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acute acalculous cholecystitis complicated by mrcp confirmed mirizzi syndrome a case report

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Introduction MirizzisyndromeMSisarelativelyrarecomplicationof gall-stonedisease.Nowadays,itisconsiderednotonlyasanextrinsic compressionofthecommonhepaticductbyastoneimpactedat thecysticd

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jo u r n al h om ep a g e :w w w e l s e v i e r c o m / l o c a t e / i j s c r

Acute acalculous cholecystitis complicated by MRCP-confirmed Mirizzi

syndrome: A case report

Yuri N Shiryajeva,b,∗, Anna V Glebovaa,b, Tatyana V Koryakinac, Nikolay Y Kokhanenkoa

a Department of Faculty Surgery named after Professor A.A Rusanov, Saint-Petersburg State Pediatric Medical Academy, Saint-Petersburg, Russian Federation

b Sixth Department of Surgery, Mariinsky Hospital, Saint-Petersburg, Russian Federation

c Department of MRI, Mariinsky Hospital, Saint-Petersburg, Russian Federation

a r t i c l e i n f o

Article history:

Received 30 October 2011

Received in revised form 5 November 2011

Accepted 15 November 2011

Available online 23 November 2011

Keywords:

Acute acalculous cholecystitis

Mirizzi syndrome

Magnetic resonance

cholangiopancreatography

a b s t r a c t

© 2011 Surgical Associates Ltd Published by Elsevier Ltd All rights reserved

1 Introduction

Mirizzisyndrome(MS)isarelativelyrarecomplicationof

gall-stonedisease.Nowadays,itisconsiderednotonlyasanextrinsic

compressionofthecommonhepaticductbyastoneimpactedat

thecysticductorgallbladderneck,butalsoaresultofa

cholecys-tobiliaryfistulacausedbyinflammationandpressurenecrosisof

thegallbladderandcommonhepaticductwallsbytheimpacted

stone.1–3 The most typical clinical symptoms in MS are upper

abdominal(especially rightupper quadrant)pain,jaundice and

fever.4–5SurgeryisthemosteffectivetreatmentofMS,butitis

accompanied withan increasedrisk of bile duct injury Severe

inflammationandadhesionsinthesubhepaticareaarevery

com-monandalmostalwaysinvolvethehepatoduodenalligamentand

Abbreviations: MS, Mirizzi syndrome; AAC, acute acalculous cholecystitis;

ERCP, endoscopic retrograde cholangiopancreatography; MRCP, magnetic

reso-nance cholangiopancreatography; US, ultrasonography.

∗ Corresponding author at: Department of Faculty Surgery named after Professor

A.A Rusanov, Saint-Petersburg State Pediatric Medical Academy, Litovskaya Str., 2,

194100 Saint-Petersburg, Russian Federation Tel.: +7 812 2757362;

fax: +7 812 2757326.

E-mail addresses: shiryajev@yandex.ru (Y.N Shiryajev), glebova.anna@mail.ru

(A.V Glebova), KoryaTV@yandex.ru (T.V Koryakina), kohanenko@list.ru

(N.Y Kokhanenko).

theydistortthenormalanatomicrelationshipsandproportions Therefore, it is highly desirable todiagnose MS preoperatively

to improve orientation in the surgical field and provide maxi-mal safety of the procedure The clinical symptoms of MS are non-specific, and are very similar to the symptoms of chole-docholithiasis.Ultrasonography(US)andcomputedtomography have limited sensitivity,3,6 and they are considered as screen-ingtests.AdiagnosisofMShastobeconfirmedbyothertests Themosthelpfuldiagnostictools toshow thevariationsinMS areeitherendoscopicretrogradecholangiopancreatography(ERCP)

orpercutaneoustranshepaticcholangiography.1,4However,these examinationsareinvasiveandmayleadtocomplicationsandeven fataloutcomes.Duringthelastdecademagneticresonance cholan-giopancreatography(MRCP)hasbeenwidelyusedanditisnow consideredasa goodnon-invasiveand averyaccurateimaging modalityinMSandinmanyotherdiseasesofthe hepatopancre-atobiliaryarea.7,8

Acuteacalculouscholecystitis(AAC)canleadtothe develop-mentofaconditionwhichisverysimilartoMSinitsclinicalcourse and imaging findings.The commonhepaticduct is compressed notbyastoneatthecysticductorHartmann’spouch,butbythe severelyinflamedandenlargedgallbladder.Suchcasesare exclu-sivelyrare.Wewereabletoidentifyonlythreewell-documented casesintheliterature.9–11Allthesepatientswereoperatedon,and

inallthreecasesapreoperativediagnosiswasmadebyERCP

2210-2612/$ – see front matter © 2011 Surgical Associates Ltd Published by Elsevier Ltd All rights reserved.

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Fig 1. MRCP demonstrated extrinsic compression of the common hepatic/common

bile duct by an inflamed, enlarged gallbladder without any stones in the biliary

system.

Recently,theauthorsobservedapatientwithjaundice,

devel-opedonthebackgroundofacutecholecystitis.OnUSexamination

stones in the gallbladder and bile ducts were not found The

signsofbiliaryobstructiondisappearedpromptlyonconservative

treatment.MRCPconfirmedsignificantcompressionofcommon

hepatic/commonbileductbytheinflamedandenlarged

gallblad-der,aswellasabsenceofstonesinthebiliarysystem.Conservative

treatmentwasundertaken,andsymptomsofacutecholecystitis

andjaundicegraduallyresolved

A52-yearmalepatientN.wasadmittedurgentlyon24.02.2011

withsevereupperabdominalpain,whichwasespeciallymarkedin

therighthypochondrium.Hehadnauseaandrepeatedbile

vom-iting.Thesecomplaintsstartedtwodaysagoandtheyincreased

withtime.Thiswashisfirstepisodeofsuchanattack.Pastmedical

historywasunremarkable.Physicalexaminationshowed

moder-atetendernessintherighthypochondriumandepigastrium.There

werenoperitonealsigns.Ortner’ssignandMurphy’s signwere

weaklypositive.Laboratorydatarevealedleucocytosis12,600/ml

USfindings:gallbladder103mm×38mm,anditswallwas

thick-enedto5mm.Thecommonbileductwasdilated(12mm);there

werenostonesinthegallbladderandinbileducts.After

crystal-loidinfusionwithdrotaverinandmetamizolesodiumthepatient’s

paindecreased,andvomitingstopped.Onthenextmorningthe

painincreasedagain,and hisskinandsclera becameyellowish

Palpationshowedseveretendernessandmildmuscularrigidityin

therighthypochondrium.Ortner’ssignandMurphy’ssignbecame

highlypositive.Biochemicalbloodtestsrevealedincreasein

biliru-bin(total 101␮mol/l, direct25␮mol/l)and transaminases(ALT

295IU/l(normalrange 0–55),AST123IU/l(5–34)), whereashis

amylaselevelremainednormal(46IU/l,normalrange25–110).A

repeatedUSshowedthegallbladdertobe110mm×43mm,itswall

wasnotthickened,intrahepaticbileductsandcommonbileduct

(3mm)werenotdilated.Gastroduodenoscopyshowedno

signifi-cantchanges

AAC,complicatedwithjaundice,wasdiagnosedonthebasisof

clinical,laboratoryandinstrumentalfindings.Conservative

man-agementwascontinuedwithadditionofantibiotics.MRCPonthe

Fig 2.Check MRCP 5 months later demonstrated post-inflammatory changes of the gallbladder which became reduced in size and was deformed However its wall was not thickened The intra- and extrahepatic bile ducts were not dilated and free of stones.

3rddayafteradmissiondemonstrated enlargementofthe gall-bladderwithitswallthickeningandextrinsiccompressionofthe commonbileductwithoutanystonesinthebiliarysystem(Fig.1) Thepatient’spainandjaundicegraduallyresolvedwith conserva-tivetreatment,andlaboratoryfindingssignificantlyimprovedto normal/subnormalranges.Hewasdischargedontheninthday

Oncheck-upintheclinic5monthslaterthepatient demon-strated no complaints, no jaundice,his abdomen wassoft and notpainful.USfindingswereunremarkable.ThefollowupMRCP (Fig.2)showedthegallbladdertobereducedinsizeandit was deformed.Itswallwasnotthickened(3mm)andwithoutany fill-ingdefects.Theintra-andextrahepaticbileductswerenotdilated andfreeofstones

3 Discussion

AAC can be complicated with extrinsic compression of the commonhepatic/commonbileductbytheenlargedandinflamed gallbladder,whichwasfollowedbyjaundice.Itsmechanismisvery similartoMS,whenthebileductiscompressedfromoutsidedue

toastoneimpactedatthegallbladderneckorcysticduct.Thefirst descriptionofthisconditionusingUSandERCPwaspublishedby R.J.Ippolitoin1993.9 Averysimilarcasewasdescribed by Ital-ianauthorsin1992.12Theydescribedayoungmalepatientwho complained of repeatedattacksof right upperabdominal pain, accompaniedwithjaundice,though,thebiliaryobstructionwas notcausedbyAAC,butbyanextremelyenlarged“congestive” gall-bladderdueto“cysticductsyndrome”.Compressionofthecommon hepatic/commonbileductwasconfirmedbyERCPand intraopera-tively.Intheliteraturewemanagedtofindonlytwoadditionalcase reportsofAAC,complicatedwiththebileductcompressionand jaundice.10,11Thus,thispathologicalentityisexceptionallyrare

Itisinterestingtonotethat theterminologytodescribethis conditionhasnotbeenagreedupon.Theauthorsofthesethree publishedcasereports useddifferentterms.InIppolito’spaper9

hecalledthecondition“Acuteacalculouscholecystitisassociated withcommonhepaticductobstruction:avariantofMirizzi’s syn-drome”.K.Mergeneretal.,10entitledtheirarticle“Pseudo-Mirizzi syndromeinacutecholecystitis”,whileS.Ahlawat11usedthetitle –“AcuteacalculouscholecystitissimulatingMirizzisyndrome:a

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canbeconsideredtobeaspecialkindofMS

OneMS classificationproposedbyJapanese authorsin1997

basedontheirexperiencein30cases.5TheydefinedtypeIVofMS

as“hepaticductstenosisasacomplicationofcholecystitisinthe

absenceofcalculiimpactedinthecysticductortheneckofthe

gall-bladder”.Therewere8suchcasesintheauthors’series.However,

neitherthefigure,northepresentationofatypicalcaseallowed

thereaderstosaywhethersomeofthese8patientshadacute

acal-culouscholecystitisornot.WeconsiderthatthecasesofMSwith

acalculouscholecystitiscanbeclassifiedasNagakawa’stypeIV

T.Nagakawaetal.believe,that“forTypeIVdisease,whichoften

presentswithseverestenosisofthehepaticduct,reconstructionof

thebiliarytractfollowingexcisionofthestrictureis indicated”

However,wethink, sucha procedureistoomajorand

unjusti-fied.Inflammatoryhepaticocholedochealstrictureshealwellafter

simplecholecystectomy,whencombinedwithbiliarydrainage(by

transhepaticornasobiliarydrainageasperformedinthefirststage

ofsurgicaltreatment,orbyoperativeT-tubedrainage).R ˇColovi ´c

etal.proposedtheirownclassificationforMS.13Theseauthors’MS

typeIVisthesameasNagakawa’sclassification.However,their

conceptionofsurgeryissignificantlydifferent,5anditisvery

sim-ilartoourapproach

The diagnosis of MS in acalculous cholecystitis is usually

suggestedbyUSandERCP,9–12orsometimesbypercutaneous

tran-shepaticcholangiography.12Asthefirststageofsurgicaltreatment,

choledochalstentingwasused10torelievejaundice.Allthepatients

wereoperatedon:inthreecasesopencholecystectomy,9,11,12 in

one laparoscopic cholecystectomy10 was performed In all the

three cases of AACthere was gangrenousinflammation of the

gallbladder.9–11 Despite the absence ofbiliary drainage (itwas

placedonlyin1of4patients),jaundiceandothersymptoms

suc-cessfullyresolved

4 Conclusion

Ourcase canbeconsideredasextraordinarybecauseof two

factors.First,thediagnosisofMSinacalculouscholecystitiswas

confirmedbyMRCP,whichappearstobethefirsttimereported

intheliterature.Second,conservativetreatmentwaseffectiveand

surgerywasavoided.ItallowedustoperformacheckMRCP,which

showedresolutionofacuteinflammationofthegallbladder,aswell

asdecompressionofthecommonhepatic/commonbileduct

Conflict of interest

None

Funding

None

Ethical approval

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereport.Acopyofthewrittenconsentis avail-ableforreviewbytheEditor-in-Chiefofthisjournalonrequest

Author’s contributions

YuriN Shiryajev:ideaof thearticle,study design,data col-lection, data analysis,literature search, writing, revision of the manuscript

AnnaV.Glebova:datacollection,dataanalysis,literaturesearch TatyanaV.Koryakina:datacollection,dataanalysis,makingand interpretationofultrasoundandMRIexamination

NikolayY.Kokhanenko:criticalrevisionandsupervisionofthe work

Allauthorsreadandapprovethefinalversionofthemanuscript

Acknowledgements

None

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