Peds - Spina Bifida

Question 1

Name the 5 environmental risk factors for spina bifida

Answer 1

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

Question 2

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

post-neurulation phase occurs _____ which produces skin covered lesions

Answer 2

3rd - 4th week

4-7 weeks,

Question 3

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

Answer 3

3rd

Question 4

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

Answer 4

26

Question 5

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

Answer 5

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

Question 6

Two markers associated with NTD

Answer 6

alpha-fetoprotein
acetylcholinesterase

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

Question 7

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

Answer 7

16-18
closed NTDs without leakage of fetal CSF

Question 8

Frequent signs of SB occulta
(5)

Answer 8

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

Question 9

three types of SP cystica

Answer 9

meningocele
myelomeningocele
myelocele

Question 10

What does “spina bifida aperta” mean?

Answer 10

any NTD lesion which the deformity is open to the environment

Question 11

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

Answer 11

sacral lipoma and tethered cord

Question 12

most common location of lesion for SB occulta?

Answer 12

L5/S1

Question 13

Name the contents of the cystic sac in the following:
1. meningocele
2. myelomeningocele
3. myelocele

Answer 13

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

Question 14

myelomeningocele is associated with _____ >90% of cases

Answer 14

arnold-chiari malformation complicated by hydrocephalusq

Question 15

which is the most common type of SB cystica

Answer 15

myelomeningocele

Question 16

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

Answer 16

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

Question 17

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

Answer 17

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

Question 18

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

Answer 18

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

Question 19

Spina bifida
level of injury: S1-2
percentage
deficits

Answer 19

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

Question 20

Spina bifida
level of injury S3/4
percentage
deficits

Answer 20

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

Question 21

signs and symptoms of a tethered cord

Answer 21

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

Question 22

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

Answer 22

divisions. usually associated with bony spur

spina bifida

Question 23

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.

Answer 23

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

Question 24

most frequent single cause of death in myelodysplasia?

Answer 24

central respiratory dysfunctino

Question 25

3 types of visual impairments in SB

Answer 25

strabismus lateral rectus palsy nystagmus

Question 26

What is "cocktail party personality?"

Answer 26

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 >> visual motor activityes or math/written/visual perception abilities

Question 27

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

Answer 27

2 weeks

Question 28

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

Answer 28

20cc

Question 29

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

Answer 29

anticholinergics, alpha adrenergic agents

Question 30

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

Answer 30

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

Question 31

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

Answer 31

3 yoa

Question 32

employment rate of kids with SB is _____

Answer 32

25-50%

Question 33

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

Answer 33

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

Question 34

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

Answer 34

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

Question 35

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

Answer 35

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

Question 36

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

Answer 36

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

Question 37

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

Answer 37

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

Question 38

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

Answer 38

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