Dastagirzada Yosef, Neifert Sean, Kurland David B, Kim Nora C, Panicucci-Roma Tania, Frempong-Boadu Anthony, Lau Darryl
Department of Neurosurgery, New York University, New York , New York , USA.
Oper Neurosurg. 2025 Jun 1;28(6):762-771. doi: 10.1227/ons.0000000000001373. Epub 2024 Sep 20.
Neurofibromatosis-1 (NF1) dystrophic scoliosis is a challenging disease to manage surgically, with multiplanar curves progressing rapidly and unpredictably. Conservative management with bracing is often unsuccessful, and many patients necessitate instrumented fusion to halt progression of their curves. In rare cases, patients can present with spontaneous vertebral subluxation, significantly complicating the surgical management of this already complex disease process. The objective here was to describe 2 cases of vertebral subluxation in NF1-associated dystrophic scoliosis along with their surgical corrections and clinical courses.
A retrospective review of 2 cases at the authors' institution was performed to describe their preoperative symptom complexes, surgical corrections, and postoperative courses. A narrative review of the literature surrounding NF1-associated dystrophic scoliosis and subluxation is also presented.
Two cases of vertebral subluxation at T4-5 and C7-T1 are presented. Both patients had significant dystrophic features throughout their spines, and halo-gravity traction was unsuccessful in 1 patient and led to vertebral and subclavian artery injuries in the other. One patient underwent an uncomplicated deformity correction with partial vertebral column resection to facilitate his deformity correction. The other patient, after her vascular injuries, ultimately suffered a spinal cord injury after a fall and underwent emergent instrumentation, decompression, and partial vertebral column resection at the site of subluxation, with improvement in her neurological function afterward.
Dystrophic scoliosis in NF1 remains a difficult disease to treat, and deformity correction in patients with subluxation is particularly complex. These cases here highlight the unpredictability and possible complications of halo-gravity traction, need for good fixation to facilitate subluxation reduction, high chance of hardware complications and proximal or distal failure, and importance of cooperative management of these patients in conjunction with other surgical services.
神经纤维瘤病1型（NF1）营养不良性脊柱侧凸是一种手术治疗颇具挑战性的疾病，其多平面弯曲进展迅速且难以预测。支具保守治疗往往效果不佳，许多患者需要进行器械融合以阻止弯曲进展。在罕见情况下，患者可出现自发性椎体半脱位，这使得这个本就复杂的疾病过程的外科治疗显著复杂化。本文目的是描述2例NF1相关营养不良性脊柱侧凸合并椎体半脱位病例及其手术矫正和临床过程。
对作者所在机构的2例病例进行回顾性分析，以描述其术前症状、手术矫正及术后过程。同时对围绕NF1相关营养不良性脊柱侧凸和半脱位的文献进行叙述性综述。
报告了2例分别发生于T4 - 5和C7 - T1的椎体半脱位病例。两名患者整个脊柱均有明显的营养不良特征，1例患者颅骨牵引失败，另1例导致椎体及锁骨下动脉损伤。1例患者通过部分脊柱切除术顺利完成畸形矫正，以利于畸形矫正。另1例患者在血管损伤后，跌倒后最终发生脊髓损伤，随后在半脱位部位进行了急诊器械置入、减压及部分脊柱切除术，术后神经功能有所改善。
NF1相关营养不良性脊柱侧凸仍是一种难以治疗的疾病，半脱位患者的畸形矫正尤为复杂。这些病例突出了颅骨牵引的不可预测性及可能的并发症、需要良好的固定以促进半脱位复位、硬件并发症及近端或远端失败的高发生率，以及与其他外科服务协同管理这些患者的重要性。
