先天性巨結腸與膽道剖析課件

2022/10/18先天性巨結腸(jiécháng)

Congenitalmegacolon第一頁，共四十四頁。定義(dìngyì)

Defination由于直腸或者結腸(jiécháng)遠端的腸管持續(xù)痙攣，糞便淤積在近端結腸(jiécháng)，使該腸管肥厚、擴張Rectal

or

colonic

intestinalhaspersistent

spasmandfecal

depositioninthe

proximalcolonresultsinhypertrophyand

dilatationinproximalsegment.第二頁，共四十四頁。

Hirschsprung’s

Disease,HD

赫什朋氏病

Aganglionosis先天性無神經節(jié)細胞(xìbāo)癥英文名稱Englishname第三頁，共四十四頁。概述(ɡàishù)

Summary

一種常見的消化道畸形Acommondigestive

tractmalformation有遺傳(yíchuán)傾向，約1.4-7.8%Geneticpredisposition,

about1.4-7.8%發(fā)病率為1:2000-5000Incidencerateof1:2000-5000男:女=4:1Male:female=4:1第四頁，共四十四頁。病因Etiology病變(bìngbiàn)腸管肌間及粘膜下神經節(jié)細胞缺如，是一種發(fā)育停頓，停頓越早，無神經節(jié)細胞段越長.Absenceof

ganglioncellsinmuscle

and

submucosaoflesionbowel.Itisadevelopment

pause.

Themoreearlythepauseoccurs,thelongerintestinalsegmentwithoutganglioncellsis.第五頁，共四十四頁。胚胎(pēitāi)期第5周消化道神經母細胞從頭端向尾端移行Neuroblastoma

cellsofdigestivetractmigrate

fromthebeginningtotheendInthefifthweekoffetal.第六頁，共四十四頁。Normal

defecationphysiology

直腸(zhícháng)壺腹潴便經大腦整合(zhěnɡhé)，決定排便與否便意直腸肛管抑制反射肛管感受(gǎnshòu)糞便性質骶髓低級中樞腸壁感受器第七頁，共四十四頁。Absenceof

ganglioncells

Smoothmuscle

oflesionintestinesustainescontraction

Anorectal

reflex

loop

isinterruptedStool

cannotbedischarged.

Proximal

normal

intestinehas

compensatorydilatation

and

hypertrophyandformshugeexpansiveintestinalsegment

Pathology

ofcongenitalmegacolon

第八頁，共四十四頁。

病變(bìngbiàn)腸段呈痙攣樣改變(Spasmsegment）近端腸管擴張肥厚(féihòu)，形成巨大結腸(Enlargedsegment)二者之間過度(guòdù)腸段呈漏斗狀稱移行段(Transformedsegment)正常結腸Normalcolon

Pathological

anatomyofcongenitalmegacolon第九頁，共四十四頁。

按病變長度(chángdù)Accordingtothelengthoflesionsegment常見型Ordinarytype

(common

type)

megacolon(85%):Lesionis

limitedin

rectumandsigmoidcolon.短段型Short

segmenttypemegacolon(10%):Lesionis

limitedinthe

distal3-4

cm

ofrectum.長段型Long

segmenttypemegacolon(10%):Lesionreachessplenicflexure,

andeven

theentirecolon.全結腸型Totalcolonic

type(1%):Lesionreathesentire

colonor

evevterminalileum.分型Pathologictypesofcongenitalmegacolon第十頁，共四十四頁。臨床表現(xiàn)Clinicalmanifestations

新生兒期Theneonatalperiod:

acutelow

incompleteintestinalobstruction

Delayoffetal

dischargeAbdominaldistention

andvomitingConstipationWasting

andmalnutrition第十一頁，共四十四頁。Clinicalmanifestations嬰幼兒期：慢性(mànxìng)低位腸梗阻

Infantand

childhood:

subacuteorchronic

low

incompleteintestinalobstruction

反復便秘Recurrent

constipation進行性腹脹Progressiveabdominaldistension發(fā)育遲緩，營養(yǎng)不良Growthretardation,malnutrition第十二頁，共四十四頁。并發(fā)癥Complications多在2個月內發(fā)生Occurring

within2months腸梗阻Intestinalobstruction小腸(xiǎocháng)結腸炎Enterocolitis腸穿孔，腹膜炎Bowelperforationandperitonitis繼發(fā)敗血癥，肺炎Secondarysepsisandpneumonia

第十三頁，共四十四頁。1、肛門指診

Rectaltouch2、鋇灌腸Bariumenema3、直腸(zhícháng)肛管測壓Anorectalmanometry4、直腸活檢Biopsy5、肌電圖Electromyogram

輔助(fǔzhù)檢查Accessoryexamination

第十四頁，共四十四頁。第十五頁，共四十四頁。第十六頁，共四十四頁。鋇灌腸(guàn〃cháng)X表現(xiàn)BariumenemaX-ray葛X，2y，長段型巨結腸(jiécháng)，soave第十七頁，共四十四頁。第十八頁，共四十四頁。Anorectalmanometry:

reflection

ofnormalanorectal

reflexdisappears

正常直腸(zhícháng)肛管反射第十九頁，共四十四頁。第二十頁，共四十四頁。腸壁粘膜(zhānmó)腺體Intestinal

mucosalglands酶陽性(yángxìng)神經Enzyme

positivenerve

正常人Normalchild巨結腸(jiécháng)患兒Megacolonchild第二十一頁，共四十四頁。診斷Diagnosis1、病史Medicalhistory2、鋇灌腸Bariumenema3、直腸肛管測壓力Anorectalmanometry3、直腸黏膜組織(zǔzhī)活檢

Rectalmucosal

biopsy第二十二頁，共四十四頁。1、胎糞性便秘Meconiumconstipation

2、新生兒腸閉鎖Neonatal

intestinalatresia3、特發(fā)性巨結腸Idiopathic

megacolon4、巨結腸類緣病Neuronalintestinaldysplasia5、肛門(gāngmén)內括約肌失弛癥Internalanalsphincterachalasia6、繼發(fā)性巨結腸Secondarymegacolon7、內分泌性Endocrinedisease8、乙狀結腸過長癥Redundantsigmoidcolon

鑒別(jiànbié)診斷Differentialdiagnosis第二十三頁，共四十四頁。先天性巨結腸(jiécháng)Congenital

megacolon繼發(fā)性巨結腸(jiécháng)Secondarymegacolon第二十四頁，共四十四頁。治療Treatment治療原則(yuánzé):手術治療,切除病變腸段以及擴張肥厚的腸管Treatmentprinciple:Operation

treatment.Resectionofthelesion

boweland

dilatation

bowel.第二十五頁，共四十四頁。術前準備（保守治療）Preoperativepreparation

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

Enlarge

anusandinducingdefeation3、緩瀉藥ApplicationofLaxativedrug第二十六頁，共四十四頁。手術(shǒushù)方式Operation

methods

Sewnon’soperationSoave’soperationDuhamel’soperationRehbein’soperationMartin’soperation第二十七頁，共四十四頁。Swenson改良術：

結腸(jiécháng)經直腸內拖出

（Pull-through）切除術第二十八頁，共四十四頁。Soaveprocedure

手術(shǒushù)的基本步驟第二十九頁，共四十四頁。

Duhameloperation第三十頁，共四十四頁。

術后并發(fā)癥Postoperativecomplications

吻合口感染、泄漏Anastomotic

infectionandleakage尿潴留Retentionofurine小腸結腸炎Enterocolitis吻合口狹窄，便秘復發(fā)(fùfā)

Anastomoticstenosisandrecurrentconstipation肛門內括約肌損傷Internalanal

sphincterinjury遠期生活質量下降Declinedqualityoflifeinlong-term第三十一頁，共四十四頁。要點(yàodiǎn)Keypoints1、概念Conceptofmegacolon2、分型Pathologicaltypeofmegacolon3、臨床特征(tèzhēng)Clinicalcharacteristicsand

diagnosisofmegacolon4、治療原則Thesurgicalprinciplesofmegacolon

第三十二頁，共四十四頁。肛門直腸(zhícháng)畸形的分類

Classification

ofanorectalmalformation

第三十三頁，共四十四頁。沒有X影像學資料(zīliào)前不要輕易手術

Weshould

notdooperationeasilywithoutX-ray

第三十四頁，共四十四頁。MR第三十五頁，共四十四頁。針形電刀Needle

typeelectric

knife電刺激(cìjī)儀Electricalstimulation

instrument第三十六頁，共四十四頁。先天性膽道閉鎖(bìsuǒ)

Congenitalbiliaryatresia

第三十七頁，共四十四頁。Diagnosisanddifferentialdiagnosis

MRCP,laparoscopic

exploration

andintraoperativecholangiography

Cholestasis

syndrome第三十八頁，共四十四頁。Operation

treatment

ofcongenitalbiliaryatresia

纖維(