Care recommendations for the respiratory complications of esophageal atresia‐tracheoesophageal fistula

Abstract

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on “strong”, “moderate” or “weak” agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long‐term follow‐up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients’ age were also developed.

Noncystic fibrosis bronchiectasis in children and adolescents: Follow‐up over a decade

“…The result is likely to refer to the persistent airway inflammation and bronchial hyperreactivity that can remain after an infectious airway episode 27 . As expected, GER was detected more frequently in EA ( ± TEF) group due to the well‐known esophageal dysmotility that contributes to the respiratory morbidity in this population 14 …”

“…27 As expected, GER was detected more frequently in EA ( ± TEF) group due to the well-known esophageal dysmotility that contributes to the respiratory morbidity in this population. 14 Focusing on type and localization of bronchiectasis, the cylindrical-the mildest form of bronchiectasis-was the most frequent type affecting significantly PCD and postinfectious groups.…”

Quantifying Upper Aerodigestive Sequelae in Esophageal Atresia/Tracheoesophageal Fistula Neonates

“…Tracheomalacia has been shown to contribute to most other respiratory complications (cyanotic spells, recurrent respiratory tract infections, impaired airway clearance, bronchiectasis, aspiration, and gastroesophageal dysmotility) seen in patients with EA/TEF 4 . Early diagnosis and management of tracheomalacia are therefore important in minimizing a patient's complication burden.…”

“…Aerodigestive complications are common and often most severe during the first years of life. However, initial diagnosis or persistence of disease can occur throughout adulthood 1,2,4–9 …”

“…laryngeal clefts), iatrogenic causes (e.g. recurrent laryngeal nerve injury), and functional abnormalities 4 . While many patients are treated with conservative measures, some will require additional diagnostic direct laryngoscopy and/or bronchoscopy (DLB) with or without intervention, tracheostomy, esophageal dilation, and major operations like fundoplication, gastrostomy tube placement, aortopexy, laryngotracheoplasty, or EA/TEF revision 1,10,11 .…”