INTRODUCTION
Congenital tracheoesophageal fistula (TEF) is almost always (92% of cases) associated with esophageal atresia (EA) (commonly referred to as EA-TEF)1 with an estimated incidence of approximately 1 in 3500-4500 live births 2,3.
The trachea and the esophagus share a common embryologic origin from an outgrowth of the foregut, the “lung bud”, that appears around the fourth week of gestation. By the end of the fifth week a septum divides it in two parts and it separates from the foregut 4. The TEF is caused by a defect in the septum that preserves the communication between the esophagus and the lower trachea. Rare variants include a communication between the esophagus and the right main stem bronchus or the connection of the trachea and esophagus at proximal and distal sites by two or even three fistulae5.
Repair of the TEF consists of the separation of the esophagus from the trachea and closure of the defect of the tracheal wall. There is usually a visible “ridge” at the site of the repair and often a blind pouch that can be intermittently open (Image 1A). The repair of the esophagus is far more complex, particularly if there is a long gap between the two atretic segments6. Despite the repair of the defect, the condition carries a very high morbidity primarily associated with the presence of severe tracheomalacia (TM) or tracheobronchomalacia (TBM). The complications of TEF are most prevalent and severe during the first few years of life but may last in varying degrees throughout life7–18.
Improvement in the survival of infants born with EA-TEF has highlighted the need to optimally manage their symptoms, reduce morbidity, and improve quality of life. The International Network on Esophageal Atresia (INoEA), comprised of health professionals involved in the care of children with EA-TEF, published guidelines for the evaluation and treatment of gastrointestinal and nutritional complications in EA-TEF and on the surgical management of long-gap EA6,19,20. The Respiratory Complications Working Group (RCWG) was formed at the invitation of the INoEA Steering Committee, to develop a similar framework for the management of the respiratory complications of TEF. The RCWG consists of 10 academic Pediatric Pulmonologists from Canada, Italy, Australia, and the USA, with experience in the diagnosis and management of infants and children with EA-TEF.
The document is intended for all health care professionals who are involved in the care of patients with EA-TEF in or outside the hospital setting. It may also be useful for the families of patients with EA-TEF in understanding the rationale behind the multiple diagnostic and therapeutic interventions that may be necessary throughout childhood and adolescence.