后路椎弓根钉系统治疗Ⅰ型神经纤维瘤病合并小儿脊柱畸形

目的 回顾性研究16例神经纤维瘤病性脊柱畸形患儿的外科治疗结果,分析侧后弯分类与手术效果的关系.方法 2004至2008年资料完整的NF-1型脊柱畸形患儿16例,男12例,女4例,平均年龄8.4岁,平均随访时间2.6年.16例均为营养不良型.按照顶椎位置分为3组:胸弯组、胸腰弯组、单纯腰弯组.根据畸形类型和位置选择后路椎弓根钉系统矫形同定脊柱融合或前后路脊柱融合.所有病例行完整的影像学检查,并进行侧弯角度与手术效果之间关系的评估.结果 观察指标为脊柱冠状面及矢状面Cobb角的变化.冠状面和矢状面的矫形率分别达到65.3%和51.2%,侧弯最大矫正率89.8%,最小20%,后弯最大矫正率89.6%,最小25.9%,随访中丢失角度为5.8°和2.7°.无断棒、断钉、融合不良及内固定失败,随访期间未发现假关节存在,1例应用MOSS-MIAMI器械患儿手术半年后返院,因需增加融合范围再次行后路植骨手术.结论 神经纤维瘤病性脊柱畸形治疗较为复杂,难度较大,应用椎弓根钉系统后路矫形固定治疗此类畸形尤其是营养不良型NF-1型侧弯及后弯可获得较好的治疗效果.

Posterior pedicle screw system for children with scoliosis caused by neurofibromatosis

Objective To analyze the relationship between the curve types and clinical results of scoliosis caused by neurofibromatosis.Methods Sixteen patients (12 male and 4 female) with scoliosis caused by neurofibromatosis were treated surgically from 2004 to 2008 with mean age of 8.4 years and mean follow-up period of 2.6 years.There were 16 dystrophic curves depending on the characters by radiographs.According to the locations of apical vertebra,the dystrophic curves were divided into three subgroups:thoracic curve,thoraco-lumbar curve,and single lumbar curve.All the patients were treated by posterior pedicle screw system spinal fusion or anterior and posterior spinal fusion according to curve types and positions.Clinical and radiographic manifestations were investigated and the relationship between them was assessed.Results The correction rates in coronary and sagittal plane were 65.3% and 51.2% respectively,the ratio of correction was from 20% to 89.8% in scoliosis,the ratio of correction was from 25.9% to 89.6% in kyphosis,and mean lost of Cobb angle were 5.8 degrees and 2.7 respectively at follow-up.There was no stick or nail broken,no internal fixation failure,no fake joint formation.One patient underwent posterior bone grafting to increase the range of fusion half year after MOSS-MIAMI operation.Conclusions The treatment of scoliosis caused by neurofibromatosis is complex and difficult.Posterior pedicle screw system is the key point for treating this scoliosis especially dystrophic curve.