Case 1: A 12-year-old boy with difficulty swallowing

A 12-year-old boy of Nigerian descent presented to the emergency department with mid-chest discomfort and the sensation of food getting ‘stuck’ in his throat. His symptoms had become progressively worse since beginning three months previously and he had lost 6.8 kg in weight over this time. There was no history of food or other allergies and no history of feeding difficulty or reflux as an infant. He was constipated but experienced no regurgitation, choking, coughing, respiratory compromise or vocal changes. He had no history of constitutional symptoms, foreign body ingestion or neck masses. He was only able to drink liquids and could not tolerate solids. He had asthma as a child but had been otherwise healthy. On examination, he was well nourished and hydrated, with normal vital signs. His weight was 49.1 kg (50th to 75th percentile) and his height was 152.6 cm (25th to 50th percentile). He exhibited nasal congestion with slightly enlarged nasal turbinates. Investigations revealed a normal white blood cell count (5.9×109/L) with an elevated eosinophil count of 0.77×109/L (normal range 0.02×109/L to 0.05×109/L). Urate and lactate dehydrogenase levels were normal, as was a chest x-ray. An upper gastrointestinal series was abnormal, with the radiologist concerned about a possible obstruction from an underlying coarctation of the aorta; however, four-limb blood pressures, electrocardiography and echocardiography were normal. Further investigation revealed the diagnosis. Endoscopy revealed erythematous mucosal tissue with nodularity throughout the esophagus, with mild trachealization and narrowing whitish exudates (Figure 1). Esophageal biopsies of the upper, mid- and distal sections were remarkable for increased eosinophils, with intraepithelial eosinophil counts of 37, 40 and 87 per high-power field (hpf), respectively (hpf refers to the area of the slide that is visible by light microscopy at the maximum magnification of the objective being used). Duodenal, stomach and antral biopsies showed no other pathology. These findings, in combination with his clinical symptoms, suggested a diagnosis of eosinophilic esophagitis (EoE). The patient was treated with inhaled fluticasone 250 μg, two puffs four times per day or 500 μg two times per day, and dietary restriction. Within two weeks, he experienced significant improvement in his symptoms, was regaining his lost weight and was back on his regular diet. He was referred to an allergist. Endoscopic images in eosinophilic esophagitis showing trachealization (1) and furrowing (2), whitish exudates (3) and nodules (4) of the esophageal mucosa Dysphagia can be defined as any difficulty or abnormality with swallowing and is categorized based on several factors. EoE is an important cause of dysphagia and can be intermittent or progressive. EoE is defined as a chronic immune or antigen-mediated esophageal disease. The diagnosis of EoE is reached based on clinical as well as histological criteria (1). There is overlap in symptoms and response to treatment with gastroesophageal reflux disease. Initially, response to proton pump inhibitors was believed to separate the two but it is now known that a subset of EoE patients is responsive to proton pump inhibitors. Histologically, occasional eosinophils are also observed in patients with gastroesophageal reflux disease (2). In the gastrointestinal tract, eosinophils are an integral component of the innate immune system, acting as a first line of defence. In individuals who are predisposed to allergen hypersensitivity, T cell stimulation (specifically T-helper type 2 cells) along with eosinophil differentiation and proliferation (Figure 2) leads to eosinophil-mediated tissue injury and chronic inflammation. These processes are believed to lead to esophageal wall fibrosis and thickening (2). Increased eosinophils in the mid-esophagus. The arrows indicate scattered eosinophils in the biopsy (40× objective) Clinically, infants and toddlers tend to present with feeding difficulties and failure to thrive, while school-age children frequently present with vomiting or reflux-type symptoms (2). In older children and adolescents, such as our patient, solid food dysphagia is the predominant symptom. In adults, food impaction is a common presentation. EoE should be suspected in any individual with esophageal foreign body impaction (1). Other symptoms in this group include food impaction, heartburn and chest pain. In most children, EoE is associated with other atopic disease including food allergy, asthma, eczema, chronic rhinitis and environmental allergies (2). Endoscopy with biopsy is the only reliable diagnostic test. Esophogeal abnormalities on endoscopic study (Figure 1) include fixed or transient esophageal rings, whitish exudates, longitudinal furrows, edema and diffuse esophageal narrowing; however, none are pathognomonic for EoE (2). According to the latest consensus statement, the histopathological changes must include >15 eosinophils/hpf (peak value) for the diagnosis of EoE, but absolute numbers remain controversial (2). EoE is usually isolated to the esophagus but may be part of a generalized gastrointestinal eosinophilic disorder, and other causes of EoE should be ruled out. Symptoms respond to therapy, but the disease tends to relapse and remit and often lasts into adulthood (1). EoE should remit with appropriate therapy (2). EoE is treated with dietary therapy, topical or systemic corticosteroids, and may even require esophageal dilation (1). Dietary therapy ranges from a strict elemental diet using hypoallergenic formula to an empirical diet in which foods that are typically associated with EoE (milk, soy, wheat, eggs, nuts and fish) are removed (2). An allergy evaluation is warranted in individuals diagnosed with EoE, and dietary restriction based on multimodal allergy testing is another dietary therapy used. Although complete resolution of clinical and histological abnormalities has been shown with all three dietary therapies, a strict elemental diet continues to be the most effective therapy (2). Adherence to dietary restriction can be problematic. Topical swallowed corticosteroids can be administered using either inhaled or nebulized preparations. Fluticasone (88 μg to 440 μg, two to four times per day) and budesonide (1 mg to 2 mg per day) are the most common topical steroids used to treat EoE (2). Systemic steroids can be used in severe cases. Topical steroid therapy is as effective as dietary therapy, but symptoms tend to recur once therapy is discontinued (2). Treatment of EoE is lifelong, although maintenance therapy has not yet been standardized. Children should be monitored through routine office follow-up visits because symptoms often recur (1). The clinical presentation of EoE may vary with age. Younger children often present with a feeding disorder, vomiting or reflux symptoms, and failure to thrive. Abdominal pain, dysphagia and food impaction are typical in older children, adolescents and adults. The diagnosis of EoE is based on clinical features and defined histopathological findings of >15 eosinophils/hpf. The initial approach to treatment of EoE should include an allergy evaluation, dietary therapy and/or corticosteroid treatment. The authors acknowledge the contribution of Dr Achiya Amir and Dr Glenn Taylor from the Divisions of Gastroenterology, Hepatology and Nutrition, and Pediatric Laboratory Medicine, respectively.

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