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.