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spina bifida Flashcards

1
Q

what is spina bifida

A

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

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2
Q

prevalance of spina bifida

A

0.4-0.9 per 1,000 babies (less than CP)
higher prevalance in hispanics and caucasians compared to african americans and asians
90-95% have no family history

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3
Q

open spinal dysraphism (OSD)

A

meninges and/or nerve tissue are exposed to the external environment. most prevalant

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4
Q

closed spinal dysraphism (CSD)

A

meninges and nerve tissue are covered by skin. mildest form of the condition

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4
Q

prenatal diagnosis at 13-15 weeks

A

blood lab - alpha fetoprotein (AFP), 80% reliable

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5
Q

prenatal diagnosis 16 - 18 weeks

A

amniocentessis -100%

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5
Q

prenatal diagnosis 16-24 weeks

A

fetal ultrasound >90% reliable

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6
Q

most common location

A

L5-S1

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7
Q

complexity

A

Complications can range from minor to severe
physical and mental disabilities.
Impact determined by:
* Size/Location of malformation
* Open vs closed
* Which spinal nerves are involved

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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8
Q

causes

A

genetic
environmental
nutritional

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9
Q

folic acid relationship

A

(vitamin b9) when taken by the mother prenatally reduces the risk of neural tube defects by up to 70%

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10
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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11
Q

NM, 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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12
Q

skeletal impairments

A

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

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13
Q

other possible complications

A

Hydrocephalus
* Arnold Chiari
Malformation
* Seizures
* Cognitive impairments
* Tethered Cord
* Bowel/bladder
complications
* Latex allergy (73%)

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14
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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15
Q

hydrocephalus signs and symptoms

A

headache
nausea
lethargy
vision problems
hoarse cry
swallow issues
changes in speech
seizures

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16
Q

arnold-chiari malformation type 2

A

portion of the brain stem descends into the cervical spine

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17
Q

arnold-chiari malformation type 2 : signs and symptoms

A

changes in breathing pattern
swallowing problems (gagging)
ocular muscle palsies
weakness/spasticity in arms
bradycardia

18
Q

club foot

A

20-50% of infants w spina bifida

many factors:
- muscle imbalance
- contractures
-intrauterine positioning
- spasticity

19
Q

thethered 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

20
Q

signs and symptoms tethered cord

A

weakness
scoliosis
pain
orthopedic deformity
urologic dysfunction

dimple or y gluteal cleft

21
Q

motor testing

A

motor level determined by which muscle group can be graded 3/5 MMT scale

22
Q

common assessments - body function and structure

A

goni - ROM
myotomes and dermatomes
HHD or MMT
gait

23
common assessments - acticity and participation
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
24
motor development first 6 months
motor development is close to typical (head control and hand use) but less LE movement
25
motor development - rolling
children with THORACIC level SB usually roll by 18 months with compensatory strategies, many will progress to crawling
26
motor development - sitting
children with MID LUMBAR SB can sit with support with delay and increased lordosis, typically development in L4-L5 lesions
27
motor development - pull to stand/cruise
children with LOW LUMBAR lesions, may progress to ambulation with AD with an average onset of age 3
28
ambulation prediction for lower motor level
lower motor level = need good quad strength no history of shunt = cognitive function necessary for ambulation no history of hip or knee contracture surgery
29
functional ability by motor level T6-T12
wc for mobility non-functional ambulation and standing degree achieving community ambulation 0-33%
30
functional ability by motor level L1-L3
wc for mobility limited household ambu dg acheiving community ambu ~30%
31
functional ability by motor level L3-L4
household ambu limited community ambu wc for long distances dg achieving community ambu ~30%
32
functional ability by motor level L4-L5
household and community ambu wc for long distances dg achieving community ambu 38%
33
functional ability by motor level S1-S2
community ambu ALL
34
trendelenberg gait
Hip abductor weakness (Motor innervation L4-S1) * Associated with weak hip extensors and plantar flexors * Trunk sways side to side * Knee valgus
35
medical management - first step
fetal or post natal surgery The Management of Myelomeningocele Study (MOMS): Fetal surgery leads to improved mobility and fewer surgeries for shunt placement (Houtrow, 2020) * Fetal surgery is higher risk to the baby and the mother
36
medical management next step
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
37
medical management orthopedics - scoliosis and/or kyphosis
70% with lesions above L2 once detected - obtain spine x ray every 1-2 years may consider surgery when Cobb angle > 40 dg surgical options include fusion or growing rods
38
medical management orthopedics - hip sublux and dislocation
fractures 11-30% in children with SB related to osteopenia (most common: distal femur and femoral neck)
39
SB early intervention
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
40
partial BW supported treadmill training
* 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 ~2months sooner
41
age 1-5 rec
monitor for change - neuro level, strength, gait, sensation, bowel/bladder, MSK get them upright and WB everyday - standing frame and mobility device if not pulling to stand discuss mobility options - ambu w orthotics and or AD manual vs power wc
42
6-12 age recc
monitor for change - neuro level, strength, gait, sensation, bowel/bladder, MSK discuss benefits of diff mobility devices for diff settings - school, home, community flexibilty and strengthening exercises - emphasize UE strength to improve wc mobility and functional activities explore adapted recreational sports options - promote daily PA and WB activities
43
ambulation in children w SB
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
44
motor function into adulthood w SB
considerations for PT management and family ed: increasing body size joint degradation decrase in bone density painful movement motivation independent living 30-60% employment rate 25-50%
45

neuro peds (8 decks)

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