Type B intestinal neuronal dysplasia
Type B intestinal neuronal dysplasia
复制标题
B型肠神经元发育不良
DOI：
10.1111/j.1442-200x.2005.02067.x
复制
发表时间：
2005
影响因子：
1.4
通讯作者：
K. Osawa
中科院分区：
文献类型：
--
作者：
Y. Kanamori;K. Hashizume;M. Sugiyama;T. Tomonaga;K. Terawaki;K. Osawa
肠神经元发育不良最早由 Meier-Ruge 于 1971 年报道 1 。类似病例主要在欧洲报道。相反，日本尚未报告的病例很少。我们介绍一名患有慢性严重便秘的三岁日本男孩的病例。他接受了改良的索阿维手术。切除的直肠粘膜显示肠神经元发育不良的典型表现，包括粘膜下神经节增多、巨神经节、异位神经节细胞以及固有层神经纤维中乙酰胆碱酯酶活性增加。 2 病理检查显示，切除的结肠近端仍具有不典型增生的特征。因此，发育不良的肠道并未完全切除。然而，便秘大大改善，现在他正在门诊接受随访。
Intestinal neuronal dysplasia was first reported by Meier-Ruge in 1971 1 . Similar cases have been mainly reported in Europe. Conversely, very few cases have yet to be reported in Japan. We present the case of a three-year-old Japanese boy with chronic and severe constipation. He underwent a modified Soave operation. The resected rectal mucosa showed typical findings of intestinal neuronal dysplasia, which included submucosal hyperganglionosis, giant ganglia, ectopic ganglion cells, and increased activity of acetylcholine-esterase in the lamina propria nerve fibers. 2 Pathological examination showed that the proximal end of the resected colon still had characteristics of dysplasia. Therefore, the dysplastic intestine was not completely resected. However, the constipation greatly improved and he is now followed on an outpatient basis.