Tanaka Masato, Nakanishi Kazuo, Sugimoto Yoshihisa, Misawa Haruo, Takigawa Tomoyuki, Nishida Keiichiro, Ozaki Toshifumi
Department of Orthopaedic Sugery, Okayama University Hospital, Okayama, Japan.
Acta Med Okayama. 2009 Dec;63(6):373-7. doi: 10.18926/AMO/31823.
Scoliosis is a common clinical manifestation of Rett syndrome, a neurodevelopmental disorder that almost exclusively affects females. The spinal curve in patients with Rett syndrome is typically a long C curve of a neuromuscular type. As the onset of the scoliosis is very early and shows rapid progression, early surgical intervention has been recommended to prevent a life-threatening collapsing spine syndrome. However, there are high perioperative risks in Rett syndrome patients who undergo spinal surgery, such as neurological compromise and respiratory dysfunction due to rigid spinal curve. We herein report the surgical result of treating severe rapid progressive thoracic scoliosis in a 16-year-old girl with Rett syndrome. Posterior segmental pedicle screw fixation was performed from T1 to L3 using a computer-assisted technique. Post-operative radiography demonstrated a good correction of the curve in both the sagittal and coronal alignment. There were no postoperative complications such as neurological compromise. The patient had maintained satisfactory spinal balance as of the 3-year follow-up examination.
脊柱侧弯是雷特综合征的常见临床表现，雷特综合征是一种几乎仅影响女性的神经发育障碍疾病。雷特综合征患者的脊柱侧弯通常是神经肌肉型的长C形曲线。由于脊柱侧弯发病非常早且进展迅速，因此建议早期进行手术干预，以预防危及生命的脊柱塌陷综合征。然而，接受脊柱手术的雷特综合征患者围手术期风险很高，例如由于僵硬的脊柱侧弯导致神经功能受损和呼吸功能障碍。我们在此报告一名16岁雷特综合征女孩严重快速进展性胸椎侧弯的手术治疗结果。采用计算机辅助技术从T1至L3进行后路节段性椎弓根螺钉固定。术后X线片显示矢状面和冠状面的曲线均得到良好矫正。没有出现如神经功能受损等术后并发症。截至3年随访检查，患者保持了满意的脊柱平衡。
