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1、2019/8/15,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 Rectal or colonic intestinal has persistent spasm and fecal deposition in the proximal colon results in hypertrophy and dilatation in proximal segment.,Hirschsprungs Disease,HD 赫什朋氏病 Aganglionosis 先天性无神经节细胞症,英文
2、名称 English name,概述 Summary,一种常见的消化道畸形 A common digestive tract malformation 有遗传倾向， 约1.4-7.8% Genetic predisposition, about 1.4-7.8% 发病率为1:2000-5000 Incidence rate of 1:2000-5000 男:女 = 4:1 Male: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 of ganglion cells in muscle and su
3、bmucosa of lesion bowel. It is a development pause. 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 Neuroblastoma cells of digestive tract migrate from the beginning to the end In the fifth week of fetal.,Normal defecation physiology,直
4、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 of ganglion cells,Smooth muscle of lesion intestine sustaines contraction,Anorectal reflex loop is interrupted,Stool can not be discharged. Proximal normal intestine has compensatory dilatation and hypertrophy and forms huge expansive int
5、estinal segment,Pathology of 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,Pathological anatomy of congenital megacolon,按病变长度According to the length of lesion segment 常见型Ordinary type (common type) megacol
6、on (85%): Lesion is limited in rectum and sigmoid colon. 短段型Short segment type megacolon(10%): Lesion is limited in the distal 3-4 cm of rectum. 长段型Long segment type megacolon(10%): Lesion reaches splenic flexure, and even the entire colon. 全结肠型Total colonic type(1%):Lesion reathes entire colon or e
7、vev terminal ileum.,分型 Pathologic types of congenital megacolon,临床表现Clinical manifestations,新生儿期The neonatal period: acute low incomplete intestinal obstruction,Delay of fetal discharge Abdominal distention and vomiting Constipation Wasting and malnutrition,Clinical manifestations,婴幼儿期：慢性低位肠梗阻 Infan
8、t and childhood: subacute or chronic low incomplete intestinal obstruction 反复便秘Recurrent constipation 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 Occurring within 2 months 肠梗阻 Intestinal obstruction 小肠结肠炎 Enterocolitis 肠穿孔，腹膜炎 Bowel perf
9、oration 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 enema 3、直肠肛管测压 Anorectal manometry 4、直肠活检 Biopsy 5、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: reflection of normal anorectal reflex di
10、sappears,正常直肠肛管反射,肠壁粘膜腺体 Intestinal mucosal glands,酶阳性神经 Enzyme positive nerve,正常人 Normal child,巨结肠患儿 Megacolon child,诊 断 Diagnosis,1、病史 Medical history 2、钡灌肠 Barium enema 3 、直肠肛管测压力 Anorectalmanometry 3、直肠黏膜组织活检 Rectal mucosal biopsy,1、胎粪性便秘 Meconium constipation 2、新生儿肠闭锁 Neonatal intestinal atresi
11、a 3、特发性巨结肠 Idiopathic megacolon 4、巨结肠类缘病Neuronal intestinal dysplasia 5、肛门内括约肌失弛症 Internal anal sphincter achalasia 6、继发性巨结肠 Secondary megacolon 7、内分泌性 Endocrine disease 8、乙状结肠过长症 Redundant sigmoid colon,鉴别诊断 Differential diagnosis,先天性巨结肠 Congenital megacolon,继发性巨结肠 Secondary megacolon,治 疗 Treatment
12、,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: Operation treatment. Resection of the lesion bowel and dilatation bowel.,术前准备（保守治疗） Preoperative preparation (conservative treatment) 1、洗肠 Intestinal lavage 2、括肛、通便 Enlarge anus and inducing defeation 3、 缓泻药 Application of Laxative drug,手术方式 Operation methods,Sewnons operation Soaves operation Duhamels operation Rehbeins operation Martins 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,
