O2191: Long term pulmonary outcomes for esophageal atresia with distal tracheoesophageal fistula: A Midwest Pediatric Surgery Consortium study

Background: Little data exist on long term airway, pulmonary, and vocal cord complications for infants born with Type C esophageal atresia with tracheoesophageal fistulas (EA/TEF). The aim of this study was to characterize long term pulmonary and respiratory outcomes within a cohort of EA/TEF patients.

Methods: This is a secondary analysis of prospectively collected data from a multi-institutional quality improvement initiative implementing a standardized management protocol for EA/TEF patients across 11 institutions from January 2016 through January 2019. This analysis focuses on long term pulmonary and respiratory outcomes including tracheomalacia, pulmonary complications, and vocal cord impairments.

Results: After excluding 12 deaths during the follow up period, 153 patients were included and reviewed at 5-8 years of age. Tracheomalacia was diagnosed in 56% (n=86) at any point during their follow-up, and of those, 66% (57/86) were managed conservatively, 23% (20/86) were managed with a variety of surgical approaches including tracheostomy, aortopexy, tracheopexy, and supraglottoplasty, and management was unknown in 11% (9/86). There was no significant difference in number of symptoms after a trial of conservative management as compared to surgical intervention (p=0.750). Dysphagia in one patient was the only complication in the operative group.

Pulmonary symptoms were reported in 63% (n=97), including cough/wheeze, stridor, recurrent pneumonias, and respiratory distress. Only 17% (n=26) underwent pulmonary function testing, and 8% (n=12) were discharged from their initial hospitalization on supplemental oxygen. Patients discharged on oxygen were significantly more likely to be premature (35.5 vs 37.0 weeks EGA, p=0.001) or require prolonged intubation during their initial hospitalization (14.5 vs 4 days, p< 0.00001). Asthma, prolonged intubation, and multiple intubations during the initial hospitalization were associated with respiratory re-hospitalizations (Table 1).

Vocal cord impairment occurred in 10% of patients (n=16). Of those, 85% (11/16) were managed conservatively, and the others were managed with injection laryngoplasty (1/16), tracheostomy (2/16), laryngeal nerve reinnervation (1/16), or vocal cord mass excision (1/16). There was no significant difference in the use of a feeding tube at discharge between patients with and without vocal cord impairment (63% vs. 41%; p=0.119), but more patients with vocal cord impairment had a feeding tube at one year (73% vs. 30%; p=0.003). Ultimately, out of all patients with vocal cord impairment, 85% (11/16) had documented resolution of symptoms.

Conclusion: This study characterizes the natural history and long-term airway, pulmonary, and vocal cord outcomes in a large cohort of patients with Type C EA/TEF. Tracheomalacia and vocal cord impairment were common findings; however, the majority were successfully managed conservatively. These findings support improved counseling for patients and their families regarding expected outcomes and management strategies.