Eosinophilic esophagitis in esophageal atresia patients: current evidence and clinical implications

Background Eosinophilic esophagitis (EoE) is increasingly recognized in children with esophageal atresia (EA), but its epidemiology, clinical phenotype, risk factors, and optimal management in this population remain incompletely defined. We reviewed the available evidence on EoE associated with EA to clarify its clinical relevance and identify areas requiring further investigation.Methods A narrative review was conducted based on a systematic PubMed search of studies published through May 1, 2025, using terms related to EA, tracheoesophageal fistula, and EoE. Additional studies were identified through backward citation tracking. Studies reporting patients with both EA and EoE were included.ResultsTwenty-one studies were included, reporting 253 patients with EA-associated EoE. Reported prevalence of EoE in EA exceeded 10% in most studies using systematic esophageal biopsies, although estimates may be influenced by the high frequency of endoscopic surveillance in EA. EoE appeared to be diagnosed at a younger age than in the general pediatric EoE population, with diagnosis frequently occurring between 2 and 3 years of age. Dysphagia, feeding difficulties, and food-related symptoms were the most common presenting features. Endoscopic abnormalities typical of EoE were frequently observed, while histology consistently demonstrated significant eosinophilic infiltration and frequent fibrosis. Available comparative data suggest that EA-associated EoE may represent a distinct phenotype characterized by earlier onset, lower peak eosinophil counts, fewer allergic comorbidities, and impaired mucosal integrity. First-line treatment achieved remission in approximately 63% of patients, with responses to proton pump inhibitors and topical corticosteroids comparable to those reported in pediatric EoE.ConclusionEoE is an important comorbidity in children with EA and may contribute to longterm esophageal morbidity. Systematic staged esophageal biopsies should be considered during endoscopic evaluation of EA patients. Well-powered comparative studies are needed to define specific risk factors, establish the phenotype of EA-associated EoE, and optimize management.