Laryngotracheal anomalies associated with esophageal atresia: importance of early diagnosis.

ABSTRACT

OBJECTIVE: Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. STUDY DESIGN: Retrospective study. MATERIALS AND METHODS: Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery. RESULTS: One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2%) and other laryngotracheal anomalies in 43 patients (27.2%). Ninety-six patients (60.7%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22%). CONCLUSION: Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.