3/9/2018
Disclosures Cerebral Palsy: 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
1 3/9/2018
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 disability 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) disabilities 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
2 3/9/2018
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
3 3/9/2018
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)
4 3/9/2018
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
5 3/9/2018
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.
6 3/9/2018
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
7 3/9/2018
Physical Therapy
Neurodevelopmental Therapy Technology Hippotherapy Equipment Promise and Challenges
Challenges Robotics
Cost Training Upkeep Specificity for each child
8 3/9/2018
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
9 3/9/2018
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, Athetosis constipation, dumping after bowel surgery Kidney Stones Ataxia Skin ulceration
Oral Health Dystonia
Intellectual Disability
Etc. Etc, Etc
10 3/9/2018
Ataxia
Dystonia and Choreoathetosis Choreoathetosis
11 3/9/2018
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.
12 3/9/2018
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
13 3/9/2018
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.
14 3/9/2018
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
15 3/9/2018
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
16 3/9/2018
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
17 3/9/2018
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
18 3/9/2018
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
19 3/9/2018
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
20 3/9/2018
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
21 3/9/2018
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
22 3/9/2018
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
23 3/9/2018
Thank you
24