Download - Inflammatory Bowel Disease - Open Access eBooksopenaccessebooks.com/inflammatory-bowel-disease/role-of-surgery … · Inflammatory Bowel Disease 3 Figure 2: Natural history of disease,

Role of surgery for idiopathic inflammatory bowel disease with a focus on postoperative

events and their managementGunjan S Desai*; Prasad Pande; Aniruddha Phadke

Department of Gastrointestinal surgery, Lilavati hospital and research centre, Maharashtra, India.

*Correspondence to: Gunjan S Desai, Department of gastroenterology, Lilavati hospital and research centre,

Mumbai, Maharashtra, India 400050.

Email: [email protected]

Chapter 4

Inflammatory Bowel Disease

Keywords: Ileal pouch; Crohn’s disease; Ulcerative colitis; Surgery

1. Spectrum of Inflammatory Bowel Disorders [IBD]

Theinflammatorydisordersofbowelareverycommoningastrointestinalclinics.ThesearecharacterizedbyintermittentrelapsingandremittingcourseorchronicinflammatorycourseaffectingthegastrointestinaltractandcompriseofaspectrumofdisordersasshowninFigure 1[1].

Inthischapter,thefocusisonunderstandingidiopathicIBD,especiallyulcerativecoli-tis(UC)andcrohn’sdisease(CD)fromasurgeon’sperspectivewithspecificfocusonlifeaftersurgeryforthisIBD.

2. Natural History of the Disease and its Relevance to Clinical Practice

IdiopathicIBDis relapsingandremittingorchronicprogressivediseasewherein thediseasenaturalhistorycanbedividedinto4phasesbasedonthediseaseactivity.

PhaseI:Detection/diagnosisofdiseasebasedonclinicalpresentation:Activeorcomplicateddisease

PhaseII:Initiationoftreatmentandachievingthephaseofremission

PhaseIII:Phaseofmonitoringtomaintainremissionandearlydetectionofrelapse/complica-

InflammatoryBowelDisease

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www.openaccessebooks.comDesaiGS

tions

PhaseIV:Treatmentof relapseandmonitoringforprogressionofdiseaseorcomplications[2].

Figure 1:Spectrumofinflammatoryboweldisorders

Crohn’sdiseaseisoftenmisdiagnosedinitially.Nearly25%ofthepatientsarelabeledasirritablebowelsyndromeandmeantimetodiagnoseCDoftenreachedupto2yearsfromthefirstsymptom.Itisprogressiveinupto75%patientsandaspertheViennaclassification,canbeinflammatory,stricturingorpenetrating.Itprogressesinthesegmentwhereitbeganandhence,disease location is an important consideration.Progression toneoplasia is nowknowntobeassignificantpartofnaturalhistoryasinUC[3]. Ulcerativecolitis,ontheotherhand,progressesasachronicinflammatorydiseasestateaffectingthelargeintestineandhasnoothersubtypes.StricturingdiseaseinUCismoresug-gestiveofmalignancy.RiskofmalignancyisawellknownphenomenoninUC.Thenaturalhistory,itsclinicalsignificanceandtheeffectonpostoperativecomplicationsisshowninFig-ure 2[4]. InboththeseIBDs,progressiontocolorectalmalignancyisknownandthenaturalpath-way of progression tomalignancy is different from the sporadic colorectal cancer [CRC].ThisisshowninFigure 3.Apartfromthis,Crohn’sdiseasealsohaschronicfistulaewhichcanresultintosquamouscellcarcinomasatthosesitesandalsohasanincreasedriskoflungcancerandsmallintestinaladenocarcinoma.Also,theautoimmune,geneticandenvironmentalfactorsthataffectthegastrointestinaltract,alsoaffecttheextra-intestinaltissuesandproducetheextra-intestinalmanifestationsofthedisease[5,6].


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Figure 2:Naturalhistoryofdisease,pathophysiologicalbasisofclinicalpresentationsandcomplicationsaftersurgery

3. Disease Classification and Measurement of Severity Indices

ClassificationofCDisbasedontheMontrealclassificationortheParisclassification[7,8].Thediseasesubdivisionineitheroftheseisbasedonageatdiagnosis,diseaselocationandbehaviour[inflammatory/stricturing/penetrating]andwhetherthereisgrowthretardationornot.Also,attemptstogaugetheseverityofdiseasebyusingdifferentscoringsystemssuchasCrohn’sdiseaseactivityindex,HarveyBradshawindex,Oxfordindexetc.,havebeenmade[9].

Figure 3:DiseaseprogressiontocarcinomainIBD

UlcerativecolitisisclassifiedonthebasisofdiseaseextentandseveritybyMontrealclassification.SeveritygradinghasalsobeenattemptedusingTrueloveandWittsclassificationintomild,moderateandseverediseaseaswellasbySutherlandindex.However,theseveritygradingsaremoreacademicanddon’t actuallyguide the treatmentpathways.Clinical rel-evanceoftheseclassificationsandseverityscoringsisnotyetidentified[10].


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4. A Surgeon’s Understanding of the Medical Options of Management

ThefirstepisodeofulcerativecolitisismildinmajorityofcasesandsevereUCisseenonfirstpresentationinonlyabout15-20%cases.Morethan50%ofallcasesachieveremis-sionwithfirstlinemanagementwithASAanalogues.Upto35%ofthepatientsrelapseinthefirstyearaftertreatment.Ifthepatientsdonotrelapseinfirstyear,itisamarkerofquiescentdiseasewhereinthechancesofrelapseinthenextyearisaround20%[1,11].

InCrohn’sdiseaseon theotherhand, relapsesare seen inupto20%patients infirstyear,40%within2yearsandaround80%within10yearsofdiseasediagnosis.InbothUCandCD,incidenceofcolorectalcancersis2-5%at10yearsofdiagnosis,5-10%at20yearsofdiagnosisand12-20%at30yearsfromdiagnosis.InCD,nearly28%patientsmaydevelopsmallbowelcarcinomaand1-2%patientsareatriskforlymphoma,lungcancerand/orcervi-caldysplasia.Somestudieshavealsoconfirmedriskofprostatecanceratahigherincidenceinthesepatients[12].

Themanagementofthesepatientsisbasedontheclinical,radiological,endoscopicandhistopathologicalnatureofthediseaseineachpatient.Theoptionsformanagingthesepatientsaremedical,endoscopicandsurgical.Completediscussionofmedicalandendoscopicmeansofmanagingthesepatientsisoutofscopeofthischapterandonlytheclinicallyandsurgicallyrelevantpointsarediscussedfurtherinthesetwooptions.

The goals of the management in these patients are

• AchievethecorrectdiagnosisasthemanagementofdifferentIBDsisdifferent

• Toinduceandmaintainremission–Remissioncanbedefinedclinicallyorintermsofmucosalhealing

• Assessfordiseaseprogression,complicationsandcarcinoma

• Ensureagoodqualityoflife[QOL]whiletreatingthesepatients[13].

SalientfeaturesofthevariousmedicaloptionsforIBDareasshownin Table 1.

5. Algorithm of Medical Management of IBD at Our Centre

Our algorithmic approach for themedicalmanagement of these patients is summarized inFigure 4[14,15,16,17,18].


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AGENT SALIENT FEATURESROLE IN

MANAGEMENT

5- ASA analogues

(Sulfasalazine, mesalazine) 19,20

DoesnothelpinCDformaintainingremission•Hasadosedependentaction•Topical+oralismoreeffectivethaneitheralone.Oraland•topicalaloneareequallyeffective15%patientscannottolerateit•Doesnotalterthesurgicaloutcomes•Maleinfertilityisaconcernwithsulfasalazine•

InductionandmaintenancetherapyinUC.

InductiontherapyinCD.Notformaintenance.

Glucocorticoids 21

Doesnothelpinmaintenancetherapy

Steroidresistance*-20%

Steroiddependence**-40%

Theremaininghavealongtermresponseonsteroids

Affects surgical outcomes adversely

Induction therapy in UC

6-Mercaptopurine (6 MP)/ Azathioprine(AZA) 14,17

Takenearly6monthstoshowresponse–needcoverduring•thattimewithsteroids/methotrexate/cyclosporine.Steroidsparingforsteroiddependentpatients[better•outcomesincombinationwithinfliximab].Ifmorethan2coursesofsteroidsarerequiredinayearorif•parenteralsteroidsarerequiredtoachieveremission,theseareindicated.Reduce colectomy rates• inthesepatientswithsevere,refractorydisease.TPMT(Thiopurinemethyltransferaseenzyme)mutation•needstoberuledoutbeforestartingtreatment–Morechancesofcholestasis,bonemarrowsuppression,pancreatictoxicityandnodularregenerativehyperplasiainthesecases.Mildleucopeniaisagoodindicatorofresponseandis•desirable.Mantainancetherapyisusuallycontinuedupto3.5years.•10%patientscannottolerateit•Relapserateis8%•Does not affect surgical outcomes•

InductionandmaintenancetherapyinUCandCD

Infliximab 15,16

Mucosalhealingisbetterthananyotheragents

Effectiveoptionforsteroidrefractoryaswellas

immunomodulatorrefractorysevereUC/CDcasesforinductionaswellasmaintenanceScreeningrequiredfortuberculosis,HepatitisB/C,HIVas

wellasriskoflymphomaneedstobediscussedFormaintenancetherapy,itisusedwithsteroidor

immunomodulatorstopreventthedevelopmentofAnti-drugantibodiesPositive influence on surgical outcomes

InductionandmaintenancetherapyinCD>UC

In CD - Earlyaggressivemedicaltherapyistheparadigmshift

Other biological agents

22,23

Adalimumab•Natalizumab–Notusedduetoriskofprogressive•multifocalleukoencephalopathy[PML]Certolizumab/Vedolizumab•Ustekinumab–SubcutaneousdrugactiveagainstIl-12and•Il-23

Maybecomefrontlineinfuture

Table 1:SalientfeaturesandroleofdifferentmedicalagentsfortreatmentofIBD


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Figure 4:OuralgorithmicapproachtomedicalmanagementofIBD

6. Surgical Management of IBD

Inulcerativecolitis,20%ofthepatientsneedsurgeryduringthefirst10yearsafterdis-easediagnosisandnearly1/3rdofthepatientsneedsurgeryduringthefirst25yearsofdiseasediagnosis.Relapseratesareminimalinulcerativecolitis.InCrohn’sdiseaseontheotherhand,nearly80%ofthepatientsneedsurgeryatsomepointintheirlifewhichissignificantlyhigherthanulcerativecolitis.Also,afterthefirstsurgery,1/3rdofthepatientsrelapsewithin3yearsand2/3rdoftheserequireatleastoneothersurgeryduringtheirlife.1/3rdofthepatientswithCrohn’sdiseaseneedmorethantwosurgeriesduringtheirlifetime.10%ofpatientshavedis-easethatdoesnotrespondtoanytherapyandisreferredtoasdisablingdisease.Indications for surgeryareasshowninTable 2[24,25,26].

*-Activediseasepersistsdespitegivingasteroiddoseof0.75mg/kg/dayprednisoloneequivalent,**-Inabilitytore-ducesteroiddoseto<10mg/dayprednisoloneequivalentwithin3monthsofstartingsteroidwithoutadiseaserelapsewithin3monthsofstoppingthetherapy.


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Table 2:IndicationsofsurgeryinIBD

7. Options for Surgical Management and Surgical Techniques

BasicprinciplesofsurgeryforintestinesremainthesameforsurgeryeveninIBD–Toachievediseasefreemarginandestablishcontinuityofthegutbyatensionfree,vascularanas-tomosis.InCD,theprincipleisofbowelpreservationtoavoidshortbowelsyndromeduetotheneedforrepeatedsurgeries.Also,inCD,patientsundergoingsurgeryearlyafterdiagnosishaveanincreasedprobabilityofbeingre-operated[24,26].

Owingtothisobservationandalsoonseeingtheexcellentresponsetoimmunomodula-torsinCDforvariousindications,thenewerapproachescameinCDwhereinearlyandaggres-siveinitiationofimmunomodulator/infliximabtherapyledtoreductioninratesofsurgeries.However,forthepatientswhohavealreadyundergoneearlysurgery,theearlyinitiationofthisdrugtherapydoesnotseemtoprotectagainstthere-surgeryrates.Hence,itisrecommendedtoavoidearlysurgeryandbeginimmunomodulator/infliximabtherapyasearlyasfeasibleinCDwhichisabigparadigmshiftinthemanagementofCD[25].

There are various surgical optionstotakecareofthevariedclinicalpresentationsandvarieddiseaselocationinIBD.For CD,segmentalbowelresections,divertingloopileosto-myandsubtotalcolectomywithileostomyaretheemergencysurgeries.Forperianalfistulas/abscess, incisionanddrainageofabscess,fistulotomy/setonplacement/fistulectomy,divert-ingstomasandadvancementflapsareutilized.ForstricturesduetoCD,HeinekeMickuliczstricturoplasty and Finney stricturoplasty or side to side isoperistaltic stricturoplasty are the options[1,27].

ForUC,subtotalcolectomywithileostomyisthesurgicaloptionofchoiceinemergencywhereastotalproctocolectomywithBrooke’sorKock’sileostomyorsubtotalcolectomywithileorectalanastomosis[IRA]orrestorativeproctocolectomywithilealpouchanalanastomosis[RPAwithIPAA]arethetreatmentoptionsinelectivecases.Ofthese,thesurgeriesapartfrompouchareroutinelyperformedforotherindicationsalsoandarenotdiscussedatlengthinthis

Emergency Surgery Elective Surgery

GastrointestinalbleedingIntestinalobstructionIntestinalperforationSevere colitis/Toxicmegacolon not responding tomedicaltherapyin72hoursofstartingtreatment

Steroid dependenceSteroidrefractorydiseaseNon-compliancetomedicaltherapySuspected/confirmedmalignancyFinancialconstraintstomedicaltherapyNottoleratingadverseeffectsofmedicines

Indications specific to Crohn’s Disease

Intractablefistula[Enterocutaneous,entero-enteric,enterovesical,enterovaginal]•Complex/Simpleperianalfistula•Growthretardation•Intra-abdominal/Pelvicabscessnotrespondingtomedical/percutaneoustreatment•


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chapter.AwordismentionedhereontechnicaldetailsofpouchsurgeriesforUC[28,29].

7.1. IPAA for IBD

TheIPAAcanbedonebyadoublestapledtechniquewhereinarectalmucosalcuffofupto2cmremainsabovetheanaltransitionzoneoritcanbedonebyhandsewnanastomosisafteramucosectomywhichtheoreticallyremovesall therectalmucosaandhence,protectsagainstfutureriskofmalignancy.Hence,thedoublestapledtechniqueiscontra-indicatedincaseswithdysplasiainlower2/3rdofrectum.Studieshowever,haveshownnodifferenceinoncologicoutcomeswhenthetwotechniquesarecompared[30].

Theproblemwithmucosectomyisthatitisnotalwayscompleteandislandsoftissuesareoftenleftbehindwhichmayleadtomalignancy.Thisrectalcuffisburiedbehindtheanas-tomosisandhence,isnotamenabletoendoscopicsurveillanceorbiopsyacquisition.Also,duetoextensiveretractionduringsurgery,thereisfoundtobehigherriskofsphincterdamageandalso,becauseoflossofcompleterectalmucosa,thereislossofdiscriminationbetweenflatusand stool and results in incontinence.Studieshave shownahigher rateofnocturnal seep-ageascomparedtothedoublestapledtechnique.Inadditiontotheseproblems,itisdifficultintraoperativelybecausecompletemucosectomyandhandsewnileo-analanastomosisneedadditional2-4cmofmobilizationofpouchtomakeitreachthelowerstumpwhichmaybedifficultinsomecases–Mayleadtotensionontheanastomosisandproblemswithitsbloodsupplywhichmaypredisposetopostoperativepouchrelatedcomplications[31,32].

TheshapeofthepouchconstructedcanbeS,WorJshaped.SandWshapedpouchesarecomplextoconstruct.Also,theyoftendilateexcessivelyovertimeandleadtofecalstasisandanastomoticstenosisattheileo-analend.TheSpouchhasalongoutflowlimbandthiscancauseproblemswiththeemptyingofthepouch.FailureratedofSandWpoucharehighatnearly50-60%.Jpouchiseasiertoconstruct,haslesscomplicationsthantheothertwobut,hasmorediarrheaepisodesinitially.TheanatomyoftheJpouchisasshownintheFigure 5 below[33].

7.2. Minimally invasive surgery for IBD

LaparoscopyhasevolvedslowlyforIBDwhencomparedtotheotherindications.Thisisbecauseofseveralfactors.Thediseaseischaracterizedbyinflamedtissues,multipleop-erationsandbadplanesdue to inflammationandprevious surgery.Also,patientsareoftenmalnourishedwith low albumin, are anemic,may be on chronic steroids andmay have astronghistoryofsmoking,allofwhicharedetrimentaltosurgicaloutcomes.Incurrenttimes,laparoscopyisconsideredfeasibleandsafeforfirstelectivesurgeryaswellasforemergencysurgeries for idiopathic IBD in expert handswith equivalent surgical outcomes.However,nostudieshavebeenabletodemonstrateconclusivelythataddedbenefitsoflaparoscopyon


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postoperativescoresandpainscorestranslateintopracticeandthesebenefitshavenotreachedstatisticallysignificantlevelsacrossstudies[34].

Laparoscopyisassociatedwithhigheroperativetimes,but,lowerbloodloss.Penetrat-ingtypeofIBDhasbeenshowntobeassociatedwithhigherconversionratesandhigherratesofstomacompared to laparoscopyforother indications inIBD.Technologicaladvances inlaparoscopyandtheadventofroboticsurgeryhaveencouragedsurgeonstousethesemodali-tiesinpatientsofIBDalso[35].

Both hand assisted laparoscopy (HALS) and Single incision laparoscopy (SILS) aswellasnaturalorificespecimenextraction(NOSE)andtransanalminimallyinvasivesurgery(TAMIS)haveallbeenattemptedforIBDsurgeryandallhaveclearedthesafetyandfeasibil-itystage.HALSrestorativeproctocolectomyisassociatedwithshorteroperativetimeswithnoothersignificantdifferencecomparedtocompletelaparoscopicsurgery.SILShasnotgainedfamesofarandstudiesarescantyforthisindication.Whetheritisbeneficialstatisticallyisnotyetestablished.TAMIShasbeenusedfortotalmesorectalexcisiontoachievetherightplanefromperinealsideincombinationwithabdominalsurgeryinrectalcancer.FeasibilityinIBDforrectaldiseaseandcomplexfistulashasbeenestablishedwhereaslongtermresultsonout-comesareawaited[34,35].

Roboticsurgeryhasalreadydemonstratedbenefitforrectalsurgeriesowingtothedex-teroushandofrobottoworkinthenarrowpelvis.Nervepreservationratesarehigherwithrobotic pelvic dissections for rectum.Hence, robotic completion proctectomy is a feasibleandgoodoption.Ontheotherhand,forothersurgeriesofIBD,roboticinstrumentswillberequiredinmorethanoneabdominalquadrantandthecostandtimerequiredforthesestepsmaynotbeasbeneficial[36].

8. Life after first Surgery

Thisistheperiodwherethepatienthasachievedcontrolofthediseaseandisonmain-tenanceprotocols.Theissueshereincludemonitoringforprogressionofdisease,relapse,dys-plasiaandcancerorextra-intestinalmanifestationsaswellasthecomplicationsofthesurgery.Focusalsoneedstobeonassessmentforlifestylechangesandqualityoflifeissues.Thesepointsarenowdiscussedintheremainingchapterandthemanagementofassociatedproblemsarepresented.

Alotofparametershavebeenevaluatedforuseasmarkersofdiseaserelapseorpro-gressionaswellastoevaluatetheresponsetotherapysuchasc-reactiveprotein,erythrocytesedimentationrate,fecallactoferrin,fecalneopterinetc.Onlyfecalcalprotectinlevelshavebeenshowntobehelpfulinthisregard.Thiscalciumandzincbindingproteinisproducedbyneutrophils, remains stable inunprepared samples forupto7days,helps indifferentia-


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tionbetweenIBDandirritablebowelsyndromeandismoreaccuratethanCRPandESRformonitoringfordiseaserelapse.Elevationabovenormalvaluefor2consecutivevaluespredictarelapsewithinthenext3monthswithasensitivityof95%andspecificityof91%.Regimesfortestinghavebeenmonthlyto3monthlyacrossdifferentstudies[2].

Response isusuallymonitoredbyclinicalparameters.Endoscopicdocumentationofmucosalhealingisnotmandatory.However,incaseofdoubt,colonoscopicevaluationinCDorlimitedsigmoidoscopicevaluationafterpouchforUCtoevaluatemucosalhealingisap-propriate[1,28].

Figure 5:PartsofJpouchandrelatedpostoperativeeventsatspecificpoints

Studieshaveshownthatifapatientachievesaclinicalremission,thetypeofsur-geryi.e.stomaversuspouchdoesnotmakeamajordifferenceinthequalityoflifeandmorethan95%ofthesepatientsdorecommendsurgerytootherpatientswhenaskedafter5yearsoftheirfirstsurgery.Onevaluationofdailyactivitiesthough,theJpouchperformedbetterintermsofsocialinteraction,recreationalactivities,sexuallife,participationinsportsandalliedactivitiesetc.However,thedifferencewasnotsignificantoverall[37].

Physiologically,thesepatientshavealimitedphysiologicalreservetofightsituationswithfluid losses.This isbecausecoloncan increase its absorptivecapacity from1-1.5 lit/daytoupto5lit/dayincaseofneedforwater.Similarly,coloncancausesaltreabsorptiontoexcretelessthan2meq/dayincaseofneed.Withileostomyorpouchdiarrheainabsenceofcolon,thesepatientshaveanobligatoryfluidlossof500-800ml/dayandsodiumlossof30-40meq/daywhichisnotmodifiable.Also,thepatientsdevelopvitaminB12,folateandsecond-


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arybileaciddeficiencyduetolossofterminalileumandcolonandtheseneedtobereplaced.[37,38].

Numberofdaytimeandnighttimedefecationepisodesrangefrom6/dayand1-2/night.Incontinenceusuallyincreaseswithtimeandmaydoubleoverthenext15-20yearsaftersur-gery.However,theoverallsuccessrateofpouchisstillupto90%.SexualdysfunctionafterIPAAoccursintheformofdyspareuniain5-7%womenandretrogradeejaculationandimpo-tencein4%and2%malesrespectively.Overalllifeexpectancyhowever,isnotchangedafterthesurgeryinthelongtermprovidedtherearenoothercomplications[39].

9. Complications and their Management

Theoverallmorbidityrateshavecomedownduetoadvancesinsurgicaltechnology,equipmentsaswellasimprovedperioperativecareofthepatient.However,themorbidityrateisstillreportedaround30-60%andthemortalityrateisreportedanywhereintherangeof2-17%acrossthevariousstudiesonIPAA.Thecomplications/adverseevents/morbiditiesthatcanoc-curafterthefirstsurgeryforidiopathicIBDareasshowninthe Figure 6[28,31,32,40].

9.1.1. Pouch related septic complications

Nearly20%ofthepatientshavesepticcomplicationsofwhichuptohalfhaveabscesswhichcanbepelvicabscess,intra-abdominalabscessoranastomoticcuffabscess.Theprocessusuallystartsasananastomoticleakin1/3rdofthesepatientswhichcanbefrompouchanalanastomosis>reservoirstapleline>endoftheappendage.

Anastomotic leakismorecommoninobesepatients,patientsoperatedatanage>50yearsandthoseonlongtermsteroiduse.Surgeoninexperienceisalsoassociatedwiththiscomplication.ClinicalpresentationissimilartocasesofGIleakusuallywithpatientdevel-opingfeaturesofsepsistowardstheendofthefirstpostoperativeweekormaypresentwithaltereddrainoutputorwounddischarge.Someanastomoticleakspresentlaterasapersistentsmallenterocutaneousfistula.Diagnosiscanbeachievedbyusingcomputedtomography[CT]scanwithrectalcontrast{maydemonstratealeak/mesentericstranding/extravasationofcon-trast/extraluminalair}.Gentlepouchoscopycanvisualizetheleakincaseofdoubt.Antibiot-ics,bowelrestandpercutaneousdrainageincaseofneedaretheintialmanagementoptions.However,morethanhalfthecasesmayneedsurgerywherein,ifthedefectiseasilyvisibleandsmallwithgoodbleedingedges,aprimaryrepairwithdiversionloopileostomycanbedone.Inallothercases,optionsincludelavageanddiversionorpouchexcisionwithpermanentileo-stomyasalastresort[28,41].


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Figure 6:ComplicationsinthepostoperativelifeafterfirstsurgeryforidiopathicIBD

Abscessescanbeintra-abdominal,pelvicoranastomoticcuffabscess.Anastomoticcuffabscessisuniquetohandsewnpouchanalanastomosiswithmucosectomywhereinbleedingaftermucosectomyandresultanthematomaandanastomoticdehisencewithsecondaryinfec-tion fromabovevia thepelvicspaceorascending infectionfromtheanastomotic leaksitemayleadtothisabscess.Anastomoticleaksandvascularcompromiseofthepouchresultinanabscesssoonaftersurgery.DelayedpresentationshouldraiseaflagtoevaluateforCD.CTscanwithrectalcontrast,magneticresonanceimaging[MRI]ofpelvisforperianalsepsisand/orfistula,examinationunderanesthesiaand/orpouchogramareusefulinvestigationsheretoachieveadiagnosis.Thesearepreferablydrainedtransanallyiffeasibletoavoidfistulacre-ationwhichisapossibilityaftertrans-perinealortrans-vaginaldrainage.Ifitdoesnotresolveorincasesofabdominalabscess,laparotomymayberequiredtodraintheabscesswith/outdivertingstomaordelayofthestomareversalincaseofapre-existingstoma,incaseofaleak.Itisamajorcauseofpouchfailureinupto30-40%cases[42].

Pouch fistulas can arise from appendage, afferent or efferent limb, reservoir suturelineorfromthepouch-analanastomosis.Theotherendofthefistulousopeningcanbeskin,vagina,urinarybladderorotherintestinalloop.Mostcommonoftheseispouch-vaginalfis-tula.Overallincidenceisaround3.5%acrossstudies.Fistularesultingfromanastomoticleakfollowedbyabscessisthemostcommonevent.OtherfactorsresultingintoafistulaincludeimproperapplicationofstaplerduringpouchcreationorCDof thepouch.On thebasisofcause,presentationcanbeanastomotic site fistulaincasesofsurgicalerrororanastomoticleak,perianal fistulaincaseofCDandfistulaatandaroundthedentate lineincasesofcryp-toglandularorigin[43,44].


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Clinicalpresentationissoonaftersurgeryincaseoftechnicalerrors.However,delayedpresentationnearlyafteroneyearofsurgeryismorecommon.Examinationunderanesthesiawithpervaginalandperrectalexamination,pouchoscopyandcontrastenemaorCTwithrec-talcontrastaretheusefulinvestigations.MRIisveryusefulincasesofcomplexfistulasorincasesofdiagnosticconfusion.BiopsyisrequiredincasesofsuspectedCD[40].

Managementofafistulafollowsthesameprotocolsasforanyothercauseoffistulaviz.fluidandelectrolytebalance,sepsiscontrol,woundmanagement,nutrition,evaluationandplan-ningfordefinitivesurgeryandfinallypost-surgerycare.SpecificallyinIBD,local(transvaginalorperineal)approachispreferredforlowlyingfistulasatthepouch-analanastomosiswith/outlocaladvancementflaps.Abdominalapproachtorepairisutilizedinhighfistulas(abovethepouch-analanastomosissite)whereinthepatientsmayalsorequiredivertingloopileostomyforsepsiscontrol.Collagenplugswith/outbuttonstoplugthefistulas,excisionoffistulaandprimaryrepairwithmeshpartitionofthetwoorgansareotheroptions[40,43,44].

Nearly20-25%patientsendupwithpouchfailureleadingtopermanentileostomybe-causeofrecurrentorrefractoryfistulas.Medicalmanagementwithinfliximabhasbeenshowntobenefitthesepatients.Surgicaltimingwithinfliximabdosingisessential.Initialdosingisat0,2and6weeksfollowedby8weeklydoseswhereinsurgeryisplanned4weeksafterthedoseandthenextdoseisgiven4weeksafterthesurgery[44,45].

9.1.2. Non-septic pouch related complications

Pouchitisisanidiopathic,nonspecificinflammationoftheilealpouch.Itisthemostcom-monofthepouchrelatedcomplicationsseeninupto40%ofthecasesforulcerativecolitisandonlyaround8-10%casesafterpouchconstructionforfamilialadenomatouspolyposis(FAP).Itsincidenceincreaseswiththedurationaftersurgeryandupto75%ofthepatientssufferfrompouchitisatleastoncewithin20yearsofthesurgeryforitscreation.Themostcommontimeperiodisthepostoperative6monthperiodafterdivertingstomaclosureifitwascreated.Etiol-ogyisnotknown.Riskfactorsincludedurationofulcerativecolitispriortosurgery,durationofstomapriortoclosureandnumberofsurgeriesrequiredbeforepouchcreationorforpouchcreation.Itisoftwotypes–AcuteandChronicasshowninTable 3.Itmustberememberedthatacuteandchronicarebasedonsymptomduration[46,47].


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Table 3:Differencesbetweenacuteandchronicpouchitis

Clinically, these patients have intermittent or persistentsymptomsrelatedtopouchaswellassystemicsymptomsrelatedtotheinflammatoryprocessanditssystemiceffects.Local symptomsincludeabdominalcramps,fecalurgency,bleedingperrectumandtenesmus.Sys-temic symptomsincludefever,anemia,electrolytedisturbancesandgeneralizeddiscomfortandmalaise[47,48].

DiagnosisisaidedbythescoringsystemssuchasPouchitisdiseaseactivityindex(PDAI)wherebyclinical,endoscopicandhistologicfeaturesareclubbedtogetherandascore≥7isdiagnosticforpouchitis.Endoscopically,themucosaisedematous,friablewithlossofvascu-larpatternduetoedema.Also,theremaybemucosalgranularity,mucoidexudatescoveringthemucosaormucosalulcers.Biopsyfeaturesshowvillousatrophyordistortionofcryptar-chitechtureormucosalpolymorphonuclearinfiltrationandulceration.ManagementalgorithmisasshowninFigure 7[28,48,49].

Cuffitisischronic,nonspecificinflammationoftheretainedcuffofrectalmucosajustabovetheanaltransitionzoneindoublestapledtechniqueofpouchreconstruction.Incidenceislowerthanpouchitisandisaround15%.Clinicalpresentationissimilartopouchitisanditsmedicalmanagementisonthesamelinesasforpouchitisexceptthattheantibioticsarenotusefulforcuffitisandhencethemanagementprotocolstartswithtopicalsteroidsandmesacolenemas.Refractorycuffitis ismanagedsurgicallybycombinedabdominalandperinealap-proachtoperformcompletemucosectomyandpouchadvancementwithre-anastomosisandalmostalwaysadivertingloopileostomytobeclosedatalaterdate[28,46].

Small bowel obstructionisseeninupto20%cases.Intestinalobstructioninthesecasescanbe because of structural reasons or non-adhesive obstruction and adhesive obstructionwhichcanpresentwithin90days(early)orafter90days(late).Usually,structuralcausesleadtoearly intestinalobstructionwhereasadhesiveobstructionpresents late.Themanagementofadhesiveobstructionfollowsthesameprinciplesasforanyadhesiveintestinalobstruction[50].Structuralcausesneedspecificmanagementandthisisasfollows:

Acute pouchitis Chronic pouchitis

Symptomduration<4weeks Symptomduration>4weeks

Presentationisdelayedafterstomaclosure Presentationisearlyafterstomaclosure

Incidence–7-8% Incidence–10-12%

PatientshavelowlevelofpANCAactivity PatientshavehighlevelofpANCAactivity

Extra-intestinalmanifestations–Primarysclerosingcholangitis,historyoflongtermsteroiduseandsmokingpredispose

Postoperativecomplicationsafterpouchsurgerypredispose.

Smokingisprotective

Antibioticresponsive Antibioticdependentorrefractory


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Anastomoticstenosisatpouch-analanastomosis–RepeatedanaldilatationswithHe-gar’s dilators

Redundantandlongdilatedappendage(>2cm)–Revisionpouchsurgerywithexcisionoftheexcessappendage

Floppypouchreservoir–Laparotomywithpouchpexytosacrumandinseverecases,revisionpouchsurgerywithcreationofajejunapouchorpouchexcisionwithpermanentileo-stomy

Pouchvolvulus–Untwistingofpouchandpouchpexyorpouchexcision

Pouchprolapse–Mucosalprolapsedcanbemanagedwith stoolbulkingagentsandbiofeedbacktherapyandifitdoesnotresolveonthat,trans-analmucosalexcisioncanbeper-formed.Fullthicknessprolapsedrequireslaparotomywithpouchpexyorpouchexcisionandpermanentileostomy[28].

Figure 7:Treatmentalgorithmforpouchitis


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Postoperative stricturesoccuratanincidenceof10-15%afterthesesurgeries.Patientfactors(obesity, smoking),surgeonfactors (handsewnanastomosis,anastomosisunder ten-sion,poorbloodsupplyofpouch)andpresenceofdiverting ileostomyare the risk factorsforpostoperativestrictures.Stapledanastomosisusuallyresultinshort,non-fibroticstrictureswhicharemanageablewithendoscopicdilatationwhereashandsewnanastomosiswithmuco-sectomyresultsinlongandfibroticstrictureswhicharedifficulttomanageendoscopicallyandaremanagedwithtransanaladvancementflapanoplasty.Ifthestrictureisproximaltoafferentlimb,strongsuspicionshouldbemadeforCDandifidentified,itismanagedasforCDstric-turesbystrictureplasty/bypassandmedicalmanagement.The last resort ispouchexcision.[51].

Dysplasia and carcinomaarealso reportedafter IPAA.Squamouscellcancerat theperianalregion,adenocarcinomaofthepouchortheafferentlimbareallpossibilities.Hence,surveillanceisrecommendedforpatientsathighrisksfortheseeventsviz.patientswithhis-toryofprimarysclerosingcholangitis,orcancerintheresectedcolonicspecimenorhistoryofulcerativecolitismorethan10yearsduration.Surveillancescopyinthesepatientsisrecom-mendedeveryyear.Allotherpatientscanbefollowedupwithendoscopy5yearly[52,53].

Crohn’s disease of the pouchisoneofthemostcommonreasonsforpouchfailureanditsincidenceisaround10%.AsdiscussedinnaturalhistoryofCD,inpouchalso,thediseaseissuspectedwheninflammatory,fibroticorpenetratingdiseaseoccursinpouchoritsvicinity.Thus,CDofpouchissuspectedwhenpatienthasinflammatorydiseasecharacterizedbyrecur-rent(>4)episodesofpouchitisfor2consecutiveyearswhichmaybeantibioticresistantorhaspenetratingdiseaseinformofperianalorsmallbowelfistulasorhasfibroticdiseasewithafferentlimbstricturesoranysmallbowellongsegmentstricture[54].

Riskfactorsincludeapouchsurgeryforindeterminatecolitispreoperativelyandapa-tientwithfamilyhistoryofCDorhavinghistoryofperianalfistulasorintestinalstricturesorinactivesmokers.Thediseaseusuallymanifestsitselfafterthedivertingstomaisreversedandcanbeearlyonset(withinmonths)orlateonset(withinyears)afterthestomaclosure.

TreatmentissameasforCD.Patientswithrefractorydiseasetoconventionaltreatment,youngage,historyofsteroiduse,fistulizingdiseaseespeciallythepouch-vaginalfistulahaveapoorprognosisforpouchpreservation.Nearly30-80%willeventuallyrequirepouchexcision.6MP/AZAhasachievedgoodresponseratesforfibroticCDwhereasinfliximabhasachievedagoodresponserateforallthetypesofCDnotrespondingtoconventionaltreatment.Thewidespreadtrendtowardsearlyaggressivemedical therapyinCDmaytranslate into lowerratesofpouchfailureinfuture[54,55].


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9.2. Stoma related complications

General complicationsrelatedtosmallbowelstomasuchasstomadiarrhea,peristomalexcoriations,stomaproplapse,mucocutaneousseparation,parastomalherniaandstomalob-structionareallpossibleafterthissurgeryandthemanagementisthesameasforothercases.SpecificissuesrelatedtoIBDpatientswithstomaarediscussedbelow.

Prestomal ileitiscanoccursecondarytoIBDrecurrenceorduetobowelobstructionandcanpresentwithsystemicsignsofsepsis,anemiaandendoscopywillshowmultipleulcersinthepre-stomalileum.ItismorecommoninCDthaninUCandthemanagementwilldependonthecause.

Pyoderma gangrenosumisasevere,debilitatingdermatologicmanifestationmorecom-monwithUCthanwithCDthatpresentswithsterilepustulareruptionswith/outulcerationswhichmaygetsecondarilyinfected.Medicalmanagementispreferredwithsteroidsinitiallyandimmunomodulatorsandinfliximabarereservedforrefractorycases.Relocationofstomadoesnothelpinmostcasesbecausethediseasecanrecuratthenewsite[28,50].

9.3. Events related to the natural history of the disease

Thepresentationandmanagementofextra-intestinalmanifestationsissimilarinpre-surgeryandpost-surgeryperiodandisnotdiscussedatlengthhere.Instead,thefocusisonintestinaldiseaseprogressionandassociatedmanifestationsandtheirmanagement.

9.3.1. Recurrent strictures

RecurrentstricturesisthemostcommondiseaserelatedeventinpatientsaftersurgeryforCD.Theratesgoupto35-80%inthesepatients.Riskfactorsincludehistoryofsmoking,multiplepreviousintestinalresectionsandanastomosis,presenceofilealdiseaseandpresenceof>50cmdiseasedbowel(extensivedisease).CTscanwithcontrasthelpsindifferentiatinginflammatoryfromfibroticstrictures.Endoscopy,withitsadvancedtechniquessuchasdoubleballoonorsingleballoonendoscopyandpushenteroscopy,helpinachievingdiagnosisaswellastreatment.Also,endoscopycanbeperformedintraoperativelyincasesofneed[56].

Medicalmanagementworkswellforinflammatorystrictures.Gentleendoscopicdila-tationwith/out self expandingmetal or bioprosthetic stents for 4weeks followed by stentremovalisalsoafeasibleoptionforendoscopicmanagementofthesecases.Foranastomoticstrictures, endoscopic dilatation and intralesional steroid injection followed by endoscopicneedleknifeelectro-incisionunderultrasoundguidancearethetreatmentoptions.Surgeryisindicatedincaseswithfibroticstricturesorinstricturesassociatedwithfistulas/abscess/ma-lignancyaswellasstrictureslongerthan5cmorstricturesclosetoileocecaljunctionwhereendoscopicmanagementwillnotbepossible[57].


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Surgicaloptionsincludestrictureplastyorresectionandanastomosis.HeinekeMicku-liczstrictureplastyisusedforstrictures<10cmlength,Finney’sstrictureplastyisusedforstricturesbetween10-20cmlengthandsidetosideisoperistalticstrictureplastyispreferredforlongerstrictures.Asmentionedbefore,strictureratesarehighandthesepatientsarepronetoshortbowelsyndrome.Hence,principlesofbowelconservationareofutmostimportanceinthesepatients[56,57,58].

9.3.2. Fistulas

Fistulasinthesepatientscanbeperianalorabdominal–entero-enteric,entero-cuta-neous,entero-vesicalorenterovaginal.Forallthefistulasapartfromtheperianalfistulas,themanagement is the sameas forother cases.Perianalfistulas arediscussednext.These aredebilitating,recurrenteventsinCD.Theycanbesimpleorcomplexsameasinotherfistulas.Thediseasecanbeassociatedwithabscess,stricture,fissureorulcerintheperianalregion.PerianaldiseaseisassociatedmostcommonlywithcolorectalCD(40-45%),smallbowelin-volvement(25%)andisolatedperianaldiseaseintheremainingpatients[59].

Examinationunderanesthesiaisthebesttodiagnosethepresenceandextentoffistula.MRIpelvisisthebestmodalityfordiagnosisandnatureofthefistula.Endoscopicultrasound(EUS)isthebestinvestigationfortheassessmentofinvolvementoflowerpelvicmusculature.Managementbeginswithsepsiscontrolandantibiotics.1/3rdto½ofthepatientsneedsurgeryforsepsiscontrolinspiteofantibiotics.Thealgorithmicapproachthatweusetomanageperia-nalfistulasinthesepatientsisshowninFigure 8[60].

9.3.3. Short bowel syndrome

Short bowel syndromehas the samemanifestations,diagnosticcriteriaandmanage-mentoptionsasforanyothercaseandhence,isnotdiscussedindetailhere.Thesepatientsarealsoatriskforgall stones and renal stonesandthemanagementoutlineissimilartoothercasesduetodifferentetiologies.

9.3.4. Dysplasia and cancer

Dysplasiaandcancerareknownevents in thenaturalhistoryofIBD.Longstandingcolitis(>10years),extensivecolitis(>50%coloninvolvement),pancolitis(diseaseuptoorproximaltohepaticflexure),youngmales(<45yearsage),colitisassociatedwithdysplasiaonbiopsyandhistoryofprimarysclerosingcholangitisareknownriskfactorsformalignanttransformation.UCandCDhavethesameriskofcarcinogenesis[61,62,63].

Thediseaseisimportanttorecognizebecausethemeanageofcancerdiagnosisinthesepatientsisless(10-20yearsearlier),diseaseismorecommonlymulticentric(nearlydoubleincidenceofsynchronousdisease),associateddysplasiacanbepresentawayfromcancersites


19

inIBD,p53mutationsaremorecommonandRasmutationsarelesscommonandlateandthetumorsareoftenpoorlydifferentiated,anaplasticandmucinouswhencomparedtosporadiccancers.

DiagnosisandmanagementisbasedontherecentSCENICrecommendations.Surveil-lanceisbeganafter8yearsofdiagnosisofpancolitisandafter15yearsofdiagnosisofleftsidedcolitisastheriskofmalignancyislowinleftsidedcolitis.Foralltheabovementionedriskfactors,thesurveillanceendoscopyisdoneyearly.Inallothers,thetestisperformed5yearly.Also,thepatientswithfirstdegreerelativewithcolorectalcanceratage<50areathighriskandthesurveillanceintheseisalsocarriedoutyearly.Chromoendoscopyandtargetedbiopsyisnowthepreferredendoscopicmethodofsurveillanceifavailableasithasshowntoincreasethedysplasiadetectionrateby7%.Thepreviouslyused4quadrantbiopsyevery10cmrecommendationisnolongeranabsoluterequirementforsurveillancenow[64].

Figure 8:AlgorithmicapproachtomanagementofperianalfistulasassociatedwithCD


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Ifalesionisidentifiedonendoscopy,theParisclassificationisfollowedforthelesionsidentificationandtheirmanagementwhichisasfollows:

IporIs(Polypoidlesionpedunculatedorsessile)–Endoscopicmucosalresection•

Non-polypoidlesion–flatbutelevatedwith/outmildcentraldepression(IIa,IIb)–•Endoscopicmucosalresection

Non-polypoid lesionbut IIc (flatandnonelevated,mucosaldepressionand raised•edge)–Endoscopicsubmucosaldissection

Margins not distinct, high grade dysplasia, carcinoma – Total proctocolectomy•anddependingonthelowertwo-thirdsofrectum,pouchifnocancerorhighgradedysplasia there, ileostomy if cancer is present there and handsewn pouch withmucosectomyifhighgradedysplasiaispresentthere[64,65,66].

10. Conclusion

TherearealotofissuestotakecareofinpatientswithIBDevenaftertheirfirstattemptatmedicalandsurgicalmanagementisoverandremissionisachieved.Theseeventsneedtobeunderstoodandstandardizedmanagementguidelinesneedtobeunderstoodtotreatthesecomplexsituationsandprovidethesepatientswithadiseasefreegoodqualityoflife.

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