先天性巨結(jié)腸與膽道剖析課件

1、先天性巨結(jié)腸與膽道剖析先天性巨結(jié)腸與膽道剖析先天性巨結(jié)腸與膽道剖析定義 Defination由于直腸或者結(jié)腸遠(yuǎn)端的腸管持續(xù)痙攣，糞便淤積在近端結(jié)腸，使該腸管肥厚、擴(kuò)張Rectalorcolonicintestinal has persistentspasm and fecaldeposition in the proximal colon results in hypertrophy anddilatation in proximal segment.先天性巨結(jié)腸與膽道剖析先天性巨結(jié)腸與膽道剖析先天性巨結(jié)腸與膽定義 Defination由于直腸或者結(jié)腸遠(yuǎn)端的腸管持續(xù)痙攣，糞便淤積在近端結(jié)腸，使

2、該腸管肥厚、擴(kuò)張Rectalorcolonicintestinal has persistentspasm and fecaldeposition in the proximal colon results in hypertrophy anddilatation in proximal segment.定義 Defination由于直腸或者結(jié)腸遠(yuǎn)端的腸管持續(xù)痙攣Hirschsprungs Disease,HD 赫什朋氏病Aganglionosis 先天性無神經(jīng)節(jié)細(xì)胞癥英文名稱 English nameHirschsprungs Disease,HD 赫什朋概述 Summary 一種常見的消化

3、道畸形A common digestivetract malformation 有遺傳傾向， 約1.4-7.8%Genetic predisposition,about 1.4-7.8% 發(fā)病率為1:2000-5000Incidence rate of 1:2000-5000 男:女 = 4:1Male:female=4:1概述 Summary 一種常見的消化道畸形病 因Etiology病變腸管肌間及粘膜下神經(jīng)節(jié)細(xì)胞缺如，是一種發(fā)育停頓，停頓越早，無神經(jīng)節(jié)細(xì)胞段越長.Absence ofganglion cells in muscleandsubmucosa of lesion bowel.

4、It is a developmentpause.The more early the pause occurs, the longer intestinal segment without ganglion cells is.病 因Etiology病變腸管肌間及粘膜下神經(jīng)節(jié)細(xì)胞缺如，胚胎期第5周消化道神經(jīng)母細(xì)胞從頭端向尾端移行Neuroblastomacells of digestive tract migratefrom the beginning to the end In the fifth week of fetal.胚胎期第5周消化道神經(jīng)母細(xì)胞從頭端向尾端移行Normaldefec

5、ation physiology 直腸壺腹潴便經(jīng)大腦整合，決定排便與否便意直腸肛管抑制反射肛管感受糞便性質(zhì)骶髓低級(jí)中樞腸壁感受器Normaldefecation physiology 直Absence ofganglion cells Smooth muscleof lesion intestine sustaines contraction Anorectalreflexloopis interruptedStoolcan not be discharged.Proximalnormalintestine hascompensatory dilatationandhypertrophy an

6、d forms huge expansive intestinal segment Pathologyof congenital megacolon Absence ofganglion cells Smoo 病變腸段呈痙攣樣改變(Spasm segment）近端腸管擴(kuò)張肥厚，形成巨大結(jié)腸(Enlarged segment)二者之間過度腸段呈漏斗狀稱移行段(Transformed segment)正常結(jié)腸Normal colon Pathologicalanatomy of congenital megacolon病變腸段呈痙攣樣改近端腸管擴(kuò)張肥厚，形成巨大結(jié)腸二者之間過度 按病變長度Acco

7、rding to the length of lesion segment常見型Ordinary type(commontype)megacolon (85%): Lesion islimited inrectum and sigmoid colon.短段型Shortsegment type megacolon(10%): Lesion islimited in thedistal 3-4cmof rectum.長段型Longsegment type megacolon(10%): Lesion reaches splenic flexure,and eventhe entire colon.

8、全結(jié)腸型Total colonictype(1%):Lesion reathes entirecolon orevev terminal ileum.分型Pathologic types of congenital megacolon 按病變長度According to the length 臨床表現(xiàn)Clinical manifestations 新生兒期The neonatal period:acute lowincomplete intestinal obstruction Delay of fetaldischargeAbdominal distentionand vomitingCon

9、stipation Wastingand malnutrition 臨床表現(xiàn)Clinical manifestations 新生Clinical manifestations嬰幼兒期：慢性低位腸梗阻 Infant andchildhood:subacute or chroniclowincomplete intestinal obstruction 反復(fù)便秘Recurrentconstipation 進(jìn)行性腹脹Progressive abdominal distension發(fā)育遲緩，營養(yǎng)不良Growth retardation, malnutritionClinical manifestati

10、ons嬰幼兒期：慢性并發(fā)癥 Complications多在2個(gè)月內(nèi)發(fā)生 Occurringwithin 2 months腸梗阻 Intestinal obstruction 小腸結(jié)腸炎 Enterocolitis 腸穿孔，腹膜炎 Bowel perforation and peritonitis 繼發(fā)敗血癥，肺炎 Secondary sepsis and pneumonia 并發(fā)癥 Complications多在2個(gè)月內(nèi)發(fā)生 Occu1、肛門指診 Rectal touch 2、鋇灌腸 Barium enema3、直腸肛管測壓 Anorectal manometry 4、直腸活檢 Biopsy5

11、、肌電圖 Electromyogram 輔助檢查 Accessory examination 1、肛門指診 Rectal touch 輔助檢查 Acce先天性巨結(jié)腸與膽道剖析課件先天性巨結(jié)腸與膽道剖析課件鋇灌腸X表現(xiàn) Barium enema X-ray葛X，2y，長段型巨結(jié)腸，soave 鋇灌腸X表現(xiàn) Barium enema X-ray葛X，2y先天性巨結(jié)腸與膽道剖析課件Anorectal manometry:reflectionof normal anorectalreflex disappears 正常直腸肛管反射Anorectal manometry:reflectio先天性巨結(jié)腸與

12、膽道剖析課件腸壁粘膜腺體Intestinalmucosal glands酶陽性神經(jīng)Enzymepositive nerve 正常人Normal child巨結(jié)腸患兒Megacolon child腸壁粘膜腺體酶陽性神經(jīng)正常人巨結(jié)腸患兒診 斷 Diagnosis1、病史 Medical history2、鋇灌腸 Barium enema 3 、直腸肛管測壓力 Anorectalmanometry3、直腸黏膜組織活檢 Rectal mucosalbiopsy 診 斷 Diagnosis1、病史 Medical his1、胎糞性便秘 Meconium constipation 2、新生兒腸閉鎖 Neo

13、natalintestinal atresia 3、特發(fā)性巨結(jié)腸 Idiopathicmegacolon 4、巨結(jié)腸類緣病Neuronal intestinal dysplasia5、肛門內(nèi)括約肌失弛癥 Internal anal sphincter achalasia6、繼發(fā)性巨結(jié)腸 Secondary megacolon7、內(nèi)分泌性 Endocrine disease8、乙狀結(jié)腸過長癥 Redundant sigmoid colon 鑒別診斷 Differential diagnosis1、胎糞性便秘 Meconium constipation 先天性巨結(jié)腸Congenitalmegaco

14、lon 繼發(fā)性巨結(jié)腸Secondary megacolon 先天性巨結(jié)腸繼發(fā)性巨結(jié)腸治 療 Treatment治療原則:手術(shù)治療,切除病變腸段以及擴(kuò)張肥厚的腸管Treatment principle: Operationtreatment. Resection of the lesionbowel anddilatationbowel.治 療 Treatment術(shù)前準(zhǔn)備（保守治療）Preoperative preparation(conservative treatment) 1、洗腸 Intestinal lavage 2、括肛、通便Enlargeanus and inducing defe

15、ation 3、 緩瀉藥 Application of Laxative drug術(shù)前準(zhǔn)備（保守治療）手術(shù)方式 Operationmethods Sewnons operationSoaves operationDuhamels operationRehbeins operationMartins operation手術(shù)方式 Operationmethods SewnonSwenson 改良術(shù)：結(jié)腸經(jīng)直腸內(nèi)拖出（Pull-through）切除術(shù)Swenson 改良術(shù)：結(jié)腸經(jīng)直腸內(nèi)拖出（Pull-thSoave procedure手術(shù)的基本步驟Soave procedure手術(shù)的基本步驟 Duh

16、amel operation Duhamel oper術(shù)后并發(fā)癥 Postoperative complications 吻合口感染、泄漏 Anastomoticinfection and leakage尿潴留 Retention of urine 小腸結(jié)腸炎 Enterocolitis 吻合口狹窄，便秘復(fù)發(fā) Anastomotic stenosis and recurrent constipation 肛門內(nèi)括約肌損傷 Internal analsphincter injury 遠(yuǎn)期生活質(zhì)量下降 Declined quality of life in long-term 術(shù)后并發(fā)癥 Post

17、operative complicat要點(diǎn)Key points1、概念Concept of megacolon 2、分型Pathological type of megacolon3、臨床特征Clinical characteristics anddiagnosis of megacolon4、治療原則The surgical principles of megacolon 要點(diǎn)Key points1、概念Concept of meg肛門直腸畸形的分類Classificationof anorectal malformation 肛門直腸畸形的分類Classificationof an沒有X影

18、像學(xué)資料前不要輕易手術(shù)We shouldnot do operation easily without X-ray 沒有X影像學(xué)資料前不要輕易手術(shù)We shouldnot MRMR針形電刀Needletype electricknife 電刺激儀Electrical stimulationinstrument 針形電刀先天性膽道閉鎖Congenital biliary atresia 先天性膽道閉鎖Congenital biliary atrDiagnosis and differential diagnosis MRCP,laparoscopicexplorationand intraoperative cholangiography Cholestasissyndrome Diagnosis and differential diaOperationtreatmentof congenital biliary atresia 纖維三角板的切除 Excision of fibroustriangleplate 肝門部與腸吻合 Anastomosis of jejunumand Hilar Operationtreatmentof congeni膽道閉鎖術(shù)后用藥 Medication aft