Division of Orthopaedics, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.
Pediatric Orthopaedics, UCSF Benioff Children's Hospital Oakland, San Francisco, CA, USA.
Eur Spine J. 2021 Mar;30(3):768-774. doi: 10.1007/s00586-020-06564-8. Epub 2020 Aug 18.
Patients with spinal muscular atrophy (SMA) are often treated with growth friendly devices such as vertical expandable prosthetic titanium rib(VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, thorax morphology and pulmonary outcomes.
This is a retrospective analysis of prospectively collected data. We included patients with chest wall deformity and scoliosis secondary to SMA who were treated with growth friendly interventions and had two-year follow-up. Descriptive statistics and univariate analyses were performed.
This study included 66 patients (25% MCGR, 73% VEPTR, 2% unknown). Approximately 23% of constructs included CWS. The average Cobb angle improved from 67° (SD: 27°) to 50° (SD: 26°) at 2 years in patients with CWS (p = 0.02), and from 59° (SD: 20°) to 46° (SD: 15°) at 2 years in patients without CWS (p < 0.01). Hemithorax height improved in patients treated with and without CWS (p = 0.01), but hemithorax width only improved in patients with CWS (p = 0.01). One patient with CWS and two patients without CWS required additional respiratory support at 2 years. The rates of postoperative complications were not significantly different in patients treated with and without CWS (p = 0.31).
Growth friendly constructs improve spinal deformity and may be effective in altering the progression toward respiratory failure in patients with SMA. Patients treated with CWS have significant improvements in thorax morphology compared to patients without CWS.
脊髓性肌萎缩症（SMA）患者常采用生长友好型装置（如垂直可扩张假体钛肋骨[VEPTR]和磁控生长棒[MCGR]）治疗脊柱畸形，改善肺功能。关于这个主题的数据有限，本研究的目的是评估这些结构以及胸廓支撑（CWS）的加入对脊柱畸形、胸廓形态和肺部结果的影响。
这是一项对前瞻性收集数据的回顾性分析。我们纳入了因 SMA 导致胸廓畸形和脊柱侧凸的患者，他们接受了生长友好型干预，并进行了两年的随访。进行了描述性统计和单变量分析。
本研究共纳入 66 例患者（25%为 MCGR，73%为 VEPTR，2%为未知）。约 23%的器械包括 CWS。在接受 CWS 的患者中，Cobb 角从 2 年时的 67°（SD：27°）改善至 50°（SD：26°）（p=0.02），在未接受 CWS 的患者中，Cobb 角从 59°（SD：20°）改善至 46°（SD：15°）（p<0.01）。接受和未接受 CWS 治疗的患者的半胸廓高度均有所改善（p=0.01），但仅接受 CWS 治疗的患者的半胸廓宽度有所改善（p=0.01）。1 例接受 CWS 的患者和 2 例未接受 CWS 的患者在 2 年内需要额外的呼吸支持。接受和未接受 CWS 治疗的患者的术后并发症发生率无显著差异（p=0.31）。
生长友好型器械可改善脊柱畸形，并可能有效改变 SMA 患者向呼吸衰竭的进展。与未接受 CWS 治疗的患者相比，接受 CWS 治疗的患者的胸廓形态有显著改善。
