Process measures and patient/parent evaluation of surgical management of spinal deformities in patients with progressive flaccid neuromuscular scoliosis (Duchenne's muscular dystrophy and spinal muscular atrophy).

摘要

STUDY DESIGN: 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. OBJECTIVE: 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. SUMMARY OF BACKGROUND DATA: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.