2022年医学专题—先天性巨结肠与胆道剖析(1).ppt

2023/6/20,先天性巨结肠(jichng)Congenital megacolon,第一页，共四十四页。,定义(dngy)Defination,由于直肠或者结肠(jichng)远端的肠管持续痉挛，粪便淤积在近端结肠(jichng)，使该肠管肥厚、扩张Rectalorcolonicintestinal has persistentspasm and fecaldeposition in the proximal colon results in hypertrophy anddilatation in proximal segment.,第二页，共四十四页。,Hirschsprungs Disease,HD 赫什朋氏病Aganglionosis 先天性无神经节细胞(xbo)症,英文名称 English name,第三页，共四十四页。,概述(i sh)Summary,一种常见的消化道畸形A common digestivetract malformation 有遗传(ychun)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,第四页，共四十四页。,病 因Etiology,病变(bngbin)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.,第五页，共四十四页。,胚胎(piti)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.,第六页，共四十四页。,Normaldefecation physiology,直肠(zhchng)壶腹潴便,经大脑整合(zhn h)，决定排便与否,便意,直肠肛管抑制反射肛管感受(gnshu)粪便性质,骶髓低级中枢,肠壁感受器,第七页，共四十四页。,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,第八页，共四十四页。,病变(bngbin)肠段呈痉挛样改变(Spasm segment）,近端肠管扩张肥厚(fi hu)，形成巨大结肠(Enlarged segment),二者之间过度(gud)肠段呈漏斗状称移行段(Transformed segment),正常结肠Normal colon,Pathologicalanatomy of congenital megacolon,第九页，共四十四页。,按病变长度(chngd)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,第十页，共四十四页。,临床表现Clinical manifestations,新生儿期The neonatal period:acute lowincomplete intestinal obstruction,Delay of fetaldischargeAbdominal distentionand vomitingConstipation Wastingand malnutrition,第十一页，共四十四页。,Clinical manifestations,婴幼儿期：慢性(mn xng)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,第十二页，共四十四页。,并发症 Complications,多在2个月内发生 Occurringwithin 2 months肠梗阻 Intestinal obstruction 小肠(xiochng)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,第十三页，共四十四页。,1、肛门指诊 Rectal touch 2、钡灌肠 Barium enema3、直肠(zhchng)肛管测压 Anorectal manometry 4、直肠活检 Biopsy5、肌电图 Electromyogram,辅助(fzh)检查 Accessory examination,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(gunchng)X表现 Barium enema X-ray,葛X，2y，长段型巨结肠(jichng)，soave,第十七页，共四十四页。,第十八页，共四十四页。,Anorectal manometry:reflectionof normal anorectalreflex disappears,正常直肠(zhchng)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(zhn m)腺体Intestinalmucosal glands,酶阳性(yngxng)神经Enzymepositive nerve,正常人Normal child,巨结肠(jichng)患儿Megacolon child,第二十一页，共四十四页。,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(zzh)活检 Rectal mucosalbiopsy,第二十二页，共四十四页。,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(gngmn)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(jinbi)诊断 Differential diagnosis,第二十三页，共四十四页。,先天性巨结肠(jichng)Congenitalmegacolon,继发性巨结肠(jichng)Secondary megacolon,第二十四页，共四十四页。,治 疗 Treatment,治疗原则(yunz):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.,第二十五页，共四十四页。,术前准备（保守治疗）Preoperative preparation(conservative treatment)1、洗肠 Intestinal lavage 2、括肛、通便(tn bin)Enlargeanus and inducing defeation 3、缓泻药 Application of Laxative drug,第二十六页，共四十四页。,手术(shush)方式 Operationmethods,Sewnons operationSoaves operationDuhamels operationRehbeins operationMartins operation,第二十七页，共四十四页。,Swenson 改良术：结肠(jichng)经直肠内拖出（Pull-through）切除术,第二十八页，共四十四页。,Soave procedure手术(shush)的基本步骤,第二十九页，共四十四页。,Duhamel operation,第三十页，共四十四页。,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(f f)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,第三十一页，共四十四页。,要点(yodin)Key points,1、概念Concept of megacolon 2、分型Pathological type of megacolon3、临床特征(tzhng)Clinical characteristics anddiagnosis of megacolon4、治疗原则The surgical principles of megacolon,第三十二页，共四十四页。,肛门直肠(zhchng)畸形的分类Classificationof anorectal malformation,第三十三页，共四十四页。,没有X影像学资料(zlio)前不要轻易手术We shouldnot do operation easily without X-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Needletype electricknife 电刺激(cj)仪Electrical stimulationinstrument,第三十六页，共四十四页。,先天性胆道闭锁(b su