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Esophageal Atresia and Tracheoesophageal Fistula


KEY FACTS

Terminology

  • Atresia: Congenital occlusion of lumen

  • Fistula: Anomalous connection between 2 lumens

Imaging

  • 5 major anatomic variations of esophageal atresia-tracheoesophageal fistula (EA-TEF)

  • Fistula level variable depending on type of EA-TEF

    • Most commonly above/near carina

  • Atretic segments variable in length

    • Gap often long in EA without TEF

  • Radiographs

    • Air-distended upper esophageal pouch

    • Enteric tube tip near thoracic inlet in pouch

    • EA with TEF: Gas in stomach & bowel

    • EA without TEF: Gasless stomach & bowel

  • Limited indications for preoperative esophagram (except isolated TEF)

  • Postoperative esophagram

    • Esophageal anastomotic leak, anastomotic stricture, recurrent/additional TEF, esophageal dysmotility, gastroesophageal reflux

Top Differential Diagnoses

  • Tube malposition

  • Laryngotracheal cleft

  • Esophageal strictures of various etiologies

Clinical Issues

  • Presentation: Excessive oral secretions, cyanosis, choking, coughing during feeding, recurrent pneumonia; nasogastric tube fails to reach stomach

    • 47-75% have associated anomalies

  • Treatment: Bronchoscopy, esophagoscopy to visualize fistula(s) + extrapleural ligation of fistula + anastomosis of esophageal segments; may require staged surgeries

  • Postsurgical survival: 75-95% (depends on associated cardiac anomalies, birth weight)

Graphic shows the Gross classification of esophageal atresia with tracheoesophageal fistula (EA-TEF), including the isolated TEF without EA (H-type fistula, type E) & the isolated EA without TEF (type A).

Sagittal T2 SSFSE MR of a 26-week gestation fetus imaged for polyhydramnios shows a collection of fluid in the region of the upper esophagus
that is suggestive of an atretic esophageal pouch, which was confirmed during surgery in the newborn period.

AP radiograph of a premature newborn boy with a history of not handling secretions shows a nasogastric tube (NGT) tip
overlying the thoracic inlet due to EA. The bowel gas confirms the presence of a distal TEF. The cardiomegaly suggests congenital heart disease.

AP chest radiograph in a newborn shows an NGT coiled at the thoracic inlet
, suggesting EA. Note the lack of gas in the upper abdomen, which is typical of EA without a distal TEF.

TERMINOLOGY

Definitions

  • Esophageal atresia (EA): Congenital occlusion of upper esophagus

  • Tracheoesophageal fistula (TEF): Single (less commonly multiple) anomalous congenital connection(s) from esophagus to trachea

IMAGING

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