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目的探讨Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)伴脊柱侧凸患者胸椎椎弓根分型的特点。方法 2005年7月至2012年3月,我院收治的100例NF1伴脊柱侧凸者采用螺旋CT行胸椎连续扫描。在Lenke分型基础上对其进一步细化,将椎弓根形态分型为:A型(正常型);B型(狭窄型);C型(峡部硬化型);D型(完全硬化型);E型(椎弓根缺如型)。A、B、c、D、E型胸椎椎弓根分别为911个、561个、334个、422个和172个,共2400个。按照上述分型标准,在图像编档和通信系统(PACS)上逐层阅片,选择胸椎椎弓根显示最清楚的层面对椎弓根横径进行测量并分型,分别统计不同组别的NF1伴脊柱侧凸(scoliosis secondary to neurofibromatosis type 1,NF1-S)患者胸椎椎弓根类型发生率,并对其畸形率进行统计学分析。结果 100例NF1-S中,畸形率(B~E)为62%;其中非萎缩性脊柱侧凸15例,胸椎椎弓根畸形率为41.1%;萎缩性脊柱侧凸85例,胸椎椎弓根畸形率为65.7%,萎缩性脊柱侧凸的胸椎椎弓根畸形率明显高于非萎缩性脊柱侧凸(x~2=78.8,P<0.001)。凹侧、中立椎和凸侧椎体的胸椎椎弓根畸形率依次下降(80.8%>66.8%>47.9%,P<0.001)。(40°~59。)组、(60°~79°)组与≥80°组间胸椎椎弓根畸形率依次上升(50.7%<59.6%<69.2%,P<0.001)。≥18岁组的胸椎椎弓根畸形率(55.3%)明显低于<18岁组(63.5%,P=0.001);此外,不同性别问胸椎椎弓根畸形率差异无统计学意义(男63.4%,女60.4%,P=0.13)。结论在NF1-S患者中,胸椎椎弓根畸形率为62%。其中萎缩性脊柱侧凸中胸椎椎弓根畸形率明显高于非萎缩性脊柱侧凸;凸侧胸椎椎弓根畸形率明显低于凹侧;随着Cobb’s角的增大,胸椎椎弓根畸形率明显增加;在成人NF1-S患者中胸椎椎弓根畸形率明显低于未成年患者;不同性别NF1-S患者胸椎椎弓根的畸形率相似。
Objective To investigate the characteristics of pedicle of thoracic pedicle in patients with type 1 neurofibromatosis type 1 (NF1) and scoliosis. Methods From July 2005 to March 2012, 100 cases of NF1 with scoliosis admitted to our hospital were scanned by spiral CT. On the basis of Lenke classification further refinement of the pedicle morphology is divided into: A type (normal type); B type (stenosis type); C type (isthmic sclerosis type); D type (fully sclerosing type) ; E type (pedicle absent type). A, B, C, D, E thoracic pedicle were 911, 561, 334, 422 and 172, a total of 2400. According to the above classification criteria, the image archiving and communication system (PACS) by layer reading, select the thoracic pedicle showed the clearest level of pedicle diameter measurement and classification, respectively, statistics of different groups NF1 with scoliosis secondary to neurofibromatosis type 1 (NF1-S) in patients with thoracic pedicle type incidence, and the rate of deformity were statistically analyzed. Results 100 cases of NF1-S, the deformity rate (B ~ E) was 62%; of which 15 cases of non-atrophic scoliosis, thoracic pedicle deformity rate was 41.1%; 85 cases of atrophic scoliosis, thoracic vertebral arch The rate of root deformity was 65.7%. The rate of thoracic pedicle deformity in atrophic scoliosis was significantly higher than that in non-atrophic scoliosis (x ~ 2 = 78.8, P <0.001). The rate of thoracic pedicle deformity in concave side, neutral side and convex side decreased in turn (80.8%> 66.8%> 47.9%, P <0.001). (50.7% <59.6% <69.2%, P <0.001) in the group of 40 ° ~ 59. (60 ° ~ 79 °) and ≥80 ° group. The rate of thoracic vertebral pedicle deformity (55.3%) was significantly lower in 18-year-old group than in 18-year-old group (63.5%, P = 0.001). In addition, there was no significant difference in the rate of pedicle deformity between male and female %, Female 60.4%, P = 0.13). Conclusions In NF1-S patients, the rate of thoracic pedicle deformity was 62%. The rate of deformity and thoracic vertebral pedicle deformity in atrophic scoliosis was significantly higher than that in non-atrophic scoliosis. The rate of deformity in lower thoracic vertebral pedicle was significantly lower than that in concave side. With the increase of Cobb’s angle, The rate of thoracic pedicle deformity was significantly lower in adults with NF1-S than that in juvenile. The deformity rate of thoracic pedicle was similar in NF1-S patients.