對脊髓性肌肉萎縮症第二型病人開刀矯正脊椎側彎後肺功能之影響

The effect of scoliosis surgery on pulmonary function of spinal muscular atrophy type II patients

周世祥(Shih-Hsiang Chou)

指導教授 ： 林高田

高雄醫學大學/醫學院/醫學研究所碩士班/碩士(2015年)

摘要

研究背景: 脊椎側彎 (scoliosis)在脊髓性肌肉萎縮症第二型病人 (spinal muscular atropy type II) 是很常見的。而且，它會造成肺功能限制性的影響。很少有論文提及開刀矯正脊椎側彎後對脊髓性肌肉萎縮症第二型病人之肺功能變化。
研究方法: 以病例回溯性的方式，記錄從西元1993年到2010年間，有開刀矯正脊椎側彎的脊髓性肌肉萎縮症第二型病人。記錄內容包括，手術前、手術後的肺功能,肺功能包含有1.用力肺活量 Forced vital capacity，以下簡稱FVC,和一秒用力呼氣容積 Forced expiratory volume in 1 second，以下簡稱FEV1。手術後的肺功能歸納區分為短期，中期，和長期。另外記錄了體重和問題回答，問題包含是否降低了呼吸道感染頻率，和能否忍受坐姿在學校超過六小時的時間。
研究結果: 有10位脊髓性肌肉萎縮症第二型病人，在統計分析後得到，肺功能之變化在平均11年的追蹤中，手術後和手術前比較並無統計上有意義地減少。但是在中期至長期間，仍會有明顯地肺功能下降。明顯的體重增加在短期、中期、長期中都可發現，而BMI在術後短期有增加。而體重和肺功能的關係呈現中度的正相關。問題回答有百分之八十有明顯減少呼吸道感染機會，百分之百可以達到學校坐姿有六小時以上。
研究結論: 開刀矯正脊椎側彎對脊髓性肌肉萎縮症第二型病人術後的肺功能可達維持之效果。此外，開刀的另外好處包含有體重的增加，較好的坐姿及坐姿忍受時間和減少呼吸道感染的機會。

關鍵字

脊髓性肌肉萎縮症第二型 ； 脊椎矯正

並列摘要

BACKGROUND:
Scoliosis in spinal muscular atrophy type II (SMA II) patients is common and has important clinical consequences for pulmonary function. Few studies have examined the pulmonary function of SMA II patients following spinal correction procedures. This study was aimed to determine the value of the scoliosis surgery on spinal muscular atrophy type II patients regarding to pulmonary function, frequency of respiratory infection, body weight and the sitting tolerance at school.
METHODS:
SMA II patients undergoing spinal correction procedures during 1993-2010 were identified. Clinical data and pre-operation and post-operation pulmonary function, including forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) data were collected. The data of pulmonary function was divided into pre-operative (Pre-OP), post-operative short-term (0-5 years), mid-term (5-10 years), and long-term (> 10 years). Statistical comparisons were made using Wilcoxon test for pulmonary function and body weight analysis. The relationship between body weight and pulmonary function was determined by Spearman's rank correlation coefficient method. Questions were answered by parents about how influenced to the frequency of respiratory infection and more than six hours sitting well at school or not.
RESULTS:
Ten SMA II patients were enrolled. The mean correction rate was 62% (range 53.3-75.7%). The average length of postoperative pulmonary function follow-up was 131 months (range 59-191 months). The average Pre-OP FVC and FEV1 were 0.87 ± 0.40 and 0.91 ± 0.42 (mean ± standard deviation). The average postoperative short-term, mid-term and long-term of FVC were 0.91 ± 0.38, 0.82 ± 0.39, and 0.78 ± 0.33. The average FEV1 data were 0.93± 0.42, 0.85 ± 0.37, and 0.71 ± 0.31. There was not significant different between preoperative and each postoperative periods in FVC, neigher in FEV1. However, the significant decline from mid-term to long-term were observed on both of FVC and FEV1 (p=0.028). The body weight increased significantly in all postoperative period compared with preoperative period and the body mass index of short-term as well. Body weight and pulmonary function was moderate correlated (r= 0.526 in FVC; 0.518 in FEV1 respectively). The regression equation was y = 0.305+ 0.011x (r2= 0.26; p< 0.001), where y is the FVC in liter and x is the body weight in kilogram. The answer of questions revealed 80% obvious improved in the frequency of respiratory infection and 100% sitting well more than 6 hours at school.
CONCLUSIONS:
Surgical correction for scoliosis of SMA II patient results in pulmonary function maintained under long-term follow-up. In addition, the advantage of surgery also includes body weight gain and improved trunk alignment and better sitting tolerance and less frequency of respiratory infection.

並列關鍵字

spinal muscular atropy type II ； scoliosis correction

參考文獻

第六章 參考文獻

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