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

The efficacy of dual growing rod technique in the treatment of early onset dystrophic scoliosis with neurofibromatosis type 1

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。

Objectives： To evaluate the clinical efficacy of dual growing rod（GR） technique in the treatment of early onset dystrophic scoliosis（EOS） with neurofibromatosis type 1（NF-1）. Methods： Eight patients （2 males, 6 females） receiving dual GR technique for EOS with NF-1 in our hospital, from May 2010 to March 2014, were retrospectively reviewed. The average age was 7.4±1.4 years old（range, 5-9 years）. The medical records of all these patients were reviewed. The parameters included age at initial surgery and the final follow-up, number and frequency of lengthening, and complications. Radiographic evaluation included scoliosis Cobb angle, thoracic kyphosis, lumbar lordosis, trunk shift, length of T1-S1, and Campbell′s space available for lung ratio（SAL）. Results： The mean follow-up was 36.9±13.5（range, 24-64） months. A total of 41 surgeries were performed, and 33 of them were lengthening procedures, with an average of 4.1 lengthenings for each patient. The mean scoliosis Cobb angle improved from 75.4°±26.4° to 31.6°±10.8° after initial surgery and was 30.9°±11.4° at the final follow-up. The average growth of T1-S1 length was 1.66±0.43cm/y. The SAL increased from 0.92±0.10 to 0.99±0.02 after initial surgery and was 1.00±0.05 at the final follow-up. Five complications occurred in 3 patients and most of them were implant-related （4 complications）. No infection and nervous system complications occurred. Conclusions： The dual GR technique is safe and effective for patients with early onset dystrophic scoliosis secondary to NF-1. It maintains correction achieved at initial surgery while allowing the growth of the spine and thoracic cage, without significant increase of total complication rate.