Cerebral Palsy: Personal Disclosures : A View from Both Sides - - PowerPoint PPT Presentation

3/9/2018 1

Cerebral Palsy: A View from Both Sides

Hank Chambers, MD

David H Sutherland Chair of Cerebral Palsy Program Rady Children’s Hospital San Diego Professor of Clinical Orthopedic Surgery University of California at San Diego

Disclosures

 Personal Disclosures:

 Consultant: Allergan Corporation,

Orthopediatrics, 3D4Medical Corp.  Institutional Research Support: NIH, Orthopedic Research and Education Foundation, Major League Baseball, Rady Children’s Hospital, DePuy Spine, Allergan, Axial Biotech, Ellipse, Alphatec Spine, KFx, Magellan Spine, Zimmer, KCI, Synthes, Syntaxin, K2M,  Institutional Education Support: Rady Children’s Hospital, DePuy Spine

Off-Label Use

 Botulinum Toxin (Botox, Myobloc, Xeomin, etc)

are not approved for use in children for spasticity by the FDA. Dysport has recently received approval for lower extremity spasticity in children

 Intrathecal Baclofen is not approved for use in

dystonia

 However, 50% of all drugs that are used in

children are not specifically indicated

What Is Cerebral Palsy?

 Is it brain damage due to obstetrical trauma?  Was the baby too big or too small?  Occurs before the age of 3  Cerebral palsy (CP) 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 of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, behavior, by epilepsy and by secondary musculoskeletal problems

Modified after Bax et al. DMCN 2005

3/9/2018 2 Epidemiology: The Cerebral Palsies

 Risk is 25-30 times in neonates < 1500g

 1 in 3 children with VLBW will have CP  Most children with CP were not premature  10% of <28 week premature patients will have CP

 Prevalence in 8 year olds: 3-4 patients/1000

 (1 in 278)  10,000 new diagnoses each year

 Prevalence: ~950,000 Americans with CP  87% 30-year survival rate  Much higher prevalence in black population  There are now more adults with CP than children

Some Statistics

 54 million Americans have a disability  72 percent of unemployed adults with

disabilities would like to work

 Lifetime cost of child born today with

CP:

$1 million

Etiology of Cerebral Palsies

 Prematurity

 Multiple Births: Assistive Reproduction,

• lder mothers, teen pregnancy

 Chromosomal and Brain

Abnormalities

 Genetic Influences  Metabolic Influences

 Hormonal  Heat  Inflammation

 Hemostatic Disorders  Infection: bacterial, viral  Trauma  Epigenetic factors such as maternal

deparession

 Remember: Correlation does not

imply causation

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Tractography Preventive Strategies

 Magnesium Sulfate  Infant and brain cooling  EPO  Antiinflammatories  Thyroid Hormone  Erythropoiesis Stimulating Agents  Avoidance of toxic substances: nicotine, drugs,

alcohol

 Question Assistive Reproduction Technology  Prevention of Non accidental Trauma, automobile

accidents, near drowning

Career Choices

 United Cerebral Palsy Telethon  Pediatric Rotation  Birth of my son, Sean in 1982 while I was an intern

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Orthopedic Education

 Residency taught me the “fundamentals” of

cerebral palsy care, which essentially was heel cord lengthening, percutaneous adductor and hamstring lengthening and prolonged casting

 Therefore these were the procedures that I

learned and these were the procedures that my son had.

Fellowship in San Diego

 Introduction to Gait Analysis by Dr. David

Sutherland

 Other teachers included forward thinkers

such as Scott Mubarak and Dennis Wenger who encouraged me to work in the field

 Meeting other great thinkers like Freeman

Miller, Mike Sussman, Mike Aiona, Jim Gage, Kerr Graham, etc

Gait Analysis

 What is gait analysis?  Why is it important for the individual patient and

the overall care of children with cerebral palsy?

 Why is there a controversy?

Classification Systems

 The diplegia, quadriplegia, hemiplegia

system has poor intra and interobserver reliability

 Unilateral vs Bilateral (Surveillance of

Cerebral Palsy in Europe)

 Levels of ambulation: household,

therapy, community also has limitations

 Gross Motor Functional Classification

System (GMFCS)

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Gross Motor Function Measure (GMFM)

 Series of tests given to ascertain the level of gross

motor involvement in children with cerebral palsy.

GMFCS

• 1. Hip displacement: incidence, type
• 2. Success of hip surgery: STR vs VDROs
• 3. Mortality & Morbidity
• 4. Contracture and bony deformity
• 5. Success of Gait Correction Surgery
• 6. Choice of procedures: Rectus Femoris

Transfer, Varus foot surgery

GMFCS and Musculoskeletal Problems Functional Mobility Scale

Function at 5, 50 and 500 meters

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Other Classifications

 Manual Ability Classification System

 For Upper Extremity Problems

 Communication Functional Classification System

 I Effective Sender and Receiver with unfamiliar and familiar

partners

 II Effective but slower paced Sender and/or Receiver with

unfamiliar partners

 III Effective Sender and Receiver with familiar partners  IV Inconsistent Sender or Receiver with familiar partners  V Seldom Effective Sender and Receiver even with familiar

partners

Dimensions of Disability

 International Classification of Functioning,

Disability and Health (ICF) WHO

 Body Functions  Body Structures  Activities and Participation  Environmental Factors

Participation The NCMRR Model of Disablement

Societal Limitation

The Person with a Disability and the Rehabilitation Process

National Center for Medical Rehabilitation Research. Bethesda, MD.

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Treatment Paradigms

 Goal Setting  Team Approach  Management of Movement Disorders  Therapies: Physical, Occupational, Speech  Role of Technology  Timing of Orthopedic Surgery  Bony and Soft Tissue Surgery

First We Must Set Goals

Independence Working Communication Activities of Daily Living Mobility Walking

Integrated Treatment Approach in the Child with Cerebral Palsy

Alternative Treatment Orthopedic Surgery Phenol Injection

Child With Spasticity

PT OT Casting Bracing Orthotics Oral Medications Botulinum Toxin Alcohol Injection Intrathecal Baclofen Pump Rhizotomy

Therapies

Occupational Speech and Language Management of Drooling Visual Impairment

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Physical Therapy

 Neurodevelopmental

Therapy

 Hippotherapy  Equipment

Technology

Promise and Challenges

Challenges

Cost Training Upkeep Specificity for each child

Robotics

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Speech Therapy

Communication Devices

Simple Computer

Mobility

 Getting from Point A-Point B  May mean walking, using assistive devices,

wheelchairs or the means of accessing private or public transportation

Role of Standing Wheelchairs

 Simple sling chairs  Custom Manual Chairs  Custom Powered Chairs

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Mobility: Wheelchairs and Seating Systems Transportation Medical Management of Cerebral Palsy

 Growth Retardation  Seizure Disorders  Management of Reflux  Management of other GI issues such as Gall stones,

constipation, dumping after bowel surgery

 Kidney Stones  Skin ulceration  Oral Health  Intellectual Disability  Etc. Etc, Etc

Spasticity Choreo-

Athetosis

Ataxia Dystonia

Movement Disorders

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Ataxia Choreoathetosis Dystonia and Choreoathetosis

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Spasticity vs Dystonia

 New understandings of the definitions and

therefore the natural history of children with cerebral palsy.

Other Important Problems

Loss of Selective Motor

Control

Sensory Deficits Weakness

Current Spasticity Treatment Options: General

Exercise and physical

modalities

Systemic drugs

Diazepam (Valium) Baclofen (Lioresal) Trihexyphenidyl (Artane) Etc.

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 Anesthetic and

neurolytic injections

Phenol Alcohol  Chemodenervation

injections

Botulinum Toxin A, B

Cannibis CBD Oil

Orthopedic and Neurosurgical Methods

Tendon lengthenings altering the

muscle receptors

Osteotomies

 Lever Arm Syndrome

Neurotomies Fusion especially spinal fusion

stabilizes the trunk Intrathecal

drugs

Intrathecal

Baclofen Selective Dorsal Rhizotomy

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Deep Brain Stimulation

Principles of Orthopedic Surgery

Single event, multilevel

surgery

Delay surgery as long as

possible (> 6 years)

Use spasticity management

as adjunct to surgery

Timing of Orthopedic Surgical Interventions

Boyd, et al. Eur J Neur 1999;6:S37-43.

5 7.5 10 15 Years

Isolated soft tissue and/or bony surgery for hip stability

Casting + BTX-A Casting Casting and surgery

Casting after surgery Isolated use with repeated surgery, where indicated

Surgery (SEMLS) BTX-A + motor training and

• rthoses

Relative frequency of treatment type in cerebral palsy management program

GMFCS Level 1 GMFCS Level 1

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GMFCS Level 1 GMFCS Level 1

• High level physical functioning: spastic

hemiplegia, mild spastic diplegia

• Seizures, occasionally
• Learning difficulties
• Behavioral problems
• Autistic spectrum disorders

From H. Kerr Graham, MD

GMFCS Level I GMFCS Level I

• Mild gait dysfunction
• Many benefit from botulinum toxin
• Few need any orthopaedic surgery
• Too mild for SDR or ITB
• No hip displacement, no scoliosis
• UL Surgery in Hemiplegia

GMFCS Level II GMFCS Level II

GMFCS Level II GMFCS Level II

• Mostly spastic diplegia of prematurity
• Some have severe hemiplegia
• Wide range of gait dysfunction
• Significant spasticity
• Significant deformities
• Mild hip disease, no scoliosis

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GMFCS Level II GMFCS Level II

• Botulinum Toxin very useful
• SDR: a very few , highly selected cases
• No ITB
• Single level orthopaedic surgery: UL & LL
• Hip screening and preventative surgery
• SEMLS: Multilevel surgery

GMFCS Level III GMFCS Level III

GMFCS Level III GMFCS Level III

• Severe diplegia, mild quadriplegia
• Spastic-dystonia
• Botulinum toxin + Phenol are useful,

some ITB

• Hip displacement common & important
• Screen and prevent hip displacement
• Gait correction surgery: hips and feet

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Treatment of Lever Arm Syndrome

 Femoral Osteotomies

 Proximal  Distal

 Tibial rotational osteotomies  Correction of foot valgus

GMFCS Level IV GMFCS Level IV

GMFCS Level IV GMFCS Level IV

• Spastic quadriplegia: mild-moderate
• Spastic-dystonia
• Botox and ITB
• Hip displacement and scoliosis
• Screen and prevent hip displacement
• Orthopaedic surgery for standing, sitting
• May need hip and knee surgery

Hip Displacement (MP>30%) by GMFCS.

Soo et al JBJS(A) Jan 2006

Hip Displacement (MP>30%) by GMFCS.

Soo et al JBJS(A) Jan 2006

I II III IV V

90 10

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Hip Subluxation & Dislocation

Hip Subluxation Acetabular Dysplasia

Acetabular Dysplasia

 Usually posterior dysplasia  Should assess with CT Scan

Anterior 29%

Posterior 37%

Mid-superior 15%

Kim and Wenger JPO 1997

Proximal Femoral Varus Derotational Osteotomies

Indications for surgery:

‘d valgus ‘d femoral anteversion

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GMFCS Level V GMFCS Level V

GMFCS Level V GMFCS Level V

• Spastic quadriplegia
• Multiple medical co-morbidities
• Significant excess mortality in each decade
• Dystonia, spasticity: Botox, phenol, ITB
• 90% will develop hip disease and scoliosis
• Comfortable sitting

GMFCS Level V GMFCS Level V

• Optimizing health
• Minimizing co-morbidities
• Goal setting
• Hip and spine surveillance
• Preventative, reconstructive surgery
• Child and care giver quality of life

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VALGUS OSTEOTOMY

 Advantages

Increased motion

 Pain relief

 Disadvantages

 May be painful

PROXIMAL FEMORAL RESECTION

TOTAL HIP REPLACEMENT

 Advantages

 Pain relief  Motion

 Disadvantages

 Re-dislocation  Difficult  Infection

Shoulder Arthroplasty

Flynn, J and Miller F: Management of Hip Disorders in Patients with Cerebral Palsy. JAmAcadOrthopSurg 2002 !): 196-209

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Botulinum Toxin for Pain in Dislocated Hips

 Current study at Rady Children’s Hospital  32 patients with painful dislocated hips or

previously surgically treated hips

 400 Units of Botox in muscles about the hip (16

separate sites)

 Marked improvement in pain in 90%  Must be repeated every 4-5 months.

Fuse Joints for Stability

Orthopedic Surgical Interventions: Spine

Intervention to

correct

Scoliosis Spondylolisthesis Hyperkyphosis Hyperlordosis

Unconventional or Alternative Treatments

 Hyperbaric Oxygen  Adeli Suit  Biofeedback  Conductive Education  Facilitated Communication  Doman-Delicato  Many, many more

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Caregiver Stress

 Increased physical strain

 Higher incidence of back pain

 Increased mental strain  More time to care for child, including feeding  Increased marital stress

 85% divorce rate (in articles, but not true)

 Mothers (usually) giving up job/career  Sleep Disorder  Sibling stress

Other Stressors: New Interventions with little or no evidence

 Hyperbaric Oxygen  Different therapies Doman Delicato Conductive Therapy Etc  Stem Cell treatment

Transition Issues Adult Clinic

 5100 Patients treated in last 25 years.  Multitude of new problems including:

 Increased pain  Arthritis  Difficulty sitting  Progression of movement disorder  Bipolar disease (45% of all my adult

patients are on antidepressants)

 Loss of ambulation  Cervical Spine Problems  Progressive Hydrocephalus

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Stages of Grief So, What have I learned in 35 years

 Parents are always seeking a cure for their child  Hyperbaric Oxygen  Stem Cells  ?????? And $$$$$$  Simple insights lead to great changes in care  Definition of dystonia  GMFCS  Little money available for research  There is a huge disparity between health care for

children and adults with disabilities

 Treatment is important, but prevention is the real

hope

 There are true heroes who have no vested interest

• ther than the care of children who have

dedicated their careers to the understanding and treatment of this disorder

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Thank you