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

中文关键词:  1型神经纤维瘤病  早发性脊柱侧凸  营养不良型  双生长棒技术  非融合技术  并发症

英文关键词:Neurofibromatosis type 1  Early onset scoliosis  Dystrophic  Dual growing rod technique  Fusionless technique  Complications

中文摘要:

  【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。

英文摘要:

  【Abstract】 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.