Ⅰ型神经纤维瘤病伴脊柱侧凸患者胸椎椎弓根形态学分型研究

Morphologic classification of thoracic pedicles in the patients with scoliosis secondary to neurofibromatosis type 1

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 features of morphologic classiifcation of thoracic pedicles in the patients with scoliosis secondary to neurofibromatosis type 1 （ NF1-S ）. Methods Successive spiral CT thoracic vertebrae scans of 100 patients with NF1-S who were adopted from July 2005 to March 2012 were obtained. The morphology of thoracic pedicles was classiifed based on the Lenke classiifcation system. There were type A （ normal type ）, type B （ stenotic type ）, type C （ isthmic type ）, type D （ complete sclerosis type ） and type E （ pedicle absent type ）. The numbers of thoracic pedicles of type A, B, C, D and E among the 100 patients with NF1-S were 911, 561, 334, 422 and 172 respectively, and 2400 in all. According to the above classiifcation standard, all the patients’ transverse pedicle diameters of the thoracic vertebrae were measured and classiifed based on the clearest images with the software of picture archiving and communication system （ PACS ）. The incidence of different types of thoracic pedicles in the patients with NF1-S was recorded respectively, and the malformation rate was analyzed statistically. Results The malformation rate （ type B-E ） was 62%in the 100 patients with NF1-S. Nondystrophic scoliosis was found in 15 patients whose malformation rate of thoracic pedicles （ MRTP ） was 41.1%and dystrophic scoliosis was found in 85 patients whose MRTP was 65.7%. The MRTP in the dystrophic group was obviously higher than that in the nondystrophic group （χ2=78.8, P＆lt;0.001 ）. The MRTP was decreased in turn in the concavity group, the neutral vertebrae group and the convexity group （ 80.8%＆gt;66.8%＆gt;47.9%, P＆lt;0.001 ）. The MRTP was increased in turn in the （ 40＆#176;-59＆#176; ） group, （ 60＆#176;-79＆#176; ） group and≥80＆#176; group （50.7%＆lt;59.6%＆lt;69.2%, P＆lt;0.001 ）. The MRTP of the patients equal to or more than 18 years old （ 55.3% ） was lower than that of the patients less than 18 years old （ 63.5% ） （ P=0.001 ）. In addition, there were no signiifcant differences between males （ 63.4%） and females （ 60.4%） in terms of the MRTP （ P=0.13 ）. Conclusions The MRTP in the patients with NF1-S is 62%. The MRTP is obviously higher in the dystrophic scoliosis group than in the nondystrophic scoliosis group. The MRTP is obviously lower in the convexity group than in the concavity group. The MRTP is signiifcantly increased as the Cobb’s angle is increased. The MRTP is obviously lower in adults than in adolescents. The MRTP is not affected by the sex.