Overview
Definition:
Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease characterized by the accumulation of eosinophils in the esophageal mucosa, leading to dysphagia, feeding difficulties, and other esophageal symptoms.
Epidemiology:
EoE is a leading cause of food-bound recurrent abdominal pain and dysphagia in children and adolescents
Prevalence is increasing globally, with estimates ranging from 1 in 1000 to 1 in 5000 children
It is strongly associated with other atopic conditions like asthma, allergic rhinitis, and atopic dermatitis.
Clinical Significance:
EoE significantly impacts a child's quality of life, affecting feeding, growth, and social development
Prompt diagnosis and effective management are crucial to prevent long-term complications such as esophageal strictures, food impaction, and Barrett esophagus
Understanding treatment options like dietary elimination and topical steroids is vital for pediatricians and gastroenterologists preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
In infants and young children: poor feeding, vomiting, failure to thrive, abdominal pain
In older children and adolescents: dysphagia (especially to solids), odynophagia, chest pain, food impaction, heartburn, nausea, vomiting.
Signs:
Physical examination is often unremarkable
In severe, long-standing cases, signs of malnutrition or esophageal stricture may be present
Associated atopic conditions (eczema, asthma, rhinitis) are common.
Diagnostic Criteria:
The diagnostic criteria for EoE include: 1
Symptomatic esophageal dysfunction
2
Esophageal eosinophil count of ≥15 eosinophils per high-power field (eos/hpf) in biopsy specimens, with no other identifiable cause for esophageal eosinophilia
3
Exclusion of other causes of esophageal eosinophilia such as proton pump inhibitor (PPI) responsive esophageal eosinophilia (PPI-REE).
Diagnostic Approach
History Taking:
Detailed history of feeding difficulties, food intolerances, symptoms of dysphagia, odynophagia, abdominal pain, and vomiting
Assess for associated atopic conditions and family history of allergies or EoE
Inquire about previous diagnoses and treatments for gastrointestinal or allergic symptoms.
Physical Examination:
General assessment for growth and nutritional status
Examination of skin for eczema, respiratory system for wheezing (asthma), and nasal passages for rhinitis
Abdominal examination for tenderness or masses
Oropharyngeal examination to rule out other causes of dysphagia.
Investigations:
Upper endoscopy with esophageal biopsies is the gold standard for diagnosis
Biopsies should be taken from the proximal and distal esophagus to assess for eosinophilic infiltration (typically ≥15 eos/hpf)
Allergy testing (skin prick tests or specific IgE) can help identify potential triggers
Esophageal manometry may be considered in select cases to evaluate motility disorders.
Differential Diagnosis:
Gastroesophageal reflux disease (GERD), infectious esophagitis (e.g., Candida), eosinophilic gastroenteritis, celiac disease, peptic strictures, achalasia, foreign body ingestion, functional dysphagia, and PPI-REE.
Management
Initial Management:
The initial management typically involves addressing symptoms and ruling out other causes
A trial of proton pump inhibitors (PPIs) is often recommended for 4-8 weeks to rule out PPI-REE
If PPIs are ineffective, or if EoE is strongly suspected based on symptoms and findings, further investigation and management for EoE are initiated.
Medical Management:
Two primary treatment modalities are: 1
Dietary Elimination: The six-food elimination diet (SFED), which removes milk, egg, wheat, soy, peanuts/tree nuts, and fish/shellfish, is highly effective
Other elimination diets, such as the four-food elimination diet (removing milk, egg, wheat, soy), are also used
Dietitian consultation is essential for implementation and monitoring
2
Topical Steroids: Swallowed topical corticosteroids (e.g., fluticasone propionate or budesonide) are delivered directly to the esophagus
Doses vary by age and formulation, but typical pediatric doses are 0.25-0.5 mg twice daily, swished and swallowed
These are effective in reducing esophageal eosinophilia and improving symptoms.
Surgical Management:
Surgical management is generally reserved for refractory cases or complications such as severe esophageal strictures
Esophageal dilation (balloon or Savary dilation) can be performed to relieve strictures
Surgical myotomy is rarely indicated for EoE.
Supportive Care:
Nutritional support is crucial, especially in children with feeding difficulties or failure to thrive
Long-term management may involve ongoing dietary counseling, regular endoscopies for monitoring, and management of comorbid atopic conditions
Psychological support for patients and families is also important.
Complications
Early Complications:
Food impaction is a common early complication requiring emergent endoscopy for removal
Acute esophageal obstruction can occur
Severe symptoms can lead to dehydration and malnutrition.
Late Complications:
Chronic inflammation can lead to esophageal remodeling, resulting in fibrosis, stricture formation, and increased risk of Barrett esophagus and, rarely, esophageal adenocarcinoma
Long-term feeding difficulties can impede growth and development.
Prevention Strategies:
Early diagnosis and consistent adherence to treatment (dietary elimination or topical steroids) are key to preventing complications
Regular follow-up with a gastroenterologist and allergist is essential
Prompt management of food impactions is critical.
Prognosis
Factors Affecting Prognosis:
Prognosis is generally good with appropriate management
Factors influencing prognosis include the severity of initial symptoms, adherence to treatment, presence of complications, and effectiveness of allergy management
Early intervention often leads to better long-term outcomes.
Outcomes:
Both dietary elimination and topical steroids are effective in inducing remission and improving symptoms in the majority of pediatric patients
Long-term outcomes depend on sustained remission and preventing complications
Some children may require lifelong management.
Follow Up:
Follow-up typically involves regular clinical assessment, evaluation of symptoms, and periodic endoscopies with biopsies to monitor disease activity and treatment response
The frequency of follow-up is tailored to the individual patient's needs and treatment plan
Long-term monitoring for esophageal remodeling and potential development of metaplasia or dysplasia is advised.
Key Points
Exam Focus:
Differentiate EoE from GERD and PPI-REE
Understand the diagnostic criteria (symptoms + biopsy findings)
Recognize the two main treatment pillars: dietary elimination (SFED) and topical steroids
Know the common complications like food impaction and strictures.
Clinical Pearls:
Always consider EoE in children with unexplained dysphagia or feeding issues, especially those with a history of atopy
Collaborate closely with dietitians for effective dietary elimination
Swallowed steroids bypass systemic absorption, minimizing side effects compared to oral corticosteroids
Emphasize patient/family education for adherence.
Common Mistakes:
Attributing all eosinophilic esophagitis symptoms to GERD without proper investigation
Inadequate biopsy sampling (number or location)
Failure to involve a dietitian in dietary elimination therapy
Inconsistent follow-up and monitoring of treatment efficacy.