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目的探讨肠神经元发育异常的病理特点及其与术后并发症之间的关系。方法总结324例肠神经元发育异常患儿的术后病理检查的特点,并对照相应的治疗效果和术后并发症,进行统计分析。结果324例患儿中,先天性巨结肠(H irschsprung′s d isease,HD)210例,肠神经元发育不良(intestinal neuronal dysp lasia,IND)38例,HD伴IND 45例,神经节细胞减少症8例,HD伴神经细胞节减少22例,神经节细胞未成熟症1例。不同病理类型在扩张段神经元正常的比例分别为HD88.1%,HD伴IND 24.4%,IND 18.4%,神经节细胞减少症4/8,HD伴神经细胞节减少27.7%,神经节细胞未成熟症0/1。全组有46例患儿术后出现反复小肠结肠炎(EC)。HD、HD伴IND、IND患儿的术后反复EC发生率分别为6.7%、35.6%、28.9%;切缘正常组与切缘IND组术后反复EC发生率分别为8.7%、38.2%;经肛门手术和经腹手术术后EC的发生率分别为18.0%和8.3%。术后仍有反复腹胀、便秘或严重的EC行再次手术9例,其中4例为HD伴IND,1例为IND,3例HD,1例HD伴神经细胞节减少。结论肠神经元发育异常的神经元分布与大体病理改变有不平衡性,巨结肠同源病较HD神经元分布更不典型;单纯HD的治疗效果较好,术后小肠结肠炎发生率低;切缘仍有IND病变以及经肛门手术是术后反复EC的危险因素;术中冰冻切片对判断切缘神经元有重要意义;IND的切除范围仍有不确定性。
Objective To investigate the pathological features of intestinal neuronal dysplasia and its relationship with postoperative complications. Methods 324 cases of intestinal neurodevelopmental dysplasia in children with postoperative pathological features, and the corresponding control efficacy and postoperative complications, statistical analysis. Results Among the 324 children, 210 cases of Hirschsprung’s disease (HD), 38 cases of intestinal neuronal dysplasia (IND), 45 cases of HD with IND, gangliocytopenia 8 cases, HD with 22 cases of neuronal cell reduction, 1 cases of ganglion cell immature. The percentages of normal neurons in different pathological types were HD 88.1%, HD with IND 24.4%, IND 18.4%, gangliocytopenia 4/8, HD with 27.7% reduction in ganglion cells, Mature 0/1. Forty-six children in the group developed recurrent enterocolitis (EC). The incidences of recurrent EC in children with HD and HD with IND and IND were 6.7%, 35.6% and 28.9%, respectively. The incidences of recurrent EC were 8.7% and 38.2% in patients with normal margin margin and margin margin IND respectively. After anal surgery and abdominal surgery, the incidence of EC were 18.0% and 8.3%. There were still 9 cases of recurrent abdominal distension, constipation or severe reoperation after EC. Among them, 4 were HD with IND, 1 with IND, 3 with HD, and 1 with HD. Conclusions The distribution of neurons in the abnormal development of enteric neurons is unbalanced with the general pathological changes. The distribution of homoeopathies in patients with homomorphic gonorrhea is more atypical than that in HD neurons. The treatment of simple HD is better and the incidence of enterocolitis is lower. There are still IND margin lesions and anal surgery after repeated EC risk factors; intraoperative frozen sections of the judgment of the marginal neurons of great significance; IND resection range is still uncertain.