分期手术治疗先天性脊柱侧凸合并脊髓纵裂和脊髓拴系的安全性与近期疗效

Safety and efficacy of 2-staged surgery for congenital scoliosis combined with split spinal cord malformation and tethered cord

目的：评估分期手术治疗先天性脊柱侧凸合并脊髓纵裂和脊髓拴系患者的安全性与近期疗效。方法 ：回顾性分析我院2009年1月-2014年1月收治的66例先天性脊柱侧凸合并脊髓纵裂和脊髓拴系患者的临床资料。其中男20例,女46例,年龄17.2±4.5岁（7-26岁）。5例患者术前存在下肢神经功能障碍。脊柱侧凸冠状位主弯Cobb角97.6°±23.5°（50°-165°）,主弯位于颈胸段1例,胸段58例,胸腰段7例。23例同时存在矢状位后凸畸形,Cobb角89.5°±13.9°（47°-165°）。伴有Ⅰ型脊髓纵裂45例,Ⅱ型脊髓纵裂21例,均合并脊髓拴系。所有患者均行分期手术治疗：Ⅰ型脊髓纵裂组患者一期切除骨性分隔、松解脊髓拴系,Ⅱ型脊髓纵裂组患者一期松解脊髓拴系;一期术后3-4周,二期行侧凸矫形手术。结果：一期手术时间208.7±107.2min（60-505min）,术中出血量297.1±192.6ml（20-2000ml）;二期手术时间392.6±150.7min（196-600min）,术中出血量2158.8±1158.4ml（450-6000ml）。术前存在下肢神经功能障碍的5例患者中,1例术后下肢肌力提高2级,感觉功能有所恢复;其余4例术后神经功能无明显变化。2例术前神经功能正常患者二期术中出现脊髓损伤（3.0%,2/66）,其中1例术后双下肢肌力降为4级,感觉稍减退,术后1周感觉、运动功能完全恢复正常;另1例术后双下肢肌力降低至2级,双下肢及会阴部感觉减退,经脱水、激素冲击治疗及高压氧治疗,术后1个月双下肢肌力恢复至3级,术后2年随访时双下肢肌力恢复至4级,双下肢残留轻度麻木感,大小便功能正常。4例（6.1%,4/66）患者二期术后并发胸腔积液。均获得随访,随访时间12.4±3.5个月（6-24个月）。随访期间未发现椎弓根螺钉松动及断裂现象。脊柱侧凸矫形术后冠状位Cobb角为41.6°±17.8°（12°-107°）,矫正率为（61.3±14.3）%;末次随访时冠状位Cobb角为43.7°±16.6°（15°-108°）,丢失率为（1.9±1.1）%。术后矢状位后凸Cobb角为38.4°±11.0°（2°-78°）,矫正率为（67.6±23.4）%,末次随访时矢状位Cobb角为39.7±11.2°（3°-87°）,丢失率为（2.3±1.3）%。结论：分期手术治疗合并脊髓纵裂和脊髓拴系的先天性脊柱侧凸具有较高的手术安全性,并可获得较满意的矫形效果。

Objectives： To evaluate the safety and short term outcome of 2-staged surgery for congenital scoliosis combined with split spinal cord malformation（SSCM） and tethered cord. Methods： Data of 66 patients, who had undergone surgical treatment for congenital scoliosis combined with SSCM and tethered cord from January 2009 to January 2014 in our hospital, were retrospectively analyzed. 20 of these patients were males and 46 were females with an average age of 17.2±4.5 years（range, 7-26 years）. The mean major coronal Cobb angle ranged from 50° to 165°（average, 97.6°±23.5°）. 23 patients were also accompanied by kyphosis with the Cobb angle ranging from 47° to 165°（average, 89.5°±13.9°）. There were 45 patients with type I SSCM and 21 patients with type II SSCM, all patients were combined with tethered cord. All the surgeries were operated in 2 stages. At the first surgery, bony spur was resected and tetherd cord was released for the patient with type I SSCM, and only tetherd cord was released for the patient with type II SSCM. 3-4 weeks after the first surgery, the scoliosis was corrected. Results： All the patients were followed up for 6 months to 24 months（average, 12.4±3.5 months）. The average operation time was 208.7＋107.2min（range, 60- 505min） and the average blood loss was 297.1＋192.6ml（range, 20-2000ml） for the first surgery. The average operation time was 392.6±150.7min （range, 196-600min） and the average blood loss was 2158.8＋1158.4ml （range, 450-6000ml） for the second surgery. There were 2 patients（3.0%, 2/66） experiencing neurological de- terioration at the second surgery, 4 patients（6.1%, 4/66） had pleural effusion after the second surgery and no patient presented infection. There was no instrument failure at final follow-up. The average coronal Cobb an- gle after surgery was 41.6°＋17.8°（range, 12°-107°）, the average correction rate was （61.3±14.3）%. The average coronal Cobb angle at final follow-up was 43.7°±16.6°（range, 15°-108°）, the average lost rate was （1.9° 1.1）%. The average kyphosis Cobb angle after surgery was 38.4°±11.0°（range, 2°-78°）, the average correction rate was （67.6±23.4）%. The average kyphosis Cobb angle at final follow-up was 39.7°±11.2°（range, 3°-87°）, the average lost rate was （2.3±1.3）%. Conclusions： Two-staged surgery is safe and effective for congenital scoliosis combined with SSCM and tethered cord.