Orthopedic Issues in Children with Special Healthcare Needs

Transcription

1 Orthopedic Issues in Children with Special Healthcare Needs Kathryn A Keeler, MD Assistant Professor University of Missouri-Kansas City School of Medicine, Department of Orthopaedic Surgery and Department of Pediatrics Children s Mercy Kansas City, Division of Orthopaedics and Section of Sports Medicine The Children's Mercy Hospital, 2016

2 Disclosures I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity. I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation 2

3 Special Needs An individual who requires assistance for disabilities that may be medical or emotional 3

4 Special Needs Movement disorders (i.e. Cerebral palsy) Genetic syndromes (i.e. Trisomy 21) Spina bifida 4

5 Cerebral Palsy 3.6 per 1000 live births in the U.S Most common cause of physical disability affecting children in developed countries Wide range of causes including: Prematurity Anoxic injuries Intrauterine factors 5

6 Cerebral Palsy Non-progressive upper motor neuron disorder (static encephalopathy) due to injury to the immature brain Movement/Motor disorder Muscle imbalance with a mixture of weakness and spasticity Abnormal reflex arch Differs in distribution, presentation and severity 6

7 Cerebral Palsy Primary abnormality (abnormal muscle tone, muscle imbalance, weakness, poor selective motor control) Muscle contracture (dynamic or static) Effect on Growth and Development Bony deformity (Femoral neck-shaft angle) Joint instability 7

8 Tone Disorder Classification of Cerebral Palsy Spasticity: Characterized by resistance to stretch, may be either or both velocity and position dependent. Dystonia: Characterized by sustained muscle contractions or postures, often twisting or repetitive in nature. Mixed Usually mixed spastic and dystonic features and involves the entire body 8

9 Gross Motor Function Classification Scale (GMFCS) (Palisano et al 1997) GMFCS I: Walks and runs GMFCS II: Walks, no aids GMFCS III: Walks with aids GMFCS IV: Stands, wheelchair GMFCS V: No functional wt bearing 9

10 Hip displacement (MP > 30%) & GMFCS Proportion of children (%) with MP < 33% (green), 33 39% (yellow) and > 40% (red) in relation to GMFCS level. BMC Musculoskelet Disord. 2007;8:

11 Limited hip ROM interferes with: Dressing, Perineal care, Diapering Sitting imbalance and discomfort Pelvic obliquity, Prominence Pressure ulcers Hip pain 49% of institutionalized adults with CP (Noonan et al, JBJS A 12, 2004) 11

12 Hip Surveillance Serial monitoring with: Repeated, recorded, physical examination Pelvic x-ray with standardized positioning 1 st xray at months Gait videos for ambulant patients 12

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14 Trisomy 21: Orthopaedic Aspects Ligamentous laxity Scoliosis Hip instability Patella-femoral instability Foot deformities

15 Trisomy 21: Cervical Spine Atlanto-axial instability (C1-2) is the primary orthopaedic concern of individuals with Down Syndrome. While there is significant incidence of AAI in Down Syndrome, the clinical significance of this finding and its management remain controversial.

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17 Trisomy 21: AAI 1983: Lateral neck radiographs required for participants in Special Olympics. 1984: American Academy of Pediatrics publishes position statement supporting the requirement.

18 Trisomy 21: AAI 1995: American Academy of Pediatrics retracts its previous statement of support for routine screening. Special Olympics has not removed its requirement that radiographs of the cervical spine be obtained in all athletes with Down Syndrome.

19 Trisomy 21: AAI Health care guidelines for individuals with Down Syndrome screening cervical spine radiographs at 3 years, 12 years, and once in adulthood if asymptomatic. Cohen: Down Syndrome Medical Interest Group

20 Trisomy 21: AAI A significant amount (10-30%) of people with Down Syndrome have an increased ADI on screening radiographs ( asymptomatic AAI? ) A small amount of people with Down Syndrome (1% or less) will develop neurologic impairment from cervical spine instability. No evidence that patients with asymptomatic AAI will progress to symptomatic AAI with neurologic impairment.

21 Spina Bifida Spina bifida occulta Neural tube defects Meningocele Myelomeningocele

22 Myelomeningocele: Incidence In general, cases per 100,000 live births Difference in incidence occurs amongst different ethnic populations Folic acid supplementation as affected the incidence of neural tube defects Pre-natal diagnosis and termination as influenced incidence as well

23 Spina Bifida: Etiology Primary embryologic problem in all neural tube defects is a failure of the neural folds to fuse in the midline and form the neural tube This event occurs at days of gestation (all neural tube defects) Myelomeningocele occurs due to failure of closure of the caudal neuropore at days of gestation

24 Management of Thoracic and Upper Lumbar Level: Early Walking or Early Wheelchair? Spina bifida patients who stand or walk early in life, even if they become non-ambulators later: Have fewer fractures Have fewer pressure sores Were better able to transfer Were more independent, but...

25 Management of Thoracic and Upper Lumbar Level: Early Walking or Early Wheelchair? Spent more time in the hospital Had more surgical procedures There were no differences regarding: Skills of daily living Upper extremity function Frequency or severity of obesity

26 Fractures in Children with Myelomeningocele Common due to osteopenia and lack of protective sensation Often present clinically with swelling and erythema, but not pain Immobilization necessary, but must be as limited as possible to prevent further disuse osteopenia

27 Latex Allergy in Spina Bifida Patients Allergy is to the residual plant proteins in natural latex products IgE-medicated, immediate type of hypersensitivity 15-30% incidence of latex allergy in spina bifida population Avoidance of latex by all children with spina bifida may reduce the prevalence of the allergy, or perhaps severity

28 Thank you 28