DDSEP® 8 Quick Quiz

March 2017 Quiz 1

Q1: A 14-year-old boy with a history of mild seasonal allergies presents to the emergency room with chest pain and discomfort after eating a steak 2 hours ago. He is having trouble swallowing and feels there is a piece of food stuck in his chest, and he points to his mid-sternum. He tells you this has happened several other times over the past year, and he felt better after he vomited. His physical examination is entirely normal. He is taken to the operating room for emergency endoscopy where a large piece of steak is removed from his mid-esophagus, without complication. Biopsies of the mid-esophagus demonstrate acute and chronic inflammatory changes in the lamina propria with 35 eosinophils per high-powered field.

What is the most likely diagnosis?

Eosinophilic esophagitis

GERD.

Inflammatory bowel disease.

Fungal esophagitis.

Achalasia.

Q1: Answer: A

Critique: This is a classic presentation of eosinophilic esophagitis (EoE). As many as half of older children with food impactions suffer from EoE. EoE is characterized by a severe, eosinophilic infiltration of the esophagus that may respond to acid inhibition, systemic or topical steroid therapy, or removal of dietary allergens. Epidemiologic studies suggest a rising incidence in the United States in both children and adults, with at least one case occurring in every 10,000 children each year. Treatment is aimed at alleviating symptoms and healing esophageal inflammation. Allergy testing should be performed at the time of diagnosis; however, radioallergosorbent tests and skin-prick tests are often negative, and only half of affected children have a antecedent history of other allergic symptoms.

A five-food elimination diet can be helpful for many affected children and adults, although adherence to the diet can be difficult. There is a group of affected children who respond to high doses of proton pump inhibitors, and most patients respond to either systemic or topical steroid therapy. Even with therapy, some patients go on to develop esophageal strictures and may need serial or repeated dilatations.

While eosinophilic infiltration and inflammation may be present with gastroesophageal reflux disease and associated esophagitis, the number of eosinophils seen in this boy’s biopsies is much more consistent with EoE. Moreover, stricture formation as a result of peptic esophagitis in a child this age would be extremely rare. While inflammatory bowel disease may be associated with eosinophilic infiltration of the intestinal tract, isolated esophageal Crohn’s disease would be extraordinarily rare.Our patient has no history of any immune deficiency or steroid use that would predispose to fungal esophagitis. Achalasia typically presents with gradually worsening symptoms, and the obstruction would be at the lower esophageal sphincter, not in the mid-esophagus.

Reference

1. Liacouras C., Furuta G., Hirano I., et al. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol. 2011;128:3-20.
2. Furuta G., Liacouras C., Collins M., et al. Eosinophilic esophagitis in children and adults: A systematic review and consensus recommendations for diagnosis and treatment. Gastroenterology. 2007;133:1342-63.

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