Liver transplantation for “mass-forming” sclerosing cholangitis after laparoscopic cholecystectomy

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International

Journal

of

Surgery

Case

Reports

j o ur n a l ho m e p a g e :w w w . c a s e r e p o r t s . c o m

Liver

transplantation

for

“mass-forming”

sclerosing

cholangitis

after

laparoscopic

cholecystectomy

夽

Damiano

Patrono

_,

_Elena

_Mazza

_,

_Gianluca

_Paraluppi

_,

_Paolo

_Strignano

_,

_Ezio

_David

_,

Renato

Romagnoli

_,

_Mauro

_Salizzoni

a_{GeneralSurgery2andLiverTransplantationCenter,UniversityofTurin,A.O.CittàdellaSaluteedellaScienzadiTorino,CorsoBramante88,10126Turin,}

Italy

b_{PathologyUnitII,UniversityofTurin,A.O.CittàdellaSaluteedellaScienzadiTorino,CorsoBramante88,10126Turin,Italy}

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Articlehistory: Received22April2013

Receivedinrevisedform3June2013 Accepted22July2013 Available online xxx Keywords: Biliaryobstruction Biliarystent Cholangiocarcinoma Gallstones Portalhypertension

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INTRODUCTION:Chronicbiliaryobstructionconsequenceofabileductinjurymayrequireliver

transplan-tation(LT)incaseofsecondarybiliarycirrhosis,intractablepruritusorreiterateepisodesofcholangitis.

“Mass-forming”sclerosingcholangitisleadingtosecondaryportalveinthrombosisandpre-sinusoidal

portalhypertensionhasnotbeenreportedsofar.

PRESENTATIONOFCASE:Wepresentthecaseofapatientwhounderwentlaparoscopiccholecystectomy

forMirizzisyndrome.Thepersistentbileductobstructionduetoaresidualgallstonefragmentwas

treatedbyaprolongedbiliarystenting.Followingrepeatedboutsofcholangitis,afibrouscentrohepatic

scardeveloped,conglobatingandobstructingthemainbranchesoftheportalveinandofthebiliarytree.

Thepatientdevelopedsecondaryportalveinthrombosisandportalhypertension.Afteranextensive

diagnosticwork-up,includingsurgicalexplorationtoruleoutmalignancy,thecasewassuccessfully

managedbylivertransplantation.

DISCUSSION:Mass-formingsclerosisofthebileductandbiliarybifurcationmaydevelopasa

conse-quenceofchronicbiliaryobstructionandprolongedstenting.Secondaryportalveinthrombosisand

pre-sinusoidalportalhypertensionrepresentsanunusualcomplication,mimickingKlatskintumor.

CONCLUSION:Atimelyandpropermanagementofpost-cholecystectomycomplicationsisofmainstay

importance.Earlyreferraltoaspecializedhepato-biliarycenterisstronglyadvised.

© 2013 The Authors. Published by Elsevier Ltd on behalf of Surgical Associates Ltd. All rights reserved.

1. Introduction

Wepresentthecaseofapatientwhodevelopedasclerosisof thebileductandofthebiliarybifurcationasaconsequenceofa residualgallstonefragmentafterlaparoscopiccholecystectomyfor Mirizzisyndrome(MS)andprolongedbiliarystenting.The thicken-ingofthebiliaryductsatthehilarplateconglobatedtheportalvein branchesleadingtoportalveinthrombosisandportalhypertension andwasfinallytreatedbylivertransplantation(LT).

2. Presentationofcase

A36-year-oldpatientunderwentlaparoscopiccholecystectomy forMirizzisyndromeinatownhospitalinJuly2010.Priortothe

夽 Thisisanopen-accessarticledistributedunderthetermsoftheCreative Com-monsAttribution-NonCommercial-NoDerivativeWorksLicense,whichpermits non-commercialuse,distribution,andreproductioninanymedium,providedthe originalauthorandsourcearecredited.

∗ Correspondingauthorat:GeneralSurgery2andLiverTransplantationUnit, Uni-versityofTurin,SanGiovanniBattistaHospital,A.O.CittàdellaSaluteedellaScienza, CorsoBramante88,10126Turin,Italy.Tel.:+390116334374.

E-mailaddress:mauro.salizzoni@unito.it(M.Salizzoni).

operation,abiliarystenthadbeenplacedbyendoscopicretrograde cholangio-pancreatographytorelievebiliaryobstruction.During theoperation, duetothepresenceof importantadhesions pre-cludingasafedissectionoftheCalot’striangle,thegallbladderwas openedintheattempttoremoveahugegallstoneimpactedinthe infundibulum.Asthegallstonewasfirmlyadheringtothe gall-bladderwallandimpossibletodislodge,itwasfragmentedusing acadmiumlaser.Cholecystectomywasachievedbylaparoscopy leaving in place a part of the gallbladder infundibulum. Intra-operative cholangiography was not even attempted. The early postoperativecoursewasuneventfulandthefirstbiliarystentwas removed,butthepatientsufferedtwoepisodesofcholangitisdue torecurrentbileductobstructioninthefollowingthreemonths, whichweretreatedagainbyendoscopicstentingofthebileduct.A firstplasticstentwasreplacedbyafullycoveredmetallicstent, which wasleft in placeuntil March2012,when it was substi-tutedafterthepatientpresentedafurtherepisodeofcholangitis. Thetotaldurationofthebiliarystentingwas21months.InApril 2012,Dopplerultrasonographyshowedapreviouslyunrecognized splenomegalyandpartialthrombosisofintrahepaticportalvein branches,thuslowmolecularweightheparintherapywasstarted. OnlyinJune2012,whenhepresentedanepisodeofupper gastroin-testinalbleedingfromlargegastricfundusvarices,thepatientwas referredtoourInstitution.

2210-2612/$–seefrontmatter © 2013 The Authors. Published by Elsevier Ltd on behalf of Surgical Associates Ltd. All rights reserved.

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908 D.Patronoetal./InternationalJournalofSurgeryCaseReports4 (2013) 907–910

Computed tomography and magnetic resonance imaging showedtheobstructionoftheintrahepaticportalveinbranches relatedtothepresenceofathick,densetissueinthehilarplate region,determiningalsothestenosisoftheproximalportionof thecommonbile duct and ofthebifurcation (Fig. 1).This pre-sentationwasdeemedconsistentwithalocallyadvancedKlatskin tumourinvolvingtheintra-hepaticportalveinbrancheswith sec-ondarythrombosis.Nevertheless,CA19.9levelwasnormaldespite theincreased bilirubin and positron emission tomography was consistentwithaninflammatory reaction atthe hepatichilum. Furthermore,severaltrans-luminalandpercutaneous ultrasound-guidedbiopsieswerenegativeformalignancy.Inordertoformally ruleoutcholangiocarcinomaweproceededtoopensurgical explo-ration.Atoperation,theliverwascholestaticbutwithnosignof cirrhosis,thehepatic pediclepresented a cavernomatous trans-formationandadensescar-liketissueoccupiedandretractedthe hilarplate.Afterdissectionofthehepaticpediclecavernomaand removalofaresidualgallstonefragment(Fig.2A),wecarriedout anextensivesamplingoftheextremelystiffhilartissue.Pathologic examinationwasagainnegativeforcancer.Thedissectionofthe biliarybifurcationandofthehilarplatewasabandonedwhenit becameevidentthattheintra-hepaticinvolvementofthebiliary ductsprecludedanyattemptata bilio-entericderivation.Thus, consideringthe portal hypertensionstatus, the complexbiliary lesionnotamenabletoastandardrepairbyhepatico-jejunostomy andtheabsenceofanyargumentinfavourofmalignantdisease, weenlistedthepatientforlivertransplantation(LT).Duetothe complicatedsurgicalhistoryandthereiterateepisodesof cholan-gitis,thepatientwasgrantedanupgradeonthewaitinglistand wastransplantedinAugust 2012witha biochemicalModel for End-stageLiverDiseasescoreof16.Nomajorpost-operative com-plicationoccurred,andheiscurrentlyalivewithgoodliverfunction tenmonthsafterLT.Macroscopic(Fig.2BandC)andpathological (Fig.3)examinationoftheexplantedliverconfirmedtheabsenceof malignancyandthepresenceofa“mass-forming”sclerosisofthe hilarbileductsencasingtheportalveinbranches.

3. Discussion

LTrepresentsthelastresource inthetreatmentofbile duct injuries (BDI) in case of secondary biliary cirrhosis, repeated episodesof cholangitis,intractablepruritus andpoor qualityof life.1 _{Several cases of LT after a BDI occurring during} laparo-scopiccholecystectomyhavebeenreported.ThetimingofLTvaries accordingtothetype ofinjury:patientswithassociated vascu-larinjuries maydevelop afulminanthepaticfailureconsequent tothemassivehepaticnecrosis,thusrequiringurgentLT.2_Onthe otherhand,patientswithcomplexbiliaryinjuriesnotamenableto bilio-entericderivation3_{orhepaticresection,}4_orwhodevelop sec-ondarybiliarycirrhosis,usuallyundergoLTmonthstoyearsafter theBDI.1,5,6

Mirizzi syndrome is the obstruction of the common bile duct due to theextrinsiccompression by a gallstone impacted in the gallbladder neck. Surgical treatment depends on the localanatomyandspacesfromlaparoscopiccholecystectomyto hepatico-jejunostomy.7,8_{Theroleoflaparoscopiccholecystectomy} inthetreatmentofMSisstilldebated.Duetothepresenceof tena-ciousinflammatoryadhesions,graspingoftheHartmann’spouch

Fig. 1. Pre-operative work-up. (A) Contrast-enhanced computed tomography showingtheportalveinoccludedbydensetissueattheportahepatis(thickarrow). Notethepresenceofvisceralvarices,thesplenomegalyandthebiliarystent;(B) thehypodensemassconglobatesthehepaticvessels(thinarrow)andthebileducts atthehilarplate;(C)magneticresonancecholangiopancreatographyshowingthe stenosisoftheproximalcommonbileductextendedtothejunctionofthemain biliarybranches(arrowheads).

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Fig.2.Explantedliver.(A)Residual2-cmgallstone;(B)thickenedportalvein(arrowhead)andbileduct(arrow);(C)internalaspectoftheportalveinafterlongitudinal

section,withthestenoticorificesoftheright(arrow)andleft(arrowhead)intra-hepaticbranches;(D)theportalveinhasbeenseveredlongitudinallyalongwiththecaudate

lobe:abnormalthickfibroustissuesurroundingandconglobatingthevenousbranches(arrowheads)andthebiliaryorifices(arrows).

andcorrectexposureanddissectionoftheCalot’striangleis some-timesimpossible.9_{Incomplexcases,someAuthorssuggestawide} openingofthegallbladder,extractionofthegallstonesandsubtotal cholecystectomy,leavingthegallbladderinfundibuluminplace.10 According to Antoniou et al.,9 _{laparoscopic cholecystectomy is} achievedin59%ofcases,witha0.8%mortality,16%complication rate,and5%reoperationrate.Noteworthy,25%ofcomplications isrepresentedbyresidualstones.PreoperativediagnosisofMSis associatedwithalowerconversionandpostoperativecomplication rate.11_{Nevertheless,althoughseveralcasesofsuccessful} manage-mentofMSbylaparoscopiccholecystectomyhavebeenreported, mostAuthorsremaincautiousinrecommendingthelaparoscopic approach,especiallyfortypeIIMS(i.e.presenceofa cholecysto-biliaryfistula).8_{Thus,conversiontoanopenapproachshouldbe}

Fig.3. Explantedliverhistology(haematoxylinandeosinstaining;original mag-nification1×).Sectionofthehilarplatedemonstratingthepresenceofadense, star-shapedfibroustissueconglobatingtheportalvein(arrowhead),thebileducts (thinarrows),andthearteries(thickarrow).Inset:biliaryductssurroundedbythe fibroustissue.

preferredwhendissectionbylaparoscopybecomeshazardous,as itwasprobablythecaseforourpatient.

Thiscasedepictsa peculiarindicationforLT,which wasnot secondarybiliarycirrhosis,pruritusorrecurrentcholangitisasin mostcasesoflateLTafterBDI.Thepatientdidnotdevelopportal hypertensionasaconsequenceofhepaticfibrosis,asdemonstrated by thefact that liver parenchyma washistologically normal. A fibrous scar, issueof reiterate episodes of cholangitis and pro-longedbiliarystenting,surroundedandnarrowedtheintra-hepatic mainbranchesoftheportalvein,causingsecondary portalvein thrombosisandpre-sinusoidalportalhypertension.Ourfirst diag-nostic hypothesis wasa locallyadvanced Klatskintumour. This hypothesis wassupported by the fact that recurrent cholangi-tisis awell-known risk factorfor cholangiocarcinoma andthat bile ductsclerosis-related portalveinencasementhasnot been reportedbefore. Thiscasehighlightstheimportanceofaproper initialmanagementof Mirizzisyndromeand,in general,of any caseofbiliaryobstruction.Evenincomplexcases,theprinciples of“criticalviewofsafety”12_{mustberespectedasmuchaspossible} andthethresholdforconversiontoopencholecystectomyshould bekeptverylowifasafedissectionbylaparoscopyisimpossible. Intra-operativecholangiographyrepresentsapreciousaidin defin-ingbileductanatomyandindiagnosingbileductinjuries.13_Inthis case,ifsuccessfullyperformed,itwouldhaveallowedimmediate detectionofpersistentbileductstenosisandconsequentlyledto acorrecttimelyrepair.Finally,itcouldbearguedthataprompt treatment of post-cholecystectomy complications (i.e. removal of the residual gallstone fragmentand bilio-enteric derivation) wouldhave avoidedtheprogressiontowardsthis unusualform of“mass-forming”sclerosingcholangitisandpreventedtheneed forLT.

4. Conclusion

Chroniccholangitisduetoprolongedbiliaryobstructionmay leadtoportalveinobstructionandthrombosis.Properinitial man-agementofMirizzisyndromeandbiliaryobstructionisofmainstay

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importance. The referral to an experienced hepatobiliary unit beforetheonsetofseverecomplicationsofchroniccholangitisis stronglyadvised.

Conflictofintereststatement

None.

Funding

None.

Ethicalapproval

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontributions

DamianoPatronowrotethepaper,obtainedconsent,processed digital images and performed literature search; Elena Mazza wastheresident in charge of patientcare and helped in writ-ingthepaper; Gianluca Paraluppiwasthe secondsurgeon and jointly performed both operations; Paolo Strignano acquired photos during the operations and contributed to manuscript drafting;EzioDavid wasthepathologistincharge of patholog-ical examination; Renato Romagnoli performed the transplant operationandcriticallyrevisedthemanuscript;MauroSalizzoni was the overall responsible for the care of the patient, per-formedthefirstoperation,supervised andcriticallyrevisedthe manuscript.

Acknowledgment

TheAuthorsthankMissElizabethClarkeforherkindassistance inrevisingthemanuscript.

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