传统生长棒治疗合并肋骨头脱入椎管的早发性营养不良性1型神经纤维瘤病脊柱侧凸  

Efficiency and safety of traditional growing rod technique in the treatment of early onset dystrophic scoliosis secondary to type 1 neurofibromatosis with intraspinal rib head in children

作　　者：高荣轩[1] 张学军[1] 郭东[1] 曹隽[1] 姚子明 白云松[1] 祁新禹[1] Gao Rongxuan;Zhang Xuejun;Guo Dong;Cao Jun;Yao Ziming;Bai Yunsong;Qi Xinyu(Department of Orthopaedics,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院骨科,北京100045

出　　处：《中华骨科杂志》2022年第20期1373-1381,共9页Chinese Journal of Orthopaedics

基　　金：国家重点研发计划(2016YFC1000806);北京市医管局青苗人才培养项目(QMS20211209)。

摘　　要：目的探讨传统生长棒治疗合并肋骨头脱入椎管的早发性营养不良性1型神经纤维瘤病脊柱侧凸(dystrophic scoliosis secondary to type 1 neurofibromatosis,NF1-DS)的疗效及安全性。方法回顾性分析2006年9月至2020年5月采用传统生长棒治疗20例合并肋骨头脱入椎管的早发性NF1-DS患儿资料,男13例、女7例,初次手术年龄(7.0±1.6)岁(范围4.1~9.8岁);单纯左胸弯7例、单纯右胸弯9例、双胸弯4例;13例存在不同程度的胸椎后凸畸形。术前2例患儿存在神经症状,美国脊髓损伤协会损伤量表(American Spinal Injury Association Impairment Scale,AIS)神经功能分级均为D级。术前、术后即刻和末次随访时测量肋骨头脱入椎管比例(intraspinal rib proportion,IRP)、主胸弯Cobb角、顶椎旋转角度、顶椎偏移距离、躯干偏移距离、胸椎后凸角、腰椎前凸角、矢状面平衡及T1~S1高度。结果20例患儿均获得随访,随访时间(41.6±23.8)个月(范围24~99个月),共接受85次手术,其中63次为撑开手术。IRP术后(22.2%±11.3%)较术前(33.1%±17.5%)减小(P<0.001),末次随访时(23.7%±12.4%)与术后比较无明显变化(P>0.05);主胸弯Cobb角由术前75.9°±26.7°减少至术后45.0°±18.5°,差异有统计学意义(P<0.001),且末次随访时(41.0°±17.2°)较术后仍有改善(P<0.05);术后顶椎旋转角度(33.0°±10.1°)较术前(39.3°±13.3°)减小(P<0.001),末次随访时(40.1°±11.4°)较术后增大(P<0.05);T1~S1高度由术前(259.8±70.7)mm增加至术后(296.9±78.4)mm(P=0.001),末次随访时为(339.9±83.4)mm,较术后仍有增加(P<0.001),每年增长(12.4±3.2)mm。术后顶椎偏移距离、胸椎后凸角、腰椎前凸角和矢状面平衡较术前明显改善(P<0.05),末次随访时与术后比较差异无统计学意义(P>0.05)。7例患儿出现10次并发症,其中椎弓根螺钉松动5次、螺栓脱落1次、断棒2次、远端交界性后凸1次、远端附加现象1次。2例术前存在神经症状的患儿,术后逐渐恢Objective To investigate the efficiency and safety of traditional growing rod in the treatment of early onset dystrophic scoliosis secondary to type 1 neurofibromatosis(NF1-DS)with intraspinal rib head in children.Methods From September 2006 to May 2020,this study recruited 20 children with intraspinal rib head with early onset NF1-DS who had received traditional growing rods.There were 13 boys and 7 girls and the age of the initial operation was 7.0±1.6 years(range,4.1-9.8 years).There were 7 cases of simple left chest bend,9 cases of simple right chest bend,and 4 cases of double chest bend;13 patients had varying degrees of kyphosis deformity.Two children had neurological symptoms before surgery,American Spinal Injury Association Impairment Scale(AIS)were grade D.The proportion of the intraspinal rib head(IRP),the Cobb angle of the main chest bend,apical vertebra rotation(AVR),apical vertebral translation(AVT),trunk shift(TS)and sagittal TK,lumbar lordosis(LL),sagittal balance and T1-S1 height were measured before and after first time internal fixation and at last follow-up,and the complications were also evaluated.Results All 20 patients were followed up and the average follow-up time was 41.6±23.8 months(range,24-99 months).A total of 85 operations was conducted including 63 protrude operations.After operation,the IRP was significantly lower than that before operation(preoperative 33.1%±17.5%vs.postoperative 22.2%±11.3%,P<0.001)and no significant correction loss was found at last follow-up 23.7%±12.4%(P>0.05).The mean Cobb angle decreased from 75.9°±26.7°preoperatively to 45.0°±18.5°postoperatively(P<0.001)and there was still significant improvement at the last follow-up(41.0°±17.2°)compared with postoperatively(P<0.05).The AVR was significantly reduced after surgery compared with preoperatively(33.0°±10.1°vs.39.3°±13.3°,P<0.001),and the last follow-up(40.1°±11.4°)was significantly improved compared with postoperative(P=0.005).The T1-S1 height increased from 259.8±70.7 mm preoperativel

关 键 词：脊柱侧凸 神经纤维瘤病 肋骨 椎管 

分 类 号：R687.3[医药卫生—骨科学]