Case Report Esophageal Rupture as a Primary Manifestation...

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.

References

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[3] G. T. Furuta, C. A. Liacouras, M. H. Collins et al., “Eosinophilicesophagitis in children and adults: a systematic review andconsensus recommendations for diagnosis and treatment,”Gastroenterology, vol. 133, no. 4, pp. 1342–1363, 2007.

[4] A. J. Lucendo and L. de Rezende, “Endoscopic dilation ineosinophilic esophagitis: a treatment strategy associated with ahigh risk of perforation,” Endoscopy, vol. 39, no. 4, article 376,2007.

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[8] J. W. Jacobs Jr. and S. J. Spechler, “A systematic review of therisk of perforation during esophageal dilation for patients witheosinophilic esophagitis,” Digestive Diseases and Sciences, vol.55, no. 6, pp. 1512–1515, 2010.

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[10] A. J. Lucendo, A. B. Friginal-Ruiz, and B. Rodrıguez, “Boer-haave’s syndrome as the primary manifestation of adulteosinophilic esophagitis. Two case reports and a review of theliterature,” Diseases of the Esophagus, vol. 24, no. 2, pp. E11–E15,2011.

[11] J. D. Predina, R. B. Anolik, B. Judy et al., “Intramural esophagealdissection in a young man with eosinophilic esophagitis,”Annals ofThoracic and Cardiovascular Surgery, vol. 18, no. 1, pp.31–35, 2012.

[12] J. Quiroga, J. M. G. Prim, M. Moldes, and R. Ledo, “Sponta-neous circumferential esophageal dissection in a young manwith eosinophilic esophagitis,” Interactive Cardiovascular andThoracic Surgery, vol. 9, no. 6, pp. 1040–1042, 2009.

[13] C. Robles-Medranda, F. Villard, R. Bouvier, J. Dumortier, andA.Lachaux, “Spontaneous esophageal perforation in eosinophilicesophagitis in children,” Endoscopy, vol. 40, article E171, 2008.

[14] P. J. Riou, A. G. Nichoison, and U. Pastorino, “Esophagealrupture in a patient with idiopathic eosinophilic esophagitis,”The Annals of Thoracic Surgery, vol. 62, pp. 1854–1856, 1996.

[15] H. Giles, L. Smith, D. Tolosa, M. J. Miranda, and D. Laman,“Eosinophilic esophagitis: a rare cause of esophageal rupturein children,”The American Surgeon, vol. 74, no. 8, pp. 750–752,2008.

[16] G. A. Prasad and A. S. Arora, “Spontaneous perforation in theringed esophagus,” Diseases of the Esophagus, vol. 18, no. 6, pp.406–409, 2005.

[17] T. W. Spahn, M. Vieth, and M. K. Mueller, “Paracetamol-induced perforation of the esophagus in a patient witheosinophilic esophagitis,” Endoscopy, vol. 42, no. 2, pp. E31–E32,2010.

[18] M. S. Cohen, A. Kaufman, A. J. DiMarino Jr., and S.Cohen, “Eosinophilic esophagitis presenting as spontaneousesophageal rupture (Boerhaave’s syndrome),”Clinical Gastroen-terology and Hepatology, vol. 5, no. 2, p. A24, 2007.

[19] S. Gomez Senent, L. Adan Merino, C. Froilan Torres, R. PlazaSantos, and J. M. Suarez de Parga, “Spontaneous esophagealrupture as onset of eosinophilic esophagitis,” GastroenterolHepatol, vol. 31, pp. 50–51, 2008.

[20] G. Liguori, M. Cortale, F. Cimino, andM. Sozzi, “Circumferen-tial mucosal dissection and esophageal perforation in a patientwith eosinophilic esophagitis,” World Journal of Gastroenterol-ogy, vol. 14, no. 5, pp. 803–804, 2008.

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