case report esophageal rupture as a primary manifestation...
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Case ReportEsophageal Rupture as a Primary Manifestation inEosinophilic Esophagitis
Natalia Vernon, Divyanshu Mohananey, Ehsan Ghetmiri, and Gisoo Ghaffari
Penn State Hershey Medical Center, 500 University Drive, MC H041, Hershey, PA 17033, USA
Correspondence should be addressed to Gisoo Ghaffari; [email protected]
Received 21 February 2014; Accepted 1 May 2014; Published 11 May 2014
Academic Editor: Timothy J. Craig
Copyright © 2014 Natalia Vernon et al.This is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and,histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, andchest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause foodimpaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to thefriability of the esophagealmucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave’s syndrome, as a primarymanifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initialmanifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 ofthe patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majorityof previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history ofdysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE.
1. Introduction
Eosinophilic esophagitis (EoE) is a chronic inflammatoryprocess and a clinicopathological diagnosis. EoE is clini-cally characterized by symptoms of esophageal dysfunction.Histopathologically, eosinophilic infiltration of the esopha-gus is the characteristic finding [1]. The diagnosis is madein symptomatic patients after a biopsy that confirms at least15 eosinophils per high power field (HPF) in the absence ofother causes [1–3]. EoE can present in children as well as inadults. Presenting symptoms include dysphagia, odynopha-gia, food impaction, heartburn, and chest or abdominal pain[3]. The main complication results from chronic inflamma-tion leading to a segmental or diffuse narrowing of esophageallumen. The narrowing may cause food impaction whichmay induce vomiting and/or require surgical interventions.Esophageal dilatation through endoscopic procedures toresolve food impactionmay lead to partial or complete tear ofthe already friable esophagus [4]. Partial or complete tear ofthe esophagus as a primary manifestation of EoE is rare. Thispaper presents two adult cases of spontaneous esophageal
rupture as a primary manifestation of EoE along with aliterature review of this rare complication.
2. Review of Two Cases
2.1. Case 1. A 48-year-old man presented to the emergencydepartment (ED) with progressive chest pain which inten-sified with inspiration. Earlier that day for the first time, hehad had an episode of impaction of a fish oil tablet which wasresolved by inducing vomiting. This is when the chest painstarted and progressively increased. In the ED, hewas initiallyworked up for a cardiac event and pulmonary embolus, bothof which were negative. A computed tomographic (CT) scanof his chest revealed thickening of the esophageal wall as wellas evidence of air/gas that appeared to dissect a plane of theesophagus. The CT also revealed extraluminal air suggestiveof esophageal perforation. An upper endoscopy with stentplacement was performed. The esophageal biopsy taken atthis time showed inflammation with increased eosinophilsin both the upper and lower esophagus parts. Histological
Hindawi Publishing CorporationCase Reports in MedicineVolume 2014, Article ID 673189, 5 pageshttp://dx.doi.org/10.1155/2014/673189
2 Case Reports in Medicine
sections of the esophagus showed basal cell hyperplasia,interstitial edema, and intraepithelial eosinophils up to 30eosinophils per high power field (HPF) in the proximal anddistal esophagus. The patient was discharged on a liquiddiet, antibiotics, and a proton pump inhibitor. The stentwas left in situ for 17 days and then was removed withoutdifficulty. A repeat endoscopy three months later showedbasal cell thickening, intercellular edema, and up to 20eosinophils/HPF in the proximal and distal esophagus. Thisendoscopy also revealed stricture in the total esophagus and aringed esophagus consistent with EoE. He was then referredto the allergy/immunology clinic for further evaluation.The clinical picture along with pathological findings wasconsistent with a diagnosis of EoE. Following the diagnosisof EoE, the patient began treatment with topical fluticas-one propionate with complete symptom resolution. Repeatendoscopy on topical steroid for several months showed upto 3 eosinophils/HPF and mild inflammation and the patienthad resolution of his symptoms.
The patient had a long standing history of dysphagia forthe past 20–25 years predominantly with solid foods. Heusually needed to chew his food for extended duration toswallow it with ease. He also reported intermittent symptomsof gastroesophageal reflux in the past which were neitherevaluated nor treated. He has had no history of food allergies,asthma, or allergic rhinitis. His past medical history was sig-nificant only for hypercholesterolemia. There was no familyhistory of atopic diseases. He consumes alcohol occasionallyand does not smoke. A skin prick test done as part of theallergy workup was mildly positive to shellfish.
2.2. Case 2. A 63-year-old woman presented to the ED withacute onset of chest pain, nausea, and vomiting after eatingdinner. Acute coronary syndrome and pulmonary embolismwere ruled out. She then developed several episodes of coffee-ground emesis while in the ED and 50cc of bright redblood was drained through a nasogastric tube. An emergencyupper endoscopy revealed a feline esophagus and a linearesophageal tear. No biopsies were taken at that time dueto acute bleeding. The esophageal tear was treated withepinephrine and three surgical clips. An esophagogastro-duodenoscopy was performed four weeks later. Histologicalsections of the esophagus showed basal cell hyperplasia andintraepithelial eosinophils greater than 20/HPF. The patientwas treated with a proton pump inhibitor and a swallowabletopical steroid intermittently with variable response. Shewas referred to the allergy/immunology clinic for furtherevaluation three years later. She was initially restarted on aproton pump inhibitor and repeat endoscopy 8 weeks latershowed possible esophageal rings consistent with EoE and 20eosinophils/HPF.The clinical picture along with pathologicalfindings was consistent with a diagnosis of EoE. She iscurrently awaiting reevaluation for additional medicationtreatment.
After the initial presentation of the esophageal tear, thepatient started to notice symptoms of dysphagia predomi-nantly with solid foods. At least once or twice weekly, shefelt that the food was impacted in her upper chest and this
sensation was relieved by inducing emesis. She also reportedintermittent symptoms of heartburn and oral pruritus withstrawberries and eggs. She has no history of asthma orallergic rhinitis. Her past medical history was significant foresophageal spasms, diverticulitis, hiatal hernia, hypercholes-terolemia, and hyperlipidemia. Family history was significantfor asthma, allergic rhinitis, and atopic dermatitis. She deniedany alcohol or tobacco use. A skin prick test done as part ofthe allergy evaluation was negative for all foods tested andpositive for trees and dust mites.
3. Literature Review
A PubMed based search was done combining the terms“eosinophilic esophagitis complications,” “esophageal rup-ture,” “Boerhaave’s syndrome,” and “Mallory-Weiss tear”(Table 1). We found that esophageal rupture had been previ-ously reported in 13 patients (11 adults and 2 children) andonly 1 of them had been previously diagnosed with EoE.A history of dysphagia and food impaction was seen inthe majority of these patients despite no prior diagnosis ofEoE. Our results were primarily in the adult population, asBoerhaave’s syndrome in children younger than 18 years ofage is rare andmore often is caused by trauma or is iatrogenic.
4. Discussion
This paper presents two adult patients with spontaneoustransmural esophageal rupture as the primary manifestationof EoE. The syndrome previously known as Boerhaave’ssyndrome is typically seen in middle aged men with 40to 60 years of age [5]. The clinical presentation usuallyinvolves a history of alcoholism or overindulgence in foods.The triad of vomiting, lower chest pain, and subcutaneousemphysema may also be seen [6]. Esophageal rupture is nota common primary manifestation of EoE. When it occurs asa primary manifestation, it is usually preceded by an episodeof food impaction with induced vomiting. Mucosal tears andlacerations have been reported in EoE patients suggestingincreased fragility of the esophageal mucosa from eosinophilinfiltration and subsequent remodeling. Eosinophil granulescontain cytotoxic proteins that can lead to tissue damage,and transmural eosinophilic infiltration has been shown inadult EoE patients [7]. However, despite the high frequencyof mucosal tears in these patients, studies have not showna higher frequency of esophageal perforation from dilationprocedures [8].
One study by Cohen et al. showed that risk factors forendoscopic complications included a prolonged history ofdysphagia, presence of esophageal stenosis, and a higherdensity of eosinophilic infiltration [9].These findings all haveimportant clinical implications as they highlight the impor-tance of dysphagia as a presenting symptom and also therole of the eosinophil. Early treatment of inflammation withtopical steroids may prevent complications such as segmentalesophageal stricture, narrowed lumen of the esophagus, andesophageal rupture since topical steroids can reduce theamount of eosinophils.
Case Reports in Medicine 3
Table1:Literature
review
ofesop
hagealruptureineosin
ophilic
esop
hagitis.
Reference
Patie
ntage
Previous
diagno
sisof
EoE
Priorsym
ptom
sPresentatio
nIm
aging/endo
scop
yTreatm
ent
Lucend
oet
al.[10]
36No
Interm
ittentesoph
agealsym
ptom
ssin
cechild
hood
with
frequ
ent
episo
deso
fcho
king
;seasonal
bron
chialasthm
aand
know
nsensitivityto
mustard,peanu
ts,grasses,andolivep
ollen
Meatimpactionresolved
byindu
cing
vomiting
follo
wed
byintenser
etroste
rnalpain
CTwith
contrastshow
edextensive
mediastinaland
subcutaneous
emph
ysem
aam
ongsto
ther
finding
ssug
gestiveo
fperfo
ratio
nof
esop
hagus;an
endo
scop
ydo
ne9mon
thslater
show
ednarrow
ingof
middlee
soph
agus
with
linearfurrowsa
ndcobb
lesto
ning
Thoracotom
ywith
closure
ofperfo
ratio
n
Lucend
oet
al.[10]
65No
Several-y
earh
istoryof
interm
ittent
esop
hagealsymptom
snot
requ
iring
treatment
Intensea
bdom
inalpain
after
chok
ingon
apiece
ofplum
which
was
relieved
after
indu
cing
vomiting
Endo
scop
yatthetim
esho
wed
adeepulcer
inthed
istalthird
ofthee
soph
agus
anda
CXRshow
edaleft
pleuraleffu
sionandfre
eaira
roun
dtheg
astricfund
us
Laparotomywith
closure
ofperfo
ratio
n
Predinae
tal.[11]
19No
Three-year
histo
ryof
dysphagiaa
ndseason
alallergies
Retching
follo
wingdinn
er,
follo
wed
byhematem
esis
andmele
na14
hourslater
Endo
scop
yrevealed
presence
oftwo
Mallory-W
eisstearsjustsup
eriortoGE
junctio
nandcorrugationof
esop
hagus
Endo
scop
icclipp
ing
with
epinephrine
injection
Quiroga
etal.[12]
24Yes
Allergyto
pollenandan
esop
hageal
stric
ture
inthem
iddlethird
ofthe
esop
hagussecon
dary
toeosin
ophilic
esop
hagitis
Progressivec
hestpain,
nausea,vom
iting
,and
fever
SpiralCT
show
edintram
ural
circum
ferentiald
issectio
nof
thoracic
esop
hagusa
ndperie
soph
agealm
ediastinal
abscessformation
Con
servative
managem
entw
ithantib
iotic
sand
parental
nutrition
;corticosteroid
therapy
was
initiated
after
abscessresolutionwas
demon
strated
onaC
TRo
bles-
Medrand
aetal.[13]
9No
Historyof
asthmaa
ndinterm
ittent
solid
food
dysphagia
Chestp
ain,
pyrosis
,and
fevera
ftera
nepiso
deof
food
blockage
CXRwas
norm
al;C
Tshow
eda
retro
esop
hagealperfo
ratio
nwith
perie
soph
agealfl
uidcollection
Con
servative
managem
entw
ithantib
iotic
s
Riou
etal.
[14]
26No
Long
histo
ryof
dysphagiaa
ndesop
hagealob
structionas
achild
andalso
hadhisto
ryof
idiosyncratic
reactio
nsto
cham
pagn
eand
red
wine
Severe
constant
epigastric
pain
follo
wingfood
impaction
CXRconfi
rmed
airincervicaltissues
and
CTshow
edpn
eumom
ediastinum
;gastrografi
nsw
allowshow
edfre
econ
trast
inperiton
ealcavity
;sub
sequ
entend
oscopy
show
edste
nosis,circ
ular
rings,and
an8c
mlong
long
itudinaltearo
nther
ight
lateral
wallofthe
esop
hagus
Subtotal
esop
hagectom
yand
cervical
esop
hagogastr
icanastomosiswere
perfo
rmed
4 Case Reports in Medicine
Table1:Con
tinued.
Reference
Patie
ntage
Previous
diagno
sisof
EoE
Priorsym
ptom
sPresentatio
nIm
aging/endo
scop
yTreatm
ent
Gilese
tal.
[15]
12No
N/A
Sore
throat,dysph
agiawith
solid
s,andretro
sternalpain
thatpersisted
after
chok
ing
onap
iece
ofcorn
CTwith
IVcontrastrevealed
asmall
containedperfo
ratio
nwith
outm
ediastinitis
orpleuraleffu
sion
Non
operative
managem
entw
ithbroadspectrum
antib
ioticsa
ndtotal
parentalnu
trition
was
used
Prasad
etal.
[16]
54No
Interm
ittenth
istoryof
solid
food
dysphagia,heartburn,
andasthma
Presentedwith
retro
sternal
pain
after
anepiso
deof
food
impaction;
heindu
ced
emesisto
relieve
thefoo
dim
paction
CTdemon
strates
freea
irin
the
mediastinum
with
pleuraleffu
sions
and
inflammatorychangesa
roun
dthed
istal
esop
hagus;up
pere
ndoscopy
revealsa
large
tear
inthed
istalesop
hagus
Con
servative
managem
entw
ithIV
antib
ioticsa
ndbo
wel
rest
Spahnetal.
[17]
41No
Historyof
multip
leepiso
deso
fdysphagia
Presentedwith
dysphagia
18ho
ursa
ftering
estin
gacetam
inop
hen
Esop
hago
scop
yshow
edstr
icture
and
hemorrhage;CT
show
edmediastinalair
consistentw
ithperfo
ratio
nNot
mentio
ned
Coh
enetal.
[18]
56No
Historyof
heartburn,
asthma,and
season
alallergies
Progressiven
ausea,
vomiting
,and
epigastric
andchestp
ain
CTscan
revealed
aira
ndflu
idsurrou
nding
thee
soph
agus
Closureo
fthe
perfo
ratio
n
Gom
ez-
Senent
etal.
[19]
35No
N/A
Dysph
agia,vom
iting
,and
epigastricpain
Upp
erendo
scop
yrevealed
impacted
bean;
CTscan
show
edfre
eliquidarou
ndesop
hagusa
ndpn
eumom
ediastinum
Con
servative
managem
entw
ithantib
iotic
s
Ligouriet
al.[20]
32No
Mild
solid
food
dysphagia
Presentedwith
food
impaction
Upp
erendo
scop
yrevealed
mucosal
disrup
tion;CT
scan
show
edcircum
ferential
dissectio
nandmediastinalemph
ysem
a
Righttho
racotomy,
totalesoph
agectomy
with
esop
hagogastroplasty,
andjejuno
stomy
Straum
ann
etal.[21]
28No
Ten-year
histo
ryof
dysphagia
Severe
vomiting
and
hematem
esis
Upp
erendo
scop
yshow
eddeep
mucosal
tear;C
Tscan
show
edpn
eumom
ediastinu
mSurgeryandantib
iotics
Case Reports in Medicine 5
Our two cases and the literature review revealed that themajority of EoE patients diagnosed only after presenting witha nontraumatic esophageal perforation had a previous historyof dysphagia. Patients with a history of dysphagia shouldwarrant an evaluation for EoE as well as patients who presentwith spontaneous esophageal perforation to potentially avoidother complications of untreated EoE.
Conflict of Interests
The authors declare that there is no conflict of interestsregarding the publication of this paper.
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