喉丛状神经纤维瘤:一个具有挑战性的管理困境

N. Nerurkar, G. Kapre, P. Murthy
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引用次数: 0

摘要

简介:在导致儿童喘鸣的众多原因中,喉肿瘤是相对罕见的。更罕见的是出现喉内神经纤维瘤。丛状神经纤维瘤与I型神经纤维瘤病(NF1)有关。它们的典型特征是弥漫性肿瘤,边缘不清晰,这使得完全切除成为一项挑战,复发的机会更高。目的:记录我们对咽内神经纤维瘤的治疗经验,并探讨这种罕见疾病的治疗方案。方法:我们报告2例小儿丛状神经纤维瘤。两例患儿均有声门上肿物,妨碍呼吸和吞咽。他们也符合I型神经纤维瘤的标准。两例患者均行微清喉入路及激光辅助手术切除。结果:手术后,两例患者均顺利恢复,无呼吸或吞咽困难。结论:咽内神经纤维瘤治疗的两难选择是咽内激光手术还是积极的外入路手术。对有限的现有文献的简短回顾表明,将手术限制在尽可能完整的内窥镜切除是更明智的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Plexiform Neurofibroma of the Larynx: A Challenging Management Dilemma
Introduction: Among the many causes responsible for stridor in the pediatric population, tumors of the larynx are relatively rare. Rarer still is the presence of endolaryngeal neurofibromas. Plexiform neurofibromas are associated with type I neurofibromatosis (NF1). Their typical characteristic is that they are diffuse tumors with indistinct margins, which makes complete resection a challenge and the chances of recurrences higher. Objective: To document our experience with endolaryngeal neurofibromas and to discuss the treatment options available for this rare condition. Methods: We present two cases of plexiform neurofibromas in pediatric patients. Both children presented with large supraglottic masses which interfered with breathing and swallowing. They also fulfilled the criteria for type I neurofibroma. Endolaryngeal approach with microdebrider and laser-assisted surgical excision was performed in both cases. Result: Following surgery, both patients had uneventful recovery and no subsequent breathing or swallowing difficulties. Conclusion: The dilemma in the management of endolaryngeal neurofibroma is the choice between endolaryngeal laser and aggressive external approach surgery. A short review of the limited existing literature shows that it is wiser to limit the surgery to as complete a resection as is possible endoscopically.
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