High rate of pulmonary exacerbations and neutrophilic airway inflammation in children with repaired esophageal atresia with or without tracheoesophageal fistula
Year: 2026
Type: article
Study design: Observational Study
Abstract: BACKGROUND: Children with repaired esophageal atresia with or without tracheoesophageal fistula (EA/TEF) frequently have chronic respiratory morbidity, but airway inflammation is poorly defined. We described bronchoalveolar lavage (BAL) cell patterns in symptomatic EA/TEF and compared them with primary ciliary dyskinesia (PCD), protracted bacterial bronchitis (PBB), and healthy controls (HC). METHODS: BAL profiles from symptomatic EA/TEF patients (n = 44) were analyzed against PCD (n = 10), PBB (n = 21), and HC (n = 16). Endoscopic bronchitis severity was assessed using the BScore. Associations between BScore and BAL profiles were assessed using multivariable modeling. RESULTS: All EA/TEF patients had pulmonary exacerbations. BAL neutrophils were higher in EA TEF than HC (median 8.0%, IQR 3-25 vs 1.0%, IQR 0-2, p < 0.001) and similarly elevated in PBB and PCD. BScore correlated with neutrophils (Spearman rho 0.69, p < 0.001) and independently predicted neutrophilic inflammation (beta 4.53, 95% CI 1.78-7.28, p = 0.002). Pathogenic bacteria were cultured in 61%, most commonly Haemophilus influenzae (29.5%) and Staphylococcus aureus (25.0%). CONCLUSIONS: This is the first study to systematically demonstrate neutrophil-driven airway inflammation in symptomatic children with repaired EA/TEF. These findings underscore the need for structured pulmonary follow up and the evaluation of anti inflammatory strategies in EA/TEF patients. IMPACT: Children with repaired EA/TEF and respiratory symptoms show frequent pulmonary exacerbations and neutrophil driven BAL inflammation. Neutrophil levels resemble protracted bacterial bronchitis and primary ciliary dyskinesia and track bronchoscopic bronchitis severity. Bronchoscopic bronchitis severity tracks neutrophilic inflammation and independently predicts it. Bacterial pathogens are common in BAL of EA/TEF children, supporting infection related airway injury risk. Results inform phenotype driven care pathways to reduce exacerbations and long term airway damage in EA/TEF children.
Source: Pediatric Research
Authors: Illya Martynov, Amitai Hecht Nofar, Richard Wagner, Jan-Hendrik Gosemann, Steffi Mayer, Robin Wachowiak, Manuela Siekmeyer, Tobias Piegeler, Maximilian von Laffert, Tobias Lipek, Norman Lippmann, Martin Lacher, Freerk Prenzel, Holger Stepan, Franz Wolfgang Hirsch, Ulrich Thome, Jan Gosemann, Tobias Piegeler, Manuela Siekmeyer, Sebastian Krämer, Martin Kostelka, Marcel Vollroth, Andreas Dietz, Michael Fuchs, Sylvi Meuret, Freerk Prenzel, Gunter Flemming, Christoph Heyde, Georg von Polier, Annick Martin, Ulrike Knauf
Language: English
Topic: Esophageal and GI Pathology
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,390
OpenAlex ID: W7162485614