Eosinophilic Esophagitis (EoE) in Children

What Happens if Eosinophilic Esophagitis (EoE) is Not Treated? 

Left untreated the chronic inflammation from EoE will lead to scarring of the esophagus which leads to narrowing of the part where food travels (referred to as stricture). This leads to the sensation of food being stuck in the neck or chest. In some cases, emergent endoscopy can be required to retrieve the food that is stuck (referred to as food impaction).  

What Are the Symptoms of Eosinophilic Esophagitis in Children?

EoE is characterized by food impaction (food stuck in the throat, neck or chest), dysphagia (difficulty swallowing or feeling food pass slowly), vomiting, and gastroesophageal reflux disease (GERD).

Less common symptoms include:

• Poor growth
• Food aversion
• Cough or throat clearing with eating
• Regurgitation of food
• Chest discomfort with eating
• Slow eating, increased fluids to wash down solids, and requiring small bites or excessive chewing

What Causes Eosinophilic Esophagitis in Children?

EoE is caused by a dysregulated immune response in the esophagus to certain foods. EoE is caused by a combination of multiple subtle genetic abnormalities along with environmental exposures.  While the environmental exposures are not entirely clear, there are large number of potential genetic abnormalities found in patients with EoE. As no individual or combination of abnormalities is causative, genetic testing is not performed. Because of the genetic component, EoE can run in families, with siblings having nearly 10 fold increased risk.

In addition, many patients with EoE have another allergic disease including asthma, eczema, nasal allergies or IgE-mediated food allergy. Patients with IgE-mediated food allergy have the highest risk of developing EoE with one study showing 5% of patients with IgE-mediated food allergy were diagnosed with EoE.  

Inflammation in EoE is largely caused by one or more foods in the diet with the most common food triggers being dairy and wheat. Over time, ongoing exposure to inciting food(s) and resultant chronic inflammation leads to symptoms of esophageal dysfunction. The inflammation cannot be readily identified by history, nor blood or skin testing. 

How Is Eosinophilic Esophagitis in Children Diagnosed?

Diagnosis of EoE requires upper endoscopy with multiple esophageal biopsies. This procedure is performed by a gastroenterologist under general anesthesia, supervised by an anesthesiologist. The characteristic finding on esophageal biopsies in EoE is high numbers of a type of white blood cell called the eosinophil infiltrating the surface lining of the esophagus. A biopsy showing at least 15 eosinophils per high-power field, along with symptoms of esophageal dysfunction, is diagnostic of EoE.  

Often on endoscopy, the esophagus looks abnormal with vertical lines (furrows), paleness (edema), white specks (exudate), rings (developing scar tissue), or stricture (scar tissue that has progressed to narrowing). Eosinophils may not be found in areas of scarring. 

How Is Eosinophilic Esophagitis in Children Treated?

The current standard-of-care treatment options for EoE in children are: 

1. Proton pump inhibitors (PPI) such as omeprazole or lansoprazole. These are medications commonly used to treat acid reflux that were found to have specific therapeutic effect in treating EoE. 
2. Removal of foods from the diet commonly known to cause inflammation with the guidance of a registered dietitian. Learn more about food elimination diets. 
3. ‘Topical’ corticosteroids (typically twice daily) such as fluticasone or budesonide to coat the esophagus and control inflammation.
4. Dupilumab (brand name: Dupixent): this is a biologic medication that specifically targets the inflammation pathways involved in EoE. Dupilumab are self-administered injections given at regular intervals that have been shown to be effective in treating EoE and other allergic conditions, such as severe eczema or asthma.
5. Esophageal dilation to stretch out narrowed segments of esophagus guided by EndoFLIP (endoluminal functional lumen imaging probe). EndoFLIP is a tool used during endoscopy to measure the size and function of the esophagus to identify narrowed areas that may benefit from dilation. 

EoE is a lifelong disease that requires treatment. The goal of EoE treatment is to heal the esophagus and prevent complications of esophageal inflammation, including scarring and narrowing of the esophagus. 

What Is the Long-Term Outlook for Children with Eosinophilic Esophagitis? 

EoE is a lifelong condition that can result in chronic symptoms associated with eating, significantly impacting the quality of life of patients. Identification of EoE allows for initiation of treatment to prevent long-term damage (scarring), support normal growth, and improve quality of life.  EoE and its symptoms can be well-controlled through diet and medications, and many patients lead a healthy and active life with the right treatment. For children with scarring in the esophagus, dilation can be very beneficial to relieve symptoms. Regular follow-up with EoE specialists is important to identify the right treatment for your child and support their changing needs. 

What Is Lurie Children’s Approach to Caring for Children With Eosinophilic Esophagitis? 

Lurie takes a comprehensive approach that considers all aspects of how Eosinophilic Esophagitis impacts a patient and their family. We partner with our patients and families to develop the right treatment plan for them that is tailored to their specific needs. Our team consists of 4 pediatric gastroenterologists, an advanced nurse practitioner, 2 nurses, and a dietitian, all with specific expertise in EoE management.

We engage in multi-disciplinary care for our patients with other allergic diseases or undergoing Oral Immunotherapy (OIT) through partnership with our Lurie and community-based pediatric allergists. We offer state-of-the-art endoscopic technology in diagnosing and monitoring EoE. Our center has broad experience assessing for scar tissue and narrowing using the EndoFLIP (endoluminal functional lumen imaging probe) technology along with therapeutic dilation. EndoFLIP is a tool used during endoscopy to measure the size and function of the esophagus to enhance the safety and effectiveness of esophageal dilation and to monitor treatment response. We offer transnasal endoscopy (TNE) utilizing the latest technology that is performed without sedation for patients and families that are interested in minimizing anesthesia exposure.  

We offer both telehealth and in-person visits at 2 centers (Lincoln Park and North suburbs of Glenview or Northbrook) and endoscopies at 3 locations (Chicago, Westchester, and Northbrook). We take the whole person into account by assessing quality of life through validated surveys and addressing co-occurring conditions such as eosinophilic gastrointestinal disorders outside the esophagus (eosinophilic gastritis, duodenitis, colitis), Postural orthostatic tachycardia syndrome (POTS), and Ehlers-Danlos syndrome (EDS). Our team is actively involved in cutting edge research (link to Wechsler lab and publications) to better understand disease mechanism and improve treatment options for patients, all toward the goal of finding a cure for EoE. 

Learn more about The Eosinophilic Gastrointestinal Diseases (EGID) Program