3/9/2018

Disclosures :  Personal Disclosures: A View from Both Sides  Consultant: Allergan Corporation, Orthopediatrics, 3D4Medical Corp.

 Institutional Research Support: NIH, Orthopedic Research and Education Foundation, Major League Baseball, Rady Hank Chambers, MD Children’s Hospital, DePuy Spine, Allergan, Axial David H Sutherland Chair of Cerebral Palsy Program Biotech, Ellipse, Alphatec Spine, KFx, Magellan Rady Children’s Hospital San Diego Spine, Zimmer, KCI, Synthes, Syntaxin, K2M, Professor of Clinical Orthopedic Surgery University of California at San Diego  Institutional Education Support: Rady Children’s Hospital, DePuy Spine

Off-Label Use What Is Cerebral Palsy?  Is it brain damage due to obstetrical trauma?  Was the baby too big or too small?  Botulinum Toxin (Botox, Myobloc, Xeomin, etc) are not approved for use in children for spasticity  Occurs before the age of 3 by the FDA. Dysport has recently received  Cerebral palsy (CP) describes a group of approval for lower extremity spasticity in children permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive  Intrathecal Baclofen is not approved for use in disturbances that occurred in the developing dystonia fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition,  However, 50% of all drugs that are used in communication, behavior, by epilepsy and by children are not specifically indicated secondary musculoskeletal problems

Modified after Bax et al. DMCN 2005

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Epidemiology: The Cerebral Palsies Some Statistics  Risk is 25-30 times in neonates < 1500g  1 in 3 children with VLBW will have CP  Most children with CP were not premature  54 million Americans have a  10% of <28 week premature patients will have CP  72 percent of unemployed adults with  Prevalence in 8 year olds: 3-4 patients/1000  (1 in 278) would like to work  10,000 new diagnoses each year  Lifetime cost of child born today with  Prevalence: ~950,000 Americans with CP CP:  87% 30-year survival rate  $1 million  Much higher prevalence in black population

 There are now more adults with CP than children

Etiology of Cerebral Palsies

 Prematurity  Multiple Births: Assistive Reproduction, older 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 Career Choices  Magnesium Sulfate   Infant and brain cooling United Cerebral Palsy Telethon

 EPO  Pediatric Rotation

 Antiinflammatories  Birth of my son, Sean in 1982 while I was an intern  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

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Orthopedic Education Fellowship in San Diego  Introduction to Gait Analysis by Dr. David Sutherland  Residency taught me the “fundamentals” of cerebral palsy care, which essentially was heel  Other teachers included forward thinkers cord lengthening, percutaneous adductor and such as Scott Mubarak and Dennis hamstring lengthening and prolonged casting Wenger who encouraged me to work in the field  Therefore these were the procedures that I learned and these were the procedures that my  Meeting other great thinkers like Freeman son had. Miller, Mike Sussman, Mike Aiona, Jim Gage, Kerr Graham, etc

Gait Analysis Classification Systems

 The diplegia, quadriplegia, hemiplegia  What is gait analysis? system has poor intra and interobserver reliability  Why is it important for the individual patient and the overall care of children with cerebral palsy?  Unilateral vs Bilateral (Surveillance of Cerebral Palsy in Europe)  Why is there a controversy?  Levels of ambulation: household, therapy, community also has limitations  Gross Motor Functional Classification System (GMFCS)

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Gross Motor Function GMFCS Measure (GMFM)  Series of tests given to ascertain the level of gross motor involvement in children with cerebral palsy.

GMFCS and Musculoskeletal Problems 1. Hip displacement: incidence, type Functional 2. Success of hip surgery: STR vs VDROs Mobility Scale 3. Mortality & Morbidity Function at 5, 50 4. Contracture and bony deformity and 500 meters 5. Success of Gait Correction Surgery 6. Choice of procedures: Rectus Femoris Transfer, Varus foot surgery

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Other Classifications Dimensions of Disability  Manual Ability Classification System  International Classification of Functioning,  For Upper Extremity Problems Disability and Health (ICF) WHO  Body Functions  Body Structures  Activities and Participation

 Communication Functional Classification System  Environmental Factors  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

Participation The NCMRR Model of Disablement

The Person with a Disability and the Rehabilitation Process

Societal Limitation

National Center for Medical Rehabilitation Research. Bethesda, MD.

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Treatment Paradigms First We Must Set Goals  Goal Setting  Team Approach Independence  Management of Movement Disorders Working  Therapies: Physical, Occupational, Speech Communication  Role of Technology Activities of Daily Living  Timing of Orthopedic Surgery Mobility  Bony and Soft Tissue Surgery Walking

Integrated Treatment Approach in the Child with Cerebral Palsy Therapies

Alternative PT Treatment Orthopedic Surgery Occupational OT Intrathecal Baclofen Pump Speech and Language Casting Child With Rhizotomy Spasticity Management of Drooling Bracing Alcohol Injection

Orthotics Botulinum Visual Impairment Toxin Oral Phenol Medications Injection

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Physical Therapy

 Neurodevelopmental Therapy Technology  Hippotherapy  Equipment Promise and Challenges

Challenges Robotics

Cost Training Upkeep Specificity for each child

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Speech Therapy Mobility  Getting from Point A-Point B Communication Devices  May mean walking, using assistive devices, wheelchairs or the means of accessing private or public transportation

Simple Computer

Role of Standing Wheelchairs

 Simple sling chairs

 Custom Manual Chairs

 Custom Powered Chairs

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Mobility: Wheelchairs and Transportation Seating Systems

Medical Management of Cerebral Palsy Movement Disorders  Growth Retardation Spasticity  Seizure Disorders

 Management of Reflux Choreo-  Management of other GI issues such as Gall stones, constipation, dumping after bowel surgery  Kidney Stones   Skin ulceration

 Oral Health Dystonia

 Intellectual Disability

 Etc. Etc, Etc

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Ataxia

Dystonia and Choreoathetosis 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 Current Spasticity Treatment Options: General Loss of Selective Motor Exercise and physical Control modalities Sensory Deficits Systemic drugs Diazepam (Valium) Weakness Baclofen (Lioresal) Trihexyphenidyl (Artane) Etc.

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Orthopedic and Neurosurgical Methods  Anesthetic and neurolytic injections Tendon lengthenings altering the  Phenol muscle receptors  Alcohol Osteotomies  Chemodenervation  Lever Arm Syndrome injections Neurotomies  Botulinum Toxin A, B Cannibis CBD Oil 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 GMFCS Level 1 Surgical Interventions Relative frequency of treatment type in cerebral palsy management program BTX-A + motor training and Surgery (SEMLS) orthoses

Casting Casting Casting and + BTX-A surgery

Isolated soft tissue Casting Isolated use with and/or bony surgery after repeated surgery, for hip stability surgery where indicated 0 5 7.5 10 15 Years Boyd, et al. Eur J Neur 1999;6:S37-43.

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GMFCS Level 1 GMFCS Level I

• High level physical functioning: spastic • Mild gait dysfunction hemiplegia, mild spastic diplegia • Many benefit from botulinum toxin • Seizures, occasionally • Few need any orthopaedic surgery • Learning difficulties • Too mild for SDR or ITB • Behavioral problems • No hip displacement, no scoliosis • Autistic spectrum disorders • UL Surgery in Hemiplegia

From H. Kerr Graham, MD

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

• 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

• 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 GMFCS Level IV

 Femoral Osteotomies  Proximal  Distal

 Tibial rotational osteotomies

 Correction of foot valgus

GMFCS Level IV Hip Displacement (MP>30%) by GMFCS. Soo et al JBJS(A) Jan 2006

• Spastic quadriplegia: mild-moderate 90 • Spastic-dystonia • Botox and ITB • Hip displacement and scoliosis • Screen and prevent hip displacement • Orthopaedic surgery for standing, sitting 10 • May need hip and knee surgery

I II III IV V

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Acetabular Dysplasia Hip Subluxation & Dislocation

 Usually posterior dysplasia

 Should assess with CT Scan Acetabular Dysplasia

Hip Subluxation

Anterior Posterior Mid-superior 29% 15% Kim and Wenger JPO 1997 37%

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

• Spastic quadriplegia • Optimizing health • Multiple medical co-morbidities • Minimizing co-morbidities • Significant excess mortality in each decade • Goal setting • Dystonia, spasticity: Botox, phenol, ITB • Hip and spine surveillance • 90% will develop hip disease and scoliosis • Preventative, reconstructive surgery • Comfortable sitting • Child and care giver quality of life

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VALGUS OSTEOTOMY PROXIMAL FEMORAL RESECTION

 Advantages

 Increased motion

 Pain relief

 Disadvantages

 May be painful

TOTAL HIP REPLACEMENT Shoulder Arthroplasty

 Advantages

 Pain relief

 Motion

 Disadvantages

 Re-dislocation

 Difficult

 Infection

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 Fuse Joints for Stability 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.

Orthopedic Surgical Unconventional or Interventions: Spine Alternative Treatments  Hyperbaric Oxygen Intervention to  Adeli Suit correct  Biofeedback Scoliosis  Conductive Education Spondylolisthesis  Facilitated Communication Hyperkyphosis  Doman-Delicato Hyperlordosis  Many, many more

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Caregiver Stress Other Stressors: New Interventions with little or  Increased physical strain  Higher incidence of back pain no evidence  Increased mental strain  Hyperbaric Oxygen  More time to care for child, including feeding  Different therapies  Increased marital stress  Doman Delicato  85% divorce rate (in articles, but not true)  Conductive Therapy  Mothers (usually) giving up job/career  Etc  Sleep Disorder  Stem Cell treatment  Sibling stress

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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So, What have I learned in 35 Stages of Grief 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 other than the care of children who have dedicated their careers to the understanding and treatment of this disorder

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Thank you

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