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Scoliosis in Rett’s Syndrome

Alex Gibson FRCS Consultant Spine Surgeon Royal National Orthopaedic Hospital, Stanmore. Rett UK Regional Day Liverpool 2016. Scoliosis in Rett’s Syndrome. Stanmore. What is Scoliosis?. Abnormal lateral curve 3 D deformity Rotational element. Normal Curvatures.

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Scoliosis in Rett’s Syndrome

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  1. Alex Gibson FRCS Consultant Spine Surgeon Royal National Orthopaedic Hospital, Stanmore Rett UK Regional Day Liverpool 2016 Scoliosis in Rett’s Syndrome

  2. Stanmore

  3. What is Scoliosis? • Abnormal lateral curve • 3 D deformity • Rotational element

  4. Normal Curvatures • Normal side profile • Cervical lordosis • Thoracic kyphosis • Lumbar lordosis

  5. Classification • Idiopathic – most common (85%) • No known underlying cause • Neuromuscular (10%) • Cerebral palsy, Rett’s syndrome, Muscular Dystrophy • Congenital • Abnormal ½ vertebrae or unilateral bars • Other syndromic

  6. Scoliosis in Rett Syndrome • Common • Not all Rett girls need surgery • Not present at birth • Develops due to muscle imbalance • High tone • Low tone • Average age onset 7-9

  7. Scoliosis - Detection • Have high index of suspicion • Regular spine examination • X-Ray if thought to be deformity • Refer to local scoliosis service

  8. Scoliosis - Treatment • Encourage walking • Physiotherapy • Joint contractures • Postural management • Good seating • Special Seating Service

  9. Treatments • Bracing • May help seating • Probably doesn’t stop curve progressing • May cause problems – skin pressure, reflux

  10. Scoliosis - Surgery • Curves can be rapidly progressive • Need careful monitoring with X Rays • Surgery considered when >40-50°

  11. Pre-operative assessment • 1 or 2 night stay • Medical • Anaesthetic – sleep study • Surgical • Occupational therapy • Physiotherapy • Dietetics • Nursing

  12. Surgery - Aims • Correct deformity • Balanced Spine • Level Pelvis • Sitting comfort • May change hip / leg position • Fused for long term stabilty • Prevent progression • As safely as possible

  13. Surgery - Risks • Wound problems • Infection, Haematoma, skin breakdown • Bleeding – cell saver / transfusion • Non-Fusion – rod or screw breakage / pullout • Spinal cord damage – Paralysis • Respiratory problems • Long time ITU, Tracheostomy • Others – Death, Blindness, Clots

  14. Surgery - Techniques • All posterior fusion • Ideal – shorter, safer • Anterior and posterior fusion • 2 operations • May be required for large curves • Can be same day or staged 1 week apart

  15. Posterior Fusion • Large incision and exposure • Spinal cord monitoring • Excision of small joints • Pedicle screws or Hooks • Sublaminar wires can be used • Iliac screws if Pelvic fixation • Rods to force spine straight • Donor bone to encourage fusion

  16. Pedicle Screws

  17. Hook

  18. Questions?

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