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Surgery for scoliosis in Duchenne muscular dystrophy

  • Review
  • Intervention

Authors


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. This is an updated review and an updated search was undertaken in which no new studies were found.

Objectives

The objectives of this systematic review were to determine the effectiveness and safety of spinal surgery in people with Duchenne muscular dystrophy with scoliosis. We intended to test whether spinal surgery is effective in increasing survival, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects.

Search methods

We searched the specialized registers of the Cochrane Neuromuscular Disease Group (July 2010) and Cochrane Back Group (August 2010), MEDLINE (1966 to July 2010), EMBASE (1947 to July 2010), Cochrane Central Register of Controlled Trials (CENTRAL) (July 2010), CINAHL (1982 to July 2010), Proquest Dissertation and Thesis Database (1980 to August 2010), and the National Institute of Health Clinical Trials Database (August 2010). No language restrictions were imposed.

Selection criteria

We planned to include controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in people with Duchenne muscular dystrophy 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

On electronic search, 45 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. People with scoliosis 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 quality of life, functional status, respiratory function and life expectancy.

Plain language summary

There is currently no high quality evidence for scoliosis surgery for people with Duchenne muscular dystrophy

Scoliosis, curvature of the spine, is common in people with Duchenne muscular dystrophy. It is usually progressive and surgery is often performed aiming to halt its progression, improve cosmetic appearance, facilitate care, preserve upper limb and respiratory function, and hopefully increase life expectancy. However, there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery. Randomized controlled clinical trials are needed in this group of patients to evaluate the benefits and risks of different surgical treatments. This is an updated review and an updated search was undertaken in which no new studies were found.

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