先天性巨结肠与胆道剖析.pptxVIP

先天性巨结肠与胆道剖析;定义 Defination;Hirschsprung’s Disease,HD 赫什朋氏病Aganglionosis 先天性无神经节细胞症;概述 Summary ;病 因Etiology;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.;;Absence of?ganglion cells ;; 按病变长度According to the length of lesion segment 常见型Ordinary type?(common?type)?megacolon (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?evev terminal ileum. ;临床表现Clinical manifestations ;Clinical manifestations;并发症 Complications;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 ;;第16页/共44页;钡灌肠X表现 Barium enema X-ray;第18页/共44页;Anorectal manometry:?reflection?of normal anorectal??reflex disappears ;第20页/共44页;;诊 断 Diagnosis;1、胎粪性便秘 Meconium constipation 2、新生儿肠闭锁 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 ;先天性巨结肠 Congenital?megacolon ;治 疗 Treatment;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 ;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; ; ;要点Key points;肛门直肠畸形的分类Classification?of anorectal malformation ;没有X影像学资料前不要轻易手术We should?not do operation easily without X-ray ;MR;针形电刀 Needle?type electric?knife 电刺激仪 Electrical stimulation?instrument ;先天性胆道闭锁Congenital biliary atresia ;Diagnosis and differential diagnosis ;Operation?treatment?of congenital biliary atresia ;胆道闭锁术后用药 Medication after?biliary atresia operation;先