摘要目的 分析I型神经纤维瘤病(NF1)合并脊柱侧凸患者肺功能损害的模式,以及影响其肺功能的影像学因素.方法 选取2003年1月至2009年6月间收治的NF1合并脊柱侧凸患者36例(NF1组),特发性脊柱侧凸(IS)患者64例(IS组),术前检测患者的肺活量(VC)、用力肺活量(FVC)、第1秒用力呼气容积(FEV1)、最大呼气中期流量(MMEF)、最大自主通气量(MVV).比较两组肺功能参数的差异.按照弯型部位及有无萎缩性改变将NF1组患者分类并分类比较肺功能损害的差异,分析影响肺功能的影像学指标.结果 两组肺功能参数VC、FVC、FEV1、MMEF、MVV差异均无统计学意义(P>0.05).NF1组胸弯患者肺功能显著低于非胸弯患者;营养不良型患者与非营养不良型患者肺功能损害差异无统计学意义(P>0.05);顶椎位置以及Cobb角与肺功能参数显著相关(P<0.05).结论 NF1合并脊柱侧凸患者的肺功能损害模式与IS患者类似,胸弯患者比非胸弯患者肺功能损害严重,侧凸位置以及Cobb角大小是影响患者肺功能的主要因素.

abstractsObjective To investigate the pulmonary dysfunction patterns in patients of scohosis associated with neurofibmmatosis type I(NFI)and to identify factors affecting the pulmonary function in patients with scoliosis associated with NF1.Methods Preoperative pulmonary function tests(Pyrs)were evaluated in 100 patients with scoliosis[NF1 group,36 cases;idiopathic scoliosis(IS)group,64 cases]from January 2003 to June 2009.According to location of apical vertebra and dystrophic change in patients with NF1,the parameters of pulmonary function[vital capacity(VC),forced vital capacity(FVC),forced expiratory volume in one second(FEV1),maximal mid-expiratory flow(MMEF),maximal voluntary ventilation(MVV)]werc compared between NF1 group and IS group,and between the subgroups of NF1.The correlation between pulmonary function parameters and radiographic parameters of scoliosis was analyzed.Results The VC,FVC,FEV1,MMEF,MVV in NF1 group and IS group were of no significant difference(P>0.05).In NF1 patients,the pulmonary dysfunction wag more severe in thoracic subgroup than non-thoracic subgroup(P＜0.05).while there was no difference between dystrophic scoliosis and nondystrophic scohosis(P>0.05).The location of apical vertebra and the severity of acoliosis correlated significantly with the pulmonary dysfunction in NFI group.Conclusions The pattern of pulmonary dysfunction in scoliosis associated with NF1 is similar with IS.Pulmonary dysfunction is more severe in thoracic scohosis.The location of apical vertebra and the severity of acohosis are the risk factors influencing the pulmonary dysfunction.