1、先天性巨结肠与胆道剖析先天性巨结肠与胆道剖析由于直肠或者结肠远端的肠管持续痉挛，粪便淤积在近端结肠，使该肠管肥厚、扩张Rectalorcolonicintestinalhaspersistentspasmandfecaldepositionintheproximalcolonresultsinhypertrophyanddilatationinproximalsegment.英文名称 English name一种常见的消化道畸形Acommondigestivetractmalformation有遗传倾向，约1.4-7.8%Geneticpredisposition,about1.4-7.8%发
2、病率为1:2000-5000Incidencerateof1:2000-5000男:女=4:1Male:female=4:1病变肠管肌间及粘膜下神经节细胞缺如，是一种发育停顿，停顿越早，无神经节细胞段越长.Absenceofganglioncellsinmuscleandsubmucosa of lesion bowel.It isadevelopmentpause.Themoreearlythepauseoccurs,thelongerintestinal segment without ganglion cells is.胚胎期第5周消化道神经母细胞从头端向尾端移行Neuroblastom
3、acellsofdigestivetractmigratefromthebeginningtotheendInthefifthweekoffetal.直肠壶腹潴便经大脑整合，决定排便与否便意直肠肛管抑制反射肛管感受粪便性质骶髓低级中枢肠壁感受器Smoothmuscleoflesionintestinesustainescontraction AnorectalreflexloopisinterruptedStoolcannotbedischarged.Proximalnormalintestinehascompensatorydilatationandhypertrophyandformshu
4、geexpansiveintestinalsegment Pathologyofcongenitalmegacolon 病变肠段呈痉挛样改变(Spasm segment）近端肠管扩张肥厚，形成巨大结肠(Enlarged segment)二者之间过度肠段呈漏斗状称移行段(Transformed segment)正常结肠Normalcolon Pathologicalanatomyofcongenitalmegacolon 按病变长度Accordingtothelengthoflesionsegment常见型Ordinarytype(commontype)megacolon(85%):Lesion
5、islimitedinrectumandsigmoidcolon.短段型Shortsegmenttypemegacolon(10%):Lesionislimitedinthedistal3-4cmofrectum.长段型Longsegmenttypemegacolon(10%):Lesionreachessplenicflexure,andeventheentirecolon.全结肠型Totalcolonictype(1%):Lesionreathesentirecolonorevevterminalileum.分型Pathologictypesofcongenitalmegacolon新生儿
6、期新生儿期Theneonatalperiod:acutelowincompleteintestinalobstructionDelayoffetaldischargeAbdominaldistentionandvomitingConstipationWastingandmalnutrition婴幼儿期：慢性低位肠梗阻Infantandchildhood:subacuteorchroniclowincompleteintestinalobstruction 1.反复便秘Recurrentconstipation2.进行性腹胀Progressiveabdominaldistension3.发育迟缓
7、，营养不良Growthretardation,malnutrition多在2个月内发生Occurringwithin2months肠梗阻Intestinalobstruction小肠结肠炎Enterocolitis肠穿孔，腹膜炎Bowelperforationandperitonitis继发败血症，肺炎Secondarysepsisandpneumonia 1、肛门指诊Rectaltouch2、钡灌肠Bariumenema3、直肠肛管测压Anorectalmanometry4、直肠活检Biopsy5、肌电图Electromyogram 辅助检查辅助检查 Accessoryexamination
8、 葛X，2y，长段型巨结肠，soave正常直肠肛管反射正常直肠肛管反射肠壁粘膜腺体肠壁粘膜腺体Intestinal mucosal glands酶阳性神经酶阳性神经Enzyme positive nerve 正常人正常人Normal child巨结肠患儿巨结肠患儿Megacolon child1、病史 Medical history2、钡灌肠 Barium enema 3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织活检 Rectal mucosalbiopsy 1、胎粪性便秘 Meconiumconstipation2、新生儿肠闭锁Neonatalintestinala
9、tresia3、特发性巨结肠Idiopathicmegacolon4、巨结肠类缘病Neuronalintestinaldysplasia5、肛门内括约肌失弛症Internalanalsphincterachalasia6、继发性巨结肠Secondarymegacolon7、内分泌性Endocrinedisease8、乙状结肠过长症Redundantsigmoidcolon 鉴别诊断鉴别诊断 Differential diagnosis先天性巨结肠Congenital megacolon 继发性巨结肠Secondary megacolon 治疗原则:手术治疗,切除病变肠段以及扩张肥厚的肠管Tre
10、atmentprinciple:Operationtreatment.Resectionofthelesionbowelanddilatationbowel.术前准备（保守治疗）Preoperativepreparation(conservativetreatment)1、洗肠Intestinallavage2、括肛、通便Enlargeanusandinducingdefeation3、缓泻药ApplicationofLaxativedrugSewnonsoperationSoaves operationDuhamels operationRehbeins operationMartins o
11、peration Duhameloperation术后并发症Postoperativecomplications 吻合口感染、泄漏Anastomoticinfectionandleakage尿潴留Retentionofurine小肠结肠炎Enterocolitis吻合口狭窄，便秘复发Anastomoticstenosisandrecurrentconstipation肛门内括约肌损伤Internalanalsphincterinjury远期生活质量下降Declinedqualityoflifeinlong-term1、概念Conceptofmegacolon2、分型Pathologicalty
12、peofmegacolon3、临床特征Clinicalcharacteristicsanddiagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon 针形电刀Needletypeelectricknife电刺激仪ElectricalstimulationinstrumentMRCP,laparoscopicexplorationandintraoperativecholangiography Cholestasissyndrome纤维三角板的切除 Excisionoffibroustriangleplate 肝门部与肠吻合 AnastomosisofjejunumandHilar激素应用激素应用Hormonedrugs抗生素应用抗生素应用Antibiotics利胆药应用利胆药应用Cholagogue
