Operative treatment of severe scoliosis and pelvic obliquity in patients with spinal muscular atrophy: assessment of outcomes and complications.

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Heng Sun, Yizhen Huang, Yulei Dong, Zhen Wang, Junduo Zhao, Xuan Huang, Weiyun Chen, Jianxiong Shen
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引用次数: 0

Abstract

Background: Few reports exist that focus on patients with spinal muscular atrophy (SMA) and severe spinal deformity. In this study, we aimed to report surgical outcomes and complications for SMA patients with severe scoliosis and pelvic obliquity.

Methods: A retrospective review of data on operatively treated SMA patients with severe scoliosis and pelvic obliquity (minimum major coronal curve Cobb angle > 100° and pelvic obliquity > 20°) was performed. Radiography findings, pulmonary function, motor status, the sitting function score, and perioperative and postoperative complications were the main clinical outcomes examined. Muscular dystrophy spine questionnaire (MDSQ) responses and caregiver responses to four anchor questions (quality of life/comfort/ease of care/overall health) using Likert scales were recorded.

Results: Of 28 consecutive patients, 22 (79%) completed the minimum 2-year follow-up (mean age at surgery = 16.1, 68% female). The mean follow-up duration was 40.3-mo. All patients underwent one-stage posterior spinal fusion (PSF) with pelvic fixation. Radiographic measurements (main coronal curve, kyphosis, pelvic obliquity) were significantly corrected (all p < 0.001) and were maintained at the last follow-up. The mean forced vital capacity (FVC) remained stable during follow-up, with 50% of patients showing improvement. The percentage of patients who could sit independently increased significantly from 22.7% preoperatively to 77.3% postoperatively (p < 0.001). The total sitting-related MDSQ score significantly increased from 8.5 to 12.5 at 6 months postoperatively, and to 15.0 at the last follow-up (p < 0.001). Six instances of complications (two instances each of pneumonia, epiglottic edema, and delayed wound healing) occurred perioperatively in six patients (27.3%), but no surgical intervention was required.

Conclusion: Operative treatment significantly improved radiographic parameters and sitting function and maintained pulmonary function without serious complications in SMA patients with severe scoliosis and pelvic obliquity.

脊髓性肌萎缩患者重度脊柱侧凸和骨盆倾斜的手术治疗:结果和并发症的评估。
背景:关于脊髓性肌萎缩(SMA)和严重脊柱畸形的报道很少。在这项研究中,我们旨在报道SMA患者合并严重脊柱侧凸和骨盆倾斜的手术结果和并发症。方法:回顾性分析经手术治疗的伴有严重脊柱侧凸和骨盆倾斜(最小主冠状曲线Cobb角> 100°和骨盆倾斜> 20°)的SMA患者的资料。影像学表现、肺功能、运动状态、坐位功能评分、围手术期和术后并发症是检查的主要临床结果。使用李克特量表记录肌肉萎缩症脊柱问卷(MDSQ)的回答和护理者对四个锚定问题(生活质量/舒适/护理便利/整体健康)的回答。结果:在28例连续患者中,22例(79%)完成了至少2年的随访(手术时平均年龄为16.1岁,68%为女性)。平均随访时间为40.3个月。所有患者均行一期后路脊柱融合术(PSF)伴骨盆固定。x线测量(主冠状曲线、后凸、骨盆倾斜)得到显著纠正(均p < 0.001),并在最后一次随访时保持不变。平均用力肺活量(FVC)在随访期间保持稳定,50%的患者表现出改善。能够独立坐位的患者比例从术前的22.7%显著增加到术后的77.3% (p < 0.001)。术后6个月,与坐姿相关的MDSQ总分从8.5上升到12.5,最后一次随访时上升到15.0 (p < 0.001)。6例患者(27.3%)围手术期出现6例并发症(肺炎、会厌水肿和伤口延迟愈合各2例),但不需要手术干预。结论:SMA合并重度脊柱侧凸和骨盆倾斜患者手术治疗可显著改善影像学参数和坐位功能,维持肺功能,无严重并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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