Workshop reportMuscular Dystrophy Campaign Funded Workshop on Management of Scoliosis in Duchenne Muscular Dystrophy 24 January 2005, London, UK
Section snippets
Literature review
The literature on this subject consists mainly of retrospective cohort studies, and care is required with their interpretation in the light of the developments in surgical techniques over the years, in particular improvement in the instrumentation and reduction in operating time and peri-operative blood loss. Changes in the natural history of DMD due to improvements in management including the treatment of respiratory and cardiac insufficiency, the use of callipers, and the use of
Is spinal surgery viable in DMD?
There are many years of experience and published case series confirming that spinal surgery is a safe and effective way to manage progressive scoliosis in DMD when it is carried out in experienced centres with multidisciplinary support. Mortality and complication rates in experienced centres and in patients managed by multidisciplinary teams are low and satisfaction levels high.
What is the aim of spinal surgery in DMD?
The primary aim of spinal surgery in DMD is to correct scoliosis and prevent further progression of spinal deformity.
Acknowledgements
This workshop was made possible by the generous contribution of the Muscular Dystrophy Campaign of Great Britain and Northern Ireland. The authors wish to thank all the colleagues, the patient and charity representative for their contribution to this workshop and to the preparation of this report.
List of participants:
- 1.
Peter Baxter, Consultant Pediatric Neurologist, Sheffield
- 2.
Rob Bullock, Consultant Anesthesist, Newcastle
- 3.
Kate Bushby, Consultant Geneticists, Newcastle
- 4.
Nicky Courtier, Pediatric
References (54)
- et al.
Imaging in scoliosis: what, why and how?
Clin Radiol
(2002) - et al.
Long-term results of spine surgery in Duchenne muscular dystrophy
Neuromuscul Disord
(1996) - et al.
Impaired primary hemostasis with normal platelet function in Duchenne muscular dystrophy during highly-invasive spinal surgery
Neuromuscul Disord
(2005) - et al.
Echocardiographic evaluation of left ventricular function in Duchenne's muscular dystrophy
Am J Med
(1980) - et al.
Cardioprotection for Duchenne's muscular dystrophy
Am Heart J
(1999) - et al.
Ventricular arrhythmia in Duchenne muscular dystrophy: prevalence, significance and prognosis
Neuromuscul Disord
(1993) - et al.
Cardiac function, metabolism and perfusion in Duchenne and Becker muscular dystrophy
Neuromuscul Disord
(1996) - et al.
The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy
Int J Cardiol
(1990) - et al.
Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy
Am J Cardiol
(2002) - et al.
Pulsed Doppler tissue imaging in dystrophinopathic cardiomyopathy
J Am Soc Echocardiogr
(2002)
Clinical relevance of plasma brain natriuretic peptide in patients with Duchenne and Becker muscular dystrophy
Neuromuscul Disord
107th ENMC international workshop: the management of cardiac involvement in muscular dystrophy and myotonic dystrophy. 7th-9th June 2002, Naarden, The Netherlands
Neuromuscul Disord
Steroid treatment and the development of scoliosis in males with duchenne muscular dystrophy
J Bone Joint Surg Am
Neuromuscular scoliosis: causes of deformity and principles for evaluation and management
Semin Neurol
Progression of scoliosis in Duchenne muscular dystrophy
J Bone Joint Surg Am
Scoliosis associated with Duchenne muscular dystrophy
J Pediatr Orthop
Lung function in Duchenne muscular dystrophy
Eur Spine J
Spinal stabilisation in Duchenne muscular dystrophy
J Bone Joint Surg Br
The treatment of scoliosis in Duchenne muscular dystrophy
Muscle Nerve
Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy
Neurology
Longitudinal study of spinal deformity in Duchenne muscular dystrophy
J Pediatr Orthop
Prevention of rapidly progressive scoliosis in Duchenne muscular dystrophy by prolongation of walking with orthoses
J Child Neurol
The natural history of spine curvature progression in the nonambulatory Duchenne muscular dystrophy patient
Spine
Orthotic management of scoliosis in Duchenne muscular dystrophy
Arch Phys Med Rehabil
Corticosteroid treatment and functional improvement in duchenne muscular dystrophy: long-term effect
Am J Phys Med Rehabil
Profiles of neuromuscular diseases. Duchenne muscular dystrophy
Am J Phys Med Rehabil
Cited by (27)
The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy
2015, European Journal of Paediatric NeurologyCitation Excerpt :Longer survival is associated with higher incidences of cardiomyopathy and an increasing number of deaths in DMD are now attributed to poor cardiac function.9–11 Orthopaedic complications such as ankle deformities and scoliosis affect the functional abilities of boys with DMD and often require tenotomy or spinal surgery.8,12 However, major surgical procedures pose an increased risk of perioperative morbidity and mortality due to anaesthetic reactions, exacerbation of respiratory failure or worsening of cardiomyopathy.13–15
Neuromuscular scoliosis
2013, Orthopaedics and Traumatology: Surgery and ResearchCitation Excerpt :Table 1 presents the main causes of spinal deviation. The Scoliosis Research Society (SRS) classifies them as neuropathic, with central or peripheral motor neuron involvement or both, or myopathic [1], a heterogeneous class of pathologies of very variable functional impact [2–5]. Such “secondary” spinal deformity occurs much more frequently during the course of these pathologies than does “idiopathic” scoliosis in the general population: prevalence ranges from 25 to 100% according to etiology (Table 2).
Bone Health and Associated Metabolic Complications in Neuromuscular Diseases
2012, Physical Medicine and Rehabilitation Clinics of North AmericaMotor and respiratory heterogeneity in Duchenne patients: Implication for clinical trials
2012, European Journal of Paediatric NeurologyDeflazacort Use in Duchenne Muscular Dystrophy: An 8-Year Follow-Up
2008, Pediatric NeurologyMusculoskeletal Complications of Neuromuscular Disease in Children
2008, Physical Medicine and Rehabilitation Clinics of North AmericaCitation Excerpt :Significant progression of scoliosis is unusual while the child remains ambulant. Rapid progression of scoliosis seems to be related to the loss of walking ability and commonly corresponds with a growth spurt in adolescence [54]. The use of corticosteroids [55,56] and orthotics, such as knee-ankle-foot orthoses [57], have been shown to prolong ambulatory ability.