Eosinophilic esophagitis and intermediate esophagitis after tracheoesophageal fistula repair: a case series.

BACKGROUND: Eosinophilic esophagitis (EE) is often missed or underdiagnosed in children, particularly in the setting of reflux disease associated with tracheoesophageal fistula (TEF). Intermediate esophagitis (IE) is a recently described condition, which includes characteristics of gastroesophageal reflux disease and EE but does not present with enough eosinophils on biopsy to diagnose EE. Here we present a case series of EE and IE associated with TEF, and their clinical manifestations.

METHODS: A retrospective analysis including clinical presentation, endoscopic and pathologic findings, and treatment of 4 patients with EE and 4 patients with IE who presented between 2003 and 2007 was performed.

RESULTS: Male dominance was found equally in both groups (75%), and most patients had a personal history of atopy (87.5%). Food allergies were seen mainly in the EE group (75%). The most frequent primary symptoms in both groups were dysphagia for solids (75%) and for liquids (25%). The median age at diagnosis was 9.8 vs 11.2 years in the EE and IE groups, respectively. On endoscopy, both groups had similar findings including furrows (EE, 75%; IE, 66.6%) and white plaques (EE, 50%; IE, 33.3%). In both groups, almost all patients had basal cell hyperplasia on biopsy (EE, 100%; IE, 75%). Degranulated surface eosinophils (50%) and eosinophilic abscess (25%) were found in the EE group only. Elongated rete papillae were more often seen in EE biopsies (50%) compared with IE biopsies (25%). Peripheral serum eosinophilia was seen in all EE patients and in 33% of the IE patients. The only effective treatment with complete resolution of the symptoms was the topical or systemic application of steroids.

CONCLUSIONS: The diagnosis of EE and IE is frequently missed or delayed. Eosinophilic esophagitis should be suspected in reflux disease refractory to conventional treatment, particularly in the setting of TEF. Intermediate esophagitis represents an entity that includes findings of gastroesophageal reflux disease and EE. Endoscopic biopsies are diagnostic for both conditions and allow institution of specific medical treatment.