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Cucc - PEDs > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Name the 5 environmental risk factors for spina bifida

A
  1. low socioeconomic class
  2. Midspring conception
  3. maternal obesity
  4. in utero exposure to anticonvulsant drugs (VPA, Carbamazepine)
  5. maternal febrile illness
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2
Q

Neurulation of the anterior and posterior neuropores occurs during _____ week after conception

post-neurulation phase occurs _____ which produces skin covered lesions

A

3rd - 4th week

4-7 weeks,

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3
Q

Normal neural tube closure starts in ____ week of gestation from mid-cervical level and proceeds in both cephalad and caudad directions

A

3rd

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4
Q

Defect of neural tube closure is thought to occur around day _____ and accounts for most lesions through mid-lumbar

A

26

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5
Q

Caudal regression of embryonic tail with rostral extension, resulting in fusion with the neural tube results in formation of the spinal cord by day ____

A

53 - lesions of lower lumbar/sacral area occur before this day.

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6
Q

Two markers associated with NTD

A

alpha-fetoprotein
acetylcholinesterase

AFP is reliable in 80% of open NTDs in 13-15 weeks

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7
Q

amniocentesis is done by week _____ for detecting elevated amnitiotic fluid AFP. Will not detect _____

A

16-18

closed NTDs without leakage of fetal CSF

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8
Q

Frequent signs of SB occulta

5

A 
1. pigmented nevus
angioma
hirsute patch
dimple
dermal sinus on overlying skin
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9
Q

three types of SP cystica

A

meningocele
myelomeningocele
myelocele

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10
Q

What does “spina bifida aperta” mean?

A

any NTD lesion which the deformity is open to the environment

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11
Q

SB occulta is rarely associated with ___- and ____ and therefore must be followed

A

sacral lipoma and tethered cord

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12
Q

most common location of lesion for SB occulta?

A

L5/S1

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13
Q

Name the contents of the cystic sac in the following:

  1. meningocele
  2. myelomeningocele
  3. myelocele
A
  1. spinal fluid, meninges
  2. spinal fluide, meneges, spinal cord
  3. cystic cavity is in front of the anterior wall of the spinal cord causing protrusion of spinal cord
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14
Q

myelomeningocele is associated with _____ >90% of cases

A

arnold-chiari malformation complicated by hydrocephalusq

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15
Q

which is the most common type of SB cystica

A

myelomeningocele

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16
Q

Spina Bifida
Level of injury: T6-T12
Percentage:
Deficits:

A 
T6-T12
40%
- complete leg paralysis (flaccid) 
- Kyphosis 
- scoliosis
-hip, knee flexion (frog leg) 
- equinus foot
- bowel and bladder dysfunction
17
Q

Spina bifida
Level of injury: L1/2/3
Percentage:
Deficits:

A 
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
18
Q

spina bifida
level of injury: L4/5
percentage:
deficits:

A 
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
19
Q

Spina bifida
level of injury: S1-2
percentage
deficits

A

S1/2
10% (combined with S3/4)
- bowel and bladder dysfunction
- pes cavus foot and clawing of toes due to intrinsic muscle denervation

20
Q

Spina bifida
level of injury S3/4
percentage
deficits

A

S3/4
10% (combined with S1/2)
- bowel and bladder dysfunction
- cavus foot and clawing of toes due to intrinsic muscle denervation

21
Q

signs and symptoms of a tethered cord

A
  1. increased weakness (55%)
  2. scoliosis (51%)
  3. pain (32%)
  4. orthopedic deformity (11%) and urologic dysfunction (6%)
22
Q

What is diastematomyelia or sagittal cleavage of the spinal cord?
associated with?

A

divisions. usually associated with bony spur

spina bifida

23
Q

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.

A

syringomyelia - fluid filled central cavity in the spinal cord parenchyma lined with glial cells

24
Q

most frequent single cause of death in myelodysplasia?

A

central respiratory dysfunctino

25
Q

3 types of visual impairments in SB

A

strabismus
lateral rectus palsy
nystagmus

26
Q

What is “cocktail party personality?”

A

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&raquo_space; visual motor activityes or math/written/visual perception abilities

27
Q

when should you order a renal ultrasound to define spina bifida urinary anatomy?

A

2 weeks

28
Q

intermittent cath should be initiated in SB when residual volume is _____

A

20cc

29
Q

pharmacologic therapy of neurogenic bladder in SB includes _____ which decrease detrusor contractions and enlarge bladder storage capacity, and _____ that increase outflow resistance

A

anticholinergics, alpha adrenergic agents

30
Q

______ occurs in 59% of children with spina bifida and 55% in children who face multiple surgeries for other diagnoses

A

latex sensitivity

There is a 500 x increased risk of anaphylaxis in the operating room for children with spina bifida

31
Q

Referral to preschool programs at age ______ is mandated for children with disabilities

A

3 yoa

32
Q

employment rate of kids with SB is _____

A

25-50%

33
Q 
Level of Spina bifida: T12
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

Totally paralyzed lower limbs
Standing brace, WC
WC
WC, no ambulation

34
Q 
Level of Spina bifida: L1-2
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

Hip flexor muscles
Crutches, braces, WC
WC, household ambulation
WC, nonfunctional ambulation

35
Q 
Level of Spina bifida L3-4
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

Quadriceps muscles
crutches, braces, household ambulation, WC
Crutches, household ambulation, WC
50% wheelchair, household ambulation with crutches

36
Q 
Level of Spina bifida L5
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

medial hamstrings, anterior tibial muscles
crutches, braces, community ambulation
crutches, community ambulation
community ambulation with crutches

37
Q 
Level of Spina bifida S1
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

lateral hamstring, peroneal muscles
community ambulation
community ambulation
community amculation 50% crutch or cane

38
Q 
Level of Spina bifida S2-3
Critical motor function present: 
Mobility school age: 
Range: adults
Activity: adolescent:
A

mild loss of intrinsic foot muscles possible
normal
normal
limited endurance because of late foot deformities

Cucc - PEDs (11 decks)

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