先天性巨结肠与胆道剖析课件

2022/10/18先天性巨结肠(jiécháng)

Congenitalmegacolon第一页，共四十四页。定义(dìngyì)

Defination由于直肠或者结肠(jiécháng)远端的肠管持续痉挛，粪便淤积在近端结肠(jiécháng)，使该肠管肥厚、扩张Rectal

or

colonic

intestinalhaspersistent

spasmandfecal

depositioninthe

proximalcolonresultsinhypertrophyand

dilatationinproximalsegment.第二页，共四十四页。

Hirschsprung’s

Disease,HD

赫什朋氏病

Aganglionosis先天性无神经节细胞(xìbāo)症英文名称Englishname第三页，共四十四页。概述(ɡàishù)

Summary

一种常见的消化道畸形Acommondigestive

tractmalformation有遗传(yíchuán)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第四页，共四十四页。病因Etiology病变(bìngbiàn)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.第五页，共四十四页。胚胎(pēitāi)期第5周消化道神经母细胞从头端向尾端移行Neuroblastoma

cellsofdigestivetractmigrate

fromthebeginningtotheendInthefifthweekoffetal.第六页，共四十四页。Normal

defecationphysiology

直肠(zhícháng)壶腹潴便经大脑整合(zhěnɡhé)，决定排便与否便意直肠肛管抑制反射肛管感受(gǎnshòu)粪便性质骶髓低级中枢肠壁感受器第七页，共四十四页。Absenceof

ganglioncells

Smoothmuscle

oflesionintestinesustainescontraction

Anorectal

reflex

loop

isinterruptedStool

cannotbedischarged.

Proximal

normal

intestinehas

compensatorydilatation

and

hypertrophyandformshugeexpansiveintestinalsegment

Pathology

ofcongenitalmegacolon

第八页，共四十四页。

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

Pathological

anatomyofcongenitalmegacolon第九页，共四十四页。

按病变长度(chángdù)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第十页，共四十四页。临床表现Clinicalmanifestations

新生儿期Theneonatalperiod:

acutelow

incompleteintestinalobstruction

Delayoffetal

dischargeAbdominaldistention

andvomitingConstipationWasting

andmalnutrition第十一页，共四十四页。Clinicalmanifestations婴幼儿期：慢性(mànxìng)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第十二页，共四十四页。并发症Complications多在2个月内发生Occurring

within2months肠梗阻Intestinalobstruction小肠(xiǎocháng)结肠炎Enterocolitis肠穿孔，腹膜炎Bowelperforationandperitonitis继发败血症，肺炎Secondarysepsisandpneumonia

第十三页，共四十四页。1、肛门指诊

Rectaltouch2、钡灌肠Bariumenema3、直肠(zhícháng)肛管测压Anorectalmanometry4、直肠活检Biopsy5、肌电图Electromyogram

辅助(fǔzhù)检查Accessoryexamination

第十四页，共四十四页。第十五页，共四十四页。第十六页，共四十四页。钡灌肠(guàn〃cháng)X表现BariumenemaX-ray葛X，2y，长段型巨结肠(jiécháng)，soave第十七页，共四十四页。第十八页，共四十四页。Anorectalmanometry:

reflection

ofnormalanorectal

reflexdisappears

正常直肠(zhícháng)肛管反射第十九页，共四十四页。第二十页，共四十四页。肠壁粘膜(zhānmó)腺体Intestinal

mucosalglands酶阳性(yángxìng)神经Enzyme

positivenerve

正常人Normalchild巨结肠(jiécháng)患儿Megacolonchild第二十一页，共四十四页。诊断Diagnosis1、病史Medicalhistory2、钡灌肠Bariumenema3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织(zǔzhī)活检

Rectalmucosal

biopsy第二十二页，共四十四页。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(gāngmén)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(jiànbié)诊断Differentialdiagnosis第二十三页，共四十四页。先天性巨结肠(jiécháng)Congenital

megacolon继发性巨结肠(jiécháng)Secondarymegacolon第二十四页，共四十四页。治疗Treatment治疗原则(yuánzé):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.第二十五页，共四十四页。术前准备（保守治疗）Preoperativepreparation

(conservativetreatment)1、洗肠Intestinallavage2、括肛、通便(tōnɡbiàn)

Enlarge

anusandinducingdefeation3、缓泻药ApplicationofLaxativedrug第二十六页，共四十四页。手术(shǒushù)方式Operation

methods

Sewnon’soperationSoave’soperationDuhamel’soperationRehbein’soperationMartin’soperation第二十七页，共四十四页。Swenson改良术：

结肠(jiécháng)经直肠内拖出

（Pull-through）切除术第二十八页，共四十四页。Soaveprocedure

手术(shǒushù)的基本步骤第二十九页，共四十四页。

Duhameloperation第三十页，共四十四页。

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(fùfā)

Anastomoticstenosisandrecurrentconstipation肛门内括约肌损伤Internalanal

sphincterinjury远期生活质量下降Declinedqualityoflifeinlong-term第三十一页，共四十四页。要点(yàodiǎn)Keypoints1、概念Conceptofmegacolon2、分型Pathologicaltypeofmegacolon3、临床特征(tèzhēng)Clinicalcharacteristicsand

diagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon

第三十二页，共四十四页。肛门直肠(zhícháng)畸形的分类

Classification

ofanorectalmalformation

第三十三页，共四十四页。没有X影像学资料(zīliào)前不要轻易手术

Weshould

notdooperationeasilywithoutX-ray

第三十四页，共四十四页。MR第三十五页，共四十四页。针形电刀Needle

typeelectric

knife电刺激(cìjī)仪Electricalstimulation

instrument第三十六页，共四十四页。先天性胆道闭锁(bìsuǒ)

Congenitalbiliaryatresia

第三十七页，共四十四页。Diagnosisanddifferentialdiagnosis

MRCP,laparoscopic

exploration

andintraoperativecholangiography

Cholestasis

syndrome第三十八页，共四十四页。Operation

treatment

ofcongenitalbiliaryatresia

纤维(