Department of Orthopaedics, Peking Union Medical College Hospital, Beijing, China.
Orthop Surg. 2020 Dec;12(6):1923-1940. doi: 10.1111/os.12848. Epub 2020 Nov 13.
The aim of the present study was to explore the surgical treatment and prognosis of 27 cases of neurofibromatosis type 1 with severe dystrophic kyphosis.
We performed surgical treatment for scoliosis and kyphosis caused by dystrophic curves at Peking Union Medical College Hospital, Beijing, China from December 2015 to December 2017. The study included 21 patients with moderate to severe kyphosis, 12 males and 9 females, with an average age of 14.95 ± 6.05 years. All patients had kyphosis angles greater than 70° and had more than four skeletal developmental defects. A total of 6 patients with severe kyphosis, 2 males and 4 females, with an average age of 12.5 years, had more than five skeletal developmental defects with a kyphosis angle greater than 90° or a lumbar kyphosis angle greater than 40°. According to the patient's own situation, we adopted a low-grade surgery scheme (grades 1 or 2) or a high-grade surgery scheme (grades 3-6). The low-grade surgery was mainly lower articular surface resection or pontodestomy, and the high-grade surgery was mainly apical vertebral body or upper discectomy. All patients were followed up to determine their prognosis.
Statistical analysis showed that there was a significant difference in preoperative and postoperative scores between the two groups (P < 0.05), and scoliosis correction showed that surgical treatment had a significant effect on scoliosis kyphosis. The mean follow-up time was 66.7 months. Follow-up results showed that 50% of complications after internal fixation were related to high-level surgery. Complications included displacement of the titanium cage, removal of the lamina hook, formation of pseudoarthrosis, and internal fixation failure (with a rate of 7.7%-14.3%). In contrast, there were no associated symptoms for low-grade surgery. In addition, the results showed that gender, age, extent of resection, height, and body mass index had no significant effect on preoperative, postoperative, and prognostic indicators of patients (P > 0.05).
Early identification of dysplastic scoliosis-related deformities plays an important role in surgical planning and prognosis, and low-level surgical procedures are more favorable for patients' prognosis.
本研究旨在探讨 27 例 1 型神经纤维瘤病严重骨营养不良性后凸的手术治疗及预后。
2015 年 12 月至 2017 年 12 月，我们在我院对由骨营养不良曲线引起的脊柱侧凸和后凸进行手术治疗。研究共纳入 21 例中重度后凸患者，男 12 例，女 9 例，平均年龄 14.95±6.05 岁。所有患者的后凸角均大于 70°，且均存在 4 个以上的骨骼发育缺陷。6 例重度后凸患者，男 2 例，女 4 例，平均年龄 12.5 岁，均存在 5 个以上骨骼发育缺陷，后凸角大于 90°或腰椎后凸角大于 40°。根据患者自身情况，采用低级别手术方案（1 或 2 级）或高级别手术方案（3-6 级）。低级别手术主要为下关节面切除或 pontodestomy，高级别手术主要为顶椎椎体或上位椎间盘切除术。所有患者均进行随访以确定其预后。
统计学分析显示，两组患者术前和术后评分均有显著差异（P<0.05），且脊柱侧凸矫正显示手术治疗对脊柱后凸有显著效果。平均随访时间为 66.7 个月。随访结果显示，50%的内固定相关并发症与高级别手术有关。并发症包括钛笼移位、椎板钩取出、假关节形成和内固定失败（发生率为 7.7%-14.3%）。相比之下，低级别手术无相关症状。此外，结果表明，性别、年龄、切除范围、身高和体重指数对患者术前、术后和预后指标均无显著影响（P>0.05）。
早期识别与骨营养不良性脊柱侧凸相关的畸形对于手术规划和预后具有重要意义，且低级别手术程序更有利于患者的预后。
