双生长棒技术治疗早发性1型神经纤维瘤病营养不良型脊柱侧凸的疗效分析

发布时间：2018-09-04 11:12

【摘要】：目的:评估双生长棒技术治疗早发性1型神经纤维瘤病营养不良型脊柱侧凸的临床疗效。方法:回顾性研究2010年5月~2014年3月于我科接受双生长棒技术治疗的8例早发性1型神经纤维瘤病营养不良型脊柱侧凸患者(男2例,女6例),平均年龄为7.4±1.4岁(5~9岁)。通过复习病历,对患者年龄、撑开次数及并发症进行记录;同时对影像学资料进行测量分析。测量指标包括侧凸主弯Cobb角、胸后凸、腰前凸、T1-S1长度,对畸形的矫正情况以及脊柱、胸廓的生长指标(Campbell′s space available for lung ratio,SAL)进行评估。结果:所有患者平均随访时间为36.9±13.5个月(24~64个月)。8例患者共接受41次手术,其中33次为撑开手术,平均每位患者经历4.1次撑开手术。冠状面主弯Cobb角术前为75.4°±26.4°,术后矫正至31.6°±10.8°,末次随访时为30.9°±11.4°。T1-S1长度从术前的30.0±4.4cm增至术后的35.1±4.0cm,末次随访时为39.9±3.5cm,平均年增长率为1.66±0.43cm/y。术前SAL为0.92±0.10,术后改善至0.99±0.02,末次随访时为1.00±0.05。有3例患者发生了5例次并发症,主要为内固定相关并发症(4例次);无感染以及神经系统并发症发生。结论:双生长棒技术治疗早发性1型神经纤维瘤病营养不良型脊柱侧凸安全有效,可有效地控制脊柱畸形的进展,保留脊柱的生长潜能,同时可以在一定程度上矫正患者的胸廓畸形,总体并发症发生率并无明显增高。
[Abstract]:Objective: to evaluate the clinical efficacy of dual growth rod technique in the treatment of early onset neurofibromatosis with dystrophy. Methods: from May 2010 to March 2014, 8 patients (male 2, female 6) with early onset neurofibromatosis dystrophy received double growth rod technique in our department. The average age was 7.4 卤1.4 years (59 years). The patient's age, times of distraction and complications were recorded by reviewing the medical records, and the imaging data were measured and analyzed. The measurements included Cobb angle of main curvature of scoliosis, thoracic kyphosis, length of T1-S1 of lumbar kyphosis, and evaluation of correction of deformity and growth index of spine and thorax (Campbell's space available for lung ratio,SAL). Results: the mean follow-up time of all patients was 36.9 卤13.5 months (24 ~ 64 months). There were 41 surgeries, 33 of which were open surgery, and the average patient underwent 4.1 open surgery. The Cobb angle of the main coronal bending was 75.4 掳卤26.4 掳before operation and 31.6 掳卤10.8 掳after operation. The length of T1-S1 increased from 30.0 卤11.4 掳路T1-S1 to 35.1 卤4.0 cm at the last follow-up, and 39.9 卤3.5 cm at the last follow-up, with an average annual growth rate of 1.66 卤0.43 cm / y. SAL was 0.92 卤0.10 before operation, improved to 0.99 卤0.02 after operation, and 1.00 卤0.05 at last follow-up. There were 5 complications in 3 cases, mainly internal fixation related complications (4 cases), no infection and nervous system complications. Conclusion: double growth rod technique is safe and effective in the treatment of early onset neurofibromatosis with dystrophy, which can effectively control the progression of spinal deformity and preserve the growth potential of spinal column. At the same time, the thoracic deformity can be corrected to a certain extent, and the overall incidence of complications is not significantly increased.
【作者单位】：北京协和医学院北京协和医院骨科;
【基金】：国家自然科学基金面上项目(No.81672123)
【分类号】：R687.3

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