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