Spina Bifida Flashcards
what is the 2nd most common birth defect
spina bifida (2nd to down syndrome)
- also second to CP in causing locomotor dysfunction
what are some factors that may contribute to a baby being born with spina bifida
- genetics
- environment
- low levels of maternal folic acid
- maternal use of valproic acid/antidepressants
- maternal hyperthermia (hot tubs)
- maternal obesity
T or F: there are now high survival rates for spina bifida
T:>90% with aggressive treatment
what is the first approach to treating spina bifida
early back closure
other than early back closure, what are 3 other things that have led to higher survival rates with spina bifida
- use of antibiotics
- ventricular shunts
- intermittent catheterization to control UTIs and renal deterioration
spina bifida
a spinal defect diagnosed at or before birth by the presence of an external sac on the infants back. the sac contains meninges and spinal cord tissue protruding through a dorsal defect in the vertebrae
where does spina bifida most commonly occur
lumbar region
clinical signs of spina bifida
- absence of motor and sensory bilaterally below level of defect
- loss of neural control of B/B
are functional deficits with spina bifida permanent
yes, but they may be partial or complete
spina bifida occulta
- non-fusion of the halves of the vertebral arches
- no disturbance of underlying neural tissue
- may be distinguished by midline tuft of hair
- most mild form
4 types of spina bifida
- occulta
- meningocele
- myelomeningocele
- lipomeningocele
meningocele
- sac contains only meninges and CSF
- typically no motor or sensory deficits
myelomeningocele
- sac contains spinal cord and nerves
- may or may not have meninges
- most severe form
lipomeningocele
- superficial fatty mass in low lumbar or sacral region
- high incidence of B/B dysfunction
- less severe
these 2 types of spina bifida commonly present with tethered cord
occulta and lipomeningocele
the neural plate differentiates into the…
neural tube and neural crest
the neural ______ becomes the PNS while the neural _______ becomes the CNS
crest
tube
why does spina bifida occur
the caudal end of the neural tube fails to close during ~26th day of gestation
what happens if the cranial end of the neural tube does not close during gestation
anencephaly
abnormally high levels of this protein in the amniotic fluid may indicate a neural tube defect
alpha-fetoprotein (AFP)
what can be used to identify cranial signs of spina bifida
ultrasound
*early diagnosis helps with timely back closure surgery
if doctors know a baby has spina bifida how will they likely be delivered and why
c-section because it has a protective effect on sensitive neural tissue, reduces trauma, and lowers infection risk
T or F: back closure surgery for spina bifida can be completed in utero
T: but there are complications so sometimes it is managed right after birth
what are the 2 most common comorbidities with spina bifida? what are some other comorbidities?
- hydrocephalus and chiari malformation
- tethered cord, scoliosis, foot deformities
hydrocephalus
accumulation of CSF in the cranial vault
what can cause hydrocephalus? how is it treated?
- caused by overproduction of CSF, failure to absorb CSF, or obstruction of flow
- treated with a VP or VA shunt
what is the primary cause of hydrocephalus in most children with Spina Bifida
chiari malformation
chiari malformation
- deformity of the cerebellum, medulla, and cervical spina cord
- posterior cerebellum herniates down through foramen magnum
T or F: 90% of infants who have surgery for back closure will develop hydrocephalus
T
what are symptoms of hydrocephalus
- sunsetting eyes (downward deviation)
- separation of cranial sutures
- bulging anterior fontanelle
what are sunsetting eyes in hydrocephalus due to
pressure on cranial nerves
a VA shunt moves CSF from lateral ventrical to ______ while a VP shunt moves it to _________
right atrium
peritoneum
how long do you need to wait after shunt placement to initiate PT
24-48 hours
*handle with extreme caution during this time - no major changes in position
what are some signs of shunt malfunction
- vomiting
- irritability
- headaches
- bulging fontanelle
- lethargy
- edema/redness along shunt tract
- seizures
- memory/personality changes
what is the most common types of chiari malformation with spina bifida
2
what are some symptoms of chiari 2 malformation
- difficulty swallowing/breathing
- ataxia
- UE weakness
- abnormal eye movement
- facial palsy
hydromyelia - what is it and what does it cause
- CSF migrates into spinal cord and collects in pockets leading to pressure and necrosis of neural tissue
- causes progressive UE weakness and hypertonus
tethered cord
adhesions anchor spinal cord to site of back lesion/closure
- as child grows, the cord is stretched
what are s/s of tethered cord
- degeneration of muscle function
- scoliosis
- hypertonia at one or more LE sites
- gait changes
- B/B changes
T or F: most children with spina bifida and scoliosis will not need surgery
F: surgical fusion is inevitable for most
what causes scoliosis in spina bifida
imbalances of muscles
what are factors that influence cognition in children with spina bifida
1 - delayed treatment of hydrocephalus
2 - cerebral infarction
3 - presence of other CNS abnormalities
T or F: there is a high degree of attention deficit/distractibility in some children with spina bifida
T
deficits in children with thoracic level spina bifida
- no LE movement
- weak lower trunk
- difficulty with unsupported sitting
- decreased respiratory function
what kind of equipment may a child with thoracic level spina bifida use
- A frame for standing
- Orlau swivel walker
- WC for primary mobility
do most people with thoracic level spina bifida live independently
no, they are in supported living situations and rarely employed
children with high lumbar function have some contraction (although weak) of what LE muscles
hip flexors and adductors
what kind of equipment may children with high lumbar spina bifida use
- KAFOs/RGOs with AD for household ambulation
- WC for primary mobility
T or F: many people with high lumbar spina bifida live independently by adulthood
T: about 50%
RGO
- reciprocating gait orthosis
- trunk lean causes forward translation of leg
what LE muscles do children with L3 spina bifida have access to
- strong hip flexion/adduction
- weak hip rotation
- antigravity knee extension
what kind of equipment do children with L3 spina bifida use
- KAFOs w/ forearm crutches for household and short community distances
- WC for longer distances
what kind of equipment do children with L4 spina bifida typically use
- AFOs and forearm crutches in home and community
- WC for longer distances
T or F: calcaneal foot deformities are common in L4/5 spina bifida
T
What LE muscles do children with L5 spina bifida have access to
- hip flexors, adductors, rotators
- stronger DF
- weak hip extension
- possible weak abduction, PF, and toe movement
what type of equipment do children with L5 spina bifida typically use
- might can ambulate without KAFO/AFO but have to correct foot deformities (foot orthotic)
- AD for community distance
T or F: children with S1-3 can ambulate independently without an AD
T: may still use a foot orthotic to improve alignment but foot deformities are less common
how is motor level determined in a child with spina bifida
it is the lowest level at which the child is able to perform antigravity movement through available range
*make sure they are not compensating at other joints and that the movement is active rather than reflexive
reflex movements are most often observed at what joint in spina bifida
flexion of the knee
manual muscle test grading for spina bifida
X = strong movement
O = absent response
T = trace movement
R = reflex movement
what are common joint ROM limitations in spina bifida
- hip flexor tightness (L2-4 level)
- adductor tightness
- DF contracture
how can you help increase ROM in children with spina bifida
- slow.gradual stretching with ROM exercises
- serial casting may be needed
T or F: up to 85% of children with spina bifida have low tone, with minimal to moderate developmental delay
T
what are the most common developmental delays in children with spina bifida
- head/trunk control
- abnormal acquisition of righting and equilibrium responses
what do infants with spina bifida tent to use to compensate for lack of head/trunk control
UE
T or F: brief periods of supported standing several times per day can improve proprioception, sensory input, and acetabular development
T
the goal is generally safe and functional ambulation by _____ years of age
5-6, in preparation for mobility in school
T or F: you should change multiple things at once to an orthosis or AD
F: only one thing at a time
surgery and orthotics to prepare the child for upright positioning should be coordinated as close to _____- to ______ months of age if possible
12 to 15, typical development age
children with thoracic level spina bifida are at risk of what kind of deformity
frog-legged (abduction, ER, hip/knee flexion, PF)
how can you help prevent frog-legged deformity
- prone positioning
- daily ROM exercise
- gentle wrapping of legs in ext and adduction
- night time or naptime orthosis
thoracic level and high lumbar spina bifida may benefit from what kind of orthosis
total contact orthosis
total contact orthosis
- thoracolumbar section stabilizes pelvis and lumbar spine
- lower legs/feet hold knees in extension and ankles in neutral or plantigrade
T or F: hip subluxations and dislocations are common in high lumbar spina bifida
T
what is the single most influential factor leading to the deterioration of ambulation skills in low lumbar spina bifida
moderate to sever hip flexion contractures
what may you notice in the gait pattern of a child with a lumbosacral level lesion
- exaggerated pelvic movement
- increased stance phase hip abduction, knee flexion, and knee valgus
- increased ankle DF
what are some complications of spina bifida with aging
- osteomyelitis
- scoliosis
- amputations (skin breakdown)
- decreased ambulation
- memory/comprehension deficits
- obesity
- incontinence and recurrent UTIs
- joint pain
