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目的:探讨Ⅰ型神经纤维瘤病伴脊柱侧凸(scoliosis secondary to neurofibromatosis type 1,NF-1S)患者肋骨头脱入椎管内的影像学特征。方法:对我院1998年8月~2011年3月脊柱外科收治的142例NF-1S患者的资料进行回顾性分析,有完整影像学检查的胸弯患者共91例,其中11例患者伴有肋骨头脱入椎管,男性7例,女性4例,年龄11~21岁。测量肋骨进入椎管内程度、顶椎旋转程度和双侧肋骨头宽度比,总结其影像学特征,并分析其发生的原因。结果:NF-1S患者肋骨头脱入椎管内现象的发生率为12.1%。肋骨头脱入椎管均发生在脊柱侧凸的凸侧顶椎区。脱入水平位于T5～T11。肋骨进入椎管内程度为11.2%～50%,平均26.5%。脱入椎管内的肋骨头形态尖而细长,其宽度与对侧肋骨头宽度的比值为0.50～0.99,平均0.77;均伴有显著顶椎旋转(Nash-Moe法Ⅰ～Ⅳ度,中位数为Ⅲ度)。在MRI轴面成像上脊髓向凹侧偏移,其中5例患者脊髓紧贴凹侧椎弓根,4例伴有椎旁肿瘤。结论:NF-1S患者肋骨头脱入椎管发生在脊柱侧凸的凸侧顶椎区。肋骨头尖锐细长、椎体旋转半脱位可能是肋骨头脱入椎管内的重要原因。需手术治疗的NF-1S患者术前均应行顶椎区CT检查。
Objective: To investigate the imaging features of rib head debridement into the spinal canal in patients with type 1 neurofibromatosis scoliosis secondary to neurofibromatosis type 1 (NF-1S). Methods: The data of 142 cases of NF-1S admitted to our department from August 1998 to March 2011 were retrospectively analyzed. Thoracic patients with complete imaging examination included 91 cases, of whom 11 cases were accompanied by Rib into the spinal canal head, 7 males and 4 females, aged 11 to 21 years. The degree of the rib insertion into the spinal canal, the degree of rotation of the vertebral vertebra and the ratio of the width of the bilateral rib heads were measured to summarize the imaging features and to analyze the causes. RESULTS: The incidence of rib head debridement in the spinal canal was 12.1% in patients with NF-1S. Rib into the spinal canal head occurred in the scoliosis of the convex side of the vertebral area. Take-off level is located in T5 ~ T11. The degree of rib into the spinal canal was 11.2% ~ 50%, an average of 26.5%. The ratio of rib width to contralateral rib head width was 0.50 ~ 0.99, with an average of 0.77. All of them had significant vertebral rotation (Nash-Moe method Ⅰ ~ Ⅳ degree, medium Median is three degrees). In MRI axial imaging, the spinal cord migrated to the concave side, of which 5 patients had the spinal cord close to the concave pedicle and 4 patients with paravertebral tumors. CONCLUSIONS: The detachment of the rib head from the NF-1S patient in the convex vertebra of the scoliosis occurs in the vertebral column. Sharp and slender rib head, vertebral rotation subluxation may be an important reason for the rib head into the spinal canal. Surgical treatment of NF-1S patients should be performed before the top of the spine CT examination.