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Path Lab
Could It Be Eosinophilic Esophagitis?
Julia Dahl
Publish Date: May 13, 2008

If you handle lots of GI cases, you may have noticed a new cluster of younger patients having difficulty swallowing, or even showing food impaction. After the upper GI endoscopy comes the unfamiliar term in the pathology report: "eosinophilic esophagitis." Here's what you need to know about this emerging disease classification.

New ICD-9 Codes Take Effect in October
Effective Oct. 1, these ICD-9-CM diagnosis codes for eosinophilic gastrointestinal disorders apply.

Disease

New ICD-9

Current ICD-9

Eosinophilic esophagitis

530.13

530.19

Eosinophilic gastritis (excludes eosinophilic gastroenteritis)

535.7

535.40 or 535.41

Eosinophilic gastroenteritis and eosinophilic enteritis

558.41

558.4

Eosinophilic colitis

558.42

558.4

Disease and pathogenesis
Patients with eosinophilic esophagitis (EE) may be any age and generally present with dysphagia, food impaction and inconsistent response to therapies directed at treating GERD. There's a strong association with a personal or family history of allergy (atopy, asthma and positive skin prick tests, for example).

At endoscopy, the esophagus may have characteristic feline appearance, circumferential rings, rice paper appearance and punctate white plaques resembling Candida esophagitis. About 10 percent of patients appear normal. Swallowed steroids, stricture dilation, dietary restriction or a combination of these may be effective treatment options.

In addition to performing esophagogastroduodenoscopy (EGD), be sure to biopsy these patients. From GERD to upper gut inflammatory bowel disease, many other diseases may cause dysphagia and increased eosinophils on the surface of the esophagus. Also, you'll need a tissue diagnosis to estimate the risk of complications for dilation of strictures.

Establishing the diagnosis
Provide clinical history to the GI pathologist, including personal and family history of allergy, medications, presenting symptoms, CBC results and responses to GERD therapies. You may request an eosinophil count or ask the pathologist to comment on the presence or absence of EE features.

During EGD, have several biopsy bottles ready. Perform biopsies as suggested in "Recommended Biopsy Distribution" on page 90. Because EE is a notoriously patchy and temporal disease, a "negative" or "normal" biopsy doesn't exclude the possibility of EE. If the patient's symptoms persist or can be correlated to a particular food substance or exposure, repeat EGD with additional biopsies.

Perform multiple biopsies even if the mucosa looks completely normal. At least 10 percent of patients with EE have completely normal-appearing esophageal mucosa. Recent studies have also shown that 90 percent sensitivity requires evaluation of six separate pieces of tissue, given the patchy nature of the process.

Some tissue will remain confounding. Other tissue will feature more than one disease (asthma, EE and iatrogenic GERD). In this case, the submitting GI may request referral to a GI pathologist with experience in distinguishing among EE, GERD, upper gut IBD, infection EGE and others.

Dilation of strictures in patients with EE carries an increased risk of laceration and rupture, due to an unusual type of fibrosis that occurs. When dilation is scheduled on a patient known to have EE, proceed gently and cautiously, expecting complications.

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