摘要
目的探讨Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)伴萎缩性腰骶部畸形患者的内固定模式选择、手术疗效。方法回顾性分析2009年1月至2022年11月于南京鼓楼医院接受脊柱畸形矫形手术治疗的21例NF1伴萎缩性腰骶部畸形(L 4-sacrum)患者的资料,男11例、女10例,手术时年龄(15.4±4.7)岁(范围7~24岁)。根据患者是否采用骨盆固定[经第2骶椎骶髂螺钉(second sacral alar-iliac screw,S 2AI)或髂骨钉]进行分组,11例患者选择骨盆固定、10例选择非骨盆固定。比较两组患者手术前后及末次随访时腰骶半弯Cobb角、主弯Cobb角、局部后凸Cobb角等影像学参数的差异。结果非骨盆固定组患者年龄(13.2±4.9)岁,小于骨盆固定组的(17.5±3.5)岁,差异有统计学意义(t=2.287,P=0.034)。非骨盆固定组和骨盆固定组萎缩性改变导致的脊柱不稳定(旋转半脱位或椎体滑脱)分别为2例和8例,差异有统计学意义(χ^(2)=5.838,P=0.030)。骨盆固定组行单侧固定5例、双侧固定6例,采用髂骨钉、髂骨钉+经S 2骶髂螺钉(S 2 alar-iliac Screw,S 2AI)及S 2AI分别为2例、1例和8例。非骨盆固定组中椎板钩的密度为12.6%±11.5%,高于骨盆固定组的3.5%±6.9%,差异有统计学意义(t=2.230,P=0.038)。非骨盆固定组和骨盆固定组术前腰骶半弯Cobb角分别为13.8°±9.0°和25.5°±13.9°(t=2.228,P=0.039),术后矫正至6.3°±6.1°和6.4°±5.3°(t=0.901,P=0.969),矫正率为57.3%±13.6%和74.1%±17.8%(t=2.369,P=0.029);末次随访时为6.6°±6.6°和6.3°±4.8°(t=0.116,P=0.909)。非骨盆固定组和骨盆固定组术前主弯Cobb角分别为52.5°±15.1°和61.1°±16.9°(t=1.200,P=0.246),术后矫正至31.3°±13.8°和28.0°±8.4°(t=0.646,P=0.526),矫正率为41.3%±13.0%和53.2%±11.6%(t=2.206,P=0.037);末次随访时为32.4°±14.2°和31.7°±10.3°(t=0.133,P=0.896)。9例患者伴有局部后凸畸形,由术前的19.7°±10.9°矫正至术后的-13.6°±9.5°,末次随访时维持在-14.1°±9.6°(F=33.547,P<0.001)。非骨盆固定组和骨盆固定组应用多棒系统分别为6例和7例,差异无统计学意义(χ^(2)=0.153,P=0.926)。非骨盆固定组2例在术后3年发生冠状面失代偿,1例接受翻修手术。骨盆固定组患者发生3例断棒,其中2例接受翻修手术。结论腰骶部萎缩性旋转半脱位或椎体滑脱是NF1伴萎缩性腰骶部畸形患者行骨盆固定的主要指征,但内固定并发症并不少见。多棒钉钩混合系统(尤其跨越腰骶部区域)的联合应用有助于降低内固定失败的风险。
ObjectiveTo analyze the selection of internal fixation methods,surgical outcomes,and complications in patients with Neurofibromatosis Type 1(NF1)accompanied by dystrophic lumbosacral deformities,and to evaluate the indications for pelvic fixation.MethodsA retrospective analysis was conducted on 21 patients with NF1 and associated dystrophic lumbosacral malformations(L 4 to sacrum)who underwent spinal deformity correction surgery at Nanjing Drum Tower Hospital from January 2009 to November 2022.The cohort included 11 males and 10 females,with a mean surgical age of 15.4±4.7 years(range,7-24 years).Patients were divided into two groups based on whether pelvic fixation was performed:10 patients in the non-pelvic fixation group(NP group)and 11 in the pelvic fixation group(P group),where fixation involved second sacral alar-iliac(S 2AI)screws or iliac screws.Radiographic parameters,including the Cobb angle of the lumbosacral fractional curve,main curve,and focal kyphosis,were compared preoperatively,postoperatively,and at the last follow-up.ResultsThe NP group had a significantly lower mean age(13.2±4.9 years)compared to the P group(17.5±3.5 years;t=2.287,P=0.034).Spinal instability(rotational subluxation or spondylolisthesis)due to dystrophic changes was observed in 2 patients in the NP group and 8 in the P group,a statistically significant difference(χ^(2)=5.838,P=0.030).In the P group,five patients underwent unilateral fixation and six underwent bilateral fixation.Implant types included 2 cases with iliac screws,1 case with iliac screws plus S 2AI,and 8 cases with S 2AI screws alone.The utilization rate of hooks was significantly higher in the NP group(12.6%±11.5%)compared to the P group(3.5%±6.9%;t=2.230,P=0.038).The preoperative Cobb angle of the lumbosacral fractional curve was significantly smaller in the NP group(13.8°±9.0°)than in the P group(25.5°±13.9°;t=2.228,P=0.039).Postoperatively,the angles were corrected to 6.3°±6.1°and 6.4°±5.3°,respectively(t=0.901,P=0.969),with correction rates of 57.3%±13.6%and 74.1%±17.8%(t=2.369,P=0.029).At final follow-up,the angles remained stable(6.6°±6.6°vs.6.3°±4.8°;t=0.116,P=0.909).For the main curve,preoperative Cobb angles were 52.5°±15.1°(NP)and 61.1°±16.9°(P;t=1.200,P=0.246),corrected to 31.3°±13.8°and 28.0°±8.4°,respectively(t=0.646,P=0.526).Correction rates were 41.3%±13.0%in the NP group and 53.2%±11.6%in the P group(t=2.206,P=0.037).At the final follow-up,these values were 32.4°±14.2°and 31.7°±10.3°(t=0.133,P=0.896).Focal kyphosis,seen in 9 patients,was corrected from 19.7°±10.9°preoperatively to-13.6°±9.5°postoperatively,and remained at-14.1°±9.6°at the final follow-up(F=33.547,P<0.001).Multi-rod systems were used in 6 cases(NP group)and 7 cases(P group),with no significant difference(χ^(2)=0.153,P=0.926).Two patients in the NP group developed coronal decompensation three years postoperatively,and one required revision surgery.In the P group,rod breakage occurred in 3 patients,two of whom underwent revision.ConclusionsDystrophic rotational subluxation or spondylolisthesis of the lumbosacral spine is a primary indication for pelvic fixation in patients with NF1-associated deformities.However,complications related to internal fixation remain common.The combined use of a multi-rod screw-hook hybrid system,particularly when extending across the lumbosacral region,may reduce the risk of instrumentation failure.
作者
李松
朱泽章
周杰
毛赛虎
孙署淇
刘臻
史本龙
孙旭
乔军
邱勇
Li Song;Zhu Zezhang;Zhou Jie;Mao Saihu;Sun Shuqi;Liu Zhen;Shi Benlong;Sun Xu;Qiao Jun;Qiu Yong(Division of Spine Surgery,Department of Orthopaedic Surgery,Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School,Nanjing 210008,China)
出处
《中华骨科杂志》
北大核心
2025年第9期604-612,共9页
Chinese Journal of Orthopaedics
基金
江苏省骨科医学创新中心(CXZX202214)
南京鼓楼医院临床研究专项基金项目(LCYJ-2024-PY-10)。
关键词
神经纤维瘤病1型
腰骶部
脊椎前移
旋转半脱位
骨盆固定
多棒内固定
Neurofibromatosis 1
Lumbosacral region
Spondylolisthesis
Rotational subluxation
Pelvic fixation
Multi-rod instrumentation
