先天性巨结肠与胆道剖析

先天性巨结肠与胆道剖析第1页/共44页定义Defination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Rectal

or

colonic

intestinalhaspersistent

spasmandfecal

depositioninthe

proximalcolonresultsinhypertrophyand

dilatationinproximalsegment.第2页/共44页

Hirschsprung’sDisease,HD

赫什朋氏病

Aganglionosis先天性无神经节细胞症英文名称Englishname第3页/共44页概述Summary

一种常见的消化道畸形Acommondigestive

tractmalformation有遗传倾向，约1.4-7.8%Geneticpredisposition,

about1.4-7.8%发病率为1:2000-5000Incidencerateof1:2000-5000男:女=4:1Male:female=4:1第4页/共44页病因Etiology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.Absenceof

ganglioncellsinmuscle

and

submucosaoflesionbowel.Itisadevelopment

pause.

Themoreearlythepauseoccurs,thelongerintestinalsegmentwithoutganglioncellsis.第5页/共44页胚胎期第5周消化道神经母细胞从头端向尾端移行Neuroblastoma

cellsofdigestivetractmigrate

fromthebeginningtotheendInthefifthweekoffetal.第6页/共44页Normal

defecationphysiology

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第7页/共44页Absenceof

ganglioncells

Smoothmuscle

oflesionintestinesustainescontraction

Anorectal

reflex

loop

isinterruptedStool

cannotbedischarged.

Proximal

normal

intestinehas

compensatorydilatation

and

hypertrophyandformshugeexpansiveintestinalsegment

Pathology

ofcongenitalmegacolon

第8页/共44页

病变肠段呈痉挛样改变(Spasmsegment）近端肠管扩张肥厚，形成巨大结肠(Enlargedsegment)二者之间过度肠段呈漏斗状称移行段(Transformedsegment)正常结肠Normalcolon

Pathological

anatomyofcongenitalmegacolon第9页/共44页

按病变长度Accordingtothelengthoflesionsegment常见型Ordinarytype

(common

type)

megacolon(85%):Lesionis

limitedin

rectumandsigmoidcolon.短段型Short

segmenttypemegacolon(10%):Lesionis

limitedinthe

distal3-4

cm

ofrectum.长段型Long

segmenttypemegacolon(10%):Lesionreachessplenicflexure,

andeven

theentirecolon.全结肠型Totalcolonic

type(1%):Lesionreathesentire

colonor

evevterminalileum.分型Pathologictypesofcongenitalmegacolon第10页/共44页临床表现Clinicalmanifestations

新生儿期Theneonatalperiod:

acutelow

incompleteintestinalobstruction

Delayoffetal

dischargeAbdominaldistention

andvomitingConstipationWasting

andmalnutrition第11页/共44页Clinicalmanifestations婴幼儿期：慢性低位肠梗阻

Infantand

childhood:

subacuteorchronic

low

incompleteintestinalobstruction

反复便秘Recurrent

constipation进行性腹胀Progressiveabdominaldistension发育迟缓，营养不良Growthretardation,malnutrition第12页/共44页并发症Complications多在2个月内发生Occurring

within2months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

第13页/共44页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Accessoryexamination

第14页/共44页第15页/共44页第16页/共44页钡灌肠X表现BariumenemaX-ray葛X，2y，长段型巨结肠，soave第17页/共44页第18页/共44页Anorectalmanometry:

reflection

ofnormalanorectal

reflexdisappears

正常直肠肛管反射第19页/共44页第20页/共44页肠壁粘膜腺体Intestinal

mucosalglands酶阳性神经Enzyme

positivenerve

正常人Normalchild巨结肠患儿Megacolonchild第21页/共44页诊断Diagnosis1、病史Medicalhistory2、钡灌肠Bariumenema3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织活检

Rectalmucosal

biopsy第22页/共44页1、胎粪性便秘Meconiumconstipation

2、新生儿肠闭锁Neonatal

intestinalatresia3、特发性巨结肠Idiopathic

megacolon4、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Differentialdiagnosis第23页/共44页先天性巨结肠Congenital

megacolon继发性巨结肠Secondarymegacolon第24页/共44页治疗Treatment治疗原则:手术治疗,切除病变肠段以及扩张肥厚的肠管Treatmentprinciple:Operation

treatment.Resectionofthelesion

boweland

dilatation

bowel.第25页/共44页术前准备（保守治疗）Preoperativepreparation

(conservativetreatment)1、洗肠Intestinallavage2、括肛、通便

Enlarge

anusandinducingdefeation3、缓泻药ApplicationofLaxativedrug第26页/共44页手术方式Operation

methods

Sewnon’soperationSoave’soperationDuhamel’soperationRehbein’soperationMartin’soperation第27页/共44页Swenson改良术：

结肠经直肠内拖出

（Pull-through）切除术第28页/共44页Soaveprocedure

手术的基本步骤第29页/共44页

Duhameloperation第30页/共44页

术后并发症Postoperativecomplications

吻合口感染、泄漏Anastomotic

infectionandleakage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Internalanal

sphincterinjury远期生活质量下降Declinedqualityoflifeinlong-term第31页/共44页要点Keypoints1、概念Conceptofmegacolon2、分型Pathologicaltypeofmegacolon3、临床特征Clinicalcharacteristicsand

diagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon

第32页/共44页肛门直肠畸形的分类

Classification

ofanorectalmalformation

第33页/共44页没有X影像学资料前不要轻易手术

Weshould

notdooperationeasilywithoutX-ray

第34页/共44页MR第35页/共44页针形电刀Needle

typeelectric

knife电刺激仪Electricalstimulation

instrument第36页/共44页先天性胆道闭锁

Congenitalbiliaryatresia

第37页/共44页Diagnosisanddifferentialdiagnosis

MRCP,laparoscopic

exploration

andintraoperativecholangiography

Cholestasis

syndrome第38页/共44页Operation

treatment

ofcongenitalbiliaryatresia

纤维三角板的切除Excisionoffibrous

triangle

plate

肝门部与肠吻合Anastomosisofjejunum

andHilar第39页/共44页胆道闭锁术后用药

Medication

after

biliaryatresia