先天性巨結(jié)腸與膽道剖析

1、2024/3/13,先天性巨結(jié)腸Congenital megacolon,定義 Defination,由于直腸或者結(jié)腸遠端的腸管持續(xù)痙攣，糞便淤積在近端結(jié)腸，使該腸管肥厚、擴張Rectal or colonic intestinal has persistent spasm and fecal deposition in the  proximal colon resul

2、ts in hypertrophy and dilatation in proximal segment.,Hirschsprung’s Disease,HD 赫什朋氏病Aganglionosis 先天性無神經(jīng)節(jié)細胞癥,英文名稱 English name,概述 Summary,一種常見的消化道畸形A common digestive tract malformation 有遺傳傾向， 約1.4-7.8%Ge

3、netic predisposition, about 1.4-7.8% 發(fā)病率為1:2000-5000Incidence rate of 1:2000-5000 男:女 = 4:1Male:female=4:1,病 因Etiology,病變腸管肌間及粘膜下神經(jīng)節(jié)細胞缺如，是一種發(fā)育停頓，停頓越早，無神經(jīng)節(jié)細胞段越長.Absence of ganglion cells in muscle and

4、 submucosa 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周消化道神經(jīng)母細胞從頭端向尾端移行Neuroblastoma cells of digestive

5、 tract migrate from the beginning to the end In the fifth week of fetal.,,Normal defecation physiology,直腸壺腹潴便,,經(jīng)大腦整合，決定排便與否,便意,直腸肛管抑制反射肛管感受糞便性質(zhì),,,骶髓低級中樞,腸壁感受器,Absence of ganglion cells,Smooth muscle

6、60;of lesion intestine sustaines contraction,Anorectal reflex loop is interrupted,Stool can not be discharged. Proximal normal intestine has compensatory dilatation and h

7、ypertrophy and forms huge expansive intestinal segment,,,,,Pathology of congenital megacolon,,,,,,病變腸段呈痙攣樣改變(Spasm segment）,近端腸管擴張肥厚，形成巨大結(jié)腸(Enlarged segment),二者之間過度腸段呈漏斗狀稱移行段(Transformed segment),正常結(jié)腸Normal col

8、on,Pathological anatomy of congenital megacolon,按病變長度According to the length of lesion segment常見型Ordinary type (common type) megacolon (85%): Lesion is limited in rectum and sigmoid colon.

9、短段型Short segment type megacolon(10%): Lesion is limited in the distal 3-4 cm of rectum.長段型Long segment type megacolon(10%): Lesion reaches splenic flexure, and even the entire col

10、on.全結(jié)腸型Total colonic type(1%):Lesion reathes entire colon or evev terminal ileum.,分型Pathologic types of congenital megacolon,臨床表現(xiàn)Clinical manifestations,新生兒期The neonatal period: acute low incom

11、plete intestinal obstruction,Delay of fetal dischargeAbdominal distention and vomitingConstipation Wasting and malnutrition,Clinical manifestations,嬰幼兒期：慢性低位腸梗阻 Infant and childhood: su

12、bacute or chronic low incomplete intestinal obstruction 反復便秘Recurrent constipation 進行性腹脹Progressive abdominal distension發(fā)育遲緩，營養(yǎng)不良Growth retardation, malnutrition,并發(fā)癥 Complications,多在2個月內(nèi)發(fā)生 Occurring

13、0;within 2 months腸梗阻 Intestinal obstruction 小腸結(jié)腸炎 Enterocolitis 腸穿孔，腹膜炎 Bowel perforation and peritonitis 繼發(fā)敗血癥，肺炎 Secondary sepsis and pneumonia,1、肛門指診  Rectal touch 2、鋇灌腸 Barium enema3、直腸肛管測壓 Anorectal manom

14、etry 4、直腸活檢 Biopsy5、肌電圖 Electromyogram,輔助檢查 Accessory examination,,鋇灌腸X表現(xiàn) Barium enema X-ray,葛X，2y，長段型巨結(jié)腸，soave,Anorectal manometry: reflection of normal anorectal  reflex disappears,正常直腸肛管反射,,,,腸壁粘

15、膜腺體Intestinal mucosal glands,,酶陽性神經(jīng)Enzyme positive nerve,正常人Normal child,巨結(jié)腸患兒Megacolon child,診 斷 Diagnosis,1、病史 Medical history2、鋇灌腸 Barium enema 3 、直腸肛管測壓力 Anorectalmanometry3、直腸黏膜組織活檢

16、 Rectal mucosal biopsy,1、胎糞性便秘 Meconium constipation 2、新生兒腸閉鎖 Neonatal intestinal atresia 3、特發(fā)性巨結(jié)腸 Idiopathic megacolon 4、巨結(jié)腸類緣病Neuronal intestinal dysplasia5、肛門內(nèi)括約肌失弛癥 Internal anal sphincter ac

17、halasia6、繼發(fā)性巨結(jié)腸 Secondary megacolon7、內(nèi)分泌性 Endocrine disease8、乙狀結(jié)腸過長癥 Redundant sigmoid colon,鑒別診斷 Differential diagnosis,先天性巨結(jié)腸Congenital megacolon,繼發(fā)性巨結(jié)腸Secondary megacolon,治 療 Treatment,治療原則:手術(shù)治療,切除病變腸段以及擴張

18、肥厚的腸管Treatment principle: Operation treatment. Resection of the lesion bowel and dilatation bowel.,術(shù)前準備（保守治療）Preoperative preparation (conservative treatment) 1、洗腸 Intestinal lavage

19、 2、括肛、通便 Enlarge anus and inducing defeation 3、 緩瀉藥 Application of Laxative drug,手術(shù)方式 Operation methods,Sewnon’s operationSoave’s operationDuhamel’s operationRehbein’s operationMartin’s oper

20、ation,Swenson 改良術(shù)：結(jié)腸經(jīng)直腸內(nèi)拖出（Pull-through）切除術(shù),,,,Soave procedure手術(shù)的基本步驟,,Duhamel operation,,術(shù)后并發(fā)癥 Postoperative complications,,,吻合口感染、泄漏 Anastomotic infection and leakage尿潴留 Retention of urine 小腸結(jié)腸炎 Enterocolitis

21、 吻合口狹窄，便秘復發(fā) Anastomotic stenosis and recurrent constipation 肛門內(nèi)括約肌損傷 Internal anal sphincter injury 遠期生活質(zhì)量下降 Declined quality of life in long-term,要點Key points,1、概念Concept of megacolon 2、分型Pathological type of m

22、egacolon3、臨床特征Clinical characteristics and diagnosis of megacolon4、治療原則The surgical principles of megacolon,肛門直腸畸形的分類Classification of anorectal malformation,沒有X影像學資料前不要輕易手術(shù)We should not do operation e

23、asily without X-ray,MR,針形電刀Needle type electric knife 電刺激儀Electrical stimulation instrument,先天性膽道閉鎖Congenital biliary atresia,Diagnosis and differential diagnosis,MRCP,laparoscopic exploration

24、0;and intraoperative cholangiography,Cholestasis syndrome,Operation treatment of congenital biliary atresia,纖維三角板的切除 Excision of fibrous triangle plate 肝門部與腸吻合 Anastomosis of jejunum and Hi

25、lar,膽道閉鎖術(shù)后用藥 Medication after biliary atresia operation,激素應用Hormone drugs 抗生素應用Antibiotics 利膽藥應用Cholagogue,先天性膽總管囊腫Congenital choledochal cyst,MRCP,治療:囊腫切除，Roux-en-Y吻合，抗反流瓣Treatment: cyst excision,