Wang Jingcheng, Liu Congcong, Wang Cheng, Li Jing, Lv Guohua, A Jiancuo, Deng Youwen, Wang Wenjun
Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China.
Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China; Department of Spine Surgery, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China.
World Neurosurg. 2019 Jul;127:e1190-e1200. doi: 10.1016/j.wneu.2019.04.096. Epub 2019 Apr 17.
To evaluate the early and midterm outcomes of surgical correction for severe dystrophic cervical kyphosis in patients with neurofibromatosis type 1 (NF-1) and analyze the pathomechanics and the influence on surgical efficacy of related systemic skeletal dystrophy.
Ten patients who underwent surgical correction for NF-1-related severe dystrophic cervical kyphosis were reviewed. Radiographic parameters, including local and global Cobb angle, sagittal vertical axis, and T-1 slope, were measured. The visual analog scale score, Japanese Orthopaedic Association score, Neck Disability Index, Patient Satisfaction Index, and complications were evaluated.
The average follow-up was 50.6 months. The local and global Cobb angle improved from the preoperative average of 82.0° and 54.9° to an average of 35.6° and 29.8°, respectively, at the time of final follow-up. The C2-7 sagittal vertical axis averaged 5.8 mm before surgery and 8.9 mm at the final follow-up. The average T1 slope was -12.3° before surgery and -1.6° at the final follow-up. The visual analog scale score, Japanese Orthopaedic Association score, and Neck Disability Index improved significantly, and the overall satisfaction rate was 90.0%. One death and 4 instrumentation failures occurred, 3 patients showed progression of the kyphosis, and 2 fusion failures were observed.
Surgical correction, specifically the combined anteroposterior procedure, is essential and effective for management of NF-1-related severe dystrophic cervical kyphosis. However, high incidences of instrumentation failure, kyphosis progression, and fusion failure were observed. NF-1-related continuous skeletal dystrophy caused by multiple metabolic factors remarkably affected the midterm outcomes. Early prevention and targeted pharmacotherapy may be necessary.
评估1型神经纤维瘤病（NF-1）患者严重营养不良性颈椎后凸畸形手术矫正的早期和中期结果，并分析相关全身骨骼营养不良的病理力学及其对手术疗效的影响。
回顾10例行NF-1相关严重营养不良性颈椎后凸畸形手术矫正的患者。测量影像学参数，包括局部和整体Cobb角、矢状垂直轴和T1斜率。评估视觉模拟量表评分、日本骨科协会评分、颈部功能障碍指数、患者满意度指数及并发症情况。
平均随访50.6个月。末次随访时，局部和整体Cobb角分别从术前平均82.0°和54.9°改善至平均35.6°和29.8°。C2-7矢状垂直轴术前平均为5.8 mm，末次随访时为8.9 mm。平均T1斜率术前为-12.3°，末次随访时为-1.6°。视觉模拟量表评分、日本骨科协会评分和颈部功能障碍指数显著改善，总体满意率为90.0%。发生1例死亡和4例内固定失败，3例患者后凸畸形进展，观察到2例融合失败。
手术矫正，尤其是前后联合手术，对于NF-1相关严重营养不良性颈椎后凸畸形的治疗至关重要且有效。然而，观察到内固定失败、后凸畸形进展和融合失败的发生率较高。多种代谢因素导致的NF-1相关持续性骨骼营养不良显著影响中期结果。早期预防和靶向药物治疗可能是必要的。
