Chalas J, Francoual J, Capel L, Lindenbaum A, Odievre M, Labrune P (1999) Jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert syndrome. Arch Dis Child 81:301–303CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Greenberg CS (2002) A sugar-coated pacifier reduces procedural pain in newborns. Pediatr Nurs 28:271–277PubMedGoogle Scholar
  17. 17.
    White MC, Langer JC, Don S, DeBaun MR (1998) Sensitivity and cost minimization analysis of radiology versus olive palpation for the diagnosis of hypertrophic pyloric stenosis. J Pediatr Surg 33:913–917CrossRefPubMedGoogle Scholar
  18. 18.
    Feddock CA (2007) The lost art of clinical skills. Am J Med 120:374–378CrossRefPubMedGoogle Scholar
  19. 19.
    Jauhar S (2006) The demise of the physical exam. N Engl J Med 354:548–551CrossRefPubMedGoogle Scholar
  20. 20.
    Aspelund G, Langer JC (2007) Current management of hypertrophic pyloric stenosis. Semin Pediatr Surg 16:27–33CrossRefPubMedGoogle Scholar
  21. 21.
    Glatstein M, Carbell G, Boddu SK, Bernardini A, Scolnik D (2011) The changing clinical presentation of hypertrophic pyloric stenosis: the experience of a large, tertiary care pediatric hospital. Clin Pediatr (Phila) 50:192–195CrossRefGoogle Scholar
  22. 22.
    Gale HI, Gee MS, Westra SJ, Nimkin K (2016) Abdominal ultrasonography of the pediatric gastrointestinal tract. World J Radiol 8:656–667CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Jobson M, Hall NJ (2016) Contemporary management of pyloric stenosis. Semin Pediatr Surg 25:219–224CrossRefPubMedGoogle Scholar
  24. 24.
    Pandya S, Heiss K (2012) Pyloric stenosis in pediatric surgery: an evidence-based review. Surg Clin N Am 92:527–539 vii-viii.CrossRefPubMedGoogle Scholar
  25. 25.
    Flageole HH, Pemberton J (2015) Post-operative impact of nasogastric tubes on length of stay in infants with pyloric stenosis (POINTS): a prospective randomized controlled pilot trial. J Pediatr Surg 50:1681–1685CrossRefPubMedGoogle Scholar
  26. 26.
    Oomen MW, Hoekstra LT, Bakx R, Ubbink DT, Heij HA (2012) Open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a systematic review and meta-analysis focusing on major complications. Surg Endosc 26:2104–2110CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Mahida JB, Asti L, Deans KJ, Minneci PC, Groner JI (2016) Laparoscopic pyloromyotomy decreases postoperative length of stay in children with hypertrophic pyloric stenosis. J Pediatr Surg 51:1436–1439CrossRefPubMedGoogle Scholar
  28. 28.
    Hall NJ, Eaton S, Seims A, Leys CM, Densmore JC, Calkins CM, Ostlie DJ, St Peter SD, Azizkhan RG, von Allmen D et al (2014) Risk of incomplete pyloromyotomy and mucosal perforation in open and laparoscopic pyloromyotomy. J Pediatr Surg 49:1083–1086CrossRefPubMedGoogle Scholar
  29. 29.
    Leahy A, Fitzgerald RJ (1982) The influence of delayed feeding on postoperative vomiting in hypertrophic pyloric stenosis. Br J Surg 69:658–659CrossRefPubMedGoogle Scholar
  30. 30.
    Scharli AF, Leditschke JF (1968) Gastric motility after pyloromyotomy in infants. A reappraisal of postoperative feeding. Surgery 64:1133–1137PubMedGoogle Scholar
  31. 31.
    Sullivan KJ, Chan E, Vincent J, Iqbal M, Wayne C, Nasr A, Canadian Association of Paediatric Surgeons Evidence-Based., R (2016) Feeding post-pyloromyotomy: a meta-analysis. Pediatrics 137Google Scholar
  32. 32.
    Adibe OO, Iqbal CW, Sharp SW, Juang D, Snyder CL, Holcomb GW 3rd, Ostlie DJ, St Peter SD (2014) Protocol versus ad libitum feeds after laparoscopic pyloromyotomy: a prospective randomized trial. J Pediatr Surg 49:129–132. (discussion 132)CrossRefPubMedGoogle Scholar
  33. 33.
    Wheeler RA, Najmaldin AS, Stoodley N, Griffiths DM, Burge DM, Atwell JD (1990) Feeding regimens after pyloromyotomy. Br J Surg 77:1018–1019CrossRefPubMedGoogle Scholar
  34. 34.
    Owen RP, Almond SL, Humphrey GM (2012) Atropine sulphate: rescue therapy for pyloric stenosis. BMJ case reports 2012Google Scholar
  35. 35.
    Muramori K, Nagasaki A, Kawanami T (2007) Ultrasonographic serial measurements of the morphologic resolution of the pylorus after Ramstedt pyloromyotomy for infantile hypertrophic pyloric stenosis. J Ultrasound Med 26:1681–1687CrossRefPubMedGoogle Scholar
  36. 36.
    Takeuchi M, Yasunaga H, Horiguchi H, Hashimoto H, Matsuda S (2013) Pyloromyotomy versus i.v. atropine therapy for the treatment of infantile pyloric stenosis: nationwide hospital discharge database analysis. Pediatr Int Off J Jpn Pediatric Soc 55:488–491CrossRefGoogle Scholar
  37. 37.
    Konturek SJ, Biernat J, Oleksy J, Rehfeld JF, Stadil F (1974) Effect of atropine on gastrin and gastric acid response to peptone meal. J Clin Invest 54:593–597CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Rogers IM (2006) The true cause of pyloric stenosis is hyperacidity. Acta paediatrica 95:132–136CrossRefPubMedGoogle Scholar
  39. 39.
    Heine W, Groger B, Litzenberger M, Drescher U (1986) [Results of Lambling gastric juice analysis in infants with spastic hypertrophic pyloric stenosis (SHPS)]. Padiatrie Padologie 21:119–125PubMedGoogle Scholar
  40. 40.
    Dodge JA, Karim AA (1976) Induction of pyloric hypertrophy by pentagastrin. An animal model for infantile hypertrophic pyloric stenosis. Gut 17:280–284CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Coulie B, Tack J, Peeters T, Janssens J (1998) Involvement of two different pathways in the motor effects of erythromycin on the gastric antrum in humans. Gut 43:395–400CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Honein MA, Paulozzi LJ, Himelright IM, Lee B, Cragan JD, Patterson L, Correa A, Hall S, Erickson JD (1999) Infantile hypertrophic pyloric stenosis after pertussis prophylaxis with erythromcyin: a case review and cohort study. Lancet 354:2101–2105CrossRefPubMedGoogle Scholar
  43. 43.
    Lund M, Pasternak B, Davidsen RB, Feenstra B, Krogh C, Diaz LJ, Wohlfahrt J, Melbye M (2014) Use of macrolides in mother and child and risk of infantile hypertrophic pyloric stenosis: nationwide cohort study. BMJ 348:g1908CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Eberly MD, Eide MB, Thompson JL, Nylund CM (2015) Azithromycin in early infancy and pyloric stenosis. Pediatrics 135:483–488CrossRefPubMedGoogle Scholar
  45. 45.
    Ames MD (1960) Gastric acidity in the first ten days of life of the prematurely born baby. Am J Dis Child 100:252–256PubMedGoogle Scholar
  46. 46.
    Krogh C, Biggar RJ, Fischer TK, Lindholm M, Wohlfahrt J, Melbye M (2012) Bottle-feeding and the risk of pyloric stenosis. Pediatrics 130:e943-949CrossRefGoogle Scholar
  47. 47.
    Piotrowska AP, Solari V, Puri P (2003) Distribution of heme oxygenase-2 in nerves and interstitial cells of Cajal in the normal pylorus and in infantile hypertrophic pyloric stenosis. Arch Pathol Lab Med 127:1182–1186PubMedGoogle Scholar
  48. 48.
    Oue T, Puri P (1999) Smooth muscle cell hypertrophy versus hyperplasia in infantile hypertrophic pyloric stenosis. Pediatr Res 45:853–857CrossRefPubMedGoogle Scholar
  49. 49.
    Huang PL, Dawson TM, Bredt DS, Snyder SH, Fishman MC (1993) Targeted disruption of the neuronal nitric oxide synthase gene. Cell 75:1273–1286CrossRefPubMedGoogle Scholar
  50. 50.
    Peeters B, Benninga MA, Hennekam RC (2012) Infantile hypertrophic pyloric stenosis–genetics and syndromes. Nat Rev Gastroenterol Hepatol 9:646–660CrossRefPubMedGoogle Scholar
  51. 51.
    Krogh C, Fischer TK, Skotte L, Biggar RJ, Oyen N, Skytthe A, Goertz S, Christensen K, Wohlfahrt J, Melbye M (2010) Familial aggregation and heritability of pyloric stenosis. Jama 303:2393–2399CrossRefPubMedGoogle Scholar
  52. 52.
    Pedersen RN, Garne E, Loane M, Korsholm L, Husby S, Group EW (2008) Infantile hypertrophic pyloric stenosis: a comparative study of incidence and other epidemiological characteristics in seven European regions. J Maternal Fetal Neonatal Med 21:599–604CrossRefGoogle Scholar
  53. 53.
    Stankiewicz P, Sen P, Bhatt SS, Storer M, Xia Z, Bejjani BA, Ou Z, Wiszniewska J, Driscoll DJ, Maisenbacher MK et al (2009) Genomic and genic deletions of the FOX gene cluster on 16q24.1 and inactivating mutations of FOXF1 cause alveolar capillary dysplasia and other malformations. Am J Hum Genet 84:780–791CrossRefPubMedPubMedCentralGoogle Scholar
  54. 54.
    Everett KV, Ataliotis P, Chioza BA, Shaw-Smith C, Chung E.M.K. (2017) A novel missense mutation in the transcription factor FOXF1 cosegregating with infantile hypertrophic pyloric stenosis in the extended pedigree linked to IHPS5 on chromosome 16q24. Pediatr Res 81:632–638CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of SurgerySt. Jude Children’s Research HospitalMemphisUSA
  2. 2.Division of Pediatric Surgery, Department of SurgeryUniversity of Tennessee Health Sciences CenterMemphisUSA

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