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Esophageal atresia

Last updated: September 2, 2021

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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.

Overview of types of esophageal atresia
Type A Type B Type C Type D Type E

Description

Epidemiology [1]

  • ∼ 8% of cases
  • ∼ 1% of cases
  • ∼ 87% of cases
  • ∼ 1% of cases
  • ∼ 4% of cases

Clinical features

  • Polyhydramnios
  • Excessive secretions/foaming at the mouth
    • Choking, drooling
    • Inability to feed
X-ray findings
  • Gasless abdomen
  • Esophageal pouch
  • Large gastric bubble
  • Esophageal pouch
  • Large gastric bubble

References:[2][3]

Epidemiological data refers to the US, unless otherwise specified.

References:[5]

Overview of clinical features of types of esophageal atresia
Type A Type B Type C Type D Type E
Polyhydramnios -
Excessive secretions -
Aspiration pneumonia -
Gastric distention - -

Esophageal atresia

  • Definition: a congenital defect in which the upper esophagus is not connected to the lower esophagus and ends blindly instead
  • Prenatal features
  • Postnatal features
    • Pooling of secretions → excessive secretions/foaming at the mouth [2]
      • Choking, drooling
      • Inability to feed
      • Vomiting

Tracheoesophageal fistula

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

  • Placement of a feeding tube: the feeding tube cannot pass through the esophagus in the case of esophageal atresia [2]
  • X-ray of the thorax/abdomen [2]
    • Esophageal pouch (except with an H‑type fistula)
    • Large gastric bubble: air in the stomach (gross types A and B present with a gasless abdomen)
  • Further diagnostics concerning VACTERL anomalies

Double aortic arch

Other

Differential diagnoses of newborn swallowing disorders [2][10]
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

The differential diagnoses listed here are not exhaustive.

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.

  • The goal is to reconnect the upper esophageal pouch and the lower esophagus. [3]
  • A long gap between both ends of the esophagus may not allow primary repair. [2]

Postoperative

  • Uncomplicated surgery: transition to a normal diet after 2–3 days [3]
  • Anastomosis under tension: postoperative ventilation (for approx. 5 days)
  • Radiological examination with a contrast agent (esophagram) one week after surgery to identify complications: e.g., esophageal stricture or anastomotic leak [2]
  • Approx. 4 weeks after the procedure: Gastroscopy (EGD) and dilation of the anastomosis may be necessary.

Overall good prognosis, but surgical complications occur frequently:

  1. Clark DC. Esophageal atresia and tracheoesophageal fistula.. Am Fam Physician. 1999; 59 (4): p.910-6, 919-20.
  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. Facts about Esophageal Atresia. https://www.cdc.gov/ncbddd/birthdefects/esophagealatresia.html#ref. Updated: November 1, 2018. Accessed: November 1, 2019.
  5. Polin RA, Abman SH, Rowitch D, Benitz WE. Fetal and Neonatal Physiology. Elsevier ; 2016
  6. Pardy C, D’Antonio F, Khalil A, Giuliani S. Prenatal detection of esophageal atresia: A systematic review and meta‐analysis. Acta Obstet Gynecol Scand. 2019; 98 (6): p.689-699. doi: 10.1111/aogs.13536 . | Open in Read by QxMD
  7. Clark DC. Esophageal Atresia and Tracheoesophageal Fistula. Am Fam Physician. 1999; 59 (4): p.910-916.
  8. 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.
  9. 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
  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