Developmental Medicine & Child Neurology



Original Articles

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


Kinali  a1, Messina  a1, Mercuri  a1 a2, Lehovsky  a3, Edge  a3, AY Manzur  a1 and Muntoni  a1 c1
a1 Dubowitz Neuromuscular Centre, Department of Paediatrics, Hammersmith Hospital Campus, Imperial College London, UK.
a2 Department of Child Neurology, Catholic University, Rome, Italy.
a3 Department of Orthopaedics, Royal National Orthopaedic Hospital, Stanmore, UK.

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°) in 13% of patients. Another 13% had moderate scoliosis (31–50°) and were managed conservatively. Surgery was considered in 57% (70/123) of patients with scoliosis greater than 50° 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°), 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.

(Published Online May 15 2006)
(Accepted October 3 2005)


Correspondence:
c1 Dubowitz Neuromuscular Centre, Department of Paediatrics, Hammersmith Hospital Campus, Imperial College London, Du Cane Road, London W12 ONN, UK. E-mail: f.muntoni@imperial.ac.uk