Skip to main content

Esophageal Atresia

  • Chapter
  • First Online:
Pediatric Neurogastroenterology

Abstract

Esophageal atresia is a common congenital anomaly associated with esophageal and gastric dysmotility. The dysmotility may be caused by intrinsic factors and operative manoeuvres. Adequate understanding of the esophageal dysfunction is mandatory in order to treat and ultimately prevent complications.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Brosens E, Ploeg M, van Bever Y, et al. Clinical and etiological heterogeneity in patients with tracheo-esophageal malformations and associated anomalies. Eur J Med Genet. 2014;57:440–52.

    PubMed  Google Scholar 

  2. Castilloux J, Noble AJ, Faure C. Risk factors for short- and long-term morbidity in children with esophageal atresia. J Pediatr. 2010;156:755–60.

    PubMed  Google Scholar 

  3. Vergouwe FWT, IJsselstijn H, Biermann K, et al. High prevalence of Barrett’s esophagus and esophageal squamous cell carcinoma after repair of esophageal atresia. Clin Gastroenterol Hepatol. 2018;16:513–521.e6.

    PubMed  Google Scholar 

  4. Hsieh H, Frenette A, Michaud L, et al. Intestinal metaplasia of the esophagus in children with esophageal atresia. J Pediatr Gastroenterol Nutr. 2017;65:e1–4.

    PubMed  Google Scholar 

  5. Krishnan U, Mousa H, Dall’Oglio L, et al. ESPGHAN-NASPGHAN guidelines for the evaluation and treatment of gastrointestinal and nutritional complications in children with esophageal atresia-tracheoesophageal fistula. J Pediatr Gastroenterol Nutr. 2016;63:550–70.

    PubMed  Google Scholar 

  6. Banjar HH, Al-Nassar SI. Gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula. Saudi Med J. 2005;26:781–5.

    PubMed  Google Scholar 

  7. McKinnon LJ, Kosloske AM. Prediction and prevention of anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1990;25:778–81.

    CAS  PubMed  Google Scholar 

  8. Deurloo JA, Ekkelkamp S, Schoorl M, et al. Esophageal atresia: historical evolution of management and results in 371 patients. Ann Thorac Surg. 2002;73:267–72.

    PubMed  Google Scholar 

  9. Frohlich T, Otto S, Weber P, et al. Combined esophageal multichannel intraluminal impedance and pH monitoring after repair of esophageal atresia. J Pediatr Gastroenterol Nutr. 2008;47:443–9.

    PubMed  Google Scholar 

  10. Catalano P, Di Pace MR, Caruso AM, et al. Gastroesophageal reflux in young children treated for esophageal atresia: evaluation with pH-multichannel intraluminal impedance. J Pediatr Gastroenterol Nutr. 2011;52:686–90.

    PubMed  Google Scholar 

  11. Tong S, Mallitt KA, Krishnan U. Evaluation of Gastroesophageal reflux by combined multichannel intraluminal impedance and pH monitoring and esophageal motility patterns in children with esophageal atresia. Eur J Pediatr Surg. 2016;26:322–31.

    PubMed  Google Scholar 

  12. van Wijk M, Knuppe F, Omari T, et al. Evaluation of gastroesophageal function and mechanisms underlying gastroesophageal reflux in infants and adults born with esophageal atresia. J Pediatr Surg. 2013;48:2496–505.

    PubMed  Google Scholar 

  13. Donoso F, Lilja HE. Risk factors for anastomotic strictures after esophageal atresia repair: prophylactic proton pump inhibitors do not reduce the incidence of strictures. Eur J Pediatr Surg. 2017;27:50–5.

    PubMed  Google Scholar 

  14. Stenstrom P, Anderberg M, Borjesson A, et al. Prolonged use of proton pump inhibitors as stricture prophylaxis in infants with reconstructed esophageal atresia. Eur J Pediatr Surg. 2017;27:192–5.

    PubMed  Google Scholar 

  15. Righini Grunder F, Petit LM, Ezri J, et al. Should proton pump inhibitors be systematically prescribed in patients with esophageal atresia after surgical repair? J Pediatr Gastroenterol Nutr. 2019;69:45–51.

    CAS  PubMed  Google Scholar 

  16. Montgomery M, Frenckner B, Freyschuss U, et al. Esophageal atresia: long-term-follow-up of respiratory function, maximal working capacity, and esophageal function. Pediatr Surg Int. 1995;10:519–22.

    Google Scholar 

  17. Sistonen SJ, Koivusalo A, Nieminen U, et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann Surg. 2010;251:1167–73.

    PubMed  Google Scholar 

  18. Lemoine C, Aspirot A, Le Henaff G, et al. Characterization of esophageal motility following esophageal atresia repair using high-resolution esophageal manometry. J Pediatr Gastroenterol Nutr. 2013;56:609–14.

    PubMed  Google Scholar 

  19. Little DC, Rescorla FJ, Grosfeld JL, et al. Long-term analysis of children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2003;38:852–6.

    CAS  PubMed  Google Scholar 

  20. Pedersen RN, Markow S, Kruse-Andersen S, et al. Esophageal atresia: gastroesophageal functional follow-up in 5–15year old children. J Pediatr Surg. 2013;48:2487–95.

    PubMed  Google Scholar 

  21. Connor MJ, Springford LR, Kapetanakis VV, et al. Esophageal atresia and transitional care—step 1: a systematic review and meta-analysis of the literature to define the prevalence of chronic long-term problems. Am J Surg. 2015;209:747–59.

    PubMed  Google Scholar 

  22. Menzies J, Hughes J, Leach S, et al. Prevalence of malnutrition and feeding difficulties in children with esophageal atresia. J Pediatr Gastroenterol Nutr. 2017;64:e100–5.

    PubMed  Google Scholar 

  23. Dhaliwal J, Tobias V, Sugo E, et al. Eosinophilic esophagitis in children with esophageal atresia. Dis Esophagus. 2014;27:340–7.

    CAS  PubMed  Google Scholar 

  24. McCann F, Michaud L, Aspirot A, et al. Congenital esophageal stenosis associated with esophageal atresia. Dis Esophagus. 2015;28:211–5.

    CAS  PubMed  Google Scholar 

  25. Berthet S, Tenisch E, Miron MC, et al. Vascular anomalies associated with esophageal atresia and tracheoesophageal fistula. J Pediatr. 2015;166:1140–1144.e2.

    PubMed  Google Scholar 

  26. Tomaselli V, Volpi ML, Dell’Agnola CA, et al. Long-term evaluation of esophageal function in patients treated at birth for esophageal atresia. Pediatr Surg Int. 2003;19:40–3.

    CAS  PubMed  Google Scholar 

  27. Chapuy L, Pomerleau M, Perreault P, et al. Mucosal bridge as a cause of dysphagia after surgery for esophageal atresia. Can J Gastroenterol Hepatol. 2014;28:350.

    PubMed  PubMed Central  Google Scholar 

  28. Pham A, Ecochard-Dugelay E, Bonnard A, et al. Feeding disorders in children with oesophageal atresia: a cross-sectional study. Arch Dis Child. 2022;107(1):52–8.

    PubMed  Google Scholar 

  29. Yalcin S, Demir N, Serel S, et al. The evaluation of deglutition with videofluoroscopy after repair of esophageal atresia and/or tracheoesophageal fistula. J Pediatr Surg. 2015;50:1823–7.

    PubMed  Google Scholar 

  30. Hormann M, Pokieser P, Scharitzer M, et al. Videofluoroscopy of deglutition in children after repair of esophageal atresia. Acta Radiol. 2002;43:507–10.

    CAS  PubMed  Google Scholar 

  31. Morini F, Iacobelli BD, Crocoli A, et al. Symptomatic vocal cord paresis/paralysis in infants operated on for esophageal atresia and/or tracheo-esophageal fistula. J Pediatr. 2011;158:973–6.

    PubMed  Google Scholar 

  32. Mortellaro VE, Pettiford JN, St Peter SD, et al. Incidence, diagnosis, and outcomes of vocal fold immobility after esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) repair. Eur J Pediatr Surg. 2011;21:386–8.

    CAS  PubMed  Google Scholar 

  33. Fraga JC, Adil EA, Kacprowicz A, et al. The association between laryngeal cleft and tracheoesophageal fistula: myth or reality? Laryngoscope. 2015;125:469–74.

    PubMed  Google Scholar 

  34. Petit LM, Righini-Grunder F, Ezri J, et al. Prevalence and predictive factors of histopathological complications in children with esophageal atresia. Eur J Pediatr Surg. 2019;29:510–5.

    PubMed  Google Scholar 

  35. Krishnan U, Lijuan C, Andrew GJ, et al. Analysis of eosinophilic esophagitis in children with repaired congenital esophageal atresia. J Allergy Clin Immunol. 2019;143:1455–1464.e2.

    PubMed  Google Scholar 

  36. Chan LJ, Tan L, Dhaliwal J, et al. Treatment outcomes for eosinophilic esophagitis in children with esophageal atresia. Dis Esophagus. 2016;29:563–71.

    CAS  PubMed  Google Scholar 

  37. Kawahara H, Kubota A, Hasegawa T, et al. Lack of distal esophageal contractions is a key determinant of gastroesophageal reflux disease after repair of esophageal atresia. J Pediatr Surg. 2007;42:2017–21.

    PubMed  Google Scholar 

  38. Di Pace MR, Caruso AM, Catalano P, et al. Evaluation of esophageal motility and reflux in children treated for esophageal atresia with the use of combined multichannel intraluminal impedance and pH monitoring. J Pediatr Surg. 2011;46:443–51.

    PubMed  Google Scholar 

  39. Montgomery M, Witt H, Kuylenstierna R, et al. Swallowing disorders after esophageal atresia evaluated with videomanometry. J Pediatr Surg. 1998;33:1219–23.

    CAS  PubMed  Google Scholar 

  40. Romeo G, Zuccarello B, Proietto F, et al. Disorders of the esophageal motor activity in atresia of the esophagus. J Pediatr Surg. 1987;22:120–4.

    CAS  PubMed  Google Scholar 

  41. Hoffman I, De Greef T, Haesendonck N, et al. Esophageal motility in children with suspected gastroesophageal reflux disease. J Pediatr Gastroenterol Nutr. 2010;50:601–8.

    PubMed  Google Scholar 

  42. Dutta HK, Grover VP, Dwivedi SN, et al. Manometric evaluation of postoperative patients of esophageal atresia and tracheo-esophageal fistula. Eur J Pediatr Surg. 2001;11:371–6.

    CAS  PubMed  Google Scholar 

  43. Bozinovski J, Poenaru D, Paterson W, et al. Esophageal aperistalsis following fundoplication in a patient with trisomy 21. Pediatr Surg Int. 1999;15:510–1.

    CAS  PubMed  Google Scholar 

  44. Tovar JA, Diez Pardo JA, Murcia J, et al. Ambulatory 24-hour manometric and pH metric evidence of permanent impairment of clearance capacity in patients with esophageal atresia. J Pediatr Surg. 1995;30:1224–31.

    CAS  PubMed  Google Scholar 

  45. Di Pace MR, Caruso AM, Catalano P, et al. Evaluation of esophageal motility using multichannel intraluminal impedance in healthy children and children with gastroesophageal reflux. J Pediatr Gastroenterol Nutr. 2011;52:26–30.

    PubMed  Google Scholar 

  46. Courbette O, Omari T, Aspirot A, et al. Characterization of esophageal motility in children with operated esophageal atresia using high-resolution impedance manometry and pressure flow analysis. J Pediatr Gastroenterol Nutr. 2020;71:304–9.

    CAS  PubMed  Google Scholar 

  47. Duranceau A, Fisher SR, Flye M, et al. Motor function of the esophagus after repair of esophageal atresia and tracheoesophageal fistula. Surgery. 1977;82:116–23.

    CAS  PubMed  Google Scholar 

  48. Somppi E, Tammela O, Ruuska T, et al. Outcome of patients operated on for esophageal atresia: 30 years’ experience. J Pediatr Surg. 1998;33:1341–6.

    CAS  PubMed  Google Scholar 

  49. Lemoine C, Aspirot A, Morris M, et al. Esophageal dysmotility is present before surgery in isolated tracheoesophageal fistula. J Pediatr Gastroenterol Nutr. 2015;60:642–4.

    PubMed  Google Scholar 

  50. Nakazato Y, Wells TR, Landing BH. Abnormal tracheal innervation in patients with esophageal atresia and tracheoesophageal fistula: study of the intrinsic tracheal nerve plexuses by a microdissection technique. J Pediatr Surg. 1986;21:838–44.

    CAS  PubMed  Google Scholar 

  51. Dutta HK, Mathur M, Bhatnagar V. A histopathological study of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2000;35:438–41.

    CAS  PubMed  Google Scholar 

  52. Boleken M, Demirbilek S, Kirimiloglu H, et al. Reduced neuronal innervation in the distal end of the proximal esophageal atretic segment in cases of esophageal atresia with distal tracheoesophageal fistula. World J Surg. 2007;31:1512–7.

    PubMed  Google Scholar 

  53. Davies MR. Anatomy of the extrinsic motor nerve supply to mobilized segments of the oesophagus disrupted by dissection during repair of oesophageal atresia with distal fistula. Br J Surg. 1996;83:1268–70.

    CAS  PubMed  Google Scholar 

  54. Midrio P, Alaggio R, Strojna A, et al. Reduction of interstitial cells of Cajal in esophageal atresia. J Pediatr Gastroenterol Nutr. 2010;51:610–7.

    PubMed  Google Scholar 

  55. Li K, Zheng S, Xiao X, et al. The structural characteristics and expression of neuropeptides in the esophagus of patients with congenital esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2007;42:1433–8.

    PubMed  Google Scholar 

  56. Qi BQ, Uemura S, Farmer P, et al. Intrinsic innervation of the oesophagus in fetal rats with oesophageal atresia. Pediatr Surg Int. 1999;15:2–7.

    CAS  PubMed  Google Scholar 

  57. Qi BQ, Merei J, Farmer P, et al. The vagus and recurrent laryngeal nerves in the rodent experimental model of esophageal atresia. J Pediatr Surg. 1997;32:1580–6.

    CAS  PubMed  Google Scholar 

  58. Roman C. Nervous control of peristalsis in the esophagus. J Physiol Paris. 1966;58:79–108.

    CAS  PubMed  Google Scholar 

  59. Shono T, Suita S, Arima T, et al. Motility function of the esophagus before primary anastomosis in esophageal atresia. J Pediatr Surg. 1993;28:673–6.

    CAS  PubMed  Google Scholar 

  60. Haller JA Jr, Brooker AF, Talbert JL, et al. Esophageal function following resection. Studies in newborn puppies. Ann Thorac Surg. 1966;2:180–7.

    PubMed  Google Scholar 

  61. Nakazato Y, Landing BH, Wells TR. Abnormal Auerbach plexus in the esophagus and stomach of patients with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1986;21:831–7.

    CAS  PubMed  Google Scholar 

  62. Montgomery M, Escobar-Billing R, Hellstrom PM, et al. Impaired gastric emptying in children with repaired esophageal atresia: a controlled study. J Pediatr Surg. 1998;33:476–80.

    CAS  PubMed  Google Scholar 

  63. Romeo C, Bonanno N, Baldari S, et al. Gastric motility disorders in patients operated on for esophageal atresia and tracheoesophageal fistula: long-term evaluation. J Pediatr Surg. 2000;35:740–4.

    CAS  PubMed  Google Scholar 

  64. Bokay J, Kis E, Verebely T. Myoelectrical activity of the stomach after surgical correction of esophageal atresia. J Pediatr Surg. 2005;40:1732–6.

    PubMed  Google Scholar 

  65. Cheng W, Spitz L, Milla P. Surface electrogastrography in children with esophageal atresia. Pediatr Surg Int. 1997;12:552–5.

    CAS  PubMed  Google Scholar 

  66. Yagi M, Homma S, Iwafuchi M, et al. Electrogastrography after operative repair of esophageal atresia. Pediatr Surg Int. 1997;12:340–3.

    CAS  PubMed  Google Scholar 

  67. Holschneider P, Dubbers M, Engelskirchen R, et al. Results of the operative treatment of gastroesophageal reflux in childhood with particular focus on patients with esophageal atresia. Eur J Pediatr Surg. 2007;17:163–75.

    CAS  PubMed  Google Scholar 

  68. Michaud L, Sfeir R, Couttenier F, et al. Dumping syndrome after esophageal atresia repair without antireflux surgery. J Pediatr Surg. 2010;45:E13–5.

    PubMed  Google Scholar 

  69. Aumar M, Gottrand F, Chalouhi C, et al. Frequency of abnormal glucose tolerance test suggestive of dumping syndrome following oesophageal atresia repair. J Pediatr Gastroenterol Nutr. 2020;70:820–4.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding authors

Correspondence to Usha Krishnan or Christophe Faure .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Krishnan, U., Righini-Grunder, F., Faure, C. (2022). Esophageal Atresia. In: Faure, C., Thapar, N., Di Lorenzo, C. (eds) Pediatric Neurogastroenterology. Springer, Cham. https://doi.org/10.1007/978-3-031-15229-0_29

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-15229-0_29

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-15228-3

  • Online ISBN: 978-3-031-15229-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics