马方和类马方综合征合并脊柱侧凸患者肺功能障碍的模式及影响因素

目的 探讨合并脊柱侧凸的马方和类马方综合征患者肺功能损害的模式及其影响因素.方法 回顾性分析1998年2月至2007年9月行脊柱侧凸矫形内固定手术且有术前肺功能资料的25例马方和类马方综合征患者(A组)的临床资料,其中男性11例,女性14例;年龄11～20岁,平均15岁.分析其肺功能指标(实测值与预计值的比值)与冠状面Cobb角、胸弯顶椎位置、受累节段数以及胸椎后凸角的关系.并与同期行脊柱侧凸矫形内固定术且弯型与此匹配的38例青少年特发性脊柱侧凸(MS)患者(B组)的肺功能指标进行比较分析.结果 A组患者肺活量(VC)、用力肺活量(FVC)、第1秒最大呼气容积(FEVI)等指标与冠状面Cobb角呈显著负相关(r=0.514、-0.503、-0.464,P<0.05);VC、FVC、FEV1以及最大呼气中期流量(MMEF)等指标明显小于B组(P<0.05);顶椎位置在T_(4～8)与在T_(9～12)的患者之间肺功能指标差异无统计学意义;受累节段≥8的患者,VC、FVC、FEV1和最大自主通气量(MVV)等指标小于受累节段<8者(P<0.05);胸椎后凸角与肺功能指标之间无明显相关性.结论 合并脊柱侧凸的马方和类马方综合征患者肺功能损害较MS患者严重,其肺功能主要受胸弯受累节段数和胸弯冠状面Cobb角共同影响.

Pulmonary dysfunction patterns in patients with Marfan and Marfanoid syndrome associated with scoliosis and the influencing factors

Objective To investigate the impairment pattern and the influencing factors of pulmonary function in patients with Marfan and Marfanoid syndrome associated scoliesis (MS). Methods In this retrospective study, totally 25 MS patients (aged 11-20 years, 11 boys and 14 girls) who received posterior instrumentation and fusion (Group A) and 38 adolescent idiopathic scoliosis (AIS) patients ( Group B) ( aged 10-19 years, 11 boys and 27 girls) were included from February 1998 to September 2007. The curve pattern was matched in both groups. The preoperative pulmonary function test (Pgrs) were compared in two groups. And the parameters influencing the preoperative pulmonary function were analyzed in group A. Results In Group A, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes ( VC%, FVC% and FEV1% ) ( r = - 0. 514, - 0. 503, -0.464,P < 0.05). And the reduction of lung function parameters (VC%, FVC%, FEV1% and MMEF% ) was more severe in Group A than in Group B with compared magnitude of thoracic curve (P < 0. 05 ). In Group A, the extent of impairment of pulmonary function in patients with the number of vertebrae involved ≥8 were more severe than those involved < 8 vertebrae(P < 0. 05 ). However, there was no significant difference of deterioration of lung function between the higher apex ( T_(4～8) ) subgroup and lower apex (T_(9～12)) subgroup. And no correlation was found between thoracic kyphosis and the degrees of impairment of respiration function. Conclusions Patients with MS have mixed ventilation dysfunction, which is more severe than AIS patients with matched age and Cobb angle. The pulmonary dysfunction in MS patients can be influenced by the severity of thoracic curve and the number of involved vertebrae.