Ⅰ型神经纤维瘤病营养不良性脊柱侧凸合并肋骨头脱入椎管的术前危险因素及影响肋骨头被动复位的相关因素分析

Analysis of the preoperative risk factors of intraspinal rib head dislocation in children with dystrophic scoliosis secondary to type 1 neurofibromatosis and the related factors that affecting the passive reduction of rib head

中文关键词:  神经纤维瘤病  营养不良性脊柱侧凸  肋骨头  椎管  危险因素

英文关键词:Neurofibromatosis  Dystrophic scoliosis  Rib head  Spinal canal  Risk factors

中文摘要:

  【摘要】 目的：探讨Ⅰ型神经纤维瘤病营养不良性脊柱侧凸（dystrophic scoliosis secondary to type 1 neurofi?鄄bromatosis，NF1-DS）合并肋骨头脱入椎管的术前风险及影响肋骨头被动复位的相关因素。方法：2006年9月~2020年5月我院收治130例NF1-DS患者，34例患者合并肋骨头脱入椎管，其中20例接受生长棒置入术，14例接受后路矫形固定术。7例患者行肋骨头切除术，27例患者保留肋骨头而直接进行矫形固定。将患者术前可能与肋骨头脱入椎管比例（intraspinal rib proportion，IRP）相关的年龄、身体重量指数（body mass index，BMI）、顶椎旋转角度（apical vertebral rotation，AVR）、顶椎偏移距离（apical vertebral translation，AVT）、肋骨头脱入椎管角度、肋骨头宽度比值、主胸弯Cobb角、躯干偏移（trunk shift，TS）、胸椎后凸（thoracic kyphosis，TK）、腰椎前凸（lumbar lordosis，LL）、矢状面平衡进行Pearson相关性分析，而后通过多元线性回归分析从可能的相关因素中筛选出独立的危险因素。利用Pearson相关性分析对与IRP矫正率可能相关的AVR矫正率、AVT矫正率、主胸弯Cobb角矫正率、TS矫正率、TK矫正率、LL矫正率和矢状面平衡矫正率进行分析。结果：肋骨头脱入椎管在NF1-DS患者中的发生率为26.2%（34/130），术前IRP为5.5%~68.5%[（32.9±17.2）%]。术前IRP与术前BMI、AVR、AVT、主胸弯Cobb角和TK相关（P<0.05），与年龄、肋骨头脱入椎管角度、肋骨头宽度比值、TS、LL和矢状面平衡无相关性（P>0.05）。多元线性回归分析提示术前BMI和主胸弯Cobb角为术前IRP独立危险因素（B=-4.733，P=0.040；B=0.470，P<0.001）；而术前AVR、AVT、TK为术前IRP的非独立危险因素（P>0.05）。在27例保留了肋骨头而直接进行矫形的病例中，IRP由术前的（32.6±16.3）%显著下降至术后的（18.9±11.6）%（P<0.05），其矫正率达（46.6±19.9）%；保留肋骨头病例的IRP矫正率与主胸弯Cobb的矫正率存在正相关（r=0.443，P=0.009），与AVR矫正率、AVT矫正率、TS矫正率、TK矫正率、LL矫正率和矢状面平衡矫正率无相关性（P>0.05）。结论：术前营养状况越差、脊柱畸形严重的NF1-DS患者肋骨头脱入椎管的程度越明显；未切除肋骨头而直接矫形的患者术中应尽可能提高主胸弯Cobb角的矫正率，有助于脱入椎管肋骨头被动复位。

英文摘要:

  【Abstract】 Objectives: To explore the preoperative risks and the factors affecting the rib head passive reduction of rib head dislocation into spinal canal in children with dystrophic scoliosis secondary to type 1 neurofibromatosis(NF1-DS). Methods: From September 2006 to May 2020, 130 patients with NF1-DS were included in this study, and 34 patients were found with intraspinal dislocation of rib head. 20 patients received growing rods treatment and 14 patients received posterior spinal fusion. 7 patients underwent rib head resection and 27 patients underwent surgery directly without rib head resection. The factors that related to preoperative intraspinal rib proportion(IRP) included age, body mass index (BMI), apical vertebral rotation(AVR), apical vertebral translation(AVT), the angle of intraspinal rib, proportion of rib width, the main tho?鄄racic Cobb angle, trunk shift(TS), thoracic kyphosis(TK), lumbar lordosis(LL) and sagittal balance. Pearson cor?鄄relation analysis was used to screen out the relevant factors and preformed multivariable linear regression to determine the independent risk factors of the IRP. Pearson correlation analysis was used to screen out the factors that related to the correction rate of IRP, included the correction rate of AVR, AVT, the main tho?鄄racic Cobb angle, TS, TK, LL and sagittal balance. Results: The incidence of intraspinal rib in NF1-DS pa?鄄tients was 26.2% (34/130). The preoperative IRP was (32.9±17.2)% (5.5%-68.5%). Preoperative IRP was cor?鄄related with BMI, AVR, AVT, the main thoracic Cobb angle and TK (P<0.05), but it had no correlation with age, the angle of intraspinal rib, proportion of rib width, TS, LL and sagittal balance(P>0.05). Multiple linear regression analysis indicated that BMI and main thoracic Cobb angle were independent risk factors of the pre?鄄operative IRP(B=-4.733, P=0.040; B=0.470, P<0.001). AVR, AVT and TK were non-independent risk factors of the preoperative IRP(P>0.05). In the 27 patients without rib head resection, the IRP decreased from preop?鄄erative (32.6±16.3)% to postoperative (18.9±11.6)%(P<0.05) and the correction rate of IRP was(46.6±19.9)%. The correction rate of IRP was correlated with the correction rate of the main thoracic Cobb angle(r=0.443, P<0.009). The correction rate of IRP was not correlated with the correction rate AVR, AVT, TS, TK, LL and sagittal balance(P>0.05). Conclusions: The patients with worse preoperative nutritional status and severe spinal deformity were more serious in the degree of IRP. The correction rate of the main thoracic Cobb angle should be corrected as much as possible, so as to increase the passive reduction of the intraspinal rib head.