Spina Bifida Flashcards
1
Q
What is it? How common? What causes it?
A
- Neural tube defect causing neurologic dysfunction
- Second most common birth defect
- No definitive cause has been established
2
Q
Linked to? Also associated with?
A
- Linked to maternal lack of folic acid in first trimester
- Also associated with valproic acid (Seizure medication)
3
Q
Spinal defect diagnosed by? Contains? Can be? Most commonly located where?
A
- the presence of an external sac on the infant’s back
- Contains meninges and spinal cord tissue protruding through a dorsal defect in the vertebrae
- Can be covered by membrane or exposed
- Most commonly located in the lumbar region
4
Q
Spinal bifida occulta involves? Without?
A
- Involves nonfusion of the halves of the vertebral arches
- Without disturbance of the underlying neural tissue
5
Q
What is Myelocele? (2) Deficits?
A
- Protruding sac containing meninges and cerebrospinal fluid (CSF)
- Nerve roots and spinal cord remain intact and in their normal positions
- No motor or sensory deficits, associated hydrocephalus, or other central nervous system (CNS) problems
6
Q
What is Lipomeningocele? (2)
A
- Superficial fatty mass in the low lumbar or sacral level of the spinal cord
- Significant neurologic deficits and hydrocephalus are not expected
7
Q
Prenatal Testing and Diagnosis? (7)
A
- α-Fetoprotein levels (AFP)
» Remain abnormally high after 14 weeks gestation - Fetal ultrasound
- Fetal surgery
- Repair the exposed spine in utero
» Associated with decreased hydrocephalus
» No significant changes in motor function
8
Q
Prognosis? What is tx? (3)
A
- Greater than 90% survival with early treatment
- Closure of spine
- Shunt for hydrocephalus
- Clean, intermittent catheterization
9
Q
Impairments: are based on the level of the lesion and can include? (4)
A
- Disruption of nerve conduction below level of the lesion
- Joint contracture due to muscle imbalances
- Loss of sensation (need to brace)
- Loss of Bowel and Bladder control
10
Q
Associated disorders - neurological? (6)
A
- Hydrocephalus
- Arnold Chiari Malformation
- Hydromyelia (herniation through ventricles)
- Tethered Cord
- Decreased speech and cognitive function
- “Cocktail party” speech
11
Q
Associated disorders - musculoskeletal? (2)
A
Club Feet
Congenital Hip Dislocation
12
Q
What is Hydrocephalus? Causes? (3)
A
- An abnormal accumulation of cerebral spinal fluid (CSF) in the cranial vault
Causes:
- Overproduction of CSF
- A failure in absorption of CSF fluid
- Obstruction in the normal flow of CSF through the brain structures and spinal cord
13
Q
Management of Hydrocephalus? What is it?
A
- Shunt placed within first several days to 6 months
- Thin, flexible tube that diverts CSF away from the lateral ventricles
14
Q
Ventriculoatrial (VA) shunt moves? Ventriculoperitoneal (VP) shunt is?
A
- Ventriculoatrial (VA) shunt
Moves excess CSF from one lateral ventricle to the right atrium of the heart - Ventriculoperitoneal (VP) shunt
Preferred treatment for hydrocephalus
15
Q
Chiari II Malformation is? (3)
A
- Deformity of the cerebellum, medulla, and cervical spinal cord
- The posterior cerebellum is herniated downward through the foramen magnum
- Brainstem structures also displaced in a caudal direction
16
Q
Signs of Shunt Malfunction? (9)
A
- Headache
- Irritability
- Fever Unrelated to Illness
- Nausea
- Increased spasticity in innervated muscles
- Increased difficulty with postural control
- Decreased school performance
- Decreased level of consciousness
- “Sunset” sign of the eyes bc of increased ICP and decreased rotation
17
Q
Hydromyelia is? Causes? (2) Requires?
A
- Excess CSF collects in pockets down the spinal cord that created areas of pressure and necrosis of the surrounding peripheral nerves
- Causes scoliosis
- Progressive upper extremity weakness and hypertonus
- Requires shunting
18
Q
Tethered Spinal Cord is? (2) Causes? Assosciated?
A
- Adhesions anchor the spinal cord at the site 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 muscle function
19
Q
Tethered Spinal Cord signs? (7) Requires?
A
- Rapidly progressive scoliosis,
- hypertonus at one or several sites in the lower extremities,
- changes in gait pattern and changes in urologic function,
- increased tone on passive
- ROM,
- asymmetric changes in manual muscle testing results,
- areas of decreasing strength, or discomfort in the back
- Requires surgical repair
20
Q
Families and specialists must monitor ? Early intervention can?
A
- Families and specialists must monitor deterioration of skills/function
- Early intervention can minimize the effects of secondary complications
21
Q
Objectives of Management - facilitate? Achieve? Reduce? Maintain?
A
- Facilitate mastery of appropriate developmental skills
- Achieve highest level of functional mobility via ambulation or w/c
- Reduce contractures and prevent further contractures
- Maintenance of weight
22
Q
Objectives of Management - conduct? (2) Assist with? Investigate? Facilitate?
A
- Conduct ongoing assessment of neurological, musculoskeletal and functional assessment
- Conduct ongoing patient and parent education
- Assist with equipment acquisition and adjustment
- Investigate home / school situation and coordinate program planning with other disciplines
- Facilitate age appropriate participation in activities
23
Q
Management of the Neonate? (3)
A
- Studies support early and aggressive intervention
- Prevent infection
- Closure of the back within 72 hours of birth
24
Q
Care for the Young Child? (2)
A
- Followed by team of professionals over the lifetime
- Facilitation of communication for all involved
25
Developmental Issues? (3)
- Mild/moderate developmental delay
- Develops compensatory strategies for low tone/proprioceptive input
- Movement and exploration is limited
26
Handling Strategies for Parents? (4)
- Education should focus on gross, fine, and perceptual motor abilities
- Upper/lower extremities, head, and trunk
- Learning pace depends on child and family capacity to learn
- Emphasize upright positions
27
Physical Therapy for the Growing Child - kind of care? Based on? Repeated? Identify? intervention is?
- Long-range plan of care
- Based largely on the objective findings from the physical therapist’s evaluations
- Repeated manual muscle tests and careful observation of the child’s development
- Identify the child’s strengths and weaknesses
- Intervention is directed at the specific needs of the lower extremities and gross motor development
28
Developmental Concerns? (4)
Equilibrium and righting reactions
Prone positioning
Early weight bearing
Assessment of quality of movement
29
Infant Devices - which ones are negative? (5) Therapist should? Encourage?
- Infant walkers, jumper seats, swings, bouncer chairs, and the excessive use of infant car seats 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
30
General Principles of Bracing(2 schools of thought) - Predictable level of? Advocates? Many factors?
- mobility exists for children at each motor level
- Advocates establishing reasonable expectations for each child
- Many factors affect continuation and discontinuation of ambulation
31
General Principles of Bracing(2 schools of thought) - do what for as long as reasonable? Children?
- Early standing and gait training for as long as seems reasonable
- Children attain their optimal level of performance, regardless of their motor level, and assist them to maintain this level for as long as is feasible
32
Children with Thoracic Level Paralysis - posture? Total? Include?
- 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 the ankle in a neutral or plantigrade position
33
Thoracic Level Lesions - T-6 – T-12 - present how? (2) Tendency to have? (3) Orthosis needed? (5) Why?
- Upper trunk working, No LE Muscles working
-Tendency to have
Kyphoscoliosis
Hip abduction / external rotation (frog) contractures
Club feet
- Orthosis Needed:
TLSO, AFO’s, Night Splints or leg wraps
Parapodium
May show some potential for upright mobility when young and may out grow this potential
34
Children with High Lumbar Paralysis require? Are at?
- Require a high level of bracing for standing and ambulation
- Are at high risk for hip subluxation/dislocation
35
Lumbar Lesions L1- L3 - muscles working? (3) Tendency to have? (4) Orthosis needed? (5)
- Muscles working:
Hip flexors, adductors, minimal knee extensors
- Tendency to have
Hip flexion contractures, hip dislocation, wind blown posture, Scoliosis
- Orthosis needed
Abduction splint, or A-Frame
Parapodium early, HKAFO’s later, RGO
36
Orthotics for Children with Thoracic & High Lumbar Paralysis? (8)
- Early standing can use an A frame
- Swivel standers
- HKAFOs (hip knee ankle foot orthoses)
- RGOs (reciprocal gait orthoses)
- Rolling walkers
- Lofstrand crutches
- Swing through gait
- Wheelchair sports, recreational activities toward adolescence
37
L4 – L5 Muscles working? (5) Tendency to have? (5) Orthosis needed? (5)
- Muscles working
L4 knee extensors, ankle invertors and dorsiflexors
L5 Hip abductors, minimal knee flexors
- Tendency to have
Hip flexion contractures, hip dislocation, lumbar lordosis, calcaneovarus, calcaneous
- Orthosis needed
Night Splint (abduction), RGO, HKAFO, KAFO, AFO later
38
Orthotics for Children with Low Lumbar Paralysis(L4/L5)? (6)
- Strong hip flexors and adductors
- Calcaneal valgus/varus deformities
- Clubfoot deformity
- RGOs
- AFOs if trunk control
- KAFO if crouch present
39
S1- S2 muscles working? (5) Tendency to have? (4) Orthosis needed? (5)
- Muscles working:
Knee flexors, hip extensors, ankle evertors and plantar flexors, toe flexors
- Tendency to have:
Calcaneous, calcaneovarus, toe clawing, foot ulcers
- Orthosis needed
AFO, SMO, shoe inserts, nothing, need caution with choice of foot wear
40
Orthotics for Children with Sacral Level Paralysis? (3)
Most control of hip/knee
AFOs
Work on active trunk and lower extremity alignment
41
Casting following Orthopedic Surgery? (4)
- Usually required for 2-8 weeks after surgery
- Consider positioning while in the cast
- Prevention of pressure sores
- Decreased mobility experiences
42
Scoliosis - bracing may? Functions becomes? Other option?
Bracing may help decrease but not eliminate progression
Function becomes difficult
Surgical option for fusion
43
Common allergy? Depended on? Allergic rx can develop with?
- Latex Allergy
- Depended on for its impermeable qualities and strength while still providing sensitivity to touch
- Allergic reaction can develop with increased exposure
44
Perceptual Motor and Cognitive Performance? Some studies show? (2)
- Wide variety of cognitive levels depending on treatment of hydrocephalus, episodes of cerebral infection, and the presence of other CNS abnormalities
- Some studies show increased attention deficit
- Decreased ocular function
45
Wheelchair Mobility options? (3)
- Adapted strollers for young children
- Standard wheelchair by school age
- Power wheelchair and scooters as child ages
46
Recreation and Leisure Activities? (2)
Encourage full participation in recreational activities with adaptations as needed
Aquatics
47
Transition to adolescence and adulthood - prevention of? What other changes? Spinal stabilization procedures often occur in? Larger school communities require? Moving to a wheelchair should?
- 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 seen as failure
48
The Adult with Spina Bifida - Common complaints include? (10)
- Obesity, incontinence, recurrent urinary tract infections, chronic decubiti, joint pain, hypertension, neurologic deterioration, and depression
- Self-image is important
- Urinary tract issues are most common cause of morbidity
