Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III
Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III
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DOI：
10.1046/j.1440-1754.2003.00266.x
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发表时间：
2003-12-01
影响因子：
1.7
通讯作者：
Goh, DY
中科院分区：
文献类型：
--
作者：
Chng, SY;Wong, YQ;Goh, DY
目的：目的是评估新加坡国立大学医院需要进行脊柱侧弯手术的 II 型和 III 型脊髓性肌萎缩症 (SMA) 患者的临床病程。方法：对 1983 年至 1992 年之间 10 年间出生的 SMA II 型和 III 型患者进行回顾性分析。结果：共有 8 名患者：4 名 SMA II 型，4 名 SMA III 型。脊柱侧凸手术的平均年龄为 9 岁 7 个月（范围 7 岁 6 个月至 12 岁 4 个月）。术前平均Cobb角为65.4度（范围43-90度），术后平均Cobb角为22.6度（范围12-45度），平均矫正率为64.8%（范围47.7-77.8%）。术前预计用力肺活量 (FVC) 百分比每年下降 7.7%（95% CI：12.4% 至 3.0%），术后每年下降至 3.8%（95% CI：5.8% 至 1.9%）。术前和术后肺功能随访的平均时间分别为 6.3 个月（范围 0.03-31 个月）和 44 个月（范围 0-110 个月）。结论：本研究表明 SMA II 型和 III 型脊柱侧弯手术后肺功能继续下降，尽管下降速度不太明显。总体而言，本研究和所有其他先前发表的研究的综合结果在脊柱侧弯手术对 II 型和 IIII 型 SMA 肺功能的影响方面是相互矛盾的，尽管一半的研究（6 项中的 3 项）确实证明术后肺功能持续下降。尽管脊柱稳定，但肺功能下降可能是继发于该疾病的进行性神经肌肉无力。
Aims: The objectives were to evaluate the clinical course of spinal muscular atrophy (SMA) types II and III patients necessitating scoliosis surgery at the National University Hospital, Singapore.Methods: A retrospective review of SMA types II and III patients, born over a 10-year period between 1983 and 1992, was conducted.Results: There were eight patients: four with SMA type II and four with SMA type III. The mean age at scoliosis surgery was 9 years 7 months (range 7 years 6 months-12 years 4 months). The mean preoperative Cobb angle was 65.4degrees (range 43-90degrees) and the mean postoperative Cobb angle was 22.6degrees (range 12-45degrees), with a mean correction of 64.8% (range 47.7-77.8%). The decline in percentage predicted forced vital capacity (FVC) was 7.7% (95% CI: 12.4% to 3.0%) per year preoperatively and this was reduced to 3.8% (95% CI: 5.8% to 1.9%) per year postoperatively. The mean length of preoperative and postoperative lung function follow-up was 6.3 months (range 0.03-31 months) and 44 months (range 0-110 months), respectively.COnclusions: This study suggests that pulmonary function in SMA types II and III continues to decline after scoliosis surgery, though the rate of decline is less marked. Overall, the combined results from this study and all other previously published studies are conflicting in regard to the effect of scoliosis surgery on pulmonary function in SMA types II and IIII, though half of the studies (3 of 6) did demonstrate a continued decline in lung function postoperatively. This decline in pulmonary function despite spinal stabilization is likely secondary to the progressive neuromuscular weakness of the disease.