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What Do We Know About Pediatric Spinal Cord Injury?

What Do We Know About Pediatric Spinal Cord Injury? . MJ Mulcahey, Ph.D., OTRL Thomas Jefferson University, Jefferson School of Health Professions Department of Occupational Therapy. Disclosure of PI-RRTC Grant.

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What Do We Know About Pediatric Spinal Cord Injury?

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  1. What Do We Know About Pediatric Spinal Cord Injury? MJ Mulcahey, Ph.D., OTR\L Thomas Jefferson University, Jefferson School of Health Professions Department of Occupational Therapy

  2. Disclosure of PI-RRTC Grant • James S. Krause, PhD, Holly Wise, PhD; PT, Karla Reed, MA, and Elizabeth Walker, MPA have disclosed a research grant with the National Institute of Disability and Rehabilitation Research • The contents of this presentation were developed with support from an educational grant from the Department of Education, NIDRR grant number H133B090005. However, those contents do not necessarily represent the policy of the Department of Education, and you should not assume endorsement by the Federal Government.

  3. Accreditation • The Medical University of South Carolina is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. The Medical University of South Carolina designates this live activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™.  Physicians should claim only the credit commensurate with the extent of their participation in the activity. • In accordance with the ACCME Essentials &Standards, anyone involved in planning or presenting this educational activity will be required to disclose any relevant financial relationships with commercial interests in the healthcare industry.  This information is listed below.  Speakers who incorporate information about off-label or investigational use of drugs or devices will be asked to disclose that information at the beginning of their presentation. • The Center for Professional Development is an approved provider of the continuing nursing education by the South Carolina Nurses Association an accredited approver by the American Nurses Credentialing Center’s Commission on Accreditation

  4. Disclosure of Presenter • Dr. MJ Mulcahey does not have any financial disclosures.

  5. Objectives • Describe the population of youths with SCI • Discuss morbidity and mortality and describe associated factors • Discuss evidence in support of the International Standards for Neurological Classification (ISNCSCI) • Discuss outcomes of pediatric SCI • Describe current research Implications for management based on “Best Evidence”

  6. Description of the Population: Sources • US National SCI Model System • <1% children under 16yrs (DeVivo, 2006; 2012) • Vitale survey (2007) • Estimated 1500 children per year • Shriners Hospitals for Children SCI Data Base* • >3,500 children up to 21 years–first time exams • Longitudinal data collection – annual exams • 57% children <15 years • Primarily US based *Annual Publications from SHC

  7. Pediatric SCI • 1:1 male:female ratio in children < 5 yrs • Slightly higher in boys between 5-13 yrs • Increasing number of boys vs. girls >13 yrs. • Adult ratio 4:1 male:female Hadley et al., 1988; Hamilton & Myles, 1992; Ruge, Sinson, McLone, & Cerullo, 1988; Vogel & DeVivo, 1996, Vogel, 2004; 2012, Vitale, 2007

  8. Pediatric SCI • Neurological level and category vary as a function of age • Children < 8 yrs prone to cervical injuries • Children between 5-10 prone to paraplegia, complete injuries (lap-belt) Vogel & DeVivo, 1996, 1997, 2006; 2012

  9. Pediatric SCI • MVC primary cause of injury in children (56%) • 68% not in restraints (Vitale, 2007) • Violence remains another leading cause • Child abuse, physical fights, guns • Medical\surgical causes • Sports • Trampolines, ATV, gymnastics, skiing, snowboard Vogel & DeVivo, 1996, 1997; Shriners Hospitals for Children, 2011; Vitale, 2007

  10. Morbidity: Source of Data • Analyses of Shriners Hospital database • Followed annually until age 21 • 5,921 follow-up interviews/exams, 2,088 patients Data and slides provided by Dr. Michael DeVivo and presented at the 2012 Howard H. Steel Conference on Pediatric SCI

  11. Annual Risk of Medical Complications

  12. Annual Risk of Medical Complications

  13. Risk of Hospitalization (%)

  14. Mortality: Sources of Data N=8,668 • US SCI Model Systems and SHC Longitudinal databases • Injuries between 1935-2012 • 99% injured since 1970 • Periodic phone and mail interview • Clinic visit • Social security death index • National death index Data and slides provided by Michael DeVivo and presented at the 2012 Howard H. Steel Conference on Pediatric SCI

  15. Mortality Odds Ratios

  16. Mortality Odds Ratios

  17. Mortality Odds Ratios

  18. Mortality Odds Ratios

  19. Mortality Odds Ratios

  20. Mortality Odds Ratios

  21. Leading Cause of Death by Age (%)

  22. Leading Cause of Death by Age (%)

  23. Summary: Population Description • SCI in children is rare • No difference in SCI as a function of gender until after 13 years of age • MVC are the leading cause of pediatric SCI • LOI and severity associated with age at injury

  24. Summary: Morbidity • Three most common medical complications are UTI, pressure sore and AD • 1/3 children re-hospitalized during year one • 1/4 children re-hospitalized annually after year 1 • No difference in trends between children in adults

  25. Summary: Mortality • Risk of dying similar in children and adults • Age of injury <15 years associated with an increase risk in early death • Life expectancy below normal • No progress for year 1 survivors in 30 years • Children die from respiratory complications, heart disease, accidents and suicide

  26. Factors Associated with Morbidity and Mortality • Growth and development • Musculoskeletal conditions • Neuromuscular scoliosis • Pelvic obliquity\hip instability Vogel et al, Handbook of Clinical Neurology 2012; Mulcahey & Betz, Pediatric Spine, 2008; Mulcahey et al, Topics in SCI Rehab, 2004

  27. Prevalence: Neuromuscular Scoliosis

  28. Association Between Age-At-Injury and Scoliosis • Lancourt et al. • 0-10 years 100% • 11-16 years 19% • > 16 years 12% • Dearolf & Betz et al. 1990 • Prior to maturity: 98% (risk of surgery: 67%) • After maturity: 20% (risk of surgery: 5%)

  29. Study to Define Predictors of Neuromuscular Scoliosis in Children with SCI • 217 youths with SCI • Evaluation using motor, sensory and anorectal examinations of ISNCSCI • Cobb Angles from Plain Radiographs • Interpretation of Cobb Angles • 10> - diagnoses of scoliosis • 20> - brace treatment • 40> - surgical range Mulcahey, et al. Topics in SCI Rehab, In press

  30. Study to Define Predictors of Neuromuscular Scoliosis in Children with SCI • Multivariate analysis to determine worst Cobb Angle • Motor score, motor level, neurologic level, sensory level, AIS classification, gender, age at injury • Further evaluate effect of age • Sub-sample minimum 2 year follow up • Injured <12 and >12 • Odds of having spinal fusion

  31. Predictors of Worst Curve

  32. Predictors of Spine Fusion

  33. Odds Ratio for Eventual Spine Fusion • Minimum of 2 year follow up injured <12 (N=16) and >12 (N=27) OR=3.72 injured <12 • Excluding AIS D, minimum of 2 year follow up injured <12 (N=11) and >12 (N=26) OR=2.50 injured <12

  34. Neuromuscular Scoliosis • High Prevalence among children with SCI • Age is the only strong predictor, with statistical significance • Underscores importance of anticipatory guidance as part of rehabilitation

  35. Neuromuscular Scoliosis: So What? • Compensatory function is lost • Increase in respiratory distress and skin breakdown with increase in Cobb Angle • 98% of children injured <12 require spinal fusion (Dearolf & Betz, 1990) • Risk for pulmonary complications, psuedoarthrosis, infections very high (Sharma, et al 2010) • Vision loss reported (Samdani et al 2009)

  36. Neuromuscular Scoliosis: Implications for “Best Evidence” • Anticipatory guidance – education & prevention • TLSO • Prescription prior to onset of curve (right at time of injury) (Metah et al, 2006) • Slows progression – likely not prevent (Betz, 2010) • Wearing compliance varies (Hunter et al 2009) • Adverse effects on reachable workspace (Sison-Williamson, et al 2007) • Adverse effects on ADL (Chafetz, et al 2007)

  37. Neuromuscular Scoliosis: Implications for “Best Evidence” • Fusion • Improves respiratory function, posture, sitting balance • Greater risk for pressure sores within first year post spine fusion (Vogel, et al.) • Adverse effect on ability of children • Tetraplegia, to feed self (Mulcahey, et al) • Paraplegia, to perform bowel program (Mulcahey et al)

  38. Prevalence: Hip Instability • Pierre-Jacques & Betz et al, 1995 • 31 of 72 (43%) • Subluxation: 22 (70%) • Dislocation: 9 (30%) • Minimum 3-year followup

  39. Hip Instability: Precipitating Factors • Age at Injury • Spasticity • Flaccidity • Sepsis • Scoliosis

  40. Hip Instability • Spastic 25/31 (80%) • Flaccid 6/31 (21%) 12 year old, 4 years follow lap belt injury, T12 AIS A, flaccid paralysis, 70 Cobb Angle,

  41. Hip Instability • Increase risk • Pressure sores • Worsening spasticity • AD McCarthy & Betz, Clinic Ortho Related Research 2005

  42. Hip Instability: Implications for “Best Practice” • Prevention • Stretch – prone • Sleep prone • Release soft tissue contractures • Bone stability • Muscle balancing Parent et al 2010; Parent et al 2011

  43. Summary: Factors Associated with Morbidity and Mortality Unique to Children • Growth and development • Age at injury strongest predictor • Neuromuscular scoliosis • Respiratory decline, pressure sores, pelvic obliquity • Hip instability • Pressure sores, increased spasticity, Increase AD

  44. Use of the International Standards for Neurological Classification of Spinal Cord Injury

  45. aka “The Standards” Evaluate the neurological consequence Classify the neurological consequence Background

  46. The Standards • Reliability Studies • Adults with relatively small samples* • Pediatric studies** • Motor and sensory examinations • Classification *Cohen 1994; 1996; 1998; Donovan 1990; Marino 2004; 2008; ** Mulcahey 2007a; 2007b; 2009; Chafetz 2009; Vogel 2009; Samdani 2010; Mulcahey 2011

  47. The Standards • Lacking • Reliability of the anorectal examinations • No work with Adults • Few studies with children* • Validity of the anorectal examination** *Mulcahey 2007; Vogel 2009 **Wietek 2008; Samdani 2009; Vogel 2009; Samdani 2010

  48. The Problem • Routinely used with children • Children are “assigned” NL and classification • “Assignment” (diagnoses) travels with children • Little reliability\validity of the anorectal examinations

  49. Purpose • Evaluate reliability of the motor, sensory and anorectal examinations • Determine lower age limit • Establish pediatric guidelines

  50. Methods • Cross-sectional repeated measures multi-center study • Sample of convenience • 3 months – 21 years of age • Chronic SCI (=>3 months duration) • Exclusion • Acute injury (<3 months) • Neurological changes in last 3 months • Mechanical ventilation without ability to communicate • TBI and\or brachial plexus injury

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