Hashimoto Shogo, Suzuki Satoshi, Takeda Kazuki, Iga Takahito, Okubo Toshiki, Ozaki Masahiro, Tsuji Osahiko, Nagoshi Narihito, Matsumoto Morio, Nakamura Masaya, Watanabe Kota
Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan; Department of Orthopaedic Surgery, Ohashi Hospital, Toho University, 2-22-36 Ohashi, Meguro-ku, Tokyo, 153-8515, Japan.
J Orthop Sci. 2025 Aug 28. doi: 10.1016/j.jos.2025.08.002.
Surgical strategies for spinal deformities associated with neurofibromatosis type 1(NF-1) have been still controversial due to the wide variety of pathologies, particularly, for deformities with severe dystrophic changes in pediatric patients remained challenging with high complication and reoperation rates. The purpose of this study was to validate our surgical strategies by evaluating clinical and radiographical outcomes.
We retrospectively evaluated 22 cases who underwent correction surgeries for spinal deformities in our institute. The investigations included patient background, presence of dystrophic changes and paravertebral tumors, surgical procedure, number of surgeries, complications and reoperation, and radiographical parameters including Cobb angle and T1-S1 length.
The mean age at the first surgery was 11.6 ± 5.5 years. The mean follows up period was 84.3 ± 50.2 months. Of the 22 cases, 4 cases had cervical kyphosis and 18 cases had deformity in thoracic and/or lumbar spine. Dystrophic changes of spine were observed in all 4 cases with cervical kyphosis and 15 cases with spinal deformity in thoracic and/or lumbar spine. All cases with cervical kyphosis underwent posteroanterior fusion following halo gravity traction preoperatively. The mean local kyphosis of cervical spine improved from 83.7 ± 5.9° to 16.9 ± 2.4° after surgery and 19.4 ± 2.5° at final follow up, and 2 cases required unplanned surgery due to pseudarthrosis. In cases of deformity in thoracic and/or lumbar spine, 11 cases underwent posteroanterior fusion, 4 cases underwent only posterior fusion, and 3 cases underwent conventional growing-rod, of which 4 cases underwent halo gravity traction before correction surgery. The mean Cobb angle of scoliosis in thoracic and/or lumbar spine improved from 73.2 ± 16.9° to 30.9 ± 15.8° after surgery and 29.5 ± 13.3° at final follow up, and that of T1-S1 growth increased from 338.3 ± 13.3 mm to 406.3 ± 8.9 mm at final follow up. Four cases required unplanned surgery due to pseudarthrosis (n = 2) or progression of deformity (n = 2).
The fundamental of surgical treatment for NF-1-related spinal deformity is extensive rigid spinal fusion by adequate bone grafting. Even if these surgeries are performed, in some cases additional surgery could be necessary due to progression of deformity.
Level III, retrospective cohort study.
由于病理类型多样，1型神经纤维瘤病（NF-1）相关脊柱畸形的手术策略仍存在争议，尤其是对于小儿患者中伴有严重营养不良性改变的畸形，其并发症和再次手术率高，治疗仍具有挑战性。本研究的目的是通过评估临床和影像学结果来验证我们的手术策略。
我们回顾性评估了在我院接受脊柱畸形矫正手术的22例患者。调查内容包括患者背景、是否存在营养不良性改变和椎旁肿瘤、手术过程、手术次数、并发症和再次手术情况，以及影像学参数，包括Cobb角和T1-S1长度。
首次手术时的平均年龄为11.6±5.5岁。平均随访时间为84.3±50.2个月。22例患者中，4例有颈椎后凸，18例有胸段和/或腰段脊柱畸形。所有4例颈椎后凸患者以及15例胸段和/或腰段脊柱畸形患者均观察到脊柱营养不良性改变。所有颈椎后凸患者术前均在头环重力牵引后行前后路融合术。颈椎局部后凸平均角度从术前的83.7±5.9°改善至术后的16.9±2.4°，末次随访时为19.4±2.5°，2例因假关节形成需要再次手术。对于胸段和/或腰段脊柱畸形患者，11例行前后路融合术，4例仅行后路融合术，3例行传统生长棒手术，其中4例在矫正手术前行头环重力牵引。胸段和/或腰段脊柱侧弯的平均Cobb角从术前的73.2±16.9°改善至术后的30.9±15.8°，末次随访时为29.5±13.3°，T1-S1长度在末次随访时从338.3±13.3mm增加至406.3±8.9mm。4例因假关节形成（2例）或畸形进展（2例）需要再次手术。
NF-1相关脊柱畸形手术治疗的基本原则是通过充分植骨进行广泛的坚强脊柱融合。即使进行了这些手术，在某些情况下，由于畸形进展可能仍需要再次手术。
III级，回顾性队列研究。
