“Eosinophilic esophagitis represents a chronic immune/antigen mediated oesophageal disease characterized clinically by symptoms related to oesophageal dysfunction and histologically by eosinophil-predominant inflammation” (80), as defined by a multidisciplinary panel in 2011.
The American College of Gastroenterology define EOE as: ‘The presence of symptoms related to oesophageal dysfunction such as dysphagia, food impaction, chest pain, etc.’
There is a higher prevalence of EOE in adolescents and adults born with OA/TOF. The prevalence in the general population is <4/10000, compared with 9.5% prospectively biopsied adolescents born with OA/TOF. (75,48)
The causes of this are thought to be multifactorial. One cause is the high prevalence of GORD in the TOF population, exposing the oesophagus to injury and damaging the mucosa. This damage to the mucosal barrier allows easier penetration of allergens. Secondly, the near universal dysmotility of the oesophagus increases contact time between food/allergens and the oesophageal mucosa, leading to chronic irritation which triggers increased mucosal permeability to allergens and the influx of eosinophils and mast cells.
It is difficult to differentiate EOE and severe GORD from symptoms alone. In one study, patients with EOE had higher levels of dysphagia, retrosternal pain and food impaction, but some in patient groups with repaired OA/TOF and those without EOE reported these symptoms. (81)
This is known as the 3Ds: diet, drugs and dilatation. Either drugs or diet can be used as first-line treatment. In OA/TOF patients there may be a greater proportion of patients that respond to PPI treatment as a first-line therapy. (212)
The European Society for Paediatric Gastroenterology, Hepatology and Nutrition guidelines state that EOE needs to be excluded in OA patients of all ages with dysphagia, reflux symptoms, coughing, choking or recurrent strictures that are refractory to PPI, before proceeding to anti-reflux surgery. (39)