1、2023/1/4 先天性巨结肠 Congenital megacolon 定义定义 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 submuco
3、sa 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 直肠壶腹潴便 经大脑整
4、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 intestinal segm
5、ent 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)megacolon(85%):Lesion is
6、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 evev terminal ile
7、um.分型 Pathologic types of congenital megacolon 临床表现Clinical manifestations 新生儿期新生儿期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 Infant and childhoo
8、d:subacute or chronic low incomplete intestinal obstruction 1.反复便秘Recurrent constipation 2.进行性腹胀Progressive abdominal distension 3.发育迟缓，营养不良Growth retardation,malnutrition 并发症并发症 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 perforation
9、 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 辅助检查辅助检查 Accessory examination 钡灌肠钡灌肠X X表现表现 Barium enema X-ray 葛X，2y，长段型巨结肠，soave Anorectal manometry:reflection of normal anorectal reflex
10、 disappears 正常直肠肛管反射正常直肠肛管反射 肠壁粘膜腺体肠壁粘膜腺体 Intestinal mucosal glands 酶阳性神经酶阳性神经 Enzyme positive nerve 正常人正常人 Normal child 巨结肠患儿巨结肠患儿 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
11、闭锁 Neonatal intestinal atresia 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 鉴别诊断鉴别诊断 Differential diagnosis 先天性巨结肠 Congenital megacolon 继发性巨结肠
12、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: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 Lax
13、ative drug 手术方式手术方式 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 手术的基本步骤手术的基本步骤 Duhamel operation 术后并发症 Postoperative complications 吻合口感染、泄漏 Anastomotic infection and leakage 尿潴留 Ret
14、ention 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 anal sphincter 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 megacolon 3、临床特征Clinical characteristics and diagnosis
15、of megacolon 4、治疗原则The surgical principles of megacolon 肛门直肠畸形的分类肛门直肠畸形的分类 Classification of anorectal malformation 没有没有X X影像学资料前不要轻易手术影像学资料前不要轻易手术 We should not do operation easily without X-ray MRMR 针形电刀 Needle type electric knife 电刺激仪 Electrical stimulation instrument 先天性胆道闭锁先天性胆道闭锁 Congenital bi
16、liary atresia Diagnosis and differential diagnosis MRCP,laparoscopic exploration and intraoperative cholangiography Cholestasis syndrome Operation treatment of congenital biliary atresia 纤维三角板的切除 Excision of fibrous triangle plate 肝门部与肠吻合 Anastomosis of jejunum and Hilar 胆道闭锁术后用药胆道闭锁术后用药 Medication after biliary atresia operation 激素应用激素应用 Hormone drugs 抗生素应用抗生素应用 Antibiotics 利胆药应用利胆药应用 Cholagogue 先天性胆总管囊肿先天性胆总管囊肿 Congenital choledochal cyst MRCP 治疗治疗:囊肿切除，囊肿切除，Roux-en-Y吻合，抗反流瓣吻合，抗反流瓣 Treatment:c
