Neonatology pp 1281-1294 | Cite as

Esophageal Atresia of Newborns

  • Mario MessinaEmail author
  • Francesco Molinaro
  • Alfredo Garzi
  • Rossella Angotti
Reference work entry


Esophageal atresia includes a group of congenital anomalies characterized as interruption of the continuity of the esophagus combined with or without a persistent communication with the trachea. These congenital malformations occur in 1:2,500–3,000 live births.

Over the last 40 years, there has been a steady improvement in the overall survival rate due to early diagnosis and prompt referral, improvements in preoperative care and diagnosis and treatment of associated anomalies, advances in anesthetic techniques, and sophisticated neonatal intensive care.

There is increasing experience with the thoracoscopic approach to repair esophageal atresia (Brandigi et al., J Siena Acad Sci 5, 2013; Bax and van Der Zee, J Pediatr Surg 37: 192–196, 2002; Rothenberg, J Pediatr Surg 37: 869–872, 2002; Nguyen et al., J Laparoendosc Adv Surg Tech A 16: 174–178, 2006; Krosnar and Baxter, Paediatr Anaesth 15: 541–546, 2005; Rothenberg, Semin Pediatr Surg 14: 2–7, 2005a; Rothenberg, Esophageal atresia and tracheoesophageal fistula. In: Najmaldin et al. (eds) Operative endoscopy and endoscopic surgery in infants and children. Hodder Arnold, London, pp 89–97, 2005b), but this requires advanced skills in minimal invasive surgery.


  1. Agrawal L, Beardsmore CS, MacFadyen UM (1999) Respiratory function in childhood following repair of oesophageal atresia and tracheoesophageal fistula. Arch Dis Child 81:404–408CrossRefGoogle Scholar
  2. Babu R, Pierro A, Spitz L et al (2000) The management of esophageal atresia in neonates with right-sided aortic arch. J Pediatr Surg 35:56–58CrossRefGoogle Scholar
  3. Bax KM, van Der Zee DC (2002) Feasibility of thoracoscopic repair of esophageal atresia with distal fistula. J Pediatr Surg 37:192–196CrossRefGoogle Scholar
  4. Bax NM, van der Zee DC (2007) Jejunal pedicle grafts for reconstruction of the esophagus in children. J Pediatr Surg 42:363–369CrossRefGoogle Scholar
  5. Bianchi A, Sowande O, Alizai NK, Rampersad B (1998) Aesthetics and lateral thoracotomy in the neonate. J Pediatr Surg 33:1798–1800CrossRefGoogle Scholar
  6. Brandigi E, Molinaro F et al (2013) Thoracoscopic repair of esophageal atresia with distal fistula in a new born: a technical case report. J Siena Acad Sci 5. Published Since 1761:85–88CrossRefGoogle Scholar
  7. Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ (1980) Esophageal atresia and associated anomalies. Arch Dis Child 64:364–368CrossRefGoogle Scholar
  8. Cloud DT (1968) Anastomotic technique in esophageal atresia. J Pediatr Surg 3:561–564CrossRefGoogle Scholar
  9. Corbally MT, Spitz L, Kiely E et al (1993) Aortopexy for tracheomalacia in oesophageal anomalies. Eur J Pediatr Surg 3:264–266CrossRefGoogle Scholar
  10. Crisera CA, Connelly PR, Marmureanu AR et al (1999) Esophageal atresia with tracheoesophageal fistula: suggested mechanism in faulty organogenesis. J Pediatr Surg 34:204–208CrossRefGoogle Scholar
  11. Digilio MC, Marino B, Bagolan P et al (1999) Microdeletion 22q11 and esophageal atresia. J Med Genet 36:137–139PubMedPubMedCentralGoogle Scholar
  12. Driver CP, Shankar KR, Jones MO et al (2001) Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification. J Pediatr Surg 36:1419–1421CrossRefGoogle Scholar
  13. Duranceau A, Fisher SR, Flye M (1977) Motor function of the esophagus after repair of esophageal atresia and tracheoesophageal fistula. Surgery 82:116–123PubMedGoogle Scholar
  14. Filler RM, Messineo A, Vinograd L (1992) Severe tracheomalacia associated with esophageal atresia: results of surgical treatment. J Pediatr Surg 27:1136–1140CrossRefGoogle Scholar
  15. Filston HC, Chitwood WR Jr, Schkolne B, Blackmon LR (1982a) The Fogarty balloon catheter as an aid to management of the infant with esophageal atresia and tracheoesophageal fistula complicated by severe RDS or pneumonia. J Pediatr Surg 17:149–151CrossRefGoogle Scholar
  16. Filston HC, Rankin JS, Kirks DR (1982b) The diagnosis of primary and recurrent tracheoesophageal fistulas: value of selective catheterization. J Pediatr Surg 17:144–148CrossRefGoogle Scholar
  17. Hagberg S, Rubcnson A, Sillcn U, Werkmaster K (1986) Management of long-gap esophagus: experience with end-to-end anastomosis under maximal tension. Prog Pediatr Surg 19:88–92CrossRefGoogle Scholar
  18. Holmes SJK, Kiely EM, Spitz L (1987) Tracheoesophageal fistula and the respiratory distress syndrome. Pediatr Surg Int 2:16–18CrossRefGoogle Scholar
  19. Kluth D (1976) Atlas of esophageal atresia. J Pediatr Surg 11:901–919CrossRefGoogle Scholar
  20. Krosnar S, Baxter A (2005) Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: anesthetic and intensive care management of a series of eight neonates. Paediatr Anaesth 15:541–546CrossRefGoogle Scholar
  21. Langer JC, Hussain H, Khan A et al (2001) prenatal diagnosis of esophageal atresia using sonography and magnetic resonance imaging. J Pediatr Surg 36:804–807CrossRefGoogle Scholar
  22. Litingtung Y, Lei L, Westphal H, Chiang C (1998) Sonic hedgehog is essential to foregut development. Nat Genet 20:58–61CrossRefGoogle Scholar
  23. Livitidis A (1973) Esophageal atresia: a method of overbrindging large segment gaps. Z Kinderchir 13:298–306Google Scholar
  24. Lopez PJ, Keys C, Pierro A et al (2006) Oesophageal atresia: improved outcome in high-risk groups? J Pediatr Surg 41:331–334CrossRefGoogle Scholar
  25. Marsh AJ, Wellesley D, Burge D et al (2000) Interstitial deletion of chromosome 17 (del(l7)(q22q23.3)) confirms a link with oesophageal atresia. J Med Genet 37:701–704CrossRefGoogle Scholar
  26. Mee RBB, Beasley SW, Auldist AW, Myers NA (1992) Influence of congenital heart disease on management of oesophageal atresia. Pediatr Surg Int 7:90–93CrossRefGoogle Scholar
  27. Merci J, Hasthorpe S, Farmer P, Hutson JM (1998) relationship between esophageal atresia with tracheoesophageal fistula and vertebral anomalies in mammalian embryos. J Pediatr Surg 33:58–63CrossRefGoogle Scholar
  28. Nguyen T, Zainabadi K, Bui T et al (2006) Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: lessons learned. J Laparoendosc Adv Surg Tech A 16:174–178CrossRefGoogle Scholar
  29. Parker AF, Christie DL, Cahill JL (1979) Incidence and significance of gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula and the need for anti-reflux procedures. J Pediatr Surg 14:5–8CrossRefGoogle Scholar
  30. Poenaru D, Laberge JM, Neilson JR, Guttman FM (1993) A new prognostic classification for esophageal atresia. Surgery 113:426–432PubMedGoogle Scholar
  31. Puri P, Blake N, O’Donnell B, Guiney EJ (1981) Delayed primary anastomosis following spontaneous growth of esophageal segments in esophageal atresia. J Pediatr Surg 16:180–183CrossRefGoogle Scholar
  32. Quan L, Smith DW (1972) The VATER association: vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial dysplasia. Birth Defects 8:75–78Google Scholar
  33. Roessler E, Belloni E, Gaudenz K et al (1996) Mutations in the human sonic hedgehog gene cause holoprosencephaly. Nat Genet 14:357–360CrossRefGoogle Scholar
  34. Rothenberg SS (2002) Thoracoscopic repair of tracheoesophageal fistula in newborns. J Pediatr Surg 37:869–872CrossRefGoogle Scholar
  35. Rothenberg SS (2005a) Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula. Semin Pediatr Surg 14:2–7CrossRefGoogle Scholar
  36. Rothenberg SS (2005b) Esophageal atresia and tracheoesophageal fistula. In: Najmaldin A, Rothenberg S, Crabbe D, Beasley S (eds) Operative endoscopy and endoscopic surgery in infants and children. Hodder Arnold, London, pp 89–97CrossRefGoogle Scholar
  37. Shulman A, Mazkereth R, Zalel Y et al (2002) Prenatal identification of esophageal atresia: the role of ultrasonography for evaluation of functional anatomy. Prenat Diagn 22:669–674CrossRefGoogle Scholar
  38. Spitz L (1973) Congenital esophageal stenosis distal to associated esophageal atresia. J Pediatr Surg 8:973–974CrossRefGoogle Scholar
  39. Spitz L (1996) Esophageal atresia: past, present, and future. J Pediatr Surg 31:19–25CrossRefGoogle Scholar
  40. Spitz L, Kiely E, Brereton RJ, Drake D (1993) Management of esophageal atresia. World J Surg 17:296–300CrossRefGoogle Scholar
  41. Spitz L, Kiely EM, Morecrofl JA, Drake DP (1994) Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg 29:723–725CrossRefGoogle Scholar
  42. Spitz L, Kiely E, Pierro A (2004) Gastric transposition in children a 21-year experience. J Pediatr Surg 39:276–281CrossRefGoogle Scholar
  43. Stringer MD, McKenna KM, Goldstein RB et al (1995) Prenatal diagnosis of esophageal atresia. J Pediatr Surg 30:l258–l1263Google Scholar
  44. Yeung CK, Spitz L, Brereton RJ (1992) Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia. J Pediatr Surg 27:852–855CrossRefGoogle Scholar
  45. Zhou B, Hutson JM, Farmer PJ et al (1999) Apoptosis in tracheoesophageal embryogenesis in rat embryos with or without adriamycin treatment. J Pediatr Surg 34:872–875CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Mario Messina
    • 1
    • 2
    Email author
  • Francesco Molinaro
    • 1
    • 2
  • Alfredo Garzi
    • 2
    • 3
  • Rossella Angotti
    • 4
  1. 1.Department of Pediatrics, Obstetrics and Reproductive Medicine, Section of Pediatric SurgeryPoliclinico “Le Scotte”, University of SienaSienaItaly
  2. 2.Department of Medical, Surgical and Neurological SciencesSection of Pediatric Surgery, University of SienaSienaItaly
  3. 3.Università degli Studi di SalernoFiscianoItaly
  4. 4.Department of Pediatrics, Obstetrics and Reproductive Medicine, Section of Pediatric SurgeryUniversity of SienaSienaItaly

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