1型神经纤维瘤病伴脊柱侧凸肋骨头椎管内脱位的治疗策略

The treatment strategy for rib head protrusion into the spinal canal in type 1 neurofibromatosis scoliosis

中文关键词:  早发性脊柱侧凸  1型神经纤维瘤病  生长棒

英文关键词:Early onset scoliosis  Type 1 neurofibromatosis  Growing rod

中文摘要:

  【摘要】 目的：观察生长棒治疗1型神经纤维瘤病早发性脊柱侧凸的有效性。方法：回顾性分析13例生长棒治疗的1型神经纤维瘤病早发性脊柱侧凸患者的临床资料，其中男8例，女5例；年龄5~10岁（7.5±2.1岁）。随访时间2~10年（5.1±2.8年）。生长棒置入术后每6个月撑开1次。影像学测量指标包括侧凸Cobb角、后凸角、躯干高度（T1~S1），并统计手术并发症。结果：术前侧凸Cobb角69.0°±21.2°（43°~90°），末次随访25.3°±15.0°（12°~45°），矫正率63.3%。躯干高度术前323.0±29.1mm（288~355mm），末次随访400.5±25.7mm（361~430mm），躯干高度增加77.1±18.3mm，每年继续生长11.7±3.3mm。9例患者出现并发症，并发症发生率69.2%。共发生并发症16例次，包括内置物失败9例次（56.3%），交界区后凸5例次（31.3%），内置物外露1例次（6.2%），深部感染1例次（6.2%）。9例后凸角＜50°患者中6例（66.7%）出现并发症，4例后凸角≥50°患者中3例（75%）出现并发症，两组并发症发生率差异无统计学意义（χ2=0.123，P=0.726）。结论：生长棒治疗1型神经纤维瘤病早发性脊柱侧凸可有效改善脊柱畸形，并且保持躯干的继续生长；伴有严重后凸并未增加术后并发症的发生率。

英文摘要:

  【Abstract】 Objectives: To investigate the surgical outcome and complications of growing rods in the treatment of early onset scoliosis(EOS) with type 1 neurofibromatosis. Methods: Thirteen cases of EOS with type 1 neurofibromatosis treated with growing rods were studied retrospectively. There were 8 boys and 5 girls, aged from 5 to 10 years old(average, 7.5±2.1 ys). The average follow-up was 5.1±2.8 years(2-10 years). Exptending procedure was carried out every 6 months after first time internal fixation. The Cobb angle, kyphosis angle and trunk height were measured, and the complications were also evaluated. Results: The mean Cobb angle was corrected from 69.0°±21.2°(43°-90°) preoperatively to 25.3°±15.0°(12°-45°) at the final follow-up, the correction rate was 63.3%. The trunk height was corrected from 323.0±29.1mm(288-355mm) to 400.5±25.7mm(361-430mm), and spine grew an average of 11.7±3.3mm per year. There were 16 complications in 9 cases(69.2%), with implant failures in 9 cases(56.3%), junctional kyphosis in 5 cases(31.3%), and wound dehiscence and deep wound infection in 1 case(6.2%). Among 9 cases with kyphosis angle less than 50°, complication was found in 6 cases(66.7%); among 4 cases with kyphosis angle more than 50°, complication was found in 3 cases(75%). There was no significant difference of complication rate between kyphosis more than 50° group and that less than 50°group( χ2=0.123, P=0.726). Conclusions： The use of growing rods in patients with EOS associated with type 1 neurofibromatosis provides curve correction and allows the growth of spine. Kyphosis more than 50° does not increase complications.