不同年龄段先天性巨结肠患儿齿状线上神经节细胞、神经纤维分布的意义-现代医学

不同年龄段先天性巨结肠患儿齿状线上神经节细胞、神经纤维分布的意义

作者：莫优炼¹ 杨六成² 胡小华¹ 陈茂芳³ 段立锋⁴ 苏嘉鸿¹ 张平锋¹ 钟陈¹ 张家德¹ 冯大军¹

单位：1. 南方医科大学附属中山市博爱医院小儿外科, 广东中山 528408;
2. 南方医科大学珠江医院小儿外科, 广东广州 510280;
3. 南方医科大学附属中山市博爱医院麻醉科, 广东中山 528408;
4. 南方医科大学附属中山市博爱医院病理科, 广东中山 5284084

关键词：神经节细胞神经纤维钙视网膜蛋白染色先天性巨结肠

分类号：R656.9

出版年·卷·期（页码）：2018·46·第十期（1093-1098）

摘要：

目的：研究不同年龄段先天性巨结肠（HD）患儿齿状线上肠壁神经节细胞、神经纤维的分布，探讨其临床意义，明确经肛门直肠肌层活检的取材范围。方法：收集行经肛门巨结肠根治术患儿52例，按年龄分为A组（新生儿组）、B组（婴儿组）和C组（幼儿组）3组，标本齿状线上连续取材，行HE染色（结果阳性46例）和钙视网膜蛋白染色，观察神经节细胞、神经丛和神经纤维变化及分布情况。结果：患儿自齿状线上至痉挛段肠管缺乏神经节细胞，与扩张段存在明显差异（P<0.01）；A、B、C组分别自齿状线上1、2、2.5 cm内肠管神经丛较少，神经纤维稀疏；越过此节段肠管神经纤维增粗、变性，可见肥大神经丛，差异明显（P<0.01）；钙视网膜蛋白染色对神经节的判断有明显指导作用。结论：HD患儿存在齿状线上肠管的"生理性无神经节细胞"区域，不同年龄段患儿此区域长度不同，活检应避开此区域取材。分析神经丛和神经纤维形态，结合钙视网膜蛋白染色结果，可有效区别"生理性无神经节细胞"与巨结肠病变肠管，以免误诊。

Objective:To study the distributions and significance of enteric ganglion cells and nerve fiber above the dentate line of Hirschsprung's disease (HD) in different age groups, and to explore the exact area for transrectal myometrial biopsy. Methods:The HD patients undergoing radical operation were divided into three groups,i.e., neonatal group (group A), infant group (group B) and child group (group C). Samples were taken continuously from 2-4 cm above the dentate line and stained with HE and calretinin. The changes and the distributions of enteric ganglion cells, nerve plexus and nerve fiber above the dentate line were observed. Results:No ganglion cells from dentate line to spasmodic segments in three groups were observed, which was significantly different from those of dilated segments (P<0.01).Nerve plexus and nerve fiber in 1 cm, 2 cm and 2.5 cm above the dentate line were scarcely found in group A, group B, and group C respectively, but the thickening and denaturating nerve fiber and hyperplastic nerve plexus beyond those segments were observed. There were significant differences between the two segments in each group (P<0.01). Calretinin staining played an important role in observing ganglion cells. Conclusion:There is a "physiological non-ganglion cells" area above the dentate line of Hirschsprung's disease. The distribution of this area varies in different ages,and the area should be avoided when the specimens are taken. By analyzing the morphology of nerve plexus and nerve fiber, and by combining it with the results of calretinin staining, we can effectively distinguish "physiological non-ganglion cells" area from intestinal lesion of Hirschsprung disease and avoid misdiagnosis.

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