Management of scoliosis in Duchenne muscular dystrophy: a large 10-year retrospective study

Abstract

Scoliosis affects 75 to 90% of patients with non-ambulant Duchenne muscular dystrophy (DMD). Spinal surgery is the treatment of choice but the indication varies among centres. Some offer surgery to all non-ambulant patients, irrespective of scoliosis severity. Early surgery has the advantage of targeting DMD when cardiorespiratory function is preserved, but not all patients develop scoliosis. We report our 10-year experience of scoliosis management in 123 patients with DMD who were at least 17 years old at the time of the study. Scoliosis was absent in 10%, and mild, non-progressive (at least 30 degrees ) in 13% of patients. Another 13% had moderate scoliosis (31-50 degrees ) and were managed conservatively. Surgery was considered in 57% (70/123) of patients with scoliosis greater than 50 degrees and eventually performed in 35%. The remaining patients either refused surgery (9%) or were unfit because of cardiorespiratory compromise (13%). In a further 7%, scoliosis (greater than 50 degrees ), first noted after 14 years of age, was progressing slowly and surgery was not performed. At 17 years there was no difference in survival, respiratory impairment, or sitting comfort among patients managed conservatively or with surgery. One-third (44/123) of our patients were managed satisfactorily without receiving spinal surgery. We provide insight into the natural history of scoliosis in DMD that should help families and clinicians with decision-making when surgery is considered.

Spinal stability is improved by inducing a lumbar lordosis in boys with Duchenne Muscular Dystrophy: A pilot study

“…Surgery is currently the treatment of choice to arrest the development of scoliosis. However, a recent study of 123 patients suggested that a substantial number of boys require conservative management because of a milder scoliosis, patient choice or cardiorespiratory compromise precluding surgery [1]. This finding supports the potential significance of other techniques of spinal stabilisation in DMD boys, one of which we address in this study.…”

“…Between 70% and 90% of boys with Duchenne Muscular Dystrophy (DMD) develop progressive scoliosis [1]. Surgery is currently the treatment of choice to arrest the development of scoliosis.…”

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

“…312 Although studies have reported potential advantages of spinal surgery, including increased comfort and sitting tolerance, cosmetic improvement, and pain relief, [313][314][315][316] there is no clearly demonstrated effect of spinal fusion on the natural deterioration of respiratory function in DMD. [316][317][318][319][320][321] There is also debate about improvements in life expectancy, with both lower mortality and no difference in mortality after spinal surgery for DMD having been reported. 317,318,321,322 …”

“…[316][317][318][319][320][321] There is also debate about improvements in life expectancy, with both lower mortality and no difference in mortality after spinal surgery for DMD having been reported. 317,318,321,322 …”

Musculoskeletal Complications of Neuromuscular Disease in Children

“…In neuromuscular scoliosis, the situation is even more complex. Scoliosis 38%-64% [5,6] 20%-94% [7] 63%-90% [8,9] 100% [10] (if injured before adolescent growth spurt)…”