Neutrophilic airway inflammation in children with repaired esophageal atresia-tracheoesophageal fistula (EA/TEF)

Introduction A high proportion of children with repaired tracheoesophageal fistula (TEF) have respiratory sequelae mainly due to tracheomalacia. Pathophysiologic mechanisms with mutual dependence include airway collapse, mucus retention, bacterial colonization, chronic bronchitis, pulmonary exacerbations, and development of bronchiectasis. In other lung diseases with chronic bronchitis such as primary ciliary dyskinesia (PCD) or protracted bacterial bronchitis (PBB), bronchiectasis and exacerbations are sequelae of neutrophilic inflammation and bacterial infection. Therefore, we aimed to describe the cellular inflammatory patterns and bacterial pathogens in bronchoalveolar lavage fluid (BAL) in children with a history of TEF repair.

Methods This retrospective, monocentric case comparison study analyzes the BAL differential cell counts of children with repaired TEF and chronic or recurrent respiratory symptoms, children with PBB, and children with PCD. These groups were compared with a reference group consisting of cohorts with published BAL data from children without lung disease.

Preliminary Results Overall, 47 children were included in the study (16 patients with repaired TEF, 21 with PBB, and 10 with PCD). The mean age was 3.9±4.1 years in the TEF, 3.8±3.3 in the PBB, and 5.8±6.4 in the PCD group (p=0.94, χ² Pearson=0.13).

All children with TEF repair had evidence of bronchitis on bronchoscopy and 15/16 (94%) had tracheomalacia. Bacterial pathogens were detected in 11/16 (69%) of the BAL fluids, most commonly Haemophilus species, Staphylococcus aureus, and Moraxella catarrhalis.

The number of neutrophilic granulocytes in the BAL was significantly higher in the TEF group than in the healthy historical cohort group (27.1±31.1% vs. 1.19±1.17%, p<0.001). Neutrophilic inflammation in the PCD group was similar (27.5%, p=0.69) and lower but not statistically significantly different from the PBB group (12.3%, p=0.23).

Discussion Interdisciplinary follow-up of children with TEF including bronchoscopy and BAL is necessary. Future studies should investigate the association between neutrophilic airway inflammation and pulmonary exacerbations as well as the development of bronchiectasis and should focus on anti-inflammatory therapies.

Publication History

Article published online:
09 March 2023