Eosinophilic esophagitis in children with esophageal atresia

Abstract

Eosinophilic esophagitis (EoE) has only rarely been reported in esophageal atresia (EA) patients. A retrospective case analysis of all EA patients born at our center between January 1999 and April 2012 was performed. A total of 113 of patients were identified; 10 patients were excluded as a result of inadequate data. Eighteen patients (17%) were diagnosed with EoE. The average number of eosinophilis was 30/high-power field (HPF) (19/HPF-80/HPF). The median age for diagnosis of EoE was 1 year and 6 months (8 months-8 years and 7 months). Children with EoE had a significantly greater incidence of reflux symptoms, dysphagia, tracheomalacia, and 'hypoxic spells' (P < 0.05). EoE patients also underwent significantly more surgery including fundoplication and aortopexy when compared with those without EoE (P < 0.0001). Although the incidence of gastrostomy was greater in the EoE group (33% vs. 13%), this was not statistically significant. Half of the EoE patients had a coexisting atopic condition at time of diagnosis. The commonest condition was asthma 7/18 (38%) followed by specific food allergy 6/18 (33%). EoE was treated in 11 patients with either swallowed fluticasone or budesonide slurry. All improved clinically. Histologically, five had complete resolution and six had partial improvement. Six children with EoE were treated with acid suppression alone. All improved clinically, and 5/6 had subsequent histological resolution. One child who received acid suppression and an exclusion diet also improved. Seven patients (38%) had an esophageal stricture at time of EoE diagnosis. Five were dilated at time of the initial endoscopy, prior to the diagnosis of EoE being available. Two patients had resolution of their strictures on medical treatment of their EoE alone and did not require further dilatation. EoE was seen in 17% of children with EA in this study. EoE should be considered in EA patients with persistent symptoms on standard reflux treatment, increasing dysphagia, and recurrent strictures.

Eosinophilic esophagitis and esophageal atresia: coincidence or causality?

“…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. 6,18,22 As it is known in EoE, in all series, associated atopy was frequent (Dhaliwal: 50%; Batres: 66%; Gorter: 50%). 3,18,23 A normal endoscopy, linear furrows, edema, and white exudates were described as endoscopic findings.…”

“…6,18,22 As it is known in EoE, in all series, associated atopy was frequent (Dhaliwal: 50%; Batres: 66%; Gorter: 50%). 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.…”

“…It is secondary to esophageal dysmotility, short esophagus, lower esophageal sphincter pressure, and chronic lung disease. 4,17,18 Dysmotility is the result of intrinsic factors related to abnormal esophagus development or partial denervation during surgery. Motor patterns were described in a cohort of children with EA using a high resolution manometry.…”

“…The largest series was published by Dhaliwal et al, which established a 17% incidence (18 cases). 18 Several hypothesis explain the highest frequency of EoE and EA: 1) alterations in esophageal motility lead to a prolonged exposure to potential allergens, thus increasing the risk of local sensitization; 2) GERD causes mucosal injury and may impair the mucosal barrier function and predispose to EoE; 3) food impaction secondary to dysmotility and esophageal stricture associated with EA may damage the mucosa and cause eosinophilic infiltration; 4) long-term exposure to acid suppression, which is common among children with EA, may increase food protein allergenicity; 5) the 16q24 microdeletion involving the FOX transcription factor gene, in humans, has been associated with EA, cardiac, anorectal, renal, gastrointestinal, and vertebral anomalies. Curiously, FOXF1 genes have been identified in mice, and FOXF1 binding sites are present not only in the promoter region of genes that are critical for mesenchymal proliferation but also in the inflammation promoter region, including those for eotaxin and interleukin.…”

“…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.…”