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Esophageal atresia

Last updated: January 7, 2021

Summary

Esophageal atresia is a congenital defect in which the upper esophagus is not connected to the lower esophagus, ending blindly instead. It is caused by the abnormal development of the tracheoesophageal septum. Esophageal atresia with a fistula connected distally to the trachea is the most common kind of esophageal malformation (classified as Gross type C). It manifests immediately after birth with cyanotic attacks, foaming at the mouth, and coughing, and prevents any attempts to pass a feeding tube into the stomach. X‑ray is mandatory for classifying the atresia and should show an air‑filled pouch situated at the level of the third thoracic vertebra. Infants with suspected esophageal atresia cannot be fed orally because of the risk of aspiration pneumonia. Curative surgery must, therefore, be performed within the first 24 hours after birth.

Epidemiology

  • Incidence: approx. 1:4,300 live births in the US [1]

Epidemiological data refers to the US, unless otherwise specified.

Classification

Types Description Percentage
Type A
  • ∼ 8% of cases
Type B
  • ∼ 3% of cases
Type C
  • ∼ 84% of cases
Type D
  • ∼ 1% of cases
Type E
  • ∼ 4% of cases

References:[2][3]

Pathophysiology

References:[4]

Clinical features

Prenatal

Postnatal

Newborns usually present with symptoms directly after birth! The exception is the Gross type E fistula: The diagnosis of a small H‑type tracheoesophageal fistula may occur as late as adulthood.

References:[2][3][5][6][7]

Diagnostics


References:[2]

Differential diagnoses

Double aortic arch

Other

Differential diagnoses of newborn swallowing disorders
Differential diagnosis Findings
Esophageal atresia
Status post C‑section
  • Excessive secretions
  • Reversible condition, as opposed to esophageal atresia
Choanal atresia
  • Cyanotic attacks
  • Attacks normalize after crying or opening the mouth
Esophageal stenosis
  • Delayed diagnosis (after introduction of solid food)
  • Dysphagia
  • Regurgitation
Achalasia
  • Very rare during childhood
  • Delayed diagnosis (after introduction of solid food)
  • Dysphagia
  • Regurgitation
Defective swallowing reflex



References:[2][10][11][12]

The differential diagnoses listed here are not exhaustive.

Treatment

Preoperative

Infants who potentially have esophageal atresia should not be fed orally under any circumstances!

Surgery

Surgical treatment should be performed within the first 24 hours of birth.

Postoperative

References:[2][3][5]

Prognosis

Overall good prognosis, but surgical complications occur frequently:

References

  1. Facts about Esophageal Atresia. https://www.cdc.gov/ncbddd/birthdefects/esophagealatresia.html#ref. Updated: November 1, 2018. Accessed: November 1, 2019.
  2. Puri P, Höllwarth M. Pediatric Surgery: Diagnosis and Management. Springer ; 2009
  3. Pinheiro PF, Simões e silva AC, Pereira RM. Current knowledge on esophageal atresia. World J Gastroenterol. 2012; 18 (28): p.3662-3672. doi: 10.3748/wjg.v18.i28.3662 . | Open in Read by QxMD
  4. Polin RA, Abman SH, Rowitch D, Benitz WE. Fetal and Neonatal Physiology. Elsevier ; 2016
  5. Clark DC. Esophageal Atresia and Tracheoesophageal Fistula. Am Fam Physician. 1999; 59 (4): p.910-916.
  6. Pretorius DH, Drose JA, Dennis MA, Manchester DK, Manco-johnson ML. Tracheoesophageal fistula in utero. Twenty-two cases. J Ultrasound Med. 1987; 6 (9): p.509-513.
  7. Zacharias J, Genc O, Goldstraw P. Congenital tracheoesophageal fistulas presenting in adults: presentation of two cases and a synopsis of the literature. J Thorac Cardiovasc Surg. 2004; 128 (2): p.316-318. doi: 10.1016/j.jtcvs.2003.12.046 . | Open in Read by QxMD
  8. Shah RK, Mora BN, Bacha E, et al. The presentation and management of vascular rings: An otolaryngology perspective. Int J Pediatr Otorhinolaryngol. 2007; 71 (1): p.57-62. doi: 10.1016/j.ijporl.2006.08.025 . | Open in Read by QxMD
  9. Bhimji S. Vascular Rings Clinical Presentation. In: Talavera F, Schwartz DA, Milliken JC, Vascular Rings Clinical Presentation. New York, NY: WebMD. https://emedicine.medscape.com/article/426233-clinical. Updated: February 21, 2017. Accessed: January 31, 2019.
  10. Serrao E, Santos A, Gaivao A, Tavares A, Ferreira S. Congenital esophageal stenosis: a rare case of dysphagia. J Radiol Case Rep. 2010; 4 (6): p.8-14. doi: 10.3941/jrcr.v4i6.422 . | Open in Read by QxMD
  11. Spechler SJ. Achalasia: Pathogenesis, clinical manifestations, and diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/achalasia-pathogenesis-clinical-manifestations-and-diagnosis.Last updated: November 16, 2016. Accessed: December 22, 2016.
  12. Jadcherla SR. Neonatal Oral Feeding Difficulties due to Sucking and Swallowing Disorders. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/sucking-and-swallowing-disorders-in-the-newborn.Last updated: July 15, 2015. Accessed: January 24, 2017.