Spina Bifida Flashcards

1
Q

Etiology

A

Maternal lack of folic acid in first trimester

Also associated with valproic acid (seizure medication)

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2
Q

Myelodysplasia

A

General terminology that encompasses all of the kids

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3
Q

SBO

A

Involves non-fusion of the halves of the vertebral arches

Without disturbance of the underlying neural tissue

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4
Q

Myelocele

A

Protruding sac containing meninges and CSF

NR and SC remain intact in their normal positions

No motor or sensory deficits, associated hydrocephalus my or other CNS problems

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5
Q

Lipomeningocele

A

Superficial fatty mass in the low lumbar or sacral level of the SC

Significant neurologic deficits and hydrocephalus are NOT expected

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6
Q

Myelomeningocele

A

Spinal cord
Meninges
CSF

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7
Q

Prenatal Testing and Dx

A

AFP - remain abnormally high after 14 weeks gestation

Fetal ultrasound

Fetal surgery

Repair the exposed spine in utero - assoc with decreased hydrocephalus, no significant changes in motor fx

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8
Q

Prognosis

A

Greater than 90% survival with early treatment

  • Closure of spine
  • Shunt for hydrocephalus
  • Clean, intermittent catheterization
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9
Q

Impairments

A

Disruption of nerve conduction below level of the lesion

Joint contracture due to mm imbalances

Loss of sensation

Loss of bowel and bladder control

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10
Q

Associated Disorders

Neurological

A

Hydrocephalus

Arnold Chiari Malformation

Hydromyelia

Tethered cord

Decreased speech and cognitive function - cocktail party speech

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11
Q

Associated Disorders

MSK

A

Club feet

Congenital hip dislocation

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12
Q

Hydrocephalus

A

An abnormal accumulation of CSF in cranial vault

Causes…
Overproduction CSF
A failure in absorption of CSF
Obstruction in normal flow of CSF through brain structures and SC

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13
Q

Management of Hydrocephalus

A

90% of SB will develop this

Shunt placed within first several days to 6 mos

Thin, flexible tube that diverts CSF away from the lateral ventricles

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14
Q

VA shunt

A

Moves excess CSF from one lateral ventricle to the right atrium of the heart

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15
Q

VP shunt

A

Preferred treatment for hydrocephalus

Drains to abdomen

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16
Q

Chiari II Malformation

A

Common to children with Spina Bifida

Deformity of the cerebellum, medulla, and cervical spinal cord

The posterior cerebellum is herniated downward through foramen magnum

Brainstem structures also displaced in a caudal direction

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17
Q

Signs of shunt malfunction

A
Headache
Irritability
Fever unrelated to illness
Nausea
Increased spasticity in INN mm
Increase difficulty with postural control
Decreased school performance
Decreased level of consciousness
"Sunset" sign of the eyes
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18
Q

Sunset sign

A

Downward rotation of eyes within orbit so you only see half the iris and pupil

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19
Q

Hydromyelia

A

Excess CSF collects in pockets down the SC that creates areas of pressure and necrosis of the surrounding PERIPHERAL NERVES

Causes scoliosis

Progressive UE weakness and hypertonus

Requires shunting

20
Q

Tether Spinal Cord

A

Adhesions anchor the SC at the sight of the original lesion

Cord is not free to slide upward and reposition

Excessive stretch causes metabolic changes and ischemia of the neural tissue

Associated degeneration in mm function

21
Q

Signs of Tethered Spinal Cord

A

Rapidly progressive scoliosis

Hypertonus at one or several sites in the LEs

Changes in gait pattern

Changes in urologic function

Increase tone in passive ROM

Asymmetric changes in MMT results

Areas of decreasing strength

Discomfort in the back

Requires surgical repair***

22
Q

Management of Neonate

A

Prevent infection

Closure of the back within 72 hours of birth

23
Q

Developmental Issues

A

Mild/moderate developmental delay

Develops compensatory strategies for low tone/proprioceptive input

Movement and exploration is limited

24
Q

Handling Strategies for Parents

A

Education should focus on gross, fine, and perceptual motor abilities

UE/LE, head, and trunk

Learning pace depends on child and family capacity to learn

Emphasize upright positions

25
PT for Growing Child
Long-range plan of care Based largely on objective findings from PT's eval Repeated MMT and careful observation of child's development - ID child's strengths and weaknesses - Intervention directed at specific needs of LEs and gross motor development
26
Developmental Concerns
Equilibrium and righting rxns Prone positioning Early WB Assessment of quality of movement
27
Infant devices
Can have a negative impact on motor development and sensorimotor learning Therapist should discourage use of these Encourage active participation of these upright experiences by parents
28
General Principles of Bracing
Predictable level of mobility exists for children at each motor level - more individualized and "conservative" Early standing and gait training for as long as seems reasonable - as functional for as long as possible
29
Children with thoracic level paralysis
Posture of flaccid lower extremities and at risk for developing a frog-legged deformity Total contact body brace Include a lower leg section to hold ankle in neutral or plantigrade position
30
T6 through T12
Upper trunk working No LE mm working Tendency to have... Kyphoscoliosis Frog contractures Club feet Orthosis needed - TLSO, AFOs, night splints, leg wraps Parapodium
31
Parapodium
May show some potential for upright mobility when young and may grow out of this potential
32
Children with high lumbar paralysis
Require a high level of bracing for standing and ambulation Are at high risk for hip subluxation/dislocation
33
L1 to L3
Mm working - hip flexors, ADD, minimal knee ext Tendency to have - hip flex contracture, hip dislocation, wind blown posture, scoliosis Orthosis needed - ABD splint, A-frame Parapodium early, HKAFO later, RGO (reciprocating gait orthosis)
34
L4-L5
Mm working - Knee ext, ankle investors, ankle DF, hip ABD, minimal knee flex Tendency to have - hip flexion contractures, hip dislocations, lumbar lordosis, Calcaneovarus Orthosis needed - night splint (ABD), RGO, HKAFO, KAFO, AFO later
35
L4-L5 Presentation
Strong hip flexors and ADD Calcaneal Valgus/varus deformities Clubfoot deformity RGOs AFOs if trunk control KAFO if crouch present
36
S1-S2
Mm working - knee flexors, hip ext, ankle evertors and PFs, toe flexors Tendency to have - Calcaneovalgus or varus, toe clawing, foot ulcers Orthosis needed - AFO, SMO, shoe inserts, nothing, need caution with choice of foot wear
37
Orthotics for children with sacral level paralysis
Most control of hip/knee AFOs Work on active trunk and lower extremity alignment
38
Casting Following Orthopedic Surgery
Usually required for 2-8 weeks after surgery Consider positioning while in the cast Prevention of pressure sores Decreased mobility experiences
39
Scoliosis
Bracing may help decrease, but not eliminate progression Function becomes difficult Surgical option for fusion
40
Latex Allergy
Allergic rxn can develop with increased exposure Increased incidence for children with SB
41
Perceptual Motor and Cognitive Performance
Wide variety of cognitive levels Some studies show increased attn deficit Decreased ocular fx
42
Wheelchair mobility
Adapted strollers for young children Standard wheelchair by school age Power wheelchair and scooters as child ages
43
Transition to Adolescence and Adulthood
Prevention of weight gain and promotion of life-long health related fitness Hormonal changes, growth, weight gain can cause fatigue and loss of interest in walking Spinal stabilization procedures often occur in adolescence and can lead to loss of ambulation Larger school communities require faster mobility of the wheelchair Moving to a wheelchair should not be see as failure Educational and vocational opportunities must be explored
44
Adults with SB Common Complaints
``` Obesity Incontinence Recurrent urinary tract infections Chronic decubiti Joint pain Hypertension Neurologic deterioration Depression ``` Self-image important UTI issues most common cause of morbidity
45
Spina Bifida
Neural tube defect causing neurological dysfunction Second most common birth defect