肛门直肠畸形大课

Congenital

AnorectalMalformationsProfessorYuZuoBaiDepartmentofPediatricSurgeryShengjingHospitalChinaMedicalUniversity目前一页\总数六十八页\编于十八点PediatricSurgeryMalformationdeformityabnormalitiesInflammationTraumaTumorHandAnus目前二页\总数六十八页\编于十八点AlternativeNamesImperforateanus(indexword)CongenitalmalformationsoftheanusandrectumCongenital

anorectalmalformationAnalanomalyAnalatresia

Congenital

AnorectalMalformations目前三页\总数六十八页\编于十八点

Incidence

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

2001JAmMedGenetics1846/4618840,4.05/10000,1/2500EuropeMale:Femaleabout1:0.7MostcommongastrointestinalmalformationsGeneralIntroduction目前四页\总数六十八页\编于十八点ThecauseofanorectalmalformationsisunknownGeneticandenvironmentalfactorsinteractoneachothertogiverisetoanorectalmalformationsGeneticsplayedanimportantroleintheoccurrenceofanorectalmalformations

EtiologyandEmbryology目前五页\总数六十八页\编于十八点Thecloacaiscomposedofallantoisandhindgut(4w)

Theurorectalseptumdividesthecloacaintoananteriorurogenitalsinusandposteriorhindgut(5w)

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

Theurorectalseptumformstheperinealbody(8w)

Embryology目前六页\总数六十八页\编于十八点Iftheurorectalseptumdoesnotcompletelydividethecloaca,therectumwillconnectanteriorwithurinaryorgenitalstructures,resultinginanimperforateanuswithfistulaInsummary,congenitalanorectalmalformationsarecausedbyabnormalitiesinformationofthecloacaduringthefourthandeighthweeksofgestationEmbryology目前七页\总数六十八页\编于十八点4thweek5-6thweek7-8thweekThedevelopmentoftherectumandanus目前八页\总数六十八页\编于十八点InternalanalsphincterexternalanalsphincterlevatormusclepuborectalispubococcygeusmuscleiliococcygeusmuscleAnalsphinctermuscle目前九页\总数六十八页\编于十八点levatormuscleinternalanalsphincterexternalanalsphincter目前十页\总数六十八页\编于十八点InternalanalsphincterisathickenedcontinuationoftheinnercircularlayerofrectalmuscleIsinnervatedbyvisceralnervesResponsibleforpreventingthepassageofsolidandliquidstoolandgasSympatheticfibers---contraction--nostimulationofrectum—closetheanalcanalParasynpatheticfibers---relaxation--stimulationofrectumInternalanalsphincter目前十一页\总数六十八页\编于十八点External

analsphincterDeepExternal

analsphincterSuperficialExternal

analsphincterSubcutaneousExternal

analsphincterInnervatedbytheinferiorrectalbranchofthepudendalnerve(analnerveandperinealnerve)originatingintheanteiordivisionsofthesecondtofourthsacralnerveroots目前十二页\总数六十八页\编于十八点puborectalispubococcygeusmuscleiliococcygeusmusclelevatormuscle目前十三页\总数六十八页\编于十八点elevatestherectumpulltherectumforwardThepuborectalisistheportionmostcloselyassociatedwiththerectumAresuppliedbythefourthsacralneverandtheinferiorrectalorperinealbranchesofthepudendalnervesPuborectalis----

thethirdsphincter目前十四页\总数六十八页\编于十八点Rectoanalangle

(about80°)Thevariousportionsofthelevatoranimusclearesurroundedtheanusandrectumandtendtopulltherectumforward,toelevatetherectum,formingtheanglebetweenthelongitudinalaxisoftherectumandtheanalcanal.ThisrectoanalanglehelpstomaintaincontinencebypreventingformedstoolfromenteringtheanalcanalStriatedmusclecomplexThismusclecomplexiscomposedofafusionofthepuborectalportionofthelevatoranimuscleandexternalsphinctermuscles,includingadeepexternalsphinctercomponent,whichcannotbeidentifiedclinicallyRectoanalAngleandStriatedMuscleComplex目前十五页\总数六十八页\编于十八点RectoanalAngleandStriatedMuscleComplex目前十六页\总数六十八页\编于十八点Thelandmarkofclassificationofanorectalmalformationispubococcygealline(puborectalis)Therelationshipoftheendoftherectumtothepuborectalismuscledividestheimperforateanusintohigh,intermediateandlowtypesClassification目前十七页\总数六十八页\编于十八点ClassificationIftherectalpouchabove(supralevator)thelevatormuscle(puborectalis),itistermedahightypeIftherectalpouchat(translevator)thelevatormuscle(puborectalis),itistermedaintermediatetypeIftherectalpouchbelow(infralevator)thelevatormuscle(puborectalis),itistermedalowtype目前十八页\总数六十八页\编于十八点目前十九页\总数六十八页\编于十八点ThepropertreatmentofimperforateanusdependsonthetypethatisencounteredDeterminationofthelevelofthelesioniscriticalforappropriatemanagementEachtypeofanorectalmalformationrequiresadifferentoperationandmedicalmanagementClassification目前二十页\总数六十八页\编于十八点WingspreadClassificationofAnorectalMalformation(1984)

High

AnorectalagenesisWithrectovaginalfistula

Withoutfistula

Rectalatresia

Intermediate

Rectovestibularfistula

Rectovaginalfistula

Analagenesiswithoutfistula

LowAnovestibularfistulaAnocutaneousfistula

Analstenosis

CloacaRaremalformations

LowAnocutaneousfistula

Analstenosis

RaremalformationsFemaleMale

High

AnorectalagenesisWithrectoprostaticurethralfistula

Withoutfistula

Rectalatresia

IntermediateRectobulbarurethralfistula

Analagenesiswithoutfistula

目前二十一页\总数六十八页\编于十八点PediatrSurgInt(1986)1:200-205WingspreadClassificationofAnorectalMalformation(1984)目前二十二页\总数六十八页\编于十八点StandardsforDiagnosisInternationalClassification(Krickenbeck2005)MajorclinicalgroupsRare/regionalvariantsPerineal(cutaneous)fistulaPouchcolonRectourethralfistulaRectalatresia/stenosisProstaticRectovaginalfistulaBulbarHfistulaRectovesicalfistulaOthersVestibularfistulaCloacaNofistulaAnalstenosisJPediatricSurgery,2005,40,1525目前二十三页\总数六十八页\编于十八点OtherClassificationsAnaldeformitiesRectaldeformitiesLowtypeHightypeNointermediatetype目前二十四页\总数六十八页\编于十八点Anoperineal/anocutaneousfistula目前二十五页\总数六十八页\编于十八点Anoperineal/anocutaneousfistula目前二十六页\总数六十八页\编于十八点Rectourethralfistula目前二十七页\总数六十八页\编于十八点Rectovesicalfistula目前二十八页\总数六十八页\编于十八点Anoperinealfistula目前二十九页\总数六十八页\编于十八点Rectovestibularfistula目前三十页\总数六十八页\编于十八点Rectovaginalfistula目前三十一页\总数六十八页\编于十八点Persistentcloaca目前三十二页\总数六十八页\编于十八点PathologicalchangesareverycomplicatedSphinctermuscleNeverSacrumAssociatedanomaliesThehigherthedefect,thesevererthepathologicalchange,thelessthelikelihoodwillbeofachievingbowelcontrolPathology目前三十三页\总数六十八页\编于十八点VACTERLAssociation

VACTERLV vertebralA AnorectalC CardiacT Tracheo-esophagealfistulaE EsophagealatresiaR RenalL Limb目前三十四页\总数六十八页\编于十八点AssociatedAnomaliesCardiovascularGastrointestinalSpinalandvertebralGenitourinaryGynecologic目前三十五页\总数六十八页\编于十八点SymptomsarevariableDifferenttype:differentSymptomsThelevelofdistalpouchWithorwithoutfistulaSizeandpositionofthefistulaAssociatedanomaliesClinicalPresentations目前三十六页\总数六十八页\编于十八点Nopassageoffirststoolwithin24to48hoursafterbirthLifelonghistoryofconstipationStoolpassedbywayoffistulaAbsenceofanalopeningMisplacedanalopeningVomitingandabdominaldistention

ClinicalPresentations目前三十七页\总数六十八页\编于十八点WithoutfistulaLowerintestinalobstructionNopassageofstoolorgasAbdominaldistentionandvomitingPhysicalexamination：NoanusFlatperineumwithbulgingoncryingNoexternalsphinctercontractiononscratchingtheperineumClinicalPresentations目前三十八页\总数六十八页\编于十八点WithfistulaMale：PassageofmeconiumintheurinePassmeconiumorflatusviapenisMeconiumpassedfromthefistulaintheperineumwithlowerobstructionFemale：AbnormalanalopeningPassstoolfromvestibulumorvaginaOnlyoneorificeintheperineum----cloacaClinicalPresentations目前三十九页\总数六十八页\编于十八点Anoperinealfistula:Meconiumpassedfromthefistulaintheperineum目前四十页\总数六十八页\编于十八点Anoperinealfistula:Meconiumpassedfromthefistulaintheperineum目前四十一页\总数六十八页\编于十八点RectourethralfistulaPassageofmeconiumintheurinePassmeconiumorflatusviapenis目前四十二页\总数六十八页\编于十八点Female:imperforateanuswithfistula目前四十三页\总数六十八页\编于十八点Imperforateanuswithoutfistula目前四十四页\总数六十八页\编于十八点ImperforateAnusDiagnosis

HistoryFailuretopassmeconiumwithinthefirst24hoursoflifeLifelonghistoryofconstipationThoroughexaminationofperineumMustperformathoroughperinealinspection目前四十五页\总数六十八页\编于十八点ThediagnosisiseasilymadebyhistoryandthoroughexaminationofperineumTheappearanceoftheperineumdoesnotnecessarilypredictwhetherthelesionislow,intermediateorhighDiagnosisstudies目前四十六页\总数六十八页\编于十八点Thepurposesofspecificdiagnosisstudiesare:Todeterminetheleveloftheblindrectalpouchwhetherlow,intermediateorhightypeToidentifyanyassociatedfistulouscommunicationsTodeterminethepresenceorabsenceofanyothercongenitalanomaliesToassessthestatusofthelevatoranimuscleandanalsphinctermuscleDiagnosisstudies目前四十七页\总数六十八页\编于十八点X-rayCT/MRIFistulogramDistalcolostogramLoopogramOthersDiagnosisstudies目前四十八页\总数六十八页\编于十八点Ifnoneoftheclinicalsignstodeterminethelocationoftheanorectalanomalyareevidentby24hours,performingaradiologictestcanhelpThissituationisonlynecessaryinabout10%ofpatientsthatwithoutfistulaLateralpelvicradiographyisperformedinbabieswhohavenoexternalevidenceoffistula,whopassnomeconiumafter24hours,andwhohavenomeconiumintheurineX-ray目前四十九页\总数六十八页\编于十八点X-rayInvertogram(Wangensteen\Rice1930)UpsidedownlateralfilmPronecross-tablelateralradiographs目前五十页\总数六十八页\编于十八点I-point,isthelowestpointoftheischialtuberosity,representsthedeepestpointofthelevatoranimusclesThepubococcygeallineisthelinethatconnectstheupperborderofthesymphysispubisandsacrococcygealjunction,itrepresentstheupperlimitsofthelevatormusculature(puborectalissling).ItisthelandmarkforclassificationofanorectalmalformationThepubococcygealline(PCline)andI-point目前五十一页\总数六十八页\编于十八点PClineIpointAbovethePCline----highBelowtheIpoint-----lowBelowthePClineAbovetheIpoint----intermediateTheleveloftherectalpouch目前五十二页\总数六十八页\编于十八点目前五十三页\总数六十八页\编于十八点BowelskindistanceThedistancebetweentheendoftherectumandtheopaquemarkermeasuresmorethan2cm,itmeansthattherectumlieshigh目前五十四页\总数六十八页\编于十八点X-rayfilmsshouldbetakenmorethan12hoursoflifetoallowenoughtimeforbowelgastotheendoftheblindrectumThechildshouldbeheldverticallyupsidefor3minutesbeforethefilmistakenThehipshouldbekeptrelativelystraightPlaceanopaquemarkerontheperinealskintodenotethecutaneousleveloftheanusX-ray目前五十五页\总数六十八页\编于十八点persistentcloacaFistulogram目前五十六页\总数六十八页\编于十八点DistalColostogramRectovaginalfistulaRectourethralfistula目前五十七页\总数六十八页\编于十八点CT/MRI目前五十八页\总数六十八页\编于十八点SurgicalPrincipalThepropertreatmentofimperforateanusdependsonthetypethatisencounteredEachtypeofanorectalmalformationrequiresadifferentoperationandmedicalmanagementThetreatmentsometimesdependsontheexperienceofthesurgeonandgeneralconditionofthepatientTreatment目前五十九页\总数六十八页\编于十八点SurgicalPrincipal

Whattime?Emergentoperation:withoutfistulaorthinfistulacausingintestinalobstructionImperforate"perforate”anusDelayedoperation:withwidefistula,anoplastyuntil3-6monthsafterbirth目前六十页\总数六十八页\编于十八点

Whichprocedure?Colostomyornot?Lowtype(analdeformities) perinealanoplastywithoutcolostomyIntermediateand

hightype

(rectaldeformities) Colostomy LaparoscopyassistedpullthroughVsPSARP ClosureofColostomySurgicalPrincipal

PSARP

posteriorsagittalano-rectoplasty目前六十一页\总数六十八页\编于十八点Colostomyornot?Intermediateand

high

type

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

p