Kobayashi H, Wester T, Puri P
Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.
J Pediatr Surg. 1997 Dec;32(12):1704-7. doi: 10.1016/s0022-3468(97)90509-1.
BACKGROUND/PURPOSE: Recent reports have identified abnormal innervation of the circular muscle layer involving the fine intramuscular nerve fibers in hypertrophic pyloric stenous (HPS). HPS presenting after 3 months of age is rare. The aim of this study was to determine the distribution pattern of nerve fibers in the smooth muscle layers in HPS and correlate this with age at presentation.
Full-thickness pyloric muscle biopsy specimens were obtained from eight patients with HPS (five age 3 to 5 weeks, two age 3 months, and one age 7 months) and five controls with normal pylorus (age 5 days to 3 years). All specimens were stained with monoclonal antibody to the neural cell adhesion molecule (NCAM) using immunohistochemistry.
There were many NCAM-positive nerve fibers in the circular and longitudinal muscle layers in the controls. No NCAM positive nerve fibers were seen in the circular or longitudinal muscle layers in the five cases of HPS in which the patients were less than 5 weeks old. In the two cases in which the patients were 3 months old, occasional NCAM-positive nerve fibers were seen in the circular layer, and moderate numbers of NCAM positive fibers were seen in the longitudinal muscle layers. Moderate numbers of NCAM-positive nerve fibers in the circular muscle layer and many NCAM positive nerve fibers in the longitudinal muscle layers were identified in the 7-month-old HPS patient.
The data suggest that the pyloric muscle lacks innervation in the young HPS infant. Whereas, at 3 months of age the hypertrophied pyloric muscle is partially innervated, and at 7 months of age the pyloric muscle has practically normal innervation.
背景/目的：最近的报告已确定肥厚性幽门狭窄（HPS）中环形肌层存在异常神经支配，涉及肌内细神经纤维。3个月龄后出现的HPS很罕见。本研究的目的是确定HPS平滑肌层中神经纤维的分布模式，并将其与发病时的年龄相关联。
从8例HPS患者（5例年龄为3至5周，2例年龄为3个月，1例年龄为7个月）和5例幽门正常的对照者（年龄为5天至3岁）获取全层幽门肌活检标本。所有标本均采用免疫组织化学法用抗神经细胞黏附分子（NCAM）的单克隆抗体染色。
对照组的环形和纵形肌层中有许多NCAM阳性神经纤维。在5例年龄小于5周的HPS患者中，环形或纵形肌层未见NCAM阳性神经纤维。在2例年龄为3个月的患者中，环形层偶见NCAM阳性神经纤维，纵形肌层可见中等数量的NCAM阳性纤维。在7个月龄的HPS患者中，环形肌层可见中等数量的NCAM阳性神经纤维，纵形肌层可见许多NCAM阳性神经纤维。
数据表明，HPS幼龄婴儿的幽门肌缺乏神经支配。而在3个月龄时，肥厚的幽门肌部分受神经支配，在7个月龄时，幽门肌的神经支配几乎正常。
