Chiari畸形Ⅰ型伴脊柱侧凸患者影像学特征的自然史:一项横断面研究

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目的:探讨不同年龄段Chiari畸形Ⅰ型(Chiari malformation typeⅠ, CMI)伴脊柱侧凸患者影像学特征的自然史。方法:回顾性分析2010年6月至2018年6月诊断为CMI伴脊柱侧凸80例患者的病历资料,根据年龄分为儿童组(≤ 10岁)、青少年组(11~18岁)和成人组(≥19岁)。将脊柱侧凸弯型分为典型弯和非典型弯,记录各组患者的脊柱侧凸弯型分布情况;测量冠状位和矢状位影像学参数,包括侧凸Cobb角、顶椎偏距、冠状面平衡、胸椎后凸角、腰椎前凸角、骨盆倾斜角、骨盆入射角、骶骨倾斜角、矢状面平衡,以及在MRI上测量患者小脑扁桃体下疝程度及脊髓空洞的类型。三组间各参数进行统计学分析。结果:儿童组(10例)、青少年组(44例)和成人组(26例)典型弯发生率分别为70.0%、、84.1%和50.0%(χn 2=14.766,n P=0.141),非典型弯为30.0%、15.9%和50.0%(χn 2=2.654,n P=0.265),三组CMI患者脊柱侧凸弯型分布的差异无统计学意义。冠状位参数中主弯Cobb角(n F=16.751,n P< 0.001)、主弯柔韧性(n F=3.285,n P=0.044)、次弯Cobb角(n F=9.805,n P<0.001)和冠状面平衡(n F=5.249,n P=0.007)的差异均有统计学意义,两两比较结果显示随着年龄增长,主弯和次弯Cobb角亦增加,主弯的柔韧性下降;三组间冠状面平衡的差异无统计学意义,但青少年组冠状面平衡数值低于其他两组。矢状位参数中胸椎后凸角(n F=4.324,n P=0.017)、腰椎前凸角(n F=4.590,n P=0.013)、骨盆入射角(n F=5.501,n P=0.006)和骨盆倾斜角(n F=3.220,n P=0.045)三组间的差异有统计学意义,两两比较结果显示随着年龄的增长,各矢状位参数均明显增加,侧凸畸形加重。MRI显示越年轻的患者,高级别的小脑扁桃体下疝(χn 2=18.479,n P< 0.001)和膨胀型脊髓空洞(χn 2=23.074,,n P=0.003)的比例越高。n 结论:Chiari畸形Ⅰ型患者随着年龄增长,主弯Cobb角明显进展而柔韧性降低,提示应早期进行手术干预以降低手术风险。年长患者小脑扁桃体下疝和脊髓空洞程度优于年轻患者,提示可能存在自发性缓解。“,”Objective:To investigate the radiological presentations in relation to different ages in scoliosis associated with Chiari malformation typeⅠ(CMI).Methods:A retrospective analysis was performed on 80 patients diagnosed with scoliosis associated with CMI from June 2010 to June 2018, who were classified into three groups according to their age: Children(age≤10 years), Adolescents (age 11-18 years) and Adults (age≥19 years). Curves were classified into typical and atypical patterns in the coronal plane. The coronal and sagittal radiographical parameters were measured in the three groups. Moreover, cerebellar tonsillar descent and syringomyelia patterns were measured on MRI, and the parameters among the three groups were compared statistically.Results:The incidence of atypical curve patterns in Children (10 patients), Adolescents (44 patients) and Adults (26 patients) was 30.0%, 15.9%, and 50.0%, respectively (χn 2=2.654, n P=0.265). There was no statistical difference in the distribution of curve patterns among CMI patients with different age. In the coronal profile, Cobb angle (n F=16.751, n P<0.001) and flexibility (n F=3.285, n P=0.044) of main curve, Cobb angle of secondary curve (n F=9.805, n P<0.001) and coronal balance(CB) (n F=5.249, n P=0.007) showed statistical difference. The elderly patients tended to have larger Cobb angle of main and secondary curve with worse flexibility of main curve, and CB in Adolescents was better than the other two groups. In the sagittal profile, TK (n F=4.324, n P=0.017), LL (n F=4.590, n P=0.013), PI (n F=5.501, n P=0.006), and PT (n F=3.220, n P=0.045) showed statistical difference in the three groups, which were increasing significantly with aging. MRI parameters showed that younger patients were more likely to have a higher degree of cerebellar tonsillar descent (χn 2=18.479, n P<0.001) and distended syringomyelia (χn 2=23.074, n P=0.003).n Conclusion:With aging, Cobb angle of main curve is progressive, and the flexibility is worse, suggesting that early surgical intervention should be performed to reduce the risk of surgery. In addition, cerebellar tonsillar descent and syringomyelia in elderly patients are milder than young patients, indicating that there might be spontaneous remission.
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