肛門直腸畸形大課

Congenital

AnorectalMalformationsProfessorYuZuoBaiDepartmentofPediatricSurgeryShengjingHospitalChinaMedicalUniversity目前一頁\總數(shù)六十八頁\編于十八點(diǎn)PediatricSurgeryMalformationdeformityabnormalitiesInflammationTraumaTumorHandAnus目前二頁\總數(shù)六十八頁\編于十八點(diǎn)AlternativeNamesImperforateanus(indexword)CongenitalmalformationsoftheanusandrectumCongenital

anorectalmalformationAnalanomalyAnalatresia

Congenital

AnorectalMalformations目前三頁\總數(shù)六十八頁\編于十八點(diǎn)

Incidence

Thenumbersarequitevariable:1/5000----1/1000Theaverageincidence:1in5000livebirthsChina:1/2800

2001JAmMedGenetics1846/4618840,4.05/10000,1/2500EuropeMale:Femaleabout1:0.7MostcommongastrointestinalmalformationsGeneralIntroduction目前四頁\總數(shù)六十八頁\編于十八點(diǎn)ThecauseofanorectalmalformationsisunknownGeneticandenvironmentalfactorsinteractoneachothertogiverisetoanorectalmalformationsGeneticsplayedanimportantroleintheoccurrenceofanorectalmalformations

EtiologyandEmbryology目前五頁\總數(shù)六十八頁\編于十八點(diǎn)Thecloacaiscomposedofallantoisandhindgut(4w)

Theurorectalseptumdividesthecloacaintoananteriorurogenitalsinusandposteriorhindgut(5w)

Theurorectalseptumgrowstowardsthecloacalmembrane(5-7w)Theanalmembraneruptures,creatingtheanalopeningforthehindgut,itistheanus(8w)

Theurorectalseptumformstheperinealbody(8w)

Embryology目前六頁\總數(shù)六十八頁\編于十八點(diǎn)Iftheurorectalseptumdoesnotcompletelydividethecloaca,therectumwillconnectanteriorwithurinaryorgenitalstructures,resultinginanimperforateanuswithfistulaInsummary,congenitalanorectalmalformationsarecausedbyabnormalitiesinformationofthecloacaduringthefourthandeighthweeksofgestationEmbryology目前七頁\總數(shù)六十八頁\編于十八點(diǎn)4thweek5-6thweek7-8thweekThedevelopmentoftherectumandanus目前八頁\總數(shù)六十八頁\編于十八點(diǎn)InternalanalsphincterexternalanalsphincterlevatormusclepuborectalispubococcygeusmuscleiliococcygeusmuscleAnalsphinctermuscle目前九頁\總數(shù)六十八頁\編于十八點(diǎn)levatormuscleinternalanalsphincterexternalanalsphincter目前十頁\總數(shù)六十八頁\編于十八點(diǎn)InternalanalsphincterisathickenedcontinuationoftheinnercircularlayerofrectalmuscleIsinnervatedbyvisceralnervesResponsibleforpreventingthepassageofsolidandliquidstoolandgasSympatheticfibers---contraction--nostimulationofrectum—closetheanalcanalParasynpatheticfibers---relaxation--stimulationofrectumInternalanalsphincter目前十一頁\總數(shù)六十八頁\編于十八點(diǎn)External

analsphincterDeepExternal

analsphincterSuperficialExternal

analsphincterSubcutaneousExternal

analsphincterInnervatedbytheinferiorrectalbranchofthepudendalnerve(analnerveandperinealnerve)originatingintheanteiordivisionsofthesecondtofourthsacralnerveroots目前十二頁\總數(shù)六十八頁\編于十八點(diǎn)puborectalispubococcygeusmuscleiliococcygeusmusclelevatormuscle目前十三頁\總數(shù)六十八頁\編于十八點(diǎn)elevatestherectumpulltherectumforwardThepuborectalisistheportionmostcloselyassociatedwiththerectumAresuppliedbythefourthsacralneverandtheinferiorrectalorperinealbranchesofthepudendalnervesPuborectalis----

thethirdsphincter目前十四頁\總數(shù)六十八頁\編于十八點(diǎn)Rectoanalangle

(about80°)Thevariousportionsofthelevatoranimusclearesurroundedtheanusandrectumandtendtopulltherectumforward,toelevatetherectum,formingtheanglebetweenthelongitudinalaxisoftherectumandtheanalcanal.ThisrectoanalanglehelpstomaintaincontinencebypreventingformedstoolfromenteringtheanalcanalStriatedmusclecomplexThismusclecomplexiscomposedofafusionofthepuborectalportionofthelevatoranimuscleandexternalsphinctermuscles,includingadeepexternalsphinctercomponent,whichcannotbeidentifiedclinicallyRectoanalAngleandStriatedMuscleComplex目前十五頁\總數(shù)六十八頁\編于十八點(diǎn)RectoanalAngleandStriatedMuscleComplex目前十六頁\總數(shù)六十八頁\編于十八點(diǎn)Thelandmarkofclassificationofanorectalmalformationispubococcygealline(puborectalis)Therelationshipoftheendoftherectumtothepuborectalismuscledividestheimperforateanusintohigh,intermediateandlowtypesClassification目前十七頁\總數(shù)六十八頁\編于十八點(diǎn)ClassificationIftherectalpouchabove(supralevator)thelevatormuscle(puborectalis),itistermedahightypeIftherectalpouchat(translevator)thelevatormuscle(puborectalis),itistermedaintermediatetypeIftherectalpouchbelow(infralevator)thelevatormuscle(puborectalis),itistermedalowtype目前十八頁\總數(shù)六十八頁\編于十八點(diǎn)目前十九頁\總數(shù)六十八頁\編于十八點(diǎn)ThepropertreatmentofimperforateanusdependsonthetypethatisencounteredDeterminationofthelevelofthelesioniscriticalforappropriatemanagementEachtypeofanorectalmalformationrequiresadifferentoperationandmedicalmanagementClassification目前二十頁\總數(shù)六十八頁\編于十八點(diǎn)WingspreadClassificationofAnorectalMalformation(1984)

High

AnorectalagenesisWithrectovaginalfistula

Withoutfistula

Rectalatresia

Intermediate

Rectovestibularfistula

Rectovaginalfistula

Analagenesiswithoutfistula

LowAnovestibularfistulaAnocutaneousfistula

Analstenosis

CloacaRaremalformations

LowAnocutaneousfistula

Analstenosis

RaremalformationsFemaleMale

High

AnorectalagenesisWithrectoprostaticurethralfistula

Withoutfistula

Rectalatresia

IntermediateRectobulbarurethralfistula

Analagenesiswithoutfistula

目前二十一頁\總數(shù)六十八頁\編于十八點(diǎn)PediatrSurgInt(1986)1:200-205WingspreadClassificationofAnorectalMalformation(1984)目前二十二頁\總數(shù)六十八頁\編于十八點(diǎn)StandardsforDiagnosisInternationalClassification(Krickenbeck2005)MajorclinicalgroupsRare/regionalvariantsPerineal(cutaneous)fistulaPouchcolonRectourethralfistulaRectalatresia/stenosisProstaticRectovaginalfistulaBulbarHfistulaRectovesicalfistulaOthersVestibularfistulaCloacaNofistulaAnalstenosisJPediatricSurgery,2005,40,1525目前二十三頁\總數(shù)六十八頁\編于十八點(diǎn)OtherClassificationsAnaldeformitiesRectaldeformitiesLowtypeHightypeNointermediatetype目前二十四頁\總數(shù)六十八頁\編于十八點(diǎn)Anoperineal/anocutaneousfistula目前二十五頁\總數(shù)六十八頁\編于十八點(diǎn)Anoperineal/anocutaneousfistula目前二十六頁\總數(shù)六十八頁\編于十八點(diǎn)Rectourethralfistula目前二十七頁\總數(shù)六十八頁\編于十八點(diǎn)Rectovesicalfistula目前二十八頁\總數(shù)六十八頁\編于十八點(diǎn)Anoperinealfistula目前二十九頁\總數(shù)六十八頁\編于十八點(diǎn)Rectovestibularfistula目前三十頁\總數(shù)六十八頁\編于十八點(diǎn)Rectovaginalfistula目前三十一頁\總數(shù)六十八頁\編于十八點(diǎn)Persistentcloaca目前三十二頁\總數(shù)六十八頁\編于十八點(diǎn)PathologicalchangesareverycomplicatedSphinctermuscleNeverSacrumAssociatedanomaliesThehigherthedefect,thesevererthepathologicalchange,thelessthelikelihoodwillbeofachievingbowelcontrolPathology目前三十三頁\總數(shù)六十八頁\編于十八點(diǎn)VACTERLAssociation

VACTERLV vertebralA AnorectalC CardiacT Tracheo-esophagealfistulaE EsophagealatresiaR RenalL Limb目前三十四頁\總數(shù)六十八頁\編于十八點(diǎn)AssociatedAnomaliesCardiovascularGastrointestinalSpinalandvertebralGenitourinaryGynecologic目前三十五頁\總數(shù)六十八頁\編于十八點(diǎn)SymptomsarevariableDifferenttype:differentSymptomsThelevelofdistalpouchWithorwithoutfistulaSizeandpositionofthefistulaAssociatedanomaliesClinicalPresentations目前三十六頁\總數(shù)六十八頁\編于十八點(diǎn)Nopassageoffirststoolwithin24to48hoursafterbirthLifelonghistoryofconstipationStoolpassedbywayoffistulaAbsenceofanalopeningMisplacedanalopeningVomitingandabdominaldistention

ClinicalPresentations目前三十七頁\總數(shù)六十八頁\編于十八點(diǎn)WithoutfistulaLowerintestinalobstructionNopassageofstoolorgasAbdominaldistentionandvomitingPhysicalexamination：NoanusFlatperineumwithbulgingoncryingNoexternalsphinctercontractiononscratchingtheperineumClinicalPresentations目前三十八頁\總數(shù)六十八頁\編于十八點(diǎn)WithfistulaMale：PassageofmeconiumintheurinePassmeconiumorflatusviapenisMeconiumpassedfromthefistulaintheperineumwithlowerobstructionFemale：AbnormalanalopeningPassstoolfromvestibulumorvaginaOnlyoneorificeintheperineum----cloacaClinicalPresentations目前三十九頁\總數(shù)六十八頁\編于十八點(diǎn)Anoperinealfistula:Meconiumpassedfromthefistulaintheperineum目前四十頁\總數(shù)六十八頁\編于十八點(diǎn)Anoperinealfistula:Meconiumpassedfromthefistulaintheperineum目前四十一頁\總數(shù)六十八頁\編于十八點(diǎn)RectourethralfistulaPassageofmeconiumintheurinePassmeconiumorflatusviapenis目前四十二頁\總數(shù)六十八頁\編于十八點(diǎn)Female:imperforateanuswithfistula目前四十三頁\總數(shù)六十八頁\編于十八點(diǎn)Imperforateanuswithoutfistula目前四十四頁\總數(shù)六十八頁\編于十八點(diǎn)ImperforateAnusDiagnosis

HistoryFailuretopassmeconiumwithinthefirst24hoursoflifeLifelonghistoryofconstipationThoroughexaminationofperineumMustperformathoroughperinealinspection目前四十五頁\總數(shù)六十八頁\編于十八點(diǎn)ThediagnosisiseasilymadebyhistoryandthoroughexaminationofperineumTheappearanceoftheperineumdoesnotnecessarilypredictwhetherthelesionislow,intermediateorhighDiagnosisstudies目前四十六頁\總數(shù)六十八頁\編于十八點(diǎn)Thepurposesofspecificdiagnosisstudiesare:Todeterminetheleveloftheblindrectalpouchwhetherlow,intermediateorhightypeToidentifyanyassociatedfistulouscommunicationsTodeterminethepresenceorabsenceofanyothercongenitalanomaliesToassessthestatusofthelevatoranimuscleandanalsphinctermuscleDiagnosisstudies目前四十七頁\總數(shù)六十八頁\編于十八點(diǎn)X-rayCT/MRIFistulogramDistalcolostogramLoopogramOthersDiagnosisstudies目前四十八頁\總數(shù)六十八頁\編于十八點(diǎn)Ifnoneoftheclinicalsignstodeterminethelocationoftheanorectalanomalyareevidentby24hours,performingaradiologictestcanhelpThissituationisonlynecessaryinabout10%ofpatientsthatwithoutfistulaLateralpelvicradiographyisperformedinbabieswhohavenoexternalevidenceoffistula,whopassnomeconiumafter24hours,andwhohavenomeconiumintheurineX-ray目前四十九頁\總數(shù)六十八頁\編于十八點(diǎn)X-rayInvertogram(Wangensteen\Rice1930)UpsidedownlateralfilmPronecross-tablelateralradiographs目前五十頁\總數(shù)六十八頁\編于十八點(diǎn)I-point,isthelowestpointoftheischialtuberosity,representsthedeepestpointofthelevatoranimusclesThepubococcygeallineisthelinethatconnectstheupperborderofthesymphysispubisandsacrococcygealjunction,itrepresentstheupperlimitsofthelevatormusculature(puborectalissling).ItisthelandmarkforclassificationofanorectalmalformationThepubococcygealline(PCline)andI-point目前五十一頁\總數(shù)六十八頁\編于十八點(diǎn)PClineIpointAbovethePCline----highBelowtheIpoint-----lowBelowthePClineAbovetheIpoint----intermediateTheleveloftherectalpouch目前五十二頁\總數(shù)六十八頁\編于十八點(diǎn)目前五十三頁\總數(shù)六十八頁\編于十八點(diǎn)BowelskindistanceThedistancebetweentheendoftherectumandtheopaquemarkermeasuresmorethan2cm,itmeansthattherectumlieshigh目前五十四頁\總數(shù)六十八頁\編于十八點(diǎn)X-rayfilmsshouldbetakenmorethan12hoursoflifetoallowenoughtimeforbowelgastotheendoftheblindrectumThechildshouldbeheldverticallyupsidefor3minutesbeforethefilmistakenThehipshouldbekeptrelativelystraightPlaceanopaquemarkerontheperinealskintodenotethecutaneousleveloftheanusX-ray目前五十五頁\總數(shù)六十八頁\編于十八點(diǎn)persistentcloacaFistulogram目前五十六頁\總數(shù)六十八頁\編于十八點(diǎn)DistalColostogramRectovaginalfistulaRectourethralfistula目前五十七頁\總數(shù)六十八頁\編于十八點(diǎn)CT/MRI目前五十八頁\總數(shù)六十八頁\編于十八點(diǎn)SurgicalPrincipalThepropertreatmentofimperforateanusdependsonthetypethatisencounteredEachtypeofanorectalmalformationrequiresadifferentoperationandmedicalmanagementThetreatmentsometimesdependsontheexperienceofthesurgeonandgeneralconditionofthepatientTreatment目前五十九頁\總數(shù)六十八頁\編于十八點(diǎn)SurgicalPrincipal

Whattime?Emergentoperation:withoutfistulaorthinfistulacausingintestinalobstructionImperforate"perforate”anusDelayedoperation:withwidefistula,anoplastyuntil3-6monthsafterbirth目前六十頁\總數(shù)六十八頁\編于十八點(diǎn)

Whichprocedure?Colostomyornot?Lowtype(analdeformities) perinealanoplastywithoutcolostomyIntermediateand

hightype

(rectaldeformities) Colostomy LaparoscopyassistedpullthroughVsPSARP ClosureofColostomySurgicalPrincipal

PSARP

posteriorsagittalano-rectoplasty目前六十一頁\總數(shù)六十八頁\編于十八點(diǎn)Colostomyornot?Intermediateand

high

type

(rectaldeformities)high-----colostomyNowadays-------withnocolostomyLaparoscopyassistedpullthroughVsPSARPDependingontheexperienceofthesurgeonandgeneralconditionofthepatientPSARP

p