神经肌肉疾病患者脊柱畸形的病因学认识

E. Filatov
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摘要

目的:了解脊柱畸形的病因是正确治疗神经肌肉疾病患者脊柱畸形的基石。方法:对71例进行性脊柱畸形进行分析。他们都表现出不同形式的神经肌肉紊乱。我们继续应用主要基于不同类型NMS的病因诊断(临床表型和基因型)的快速评估标准。在评估功能独立性测量(FIM)时,我们观察到某些方面的恶化。在两种情况下评估Cobb角,即矫正手术前和术后结果。术后1年再评估。通过后路椎弓根螺钉固定进行手术矫正。结果:手术矫正c型胸腰椎侧凸。腰椎前凸过大在脑瘫患者中很常见,而胸后凸减小甚至胸前凸在杜氏肌萎缩症患者中更常见。根据功能独立性测量(FIM),功能状态有明显改善。结论:了解病因是对进行性脊柱畸形患者进行脊柱矫正手术的首要因素。我们的结论是,刚性前凸一直是并且仍然是神经肌肉疾病患者遇到的关键问题。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The Etiological Understanding of Spine Deformities in Patients with Neuromuscular Disorders
Purpose: The etiology understanding is the corner stone for proper management of the spine deformities in patients with neuromuscular disorders. Methods: Progressive spine deformities have been encountered in 71 patients. All showed different forms of neuromuscular disorders. We proceeded with applying prompt evaluation criteria which was mainly based on the etiological diagnosis of each type of NMS (clinical phenotype and genotype). Assessing the Functional Independence Measure (FIM), we observed deterioration in some aspects. The Cobb’s angle was assessed in two occasion’s i.e prior to corrective surgeries plus the post-surgery results. Followed by one year postoperative re-assessment. Surgical corrections have been performed via posterior pedicle screw fixation. Results: Surgical correction to re-align the C-shaped thoracolumbar scoliosis. Lumbar hyper lordosis were common in cerebral palsy patients, though decreased thoracic kyphosis or even thoracic lordosis occurs more frequently in Duchenne Muscular atrophy patients. There was noticeable improvement in functional status according to Functional Independence Measure (FIM). Conclusion: Etiology understanding is the paramount element in re-aligning corrective surgeries of the spine in patients with progressive spine deformities. We concluded that rigid hyper lordosis has been and still the key problem encountered in patients with neuromuscular disorders.
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