先天性脊柱侧凸合并脊髓纵裂的临床特点及手术疗效

Clinical features of split spinal cord malformations in congenital scoliosis of 69 surgical cases

中文关键词:  先天性脊柱侧凸  脊髓纵裂  手术并发症

英文关键词:Congenital scoliosis  Split spinal cord malformations  Surgery complication

中文摘要:

  【摘要】 目的：研究手术治疗先天性脊柱侧凸合并脊髓纵裂的患者的临床特点，评估其手术疗效及并发症发生情况。方法：回顾性分析2005年3月~2017年3月间我院收治并行手术治疗的先天性脊柱侧凸合并脊髓纵裂患者69例，其中女性41例，男性28例，平均年龄13.9±4.5岁（7~34岁）。所有患者术前均行全脊柱正侧位X线、CT及MRI检查，术后即刻及末次随访行全脊柱正侧位X线检查，测量影像学参数（主弯Cobb角、次弯Cobb角、主弯顶椎偏距、躯干偏移、胸椎后凸角及腰椎前凸角），分析先天性脊柱侧凸合并脊髓纵裂的影像学特点及临床表现，并评估脊柱侧凸矫形率及相关并发症[矫形率=（术前Cobb角-术后即刻Cobb角）/术前Cobb角]。结果：在69例脊髓纵裂患者中，单纯膜性纵裂50例，骨性纵裂4例，膜性合并骨性纵裂15例。合并椎板畸形38例，半椎体24例，肋骨畸形25例，37例同时存在其他椎管内畸形，椎管外畸形8例。临床表现主要有：背部毛发10例，跛行6例，腰背痛4例，截瘫2例。双下肢/双足异常8例，神经系统阳性体征20例。脊髓纵裂好发于下胸段及腰段，占72.4%（50/69），纵裂累及椎体节段平均为4.2±2.7个。所有患者中，1例在矫形前行骨嵴切除，余均未对纵裂做预防性切除，仅单纯行侧凸矫形内固定术。60例得到随访，随访率为86.9%。平均随访时间32.4±22.7个月（13~115个月）。术前主弯Cobb角平均为71.8°±29.4°，次弯Cobb角为46.4°±17.3°，胸椎后凸角为39.5°±36.1°，腰椎前凸角为50.4°±17.3°；主弯顶椎偏距为6.2±3.6cm，躯干偏移平均为2.8±3.0cm。术后即刻主弯Cobb角28.8°±21.6°，次弯Cobb角25.6°±14.5°，胸椎后凸角25.5°±19.1°，腰椎前凸角42.3°±15.4°；主弯顶椎偏距4.2±3.3cm，躯干偏移2.4±2.8cm，主弯顶椎旋转度所有患者术后即刻冠状位主弯矫形率为（59.9±22.0）%，末次随访时矫形率为（53.6±25.7）%。术后即刻与术前相比，主弯Cobb角、次弯Cobb角、胸椎后凸角、腰椎前凸角及主弯顶椎偏距均有明显统计学差异（P<0.01），主弯顶椎旋转度及躯干偏移无明显统计学差异。末次随访时主弯Cobb角平均为33.3°±25.9°，次弯Cobb角为27.1°±16.9°，胸椎后凸角为25.1°±16.1°，腰椎前凸角为45.6°±17.6°；主弯顶椎偏距为4.9±6.0cm，躯干偏移平均为2.1±2.0cm，末次随访与术后相比，均无明显统计学差异（P>0.05）。术后共6例出现神经系统并发症，发生率为8.7%，均为不完全神经损伤，无截瘫发生。内固定相关并发症3例，包括螺钉松动2例，内固定棒断裂1例。2例螺钉松动患者无任何临床症状，予以随访观察；1例内固定棒断裂患者手术翻修，未再次出现并发症。结论：先天性脊柱侧凸合并SCM手术治疗患者纵裂好发于下胸段及腰段，椎体畸形以混合型最多见；中下胸段肋骨畸形的伴发率最高。

英文摘要:

  【Abstract】 Objectives: To analyze clinical features, radiological findings and surgical outcomes of the patients with spine cord malformation(SCM) in congenital scoliosis(CS). Methods: A total of 69 patients with SCM was operated at our centre between March 2005 and March 2017. The mean age of the patients was 13.9 years and the female to male ratio was 1.46∶1. Patients′ demographic profile, radiological and operative details, complications and surgical outcomes were evaluated retrospectively. Results: Four patients had type ⅠSCM and 50 had type Ⅱ SCM. Fifteen patients had type Ⅰ and Ⅱ SCM concurrently. The dorsolumbar and lumbar regions were the most common sites for SCM. Asymmetric weakness of the lower limbs and neural axis abnormalities were presented in 10 cases. 37 patients had other intraspinal abnormalities(53.6%), including syringomyelia in 18(26.1%), tethered cord in 3(4.3%), low conus in 16(23.2%). 38 patients presented with lamina deformities(55.1%), 24 with hemivertebra(34.8%) and 25 with rib anomalies(36.2%). In total, the follow-up rate was 86.9%(60/69). The mean time of follow-up was 32.4±22.7 months(13-115 months). The average main curve, compensatory curve, thoracic kyphosis, lumbar lordosis, AVR, AVT and TS were 71.8°±29.4°，46.4°±17.3°, 39.5°±36.1°, 50.4°±17.3°, 2.1°±1.0°, 6.2±3.6cm and 2.8±3.0cm preoperatively, which improved to 28.8°±21.6°, 25.6°±14.5°, 25.5°±19.1°, 42.3°±15.4°, 1.9°±1.0°, 4.2±3.3cm and 2.4±2.8cm postoperatively. At the final follow-up, the average main curve, compensatory curve, thoracic kyphosis, lumbar lordosis, AVR, AVT and TS were 33.3°±25.9°, 27.1°±16.9°, 25.1°±16.1°, 45.6°±17.6°, 1.8°±0.9°, 4.9±6.0cm and 2.1±2.0cm. The correction rate of main curve was 59.9% and 53.3% at post-operation and follow-up period. Compared with the preoperative ones, there were significant difference in main curve, compensatory curve, AVT, thoracic kyphosis and lumbar lordosis at post-operation(P<0.01). Compared to the post-operation, there were no significant differences in terms of main curve, compensatory curve, AVT, AVR, TS, thoracic kyphosis and lumbar lordosis at the final follow-up(P>0.05). The complication rate in neural axis was 8.7%(6/69). The incidence of implants related complication was 4.3%(3/69), including 2 pedicle screw loose and 1 rod breakage. The patient with rod fracture was performed by the revision surgery. In view of no symptom, the patients with screw loose were observed at the follow-up period. Conclusions: The dorsolumbar and lumbar regions are the most common sites for SCM. Mixed type is the most common vertebral deformity. The incidence of rib anomaly is the highest in T5-T12 segments(middle and low thoracic region).