1. A Functional Approach to Serving Preschool and School Aged Children
with Spina Bifida
Presenter
Fred Klingbeil, MD
Medical Director Pediatric
Rehabilitation Medicine

2. Role of pediatric rehabilitation medicine
Functional assessment
Primary disabling disorder
Associated conditions and issues
Impairments and functional limitations
Managerial focus, objectives and expectant outcome

3. Primary Condition

4. Occurrence 0.2% of general population 5% if one child with defect
10% if one parent with defect
10% if two children with defect
21% if three children with defect
More common if Irish ancestry

5. Spina Bifida Occulta (5 percent of population)
Incomplete vertebral closure without meninges or cord protrusion, and no signs of neurologic impairment

6. Spina Bifida Occulta Clinical L5-S1 (most common)
Dermal hyperpigmentation
Hairy patch
Lump
Dermal sinus
Initially asymptomatic
Complications
Hinders spinal cord mobility with growth

7. Protrusion of sac through unfused vertebral arch defect
Spina Bifida Cystica
Protrusion of sac through
unfused vertebral arch defect
Meningocele
Incomplete vertebral closure with meninges protrusion and no sign of neurologic impairment
Myelomeningocele
Incomplete vertebral closure with meninges and cord protrusion, and signs of neurologic impairment
47% lumber
26% thoracic
20% sacral
2% cervical

8. Latex Allergy Risk factors Toys containing latex, supplies, equipment
Avocado
Banana
Water chestnut
Clinical
Sudden anaphylactic reaction
Subtle hives or rash
Management
Prevention
Education
Prevent exposure
Intervention
Diphenhydramine (Benadryl)
Epinephrine (EpiPen)

9. Hydrocephalus Occurrence 85 percent Clinical Enlarged head
Bulging forehead and fontanel
Widespread cranial sutures
Sunset eyes
Decreased activity
Poor feeding
Somnolence
Irritability
Headaches
Decreased eye-hand coordination
Decline in school performance

10. Chiari Malformation
Chiari refers to malformation and displacement of the hindbrain
into cervical vertebral canal

11. Chiari Malformation Occurrence
Occurs in all children with hydrocephalus but is symptomatic in only percent of cases
Clinical
Nystagmus, strabismus
Difficulty swallowing, slow to tolerate solid foods
Apnea spells, stridor, aspiration
Spasticity
Face, neck, extremity weakness
Loss of fine motor control
Sensory loss
Growth delay

12. Tethered Cord Clinical Deterioration of strength Changing reflex level
Change in tone and reflexes
Change in bowel and bladder function
Forming deformities
Change in sensation   
Intervention
Observe for asymptomatic cord and stable neurologic impairments
Symptomatic cord release

13. Flaccid (“Keeper”) Type
Neurogenic Bladder
Flaccid (“Keeper”) Type
Pathophysiology
Failure to empty due to ineffective bladder contractility and/or ineffective urethral relaxation
Complications
Urinary tract infection
Vesicoureteral reflux, hydronephrosis
Overflow incontinence
Management
Clean intermittent catheterization
Prophylactic antibiotics

14. Spastic (“Leaker”) Type
Neurogenic Bladder
Spastic (“Leaker”) Type
Pathophysiology
Failure to adequately store urine due to uninhibited bladder detrusor contracting and/or ineffective urethral resistance
Complications
Incontinence
Intervention
Clean intermittent catheterization
Decrease bladder contraction using (antecholinergic) medications
Increase urethral resistance using (adrenergic) medications
Surgery

15. Neurogenic Bowel Pathophysiology
Failure to adequately control bowel usually due to involuntary relaxation and/or contraction of internal anal sphincter

16. Neurogenic Bowel Clinical Absent anal sensation Incontinence
Constipation
Intervention
Dietary suggestions
Postprandial routine education
Fiber diet 
Rectal suppositories
Enema

17. Obesity Causes Decreased metabolic rate Increased body fat
Decreased lean body mass
Increased caloric intake as awards
Intervention
Caloric intake percent of normal
Low cholesterol and unsaturated fat diet
Maximize activity

18. Cardiovascular Disease
Risk factors
Obesity
Decreased activity
Hypertension due to renal dysfunction
Evaluation
Lipid profiles
Diet and weight
Exercise routine
Intervention
Encourage exercise
Modify diet
Cholesterol lowering medications

19. Orthopedic Deformities
Spine
Kyphosis
Scoliosis
Lordosis
Knee
Flexion contracture
Extension contracture
Foot/ankle
Equinovarus
Rocker bottom
Hind foot valgus

20. Cocktail Personality Verbose, irrelevant conversation
Poor pragmatic use of language
Increased use of routine social phrases

21. Learning Disorders Clinical
Achievement tests reveal deficits in arithmetic more than reading or spelling
Visuomotor dysfunction more common and severe in higher level lesions
Math and visual-spatial skills fall more behind normal peers with age
Evaluation
Neuropsychologic testing

22. Psychological Issues Family stress Increased divorce rate
Increase substance abuse
Altered relationships
Overprotected
Decreased socialization
Sexual dysfunction
Penile erection partial and unsustained
Ejaculation may be absent or retrograde
Females usually fertile
Marriage and parenting
1 in 10 risk of parenting infant with neural tube deficit

23. Projected Mobility

24. S2 Level (S2 intact, S3 absent) Motor activity Intact
Hip flexion, extension, adduction, abduction
Knee extension, flexion
Foot dorsiflexion, plantarflexion, adduction, abduction 
Absent
Foot intrinsics
Forces and deformities
Foot claw, cavovarus
Mobility
Walking with or without bracing and crutches

25. S1 Level (S1 intact, S2 absent) Mobility
Walking with or without bracing and crutches
Intervention
Hinged FRAFO  
Supramalleolar orthoses (SMO) 
Athletic shoes
Lightweight
Stable
Designed for performance

26. L5 Level (L5 intact, S1 absent) Motor activity Intact
Hip flexion, adduction, abduction
Knee extension, flexion
Foot dorsiflexion, inversion
Absent
Hip extension
Foot planter flexion, eversion
Mobility
Walking with or without bracing and crutches

27. L4 Level (L4 intact, L5 absent) Motor activity Intact
Motor activity
Intact
Hip flexion, adduction
Knee extension
Foot dorsiflexion, inversion 
Absent
Hip extension, abduction
Knee flexion
Foot plantar flexion, eversion
Mobility
Walking with or without bracing and crutches

28. L3 Level (L3 intact, L4 absent) Motor activity Intact
Motor activity
Intact
Hip flexion, adduction, rotation (weak)
Knee extension (antigravity or greater)
Mobility
Limited walking with significant bracing, crutches, and walker
Intervention
Knee-ankle-foot orthoses (KAFO) 
Floor-reaction-ankle-floor orthoses (FRAFO)

29. L2 Level (L2 intact, L3 absent) Motor activity Intact
Hip flexion, adduction
Knee extension (weak)
Forces and deformities
Mobility
Limited walking with significant bracing, crutches, and walker
Intervention
Hip abduction brace
Thoraco-hip-knee-ankle-foot orthoses (THKAFO) 
Reciprocating gait orthoses (RGO)

30. Thoracic Level Motor activity Absent lower extremity action Mobility
Wheelchair
Intervention
Thoraco-lumbar-sacral orthoses  
Parapodium

31. Focus and Desired Outcomes

32. General Desired Outcomes
Overall outcome
Maintain health status and prevent secondary conditions and complications
Specific outcomes
Control hydrocephalus
Optimize neurological functioning
Preserve renal function
Obtain urinary continence
Obtain bowel continence
Progress in growth and development
Obtain and maintain mobility
Progress in psychological and sexual development

33. General Desired Outcomes
Up to date with current immunizations
Maintain ideal body weight
Maintain good general health
Absence of repeated infection
Stable cardiovascular and respiratory status
Intact skin
Balance patterns of eating, sleeping, and exercise
Absence of orthopedic deterioration
Obtain and maintain positive attitude and self esteem
Follow latex precautions

34. Toddlerhood 1-3 Years
First to third year of life is referred to as the toddler stage. During this time the child experiments with holding on and letting go, (control issues), and begins to attach enormous value to will power. Extensive exploration of environment occurs. This is the beginning of independence. For growth and development to progress, child needs to have appropriate opportunities to express autonomy. Parents play vital role in encouraging safe, appropriate independence

35. Toddlerhood 1-3 Years Normal intracranial pressure maintained
Optimal mobility achieved
Bladder drainage and renal function remain normal
Urinary tract infections controlled
Bowel continence program initiated
Normal range for height and weight
Child enrolled in early intervention school program
Parents receiving adequate information and support
Mother aware of importance of taking folic acid
Latex precautions being followed

36. Preschool 3-5 Years
Third year through firth year of life is considered the preschool stage. During this stage the child want to know about and do everything. A beginning understanding of boy/girl roles, body image, and body boundary develops. Much is explored through imagination. Parent can do a lot to encourage child’s need to now and understand so that growth and development will progress. Learning self-care skills becomes important

37. Preschool 3-5 Years Normal intracranial pressure maintained
Optimal mobility maintained
Bladder drainage and renal function remain normal
Urinary tract infections controlled
Social continence of bowel and bladder achieved
Normal range for height and weight
Child enrolled in appropriate preschool program
Child’s overall development is progressing
Parents receiving adequate information and support
Necessary durable medical equipment, including wheelchair obtained for mobility and school bus transportation
No deterioration of skills evident
Latex precautions being followed

38. School Age 6-11 Years
Sixth year through eleventh year is referred to as the school-age stage. It is the time when the child need to feel mastery and completion of tasks and projects and be recognized for them. This will contribute to self-esteem and self-confidence. Peers become increasingly important. Parents can do a lot to facilitate peer socialization and a feeling of recognition for what is accomplished

39. School Age 6-11 Years Normal intracranial pressure maintained
Optimal mobility maintained
Secondary disability due to tethered cord due to preadolescent growth spurt prevented
Bladder drainage and renal function remain normal
Urinary tract infections controlled
Child becoming independent in bowel and bladder management, brace application, skin checks, and other self care activities
Child follows regular exercise program
Normal range for height and weight
Child enrolled in appropriate grade school with full inclusion in classroom and extracurricular activities
Parents receiving adequate information and support
Mother aware of importance of taking folic acid
Child following and able to discuss latex precautions and prevention

40. Questions?