Journal of Gastrointestinal Surgery

, Volume 21, Issue 6, pp 927–935 | Cite as

Postoperative Complications and Functional Outcome after Esophageal Atresia Repair: Results from Longitudinal Single-Center Follow-Up

  • Florian FriedmacherEmail author
  • Birgit Kroneis
  • Andrea Huber-Zeyringer
  • Peter Schober
  • Holger Till
  • Hugo Sauer
  • Michael E. Höllwarth
Original Article



Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function.


One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3–27 years). Comparative statistics were used to evaluate temporal changes between an early (1975–1989) and late (1990–2011) study period.


Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1–15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett’s esophagus were identified.


Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.


Esophageal atresia Tracheoesophageal fistula Complications Follow-up Outcome Gastroesophageal function 


Author Contributions

Study concept and design: F.F., M.E.H.

Data acquisition, analysis, and interpretation: F.F., B.K., A.H.-Z., P.S., H.T., H.S., M.E.H.

Drafting of the manuscript: F.F., B.K., H.T., M.E.H.

Final approval: F.F., B.K., A.H.-Z., P.S., H.T., H.S, M.E.H.

Agreement to be accountable for all aspects of the work: F.F., B.K., A.H.-Z., P.S., H.T., H.S., M.E.H.

Compliance with Ethical Standards

Author Disclosure Statement

The authors declare that this study was conducted in the absence of any commercial or financial relationships that could be constructed as a potential conflict of interest.


  1. 1.
    Billmyre KK, Hutson M, Klingensmith J. One shall become two: separation of the esophagus and trachea from the common foregut tube. Dev Dyn 2015;244:277–88.CrossRefPubMedGoogle Scholar
  2. 2.
    Nassar N, Leoncini E, Amar E, et al. Prevalence of esophageal atresia among 18 international birth defects surveillance programs. Birth Defects Res A Clin Mol Teratol 2012;94:893–9.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Oddsberg J, Lu Y, Lagergren J. Aspects of esophageal atresia in a population-based setting: incidence, mortality, and cancer risk. Pediatr Surg Int 2012;28:249–57.CrossRefPubMedGoogle Scholar
  4. 4.
    Pedersen RN, Calzolari E, Husby S, Garne E, EUROCAT Working group. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child 2012;97:227–32.CrossRefPubMedGoogle Scholar
  5. 5.
    Conforti A, Morini F, Bagolan P. Difficult esophageal atresia: trick and treat. Semin Pediatr Surg 2014;23:261–9.CrossRefPubMedGoogle Scholar
  6. 6.
    Zani A, Eaton S, Hoellwarth ME, et al. International survey on the management of esophageal atresia. Eur J Pediatr Surg 2014;24:3–8.CrossRefPubMedGoogle Scholar
  7. 7.
    Wang B, Tashiro J, Allan BJ, et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res 2014;190:604–12.CrossRefPubMedGoogle Scholar
  8. 8.
    Sulkowski JP, Cooper JN, Lopez JJ, et al. Morbidity and mortality in patients with esophageal atresia. Surgery 2014;156:483–91.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Sfeir R, Bonnard A, Khen-Dunlop N, et al. Esophageal atresia: data from a national cohort. J Pediatr Surg 2013;48:1664–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Koivusalo AI, Pakarinen MP, Rintala RJ. Modern outcomes of oesophageal atresia: single centre experience over the last twenty years. J Pediatr Surg 2013;48:297–303.CrossRefPubMedGoogle Scholar
  11. 11.
    Malakounides G, Lyon P, Cross K, et al. Esophageal atresia: improved outcome in high-risk groups revisited. Eur J Pediatr Surg 2016;26:227–31.CrossRefPubMedGoogle Scholar
  12. 12.
    Orford J, Cass DT, Glasson MJ. Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int 2004;20:402–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Dingemann C, Dietrich J, Zeidler J, et al. Early complications after esophageal atresia repair: analysis of a German health insurance database covering a population of 8 million. Dis Esophagus. 2016;29:780–6.CrossRefPubMedGoogle Scholar
  14. 14.
    Schneider A, Blanc S, Bonnard A, et al. Results from the French National Esophageal Atresia register: one-year outcome. Orphanet J Rare Dis 2014;9:206.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Alshehri A, Lo A, Baird R. An analysis of early nonmortality outcome prediction in esophageal atresia. J Pediatr Surg 2012;47:881–4.CrossRefPubMedGoogle Scholar
  16. 16.
    Rintala RJ, Pakarinen MP. Long-term outcome of esophageal anastomosis. Eur J Pediatr Surg 2013;23:219–25.CrossRefPubMedGoogle Scholar
  17. 17.
    Ijsselstijn H, van Beelen NW, Wijnen RM. Esophageal atresia: long-term morbidities in adolescence and adulthood. Dis Esophagus 2013;26:417–21.CrossRefPubMedGoogle Scholar
  18. 18.
    Legrand C, Michaud L, Salleron J, et al. Long-term outcome of children with oesophageal atresia type III. Arch Dis Child 2012;97:808–11.CrossRefPubMedGoogle Scholar
  19. 19.
    Connor MJ, Springford LR, Kapetanakis VV, Giuliani S. 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.CrossRefPubMedGoogle Scholar
  20. 20.
    Shah R, Varjavandi V, Krishnan U. Predictive factors for complications in children with esophageal atresia and tracheoesophageal fistula. Dis Esophagus 2015;28:216–23.CrossRefPubMedGoogle Scholar
  21. 21.
    Pedersen RN, Markøw S, Kruse-Andersen S, et al. Esophageal atresia: gastroesophageal functional follow-up in 5–15 year old children. J Pediatr Surg 2013;48:2487–95.CrossRefPubMedGoogle Scholar
  22. 22.
    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.CrossRefPubMedGoogle Scholar
  23. 23.
    Höllwarth ME, Zaupa P. Oesophageal atresia. In Puri P, Höllwarth ME (eds.). Pediatric surgery. 1st edn. Heidelberg, Berlin, New York: Springer; 2006:29--48.Google Scholar
  24. 24.
    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.CrossRefPubMedGoogle Scholar
  25. 25.
    Low DE, Alderson D, Cecconello I, et al. International Consensus on Standardization of Data Collection for Complications Associated With Esophagectomy: Esophagectomy Complications Consensus Group (ECCG). Ann Surg 2015;262:286–94.CrossRefPubMedGoogle Scholar
  26. 26.
    Vandenplas Y, Rudolph CD, Di Lorenzo C, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). J Pediatr Gastroenterol Nutr 2009;49:498–547.CrossRefPubMedGoogle Scholar
  27. 27.
    Maynard S, Bouin M. Follow-up of adult patients with repaired esophageal atresia: how, when, and for how long? Dis Esophagus 2013;26:422–4.CrossRefPubMedGoogle Scholar
  28. 28.
    Burge DM, Shah K, Spark P, et al. Contemporary management and outcomes for infants born with oesophageal atresia. Br J Surg 2013;100:515–21.CrossRefPubMedGoogle Scholar
  29. 29.
    Mortell AE, Azizkhan RG. Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg 2009;18:12–9.CrossRefPubMedGoogle Scholar
  30. 30.
    Koivusalo AI, Pakarinen MP, Lindahl HG, Rintala RJ. Revisional surgery for recurrent tracheoesophageal fistula and anastomotic complications after repair of esophageal atresia in 258 infants. J Pediatr Surg 2015;50:250–4.CrossRefPubMedGoogle Scholar
  31. 31.
    Coran AG. Diagnosis and surgical management of recurrent tracheoesophageal fistula. Dis Esophagus 2013;26:380–1.CrossRefPubMedGoogle Scholar
  32. 32.
    Zhu H, Shen C, Xiao X, Dong K, Zheng S. Reoperation for anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2015,50:2012–5.CrossRefPubMedGoogle Scholar
  33. 33.
    Guo W, Li Y, Jiao A, Peng Y, Hou D, Chen Y. Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula. J Pediatr Surg 2010;45:2351–5.CrossRefPubMedGoogle Scholar
  34. 34.
    Lévesque D, Baird R, Laberge JM. Refractory strictures post-esophageal atresia repair: what are the alternatives? Dis Esophagus 2013;26:382–7.CrossRefPubMedGoogle Scholar
  35. 35.
    Thyoka M, Barnacle A, Chippington S, et al. Fluoroscopic balloon dilation of esophageal atresia anastomotic strictures in children and young adults: single-center study of 103 consecutive patients from 1999 to 2011. Radiology 2014;271:596–601.CrossRefPubMedGoogle Scholar
  36. 36.
    Höllwarth ME. Gastroesophageal reflux disease. In: Coran AG, Adzick NS, Krummel TM, Laberge JM, Shamberger R, Caldamone AA (eds.). Pediatric surgery. Vol. 2, 7th edn. Philadelphia: Elsevier Sauders; 2012:947–58.CrossRefGoogle Scholar
  37. 37.
    Pini Prato A, Carlucci M, Bagolan P, et al. A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2015;50:1441–56.CrossRefPubMedGoogle Scholar
  38. 38.
    Koivusalo AI, Pakarinen MP, Lindahl HG, Rintala RJ. Endoscopic surveillance after repair of oesophageal atresia—longitudinal study in 209 patients. J Pediatr Gastroenterol Nutr 2016;62:562–6.CrossRefPubMedGoogle Scholar
  39. 39.
    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.CrossRefPubMedGoogle Scholar
  40. 40.
    Zamiara P, Thomas KE, Connolly BL, Lane H, Marcon MA, Chiu PP. Long-term burden of care and radiation exposure in survivors of esophageal atresia. J Pediatr Surg 2015;50:1686–90.CrossRefPubMedGoogle Scholar
  41. 41.
    Lacher M, Froehlich S, von Schweinitz D, Dietz HG. Early and long term outcome in children with esophageal atresia treated over the last 22 years. Klin Padiatr 2010;222:296–301.CrossRefPubMedGoogle Scholar
  42. 42.
    Friedmacher F, Puri P. Delayed primary anastomosis for management of long-gap esophageal atresia: a meta-analysis of complications and long-term outcome. Pediatr Surg Int 2012;28:899–906.CrossRefPubMedGoogle Scholar
  43. 43.
    Dingemann C, Meyer A, Kircher G, et al. Long-term health-related quality of life after complex and/or complicated esophageal atresia in adults and children registered in a German patient support group. J Pediatr Surg 2014;49:631–8.CrossRefPubMedGoogle Scholar
  44. 44.
    Burford JM, Dassinger MS, Copeland DR, Keller JE, Smith SD. Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series. Am J Surg 2011;202:203–6.CrossRefPubMedGoogle Scholar
  45. 45.
    Laberge JM, Blair GK. Thoracotomy for repair of esophageal atresia: not as bad as they want you to think! Dis Esophagus 2013;26:365–71.CrossRefPubMedGoogle Scholar
  46. 46.
    Pierro A. Hypercapnia and acidosis during the thoracoscopic repair of oesophageal atresia and congenital diaphragmatic hernia. J Pediatr Surg 2015;50:247–9.CrossRefPubMedGoogle Scholar

Copyright information

© The Society for Surgery of the Alimentary Tract 2017

Authors and Affiliations

  • Florian Friedmacher
    • 1
    Email author
  • Birgit Kroneis
    • 1
  • Andrea Huber-Zeyringer
    • 1
  • Peter Schober
    • 1
  • Holger Till
    • 1
  • Hugo Sauer
    • 1
  • Michael E. Höllwarth
    • 1
  1. 1.Department of Pediatric and Adolescent SurgeryMedical University of GrazGrazAustria

Personalised recommendations