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Eosinophilic Esophagitis: Serious But Misunderstood

From: ENT Today, August 2009

by Thomas R. Collins

PHOENIX-Eosinophilic esophagitis is a serious, growing problem that is little understood by many otolaryngologists, according to panelists discussing the disease. Some doctors who specialize in swallowing disorders acknowledged that they were not even aware of the problem, let alone well versed in diagnosing and treating the illness, according to results of a survey completed by 80 members of the American Broncho-Esophagological Association (ABEA).

The panelists, in an attempt to raise awareness of and educate their fellow doctors about the problem, talked about the disease's symptoms and treatments at the 88th annual meeting of the ABEA, conducted as part of the Combined Otolaryngology Spring Meeting.

Dana Thompson, MDMany of these symptoms are assumed to be associated with gastroesophageal reflux or laryngopharyngeal reflux. They often get treated for reflux disease, [but] the symptoms are refractory to traditional acid suppression.
-Dana Thompson, MD

Eosinophilic esophagitis, which occurs in both adults and children, is a disease in which eosinophils penetrate into the esophageal mucosa. In adults, it is associated with difficulty in swallowing; food impaction; chest pain; and stricture formation in the esophagus. In children, symptoms include vomiting and regurgitation, feeding disorders and oral aversion, food impaction and a general failure to thrive.

Many of these symptoms are assumed to be associated with gastroesophageal reflux or laryngopharyngeal reflux, said Dana Thompson, MD, Associate Professor of Otolaryngology at the Mayo Clinic. They often get treated for reflux disease, [but] the symptoms are refractory to traditional acid suppression.

Under the official definition, more than 14 eosinophils must be present per high-power field in an examination of the esophagus, in addition to esophageal symptoms and the exclusion of gastroesophageal reflux disease (GERD). Jeffrey Alexander, MD, Assistant Professor of Medicine in the Division of Gastroenterology at the Mayo Clinic College of Medicine, said it is not a great definition because patients with fewer than 14 eosinophils can have the same clinical syndrome, which may also be present in adult patient with concomitant GERD. I think our knowledge and understanding of this is evolving, Dr. Alexander said.

The disease was first described in 1977; there have been 338 publications about the disease in the English literature, including 240 since 2005. The first airway manifestations were described in 2000 by Orenstein et al. And the first report in otolaryngology literature about eosinophilic esophagitis was published in 2002 by Hartnick, Liu, Cotton and Rudolph (Ann Otol Rhinol Laryngol 2002;111:57).

It is still considered underreported, even though it becoming more common, possibly because of dietary changes and environmental factors, the panelists said.

Survey Says…

In the survey of ABEA members, nearly 79% of the respondents said they work in academic settings, 15% in private institutions, and just over 6% in multispecialty operations, according to results presented by Douglas Johnston, MD, of the Department of Otolaryngology-Head and Neck Surgery at Thomas Jefferson University Hospital in Philadelphia. Just under 59% treat adults, nearly 24% treat children, and almost 18% treat both.

Fifty of the 80 ABEA members who responded (62.5%) said they specialize in swallowing disorders, but 15 of them (18.8%) reported that they were not aware of the disorder. Pediatric otolaryngologists had significantly greater awareness, however.

Still, 61.5% of the respondents said that they have treated eosinophilic esophagitis and almost 30% of them said they have treated more than five patients for it.

Diagnoses of eosinophilic esophagitis were made by gastroenterologists in 62.5% of the cases, the respondents reported. The main factor leading to the diagnosis was laryngopharyngeal reflux refractory to acid suppression, which was an indicator just under 70% of the time. Trouble with swallowing solid foods was a factor 60% of the time, food impaction about 50% of the time, endoscopic signs of LPR refractory to acid suppression just over 30%, and a barium swallow with a ringed esophagus about 18% of the time, according to the survey results.

Signs of the disease seen during an endoscopic exam are linear streaking, mucosal plaques or nodularity, a ringed esophagus, an appearance similar to crepe paper, and stricture.

Four of the survey respondents reported esophagoscopy complications, including linear tears and perforation.

When otolaryngologists diagnose eosinophilic esophagitis, what do they do?

Of the 43 respondents who answered the question, 32 (just under 75 %) made a referral to a gastroenterologist, just over 72% had the patient swallow topical corticosteroids, around 51% used acid suppression and about 37% tried an elimination diet.

Symptoms and Diagnosis

Dr. Alexander said eosinophilic esophagitis is now about as prevalent as Crohn's disease. Estimates on how many people have the disease range from seven to 120 per 100,000, he said.

According to a 2006 study involving 325 patients (Eur J Gastroenterol Hepatol 2006;18:211), the average age of adults suffering from the disease is 35 to 45, with 52% of them having allergies and 31% with eosinophilia.

But the disease may be more common than is known clinically. A study in which 1000 random people in northern Sweden were studied endoscopically led researchers to conclude that 1.1% of the population in that country have more than 20 eosinophils per high-power field.

Esophageal eosinophilia may be much more common, and many of these people may be totally asymptomatic or minimally symptomatic, Dr. Alexander said.

Endoscopic findings that might signal eosinophilic esophagitis include rings, stricture, furrow or straight lines, white spots, and a small-caliber esophagus, he said. But in 30% of patients, endoscopic exams turned up normal.

Although food allergens and allergens in the air are suspected of triggering the production of eosinophils that lead to the disease's inflammation, the cause isn't known for sure.

Michael J. Rutter, MD, Associate Professor of Otolaryngology at the University of Cincinnati College of Medicine, laid out the benefits of early diagnosis of the disease, particularly in children with severe subglottic stenosis requiring laryngotracheal reconstructive surgery. In this population, if airway reconsructive surgery is attempted, when the patient actually has eosinophilic esophagitis, the odds of success are greatly diminished.

A 2003 study by Johnson et al in Transactions of the ABEA described the cases of 12 children suffering from subglottic stenosis who eventually were diagnosed with eosinophilic esophagitis with more than 24 eosinophils per high-power field.

In six of the children, 19 airway reconstruction attempts were made but failed before the eosinophilic esophagitis was diagnosed. The other six were diagnosed with the disease before reconstruction was attempted, Dr. Rutter said.

Researchers concluded that there was an 80% failure rate when patients had undiagnosed and untreated eosinophilic esophagitis, and an 80% success rate when the disease was discovered and properly managed.

He described a typical case: A six-year-old boy who was referred to him had been born prematurely, had had a patent ductus arteriosus ligation, and had been diagnosed with bronchopulmonary dysplasia.

He had left vocal cord paralysis and grade 3 subglottic stenosis, and single-stage laryngotracheal reconstruction surgery had failed at age three. There were no symptoms that suggested aspiration, reflux, dysphagia, or allergies-nothing that openly suggested eosinophilic esophagitis.

On the initial evaluation, it was observed on flex bronchoscopy that H. flu had colonized. Finally, an esophagogastroduodenoscopy (EGD) found nearly all the visual signs of the disease, including furrows and white patches. He was found to have 109 eosinophils per high-power field.

As management, the child swallowed fluticasone. He was re-evaluated three months later and found to have minimal distal esophageal inflammation and less subglottic inflammation, and the subglottic stenosis had improved to grade 2. He then had single-stage laryngotracheal reconstruction surgery with anterior and posterior costal cartilage grafts, which were nice and routine, good result, Dr. Rutter reported.

Is Routine Testing Warranted?

A study at Cincinnati Children's Hospital found that it might be well worth it to routinely test for eosinophilic esophagitis, considering the high cost of failed surgery in cases where it wasn't discovered.

The study looked at how many EGDs had to be done in that child population to find one child with the disease, what further tests had to be done after the disease was found, and the cost to treat one case of the disease.

It also looked the cost of surgical failure, including how many untreated patients failed to decannulate, how many additional surgeries were necessary to achieve decannulation, and the average cost of repeated surgeries.

The study found that the average cost of intervention to detect the disease early, before surgery, was $27,555, whereas the cost of failed surgeries, and the subsequent cost of further surgeries that resulted, amounted to an average cost of failure of $142,263.

The panelists said that otolaryngologists would be well served to sharpen their knowledge and openness to the possibility of eosinophilic esophagitis in their patients.

Dr. Thompson said, It does require knowledge, awareness, and a high index of suspicion.

News & Notes

FDA Advises Against use of Certain Zicam Products

The US Food and Drug Administration recently advised consumers to stop using three over-the-counter products marketed as cold remedies by Zicam, because they are associated with the loss of sense of smell.

The products are: Zicam Cold Remedy Nasal Gel, Zicam Cold Remedy Nasal Swabs, and Zicam Cold Remedy Swabs, Kids Size.

The FDA has received more than 130 reports of loss of sense of smell associated with the use of these three Zicam products. In these reports, many people who experienced a loss of smell said the condition occurred with the first dose; others reported a loss of smell after multiple uses of the product.

We are concerned that consumers may unknowingly use a product that could cause serious harm, said Janet Woodcock, MD, Director of the FDA's Center for Drug Evaluation and Research. The loss of smell, for example, can limit the ability to detect the smell of gas or smoke or other signs of danger in the environment.

The FDA has urged individuals who have suffered a loss of smell after the use of these products to see a health care professional, and to report adverse events via phone at 800-FDA-1088 or online at www.accessdata.fda.gov/scripts/medwatch/medwatch-online.htm .

The agency has also issued a warning letter to Matrixx Initiatives, maker of these products, stating that the products cannot be marketed without FDA approval.

©2009 The Triological Society

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