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For further information, visit mhealth.org/peds-gastroenterology

Pediatric Eosinophilic Esophagitis: Treatment Updates

Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease with symptoms of esophageal dysfunction and histologic finding of eosinophilic inflammation in the esophagus. The disease affects both adults and children. EoE has increased in prevalence over the past 20 years and has now emerged as a common cause of dysphagia.1,2 The disorder is at least 3 times more common in males than females and can affect all ethnic groups and ages.3 EoE occurs more often in developed countries, but differences in diet and lifestyle do not appear to be the sole cause of its occurrence. Its incidence in children and youth is higher than in adults, but estimates vary. One survey in Ohio suggests its incidence to be 9 to 13 new adult cases per 100,000 inhabitants and 42.9 childhood cases per 100,000 inhabitants.1 Another recent survey-based study provided an estimate of 52 cases per 100,000.2

Illustration of esophagus. ©iStock
There are many different presentations of EoE. Young children with the condition may show irritability, food aversion, and failure to thrive, and older children may have abdominal pain and symptoms of reflux. Adolescents and adults are more likely to experience dysphagia and food impaction. Clinical symptoms are nonspecific and require histological correlation.4

In children and adults, EoE is often associated with a history of atopy, including eczema, asthma, and food or environmental allergies. Patients with the above symptoms who do not respond to treatment with acid suppression or are unable to discontinue acid suppression therapy should be referred to a gastroenterology specialist for further evaluation. EoE can result in esophageal fibrosis, swelling, and stiffening4,5 and ultimately can progress to esophageal stricture if untreated. 

Definitive diagnosis requires esophagogastroduodenoscopy with biopsies to evaluate for eosinophilic inflammation.3,4 The procedure should be performed after an adequate trial on high-dose proton pump inhibitor therapy to allow differentiation between reflux and allergy. Grossly, longitudinal furrows, esophageal rings, and white exudates (eosinophilic abscesses) may be present, although they are not diagnostic for EoE. (The normal esophagus does not contain eosinophils.) After ruling out other causes of esophageal eosinophilia, individuals who have greater than 15 eosinophils per high power field on esophageal biopsy with a history of esophageal symptoms meet criteria for the diagnosis of EoE.

The goal of EoE treatment is to resolve the esophageal inflammation and prevent future complications, including esophageal strictures. Treatment options include dietary elimination and topical (swallowed) steroids. The elemental diet (an exclusive diet of amino acid/hypoallergenic formula) and 6-food elimination diet (the removal of soy, milk, wheat, egg, peanut, and seafood for 6 weeks with the gradual introduction of individual food groups to identify allergens) have shown efficacy.6 Less-restrictive diets that focus on removing the most common food allergy triggers—the 4-food or 2-food elimination diets—have also shown promise.6 Allergy testing can be used to guide dietary elimination; unfortunately, allergy testing has been shown to have a poor predictive value in identifying gastrointestinal food allergens. Consultation with a registered dietitian can ensure that patients receive sufficient nutrients and maintain the diet. Because the approach requires diet adherence and multiple follow-up endoscopies to track response, treatment compliance can be challenging, and new measures are being explored.

Potential new medications, including monoclonal antibodies to IL-5, are currently under review. Most children treated have a favorable outcome as adults.8

References

  1. Noel RJ, Putnam PE, Rothenberg ME. Eosinophilic esophagitis. N Engl J Med. 2004;351(9):940-941.
  2. Spergel JM, Book WM, Mays E, et al. Variation in prevalence, diagnostic criteria, and initial management options for eosinophilic gastrointestinal diseases in the United States. J Pediatr Gastroenterol. 2011;52(3):300-306.
  3. Lucendo AJ, Sanchez-Cazalilla M. Adult versus pediatric eosinophilic esophagitis: important differences and similarities for the clinician to understand. Expert Rev Clin Immunol. 2012;8(8):733-745. DOI: 10.1586/eci.12.68.
  4. Muir AB. Eosinophilic esophagitis. Curr Probl Pediatr Adolesc Health Care. 2018;48(3):99-101. doi: 10.1016/j.cppeds.2018.01.006.
  5. Munoz-Persy M, Lucendo AJ. Treatment of eosinophilic esophagitis in the pediatric patient: an evidence-based approach. Eur J Pediatr. 2018;177(5):649-663 DOI: 10.1007/s00431-018-3129-7.
  6. Molina-Infante J, Lucendo AJ. Dietary therapy for eosinophilic esophagitis. J Allergy Clin Immunol. 2018 Mar 6 pii: S0091-6749(18)30320-8. DOI: 10.1016/j.jaci.2018.02.028 [Epub ahead of print].
  7. Von Arnim U, Halfertheiner P. Eosinophilic esophagitis—treatment of eosinophilic esophagitis with drugs: corticosteroids. Dig Dis. 2014;32(1-2)126-129. doi:10.1159/000357089.
  8. Bohm M, Jacobs JW Jr, Gupta A, Gupta S, Wo JM. Most children with eosinophilic esophagitis have a favorable outcome as young adults. Dis Esophagus. 2017;30(1):1-6. DOI: 10.1111/dote.12454.

When to Refer

Patients who experience food impaction, dysphagia, or symptoms such as vomiting, regurgitation, abdominal pain, or heartburn and who do not respond to acid suppression should be referred for evaluation for eosinophilic esophagitis. Very young children with the condition will show signs of food aversion or failure to thrive. Our pediatric gastroenterologists and their allied specialists and dietitians have extensive experience in diagnosing and treating the condition. We provide care for infants, children, and teens, and our program at University of Minnesota Masonic Children’s Hospital is ranked among the top in the United States by U.S News & World Report.

Our physicians also treat esophagitis, gastroesophageal reflux disease, and other disorders of the esophageal tract and stomach. Our pediatric gastroenterologists provide care for patients with Crohn’s disease and ulcerative colitis, and our program is a founding member of an international care network that works to advance effective treatments for these conditions. Our specialists are members of nationally recognized, multidisciplinary care teams, including the Minnesota Cystic Fibrosis Center, the Intestinal Rehabilitation Center, the University of Minnesota Health liver and transplant programs, and the total pancreatectomy and islet auto-transplant program. We also have a pediatric fecal microbial transplant program for children with recurrent Clostridium difficile colitis.

Our physicians evaluate and manage our patients’ conditions with the help of an expert team of dietitians, care coordinators, psychologists, and pediatric endoscopy specialists. Tests and treatments are offered in a comfortable, child-friendly atmosphere. Our staff members coordinate treatment plans where subspeciality consultation is required.

The University of Minnesota Health Child-Family Life Services team is available to our patients and their families and offer help in managing the challenges of hospitalization and illness. These specialists provide age-appropriate guidance on managing stress and anxiety as well as assistance in preparing for medical procedures and tests, including endoscopy, by utilizing coping strategies and play and self-expression activities.

View clinical trials available at studyfinder.umn.edu.

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