Spina Bifida Flashcards
Spina Bifida is the most common type of ________ defect
Neural tube
_____ most common childhood abnormality/ disease causing disability after CP
2nd most common
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
Low folic acid deficiency contributes to spina bifida true or false
true
When does the neural tube close
weeks 3-4
Most severe both brain and spinal cord remain open
craniochischisis
Anterior (rostral) neuropore defect
anencephaly
posterior (caudal) neuropore defect
spina bifida
What is the double hit theory
maldevelopment of the neural tube + exposure to amniotic fluid= damage to normal spinal cord tissues
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
What are the treatments for spina bifida
post natal closure
pre natal closure
fetal surgery
fetoscopy
stem cell graft
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
Arnold chiari type 2
hydrocephalus
motor, sensory, bowel and bladder impairments
majority 75% lumbar
myelomenigocele
incomplete skin coverage= CSF leak
<10% of cases of cystica
neurologic signs may be normal
pocket w/ out nerves outside of spinal tract
meningocele
Cystic cavity anterior to spinal cord
not very commin
myelocele
50% pigmented nervus dimple dermal sinus
No neurological deficits
rarely associated with tethered cord
no neurologic or musculoskeletal changes
occulta
Neurosurgical complications occur throughout childs life
sleep apnea
palpitations
diplopia
dysphagia
arnold chiari Type 2
Neurosurgical complications occur throughout childs life
headaches
nausea
altered mental status
lethargy
personality change
vision
hoarse cry
swallow issues
hydrocephalus
motor changes
sensory changes
reflex changes
neck pain
syringomyelia
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
impaired fine motor coordination and ataxia
severe cases may lead to respiratory dysfunction and arrhythmia due to compression of brainstem
Arnold Chiari Type II
What is the most frequent single cause of death in spina bifida
central respiratory dysfunction
sleep disordered breathing affects up to ____% of people with spina bifida
81%
usually presents at birth > symptomatic during first week
hydrocephalus
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
- lower motor levels- good quad strength
- no history of shunt- especially in context of needing good cognitive function
- 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
