GI

Eosinophilic Esophagitis

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Endoscopic image of esophagus in a case of eosinophilic esophagitis. Concentric rings are termed trachealization of the esophagus. https://en.wikipedia.org/wiki/Eosinophilic_esophagitis

Background

Eosinophilic Esophagitis (EoE) is a clinicopathologic diagnosis. It is characterized by a chronic, immune-mediated esophageal dysfunction associated on biopsy with eosinophil-dominated inflammation. It was first identified in the 1960s and is increasing in incidence. It is believed that the disease is influenced by a combination genetic, environental and host immune factors. Those who are diagnosed often have a personal or family history of atopy, and it is slightly more common in males. 

Clinical Presentation

The presentation can be variable depending on severity of the condition and age of the patient. In younger patients, it can present as feeding intolerance, emesis, and abdominal pain. In teenagers and older patients, it can be mistaken for GERD and present as dysphagia, food impaction, and heartburn. The disease does not respond to a trial of PPIs in either age group. 

Differential diagnosis

  • GERD
  • Infectious esophagitis
                
    • HSV
      • Occurs most often in immunocompromised children, particularly transplant recipients, but there are reports in immunocompetent patients as well. Presents acutely with fever, odynophagia, and retrosternal pain. Diagnosis is confirmed with endoscopy and biopsy.
      • Patients are treated with acyclovir, with immunocompromised patients requiring a longer treatment course (2-3 weeks)
    • Candidiasis
           
      • Usually in patients with HIV, presents with odynophagia. Oral thrush may or may not be present. Treated with systemic antifungals
  • Medication-induced esophagitis
    • Caused by antibiotics (usually tetracycline), NSAIDs (aspirin), or other drugs (potassium chloride, iron). Inflammation is usually due to prolonged contact of the drug with the esophagus.
    • Rarely seen in children due to use of liquid medicines and not pills, but may occur in teenagers taking tetracycline for acne.
    • Presents with sudden odynophagia and retrosternal pain. Usually resolves without treatment.
  • Crohn’s disease

Workup

It is important to review the growth and development of the child in order to evaluate the severity of feeding intolerance. If there is a high clinical suspicion of EoE, an upper endoscopy with biopsies is required. The endoscopy may reveal esophageal rings, furrowing, and white papules (eosinophilic microabscesses)

 

Biopsies should be taken from both the distal and proximal esophagus to increase the sensitivity of diagnosis, as well as gastric antrum and duodenum biopsies in order to exclude other causes. On histology, a threshold of 15 eosinophils per high-powered field is used to diagnose EoE. 

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Micrograph showing eosinophilic esophagitis. H&E stain. https://en.wikipedia.org/wiki/Eosinophilic_esophagitis

Barium studies are not recommended for diagnosis, but may be helpful in identifying anatomic abnormalities. If the diagnosis of EOE vs GERD is not clear following endoscopy, esophageal pH monitoring may be indicated. 

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The barium swallow of the esophagus on the left side shows multiple rings associated with eosinophilic esophagitis. https://en.wikipedia.org/wiki/Eosinophilic_esophagitis

Patients with EoE should be referred to an allergist for evaluation due to high association with food allergies, asthma, rhinitis and eczema. The allergist may perform food allern skin tests and IgE levels. 

Treatment

A trial of PPIs should be used to help distinguish GERD from EoE. Acid suppression may be helpful in patients with EoE and concurrent GERD, however it will not treat true EoE. Children should be treated with 1mg/kg PPI, twice daily for 2-3 months as a test of clinical response.

Dietary therapy is an effective treatment in all children with EoE. Elemental formula is the most effective diet, but it is expensive and difficult to implement. Dietary restriction based on allergy testing is also effective, and will resolve symptoms in the majority of children.

Corticosteroids are the most effective medical treatment of EoE. Topical steroids are used instead of systemic steroids to reduce side effects. They are very effective in inducing remission, but they may need to be used as long-term therapy due to the high likelihood of relapse

 

References

  1. Bonis PAL and GT Furuta. Treatment of eosinophilic esophagitis. Up to Date; October 2012
  2. Bonis PAL and GT Furuta. Pathogenesis, clinical manifestations, and diagnosis of eosinophilic esophagitis. Up to Date; October 2012
  3. Bonis PAL and DF Zaleznik. Herpes simplex virus infection of the esophagus. Up to Date; December 2012
  4. Castell, DO. Medication-induced esophagitis. Up to Date; May 2011
  5. Furuta GT et al. 2007. Eosinophilic esophagitis in children and adults: a systematic review and consensus recommendations for diagnosis and treatment. Gastroenterology 133: 1342-1363
  6. Kauffman CA and JR Campbell. Overview of Candida infections in children. Up to Date; September 2012
  7. Liacouras CA et al. 2011. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol 128: 3-20.
  8. Noel RJ, PE Putnam, and ME Rothenberg. 2004. Eosinophilic esophagitis. N Engl J Med 351: 940-941.
  9. Putnam PE. 2008. Eosinophilic esophagitis in children: clinical manifestations. Gastroenterology Clinics of North America 37: 369-381.
  10. Rodrigues F et al. 2004. Herpes simplex virus esophagitis in immunocompetent children. Journal of Pediatric Gastroenterology and Nutrition 39: 560-563