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Physiotherapy Management of Neuromuscular Scoliosis

Hannah Waugh0131 536 0000 Bleep 9126Specialist Physiotherapist,

The Royal Hospital for Sick Children, Edinburgh

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

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What is Scoliosis?

• Complex three dimensional deformity where the curve is greater than 10 degrees

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Prevalence of Neuromuscular Scoliosis

• 20% of children with Cerebral Palsy

• 60% of children with Myelodysplasia

• 90% of children with Duchenne Muscular Dystrophy

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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)

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Initial Assessment06.02.200714yrs 6mth

108 o

S.G., ♂

66 o

Pelvis ? o

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06.02.200714yrs 6mth

108 o

S.G., ♂

66 o

Pelvis ? o

26.06.200714yrs 10mth

122 o

58 o

Pelvis 34 o

Progression of curve – 4 months

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Preventing Progression of Scoliosis• Prolong mobility

• Steroids

• 24 hour postural management

• Spinal bracing (not always effective particularly in progressive neuromuscular curves)

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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

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• 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

Initial Spinal Clinic

Assessment

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Cobb Angle

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Medical Management• Dependent on:

– Severity of scoliosis – Pelvic obliquity– Age/Skeletal maturity – risser grade– Rib deformity/ Impingement/ Pain– Complexity of past medical history

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

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

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

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

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

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

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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

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Spinal Surgery Pathway

Theatre list to Physio & OT

Contact made with local services & family Pre-op

assessment completed

Equipment requirements identified & commenced

AdmissionPost-op

Discharge

Local services review

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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

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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

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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

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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

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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

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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!

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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

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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

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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,

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Hospital Admission• Usually admitted the day prior to surgery

• Introduction/assessment by inter-disciplinary team

• Discussion of post operative management

31S.G., ♂

Operation – Posterior Spinal Fusion20.09.200715yrs 1mth

62 o

40 o

Pelvis 6 o

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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

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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

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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

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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

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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

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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

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School• ASL Profile provided

• Return to School – graded

• School seating• Desk height/ position• Hand function – writing skills• Manual handling/hoisting• Toileting

• Feeding

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Challenges after Discharge• Home Environment• Mobility• Self propelling wheelchairs• Change to Physiotherapy Program – Hippotherapy,

Rebound etc• Feeding• Family Support• Transport• Holidays• Anxieties

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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

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