Summary
Over 90% of patients with Duchenne muscular dystrophy develop a scoliosis when they become wheelchair bound. The scoliosis is progressive and is associated with deteriorating lung function. The purpose of this study was firstly to assess whether a standing regimen, in patients who had gone off their feet, protected against the development of scoliosis and affected their lung function, and secondly to evaluate the effect of spinal stabilisation in patients who had developed a progressive scoliosis. The results of the first part of this study showed that a standing regimen significantly delayed the progression of scoliosis and that patients who complied with the standing regimen had a significantly better lung function, as measured by vital capacity and peak expiratory flow rate, than those patients who did not stand. Spinal stabilisation prevented deterioration in the scoliosis, whereas the deformity continued to progress relentlessly in patients who did not undergo surgery. The patients who underwent spinal stabilisation maintained a significantly better lung function and had an improved survival compared with the patients who refused surgery.
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References
Burke SS, Grove NM, Houser CR, Johnson DM (1971) Respiratory aspects of pseudohypertrophic muscular dystrophy. Am J Dis Child 121:230–234
Colbert AP, Craig C (1987) Scoliosis management in Duchenne muscular dystrophy: prospective study of modified Jewett hyperextension brace. Arch Phys Med Rehabil 68:302–304
Dickson RA (1988) The aetiology of spinal deformities. Lancet 1:1151–1155
Galasko CSB (1977) Incidence of orthopaedic problems in children with muscle disease. Isr J Med Sci 13:165–176
Galasko CSB (1987) The orthopaedic management of the dystrophies, myopathies, atrophies, neuropathies and ataxias. In: Galasko CSB (ed) Neuromuscular problems in orthopaedics. Blackwell Scientific, Oxford, pp 83–105
Galasko CSB, Delaney C, Morris P (1992) Spinal stabilisation in Duchenne muscular dystrophy. J Bone Joint Surg [Br] 74: 210–214
Gardner-Medwin D (1977) Management of muscular dystrophy. Physiotherapy 63:46–51
Gardner-Medwin D (1980) Clinical features and classification of the muscular dystrophies. Br Med Bull 36:109–115
Gibson DA, Wilkins KE (1975) The management of spinal deformities in Duchenne muscular dystrophy: a new concept of spinal bracing. Clin Orthop 108:41–51
Gibson DA, Koreska J, Robertson D, Khan A III, Albisser AM (1978) The management of spinal deformity in Duchenne's muscular dystrophy. Orthop Clin North Am 9:437–450
Hsu JD (1983) The natural history of spine curvature progression in the non-ambulatory Duchenne muscular dystrophy patient. Spine 8:771–775
Jenkins JG, Bohn D, Edmonds JF, Levison H, Barker GA (1982) Evaluation of pulmonary function in muscular dystrophy patients requiring spinal surgery. Crit Care Med 10:645–649
Kurz LT, Mubarak SJ, Schultz P, Park SM, Leach J (1983) Correlation of scoliosis and pulmonary function in Duchenne muscular dystrophy. J Pediatr Orthop 3:347–353
Miller G, Dunn N (1982) An outline of the management and prognosis of Duchenne muscular dystrophy in Western Australia. Aust Paediatr J 18:277–282
Miller G, O'Connor J (1985) Spinal bracing and respiratory function in Duchenne muscular dystrophy (letter). Clin Pediatr (Phila) 24:94
Miller RG, Chalmers AC, Dao H, Filler-Katz A, Holman D, Bost F (1991) The effect of spinal fusion on respiratory function in Duchenne muscular dystrophy. Neurology 41:38–40
Noble-Jamieson CM, Heckmatt JZ, Dubowitz V, Silverman M (1986) Effects of posture and spinal bracing on respiratory function in neuromuscular disease. Arch Dis Child 61:178–181
Rennie MJ, Edwards RHT, Millward DJ et al (1982) Effects of Duchenne muscular dystrophy on muscle protein synthesis. Nature 296:165–167
Rideau Y, Glorion B, Delaubier A, Tarle O, Bach J (1984) The treatment of scoliosis in Duchenne muscular dystrophy. Musc Nerve 7:281–286
Robin GC (1975) Scoliosis and neurological disease. Wiley, New York
Rodillo EB, Fernandez-Bermejo E, Heckmatt JZ, Dubowitz V (1988) Prevention of rapidly progressive scoliosis in Duchenne muscular dystrophy by prolongation of walking with orthoses. J Child Neurol 3:269–274
Sakai DN, Hsu JD, Bonnett CA, Brown JC (1977) Stabilisation of the collapsing spine in Duchenne muscular dystrophy. Clin Orthop 128:256–260
Sibert JR, Williams V, Burkinshaw R, Sibert S (1987) Swivel walkers in Duchenne muscular dystrophy. Arch Dis Child 62: 741–742
Siegel IM (1975) Plastic moulded knee-ankle-foot orthoses in the treatment of Duchenne muscular dystrophy. Arch Phys Med Rehabil 56:322
Siegel IM (1978) The management of muscular dystrophy: a clinical review. Musc Nerve 1:453–460
Smith PEM, Calverley PMA, Edwards RHT, Evans GA, Campbell EJM (1987) Practical problems in the respiratory care of patients with muscular dystrophy. N Engl J Med 316: 1197–1205
Vignos PJ, Archibald KC (1960) Maintenance of ambulation in childhood muscular dystrophy. J Chron Dis 12:273–290
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Paper read at the ESDS meeting, Birmingham 1994, and selected for full publication.
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Galasko, C.S.B., Williamson, J.B. & Delaney, C.M. Lung function in Duchenne muscular dystrophy. Eur Spine J 4, 263–267 (1995). https://doi.org/10.1007/BF00301031
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DOI: https://doi.org/10.1007/BF00301031