Chou Shih-Hsiang, Lin Gau-Tyan, Shen Po-Chih, Lue Yi-Jing, Lu Cheng-Chang, Tien Yin-Chun, Lu Yen-Mou
Department of Orthopedics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
Department of Orthopedics, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Eur Spine J. 2017 Jun;26(6):1721-1731. doi: 10.1007/s00586-016-4828-2. Epub 2016 Nov 2.
Various results of the previous literature related to surgical effect on pulmonary function of spinal muscular atrophy (SMA) patients might be due to different SMA type, different fusion level and technique. The aim of this study was to determine the value of scoliosis surgery for SMA type II patients with regard to pulmonary function, under the same fusion level, fusion technique and average long-term follow-up.
Ten SMA II patients who underwent spinal correction procedures from 1993 to 2010 were identified. Data on clinical features and pulmonary function, including forced vital capacity (FVC) and forced expiratory volume in 1st second (FEV), were collected. The data on pulmonary function were divided into preoperative, postoperative short-term (0-5 years), mid-term (5-10 years), and long-term (>10 years). Statistical comparisons were made using the Wilcoxon test for pulmonary function and body weight analysis. Questions were answered by parents on how surgery influenced the frequency of respiratory infection and the ability to sit at school.
The average length of postoperative pulmonary function follow-up was 12.3 years (range 4.9-15.9 years). There was no significant difference in FVC or FEV between preoperative and each postoperative period. However, a significant decline from mid-term to long-term was observed (p = 0.028). Body weight increased significantly in all postoperative periods and was moderately correlated to pulmonary function (r = 0.526 for FVC). The answers to the questionnaire revealed that 80% of the patients had obvious improvement in the frequency of respiratory infection and 100% were tolerable sitting at school.
Surgical correction for scoliosis in SMA II patients results in pulmonary function being maintained during long-term follow-up. In addition, the advantages of surgery also include body weight gain, better sitting tolerance, and reduced frequency of respiratory infection.
既往文献中关于脊柱肌肉萎缩症（SMA）患者手术对肺功能影响的各种结果，可能归因于不同的SMA类型、不同的融合节段和技术。本研究的目的是在相同的融合节段、融合技术和平均长期随访条件下，确定脊柱侧弯手术对II型SMA患者肺功能的价值。
确定了1993年至2010年期间接受脊柱矫正手术的10例II型SMA患者。收集了临床特征和肺功能数据，包括用力肺活量（FVC）和第1秒用力呼气量（FEV）。肺功能数据分为术前、术后短期（0至5年）、中期（5至10年）和长期（>10年）。采用Wilcoxon检验对肺功能和体重分析进行统计学比较。由家长回答关于手术如何影响呼吸道感染频率和在学校坐立能力的问题。
术后肺功能随访的平均时长为12.3年（范围4.9至15.9年）。术前与术后各阶段的FVC或FEV无显著差异。然而，从中期到长期观察到显著下降（p = 0.028）。所有术后阶段体重均显著增加，且与肺功能呈中度相关（FVC的r = 0.526）。问卷调查的答案显示，80%的患者呼吸道感染频率有明显改善，100%的患者在学校能够耐受坐立。
II型SMA患者的脊柱侧弯手术矫正可使肺功能在长期随访中得以维持。此外，手术的优势还包括体重增加、更好的坐立耐受性以及呼吸道感染频率降低。
