Eosinophilic Esophagitis in Esophageal Atresia: Is It Really a New Disease?

Abstract

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease. Symptoms are related to mucosal eosinophilic-predominant inflammation that leads to esophageal dysfunction. Recent data suggest that esophageal atresia (EA) patients may have an increased incidence of EoE compared to the general population. As EoE symptoms may be confused with EA-related symptoms, they may significantly worsen morbidity in this specific group of patients. We investigated specific characteristics of patients with AE and EoE compared to those with EoE only. We conducted an observational retrospective monocentric study including all patients diagnosed with EoE from 1 January 2010 to 31 December 2021. For each patient, demographic, clinical and histopathological data were collected and then compared between the two cohorts (EA-EoE vs. EoE only). During the study period, 62 patients were included: 17 children were in the follow-up because of EA (18.1% of 94 EA patients screened in that period), while the other 45 presented EoE only. The demographic and clinical features of EA-EoE patients demonstrate a lower prevalence of allergic subjects (23.5% vs. 80%, p < 0.05), a lower age of presentation (3.1 vs. 12.2 years, p < 0.05), non-specific symptoms and a higher resolution rate with PPI therapy (64.7% vs. 17.8%, p < 0.05) compared to EoE-only patients. Our data confirm that EA patients are at high risk for developing EoE. As symptoms may overlap with the EA spectrum, early recognition of EoE may prevent patients from receiving unnecessary invasive therapeutic interventions and from developing complications from untreated EoE.

Oral Viscous Budesonide in Children With Eosinophilic Esophagitis After Repaired Esophageal Atresia: A Clinical Trial

“…This study demonstrated that OVB formulation has a remarkable efficacy to induce both histologic and clinical remission in EA children with EoE. Consistent with recent evidence (12,36–38), our findings support the view that esophageal eosinophilia in EA may be caused by different mechanisms than in the general population. Anyhow, we believe that OVB could be considered as the preferred first‐line therapeutic option because steroids would be expected to counteract the esophageal eosinophilic inflammation regardless of its cause.…”

“…Indeed, despite the discrepancy between endoscopic and histological appearance has been reported in EoE from general population, normal-looking esophagus has been reported only in a small proportion of children diagnosed with EoE (33), while the EREFS classification system showed an excellent accuracy in identifying both pediatric and adult EoE in the general population (34,35). In this context, there is an ongoing scientific debate among experts surrounding the pathophysiology of EoE occurring after EA repair, with some disagreeing with the guidelines on pediatric EA while supporting the development of dedicated criteria for EoE diagnosis in the EA population to better identify candidates for EoE-directed therapies (12,13,36,37). In our view, it is likely that mechanisms leading to esophageal eosinophilic infiltration in EA patients differ from those of the general population (12,13).…”

Proton pump inhibitors in esophageal atresia: A systematic review and meta‐analysis

“…We selected a total of 38 reports on the topic that met the criteria selection (Table S1), representing a cumulative 6044 patients with EA 16–53 . Fourteen studies were prospective and 24 were retrospective.…”

“…Only one study 51 has addressed the relations between neonatal pPPI use and later EoE occurrence, demonstrating a positive association between PPI duration (p = 0.018) and cumulative dose (p = 0.017) with EoE development in EA. When EoE was associated with EA, PPIs alone induced remission in 50%-66% 25,43,50,53 of patients.…”