Role of surgery for idiopathic inflammatory bowel disease with a focus on postoperative events and their management Gunjan 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] The inflammatory disorders of bowel are very common in gastrointestinal clinics. These are characterized by intermittent relapsing and remitting course or chronic inflammatory course affecting the gastrointestinal tract and comprise of a spectrum of disorders as shown in Figure 1 [1]. In this chapter, the focus is on understanding idiopathic IBD, especially ulcerative coli- tis (UC) and crohn’s disease (CD) from a surgeon’s perspective with specific focus on life after surgery for this IBD. 2. Natural History of the Disease and its Relevance to Clinical Practice Idiopathic IBD is relapsing and remitting or chronic progressive disease wherein the disease natural history can be divided into 4 phases based on the disease activity. Phase I: Detection/diagnosis of disease based on clinical presentation: Active or complicated disease Phase II: Initiation of treatment and achieving the phase of remission Phase III: Phase of monitoring to maintain remission and early detection of relapse/complica-
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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,
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].
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
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].
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].
GastrointestinalbleedingIntestinalobstructionIntestinalperforationSevere colitis/Toxicmegacolon not responding tomedicaltherapyin72hoursofstartingtreatment
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].
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
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].
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].
Clinically, these patients have intermittent or persistentsymptomsrelatedtopouchaswellassystemicsymptomsrelatedtotheinflammatoryprocessanditssystemiceffects.Local symptomsincludeabdominalcramps,fecalurgency,bleedingperrectumandtenesmus.Sys-temic symptomsincludefever,anemia,electrolytedisturbancesandgeneralizeddiscomfortandmalaise[47,48].
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:
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].
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.
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].
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].
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].
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].
64.LaineL,KaltenbachT,BarkunA,McQuaidK,SubramanianV,SoetiknoRetal.SCENICinternationalconsensusstatementonsurveillanceandmanagementofdysplasia in inflammatoryboweldisease.GastrointestinalEndoscopy.2015;81(3):489-501.e26.