Eosinophil, esophagitis, dysphagia, esophageal
obstruction, allergic conditions.
This manuscript reports on three patients
with eosinophilic esophagitis presenting with chronic symptoms
of solid food dysphagia. Eosinophilic esophagitis is an uncommon
disorder, characterized by chronic dysphagia without associated
symptoms of gastroesophageal reflux. It is often accompanied
by concomitant allergies, atopy and/or asthma (1-3). To make
the diagnosis of eosinophilic esophagitis requires a keen
index of suspicion, especially among persons with atopy and
dysphagia. Endoscopic findings include multiple mucosal rings
in a narrow, non-compliant esophagus with or without vertical
groves or lines (2-6). Contrast esophagram is remarkable for
"slender" esophagus, or focal segmental stricture (7). Eosinophilia
seen in biopsy specimens from the esophagus are required for
the diagnosis. Although the number of eosinophils per high
power field necessary for the diagnosis varies somewhat from
author to author, greater than 24 eosinophils per high power
field is generally considered diagnostic for the condition.
(1-3). Treatment includes esophageal dilation, systemic and
topical corticosteroids, and medical management of coexisting
atopic conditions (1,2,5,6).
Methods for Image Capture/Processing
Olympus Evis image management system used
for endoscopic images; Sony CD Mavica 3.3 megapixel for radiographic
images; and Olympus AO51 (BX51) microscope with U-PMTV collimator
and UCMAD3 camera for histologic images.
A 21-year-old active duty soldier was referred
to Fitzsimons Army Medical Center, Aurora, Colorado for evaluation
of "GERD" and chronic dysphagia. The patient was of good health
and upon specific questioning denied symptoms of heartburn.
He related a vivid history of solid dysphagia that began at
12 years of age. His first episode of severe dysphagia "food
impaction" occurred during a Thanksgiving dinner with turkey.
The food impaction was relieved with self-induced vomiting.
From that date on, the patient became aware that solids that
were poorly chewed or too rapidly swallowed would lead to
dysphagia and occasional food impactions. The only occasions
that the patient related symptoms of retrostenal discomfort
that were consistent with heartburn, occurred after self self-induced
vomiting used to relieve food impactions. Interestingly, the
patient denied any symptoms of heartburn. Also, the patient
had adapted his diet to avoid certain solids and had become
a "slow" eater, which diminished symptomatic episodes. The
patient presented to his unit medical officer because of the
increased frequency of symptoms of hurried meals often required
by his rapid deployment unit.
The patient's medical history was remarkable for tonsillectomy and inguinal hernia repair at 5 yrs and 8 yrs, respectively. The patient has known allergies to cats and dogs and was bothered by seasonal rhinitis, treated with antihistamines.
Physical examination revealed a very healthy 24-year-old male standing 74 inches and weighing 170 lbs. Laboratory examination showed a normal complete blood count with peripheral smear and a normal chemistry panel.
Barium esophagram revealed a subtle mid-esophageal
segment of "coiled stenosis" appearance best seen after passage
of the barium column (Figures 1A and 1B). Upper endoscopy
confirmed the "coiled spring" appearance of the esophagus
~30 cm from the incisor teeth (Figures 2A and 2B).
The luminal stenosis precluded passage of
the Olympus GIF-100 series endoscope beyond the stenosis.
The "coiled spring" stricture was dilated with through-the-scope
(TTS) balloon dilators sizes 8 mm and 10 mm. After the procedure,
the patient did complain of moderate chest discomfort and
odynophagia that resolved quickly after administration of
an intravenous narcotic. The patient was treated with Prilosec
20 mg bid and returned for repeat endoscopy and biopsy at
one week and two week intervals. Esophageal biopsies of the
"coiled spring" stenosis were remarkable for increased numbers
of eosinophils (Figures 3A and 3B). Two Maloney dilators were
passed with the second and third endoscopies (sizes 34, 36
French and then sizes 36, 38 French, respectively). With each
dilation the patient complained of moderate post procedure
chest pain requiring narcotics. The patient was followed at
1 and 6 months and related improved swallowing and his desire
to avoid further esophageal dilatation due to the post procedure
chest pain experienced with each procedure.
An 18-year-old male soldier was seen for
evaluation of dysphagia at Eisenhower Army Medical Center,
Augusta, Georgia. This patient related symptoms of solid dysphagia
since 10 years of age. He had never experienced symptoms of
heartburn and episodes of dysphagia were exclusively associated
with the ingestion of "poorly" chewed solids. The
patient had no significant medical or surgical history. He
was allergic to seafood, which caused violent vomiting. Laboratory
tests revealed a normal, complete blood count. Esophogram
showed the esophagus to be "narrow." Upper endoscopy
was performed and remarkable for a narrow non-compliant esophagus
with a "coiled spring" and the box appearance for
the length of the esophagus, (Figure 4). During endoscopy,
the endoscope was snug to the wall of the esophagus, but allowed
passage of the instrument into the stomach and duodenum with
only mild resistance. Upon withdrawal of the endoscope, a
linear mucosal tear is evident along the entire length of
the esophagus (Figure 5). After the procedure, the patient
voiced no complaints of odynophagia or pain. He returned at
two weeks for endoscopy with biopsy and Maloney bougie dilation,
sizes 34 and 36 French. Histologic findings of the esophagus
were remarkable for increased numbers of eosinophils >
24 per HPF. At four weeks, the patient returned for additional
esophageal dilation with 36 and 38 French Maloney bougies;
on this occasion, the patient complained of transient post
procedure chest discomfort. Symptoms resolved promptly without
need for medication. The patient was followed for one year
and experienced sustained improved swallowing.
A 20-year-old soldier was referred to Evans
Army Hospital, Colorado Springs, Colorado for evaluation solid
food dysphagia. He denied any symptoms of heartburn and related
the onset of symptoms to occur at about 13 years of age. With
slow and careful chewing of his food, the patient rarely experienced
dysphagia. During his enlistment in the Army, his meals were
frequently hurried requiring avoidance of solids and the need
for more liquids with meals. The patient did have a diagnosis
of exercise induced bronchospasm and allergies to pollens
and mold. He had been treated with an inhaled bronchodilator
before physical fitness testing for the last 12 months. Antihistamines
were used during the summer and fall months. A barium esophagram
performed by his referring primary care giver was reported
to be remarkable for "narrow" esophagus.
Upper endoscopy revealed diffuse esophageal rings with a parallel
band of vertical lines running the entire length of the esophagus,
(Figure 6). Biopsies of the esophagus showed increased numbers
of eosinophils. The patient underwent two esophageal dilation
sessions spaced one month apart with Maloney bougie dilators
(34 and 36 French and 36 and 38 French, respectively). Mild
chest pain and odynophagia was experienced after the second
dilation session. This patient was followed for one year with
marked improvement in swallowing.
This case series reports on three adult
males presenting with solid food dyphagia found to have eosinophilic
esophagitis. The clinical features of these patients is summarized
in Table 1. The mean age at onset of symptoms and age at initial
medial presentation were 12 yrs and 20 yrs, respectively.
Each related adaptation of his diet to slow and careful mastication
of food and avoidance of hasty meals or distractions while
eating. Variable degrees of allergic symptoms were evident
among all of the patients in this report, to include allergies
to pets, pollen, and seafood and exercised induced bronchospasm.
Complete blood counts were normal among all patients. None
of the patients described symptoms of gastroesophageal reflux
and manometry, and ambulatory esophageal pH testing performed
on two of the patients was normal. Esophagram was noted to
be remarkable for either segmental of diffuse esophageal narrowing
in each of the patients. The endoscopic appearance of the
esophagus included segmental to continuous esophageal stenosis.
The "coiled spring" appearance and
"crepe paper" like mucosal non-compliance
is unique, and associated with a unique predisposition to
tear along the length of the coiled spring. Although esophageal
dilation was associated with immediate post procedure pain
and discomfort in all of the patients, no serious complication
from the cautious dilations occurred, and relief from dysphagia
was observed to be durable up to one year of follow follow-up.
Heart burn symptoms
Age at Onset of Dysphagia
|Yes, to pollen, cats and dogs
||Yes, to seafood
|| Yes, to pollen and exercise induced bronchospasm
|| "Narrow esophagus"
||"Coiled spring," and "vertical
Pain with Esophageal dilation
Symptom resolution after dilation
Table 1: Summarization of clinical features on three adult males presenting with solid food dyphagia found to have eosinophilic esophagitis
Dobbins, et al. described the first case
of eosinophilic esophagitis more than 20 years ago (4). Since
then, ever increasing numbers of case reports, cases series,
and even a long term follow-up of 30 adult patients with primary
eosinophilic esophagitis have been published (1,2,8,10,11).
The disorder is more commonly reported among males, often
with a prolonged duration of symptoms before clinical recognition.
Symptoms of dysphagia occur exclusively with solids and coincident
atopic conditions are common (1-3).
Eosinophilic esophagitis is characterized by an inflammatory
reaction confined to the esophagus. The expression of eosinophils,
T cells, and mast cells in the mucosal of eosinophilic esophagitis
is characteristic of the disorder and strikingly similar to
that seen with in the lung of asthmatics (12). Straumann,
et al. have shown that the TH2 cytokine and IL-5 allergic
response to inhaled allergens is consistently seen in these
patients. This suggests a link between allergic asthma and
eosinophilic esophagitis (3,12).
Several large series of children with eosinophilic esophagitis
have been reported (6,11,13). Carefully conducted skin testing
for food allergies among these children have identified food
allergies in 19/26 (73%) by pin prick and 21/26 (81%) by patch
testing. With meticulous dietary elimination, these investigators
showed marked and durable improvement in dysphagia symptoms
in 18 of the 24 children with positive pinprick or patch testing.
Repeat esophageal biopsy after elimination of food allergies
showed normalization of esophageal mucosa in 12 of 24 and
decrease in mucosal eosinophil count from 55 eosinophils per
HPF to 8.4 per HPF (11). This further supports the theory
that extrinsic allergens may be responsible for some cases
of eosinophilic esophagitis.
Long standing eosinophilic esophagitis appears to be associated
with a wide gamut of radiographic and endoscopic findings.
Barium esophagrams have shown short and high grade stenosis
to very subtle narrowing often extending the entire length
of the esophagus (7,10). Importantly, the finding of a long
"small caliber" esophagus reported by Vasilopous,
et al. appears to predict the probable occurrence of long
painful mucosal tears with esophageal dilation using large
diameter dialators (10). Many endoscopists, now recommend
cautions dilation of patients with eosinophilic esopahgitis
with smaller dilators than usually used with peptic or acid
reflux related esophageal strictures (1). However, as seen
in the patients described in this report, transient post esophageal
dilation chest pain and odynphagia are common even with gentle
and conservative esophageal dilation.
The resemblance of the inflammatory reaction of eosinophilic
esopahgitis to allergic asthma has lead many to attempt treatment
with systemic or topical corticosteroid and leukotriene antagonists.
Langdon and others have reported a decrease in the number
of mucosal eosinophils with such therapy, but dysphagia symptoms
do not improve without dilation to relived esophageal non-compliance
In summary, the diagnosis of eosinophilic esophagitis should
be considered among persons with coincident atopic conditions
and symptoms of solid dysphagia. Similarly, the absence of
reflux symptoms in the person with solid dysphagia should
spark the idea of possible eosinophilic esophagitis and careful
inquiry about atopic conditions should follow. Esophagram
findings of subtle diffuse esophageal narrowing or a non-neoplastic
or reflux related esophageal stenosis should suggest the diagnosis.
These signs as well as the described endoscopic findings of
coiled spring ("feline") esophagus, segmental stricture,
and vertical lined esophagus should prompt esophageal biopsy
of the mid and proximal esophagus. The pathologist should
be alerted to the possible diagnosis of eosinophilic esophagitis,
so that the number of eosinophils per high power field is
carefully counted, as acid reflux esophagitis has been associated
with increased numbers of eosinophils, but usually < 5
per HPF. The role for elimination diets, budesonide, mucosal
injection with corticosteroids, and montelukast await further
study to define their role in the management of this disorder.
Lastly, cautious esophageal dilation with smaller dilator
diameters appear to be essential for the successful relief
of mucosal "stiffness" causing dysphagia and topical
or oral corticosteroids appear to be important in prevention
of recurrent eosinophilic inflammation and relapse.
1. Kaplan M, Mutlu EA, Kakate S, Bruninga K, Losurdo J, Losurdo J, and Keshavarzian A. Endoscopy in eosinophilic esophagitis:”feline” esophagus and perforation risk. Clinical Gastroenterology and Hepatology. 2003;1:433-7.
2. Straumann A, Spichtin H, Grize L, Bucher KA, Beglinger C, Simon H. Natural History of primary eosinophilic esophagitis: A follow-up of 30 adult patients for up to 11.5 years. Gastroenterology. 2003:125:1660-9.
3. Dobbins JW, Sheehan DG, Behar J. Eosinophilic gastroenteritis with esophageal involvement. Gastroenterol. 1977;72:1312-6.
4. Attwood SE, Smyrk TC, DeMeester TR, Jones JB. Esophageal eosinophilia with dysphagia: a distinct clinicopathologic syndrome. Dig Dis Sci. 1993:38:109-16.
5. Vitellas KM, Bennett WF, Bova JG, Johnston JC, Caldwell JH, Mayle JE. Idiopathic Eosinophilic Esophagitis (IEE). Radiology. 1993;186:789-93.
6. Gupta S, Fitzgerald JF, Chong SK, Collins MH. Vertical Lines in Esophageal Mucosa: a true endoscopic manifestation of esophagitis in children. Gastrointest Endosc. 1997;45:485-9.
7. Langdon DE. Corrugated Ringed Esophagus. Am J Gastroenterol. 1993;88:1461.
8. Langdon DE. Fluticasone in eosinophilic corrugated ringed esophagus. Am J Gastroenterol. 2001;96:926-7.
9. Vasilopoulos S, Murphy P, Auerbach A, Massey BT, Shaker R, et al. The small-caliber esophagus: an under appreciated cause of dysphagia for solids in patients with eosinophilic esophagitis. Gastrointestinal Endosc 2002;55:99-104.
10. Spergel JM, Beausolel JL, Marcarenhas M, Liacouras CA. The use of skin prick and patch test to identify causative foods in eosinophilic esophagitis. J Allerg and Clin Immunol. 2002:109:304.
11. Straumann A, et al. Idiopathic Eosinophilic Esophagitis is associated with a Th 2-type allergic inflammatory response. J Allerg Clin Immunol. 2001;108:1900.
12. Liacouras CA, Wenner WJ, Brown K, Ruchelli E. Primary eosinophilic esophagitis in children: successful treatment with oral steroids. J Pediatr Gastro Nutr. 1998:26:380-5.
13. Dobbins JW, et al. Gastro 1977;72:1312-6.
14. Landres RT, Kuster GG, Strum WB. Eosinophilic esophagitis in a patient with vigorous achalasia. Gastroenterology. 1978;74:1298-301.