Physiotherapy Management of Neuromuscular Scoliosis

1. Physiotherapy Management of Neuromuscular Scoliosis Hannah Waugh 0131 536 0000 Bleep 9126 Specialist Physiotherapist, The Royal Hospital for Sick Children, Edinburgh

2. Contents • What is Scoliosis? • Medical Management • Pre Operative Planning • Hospital Admission • Challenges post discharge

3. What is Scoliosis? • Complex three dimensional deformity where the curve is greater than 10 degrees

4. Prevalence of Neuromuscular Scoliosis • 20% of children with Cerebral Palsy • 60% of children with Myelodysplasia • 90% of children with Duchenne Muscular Dystrophy

5. Neuromuscular Scoliosis Development • Spinal curvature may begin very early in life • Often after the patient starts supported sitting • Curve may progress rapidly once patient becomes non ambulant (averaging 10 degrees/year)

6. Initial Assessment 06.02.2007 14yrs 6mth 66 o 108 o Pelvis ? o S.G., ♂

7. Progression of curve – 4 months 06.02.2007 14yrs 6mth 26.06.2007 14yrs 10mth 66o 58o 108o 122o Pelvis ? o Pelvis 34 o S.G., ♂

8. Preventing Progression of Scoliosis • Prolong mobility • Steroids • 24 hour postural management • Spinal bracing (not always effective particularly in progressive neuromuscular curves)

9. Referral Criteria • Consultant to consultant referral only • Confirmed scoliosis - requesting specialist assessment for surgical intervention • Neurological – usually after the age of 10 as surgery unlikely prior to this • DMD – when patient becomes non ambulant

10. Initial Spinal ClinicAssessment • In-depth history is taken • scoliosis progression, pain, function • past medical history • medication • social history • Objective Assessment • X-rays : standing or sitting to establish severity, bending films to identify flexibility – cobb angle, also check risser grade

11. Cobb Angle

12. Medical Management • Dependent on: • Severity of scoliosis • Pelvic obliquity • Age/Skeletal maturity – risser grade • Rib deformity/ Impingement/ Pain • Complexity of past medical history

13. Medical Management • Cardiac • Respiratory • Anaesthetics • Neurology/ Neurosurgery • Endocrinology • GI

14. Medical Management - DMD • Respiratory Function • Functional Ability • Symptoms • Quality of Life questionnaire • Reduction in surgery

15. Medical Management - CP • Respiratory Function • Functional Ability • Symptoms • Quality of Life questionnaire • Surgery

16. Medical Management - mylominingecele • Respiratory Function • Functional Ability • Symptoms • Surgery

17. Medical Management • Every case is very individual • Function • Medical Stability • MDT decision

18. Medical Management • Continue to monitor curve • Use of conservative treatment • PSF

19. Physiotherapy Service Aims • To ensure smooth pathway from pre admission to discharge • To be available for contact to reduce any anxieties throughout the patient journey • To be a resource for local therapists / services for Scotland

20. Spinal Surgery Pathway Contact made with local services & family Pre-op assessment completed Theatre list to Physio & OT Equipment requirements identified & commenced Local services review Discharge Admission Post-op

21. Physiotherapy Role • To ensure that optimal functional abilities are achieved post operatively • Those functional abilties include: • respiratory function • muscle strength • transfers/ mobility • postural management • Overall aim is to maximise independence following surgery in activities of daily living • Postural management is vital and should be considered through out all stages of spinal surgery

22. Physio Pre op Planning • Commenced as soon as the patient is listed for theatre (approx 6 weeks) • Facilitate smooth admission and discharge from hospital • Early contact with local services is essential

23. Pre-operative Planning • Unfortunately due to geographic location of clinics, unable to attend • Contact will usually be made with the family and local therapists initially by telephone • If patients admitted for respiratory tests, trial of NIV or attend for anaesthetic assessment we will meet and assess on ward if possible

24. Initial Pre-Op Assessment Physio /OT • Establish current abilities of • Seating (wheelchairs,other seating systems school, home) • Transfers (independent, assisted, hoist) • Mobility- use of walking aids • Personal Hygiene (toileting, bathing/showering, level of assistance ,specific equipment) • Respiratory function • Other ADL activities (feeding, self dressing) • School • Environmental issues (access to and within house)- child may need to live downstairs

25. Seating • Wheelchairs • Should be in suitable corrective seating system pre op- consider lateral supports, harness & head support • Tilt & recline facilities recommended pre-op for any patient with scoliosis (Bushby et al, 2005) • Tilt & recline vital post op if fused to pelvis • Moulded wheelchairs are not appropriate post op • Local services to review post op to ensure corrective seating system

26. Seating • If fused to pelvis other seating systems can be used if have recline • Local therapists to review postural support from seating systems post op • Post op head rests, lateral supports, harnesses will still be required to maintain optimal postural alignment • Sofas, beanbags are not acceptable seating systems!

27. Transfers • Hoisting • Children that are lifted pre-op may require to be hoisted • Hoisting is dependent on age, size, weight and complexity • High backed slings with head support recommended • Bones in slings not necessary • Thinner sling ideal- will be left in situ initially • Remember to consider that child may require increased sling length post op • Responsibility of local services to provide hoist training if new/ different equipment has been supplied

28. Personal Care • Toileting • Ideal is recline & tilt- limited resources may result in tilt only • Showering • Recommended in acute post op period • Alternative shower chair may be required for postural support • Bathing • Long term extra postural support in bath may be required

29. Pre-operative Respiratory Function • Extremely beneficial if families have been taught lung volume recruitment techniques and chest clearance techniques prior to admission • British Thoracic Society (www.brit-thoracic.org.uk • Scottish Muscle Network DMD Profile (www.smn.scot.nhs.uk) • Peak cough flow can be assessed by using a mask and a peak flow meter,

30. Hospital Admission • Usually admitted the day prior to surgery • Introduction/assessment by inter-disciplinary team • Discussion of post operative management

31. Operation – Posterior Spinal Fusion 20.09.2007 15yrs 1mth 40 o 62 o Pelvis 6 o S.G., ♂

32. Posterior Spinal Fusion +/- pelvic fixation • Performed via a large midline incision • Spinous processes, interspinous ligaments and facet joints excised • Pedicle Screws or hooks attached to spine • If fusing to the pelvis wires or pelvic screws are placed • Rods applied down either side of the spine and attached to screws and hooks as spinal deformity derotated • Bone grafts placed around rods – usually femoral heads from bone bank or bone substitutes • Wound is closed with redivac drain insitu

33. Anterior Release +/- posterior spinal fusion • Performed via a thoracotomy – on the convexity of scoliosis • A rib is excised for most of its length to access spine (and kept) – rib resection • Rib heads may be removed around the apex of the scoliosis to improve cosmetic result – internal costoplasty • Pleura is excised • Discs are excised and growth plates, cartilage removed • Wound closed with intercostal chest drain insitu

34. In patient Physiotherapy • Reviewed day one post op • Chest physiotherapy commenced • Passive/active assisted movements • Bed mobility – log rolling • Mobility/ hoisting once medically stable • Liaison with local therapists • Ongoing until discharge from hospital

35. Acute Post Op Challenges • Surgical considerations – e.g. pelvic fixation- reclining seating positions • Medical stability – e.g. respiratory distress • Comfort – pain control • Tone • Psychosocial – anxiety • Nutrition

36. Discharge Advice • Advise parents to cont passive/active assisted movements • To increase mobility or duration sitting in wheelchair • If wheelchair reclined- to reduce recline as tolerated • To ensure postural alignment maintained – avoid forced flexion/ extension or rotation of spine • Ongoing respiratory management – as required

37. Discharge Advice • Unable to use standing frame and some walking aids • Unable to swim/ hydrotherapy/ participate in sports • Discretion of Consultant on reviewing patient and x-rays at clinic

38. School • ASL Profile provided • Return to School – graded • School seating • Desk height/ position • Hand function – writing skills • Manual handling/hoisting • Toileting • Feeding

39. Challenges after Discharge • Home Environment • Mobility • Self propelling wheelchairs • Change to Physiotherapy Program – Hippotherapy, Rebound etc • Feeding • Family Support • Transport • Holidays • Anxieties

40. Conclusion • There is variability with each child and we aim to make the pathway as smooth as possible for the patient / carers and local therapists