Overview
Definition:
Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease characterized by esophageal eosinophilia, distinct from eosinophilic gastroenteritis or other causes of eosinophilic infiltration
It is a common cause of food impaction and dysphagia in children and adults.
Epidemiology:
Prevalence is increasing globally, with estimates ranging from 1 in 2,000 to 1 in 10,000 in Western countries
It affects males more than females and is often associated with other atopic diseases like asthma, allergic rhinitis, and atopic dermatitis.
Clinical Significance:
EoE significantly impacts quality of life due to feeding difficulties, dysphagia, abdominal pain, vomiting, and failure to thrive in children
Proper diagnosis and management, including carefully planned food reintroduction, are crucial to prevent long-term complications like esophageal strictures and fibrosis.
Clinical Presentation
Symptoms:
In infants and young children: Poor feeding
Vomiting
Abdominal pain
Failure to thrive
Esophageal reflux symptoms
In older children and adolescents: Dysphagia, particularly to solids
Food impaction
Chest pain
Odynophagia
Refusal to eat.
Signs:
Physical examination may be normal in most cases
However, signs of atopy (eczema, allergic rhinitis) may be present
Signs of malnutrition or failure to thrive in younger children.
Diagnostic Criteria:
The current consensus criteria require: 1) Symptoms suggestive of EoE
2) Esophageal eosinophil counts of ≥ 15 eosinophils per high-power field (HPF) on esophageal biopsy, excluding other causes of esophageal eosinophilia
3) Absence of other gastrointestinal eosinophilic diseases
4) Response to allergen immunotherapy or exclusion diet.
Diagnostic Approach
History Taking:
Detailed history of feeding difficulties, vomiting, abdominal pain, dysphagia, food impaction episodes
History of atopic diseases (asthma, eczema, allergic rhinitis)
Family history of atopy or EoE
Previous investigations for GERD or food allergies.
Physical Examination:
General assessment of nutritional status and growth
Examination for signs of atopy (skin, respiratory system, nasal mucosa)
Abdominal examination for tenderness or distension.
Investigations:
Esophageal endoscopy with biopsies from multiple levels (proximal, mid, distal esophagus) is essential for diagnosis
Biopsies should be stained with Hematoxylin and Eosin (H&E) to count eosinophils
Allergy testing (skin prick tests or specific IgE) may identify potential triggers
Esophageal manometry may be considered if motility disorders are suspected
Barium swallow can reveal esophageal narrowing or strictures.
Differential Diagnosis:
Gastroesophageal reflux disease (GERD)
Eosinophilic gastroenteritis
Celiac disease
Peptic strictures
Achalasia
Infectious esophagitis
Systemic mastocytosis.
Management Food Reintroduction
Elimination Diet:
Initial management typically involves a strict elimination diet, removing common food allergens (e.g., milk, soy, wheat, egg, nuts, fish, shellfish)
A 6-food elimination diet (SFED) is often the first step, followed by targeted reintroduction.
Stepwise Reintroduction:
Once symptoms resolve and eosinophil counts normalize, food reintroduction is performed systematically, typically one food group at a time
Each reintroduced food is usually consumed daily for 2-4 weeks
Symptoms and repeat endoscopy/biopsy are used to identify culprit foods.
Diagnostic Food Challenges:
In specific cases, especially when fewer than 4 food groups are removed, a targeted elimination diet may be followed by diagnostic food challenges
This involves reintroducing suspected allergens individually under medical supervision to confirm trigger foods.
Monitoring During Reintroduction:
Close monitoring for recurrence of symptoms (dysphagia, pain, vomiting) or signs of inflammation is crucial
Repeat endoscopy and biopsy are usually performed after each reintroduction phase or a set period of dietary change to assess the eosinophil count.
Long Term Dietary Strategy:
The ultimate goal is to achieve the least restrictive diet that maintains symptom control and esophageal healing
This might involve elemental diets, specific antigen avoidance, or even continued use of proton pump inhibitors (PPIs) for their anti-inflammatory effects.
Medical Management Adjuncts
Proton Pump Inhibitors:
Empirical trial of PPIs (e.g., omeprazole 1-2 mg/kg/day divided BID) is often a first step, as they can induce remission in a subset of patients and may have anti-inflammatory effects beyond acid suppression
Dosage adjustments based on weight and response are important.
Topical Corticosteroids:
Swallowed topical corticosteroids (e.g., fluticasone or budesonide) are the mainstay of medical therapy for patients not responding to PPIs or requiring more potent therapy
These are typically administered via metered-dose inhalers with spacers, with the patient swallowing the aerosolized medication
Dosing varies by age and severity
A common regimen for budesonide is 0.5-1 mg BID, often diluted with water or honey
Steroid pharyngeal applicators can also be used.
Biologics:
For refractory cases, newer biologic agents targeting specific inflammatory pathways (e.g., dupilumab) are showing promise and are increasingly used off-label or within clinical trials, especially in older children and adults
However, their role in routine pediatric EoE management is still evolving.
Complications
Early Complications:
Esophageal food impaction is a common immediate complication requiring urgent endoscopic removal
Severe dysphagia and pain can lead to malnutrition and dehydration.
Late Complications:
Chronic inflammation can lead to esophageal strictures, fibrosis, narrowing, and permanent motility abnormalities
Long-term nutritional deficiencies and impaired growth are concerns in pediatric patients.
Prevention Strategies:
Adherence to prescribed diets and medications
Regular follow-up with pediatric gastroenterologists and allergists
Prompt management of food impactions and symptoms
Avoiding triggers identified through food challenges.
Prognosis
Factors Affecting Prognosis:
Early diagnosis and consistent management are key
Patients with extensive fibrosis or strictures at diagnosis may have a poorer prognosis
The number of identified trigger foods and patient adherence to dietary recommendations also play a role.
Outcomes:
With appropriate management, most children with EoE can achieve symptom control and improve their quality of life
However, EoE is a chronic condition that often requires lifelong management
Strictures may require dilation, and in rare cases, surgical intervention.
Follow Up:
Regular follow-up with a multidisciplinary team (pediatric gastroenterologist, allergist, dietitian) is essential
This includes monitoring symptoms, adherence to diet, potential need for repeat investigations (endoscopy/biopsy), and growth parameters
Transition to adult care should be planned.
Key Points
Exam Focus:
EoE is defined by symptoms AND esophageal eosinophilia (≥ 15 HPF)
Differentiate from GERD
Management involves diet (elimination, reintroduction) and topical steroids
PPIs have a role but are not primary treatment for most.
Clinical Pearls:
Always consider EoE in children with dysphagia, food impaction, or unexplained vomiting/abdominal pain, especially with a history of atopy
Swallowed fluticasone/budesonide is the first-line medical therapy
Food reintroduction must be systematic and guided by repeat biopsies.
Common Mistakes:
Attributing all symptoms to GERD without considering EoE
Not performing adequate biopsies during endoscopy
Incomplete or unsystematic food reintroduction
Relying solely on PPIs without topical steroids for most cases
Neglecting long-term follow-up and monitoring for strictures.