Spina Bifida Flashcards by Meg Heil

Spina Bifida is the most common type of ________ defect

Neural tube

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_____ most common childhood abnormality/ disease causing disability after CP

2nd most common

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What are Risk factors for spina bifida

Polycentric inheritance- recurrence rate 2.5%- 5% after birth of one child and doubles

environment
-Low SEC
-mid spring conception
-maternal obesity
-maternal diabetes
-anticonvusent drugs
-maternal febrile illness
-nutrition

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Low folic acid deficiency contributes to spina bifida true or false

true

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When does the neural tube close

weeks 3-4

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Most severe both brain and spinal cord remain open

craniochischisis

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Anterior (rostral) neuropore defect

anencephaly

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posterior (caudal) neuropore defect

spina bifida

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What is the double hit theory

maldevelopment of the neural tube + exposure to amniotic fluid= damage to normal spinal cord tissues

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How is spina bifida diagnosed

Prenatal diagnosis at 18-20 weeks
Lemon shaped skull
banana sign- cerebellum is wrapped around the posterior brainstem due to downward traction of spinal cord

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What are the treatments for spina bifida

post natal closure
pre natal closure
fetal surgery
fetoscopy
stem cell graft

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will spina bifida present as an upper motor lesion or lower motor lesion

lower motor lesion
weakness
absent or diminished reflexes
absent babinski
flaccid bladder

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Arnold chiari type 2
hydrocephalus
motor, sensory, bowel and bladder impairments
majority 75% lumbar

myelomenigocele

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incomplete skin coverage= CSF leak
<10% of cases of cystica
neurologic signs may be normal

pocket w/ out nerves outside of spinal tract

meningocele

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Cystic cavity anterior to spinal cord
not very commin

myelocele

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50% pigmented nervus dimple dermal sinus

No neurological deficits
rarely associated with tethered cord
no neurologic or musculoskeletal changes

occulta

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Neurosurgical complications occur throughout childs life

sleep apnea
palpitations
diplopia
dysphagia

arnold chiari Type 2

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Neurosurgical complications occur throughout childs life

headaches
nausea
altered mental status
lethargy
personality change
vision
hoarse cry
swallow issues

hydrocephalus

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motor changes
sensory changes
reflex changes
neck pain

syringomyelia

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motor changes
sensory changes
reflex changes
back pain
progressive scoliosis
bowel or bladder changes
sexual changes

tethered cord- during growth spinal cord gets caught in scar tissue

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impaired fine motor coordination and ataxia
severe cases may lead to respiratory dysfunction and arrhythmia due to compression of brainstem

Arnold Chiari Type II

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What is the most frequent single cause of death in spina bifida

central respiratory dysfunction

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sleep disordered breathing affects up to ____% of people with spina bifida

81%

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usually presents at birth > symptomatic during first week

hydrocephalus

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what are the sign or gaze palsy associated with severe hydrocephalus?

sunset sign or upward gaze palsy

how is hydrocephalus treated?

with a shunt, taking fluid from one area putting it somewhere else >80% will require VP shunt with revisions infection is most frequent followed by obstruction

fluid filled central cavity symptoms change in motor and sensory function increased reflexes, pain often associated with hydrocephalus so treat the hydrocephalus first weakness in functions of the hands temperature or sensory changes

syringomyelia

weakness scoliosis pain orthopedic deformity urologic dysfunction change in function

tethered cord

When is tethered cord most common

during times of fast growth 0-4 and puberty

What are the 3 predictors of ambulation in kids with spina bifida

1. lower motor levels- good quad strength 2. no history of shunt- especially in context of needing good cognitive function 3. no history of hip or knee contracture surgery

Functional ability by level T6-T12 mobility equipment orthotic % of community ambulation

mobility: wheelchair for mobility no functional ambulation and standing equipment: wheelchair orthotic: THKAFO 0-33%

L1-L3 mobility equipment orthotic % of community ambulation

mobility: wheelchair for mobility limited household ambulation Equipment: wheelchair, walker, forearm crutches orthotic: HKAFO 31%

L3-L4 mobility equipment Orthotic % of community ambulation

mobility: household ambulation limited community ambulation wheelchair for long distances equipment: wheelchair, walker, forearm crutches orthotic: KAFO

L4-L5 mobility equipment orthotic % of community ambulation

mobility: household and community ambulation wheelchair for long distances equipment: wheelchair, forearm crutches Orthotic: AFO 38%

S1- S2 mobility equipment orthotic % of community ambulation

Community ambulation none SMO, FO all able to achieve community ambulation

Reasons for ambulation declin

obesity musculoskeletal deformity skin problems motivation energy expenditure neurologic decline spacticity

HKAFO

L1-L3

KAFO

L3-L4

AFO

L4-L5

SMAFO SMO

S1-S2

Reciprocating gait orthosis replace what

replace HKAFO patient needs active hip flexion development age 3 energy requirement= wheelchair

how can we assess strength and sensation in a child with spina bifida

utilize part of the isncsci

ISNCSCI levels

C5- elbow flexors C6- wrist extensors C7- elbow extensors C8-finger flexor T1- finger abductor L2- hip flexors L3- Knee extensors L4-ankle dorsiflexors L5- great toe extensors S1- ankle plantarflexors

T6-T12 muscle function

abdominals, paraspinals, quadratus lumborum

L1-L3 Muscle function

hip flexion, hip adduction, partial knee extension

L3-L4 muscle function

knee extension

What is a common gait pattern you might see with a patient who has an L4 myelomeningocele

trendelenburg

L4-L5 muscle function

hip abduction knee flexion ankle dorsiflexion toe extension

S1- S2 muscle function

hip abduction, knee flexion, ankle dorsiflexion, ankle inversion, toe extension

S2-S4 muscle function

sphincters

Common foot issue with T6-T12 L1-L3 L3-L4

Equinis

Common foot issue with L4-L5

calcaneovarus

Common foot issue with s1-s2 and s2-s4

cavus Claw toes

those who have a ____level lesion are at a higher risk of scoliosis

higher

true/false hip subluxation and dislocations are common

true

is a knee flexion or extension contracture more common

flexion

what can be done for equinus foot

achilles tendon lengthening

most children with SB have fecal incontinence due to poor rectal tone, absent reflex response and perianal sensory deficit

true

what is recommended for those who have difficulty with independence with a bowel program or maintaining continence

surgeries

thoracic lesions have a ____bladder sacral lesions have a _____bladder

thoracic- spastic sacral-flaccid

are UTIs common in SB

yes

what should be noted in terms of skin protection for SB

allergy to latex is common high risk for pressure injures early education for skin checks and pressure shifts

intellectual function correlates inversely with

level of spinal cord dysfunction

women with spina bifida are or arent able to get pregnant

are able to get pregnant

men with SB have ____ fertility related to poor ejaculation or erectile dysfunction

decreased

independent living is achieved by _____ of those with SB

30-60%

if desire to go to college suggest getting help from the

disability service office

most common death for older adults with SB

kidney issue