pediatric update 2013 cerebral palsy objectives - etouches · pdf filepediatric update 2013...

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Pediatric Update 2013 Cerebral Palsy

September 20, 2013Matthew Prowse, MD, FRCPC

Physical Medicine and Rehabilitation

Objectives

The participant will recognize the spectrum of Cerebral Palsy reflected in consensus definition and the current profile of Cerebral Palsy in Canada.

The participant will learn how classification systems of motor function inform prognosis

The participant will be familiar with recent guidelines for hip surveillance and bone density management in cerebral palsy

The participant will recognize the growing role for robotics and virtual reality in rehabilitation and how emerging technologies aim to further improve motor function for individuals with cerebral palsy.

Epidemiology

Largest single cause of childhood physical disability in the developed world

Overall prevalence ~2-3.5/1000 live births

Increased through 1970-1980’s which paralleled trends in survival for premature and low birth weight infants

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Cerebral Palsy: Definition

“Cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.

The motor disorders…are often accompanied by disturbances of sensation, perception, cognition, communication, and behaviour, by epilepsy and by secondary musculoskeletal problems”

The Definition and Classification of Cerebral Palsy. Dev Med Child Neurol. 2007 Feb;49(s109):1-44. PubMed PMID: 17371509

Permanent disorders

Movement and posture

Activity limitation

Non-progressive disturbance/injury

Secondary effects may be progressive

Effects of aging may lead to progressive functional impairment

Sensation

Impaired stereognosis and 2 point discrimination in 40-50%

Perception

Severe visual impairment in 20%

Cognition

Learning disability in 40%

Communication

Impaired in 40-80%

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Behaviour

5x risk of behaviour problems

Epilepsy

30-40%

Secondary musculoskeletal problems

Torsional deformities, contractures, hip subluxation, foot deformities

GMFCS

Gross Motor Function Classification System

5 Levels of function based on gross motor function measure (GMFM) scores

Classification meant to represent current usual performance (rather than capability)

5 Age bands – before age 2, 2-4 years, 4-6 years, 6-12 years, 12-18 years

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Severity by GMFCS Level

0

5

10

15

20

25

30

GMFCS 1 GMFCS 2 GMFCS 3 GMFCS 4 GMFCS 5

% o

f In

divi

dual

s w

ith

Cer

ebra

l P

alsy

Stability of GMFCS

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GMFM in Adolescence

Longitudinal study of subjects from the Ontario Motor Growth study

•Hanna SE, Rosenbaum PL, Bartlett DJ, Palisano RJ, Walter SD, Avery L, Russell DJ. Stability and decline in gross motor function among children and youth with cerebral palsy aged 2 to 21 years. Dev Med Child Neurol. 2009 Apr;51(4):295-302.

GMFM in Adolescence

Drop in GMFM-66 score related to:

Limited range of motion (hip, knee, ankle, upper extremity)

Spinal alignment

Pain

Not necessarily reflected in participation

Exercise participation low overall

•Bartlett DJ, Hanna SE, Avery L, Stevenson RD, Galuppi B. Correlates of decline in gross motor capacity in adolescents with cerebral palsy in Gross Motor Function Classification System levels III to V: an exploratory study. Dev Med Child Neurol. 2010 Jul;52(7):e155-60.

Locomotion Skills

Retrospective survey of adults with cerebral palsy

27% reported improvement in walking skills over time

Primarily before age 25

28% no change

44% reported deterioration

10% had lost their walking skills

•Jahnsen R, Villien L, Egeland T, Stanghelle JK, Holm I. Locomotion skills in adults with cerebral palsy. Clin Rehabil. 2004 May;18(3):309-16

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Locomotion Skills

Self-reported causes of deterioration

Pain

Fatigue

Lack of adapted physical activity

Deterioration strongly associated with

Older age

Delayed walking debut

Severe neurological impairment

•Jahnsen R, Villien L, Egeland T, Stanghelle JK, Holm I. Locomotion skills in adults with cerebral palsy. Clin Rehabil. 2004 May;18(3):309-16

Gait deterioration may occur in two peaks

Adolescence/young adult

Progressive crouch gait

Inability to keep up with peers

Age 40-45 Progressive fatigue

Pain

Joint deterioration

Lower risk of deterioration in highest functioning groups

Murphy KP. The adult with cerebral palsy. Orthop Clin North Am. 2010 Oct;41(4):595-605.

The Hip in Cerebral Palsy

Estimated frequency of hip displacement in cerebral palsy 2-75%

Etiology

Spasticity and/or contractures of hip flexors and adductors, and medial hamstrings

Osseous deformities due to lack of weightbearing: femoral anteversion, acetabular dysplasia

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Hip displacement defined by migration percentage: percentage of femoral head lateral to Perkin’s Line

>30% = subluxed

>100% = dislocated

Also known as migration index/Reimer’s index

Canale & Beaty: Campbell's Operative Orthopaedics, 11th ed. http://www.mdconsult.com/das/book/body/130696238-3/827516715/1584/221.html#4-u1.0-B978-0-323-03329-9..50033-7--cesec34_1228

Fig. 30-10 Subluxated left hip joint. Migration index (MI) is calculated by dividing width of uncovered femoral head A by total width of femoral head B. Acetabulum is dysplastic (type 2 sourcil), with lateral corner of acetabulum above weight bearing dome. Normal hip (left side) with acetabular index (AI) indicated. There is normal (type 1) sourcil; lateral corner is sharp and below weight bearing dome. H, horizontal axis. (From Flynn JM, Miller F: Management of hip disorders in patients with cerebral palsy, J Am Acad Orthop Surg 10:198, 2002.)

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Incidence of hip “displacement” (MP >30%) directly related to GMFCS level (overall 35%)

Soo B, Howard JJ, Boyd RN, et al. Hip displacement in cerebral palsy. J Bone Joint Surg Am 2006; 88: 121–29.

Management of the Subluxed Hip

Migration percentage

>20% - consider Botox and/or phenol, adductor and/or psoas and/or medial hamstring release/lengthening

>40% - will progress to dislocation. Requires bony procedures (femoral derotation osteotomy +/- varusosteotomy +/- acetabular procedure)

Consequences of Hip Dislocation

Lever arm dysfunction (interference with gait)

Pain

Difficulty with seating/positioning

Interference with self-care

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http://www.ausacpdm.org.au/professionals/hip-surveillance

Hip – All Levels

Refer to an orthopedic surgeon if:

MP is unstable (>10% change in 12 months) and/or progresses to >30%

There is pain related to the hip

Hip – All Levels

Initial AP pelvis radiograph at 12-24 months (or initial diagnosis if older than 24 months)

Repeat radiographs if hip subluxation or dislocation clinically suspected

Pain

New leg length discrepancy

Marked change in hip range of motion

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Hip – GMFCS I

Repeat clinical assessment at age 3 and 5

If classification of GMFCS I remains unchanged there is no need for further surveillance

Hip - GMFCS II

Repeat radiographs annually until migration percentage (MP) stabilizes

If MP is stable review at 4-5 years and 8-10 years

If MP is increasing continue with annual radiographs

Hip – GMFCS III

Repeat radiographs every 6 months until MP stabilizes

Once MP is stable reduce radiograph frequency to every 12 months

Review at 7 years

If MP is stable and <30% may discontinue radiographs until pre-puberty

Resume annual radiographs at pre-puberty and continue to skeletal maturity

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Hip – GMFCS IV

Same as for GMFCS III

If scoliosis or pelvic obliquity is present maintain 6 monthly radiographs until skeletal maturity

Hip – GMFCS V

6 monthly radiographs

At 7 years if MP is stable and <30% reduce frequency to every 12 months until skeletal maturity

If scoliosis or pelvic obliquity is present maintain 6 monthly frequency

BMD, Fractures and CP

Children with cerebral palsy are known to have low BMD compared with controls

Fragility fractures

Incidence 7-10% in severe CP

Prevalence up to 20% in non-ambulatory children and adults with CP

Most common site is distal femur

Fehlings D, Switzer L, Agarwal P, Wong C, Sochett E, Stevenson R, Sonnenberg L, Smile S, Young E, Huber J, Milo-Manson G, Kuwaik GA, Gaebler D. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: a systematic review. DevMed Child Neurol. 2012 Feb;54(2):106-16.

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Risk Factors for Low BMD and Fractures

GMFCS level

Decreased weight bearing

Anticonvulsant use

Poor nutrition

Decreased exposure to sunlight


Controversies Surrounding BMD in CP

When and how to measure BMD

How to use the information

When to incorporate Vitamin D, Calcium and Bisphosphonates

Role of weight bearing in prevention and/or management of low BMD


Evidence – Weight Bearing

6 studies using various modalities with conflicting results

Fracture rates not evaluated

No adverse events

Recommendation: Further studies are required but given other benefits of weight bearing a physiotherapy consult is recommended

Fehlings D, Switzer L, Agarwal P, Wong C, Sochett E, Stevenson R, Sonnenberg L, Smile S, Young E, Huber J, Milo-Manson G, KuwaikGA, Gaebler D. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: a systematicreview. Dev Med Child Neurol. 2012 Feb;54(2):106-16.

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Evidence – Vitamin D and Calcium

Level C evidence (possibly effective) for increasing BMD

BMD increases of 4-24%

Insufficient evidence for decreasing fragility fractures

No information on fracture rates

Recommendation: Review daily calcium intake and supplement if required; Consider starting Vitamin D; Baseline bloodwork with repeat at 6-12 months



Fehlings D, Switzer L, Agarwal P, Wong C, Sochett E, Stevenson R, Sonnenberg L, Smile S, Young E, Huber J, Milo-Manson G, KuwaikGA, Gaebler D. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: a systematicreview. Dev Med Child Neurol. 2012 Feb;54(2):106-16.

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Technology and Cerebral Palsy

Motor development is enhanced through intensive therapy especially during periods of rapid growth and after surgery to manage secondary orthopedic impairments or spasticity

The success of a rehabilitation program is dependent on:

The intensity of therapy

Repetition

Goal-oriented, task-specific

Motivation and active participation

Technology and Cerebral Palsy

Conventional therapy programs are personnel intensive and expensive

Limited resources often prevent achieving optimal therapy conditions

Robot assistance and virtually reality systems could allow for:

Reduced personnel needs

Increased repetition and frequency of therapy

Improved motivation and active participation

Robot Assisted Gait Training (RAGT)

Lokomat

Degree of assistance can be customized

Visual real-time feedback of effort

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Improvements found in:

Gait velocity and endurance

Standing and walking dimensions on the gross motor function measure (GMFM)

Possible improvements in:

Balance

Spasticity

Meyer-Heim A, van Hedel HJ. Robot-assisted and computer-enhanced therapies for children with cerebral palsy: current state and clinical implementation. Semin Pediatr Neurol. 2013 Jun;20(2):139-45.


Motivation generally remained high during therapies

May be better than conventional gait training for individuals with severe cognitive impairments

GMFCS level I and II may benefit more than III and IV


Virtual Reality Systems

Can be integrated into RAGT with further improvement in motivation and active participation


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CARENComputer Assisted Rehabilitation ENvironment

Xbox Kinect

van der Slot WM, Nieuwenhuijsen C, van den Berg-Emons RJ, Wensink-Boonstra AE, Stam HJ, Roebroeck ME; Transition Research Group South West Netherlands. Participation and health-related quality of life in adults with spastic bilateral cerebral palsy and the role of self-efficacy. J Rehabil Med. 2010 Jun;42(6):528-35.

Rutkowski S, Riehle E. Access to employment and economic independence in cerebral palsy. Phys Med RehabilClin N Am. 2009 Aug;20(3):535-47.

Young NL, Rochon TG, McCormick A, Law M, Wedge JH, Fehlings D. The health and quality of life outcomes among youth and young adults with cerebral palsy. Arch Phys Med Rehabil. 2010 Jan;91(1):143-8.

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