Spina Bifida Flashcards

1
Q

What is myelodysplasia?

A

An abnormality in development of the spinal cord

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2
Q

what is anencephaly?

A

Lack of closure in region of head

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3
Q

What is spina bifida?

A

Lack of closure below the head

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4
Q

(true/false) Most infants with anencephaly live after birth

A

false (die within a few days of birth)

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5
Q

definition: Congenital malformation of spinal cord

A

neural tube defect

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6
Q

(true/false) spina bifida differs by racial/ethnic group

A

true –> more prevalent in hispanics

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7
Q

The prevalence of spina bifida has (decreased/increased)

A

decreased

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8
Q

What is spina bifida aperta?

A

spina bifida with visible or open lesions

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9
Q

What is spina bifida occulta?

A

Spina bifida with hidden lesions

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10
Q

What is a Myelomeningocele?

A

A form of spina bifida aperta that is characterized by a Sac containing the spina cord that protrudes dorsally and is not covered by skin.

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11
Q

What form of spina bifida aperta is associated with spinal nerve paralysis?

A

Myelomeningocele

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12
Q

What is meningocele?

A

A form of spina bifida aperta that is characterized by a sac with few to no nerves and is covered by skin.

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13
Q

What form of spina bifida aperta does not have paralysis?

A

meningocele

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14
Q

What is found in the sac when meningocele is present?

A

membranes and CSF –> minimal/no nerves

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15
Q

(true/false) Spina bifida occulta has fusion of the vertebral arch.

A

FALSE

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16
Q

(true/false) Spina bifida occulta has no tissue disturbance

A

true

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17
Q

What is spina bifida occulta normally associated with?

A

mild urinary tract disorders

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18
Q

Where is spina bifida occulta commonly found?

A

lumbosacral region

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19
Q

What characteristic of spina bifida occulta is indicative of suspicion for it?

A

tuft of hair or dimple present

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20
Q

What is a lipoma?

A

A visible form of spina bifida occulta that is a subcutaneous fat mass that varies in size.

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21
Q

(true/false) Lipoma may/may not involve paralysis

A

true

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22
Q

definition: Band separates spinal cord into hemicords

A

Diastematomyelia

–> Cord tethering may result in progressive weakness

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23
Q

(true/false) Diastematomyelia may be associated with paralysis

A

true

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24
Q

What is a Myelocystocele?

A

Cysts of the spinal cord that occurs in the lumbosacral region and is skin covered.

–> may or may not be associated with nerve impairment

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25
Q

definition: Neural tube fails to close between 3rd and 4th weeks gestation

A

Abnormal neurulation

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26
Q

definition: Cells distal to S2 typically adhere together to form canals which attach to the spinal cord, this process occurs abnormally

A

Abnormal canalization

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27
Q

Who has a higher risk of having a baby with NTDs? Why?

A

a. Significantly higher risk in women who did not graduate from high school and lived in low-SES neighborhoods

b. Women in higher social groups are more likely to use folic acid

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28
Q

NTDs are common in mothers < ___ y/o and > ___y/o

A

< 19
> 40

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29
Q

(true/false) There is an increased risk of NTDs with increased total caffeine consumption during the year before pregnancy

A

true

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30
Q

Antibacterial medication (preconceptional and first trimester) and use of SSRI’s increased risk of ______.

A

anencephaly

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31
Q

What is the cause of abnormal canalization?

A

UNKNOWN

32
Q

What is the cause of abnormal neurulation?

A
  • genetics
  • teratogens
  • nutritional deficiencies
33
Q

When should mothers begin folic acid?

A

at least 3 months before conception

34
Q

Folic acid use before conception decreases risk of an open defect by ___%.

A

70%

35
Q

When is a Maternal serum alpha-fetoprotein screening positive?

A

Positive only with OPEN neural lesion

36
Q

What is ultrasound more accurate in detecting?

A

More accurate in detecting cranial malformations associated with spina bifida, than in detecting abnormalities in the spine (lemon and banana sign)

37
Q

(true/false) Those with NTDs have an associated chromosome error (Trisomy 13 or 18)

A

true (10%)

38
Q

definition: the outline of the cerebellum looks like a banana, probably because of the herniation at the spinal defect - the outline of the skull resembles a lemon

A

banana sign of spina bifida

39
Q

Why is a C-section commonly used with NTDs?

A

Cesarean section will spare exposed nerves and prevent infection by passing through the vaginal canal

Back closure can be done more rapidly

40
Q

What is chiari malformation the deformity of?

A

cerebellum, medulla, cervical spine

41
Q

What is chiari malformation?

A

Posterior cerebellum herniates downward through foramen magnum

–> May be due to small posterior fossae

42
Q

What is the cause of hydrocephalus in most children?

A

chiari malformation

43
Q

(true/false) paralysis may occur later in life

A

true

44
Q

What can cause abnormal tissue growth?

A

pressure on the nerves

45
Q

What causes the lack of tissue growth around the spinal cord?

A

ischemia and tethering of the cord

46
Q

What is the test described below?

Abduction of flexed hips (may feel clunk of relocation)

A

ortolani’s test

47
Q

What test is described below?

Pressure directed posterior on a flexed, adducted hip

A

barlow’s test

–> Feel femoral head dislocated over posterior rim of acetabulum

48
Q

What is osteoporosis caused by? What is the result of this?

A

a. hypotonia/paralysis and decreased WB

b. osteoporotic Fx

49
Q

(true/false) Often DO NOT correlate with motor levels

A

true

50
Q

What is hydrocephalus?

A

Excessive accumulation of CSF in the ventricles of the brain

51
Q

___% of children with spina bifida are born with hydrocephalus

A

25%

52
Q

An additional ___% of spina bifida cases develop hydrocephalus after surgical closure of back lesion.

A

60%

53
Q

(true/false) 80-90% of hydrocephalus cases will require a shunt

A

true

54
Q

What can prolonged shunt dysfunction lead to?

A

Functional and Cognitive decline

55
Q

definition: appear articulate and verbose although use a lot of jargon and cliché’s

A

cocktail party personality

56
Q

What are the 3 possible causes of UE dyscoordination?

–> Use UE’s for support rather than for exploration of objects

A
  1. Cerebellar ataxia related to Arnold-Chiari Type II malformation
  2. Motor cortex or pyramidal tract damage secondary to hydrocephalus
  3. Motor learning deficits due to delayed development of hand
57
Q

Cranial nerve palsies are a result of what?

A
  1. Arnold-Chiari malformation
  2. Hydrocephalus or dysplasia of the brainstem
58
Q

The majority of spina bifida cases have (UMN/LMN) presentation

A

LMN

–> 2/3 of cases had SOME UMN signs

59
Q

what are causes of progressive neuro dysfunction in those with spina bifida?

A
  • Shunt obstruction
  • Hydromyelia
  • Growth of a lipoma at the site of repair
  • Subarachnoid cysts of the cord
  • Spinal cord tethering
60
Q

(true/false) 1/3 of kids with myelomeningocele will require surgery for tethered cord

A

True

61
Q

definition: refers to an abnormal widening of the central canal of the spinal cord that creates a cavity in which cerebrospinal fluid (commonly known as spinal fluid) can accumulate and increase pressure on the spinal cord resulting in damage to the nerve cells and their connections

A

hydromyelia

62
Q

Hydromyelia occurs in __-___% of children and adolescents with spina bifida

A

10-30%

63
Q

___ medications can accentuate cognitive deficits or dyscoordination

A

anticonvulsant

64
Q

(true/false) more than 5% of spina bifida cases develop voluntary control of b/b.

A

FALSE (less than 5%)

65
Q

definition: . procedure that allows the patient to do a total washout of their colon by flushing out stool starting in the colon downward. This produces a bowel movement under the patient’s control!

A

ACE procedure

(antegrade colonic enema)

66
Q

_____ ulcers occur in 85-95% of children by time reach young adulthood

A

decubitus

67
Q

(true/false) obesity is common in children with spina bifida

A

true

68
Q

Self-catheterization can begin at age ___.

A

age 5

69
Q

How can you promote self catheterization?

A
  1. Introduce bead stringing earlier to practice
  2. Sitting balance
  3. Practice fine motor skills
70
Q

What do growth spurts increase the risk of in those with spina bifida?

A

risk of cord tethering

71
Q

When should a patient with spina bifida be independent with ADLs while using an ADL (as needed)?

A

school age

72
Q

(true/false) You may see progression in neurologic deficit when a spina bifida patient transitions into adulthood

A

true

73
Q

What is the most important factor of spina bifida prognosis?

A

motor function

74
Q

(true/false) FES may increase gait velocity and distance

A

true

75
Q

Evidence supports that ____ min/day of stepping, bouncing and treadmill training starting at 6 months can help with gait

A

10 minutes