Surgery for scoliosis in Duchenne muscular dystrophy

Abstract

BACKGROUND: Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. OBJECTIVES: The objectives of this systematic review were to determine the effectiveness and safety of spinal surgery in Duchenne muscular dystrophy patients with scoliosis. We intended to test whether spinal surgery is effective in increasing life expectancy, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects. SEARCH STRATEGY: We searched the specialized registers of the Cochrane Neuromuscular Disease Group and Cochrane Back Group, the Cochrane Central Register of Controlled Trials (January 2006), MEDLINE (January 1966 to January 2006), EMBASE (January 1980 to January 2006), Dissertation Abstracts International (1861 to Jan 2006), CINAHL (January 1982 to January 2006), and the National Institute of Health Clinical Trials Database (January 2006). No language restrictions were imposed. SELECTION CRITERIA: Controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in patients with Duchenne muscular dystrophy were to be included in the review. The control interventions would have been no treatment, non-operative treatment, or a different form of spinal surgery. DATA COLLECTION AND ANALYSIS: Two authors examined the search results and evaluated the study characteristics against inclusion criteria to decide which ones would be included in the review. MAIN RESULTS: A total of 402 studies were identified from electronic searching. Thirty-six studies were relevant but none met the inclusion criteria for the review, because they were not clinical trials but prospective or retrospective reviews of case series. AUTHORS' CONCLUSIONS: Since there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery in people with Duchenne muscular dystrophy, no evidence-based recommendation can be made for clinical practice. Patients should be informed about the uncertainty of benefits and potential risks of surgery for scoliosis. Randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of patients' quality of life, functional status, respiratory function and life expectancy.