Bridwell K H, Baldus C, Iffrig T M, Lenke L G, Blanke K
Washington University School of Medicine, Department of Orthopaedic Surgery, St. Louis, Missouri, USA.
Spine (Phila Pa 1976). 1999 Jul 1;24(13):1300-9. doi: 10.1097/00007632-199907010-00006.
Fifty-four consecutive patients with flaccid neuromuscular scoliosis (33 with Duchenne's muscular dystrophy, 21 with spinal muscular atrophy) who had undergone surgery for their disorder between 1985 and 1995 were sent questionnaires to evaluate function, self-image, cosmesis, pain, pulmonary status, patient care, quality of life, and satisfaction. Forty-eight patients returned the questionnaires.
To assess patient/parent satisfaction and clinical/functional ways in which spinal fusion helped or did not benefit these patients and to assess complications and the ultimate radiographic result.
There are only a few reports of results of spinal fusion and segmental instrumentation for flaccid neuromuscular disorders. There are no published reports regarding patient/parent evaluation of the procedure.
Results of the questionnaire were tallied, graded, and divided into eight categories. The questionnaire was validated by a Cronbach's alpha analysis, a test-retest, and a comparison with questionnaire answers from patients with idiopathic scoliosis. Radiographic data and complications also were accumulated. The follow-up periods after surgery ranged from 2 to 12.6 years (average, 7.8 years).
Except for two patients who died within 3 months of surgery, all patients seemed to have benefited from the surgery. Cosmesis, quality of life, and overall satisfaction rated the highest.
These data indicate that spinal fusion with segmental spinal instrumentation benefits most patients with Duchenne's muscular dystrophy or spinal muscular atrophy with spinal deformities in terms of all categories assessed, even though these diseases have a progressively deteriorating course.
选取了1985年至1995年间因弛缓性神经肌肉性脊柱侧弯接受手术治疗的54例连续患者（其中33例患有杜氏肌营养不良症，21例患有脊髓性肌萎缩症），向他们发送问卷以评估功能、自我形象、美观、疼痛、肺部状况、患者护理、生活质量和满意度。48例患者回复了问卷。
评估患者/家长的满意度以及脊柱融合术对这些患者有帮助或无益处的临床/功能方面，并评估并发症和最终的影像学结果。
关于弛缓性神经肌肉疾病的脊柱融合术和节段性内固定术的结果报告较少。尚无关于患者/家长对该手术评估的发表报告。
对问卷结果进行统计、评分并分为八类。通过克朗巴哈系数分析、重测以及与特发性脊柱侧弯患者的问卷答案进行比较来验证问卷。还积累了影像学数据和并发症情况。术后随访时间为2至12.6年（平均为7.8年）。
除了两名在术后3个月内死亡的患者外，所有患者似乎都从手术中获益。美观、生活质量和总体满意度评分最高。
这些数据表明，对于患有杜氏肌营养不良症或脊髓性肌萎缩症并伴有脊柱畸形的大多数患者，节段性脊柱内固定的脊柱融合术在所有评估类别方面均有益处，尽管这些疾病呈进行性恶化病程。
