Pulmonary function test difference of Marfan syndrome and Marfanoid scoliosis patients with adolescent idiopathic scoliosis patients

English Keywords:Scoliosis  Marfan syndrome  Marfanoid  Adolescent idiopathic scoliosis  Pulmonary function

English Abstract:

  【Abstract】 Objectives: To investigate the difference of the pulmonary function test between the Marfan syndrome and Marfanoid scoliosis(MMS) patients and adolescent idiopathic scoliosis(AIS) patients, as well as the possible risk factors. Methods: In this retrospective study, 40 MMS patients(aged 11-19 years, 14 boys and 26 girls) who received posterior correction(group MMS) and fusion and 80 matched AIS patients(group AIS) who received the similar surgery during the same period were included. The essential feature and severity of scoliosis were similar in both groups. The preoperative pulmonary function tests of the two groups were investigated, including forced expiratory volume in one second(FEV1), forced vital capacity(FVC) and the maximum peak expiratory flow(PEF), and the risk factors were analyzed. Differences in pulmonary function tests among 2 groups were analyzed by using U-test, and Pearsons′ correlation was used to investigate the relationship between pulmonary function and risk factors. Results: Based on the results of pulmonary function tests, the proportion of patients with severe damage in group MMS(11/40) was significantly higher than that of group (5/80)(P<0.05). MMS patients had smaller values of FEV1 and FVC than those of AIS patients(P<0.05). No significant difference of PEF was found between the two groups. In group MMS, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes(FEV1 and FVC)(r=-0.444, -0.524, P<0.05); the age was positively correlated with the percentage of predicted pulmonary volumes(FEV1, FVC and PEF)(r=0.363, 0.326, 0.348, P<0.05); the flexibility of the thoracic curve was positively correlated with the percentage of predicted FVC(r=0.321, P<0.05). FEV1 and FVC were not related with thoracic kyphosis. In group AIS, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes(FEV1, FVC and PEF)(r=-0.338, -0.293, -0.253; P<0.05). The age was positively correlated with the percentage of predicted pulmonary volumes(FEV1 and PEF)(r=0.286, 0.341; P<0.05); the Cobb angle of thoracic kyphosis was positively correlated with the percentage of predicted pulmonary volumes(r=0.238, P<0.05). Conclusions: The damage of pulmonary function is more severe in MMS patients than that in AIS patients, and the risk factors that lead to the difference include the age and the Cobb angle of thoracic curve.