Overview
Definition:
Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease characterized by symptoms of esophageal dysfunction and eosinophil-predominant inflammation on esophageal biopsy
It is often associated with a history of allergic diseases.
Epidemiology:
Prevalence is increasing worldwide, affecting both children and adults
In pediatrics, it is a leading cause of dysphagia and food impaction
Peak incidence is in early childhood and adolescence
It is more common in males and those with a personal or family history of atopy.
Clinical Significance:
EoE can lead to significant morbidity including failure to thrive, dysphagia, food impaction, chest pain, and vomiting
Accurate endoscopic diagnosis is crucial for initiating appropriate management to prevent long-term complications such as esophageal strictures and remodeling
Understanding endoscopic findings aids in stratifying severity and monitoring treatment response.
Clinical Presentation
Symptoms:
In infants and young children: Feeding difficulties
Vomiting
Irritability
Failure to thrive
In older children: Dysphagia (most common)
Food impaction
Chest pain (non-cardiac)
Abdominal pain
Refusal to eat
Nausea
Cough.
Signs:
Physical examination may be normal
Findings can include signs of atopy (eczema, asthma, allergic rhinitis)
In severe, long-standing cases, signs of malnutrition or growth delay may be present
Esophageal manometry findings can be abnormal, but are not diagnostic of EoE.
Diagnostic Criteria:
The diagnostic criteria for EoE typically include: 1
Symptoms of esophageal dysfunction
2
Esophageal biopsy showing >= 15 eosinophils per high-power field (eos/hpf) in the absence of other identifiable causes for eosinophilic infiltration
3
Response to antigen exclusion or medical therapy aimed at EoE.
Diagnostic Approach
History Taking:
Detailed history of onset and nature of dysphagia or feeding problems
History of allergies (asthma, eczema, rhinitis, food allergies)
Family history of atopy or EoE
Previous episodes of food impaction
Previous treatments and response
Red flags: acute food impaction, failure to thrive, significant weight loss, severe vomiting.
Physical Examination:
General assessment for nutritional status and growth
Examination for stigmata of atopy: skin (eczema), respiratory system (wheezing), nasal passages (rhinorrhea, nasal polyps)
Abdominal examination for tenderness or organomegaly
Cardiac examination to rule out other causes of chest pain.
Investigations:
Upper endoscopy with biopsies is the gold standard
EREFS (Endoscopic Reference Score) is used to grade endoscopic findings
Biopsies should be taken from multiple levels of the esophagus (proximal, mid, distal) and stomach/duodenum if indicated
Allergy testing (skin prick or specific IgE blood tests) can identify triggers but is not diagnostic of EoE
Esophageal dilation may be considered if strictures are present.
Differential Diagnosis:
Gastroesophageal reflux disease (GERD), achalasia, esophageal strictures (peptic, anastomotic), eosinophilic gastroenteritis, eosinophilic gastritis, celiac disease, Plummer-Vinson syndrome, infectious esophagitis, functional dysphagia, foreign body ingestion.
Endoscopic Features Erefs
Erefs Scoring:
The Eosinophilic Esophagitis Endoscopic Reference Score (EREFS) quantifies endoscopic findings to standardize reporting and assess severity
It comprises several features, each scored from 0 (normal) to 2 (severe).
Edema:
Presence of esophageal edema
Score 0: normal
Score 1: mild edema, pale mucosa
Score 2: marked edema, pale mucosa, cobblestoning.
Exudates:
Presence of whitish exudates on the esophageal mucosa
Score 0: absent
Score 1: scattered exudates
Score 2: confluent exudates.
Furrows:
Longitudinal furrows or linear depressions in the esophageal lining
Score 0: absent
Score 1: superficial furrows
Score 2: deep furrows.
Strictures:
Presence of esophageal strictures or narrowing
Score 0: absent
Score 1: mild, easily traversed stricture
Score 2: severe, difficult-to-traverse stricture.
Total Erefs Score:
The total EREFS score is the sum of the scores for edema, exudates, furrows, and strictures
A higher score indicates more severe endoscopic findings
While not diagnostic alone, it is highly correlated with histological findings and clinical severity.
Management
Initial Management:
Initial management involves identifying and avoiding trigger foods and commencing medical therapy
For acute food impaction, endoscopic retrieval is necessary
Patients with severe dysphagia or strictures may require esophageal dilation.
Medical Management:
Topical corticosteroids (e.g., fluticasone propionate, budesonide) are the first-line medical therapy
These are often delivered via metered-dose inhalers or swallowed suspensions
Proton pump inhibitors (PPIs) may be used, especially if there is concurrent GERD or for their potential anti-inflammatory effects in EoE
Dietary elimination of common allergens (e.g., milk, egg, wheat, soy) is also a key component of management, often guided by allergy testing or empiric elimination diets.
Surgical Management:
Surgery is rarely indicated for EoE
Esophageal dilation is the primary endoscopic intervention for managing strictures
Surgical myotomy or other reconstructive procedures are reserved for very rare, refractory cases with severe complications like irreversible strictures or perforation.
Supportive Care:
Nutritional support is vital, especially in children with failure to thrive
Close monitoring of growth and hydration
Management of co-existing allergic conditions
Patient and family education regarding the chronic nature of EoE and adherence to therapy.
Complications
Early Complications:
Acute food impaction is a common and potentially emergent complication requiring urgent endoscopic intervention
Esophageal perforation can occur during dilation procedures, especially in the presence of severe strictures or friable mucosa.
Late Complications:
Chronic inflammation can lead to esophageal remodeling, resulting in fibrotic strictures, narrowing, and reduced esophageal motility
Long-term dysphagia, nutritional deficiencies, and failure to thrive can significantly impact quality of life
Increased risk of Barrett esophagus and esophageal adenocarcinoma is a theoretical concern in long-standing, untreated disease, though evidence in pediatrics is limited.
Prevention Strategies:
Adherence to dietary restrictions and medical therapy can prevent symptom exacerbations and reduce the risk of food impaction
Regular endoscopic surveillance and timely dilation of strictures can prevent significant esophageal narrowing
Prompt management of acute food impactions is crucial to avoid complications.
Prognosis
Factors Affecting Prognosis:
Early diagnosis and consistent management improve prognosis
Factors associated with a poorer prognosis include severe, long-standing disease, frequent food impactions, and development of esophageal strictures
Co-morbidities like severe asthma or widespread atopy can also influence outcomes.
Outcomes:
With appropriate treatment, most patients experience significant symptom improvement and resolution of esophageal inflammation
However, EoE is typically a chronic condition requiring ongoing management
Dietary modifications and topical corticosteroids are effective in controlling symptoms and preventing histological recurrence.
Follow Up:
Long-term follow-up is essential to monitor for symptom recurrence, adherence to therapy, and potential complications
Periodic endoscopic evaluations may be necessary, especially in patients with severe disease or suboptimal response to treatment, to assess histological remission and monitor for esophageal remodeling.
Key Points
Exam Focus:
Recognize EoE as a distinct immune-mediated esophageal disease
Understand the diagnostic triad: symptoms, biopsy findings (>=15 eos/hpf), and response to treatment
Master the EREFS score components (edema, exudates, furrows, strictures) and their significance in assessing endoscopic severity
Differentiate EoE from GERD endoscopically and histologically.
Clinical Pearls:
Always biopsy multiple levels of the esophagus in suspected EoE
Consider PPIs for their anti-inflammatory effects, not just for GERD
Dietary elimination is a cornerstone of management, often requiring a stepwise approach
Be aware of the pediatric-specific presentation of feeding difficulties and failure to thrive.
Common Mistakes:
Attributing all esophageal eosinophilia to GERD
Inadequate biopsy sampling (e.g., single biopsy from one level)
Failing to consider EoE in children with unexplained dysphagia or vomiting
Discontinuing therapy prematurely due to symptom improvement without confirming histological remission.