Departments of Orthopaedic Surgery.
Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
J Pediatr Orthop B. 2022 Nov 1;31(6):572-582. doi: 10.1097/BPB.0000000000000961. Epub 2022 Feb 7.
The objective of this systematic review was to synthesize evidence regarding spinal screening recommendations, types of spinal and thoracic neurofibromatosis type 1 (NF1) tumors, medical therapy for NF1-associated neoplasms, and treatment with magnetically controlled growing rods (MCGRs) or cervical kyphosis correction in pediatric patients with NF1. We queried PubMed, Embase, Cochrane Library, Web of Science, Scopus, Clinicaltrials.gov, and medRxiv for studies reporting spinal screening recommendations, prognosis, and medical therapy for NF1-associated spinal tumors and MCGR use or cervical kyphosis correction in pediatric NF1 patients, yielding 758 publications, 33 of which were included. There is no consensus on spinal screening interval. Computed tomography is recommended for postoperative monitoring. Patients with gangliomas and spinal neurofibromas had nearly complete symptom resolution after resection. Plexiform neurofibromas were most commonly treated with resection and laminectomy; some patients reported tumor enlargement after intervention. Malignant nerve sheath tumors have high rates of metastasis even after chemoradiation and resection. MEK-inhibitors produced limited regression in tumor size. Sirolimus and thalidomide reduced tumor size but caused more severe adverse effects than MEK-inhibitors. Improvements in major curves and T1-T12 height gain were reported after MCGR intervention. Anteroposterior arthrodesis produced the greatest correction of dystrophic cervical kyphosis. There may be value in establishing standardized spinal screening protocols for pediatric NF1 patients. Surgical correction of NF1-associated spinal deformity is effective, though current medical therapies for spinal tumors have limited success. Areas for further investigation include determining appropriate screening intervals, choice of medical therapy for spinal tumors, and long-term outcomes of MCGRs. Level of Evidence: IV.
本系统评价的目的是综合有关脊柱筛查建议、脊柱和胸 NF1 型神经纤维瘤病（NF1）肿瘤类型、NF1 相关肿瘤的医学治疗以及儿科 NF1 患者使用磁控生长棒（MCGR）或颈椎后凸矫正的证据。我们在 PubMed、Embase、Cochrane 图书馆、Web of Science、Scopus、Clinicaltrials.gov 和 medRxiv 中查询了报告脊柱筛查建议、预后和 NF1 相关脊柱肿瘤的医学治疗以及儿科 NF1 患者使用 MCGR 或颈椎后凸矫正的研究，共获得 758 篇出版物，其中 33 篇被纳入。目前对于脊柱筛查间隔时间尚无共识。建议术后监测使用计算机断层扫描。神经节瘤和脊柱神经纤维瘤患者在切除后几乎完全缓解症状。丛状神经纤维瘤最常采用切除和椎板切除术治疗；一些患者报告干预后肿瘤增大。恶性神经鞘瘤即使在放化疗和切除后也有很高的转移率。MEK 抑制剂在肿瘤大小方面仅产生有限的消退。西罗莫司和沙利度胺使肿瘤缩小，但引起的不良反应比 MEK 抑制剂更严重。MCGR 干预后报告主要曲线和 T1-T12 高度增加得到改善。前后关节融合术对营养不良性颈椎后凸的矫正效果最大。为儿科 NF1 患者建立标准化脊柱筛查方案可能具有价值。NF1 相关脊柱畸形的手术矫正有效，但目前脊柱肿瘤的医学治疗效果有限。进一步研究的领域包括确定适当的筛查间隔、选择脊柱肿瘤的医学治疗以及 MCGR 的长期结果。证据等级：IV。
