Care Recommendations for the Respiratory Complications of Esophageal Atresia-Tracheoesophageal Fistula: The International Network of Esophageal Atresia, Respiratory Complications Working Group

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, 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. Non-invasive 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.

Long‐term respiratory complications of OA–TOF: The need for better data and a more specialized multidisciplinary approach

“…For a birth defect that is characterized by such morbidity surprisingly few robust multicenter data exist including care pathway guidelines. As Koumbourlis et al 2 discuss, most current data emerge from single‐center experience and, here, we agree there is a crucial need for larger‐scale collaborative studies. This manuscript is therefore timely in Pediatric Pulmonology as a “work in progress” consensus on the utility of diagnostic studies and therapeutics addressing respiratory complications of OA–TOF patients.…”

“…This current manuscript by Koumbourlis et al 2 in Pediatric Pulmonology is a step forward in the right direction towards consensus‐based management of respiratory health in OA–TOF children. We now need to work together as healthcare professionals to establish core outcomes, collate national, and international data (with registries) and ensure these special children receive the best care possible for a long and healthy life.…”

“…No matter what surgical technique(s) are deployed even with primary repair of the esophagus where anatomical correction is achieved, significant functional abnormality(s) remain thereafter for patients. There is a multitude of factors to consider which are herein discussed in the paper by Koumbourlis et al 2 in this issue of Pediatric Pulmonology. These functional abnormalities can cause life‐long morbidity and impact patients and families in many different ways.…”