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Duchenne Muscular Dystrophy: Orthopaedic Management

Duchenne Muscular Dystrophy: Orthopaedic Management. Introduction. DMD patients have weak bones, especially if on steroids Require enough calcium and vitamin D DMD patients not on steroids have 90% chance of developing progressive scoliosis

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Duchenne Muscular Dystrophy: Orthopaedic Management

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  1. Duchenne Muscular Dystrophy:Orthopaedic Management

  2. Introduction • DMD patients have weak bones, especially if on steroids • Require enough calcium and vitamin D • DMD patients not on steroids have 90% chance of developing progressive scoliosis • Daily steroid treatment reduces the risk of scoliosis, or at least delays its onset • Steroids increase the risk of vertebral fracture

  3. Spinal Care • Should involve an experienced spinal surgeon • Comprises • Monitoring for scoliosis • Support of spinal/pelvic symmetry and spinal extension by the wheelchair seating system • Monitoring for painful vertebral fractures, particularly in patients using steroids

  4. Surveillance (1) • Monitoring for scoliosis • Clinical observation while ambulatory; spinal X-ray only if scoliosis observed • Once non-ambulatory, clinical assessment for scoliosis is essential at each clinic visit • Baseline spinal radiograph assessment should be done around the time of becoming wheelchair dependent, comprising • A sitting anteroposterior full-spine radiograph • A lateral projection film

  5. Surveillance (2) • Follow-up X-rays should be done at least annually if there is a problem. Anteroposterior spinal radiograph is warranted: • Annually for curves of less than 15-20° • every 6 months for curves of more than 20° • Gaps of greater than one year risk missing a worsening of scoliosis • After growth has stopped, X-rays are only needed if there is any change clinically

  6. Prophylaxis • Attention should be paid to posture at all times • Prevention of asymmetrical contractures in ambulant boys • Proper wheelchair seating system, supporting spinal and pelvic symmetry and spinal extension • Spinal bracing is not appropriate to try to delay surgery but may be used if surgery cannot be done, or is not the chosen option.

  7. Treatment (1) • Spinal fusion • To straighten the spine • To prevent further worsening of deformity • To eliminate pain due to vertebral fracture with osteoperosis • To slow rate of respiratory decline • Anterior spinal fusion is inappropriate in DMD

  8. Treatment (2) • Posterior spinal fusion indicated • when Cobb angle >20° • in ambulatory patients who are not on steroids and have yet to reach skeletal maturity • Aim: preserve best posture for comfort and function • With steroids, less risk of deterioration. • Surgery can be left until the Cobb angle > 40° • Operation should be discussed between family and surgeon • Attention required for safe anaesthesia

  9. Bone Health Management • Bone health important in all phases of DMD • Patients have weak bones, especially if on steroids • Low bone mineral density and increased risk of fractures compared to general population • Underlying factors for poor bone health • Decreased mobility • Muscle weakness • Steroid therapy

  10. Bone Health: Interventions • Possible interventions • Vitamin D: needed if genuine deficiency. Supplement should be considered in children • Calcium: intake is best in the diet. Supplements should be consider if this is inadequate, following advice of dietician • Bisphosphonates: IV bisphosphonates recommended for vertebral fractures • Bone density may need assessment with blood tests, bone scans, and other X-rays. Further research required to establish best practice

  11. Long Bone Fracture Management • A broken bone can be a significant threat to the continued ability to walk • Surgery should be considered to allow regained mobility as soon as possible • If fracture occurs, ensure boy’s PT is notified • If an ambulant boy breaks his leg, internal fixation required to resume walking and have best chance of maintaining ambulation • In non-ambulant boys, a broken leg can be safely treated by splinting/casting. • Functional position of limb and possible development of contractures should be taken into account

  12. References & Resources • The Diagnosis and Management of Duchenne Muscular Dystrophy, Bushby K et al, Lancet Neurology 2010 9 (1) 77-93 & Lancet Neurology 2010 9 (2) 177-189 • Particularly references, p186-188 • The Diagnosis and Management of Duchenne Muscular Dystrophy: A Guide for Families • TREAT-NMD website: www.treat-nmd.eu • CARE-NMD website: www.care-nmd.eu

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