Peds - Spina Bifida Flashcards
Name the 5 environmental risk factors for spina bifida
- low socioeconomic class
- Midspring conception
- maternal obesity
- in utero exposure to anticonvulsant drugs (VPA, Carbamazepine)
- maternal febrile illness
Neurulation of the anterior and posterior neuropores occurs during _____ week after conception
post-neurulation phase occurs _____ which produces skin covered lesions
3rd - 4th week
4-7 weeks,
Normal neural tube closure starts in ____ week of gestation from mid-cervical level and proceeds in both cephalad and caudad directions
3rd
Defect of neural tube closure is thought to occur around day _____ and accounts for most lesions through mid-lumbar
26
Caudal regression of embryonic tail with rostral extension, resulting in fusion with the neural tube results in formation of the spinal cord by day ____
53 - lesions of lower lumbar/sacral area occur before this day.
Two markers associated with NTD
alpha-fetoprotein
acetylcholinesterase
AFP is reliable in 80% of open NTDs in 13-15 weeks
amniocentesis is done by week _____ for detecting elevated amnitiotic fluid AFP. Will not detect _____
16-18
closed NTDs without leakage of fetal CSF
Frequent signs of SB occulta
(5)
- pigmented nevus
angioma
hirsute patch
dimple
dermal sinus on overlying skin
three types of SP cystica
meningocele
myelomeningocele
myelocele
What does “spina bifida aperta” mean?
any NTD lesion which the deformity is open to the environment
SB occulta is rarely associated with ___- and ____ and therefore must be followed
sacral lipoma and tethered cord
most common location of lesion for SB occulta?
L5/S1
Name the contents of the cystic sac in the following:
1. meningocele
2. myelomeningocele
3. myelocele
- spinal fluid, meninges
- spinal fluide, meneges, spinal cord
- cystic cavity is in front of the anterior wall of the spinal cord causing protrusion of spinal cord
myelomeningocele is associated with _____ >90% of cases
arnold-chiari malformation complicated by hydrocephalusq
which is the most common type of SB cystica
myelomeningocele
Spina Bifida
Level of injury: T6-T12
Percentage:
Deficits:
T6-T12
40%
- complete leg paralysis (flaccid)
- Kyphosis
- scoliosis
-hip, knee flexion (frog leg)
- equinus foot
- bowel and bladder dysfunction
Spina bifida
Level of injury: L1/2/3
Percentage:
Deficits:
L1/2/3
25%
- early hip disloation
- hip flexion and adduction contractures
- scoliosis
- lordosis
- knee flexion contractures due to intrauterine positioning
- equinus foot
-bowel and bladder disfunction
spina bifida
level of injury: L4/5
percentage:
deficits:
L4/5
25%
- late hip dislocation
- scoliosis, lordosis
- calcaneovarus or calcaneus foot (ankles dorsiflexed)
- knee extension contractures
- hip, knee flexion contractures
- bowel and bladder dysfunction
Spina bifida
level of injury: S1-2
percentage
deficits
S1/2
10% (combined with S3/4)
- bowel and bladder dysfunction
- pes cavus foot and clawing of toes due to intrinsic muscle denervation
Spina bifida
level of injury S3/4
percentage
deficits
S3/4
10% (combined with S1/2)
- bowel and bladder dysfunction
- cavus foot and clawing of toes due to intrinsic muscle denervation
signs and symptoms of a tethered cord
- increased weakness (55%)
- scoliosis (51%)
- pain (32%)
- orthopedic deformity (11%) and urologic dysfunction (6%)
What is diastematomyelia or sagittal cleavage of the spinal cord?
associated with?
divisions. usually associated with bony spur
spina bifida
pt presents with deterioration of neurologic function, pain and temperature, sensory deficits, loss of motor function especially in upper extremities, increased spasticity and hyperreflexia, and pain.
syringomyelia - fluid filled central cavity in the spinal cord parenchyma lined with glial cells
most frequent single cause of death in myelodysplasia?
central respiratory dysfunctino
3 types of visual impairments in SB
strabismus
lateral rectus palsy
nystagmus
What is “cocktail party personality?”
represents spina bifida children with good verbal ability that creates the impression of higher intellectual functioning than is found on formal testing.
Verbal tasks are»_space; visual motor activityes or math/written/visual perception abilities
when should you order a renal ultrasound to define spina bifida urinary anatomy?
2 weeks
intermittent cath should be initiated in SB when residual volume is _____
20cc
pharmacologic therapy of neurogenic bladder in SB includes _____ which decrease detrusor contractions and enlarge bladder storage capacity, and _____ that increase outflow resistance
anticholinergics, alpha adrenergic agents
______ occurs in 59% of children with spina bifida and 55% in children who face multiple surgeries for other diagnoses
latex sensitivity
There is a 500 x increased risk of anaphylaxis in the operating room for children with spina bifida
Referral to preschool programs at age ______ is mandated for children with disabilities
3 yoa
employment rate of kids with SB is _____
25-50%
Level of Spina bifida: T12
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
Totally paralyzed lower limbs
Standing brace, WC
WC
WC, no ambulation
Level of Spina bifida: L1-2
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
Hip flexor muscles
Crutches, braces, WC
WC, household ambulation
WC, nonfunctional ambulation
Level of Spina bifida L3-4
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
Quadriceps muscles
crutches, braces, household ambulation, WC
Crutches, household ambulation, WC
50% wheelchair, household ambulation with crutches
Level of Spina bifida L5
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
medial hamstrings, anterior tibial muscles
crutches, braces, community ambulation
crutches, community ambulation
community ambulation with crutches
Level of Spina bifida S1
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
lateral hamstring, peroneal muscles
community ambulation
community ambulation
community amculation 50% crutch or cane
Level of Spina bifida S2-3
Critical motor function present:
Mobility school age:
Range: adults
Activity: adolescent:
mild loss of intrinsic foot muscles possible
normal
normal
limited endurance because of late foot deformities
