儿科学课件：小儿巨结肠.ppt

* Proximal part enlargement Danish physician Hirschsprung * * aproctia –无肛 * neural canal; archigaster;celenteron;coelenteron;gastrocoel;gastrocoele;priming gut;primitive gut;protogaster…bowel Embrye胚胎 peristalsis 蠕动cell differentiation; Gestation * * Amyelination无髓鞘 Normal gliocyte * 神经元，胶质细胞 gliocyte * 无神经元（肌间），神经丛增生 * 免疫组化组织蛋白酶D 阴性，无神经元 * 左，手术标本，未见神经元；右，活检标本，右侧粘膜，可见粘膜下神经元 * * 直肠ampulla壶腹部空虚 * * 测定直肠和肛门括约肌的反射性压力变化 可诊断先天性巨结肠和鉴别其他原因引起的便秘 正常小儿和功能性便秘，当直肠受膨胀性刺激后，内括约肌立即发生反射性放松，压力下降 先天性巨结肠患儿内括约肌非但不放松，而且发生明显的收缩，使压力增高 2周以内的新生儿有时可出现假阳性结果 * 活体组织检查?? 取距肛门4cm以上直肠壁粘膜下层及肌层一小块组织 检查神经节细胞的数量，巨结肠患儿缺乏节细胞 直肠粘膜组织化学检查法 根据痉挛段粘膜下及肌层神经节细胞缺如处增生、肥大的副交感神经节前纤维不断释放大量乙醯胆碱和胆碱酶，经化学方法可以测定出两者数量和活性均较正常儿童出5～6倍，有助于对先天性巨结肠的诊断 免疫组化检测神经元特异性烯醇化酶 intestinal nonrotation or incomplete rotation around the superior mesenteric artery (SMA). * ultrashort Congenital Megacolon Outline Definition and Epidemiology Etiology and Pathophysiology Clinical manifestation and complication Laboratory finding Diagnosis and Differential diagnosis Management Congenital Megacolon Aganglionosis Hirschsprung’s Disease (HD) This condition is caused by failure of ganglion cells to migrate into the distal bowel, resulting in spasm and functional obstruction of the aganglionic segment. Congenital Megacolon-History First report in 1691----Frederick Ruysch Described in detail in 1886----Hirschsprung Congenital Megacolon---Mya(1894) Tittle(1901)---- Failure of ganglion cells Ehrenpries (1964)----Etiology and Pathogenesis Congenital Megacolon-Epidemiology Incidence rate：1/2000~1/5000 No.2 of abnormality of GI tract Male: female 3~4:1 Congenital Megacolon-Etiology Developmental disorder of nervous crest Genetic factor Environment factor Developmental disorder of nervous crest Nervous crest Bowel ganglion cell Embrye：5-12week differentiation Absence or decrease of ganglion cell Loss movement/peristalsis spasticity Functional bowel obstruction Dilate of proximal colon Genetic factor Familial propensity Gender modify