324例肠神经元发育异常患儿病理特点与术后并发症关系分析  

The relationship between postoperative complication and pathological features of 324 patients with neuronal intestinal malformations

作　　者：钭金法[1] 钱云忠[1] 熊启星[1] 李民驹[1] 章希圣[1] 汤宏峰[2] 顾伟忠[2] 项志英[1] 卢红莲[1] 冯志刚[1] 朱雄凯[1] 

机构地区：[1]浙江大学医学院附属儿童医院外科,杭州310003 [2]浙江大学医学院附属儿童医院病理科,杭州310003

出　　处：《中华外科杂志》2006年第7期463-466,共4页Chinese Journal of Surgery

摘　　要：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 relationship between the morphological features of different types of neuronal intestinal malformations （NIM） and their postoperative complications. Methods The data of morphological and clinical features of 324 cases with NIM were analyzed retrospectively. Results In all 324 patients, 210 cases were Himchsprung＇s disease （HD）, 38 intestinal neuronal dysplasia（IND）, 45 mixed HD/IND, 8 hypoganglionosis, 22 combined HD/hypoganglionosis and 1 immaturity of ganglion cells. The percentages of normal neuron in bowel of different NIM were 88. 1%, 24. 4%, 18. 4%, 4/8, 27. 7% and 0/1 in HD, HD/IND, IND, hypoganglionosis, HD/hypoganglionosis and immaturity of ganglion cells respectively.There were totally 46 cases complicated with recurrent postoperative enterecolitis （EC）. Incidence of recurrent postoperative EC in HD patients was 6. 7% while in IND/HD and IND patients was 35.6% and 28. 9%, respectively. Incidences of EC in cases with the residual IND margins and with the normal margins were 38.2% and 8. 7% , respectively. Incidence of EC in cases with transanal endorectal pull-through procedure and with transabdominal procedure was 18. 0% and 8. 3%, respectively. Nine cases underwent another procedure because of severe persistent constipation or EC after operation, including 4 cases HD/IND, 1 case IND, 3 cases HD and 1 case HD/hypoganglionosis. Conclusions Neuron distribution is inconsistent with pathology of NIM. Postoperative EC are rare in the patients only with isolated HD. Furthermore, margins with residual IND and transanal endorectal pull-through procedure are risk factors to recurrent EC. However, the extension of excision about IND is uncertain and need further study.

关 键 词：HIRSCHSPRUNG病 手术后并发症 病理学 小肠结肠炎 肠神经元发育异常 

分 类 号：R726.5[医药卫生—儿科]