Eosinophilic esophagitis in patients with esophageal atresia and chronic dysphagia

Abstract

Esophageal atresia (EA) is defined as a discontinuity of the lumen of the esophagus repaired soon after birth. Dysphagia is a common symptom in these patients, usually related to stricture, dysmotility or peptic esophagitis. We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis (EoE) was made, ages ranging from 9 to 16 years. Although our patients were on acid suppression years after their EA repair, they presented with acute worsening of dysphagia. Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with EoE. Two of 4 patients improved symptomatically with the topical steroids. It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies. One of our patients developed recurrent anastomotic strictures that improved with the treatment of the EoE. A previous case report linked the recurrence of esophageal strictures in patients with EA repair with EoE. Once the EoE was treated the strictures resolved. On the other hand, based on our observation, EoE could be present in patients without recurrent anastomotic strictures. There appears to be a spectrum in the disease process. We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patients with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE.

Eosinophilic esophagitis and esophageal atresia: coincidence or causality?

“…In EA, strictures are dilated routinely; however, in EoE, it has been demonstrated that topical corticosteroids may reverse the fibrotic subepithelial process. Esophageal strictures have been reported in patients with EA and EoE at a 18,21,24 In the study carried out by Dhaliwal et al, patients with EoE significantly required a fundoplication more frequently than those without EoE, whereas no differences were observed in terms of gastrostomy requirements. 18 A retrospective study by Chan et al assessed the outcomes of treatment for EoE in children with EA.…”

“…Gorter et al have proposed that mutations in the FOX gene not only cause congenital malformations in the esophagus and lung but may also predispose to EoE, so patients with EA may be at a high risk for EoE. 3,6,21 The above mentioned observations suggest that EoE should be taken into consideration when making a differential diagnosis in patients with EA who have dysphagia and refractory gastroesophageal reflux symptoms before considering anti-reflux surgery. 3,21 The age of onset of EoE and EA ranges from 18 months to 12.5 years, and this entity is more common among men, except for the study by Dhaliwal et al, where it was predominant among women (1.6:1).…”

“…3,6,21 The above mentioned observations suggest that EoE should be taken into consideration when making a differential diagnosis in patients with EA who have dysphagia and refractory gastroesophageal reflux symptoms before considering anti-reflux surgery. 3,21 The age of onset of EoE and EA ranges from 18 months to 12.5 years, and this entity is more common among men, except for the study by Dhaliwal et al, where it was predominant among women (1.6:1). In relation to the clinical presentation, this study found a significantly higher incidence of GERD symptoms, such as vomiting and dysphagia, among patients with EoE compared to those without EoE.…”

“…3,18,23 A normal endoscopy, linear furrows, edema, and white exudates were described as endoscopic findings. 18,21,22 Multiple biopsies of both the proximal and distal parts of the anastomosis are recommended for EoE diagnosis. 19 In EA, anastomotic stricture occurs in 5-15% of patients, mainly in the first year of life.…”

Esofagitis eosinofílica y atresia esofágica: ¿casualidad o causalidad?

“…Gorter y col. postulan que mutaciones en el gen FOX no solo llevan a malformaciones congénitas de esófago y pulmón, sino que podrían predisponer a EEo, por lo cual pacientes con AE podrían tener alto riesgo de EEo. 3,6,21 Las observaciones anteriores sugieren que la EEo debe considerarse como diagnóstico diferencial en pacientes con AE que tienen disfagia y síntomas de reflujo gastroesofágico refractarios antes de considerar la cirugía antirreflujo. 3,21 La disfagia persistente en pacientes con AE debe evaluarse con esofagograma y VEDA + múltiples biopsias.…”