Vascular and pulmonary comorbidities in children with congenital EA/TEF

Pediatr Pulmonol. 2021 Feb;56(2):571-577. doi: 10.1002/ppul.25219. Epub 2020 Dec 23.


Background: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is associated with many congenital and vascular malformations; however, reports utilizing computed tomography (CT) and computed tomography angiography (CTA) are limited. The objective of this study is to review CT scans of the chest from patients with EA/TEF and report their pulmonary and vascular findings.

Methods: We completed a retrospective chart review of children with congenital EA/TEF evaluated in the aerodigestive clinic at Children's Hospital Colorado. Results of the most recent CTA or CT of the chest were investigated. Demographics, medical conditions, and bronchoscopy findings were also recorded. The ratio of tracheal lumen area between inspiratory and expiratory CTA images was measured.

Results: Of the patients with congenital EA/TEF seen in the program, 47 patients had a chest CT available for review. Eight patients (17%) had bronchiectasis. Of the contrast CT scans, 15 (58%) had a vascular abnormality and 16 (62%) demonstrated tracheal compression (38% at the level of the innominate artery, 35% from other structures). Nineteen of the CTAs had volumetric expiratory images of the trachea to evaluate tracheomalacia. The mean expiratory:inspiratory area was 0.57 (SD ± 0.23) at the level of the innominate.

Conclusion: Patients with EA/TEF frequently have vascular abnormalities that may alter airway mechanics as well as pulmonary comorbidities that may affect long-term management. For patients experiencing persistent respiratory symptoms, CTA of the chest should be considered adjunct to bronchoscopy to help with medical and surgical management of these children.

Keywords: bronchoscopy; pulmonary Imaging; tracheoesophageal fistula; tracheomalacia.

MeSH terms

  • Bronchiectasis / diagnostic imaging
  • Bronchiectasis / epidemiology*
  • Bronchoscopy
  • Child
  • Child, Preschool
  • Comorbidity
  • Esophageal Atresia / diagnostic imaging
  • Esophageal Atresia / epidemiology*
  • Female
  • Humans
  • Lung / diagnostic imaging
  • Male
  • Tomography, X-Ray Computed
  • Trachea / diagnostic imaging
  • Tracheoesophageal Fistula / diagnostic imaging
  • Tracheoesophageal Fistula / epidemiology*
  • Vascular Diseases / diagnostic imaging
  • Vascular Diseases / epidemiology*