Spina Bifida Flashcards

1
Q

Spina Bifida is the most common type of ________ defect

A

Neural tube

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2
Q

_____ most common childhood abnormality/ disease causing disability after CP

A

2nd most common

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3
Q

What are Risk factors for spina bifida

A

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

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4
Q

Low folic acid deficiency contributes to spina bifida true or false

A

true

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5
Q

When does the neural tube close

A

weeks 3-4

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6
Q

Most severe both brain and spinal cord remain open

A

craniochischisis

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7
Q

Anterior (rostral) neuropore defect

A

anencephaly

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8
Q

posterior (caudal) neuropore defect

A

spina bifida

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9
Q

What is the double hit theory

A

maldevelopment of the neural tube + exposure to amniotic fluid= damage to normal spinal cord tissues

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10
Q

How is spina bifida diagnosed

A

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

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11
Q

What are the treatments for spina bifida

A

post natal closure
pre natal closure
fetal surgery
fetoscopy
stem cell graft

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12
Q

will spina bifida present as an upper motor lesion or lower motor lesion

A

lower motor lesion
weakness
absent or diminished reflexes
absent babinski
flaccid bladder

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13
Q

Arnold chiari type 2
hydrocephalus
motor, sensory, bowel and bladder impairments
majority 75% lumbar

A

myelomenigocele

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14
Q

incomplete skin coverage= CSF leak
<10% of cases of cystica
neurologic signs may be normal

pocket w/ out nerves outside of spinal tract

A

meningocele

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15
Q

Cystic cavity anterior to spinal cord
not very commin

A

myelocele

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16
Q

50% pigmented nervus dimple dermal sinus

No neurological deficits
rarely associated with tethered cord
no neurologic or musculoskeletal changes

A

occulta

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17
Q

Neurosurgical complications occur throughout childs life

sleep apnea
palpitations
diplopia
dysphagia

A

arnold chiari Type 2

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18
Q

Neurosurgical complications occur throughout childs life

headaches
nausea
altered mental status
lethargy
personality change
vision
hoarse cry
swallow issues

A

hydrocephalus

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19
Q

motor changes
sensory changes
reflex changes
neck pain

A

syringomyelia

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20
Q

motor changes
sensory changes
reflex changes
back pain
progressive scoliosis
bowel or bladder changes
sexual changes

A

tethered cord- during growth spinal cord gets caught in scar tissue

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21
Q

impaired fine motor coordination and ataxia
severe cases may lead to respiratory dysfunction and arrhythmia due to compression of brainstem

A

Arnold Chiari Type II

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22
Q

What is the most frequent single cause of death in spina bifida

A

central respiratory dysfunction

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23
Q

sleep disordered breathing affects up to ____% of people with spina bifida

A

81%

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24
Q

usually presents at birth > symptomatic during first week

A

hydrocephalus

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25
Q

what are the sign or gaze palsy associated with severe hydrocephalus?

A

sunset sign or upward gaze palsy

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26
Q

how is hydrocephalus treated?

A

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

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27
Q

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

A

syringomyelia

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28
Q

weakness
scoliosis
pain
orthopedic deformity
urologic dysfunction
change in function

A

tethered cord

29
Q

When is tethered cord most common

A

during times of fast growth 0-4 and puberty

30
Q

What are the 3 predictors of ambulation in kids with spina bifida

A
  1. lower motor levels- good quad strength
  2. no history of shunt- especially in context of needing good cognitive function
  3. no history of hip or knee contracture surgery
31
Q

Functional ability by level
T6-T12

mobility
equipment
orthotic
% of community ambulation

A

mobility: wheelchair for mobility
no functional ambulation and standing

equipment: wheelchair
orthotic: THKAFO
0-33%

32
Q

L1-L3

mobility
equipment
orthotic
% of community ambulation

A

mobility: wheelchair for mobility
limited household ambulation

Equipment: wheelchair, walker, forearm crutches
orthotic: HKAFO
31%

33
Q

L3-L4

mobility
equipment
Orthotic
% of community ambulation

A

mobility: household ambulation
limited community ambulation
wheelchair for long distances

equipment: wheelchair, walker, forearm crutches
orthotic: KAFO

34
Q

L4-L5
mobility
equipment
orthotic
% of community ambulation

A

mobility: household and community ambulation
wheelchair for long distances

equipment: wheelchair, forearm crutches
Orthotic: AFO
38%

35
Q

S1- S2
mobility
equipment
orthotic
% of community ambulation

A

Community ambulation
none
SMO, FO
all able to achieve community ambulation

36
Q

Reasons for ambulation declin

A

obesity
musculoskeletal deformity
skin problems
motivation
energy expenditure
neurologic decline
spacticity

37
Q

HKAFO

A

L1-L3

38
Q

KAFO

A

L3-L4

39
Q

AFO

A

L4-L5

40
Q

SMAFO SMO

A

S1-S2

41
Q

Reciprocating gait orthosis replace what

A

replace HKAFO
patient needs active hip flexion
development age 3
energy requirement= wheelchair

42
Q

how can we assess strength and sensation in a child with spina bifida

A

utilize part of the isncsci

43
Q

ISNCSCI levels

A

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

44
Q

T6-T12
muscle function

A

abdominals, paraspinals, quadratus lumborum

45
Q

L1-L3
Muscle function

A

hip flexion, hip adduction, partial knee extension

46
Q

L3-L4
muscle function

A

knee extension

47
Q

What is a common gait pattern you might see with a patient who has an L4 myelomeningocele

A

trendelenburg

48
Q

L4-L5
muscle function

A

hip abduction
knee flexion
ankle dorsiflexion
toe extension

49
Q

S1- S2
muscle function

A

hip abduction, knee flexion, ankle dorsiflexion, ankle inversion, toe extension

50
Q

S2-S4
muscle function

A

sphincters

51
Q

Common foot issue with
T6-T12
L1-L3
L3-L4

A

Equinis

52
Q

Common foot issue with L4-L5

A

calcaneovarus

53
Q

Common foot issue with s1-s2 and s2-s4

A

cavus
Claw toes

54
Q

those who have a ____level lesion are at a higher risk of scoliosis

A

higher

55
Q

true/false hip subluxation and dislocations are common

A

true

56
Q

is a knee flexion or extension contracture more common

A

flexion

57
Q

what can be done for equinus foot

A

achilles tendon lengthening

58
Q

most children with SB have fecal incontinence due to poor rectal tone, absent reflex response and perianal sensory deficit

A

true

59
Q

what is recommended for those who have difficulty with independence with a bowel program or maintaining continence

A

surgeries

60
Q

thoracic lesions have a ____bladder
sacral lesions have a _____bladder

A

thoracic- spastic
sacral-flaccid

61
Q

are UTIs common in SB

A

yes

62
Q

what should be noted in terms of skin protection for SB

A

allergy to latex is common
high risk for pressure injures
early education for skin checks and pressure shifts

63
Q

intellectual function correlates inversely with

A

level of spinal cord dysfunction

64
Q

women with spina bifida are or arent able to get pregnant

A

are able to get pregnant

65
Q

men with SB have ____ fertility related to poor ejaculation or erectile dysfunction

A

decreased

66
Q

independent living is achieved by _____ of those with SB

A

30-60%

67
Q

if desire to go to college suggest getting help from the

A

disability service office

68
Q

most common death for older adults with SB

A

kidney issue