Ransom Evan R, Yoon Caroline, Manolidis Spiros
Department of Otolaryngology, Head & Neck Surgery, Columbia University, 180 Fort Washington, HP813, New York, NY 10032, USA.
Int J Pediatr Otorhinolaryngol. 2006 Jun;70(6):1055-61. doi: 10.1016/j.ijporl.2005.10.025. Epub 2006 Jan 6.
Plexiform neurofibromas of the head and neck in neurofibromatosis type 1 (NF 1) carry a significant morbidity with substantial loss of function as well as significant cosmetic problems. We describe our experience with early aggressive surgical intervention in such patients in order to avert these problems.
Retrospective review of four consecutive pediatric patients with massive head and neck plexiform neurofibromas who underwent single stage near total or sub-total tumor resections.
All four patients were referred for obstructive airway symptoms. Each patient experienced complete relief of symptoms and return of function without additional neurological deficits. There were two minor complications and no major complications of surgical resection. There have been no recurrences to date, with follow-up ranging from 15 months to 5 years.
Early surgical intervention of NF 1 patients with plexiform neurofibromas of the head and neck with a goal of near total resection avoids the loss of function associated with these tumors, such as tracheostomy dependence, swallowing difficulty, and speech problems, and prevents the inexorable progression of substantial cosmetic deformity. Successful management of these complex lesions requires detailed preoperative planning, advanced surgical techniques, and vigilant postoperative care.
1型神经纤维瘤病（NF 1）患者的头颈部丛状神经纤维瘤会导致严重的功能丧失及明显的美容问题，发病率很高。我们描述了对此类患者进行早期积极手术干预以避免这些问题的经验。
对4例连续的患有巨大头颈部丛状神经纤维瘤的儿科患者进行回顾性研究，这些患者均接受了一期近全切除或次全切除肿瘤手术。
所有4例患者均因气道阻塞症状前来就诊。每位患者症状均完全缓解，功能恢复，且无额外的神经功能缺损。手术切除有2例轻微并发症，无严重并发症。迄今为止无复发，随访时间为15个月至5年。
对患有头颈部丛状神经纤维瘤的NF 1患者进行早期手术干预，目标是近全切除，可避免与这些肿瘤相关的功能丧失，如气管造口依赖、吞咽困难和言语问题，并防止严重美容畸形的不可阻挡的进展。成功处理这些复杂病变需要详细的术前规划、先进的手术技术和警惕的术后护理。
