1. Physiotherapy Management of Neuromuscular Scoliosis
Hannah Waugh
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
14yrs 6mth
66 o
108 o
Pelvis ? o
S.G., ♂

7. Progression of curve – 4 months
14yrs 6mth
14yrs 10mth
66 o
58 o
108 o
122 o
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 Clinic Assessment
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
Physical assessment
Asymmetry of trunk
Uneven Shoulders
Uneven Pelvis
Listing to one side
Adam’s Forward Bending
Test Thoracic prominence
Lumbar prominence
Leg Length
Plumb line
From C7 spinous process to hang below buttocks
Neurological examination
Pain
Numbness
Tingling
Weakness
Muscle spasm
Bowel/bladder changes
To ensure that the patient can tolerate reconstructive spinal surgery, a detailed preoperative history and assessment should include an evaluation of respiratory competency, cardiac status, nutrition, possible feeding difficulties, seizure disorders, urologic status, and metabolic bone disease.[19]
Patients capable of cooperating should be evaluated with pulmonary function studies. Patients with vital capacities less than 30% of the predicted reference value may require postoperative ventilatory support. Performing formal pulmonary function testing is difficult in patients with neuromuscular scoliosis because patients are often unable to cooperate.[20]
Patients with Duchenne muscular dystrophy and Friedreich ataxia should be evaluated for cardiac involvement.[21]
Poor nutritional status is strongly linked to perioperative complications in these patients. Nutritional deficiencies should be corrected preoperatively through a forced nutritional improvement schedule or postoperatively with feeding tubes. Elective placement of gastric feeding tubes 3 months preoperatively dramatically improves nutritional status. The use of total parenteral nutrition (TPN) perioperatively also can be helpful in decreasing problems with wound infections.[22, 23]

11. Cobb Angle
This is taken to monitor progression but is only one part of the assessment tool. Not everybody about a certain angle gets PSF.

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
Lots of contradictory information out there – Does PSF improve respiratory function?, Does PSF stop respiratory function getting worse?
Unsure???
Functional Ability – use of ULs and flexion of the spine to feed/ play etc if flexion of spine is prevented - ?function is reduced
- can get mobile arm supports to overcome this problem.
Symptoms – PAIN – very justified to undertake PSF due to pain, particularly if some children have been down the pain management route.
- positioing, not as big a problem compared to the children with CP due to their size etc
Quality of Questionnaire – not scoring well
Reduction in Surgery – Due to the above surgery has decreased from about 5-6/yr to 1/yr

15. Medical Management - CP
Respiratory Function
Functional Ability
Symptoms
Quality of Life questionnaire
Surgery
Respiratory Function
Functional Ability – probably more often than not will improve their function due to the position change
Symptoms – Pain, Positioning, hygiene
- a lot of children with hip dislocation with a severe pelvic obliquity, PSF is required first to rectify the pelvic obliquity before pelvic and femoral osteotomies can be undertaken

16. Medical Management - mylominingecele
Respiratory Function
Functional Ability
Symptoms
Surgery
Respiratory Function
Functional Ability – floor work, often spinal surgeons request an OT and PT assessment to gauge how much function they have on the floor
Symptoms – No pain

17. Medical Management Every case is very individual Function
Medical Stability
MDT decision
Not just the size of the curve is considered.
This is a major operation and at stages different things can go wrong

18. Medical Management Continue to monitor curve
Use of conservative treatment
PSF
Intensivist
Cardiac Consultant
Neurologist

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
Spinal service relies on local therapists/ nursing teams and services to arrange provision of equipment locally

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
Manual Lifts are not recommended for a year post surgery.

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 (
Scottish Muscle Network DMD Profile (
Peak cough flow can be assessed by using a mask and a peak flow meter,
Comprehensive information on suitable respiratory techniques for neuromuscular patients can be found on the British Thoracic Society Website ( under Guidelines for the Physiotherapy Management of the Adult, Medical, Spontaneously breathing patient. The Scottish Muscle Network has also published a Paediatric Management of Duchenne Muscular Dystrophy Guideline ( where additional respiratory management guidelines can be obtained.
It is extremely beneficial if families have been taught how to perform lung volume recruitment techniques and chest clearance techniques appropriate to the patient’s stage of their condition prior to their admission for spinal fusion.
Patients will not be discharged home following spinal surgery until their chest condition is stable and families are able to manage any respiratory problems with some support from local care teams. The family will be more confident in managing any post-op short-term deterioration in respiratory function with support from the local physio if they have prior respiratory experience.
Familiarisation with face mask in preparation for potential use of Bipap/ Cough Assist.
Require 2.7l/min to clear secs
1.60l/min can clear secs with breath stacking and assisted cough
Less than 1.6l/min – may benefit from cough assist

30. Hospital Admission Usually admitted the day prior to surgery
Introduction/assessment by inter-disciplinary team
Discussion of post operative management
If bowels need prepped then admitted a couple of days prior to surgery.

31. Operation – Posterior Spinal Fusion
15yrs 1mth
40 o
Combined anterior and posterior fusion is common in the treatment of patients with neuromuscular scoliosis, either because posterior elements are absent, as in myelodysplasia, or because it is necessary to gain correction in a rigid lumbar or thoracolumbar curve and achieve a spine fused in balance over a level pelvis
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
Indicated when scoliosis is very large, rib hump very pronounced.
Skeletally very immature patients who may develop a “crankshaft deformity”
When the posterior spine is fused and the anterior spine is left to grow.

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
Ileus: Intestinal hypomotility may persist, necessitating prolonged parenteral support.
Nutritional problems: When intestinal motility returns postoperatively but the child cannot tolerate oral feedings, a feeding tube can be passed into the stomach or duodenum to allow nutritional support until oral feeding is tolerated

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
Feeding crucial to promote good healing, however complications often occur such as SMA.
Mobility may not be as good as before initially.
Hydrotherapy is not allowed.
Some kids are not allowed to self propel post op.

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