How is eosinophilic esophagitis diagnosed and managed in children and young adults?

Eosinophilic Esophagitis: Diagnosis and Management in Children and Young Adults

Diagnostic Approach

Eosinophilic esophagitis (EoE) should be suspected in all children with upper gastrointestinal symptoms and in adults presenting with dysphagia or food bolus obstruction, requiring endoscopic biopsy confirmation showing ≥15 eosinophils per 0.3 mm² field. 1

Clinical Presentation by Age

In children, symptoms vary dramatically by age and are often non-specific:

• Children under 6 years: Feeding difficulties (median age 2.8 years), failure to thrive, vomiting (median age 5.1 years), and diarrhea (median age 6-7 years) 1
• Children 6-12 years: Abdominal pain (median age 9.0 years), dysphagia (median age 10-11.1 years) 1
• Adolescents and young adults: Dysphagia, food impaction (median age 12 years), heartburn, and chest pain 1, 2

Adults typically present with solid food dysphagia in young men with atopic predisposition, and food impaction requiring endoscopic intervention is common. 2

Endoscopic Biopsy Requirements

All children undergoing endoscopy for upper GI symptoms must have esophageal biopsies taken, as macroscopic appearance is unreliable in pediatric patients. 1

In adults, biopsies are mandatory if:

• Endoscopic signs of EoE are present (furrows, rings, white plaques, mucosal edema, fragile mucosa, narrow caliber esophagus, strictures) 1
• Dysphagia or food bolus obstruction exists even with normal-appearing esophagus (7-17% of EoE cases appear endoscopically normal) 1

Obtain minimum 3-4 biopsies from distal and 3-4 from proximal esophagus, off PPI therapy for at least 2-4 weeks. 3

Key Diagnostic Pitfalls

GERD and EoE are not mutually exclusive and coexist in at least 10% of patients. 1 Consider GERD coexistence when endoscopic or clinical features suggest both conditions, as 56% of EoE patients show excess acid exposure on pH monitoring. 1

Endoscopists commonly underestimate strictures and narrow-caliber esophagus in EoE, which may be difficult to detect by simple endoscopy. 1

Treatment Strategy

First-Line Pharmacotherapy

Proton pump inhibitors are the recommended first-line treatment, achieving histological remission in 54.1% and symptom improvement in 64.9% of pediatric EoE patients. 4

• Dosing: Omeprazole 20 mg twice daily for 8-12 weeks 5
• If PPI causes unwanted side effects (diarrhea, GI infections, magnesium deficiency), switch to topical steroids or dietary therapy 1

Second-Line: Topical Corticosteroids

Topical corticosteroids (budesonide and fluticasone propionate) are significantly superior to placebo in decreasing eosinophil infiltration and relieving symptoms, with viscous budesonide formulas appearing most effective. 4, 6

Important monitoring in children and adolescents:

• Monitor bone mineral density and adrenal suppression during long-term topical steroid use 1, 6
• Candida infection occurs in a small proportion; manage with topical antifungals while continuing topical steroids 1, 6
• Systemic side effects have not been documented during long-term treatment in adults 1

Dietary Management

A two-food elimination diet (TFED) eliminating milk +/- wheat or egg for 8-12 weeks is the recommended initial dietary approach. 5

Dietary elimination must be conducted under supervision of an experienced dietitian throughout both elimination and reintroduction phases. 5, 6

Alternative dietary approaches with varying restriction levels:

• Amino acid-based elemental diet and six-food elimination diet provide best cure rates but are highly restrictive 4
• Four-food elimination (milk, gluten-containing cereals, egg, legumes) shows encouraging results with less restriction 4

The psychological impact of dietary therapy must be discussed with patients and carers, as anxiety and depression affect patients due to persistent symptoms and social restrictions. 1, 6

Monitoring and Follow-Up

Treatment Response Assessment

Endoscopy with biopsy while on treatment is mandatory to assess response, as symptoms do not always correlate with histological activity. 5, 6

Repeat endoscopy should be performed:

• No sooner than 4 weeks after therapeutic intervention 1
• When symptoms recur during treatment to assess for refractory disease, undetected fibrostenotic disease, or treatment complications like candidiasis 1

Clinical symptoms typically improve within 7 days, with histological improvement within 4 weeks of corticosteroid therapy. 5

Long-Term Management

Regular clinic visits are required to assess symptoms, compliance with therapy, and adverse effects. 1, 6

For asymptomatic patients with persistent esophageal eosinophilia, repeat endoscopy and/or barium swallow can be obtained every 2-3 years to evaluate for progressive disease. 1

Refractory Disease and Complications

Patients with EoE refractory to treatment and/or significant concomitant atopic disease should be jointly managed by a gastroenterologist and specialist allergy clinic. 1, 5, 6

Mast cell stabilizing agents, leukotriene antagonists, and antihistamines are not recommended for primary management of EoE symptoms, though they remain useful for co-existing allergic disease. 1

Perforation Management

In case of EoE perforation, CT contrast study should be performed to assess extravasation degree. 1

If limited extravasation exists, manage conservatively with multidisciplinary input from gastroenterology, surgery, and radiology specialists. 1

Transition of Care

Formal transition from pediatric to adult services may improve symptom control, concordance with therapy, and reduce emergency presentations. 1

Key transition considerations:

• 81% of young adults show symptomatic improvement compared to childhood, but 37% still report dysphagia 1
• Non-concordance with therapy and emergency presentations commonly occur after leaving pediatric care 1
• Introduce questionnaires to assess knowledge and readiness for transition, involving multidisciplinary teams (gastroenterologists, dietitians, allergists, psychologists) 1