先天性巨结肠与胆道剖析课件

1、先天性巨结肠与胆道剖析先天性巨结肠与胆道剖析先天性巨结肠与胆道剖析定义 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Rectalorcolonicintestinal has persistentspasm and fecaldeposition in the proximal colon results in hypertrophy anddilatation in proximal segment.先天性巨结肠与胆道剖析先天性巨结肠与胆道剖析先天性巨结肠与胆定义 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

2、该肠管肥厚、扩张Rectalorcolonicintestinal has persistentspasm and fecaldeposition in the proximal colon results in hypertrophy anddilatation in proximal segment.定义 Defination由于直肠或者结肠远端的肠管持续痉挛Hirschsprungs Disease,HD 赫什朋氏病Aganglionosis 先天性无神经节细胞症英文名称 English nameHirschsprungs Disease,HD 赫什朋概述 Summary 一种常见的消化

3、道畸形A common digestivetract malformation 有遗传倾向， 约1.4-7.8%Genetic predisposition,about 1.4-7.8% 发病率为1:2000-5000Incidence rate of 1:2000-5000 男:女 = 4:1Male:female=4:1概述 Summary 一种常见的消化道畸形病 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.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病变肠管肌间及粘膜下神经节细胞缺如，胚胎期第5周消化道神经母细胞从头端向尾端移行Neuroblastomacells of digestive tract migratefrom the beginning to the end In the fifth week of fetal.胚胎期第5周消化道神经母细胞从头端向尾端移行Normaldefec

5、ation physiology 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Normal colon Pathologicalanatomy of congenital megacolon病变肠段呈痉挛样改近端肠管扩张肥厚，形成巨大结肠二者之间过度 按病变长度Acco

7、rding to the length of lesion segment常见型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.长段型Longsegment type megacolon(10%): Lesion reaches splenic flexure,and eventhe entire colon.

8、全结肠型Total colonictype(1%):Lesion reathes entirecolon orevev terminal ileum.分型Pathologic types of congenital megacolon 按病变长度According to the length 临床表现Clinical manifestations 新生儿期The neonatal period:acute lowincomplete intestinal obstruction Delay of fetaldischargeAbdominal distentionand vomitingCon

9、stipation Wastingand malnutrition 临床表现Clinical manifestations 新生Clinical manifestations婴幼儿期：慢性低位肠梗阻 Infant andchildhood:subacute or chroniclowincomplete intestinal obstruction 反复便秘Recurrentconstipation 进行性腹胀Progressive abdominal distension发育迟缓，营养不良Growth retardation, malnutritionClinical manifestati

10、ons婴幼儿期：慢性并发症 Complications多在2个月内发生 Occurringwithin 2 months肠梗阻 Intestinal obstruction 小肠结肠炎 Enterocolitis 肠穿孔，腹膜炎 Bowel perforation and peritonitis 继发败血症，肺炎 Secondary sepsis and pneumonia 并发症 Complications多在2个月内发生 Occu1、肛门指诊 Rectal touch 2、钡灌肠 Barium enema3、直肠肛管测压 Anorectal manometry 4、直肠活检 Biopsy5

11、、肌电图 Electromyogram 辅助检查 Accessory examination 1、肛门指诊 Rectal touch 辅助检查 Acce先天性巨结肠与胆道剖析课件先天性巨结肠与胆道剖析课件钡灌肠X表现 Barium enema X-ray葛X，2y，长段型巨结肠，soave 钡灌肠X表现 Barium enema X-ray葛X，2y先天性巨结肠与胆道剖析课件Anorectal manometry:reflectionof normal anorectalreflex disappears 正常直肠肛管反射Anorectal manometry:reflectio先天性巨结肠与

12、胆道剖析课件肠壁粘膜腺体Intestinalmucosal glands酶阳性神经Enzymepositive nerve 正常人Normal child巨结肠患儿Megacolon child肠壁粘膜腺体酶阳性神经正常人巨结肠患儿诊 断 Diagnosis1、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 诊 断 Diagnosis1、病史 Medical his1、胎粪性便秘 Meconium constipation 2、新生儿肠闭锁 Neo

13、natalintestinal atresia 3、特发性巨结肠 Idiopathicmegacolon 4、巨结肠类缘病Neuronal intestinal dysplasia5、肛门内括约肌失弛症 Internal anal sphincter achalasia6、继发性巨结肠 Secondary megacolon7、内分泌性 Endocrine disease8、乙状结肠过长症 Redundant sigmoid colon 鉴别诊断 Differential diagnosis1、胎粪性便秘 Meconium constipation 先天性巨结肠Congenitalmegaco

14、lon 继发性巨结肠Secondary megacolon 先天性巨结肠继发性巨结肠治 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Treatment principle: Operationtreatment. Resection of the lesionbowel anddilatationbowel.治 疗 Treatment术前准备（保守治疗）Preoperative preparation(conservative treatment) 1、洗肠 Intestinal lavage 2、括肛、通便Enlargeanus and inducing defe

15、ation 3、 缓泻药 Application of Laxative drug术前准备（保守治疗）手术方式 Operationmethods Sewnons operationSoaves operationDuhamels operationRehbeins operationMartins operation手术方式 Operationmethods SewnonSwenson 改良术：结肠经直肠内拖出（Pull-through）切除术Swenson 改良术：结肠经直肠内拖出（Pull-thSoave procedure手术的基本步骤Soave procedure手术的基本步骤 Duh

16、amel operation Duhamel oper术后并发症 Postoperative complications 吻合口感染、泄漏 Anastomoticinfection and leakage尿潴留 Retention of urine 小肠结肠炎 Enterocolitis 吻合口狭窄，便秘复发 Anastomotic stenosis and recurrent constipation 肛门内括约肌损伤 Internal analsphincter injury 远期生活质量下降 Declined quality of life in long-term 术后并发症 Post

17、operative complicat要点Key points1、概念Concept of megacolon 2、分型Pathological type of megacolon3、临床特征Clinical characteristics anddiagnosis of megacolon4、治疗原则The surgical principles of megacolon 要点Key points1、概念Concept of meg肛门直肠畸形的分类Classificationof anorectal malformation 肛门直肠畸形的分类Classificationof an没有X影

18、像学资料前不要轻易手术We shouldnot do operation easily without X-ray 没有X影像学资料前不要轻易手术We shouldnot MRMR针形电刀Needletype electricknife 电刺激仪Electrical stimulationinstrument 针形电刀先天性胆道闭锁Congenital biliary atresia 先天性胆道闭锁Congenital biliary atrDiagnosis and differential diagnosis MRCP,laparoscopicexplorationand intraoperative cholangiography Cholestasissyndrome Diagnosis and differential diaOperationtreatmentof congenital biliary atresia 纤维三角板的切除 Excision of fibroustriangleplate 肝门部与肠吻合 Anastomosis of jejunumand Hilar Operationtreatmentof congeni胆道闭锁术后用药 Medication aft