A Functional Approach to Serving Adolescents with

Spina Bifida

PresenterFred Klingbeil, MD

Medical Director Pediatric Rehabilitation Medicine

Primary

Condition

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

Spina Bifida Occulta (5 percent of population)

5 percent of population

Incomplete vertebral closure without meninges or cord protrusion, and no

signs of neurologic impairment

Spina Bifida Occulta

Clinical L5-S1 (most common) Dermal hyperpigmentation Hairy patch Lump Dermal sinus Initially asymptomatic

 

Complications Hinders spinal cord mobility with growth

Spina Bifida Cystica

Protrusion of sac through

unfused vertebral arch defectMeningocele Incomplete vertebral closure with meninges protrusion

and no sign of neurologic impairmentMyelomeningocele Incomplete vertebral closure with meninges and cord

protrusion, and signs of neurologic impairment 47% lumber 26% thoracic 20% sacral 2% cervical

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)

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

Chiari Malformation

Chiari refers to malformation and displacement of the hindbrain

into cervical vertebral canal

Chiari Malformation

Occurrence Occurs in all children with hydrocephalus but is

symptomatic in only 20-30 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

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

Neurogenic Bladder

Flaccid (“Keeper”) TypePathophysiology Failure to empty due to ineffective bladder contractility

and/or ineffective urethral relaxation

Complications Urinary tract infection Vesicoureteral reflux, hydronephrosis Overflow incontinence

ManagementClean intermittent catheterizationProphylactic antibiotics

Neurogenic Bladder

Spastic (“Leaker”) TypePathophysiology 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

Neurogenic Bowel

Pathophysiology• Failure to adequately control bowel

usually due to involuntary relaxation and/or contraction of internal anal sphincter

Neurogenic Bowel

Clinical Absent anal sensation Incontinence Constipation

Intervention Dietary suggestions Postprandial routine education Fiber diet  Rectal suppositories Enema

Obesity

Causes Decreased metabolic rate Increased body fat Decreased lean body mass Increased caloric intake as awards

 

Intervention Caloric intake 50-75 percent of normal Low cholesterol and unsaturated fat diet Maximize activity

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

Orthopedic Deformities

SpineKyphosisScoliosisLordosis

 

KneeFlexion contractureExtension contracture

Foot/ankleEquinovarusRocker bottomHind foot valgus

Cocktail Personality

Verbose, irrelevant conversation

Poor pragmatic use of language

Increase use of routine social phrases

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

Reproduction

Clinical Females

Most are fertile Normal labor, delivery

Males Unsustained, partial erection Absent, retrograde ejaculation Abnormal sexual stimulation

 

Management Education of alternative sexual stimulation Obstetric consultation Artificial penile implant

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

Projected Mobility

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

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

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

L4 Level

(L4 intact, L5 absent)

 

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

L3 Level

(L3 intact, L4 absent)

 

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)

L2 Level

(L2 intact, L3 absent)

Motor activity Intact Hip flexion, adduction Knee extension (weak)

Forces and deformities Hip flexion, adduction

Mobility Limited walking with significant bracing, crutches, and walker

Intervention Hip abduction brace Thoraco-hip-knee-ankle-foot orthoses (THKAFO)  Reciprocating gait orthoses (RGO)

Thoracic Level

Motor activity Absent lower extremity action

Mobility Wheelchair

Intervention Thoraco-lumbar-sacral orthoses   Parapodium

Focus and Desired Outcomes

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

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

Adolescent stage 12-18 years

Twelve through eighteenth year of life is referred to as the adolescent stage. It is a time when a child seeks his/her own identity, (Who am I? Where am I going? What do I want to be? What can I be?), and independence. To achieve this, conflicts with parents are both natural and necessary. Peers are extremely important throughout this stage. Close parent/child communication helps the child grow and develop into responsible adulthood

Adolescent stage 12-18 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 Urinary and fecal continence maintained Awareness of sexual issues Independent in all self care, including dressing, bathing, elimination,

wheelchair transfers, brace application, and driving Teen becoming independent in bowel and bladder management,

brace application, skin checks, and other self care activities

Adolescent stage 12-18 years

Teen follows regular exercise program Normal range for height/weight ratio Teen receiving adequate information and support Teen has participated in adaptive driver’s education course Teen understands importance folic acid supplementation Teen enrolled in appropriate grade school with full inclusion in

classroom and extracurricular activities Teen following and able to discuss latex precautions and prevention Teen aware of safe sex and contraceptive options Parents receiving adequate information and support

Questions?