Clinical Course of Tracheomalacia in Children with Esophageal Atresia/Tracheoesophageal Fistula

Introduction: Tracheomalacia (TM) commonly complicates esophageal atresia +/- tracheoesophageal atresia (EA/TEF). Yet, there is limited investigation of the long-term outcomes across a population of EA/TEF patients with TM concerns. Therefore, this study aims to investigate the natural course, management, and outcomes of TM in children with EA/TEF.

Methods: A retrospective chart review of pediatric patients with TM and EA/TEF was conducted at a single tertiary academic medical center from 2006 to 2024. Data were collected from the electronic health record and stored in a REDCap database, encompassing demographics, TM severity, symptoms, management, urgent care and/or emergency department visits, and outcomes.

Results: Data from 35 patients were included. The cohort consisted of fifteen females (43%) and 20 males (57%). Mean age at TM diagnosis was 18 months. Based on endoscopy, TM severity was rated as “mild” for ten patients (29%), “moderate” for 9 (26%), “severe” for 9 (26%), and unknown for 7 (20%). Four patients (11%) experienced recurrent pneumonia, 22 (63%) had chronic cough, 16 (46%) had aspiration events, and 2 (6%) had no symptoms. Nine children (26%) required a tracheostomy, 9 (26%) required long-term outpatient CPAP, and 9 (26%) received surgery for their TM. Twenty-five children (71%) required surveillance operations for their TM. Twelve children (48%) presented to the emergency department due to a complication of TM, 11 of which required hospital admission following at least one of these visits. Four children in the study are now deceased, 2 of which died due to complications of TM.

Conclusions: The study highlights the significant health care burden of tracheomalacia in children with EA/TEF, including the need for frequent medical interventions, long-term respiratory support, and substantial morbidity. While the findings emphasize the variability in disease severity and outcomes, they underscore the importance of surveillance over time and tailored management.