Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham, UK,
Eur Spine J. 2014 Apr;23 Suppl 1(Suppl 1):S72-5. doi: 10.1007/s00586-014-3170-9. Epub 2014 Feb 1.
Rett syndrome is a progressive neurodevelopmental disorder that predominantly affects females and is associated with a high incidence of scoliosis and epilepsy. There is scant published work about intraoperative spinal cord monitoring in these patients and little more regarding the rate of perioperative complications. We investigated our institutions' experience with both.
We retrospectively reviewed the records of 11 patients with Rett syndrome who underwent surgical correction of scoliosis at our institution between 2004 and 2010.
Eleven patients underwent successful correction of their scoliosis at an average age of 12. Eight of the patients suffered one or more significant complications. The average curve was corrected from 71° to 27°. Successful spinal cord monitoring was achieved in eight of the nine patients where it was attempted. No patient suffered any neurological complications. Average inpatient stay was 18.2 days.
Scoliosis surgery in patients with Rett syndrome carries a very high rate of complications and an average hospital stay approaching 3 weeks. Both caregivers and surgeons should be aware of this when planning any intervention. These patients frequently have useful lower limb function and spinal cord monitoring is a valid tool to aid in its preservation. We would suggest aggressive optimisation of these patients prior to surgery, with an emphasis on nutrition.
雷特综合征是一种进行性神经发育障碍，主要影响女性，与脊柱侧凸和癫痫的高发病率有关。关于这些患者的术中脊髓监测，发表的文献很少，关于围手术期并发症的发生率也知之甚少。我们调查了我们机构在这两方面的经验。
我们回顾性地分析了 2004 年至 2010 年间在我们机构接受脊柱侧凸手术矫正的 11 例雷特综合征患者的记录。
11 例患者平均年龄 12 岁时成功矫正了脊柱侧凸。8 例患者发生 1 次或多次严重并发症。平均曲线从 71°矫正至 27°。在尝试进行脊髓监测的 9 例患者中，有 8 例成功。无患者发生任何神经并发症。平均住院时间为 18.2 天。
雷特综合征患者的脊柱侧凸手术并发症发生率非常高，平均住院时间接近 3 周。在计划任何干预措施时，护理人员和外科医生都应该意识到这一点。这些患者通常下肢功能良好，脊髓监测是保护其功能的有效工具。我们建议在手术前对这些患者进行积极的优化，重点是营养。
