Resolving sphincter of Oddi incontinence for primary duodenal Crohn's disease with strictureplasty

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International

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of

Surgery

Case

Reports

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

Resolving

sphincter

of

Oddi

incontinence

for

primary

duodenal

Crohn’s

disease

with

strictureplasty

G.

Alemanno

_{, A.}

_Sturiale

_{, F.}

_Bellucci

_{, F.}

_Giudici

_,

_F.

_Tonelli

DigestiveSurgeryUnit,DepartmentofClinicalPhysiopathology,UniversityofFlorenceMedicalSchool,CareggiHospital,Italy

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Articlehistory:

Received9November2012 Accepted16November2012 Available online 23 November 2012 Keywords:

DuodenalCrohn’sdisease SphincterofOddiincontinence Strictureplasty

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INTRODUCTION:Crohn’sinvolvementofduodenumisarareeventandmaybeassociatedtoproteiform symptomsanduncommonpathologicalaspectswhichmakediagnosisandtreatmentcomplex. PRESENTATIONOFCASE:Thepeculiaraspectofthiscasewasasuspectedduodeno-biliaryfistula.The patient(female,22yearsold)wasaffectedbyduodenalCrohn’sdisease.Magneticresonanceimaging showedadilatedcommonbileduct,whosefinalpartlinkedtoaformationcontainingfluid,and character-izedbyfillingofthecontrastmediumintheexcretoryphase.Abdominalultrasoundshowedintra-hepatic andintra-gallbladderaerobilia.Atsurgery,theduodenumwasmobilizedshowinganinflammatory stric-tureandaslightdilatationofthecommonbileduct,withnosignsoffistulas.Theopenedduodenumwas anastomizedsidetosidetoatransmesocolicloopofthejejunum.Aftersurgery,thegeneralconditionof thepatientimproved.

DISCUSSION:Onlytwocasesoffistulabetweenanarrowduodenalbulbandthecommonbileducthave beendescribedinliteratureandtheAuthorswerenotbeabletoverifytheoccurrenceofaduodenalbiliary fistulaatsurgery.TheassociationbetweenduodenalCrohn’sdiseaseandSphincterofOddiincontinence isaveryrarefindingwithdifferentetiology:chronicintestinalpseudo-obstruction,commonbileduct stones,progressivesystemicsclerosis.

CONCLUSION:ThetreatmenttoresolveSphincterofOddiincontinenceforprimaryduodenalCrohn’s diseaseisnotclear.Strictureplastycouldbethetreatmentofchoice,because,resolvingthestricture,the duodenalpressureislikelytodecreaseandtherefluxthroughtheincontinentsphinctercanbeavoided.

© 2012 Surgical Associates Ltd. Published by Elsevier Ltd.

1. Introduction

Crohn’sdisease(CD)canaffecttheentiregastrointestinaltract, butgastroduodenalinvolvementisrarelyobserved.Firstdescribed in1937byGottliebandAlpert,duodenallocalizationoccursin0.5% to4%ofpatientswithCD.1_{Innearlyallcasesepigastricpainor}

dys-pepsiaarethepredominantsymptoms,whichmimicpepticulceror non-ulcerdyspepsia.Inmoreadvancedphasesofthedisease,most patientshavesymptomsduetoobstructivelesions,suchas epi-gastricdistress,anorexia,nausea,vomitingandweightloss.Onlya fewhavehaematemesisormaelena.1_{Everypartoftheduodenum}

canbeinvolved,butthesecondpartismostcommonlyaffected. Differenttypesofulcer,suchasaphthous,longitudinal,transverse, anddeep,mayoccur.Abnormalfolds,stenosisandulcerationare

Abbreviations: CD,Crohn’sdisease;ESR,erythrocytesedimentationrate;CRP, C-reactiveprotein;UGI,uppergastrointestinal;MRI,magneticresonanceimaging; CKK,cholecystokinin;SO,SphincterofOddi.

∗ Correspondingauthorat:CareggiHospital,DigestiveSurgeryUnit,Largo Bram-billa3,50134Florence,Italy.Tel.:+390557947449;fax:+390557947449.

E-mailaddress:g.alemannomd@gmail.com(G.Alemanno).

a _{Authors’contribution:Allauthorscontributedequallytothiswork:G.A.,F.B.} andA.S.collectedthedata,G.A.andA.S.analyzeddata,G.A.,A.S.,F.G,F.BandF.T. wrotethemanuscript,G.A.andF.T.supervisedallthemanuscript.

themainradiographicalfeatures.2 _{Obstructionisthemost}

com-moncomplicationofduodenalCD,but fistulaearisingfromthe duodenumareextremelyrare.Itiscommonlybelieved thatthe duodenalfistulaeoriginateonlyfromotherprimarylocalizations ofdiseasedsmallorlargebowel.3_{Submittingpatientswith}

duode-nalCDtoX-rayexamination,fillingofpancreaticorbiliaryducts mayoccur.Thismaybeduetoeitherfistulaformationorreflux throughadamagedampullaofVater.4_{Consequencesofthisreflux}

canbeanobstructionoftheduodenalportionofthebileductand pancreatitis.5,6

The most frequent indication for surgery is gastroduodenal obstruction,whereasmajorhemorrage,extensivefistulaformation orsuspicionofmalignancymaywarrantresection.Dilatationof stricturesandstrictureplastymaybeviabletreatmentoptionsin selectedpatientswithstenosingduodenalCD.

2. Presentationofcase

Female,22yearsold,withduodenalCD,onsetatageof8,with diffuseabdominalpainandmalabsorptionsyndrome.Attheage of11,shewashospitalizedforpersistentabdominalpain associ-atedwithdyspepsia,constipation,fatigueand7-kgweightlossin 3months,andaBMIof15.9.BloodtestsshowedanincreasedERS, CRP15mg/dL,Hb12.1g/dLandnormalWBC,glucose,coagulation,

2210-2612 © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. http://dx.doi.org/10.1016/j.ijscr.2012.11.016

Open access underCC BY-NC-ND license.

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150 G.Alemannoetal./InternationalJournalofSurgeryCaseReports4 (2013) 149–152

Fig.1.Abdominalultrasound:intragallbladderandintrahepaticaerobilia(AandB).

serumprotein,amylase.Uppergastrointestinal(UGI)endoscopy wasperformedwithdifficultytoovercomethesuperior duode-nalknee,showinga“granulomatousandeasilybleedingduodenal mucosa,withmultipleulcerscoveredwithfibrin.”Thebiopsy con-firmedthesuspecteddiagnosisofduodenalCD.Colonoscopywas negative.Fromtheageof11–16,thepatientwastreatedwith aza-thioprineandcorticosteroidtherapy,andreportedarelativegood conditionwithsomeexacerbationepisodes.Attheageof19,the patientbegan Thalidomidetherapybecauseofpoorresponseto previousdrugs.In2009,duetoexacerbationofsymptoms, feed-ingdifficultiesandweightloss,anMRIwasperformedandshowed adilatedcommonbileduct(9mm)withitsfinalpart communi-catingwithaformation(about1cmindiameter)containingfluid, andcharacterizedbyfillingofthecontrastmediuminthe excre-toryphase.Thissituationwasattributabletoabiliaryinflammatory stricture.AnewUGIendoscopywasperformed:theduodenalbulb andthesecondduodenalportionappeareddeformedandstenotic withhyperemic and ulcerated mucosa. A pneumatic dilatation wasperformed. Atthe ageof 21, the patientsuspended treat-mentwithThalidomideand beganInfliximabwithpoorresults. In2011anotherexacerbationofsymptomsoccurred,namely: diff-usedabdominalpain,dyspepsia,weightloss,diarrhea,andfever. Abdominalultrasound showedintrahepaticandintragallbladder aerobilia(Fig.1).Gastro-duodenalfollow-throughcontrastX-ray documentedarigid,retractedandshortenedduodenum,andslight

opacificationof themain biliaryduct and ofsomeintrahepatic branches(Fig.2).Considering duodenalstricture, thefailure of previousmedicaltherapyandthesuspiciousofabiliary-digestive fistula,wedecidedtoperformsurgery.

SurgerywascarriedoutinJuly2011.Theduodenumwas com-pletelymobilizedshowinganinflammatorystricture,startingnear thepylorusuntiltheIIIportion,manygallbladderadhesions,and aslightdilatationofthecommonbileduct,withoutsignsof fistu-las.Theduodenumwasopenedlongitudinallyforalengthofabout 8cmconfirmingthewidespreadinflammatoryinvolvementofthe duodenalmucosaandwallthickening,upto8mm.Thepapillawas foundwithintheinflammatorycontextandwasprobedforsome centimeters,penetratingeasilybothintotheWirsungandthe bil-iaryduct,fromwhichclearmucusandbileflowedout(Fig.3).The openedduodenumwasanastomizedsidetosidetoa transmeso-colicloopofthejejunum,takenabout20cmfromTreitz.

About25cmfromtheRouxanastomosistherewerethree steno-sis,thatweretreatedwith12cmFinney strictureplasty(Fig.4). Belowthis,therewereother2shortjejunalstrictureswhichwere treatedwithHeineke-Mikuliczstrictureplasties.Aftersurgery,the generalconditionofthepatientimproved.Post-operativetherapy wasperformedwith5-ASA. Twelvemonthsafter theoperation therewasacompleteremissionofsymptomswithnodifficulties inintakingfoodnorsignsofocclusion.Atabdominalultrasoundno signsofintrahepaticandintragallbladderaerobiliawerefound.

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Fig.3.Atalongitudinalduodenalopening,duodenalmucosawasinflamed,the

duodenalwallwasthickenedandthepapillawasprobedforsomecentimeters,

penetratingbothintotheWirsungandthebiliaryduct,fromwhichclearmucusand

bilewereflowingout.

Fig.4.TheoutcomeoftheoperationconsistedintheRoux-en-Yduodenaljejunal

anastomosis,jejunalFinneystrictureplastyandjejunalH-Mstrictureplasty.

3. Discussion

CDoftheduodenummaybeassociatedtoproteiform symp-tomsanduncommonpathologicalaspectswhichmakediagnosis andtreatmentrathercomplex.7_{Theabovementionedpatienthad}

alonghistoryofabdominalpainandweightlossbeforediagnosis. Thepeculiaraspectofthiscasewasasuspectedduodeno-biliary fis-tula.Onlytwocasesoffistulabetweenanarrowduodenalbulband thecommonbileducthavepreviouslybeendescribedinaclinical radiologicalreport,someyearsago.8_{However,theAuthorswere}

notbeabletoverifytheoccurrenceofaduodenalbiliaryfistulaat surgery.Infact,thecommentoftheradiologicalfindingofoneof thecaseswas:“fillingofthecommonbileductandthecysticduct fromtheduodenum,probablythroughaduodenobiliaryfistula.”8

Theradiologicalpicturecouldbeduetoabiliaryfillingofduodenal contrastmediumthroughanincontinentSphincterofOddi(SO). TheassociationbetweenduodenalCD andSO incontinenceis a veryrarefindingwithdifferentetiology,suchaschronic intesti-nalpseudo-obstruction,9_{commonbileductstonesorprogressive}

systemicsclerosis.

Oddi’s Sphincter plays a major role in controlling bile and pancreatic juice flowing into the duodenum and in preventing thereflux of duodenalcontents into thebiliaryand pancreatic ducts. These functions are regulated by the SO motility. Local reflexesinvolvingtheSO havebeendemonstrated betweenthe

duodenum,10,11_{gallbladderandbileducts.}12–14_{SphincterofOddi}

motilityiscontrolledbycholecystokinin(CCK),whichisreleased intothebloodstreambytheduodenalmucosa,asa responseto duodenalluminal acidand nutrients.Oddi’sSphincter playsan importantroleindecreasingthebasalpressureandtheamplitude ofphasicwaves.ThecauseofSOincontinenceisuncertain,and onepotentialmechanismmaybeadefectoftheneural connec-tionsthatcoordinatetheinteractionbetweentheduodenum,the biliarytractandtheSO.ThereisevidencethatSOdysfunctionmay bea partofageneralizedmotordisorderofthegastrointestinal tract,suchassmallintestinaldysmotility.15_{Inonestudy,patients}

withirritablebowelsyndromeandSOdysfunctiondemonstrated paradoxicalresponsestoCCKmoreoftenthanpatientswithSO dys-functionalone.16_{Amongthecauses,thereistheassumptionthat}

increasedduodenalpressureovercomesthebaselinepressureof SO.Refluxofthecontrastmediumintothepancreaticobiliarytree wasnoticedintheuppergastrointestinalseries,inothercasesof duodenalCD,withfillingofeitherthepancreaticandbileductsor ofthebileductalone.4,17_{Inthesecases,onepatientsufferedfrom}

recurrentpancreatitiswhichwasexplainedbyperiductalfibrosis inducedbythepersistentlyopen,patulous,ampullaofVater.18_In

anothercase,therewasevidenceofcholangitiscausedbythereflux oftheduodenalcontentintothebiliarytract.19

4. Conclusion

ThetreatmenttoresolveSOincontinenceforprimaryduodenal CDisnotclearyet;thereisnotsufficientliteratureonthistopic. SurgeryforduodenalCDincludesseveralsurgicaloptionssuchas duodenalorgastro-duodenalresection,gastro-entericorduodenal entericby-passorstrictureplasty.Theproceduremostfrequently employedfor duodenalCD isgastro-jejunalbypassalthough,in aconsiderablepercentageofcases,theprocedureisfollowedby majorcomplicationsin thepostoperativecondition.20

Stricture-plastycouldbe thetreatmentof choice,because,resolving the stricture,theduodenalpressureislikelytodecreaseandthereflux through theincontinent SO canbeavoided. Furthermore,since strictureplastyinvolvesaprogressivereductionoflocal inflamma-tion,thepathogeneticmechanismthat inducesSOincontinence willalsobeinterrupted.

Conflictofinterest

Authorscertifythatthereisnoactualorpotentialconflictof interestinrelationtothisarticleandtheystatethatthereareno financialinterestsorconnections,directorindirect,orother situ-ationsthatmightraisethequestionofbiasintheworkreported ortheconclusions,implications,oropinionsstated—including per-tinentcommercialorothersourcesoffundingfortheindividual author(s)orfortheassociateddepartment(s)ororganization(s), personalrelationships,ordirectacademiccompetition.

Funding

Authorsstatethattherearenofinancialinterestsor connec-tions,direct orindirect,orothersituationsthatmightraise the questionofbiasintheworkreportedortheconclusions, implica-tions,oropinionsstated—includingpertinentcommercialorother sourcesoffundingfortheindividualauthor(s)orfortheassociated department(s)ororganization(s),personalrelationships,ordirect academiccompetition

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Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontributions

Allauthors contributed equally to this work: Giovanni Ale-manno, Francesco Bellucci and Alessandro Sturiale collected thedata, GiovanniAlemanno and Alessandro Sturiale analyzed data,GiovanniAlemanno,AlessandroSturiale,FrancescoGiudici, Francesco BellucciandFrancesco Tonelli wrote themanuscript, Giovanni Alemanno and Francesco Tonelli supervised all the manuscript.

Acknowledgement

Prof.MariaRosariaBuri,ProfessionalTranslator/AiicConference Interpreter,UniversityofSalentofortheEnglishlanguageediting.

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