Galliani C A, Matt B H
Division of Pediatric Pathology, James Whitcomb Riley Hospital for Children, Indianapolis, IN 46202-5200.
Int J Pediatr Otorhinolaryngol. 1993 Jan;25(1-3):243-8. doi: 10.1016/0165-5876(93)90059-c.
Striated muscle cells within peripheral nerve trunks, a very rare dishistogenetic lesion, was found in the arytenoid submucosa of a ten and a half week old afroamerican male with the Freeman-Sheldon syndrome (craniocarpotarsal dysplasia) who presented with obstructive apnea. Laryngomalacia was confirmed endoscopically. Supraglottoplasty offered temporary relief of the obstruction. Our histopathological observation is puzzling and perhaps adds a new dimension to the appropriateness of 'dysplasia'. It remains to be seen whether the neuromuscular lesion is a coincidental finding, a mere curiosity or bears any clinical significance.
在一名患有弗里曼-谢尔顿综合征(颅腕跗骨发育异常)且出现阻塞性呼吸暂停的10岁半非裔美国男性的杓状软骨黏膜下层,发现了周围神经干内的横纹肌细胞,这是一种非常罕见的组织发生异常病变。经内镜检查证实为喉软化症。声门上成形术暂时缓解了阻塞。我们的组织病理学观察令人困惑,可能为“发育异常”的恰当性增添了新的维度。这种神经肌肉病变是偶然发现、仅仅是一种罕见现象还是具有任何临床意义,仍有待观察。