Dieler R, Schröder J M
Institut für Neuropathologie, Klinikum der RWTH, Aachen, Federal Republic of Germany.
Acta Neuropathol. 1989;78(6):649-61. doi: 10.1007/BF00691292.
The myenteric plexus and intramuscular nerve bundles in the circular muscle layer of the pylorus from 37 children with infantile hypertrophic pyloric stenosis (IHPS) obtained at pyloromyotomy were studied by light and electron microscopy and compared to six control cases without clinical evidence of IHPS. In certain IHPS cases degenerative alterations of the axons predominated. The axonal changes consisted of (1) severely increased variability of diameters with evidence of degeneration and regeneration of some axons, (2) accumulation of electron-dense bodies, lysosomes and pleomorphic membranous cytoplasmic bodies, (3) increase in the number of maloriented neurofilaments, and (4) aggregation of glycogen granules. Degenerative changes or immaturity of perikarya of neurons and glial cells in the myenteric plexus were not a significant feature. While axonal changes predominated in some IHPS cases there were severe changes of smooth muscle cells in others suggesting that a primarily neurogenic type of IHPS can be distinguished from a predominantly myogenic type. Although the etiology of the axonal changes in IHPS is not clear, it is suggested that they play an important role in the pathogenesis of pyloric stenosis and hypertrophy.
对37例因婴儿肥厚性幽门狭窄（IHPS）行幽门肌切开术获取的幽门环形肌层中的肌间神经丛和肌内神经束进行了光镜和电镜研究，并与6例无IHPS临床证据的对照病例进行比较。在某些IHPS病例中，轴突的退行性改变占主导。轴突变化包括：（1）直径变异性严重增加，一些轴突有退变和再生迹象；（2）电子致密体、溶酶体和多形性膜性细胞质体聚集；（3）错向神经丝数量增加；（4）糖原颗粒聚集。肌间神经丛中神经元和神经胶质细胞的胞体退行性改变或不成熟并非显著特征。虽然在某些IHPS病例中轴突变化占主导，但在其他病例中平滑肌细胞有严重变化，这表明可将原发性神经源性IHPS与主要为肌源性的类型区分开来。虽然IHPS中轴突变化的病因尚不清楚，但提示它们在幽门狭窄和肥厚的发病机制中起重要作用。
