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Neuro Final > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Spina -

A

of the spine

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2
Q

Bifida –

A

split

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3
Q

Spina Bifida =

A

type of neural tube defect that occurs when one or more vertebrae fail to fuse at approximately 28 days of gestation, leaving the spinal cord unprotected in utero

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4
Q

Prevalence =

A

0.4 to 0.9 per 1000 births

Higher prevalence in Hispanics and Caucasians compared to African Americans and Asians

90-95% have no family history of Spina Bifida

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5
Q

Neural Tube Development and Spina Bifida:

A

By day 18-20 of gestation, the ectoderm (outer embryonic layer) thickens to form the neural plate, which will become the CNS

neural plate folds to form a groove, and the sides (neural folds) rise to close this groove into a tube, the neural tube

process of neural tube closure starts in the middle of the embryo and proceeds both toward the head (cranial end) and tail (caudal end), completing by day 28

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6
Q

Neural Tube Defect Leading to Spina Bifida:

A

occurs when the caudal end of the neural tube (the part that forms the spinal cord and vertebrae) fails to close properly by day 28

incomplete closure leads to the spinal cord and surrounding structures (bones, skin, etc.) being improperly formed or exposed, resulting in a range of physical and neurological impairments

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7
Q

Open spinal dysraphism (OSD) –

A

Meninges and/or nerve tissue are exposed to the external environment

due to a lack of skin or membrane covering

Most prevalent

generally more severe forms of neural tube defects

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8
Q

Closed spinal dysraphism (CSD) –

A

defect in the vertebrae or neural tube is covered by skin, and neural tissue is not exposed

Mildest form of the condition - may still cause significant functional impairments

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9
Q

Open Dysraphism (Open Neural Tube Defects) Examples:

A

Myelomeningocele
Anencephaly
Encephalocele

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10
Q

Closed Dysraphism (Closed Neural Tube Defects) Examples:

A

Spina Bifida Occulta
Lipomyelomeningocele
Tethered Cord Syndrome

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11
Q

Prenatal Diagnosis:

A

13-15 weeks: Blood Lab – Alpha-fetoprotein (AFP), 80% reliable

16-24 weeks: Fetal Ultrasound, >90% reliable

16-18 weeks: Amniocentesis, ~100% accurate

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12
Q

Complexity:

A

Complications can range from minor to severe physical and mental disabilities

The higher the malformation occurs on the back, the greater the amount of nerve damage and loss of muscle function and sensation

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13
Q

___ most common

A

L5-S1

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14
Q

Impact determined by:

A

Size/Location of malformation
Open vs closed
Which spinal nerves are involved

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15
Q

Causes and Risk Factors:

A

Exact cause is still an unknown

Genetic
Nutritional
Environmental

Folic acid

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16
Q

Genetic Factors:

A

most cases of spina bifida occur in families with no history of the condition

a family history of neural tube defects slightly increases the risk

genetic mutations or variations may affect the body’s ability to process folic acid

genes involved in the folate metabolism pathway, such as the MTHFR gene, have been linked to an increased risk

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17
Q

Nutritional Factors:

A

Folic Acid Deficiency
* essential for the proper closure of the neural tube

Women who do not get enough folic acid before and during the first trimester have a significantly higher risk of having a child with spina bifida or other NTDs

Malnutrition or deficiencies in other essential vitamins and minerals

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18
Q

Environmental Factors:

A

Some medications taken during pregnancy, especially anti-seizure drugs like valproic acid and carbamazepine

diabetes, obesity, and hyperthermia (exposure to high body temperatures, such as from fever or hot tub use during early pregnancy)

environmental toxins, such as alcohol, smoking, or certain chemicals

Older maternal age (above 35)

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19
Q

Folic acid =

A

(vitamin B9), when taken by the mother prenatally reduces the risk of neural tube defects by up to 70%

plays a key role in DNA synthesis and cell division

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20
Q

FDA/CDC Recommendation –

A

all women of childbearing age ingest 0.4 mg (400 µg) of folic acid every day

Dietary sources include dark leafy greens, whole grains, and legumes

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21
Q

Neuromuscular, Sensory and Motor Deficits:

A

Loss of sensation
Loss of muscle strength and control
Loss of bowel/bladder control
Muscle contractures

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22
Q

Skeletal Impairments:

A

Foot abnormalities (eg, club foot)
Shorter legs
Decreased bone density
Scoliosis
Hip subluxation and dislocation

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23
Q

Other Possible Complications:

A

Hydrocephalus
Arnold Chiari Malformation
Seizures
Cognitive impairments
Tethered Cord
Bowel/bladder complications
Latex allergy

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24
Q

Hydrocephalus =

A

Caused by a disturbance of formation, flow or absorption of cerebrospinal fluid

Approximately 25% at birth, which increases up to 90% who will require a ventriculoperitoneal (VP) shunt

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25
Q

Hydrocephalus Signs & Symptoms:

A

Headaches
Nausea
Lethargy
Vision problems
Hoarse cry
Swallow issues
Changes in speech
Seizures

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26
Q

Arnold-Chiari Malformation Type II:

A

Portion of the brain stem descends into the cervical spine.

structural abnormalities at the junction of the brain and spinal cord

particularly affecting the cerebellum and brainstem

strongly associated with myelomeningocele

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27
Q

Arnold-Chiari Malformation Type II Signs & Symptoms:

A

Changes in breathing pattern
Swallowing problems (e.g., gagging)
Ocular muscle palsies
Weakness/spasticity in arms
Bradycardia

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28
Q

Club Foot:

A

congenital talipes equinovarus

20-50% of infants with spina bifida

causes the foot to be twisted inward and downward, making it appear as if the person is walking on the side or top of their foot

can occur in one or both feet and ranges from mild to severe

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29
Q

Key Features of Clubfoot:

A

Equinus: The foot points downward, like a ballerina pointing the toes.

Varus: The heel is turned inward.

Cavus: The arch of the foot is elevated.

Forefoot Adduction: The front of the foot (forefoot) is turned inward.

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30
Q

Club Foot Many factors:

A

Muscle imbalance
Contractures
Intrauterine positioning
Spasticity

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31
Q

Club Foot Muscle imbalance =

A

flexor muscles (which pull the foot downward) may be stronger or tighter than the extensor muscles (which pull the foot upward),

causing the foot to point down and inward

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32
Q

Club Foot Contractures =

A

tight tendons and ligaments, particularly the Achilles tendon

can prevent the foot from moving into a normal position

pulling it into equinus (pointed downward) and varus (inward-facing) positions

soft tissues can become less elastic, making it difficult to reposition the foot without interventio

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33
Q

Club Foot Intrauterine Positioning =

A

Oligohydramnios (reduced amniotic fluid) can limit fetal movement, increasing the risk of musculoskeletal deformities

positional clubfoot occurs when the foot is forced into an abnormal position due to lack of space in utero
*more flexible and easier to correct than idiopathic

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34
Q

Club Foot Spasticity =

A

increased muscle tone and involuntary muscle contractions

can be more challenging to treat because of the ongoing muscle spasticity and abnormal reflexes, often requiring a combination of orthopedic management and spasticity treatments (e.g., botulinum toxin injections or selective dorsal rhizotomy)

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35
Q

Tethered Cord =

A

20-50% of children with spina bifida and re-tethering occurs for 10-15%

Caused by the spinal cord becoming fastened to part of the vertebral column which results in the spinal cord becoming abnormally stretched with the child’s growth

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36
Q

Tethered Cord Signs and symptoms:

A

Weakness
Scoliosis
Pain
Orthopedic deformity
Urologic dysfunction
Change in function

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37
Q

Tethered Cord dimple:

A

potential external signs

at the base of the spine, typically in the lumbosacral region

small indentation in the skin located at the base of the spine (usually above the buttocks)

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38
Q

Tethered Cord “Y” gluteal cleft:

A

groove between the buttocks

Y” shape may be visible in the lower part of the gluteal cleft due to abnormal skin changes or other indicators like a dimple, patch of hair, or fatty mass

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39
Q

Motor and Sensory Testing:

A

motor level is determined by the most caudal segment of the spinal cord that has normal function

motor Level determined by which muscle group can be graded at least 3/5 MMT scale

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40
Q

C5

A

Elbow Flexors
Biceps, brachialis

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41
Q

C6

A

Wrist extensors
Extensor carpi radialis longus, extensor carpi radialis brevis

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42
Q

C7

A

Elbow extensors
Triceps

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43
Q

T1

A

Small finger abductors
Abductor digiti minimi

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44
Q

L2

A

Hip flexors
Ilipsoas

45
Q

L3

A

Knee extensors
Quadriceps

46
Q

L4

A

Ankle dorsiflexors
Tibialis anterior

47
Q

L5

A

Long toe extensors
Extensor hallucis longus

48
Q

S1

A

Ankle plantar flexors
Gastrocnemius, soleus

49
Q

Common Assessments - ACTIVITY AND PARTICIPATION:

A

Alberta Infant Motor Scale– Motor function

Pediatric Evaluation of Disability Inventory (PEDI) – goal setting

WeeFIM – validated for use with children with spina bifida

School Function Assessment – Participation in school

Spina Bifida Health-Related Quality of Life

50
Q

Common Assessments - BODY FUNCTION & STRUCTURE:

A

Goniometry – Range of motion

Myotomes and Dermatomes

Hand-Held Dynamometry or MMT– Muscle strength

Gait and Movement Analysis – Gait deviations

51
Q

Motor Development:

A

First 6 months
Rolling
Sitting
Pull to stand/cruise

52
Q

First 6 months –

A

motor development is close to typical (head control and hand use) but less LE movement

53
Q

Rolling –

A

Children with thoracic level SB usually roll by 18 months with compensatory strategies, many will progress to crawling

54
Q

Sitting –

A

Children with mid-lumbar SB can sit with support with delay and increased lordosis, typical development in L4-L5 lesions

55
Q

Pull to stand/cruise –

A

Children with low lumbar lesions, may progress to ambulation with assistive devices with an average onset of age 3

56
Q

Ambulation Prediction:

A

Lower motor level - Need good quadriceps strength

No history of shunt - Cognitive function necessary for ambulation

No history of hip or knee contracture surgery

57
Q

Lower Motor Level:

A

Good Quadriceps Strength: critical for knee extension and stability during ambulation

If an individual has a lower motor level corresponding to at least L3 (where the quadriceps innervation occurs), and they can achieve at least 3/5 strength, it indicates a good chance for independent ambulation

58
Q

No History of Shunt:

A

Cognitive Function: The absence of a shunt often suggests that there may be less risk for hydrocephalus or associated cognitive impairments

Cognitive function is essential for safe ambulation, as it impacts the ability to understand and follow instructions, navigate environments, and react to obstacles or changes in terrain

59
Q

No History of Hip or Knee Contracture Surgery:

A

Mobility and Range of Motion: The absence of surgeries for hip or knee contractures is favorable for ambulation

Contractures can limit range of motion and functional mobility

Good joint mobility is crucial for walking mechanics and preventing compensatory patterns that may arise from limited range

60
Q

Functional Ability by Motor Level & Degree achieving community ambulation: T6-T12

A

Wheelchair for mobility

Non-functional ambulation and standing

0-33%

61
Q

Functional Ability by Motor Level & Degree achieving community ambulation: L1-L3

A

Wheelchair for mobility

Limited household ambulation

~30%

62
Q

Functional Ability by Motor Level & Degree achieving community ambulation: L3-L4

A

Household ambulation

Limited community ambulation

Wheelchair for long distances

~30%

63
Q

Functional Ability by Motor Level & Degree achieving community ambulation: L4-L5

A

Household and community ambulation

Wheelchair for long distances

38%

64
Q

Functional Ability by Motor Level & Degree achieving community ambulation: S1-S2

A

Community ambulation

All

65
Q

Gait Analysis - Trendelenburg Pattern:

A

Hip abductor weakness (Motor innervation L4-S1)

Associated with weak hip extensors and plantar flexors

Trunk sways side to side

Knee valgus

66
Q

Hip Abductor Weakness:

A

results in an inability to stabilize the pelvis during single-leg stance

affected side will drop during the stance phase of walking

muscles primarily responsible for hip abduction (gluteus medius and minimus) are innervated by the superior gluteal nerve, originating from the lumbar plexus (L4-S1)

67
Q

Weak Hip Extensors and Plantar Flexors:

A

Weakness in the hip extensors (gluteus maximus) can lead to decreased stability and propulsion during gait

Weakness in the plantar flexors (gastrocnemius and soleus) affects push-off during the gait cycle

68
Q

Trunk Sway:

A

sways side to side to compensate for the drop of the pelvis on the weak side

lateral trunk lean helps to maintain balance and center of mass over the support leg, reducing the demand on the weak hip abductors

69
Q

Knee Valgus:

A

(inward angling of the knees) often accompanies a Trendelenburg gait pattern due to compensatory strategies

occur as the individual tries to maintain balance and control over the center of mass, leading to increased stress on the medial structures of the knee and potential risk for further joint issues

70
Q

Multidisciplinary Approach:

A

Rehab Medicine
Physical Therapy
Occupational Therapy
Neurosurgery
Orthopedics
Urology
Colorectal/GI
Nurse Clinic Coordinator
Nutritionist
Social Work
Neuropsychology

71
Q

Medical Management- First Step:

A

Fetal or Post-natal Surgery

The Management of Myelomeningocele Study (MOMS): Fetal surgery leads to improved mobility and fewer surgeries for shunt placement

Fetal surgery is higher risk to the baby and the mother

72
Q

Fetal Surgery:

A

Fetal surgery for myelomeningocele is typically performed between 19 and 25 weeks of gestation and aims to repair the defect before birth

can lead to better motor outcomes in children compared to those who undergo postnatal repair

increased risks for both the mother (including complications from surgery) and the fetus (including premature birth and other complications)

73
Q

Postnatal Surgery:

A

typically occurs within the first few days after birth to close the defect in the spinal column

less risky in terms of maternal health compared to fetal surgery and can be performed safely soon after birth

poorer functional outcomes, increased need for additional surgeries, and higher rates of shunt placement for hydrocephalus

74
Q

Medical Management - Second Step:

A

Surgery to place shunt

Small hollow tube that drains cerebrospinal fluid from the brain

Additional surgeries are needed as the child outgrows the shunt or it becomes clogged or infected

10-30% have seizure activity – associated with brain malformation or CSF shunt malfunction or infection

75
Q

Orthopedics - Scoliosis and/or kyphosis:

A

70% with lesions above L2

Once detected – obtain spine x-ray every 1-2 years, may consider surgery when Cobb angles >40 deg

Surgical options include fusion or growing rods

76
Q

Orthopedics - Hip subluxation and dislocation:

A

Fractures 11-30% in children with SB related to osteopenia (most common: distal femur and femoral neck)

77
Q

SPINA BIFIDA EARLY INTERVENTION:

A

Monitor joint alignment, muscle imbalance, and development of contractures

Use stretching, orthoses, and positioning splints to maintain alignment

Strengthen trunk and neck with tummy time and prone activities

Determine orthotics for ambulation as soon as they pull to kneel/stand

Power mobility can be introduced around 12 months of age

78
Q

Partial Body Weight Supported Treadmill Training

A

Enables parents to provide high repetition and functionally relevant activity

Builds muscle strength and motor control

Improves bone density

Enhancing sensory input led to more steps

Children walked ~2 months sooner

79
Q

Spina Bifida Guidelines of Care - T6-9
T9-12
L1

possible muscle function:

A

upper trunk (abdominals), no LE function

abdominals + paraspinals = some pelvic control

complete trunk function
- lower trunk (abdominals)
- hip flexors (weak) 2/5

80
Q

Spina Bifida Guidelines of Care - T6-9
T9-12
L1

possible orthopaedic concerns:

A

kyphoscoliosis, lumbar hyperlordosis

coxa valga - hip dislocation

decreased bone density

fractures

contractures
- hip: abduction, flextion, ER
- knee: flexion, extension
- foot: heelcord, clubfoot

81
Q

Spina Bifida Guidelines of Care - T6-9
T9-12
L1

possible orthotics needed:

A

TLSO
night splints: body, hip, abduction, KAF, AF

early: parapodium, (10 months of age and up to 2 years)

later: stander, RGO, HKAFO, KAFO

caution: preserve UE function with level transfers, stable seated posture

maintain strength + flexibility of shoulders/arms

82
Q

Spina Bifida Guidelines of Care - T6-9
T9-12
L1

possible equipment for functional mobility:

A

community: wheelchair/wheelchair cushion, transfer board

home: walker/crutches, raised and padded commode seat, bath bench, mirror for skin checks

stander: 1 hour/day minimum, starting at 10-12 months of age

driving with hand controls
learn public transportation

82
Q

Spina Bifida Guidelines of Care - L2
L3

possible muscle function:

A

hip flexors 3/5
hip adductors 3/5
knee extensors 3/5

83
Q

Spina Bifida Guidelines of Care - T6-9:

A

muscle function:

orthopaedic concern:

orthotics needed:

equipment:

function:

84
Q

Spina Bifida Guidelines of Care - L2
L3

possible orthopaedic concerns:

A

scoliosis, overuse of UE’s, lumbar hyperlordosis, hip subluxation

coxa valga - hip dislocation

decreased bone density

fractures

contractures:
hip - flexion
knee - flexion, extension
foot - heelcord, clubfoot

85
Q

Spina Bifida Guidelines of Care - L2
L3

possible orthotics needed:

A

night hip abduction splint

early: parapodium (10 months - 2 years)

later: stander, RGO, HKAFO, KAFO (if quads are less than 3/5 strength)

L3-5 may be temporarily addressed by twister cables or derotations straps

86
Q

Spina Bifida Guidelines of Care - L2
L3

possible equipment for functional mobility:

A

community: wheelchair + cushion

home: stander - 1 hour/day minimum

early: may use walker or crutches

later: wheelchair in home

87
Q

Spina Bifida Guidelines of Care - executive function

A

impairments can impact educational, social, and self help skills

88
Q

Spina Bifida Guidelines of Care - cognitive function

A

can vary with degree of hydrocephalus number of shunt infections, and involvement of NS

function may not be related to level of lesion or ability to walk

support early assessment of attention difficulties, sensorimotor integration, visual perception, visual motor ability, psychosocial development in addition to fine/gross motor + communication ability

89
Q

Spina Bifida Guidelines of Care - independent living

A

OT goals: basic activities of daily living (BADLs) or bathing, dressing, grooming, bowel/bladder program, skin care, moving/transportation in your home/community

instrumental activities of daily living (IADLs)

shopping, meal preparation, use of home appliances

90
Q

Ages 1-5 Years Spina Bifida Recommendations

A

Monitor for change = Neurologic level, strength, gait, sensation, bowel/bladder function, and musculoskeletal changes

Get them upright and weight-bearing every day = Standing frame and mobility device if not pulling to stand

Discuss mobility options = Ambulation with orthotics and/or assistive device, Manual vs. power wheelchairs

91
Q

Orthotics for Children with Spina Bifida:

A

HKAFO
KAFO
GRAFO
solid AFO
SMAFO

92
Q

HKAFO (Hip-Knee-Ankle-Foot Orthosis):

A

For children with high-level lesions (typically thoracic to upper lumbar levels), where there is significant weakness or paralysis in the lower limbs and trunk

Limits independent mobility, but can enable standing and assist in gait training for children with poor trunk control or high lesion levels

93
Q

KAFO (Knee-Ankle-Foot Orthosis):

A

For children with low lumbar to high sacral lesions, where hip muscles are strong enough to function, but knee or ankle control is insufficient

Helps children walk with assistive devices like crutches or walkers, but it may also restrict movement, depending on joint rigidity

94
Q

GRAFO (Ground Reaction Ankle-Foot Orthosis):

A

Commonly used for children with weak quadriceps and lower sacral lesions

t’s designed to improve knee extension during stance phase

Aids children in walking with improved knee stability, making it easier to walk independently or with minimal assistance

95
Q

Solid AFO (Ankle-Foot Orthosis):

A

For children with sacral-level lesions who need control at the ankle and foot due to weak plantarflexors or dorsiflexors

Helps children walk more efficiently by controlling foot drop and improving alignment during gait

96
Q

SMAFO (Supramalleolar Ankle-Foot Orthosis)

A

For children with mild involvement or those with lower sacral lesions, needing mild to moderate control of foot and ankle movement

Offers minimal restriction, allowing more natural foot and ankle movement, beneficial for children who have some control but need stability for balance or walking

97
Q

Equipment for Children with Spina Bifida

A

stander
mobile stander
manual wheelchair
reverse walker
forearm crutches

98
Q

Ages 6-12 Years Spina Bifida Recommendations

A

Monitor for change = Neurologic level, strength, gait, sensation, bowel/bladder function, and musculoskeletal changes

Discuss benefits of different mobility devices for different settings = School, home, community

Flexibility and strengthening exercises = Emphasize UE strength to improve w/c mobility and functional activities

Explore adapted recreational sport options = For promotion of daily physical activity and weight-bearing activities

99
Q

Ambulation in Children with Spina Bifida:

A

Some children with spina bifida will gain the strength and motor capacity to walk. Average onset is age 3.

Most likely will need to use a mobility support device.

Many children will lose this capacity later in life. Average offset is age 11.

100
Q

Considerations for PT management and family education:

A

Increasing body size
Joint degradation
Decrease in bone density
Painful movement
Motivation

Independent living: 30-60%
Employment rate: 25-50%

101
Q

Spina Bifida is a ___ defect and the most prevalent type is ___

A

neural tube

myelomeningocele

102
Q

There are several ____ complications associated with this diagnosis

A

neuromuscular, sensory, motor, skeletal, and other medical

103
Q

___ is delayed and ___ is dependent on the lesion level

A

Motor development

functional ability

104
Q

Consider the materials/equipment you use since ___ of individuals with spina bifida have a latex allergy

A

> 70%

105
Q

Follow the Guidelines for Care from the ____

A

Spina Bifida Association

106
Q

Promote ____ and ___ activities

A

physical activity
weightbearing

107
Q

CP Recap – Dosing/Frequency

A

task specific: Daily, integrated into routine

balance: Daily, integrated into routine

strength/power: 2-3x per week, non-consecutive days

gait training: 2-5x per week, time dependent on child’s fatigue (avg. 15-45 min)

Demonstrate the exercise/activity!

Neuro Final (10 decks)

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