Section of Pulmonology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
Department of Orthopedic Surgery, Texas Children's Hospital Orthopedic Surgery Service, Baylor College of Medicine, Houston, Texas.
Pediatr Pulmonol. 2020 Apr;55(4):1037-1042. doi: 10.1002/ppul.24664. Epub 2020 Feb 3.
Scoliosis is a common complication of severe neuromuscular diseases. The aim of this study is to determine the impact of posterior spinal fusion on pulmonary function parameters in patients with severe neuromuscular disease at our medical center.
Retrospective chart review of all patients with severe neuromuscular disease who had posterior spinal fusion between 2012 and 2017 at Texas Children's Hospital. Patients with growing rods, brain injury or malformation, and/or spina bifida were excluded. Pulmonary function measures before and after spinal surgery were determined.
A total of 20 eligible patients were identified, 7 with Duchenne muscular dystrophy, 6 with spinal muscular atrophy, 3 with merosin deficient muscular dystrophy, 2 with Charcot-Marie-Tooth, 1 with central core disease, and 1 with dystroglycanopathy. The mean change in vital capacity from pre- to postspine surgery was a loss of 0.63 L for the spinal muscular atrophy patients, a loss of 0.36 L for the Duchenne muscular dystrophy patients, and a gain of 0.23 L for the merosin deficient patients. The difference between spinal muscular atrophy and merosin deficient patients was statistically significant (P = .02) CONCLUSION: In this single-center retrospective study, we found that after spine surgery for scoliosis, all patients with spinal muscular atrophy and most patients with Duchenne muscular dystrophy lost vital capacity, while the patients with merosin deficient muscular dystrophy gained vital capacity. These differences were not associated with differences is respiratory strength, body mass index, or surgical outcomes.
脊柱侧凸是严重神经肌肉疾病的常见并发症。本研究旨在确定我们医疗中心严重神经肌肉疾病患者后路脊柱融合对肺功能参数的影响。
回顾性分析 2012 年至 2017 年在德克萨斯儿童医院接受后路脊柱融合术的所有严重神经肌肉疾病患者的病历。排除使用生长棒、脑损伤或畸形和/或脊柱裂的患者。确定脊柱手术后前后的肺功能测量值。
共确定 20 名符合条件的患者，其中 7 名患有杜氏肌营养不良症，6 名患有脊髓性肌萎缩症，3 名患有 merosin 缺乏性肌营养不良症，2 名患有 Charcot-Marie-Tooth 病，1 名患有中央核疾病，1 名患有 dystroglycanopathy。脊髓性肌萎缩症患者从术前到术后脊柱手术的肺活量平均变化为损失 0.63L，杜氏肌营养不良症患者损失 0.36L，merosin 缺乏症患者增加 0.23L。脊髓性肌萎缩症和 merosin 缺乏症患者之间的差异具有统计学意义（P=0.02）。
在这项单中心回顾性研究中，我们发现脊柱侧凸后路手术后，所有脊髓性肌萎缩症患者和大多数杜氏肌营养不良症患者的肺活量均下降，而 merosin 缺乏性肌营养不良症患者的肺活量增加。这些差异与呼吸肌力、体重指数或手术结果无关。
