后路截骨减压治疗软骨发育不全所致脊柱畸形

Posterior osteotomy and decompression for spinal deformity in patients with achondroplasia

目的 探讨软骨发育不全所致脊柱畸形的手术治疗方法.方法 软骨发育不全所致脊柱畸形6例,男3例,女3例;年龄12～36岁,平均年龄18.5岁.均表现为胸腰段后凸畸形伴腰椎管狭窄,其中,4例患者合并神经系统症状.均行后路截骨矫形、椎管减压术.所有病例术前、术后和随访时拍摄站立位全脊柱X线,测量矢状面局部后凸Cobb角;通过复习病例,记录围手术期并发症;通过JOA评分评价术前术后神经系统症状的改善情况.结果 全部病例随访5～24个月,平均10.2个月.手术时间160～460 min,平均320 min.术中出血量400～3000 ml,平均1300 ml.固定融合节段5～10节,平均8节.矢状面局部后凸Cobb角由术前平均53.3°矫正至13.3°,矫正率75.0%;4例合并神经系统损伤的患者术前JOA平均4.5分,随访时平均8.5分.结论 对于软骨发育不全所致胸腰段脊柱后凸和腰椎管狭窄,后路截骨矫形、椎管减压术可取得良好的治疗效果.

Objective To evaluate the outcome of surgical treatment for spinal deformity in patients with achondroplasia. Methods Six consecutive cases of 3 males and 3 females were recruited. The average age at surgery was 18.5 years old with a range of 12 to 36 years old. All patients suffered thoracolumbar kyphosis and lumbar stenosis. Four patients had neurological deficits due to severe spinal deformity.Posterior osteotomy and decompression were performed in all cases. Long cassette supine radiographs were taken before and after surgery and at the final follow-up. Correction of kyphosis, neurological outcomes （JOA （Japanese Orthopedic Association） scores ） and operative complications were recorded and analyzed.Results The average follow-up was 10. 2 months with a range of 5 to 24 months. The average operative duration was 320 min with a mean 1300 ml of blood loss. The average number of fusion segments was 8 with a range of 5 to 10 segments. Kyphosis was corrected from 53.3° to 13. 3° with a 75.0% correction rate. The average preoperative JOA score was 4. 5 points and improved to 8. 5 points at the final follow-up. Conclusion Posterior osteotomy and decompression is a preferred surgical regiment for thoracolumbar kyphosis and lumbar stenosis in patients with achondroplasia.