Neuroscience Center, Toronto Western Hospital, Spinal Program, West Wing 4th Floor, 399 Bathurst Street, Toronto, Ontario, Canada.
J Neurosurg Spine. 2011 Mar;14(3):356-66. doi: 10.3171/2010.9.SPINE09242. Epub 2011 Jan 14.
Patients with neurofibromatosis Type 1 (NF-1) at the cervical spine present significant surgical challenges due to neural compression, multiplicity of tumors, and complex spinal deformities. Iatrogenic instability following resection of tumors is underappreciated in the literature. The focus of this study was to understand the indications for stabilization in this specific group of patients.
The authors performed a retrospective review of 20 cases involving NF-1 patients with symptomatic cervical spine neurofibromas who underwent surgical decompression and tumor resection, with or without instrumentation, between 1991 and 2008. They also included 2 additional cases involving patients treated before 1991. Imaging findings and data pertaining to clinical presentation, intraoperative management, and postoperative assessment were compiled to clarify the indications for stabilization. An ordinal pain scale based on patient self-assessment was used. Neurological function was evaluated using American Spinal Injury Association Impairment Scale scores.
The patient group comprised 13 men and 9 women. Their median age at presentation was 42.5 years; their median age at initial diagnosis of NF-1 was 30 years (range 8-74 years). The median duration of follow-up (since presentation) was 7 years (range 1-32 years). Progressive myelopathy was the main presenting symptom. Spinal cord compression was identified in 13 patients on presentation. Complete removal of the symptomatic tumors was performed in 11 patients. Ten patients underwent instrumented fusion during their first surgery. Six of these 10 required a second surgery-with fixation in 4 cases and without in 2. Of the 12 patients who did not receive instrumented fusion in their first surgery, 8 required a second surgery-with fixation in 5 cases and without in 3. Neurological deterioration due to progressive deformity was the indication for the second surgery in 3 of the 5 patients who required instrumented fusion only in their second surgery; the other 2 patients presented with neurological deterioration secondary to tumor progression. Four patients needed a third operation and instrumented fusion: 3 for deformity-related deficit and 1 for tumor progression. Based on the latest follow-up, 21 patients were stable clinically and radiologically, and 1 patient had died.
This specific group of patients represents a significant surgical challenge. In this retrospective analysis, emphasis is placed on early stabilization of the cervical spine to prevent late deformity as part of the comprehensive management of patients with NF-1.
神经纤维瘤病 1 型（NF-1）患者颈椎病变存在明显的手术挑战，主要是由于神经受压、肿瘤多发性和复杂的脊柱畸形。在文献中，肿瘤切除后的医源性不稳定往往被低估。本研究的重点是了解该特定患者群体中进行稳定治疗的适应证。
作者回顾性分析了 1991 年至 2008 年间 20 例 NF-1 伴症状性颈椎神经纤维瘤患者的病例，这些患者接受了手术减压和肿瘤切除，伴或不伴器械固定。其中还包括 2 例在 1991 年之前治疗的患者。编译影像学发现和与临床表现、术中管理和术后评估相关的数据，以阐明稳定的适应证。采用基于患者自我评估的有序疼痛量表。使用美国脊髓损伤协会损伤量表评分评估神经功能。
患者组包括 13 名男性和 9 名女性。他们的中位就诊年龄为 42.5 岁；初始 NF-1 诊断年龄为 30 岁（8-74 岁）。中位随访时间（自就诊以来）为 7 年（1-32 年）。进行性脊髓病是主要的表现症状。13 名患者在就诊时存在脊髓压迫。11 名患者完全切除了有症状的肿瘤。10 名患者在首次手术中接受了器械固定融合。其中 6 名患者需要进行第二次手术，其中 4 例固定，2 例不固定。在 12 名未在首次手术中接受器械固定融合的患者中，8 名患者需要进行第二次手术，其中 5 例固定，3 例不固定。由于进展性畸形导致的神经功能恶化是 5 名仅在第二次手术中需要器械固定融合患者进行第二次手术的适应证；另外 2 名患者因肿瘤进展而出现神经功能恶化。4 名患者需要进行第三次手术和器械固定融合：3 例为畸形相关缺陷，1 例为肿瘤进展。根据最新随访结果，21 例患者在临床和影像学上稳定，1 例患者死亡。
该特定患者群体具有重大的手术挑战。在本回顾性分析中，强调早期稳定颈椎，以防止晚期畸形，作为 NF-1 患者综合管理的一部分。
