先天性巨结肠与胆道剖析.ppt
2023/5/24,先天性巨结肠Congenital megacolon,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.,Hirschsprungs Disease,HD 赫什朋氏病Aganglionosis 先天性无神经节细胞症,英文名称 English name,概述 Summary,一种常见的消化道畸形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,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.It is a developmentpause.The more early the pause occurs,the longer intestinal segment without ganglion cells is.,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.,Normaldefecation 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,Absence ofganglion cells,Smooth muscleof lesion intestine sustaines contraction,Anorectalreflexloopis interrupted,Stoolcan not be discharged.Proximalnormalintestine hascompensatory dilatationandhypertrophy and forms huge expansive intestinal segment,Pathologyof congenital megacolon,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,按病变长度According 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.全结肠型Total colonictype(1%):Lesion reathes entirecolon orevev terminal ileum.,分型Pathologic types of congenital megacolon,临床表现Clinical manifestations,新生儿期The neonatal period:acute lowincomplete intestinal obstruction,Delay of fetaldischargeAbdominal distentionand vomitingConstipation Wastingand malnutrition,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,malnutrition,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,1、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、肌电图 Electromyogram,辅助检查 Accessory examination,钡灌肠X表现 Barium enema X-ray,葛X，2y，长段型巨结肠，soave,Anorectal manometry:reflectionof normal anorectalreflex disappears,正常直肠肛管反射,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,诊 断 Diagnosis,1、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,1、胎粪性便秘 Meconium constipation 2、新生儿肠闭锁 Neonatalintestinal 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 diagnosis,先天性巨结肠Congenitalmegacolon,继发性巨结肠Secondary megacolon,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.,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 defeation 3、缓泻药 Application of Laxative drug,手术方式 Operationmethods,Sewnons operationSoaves operationDuhamels operationRehbeins operationMartins operation,Swenson 改良术：结肠经直肠内拖出（Pull-through）切除术,Soave procedure手术的基本步骤,Duhamel operation,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,要点Key points,1、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,肛门直肠畸形的分类Classificationof anorectal malformation,没有X影像学资料前不要轻易手术We shouldnot do operation easily without X-ray,MR,针形电刀Needletype electricknife 电刺激仪Electrical stimulationinstrument,先天性胆道闭锁Congenital biliary atresia,Diagnosis and differential diagnosis,MRCP,laparoscopicexplorationand intraoperative cholangiography,Cholestasissyndrome,Operationtreatmentof 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,胆道闭锁术后用药 Medication afterbiliary atresia operation,激素应用Hormone drugs 抗生素应用Antibiotics 利胆药应用Cholagogue,先天性胆总管囊肿Congenitalcholedochal cyst,MRCP,治疗:囊肿切除，Roux-en-Y吻合，抗反流瓣Treatment:cyst excision,Roux-en-Y anastomosis,anti refluxvalve,
