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Undeleted > congenital and pediatric neurological disorders > Flashcards

congenital and pediatric neurological disorders Flashcards

1
Q

neural tube closes…

A

15-28 days post conception

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2
Q

90% of neural tube defects are…

A

spina bifida and anencephaly

  • 1 in 5000 live births
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3
Q

anencephaly

A

both cerebral hemispheres absent

  • incompatible with life, many stillborn
  • if born alive, given comfort measures, DNR
  • most pts know this in advance
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4
Q

spina bifida (in general)

A

failure of closure of neural tube

NORMAL INTELLECT should be assumed in the absence of significant hydrocephalus

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5
Q

spina bifida occulta

A

failure of vertebrae to close WITHOUT protrusion of spinal cord or meninges
- not visible externally

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6
Q

spina bifida occulta: cutaneous manifestations

A

may or may not have skin depression, tuft of hair

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7
Q

spina bifida occulta: neuromuscular disturbances

A

most often asymptomatic

- may have foot weakness, bowel/bladder sphincter issues

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8
Q

spina bifida cystica

A

visible defect with saclike protrusion

- meningocele or myelomeningocele

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9
Q

meningocele

A

rare, contains meninges and CSF only

form of spina bifida cystica

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10
Q

myelomeningocele

A

most common, contains meninges, CSF, spinal cord, and/or nerve roots

form of spina bifida cystica

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11
Q

spina bifida risks

A
  • previous pregnancies with neural tube defects
  • folic acid deficiency
  • girls > boys
  • caucasian > african american
  • diabetes in mom
  • obesity
  • anticonvulsants
  • maternal heat exposure (hot tubs!)
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12
Q

folic acid

A

big deal!

  • aap recommended daily intake: 0.4 mg
  • preconceptual folate supplement may decrease incidence of ntd by 50-70%
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13
Q

folic acid for previous ntd*

A

increase to 4.0mg

  • 1 month before pregnancy through 1st trimester
  • under provider supervision!
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14
Q

prenatal screening detection (4)

A
  • ultrasound (16-18 weeks gestation)
    • accurate for dates NOT defects
  • alpha-fetoprotein (afp)
  • fetal mri
  • amniocentesis (14-17 weeks)
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15
Q

myelomeningocele: degree of neuro impairment:

A

usually loss of sensation and complete/partial paralysis

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16
Q

hydrocephalus

A

increased accumulation of csf within ventricles of brain; acquired vs congenital

  • most frequent anomaly associated with spina bifida
  • monitor for s/s of increased intracranial pressure (icp)
  • may not be apparent until after birth and after primary closure of defect*
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17
Q

myelomeningocele: effect on bladder

A

decreased nerve supply affects both sphincter and detrusor

- can cause constant dribbling or lead to overflow incontinence

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18
Q

myelomeningocele: effect on anal sphincter tone

A

lack of bowel control

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19
Q

myelomeningocele: orthopaedic abnormalities

A

dislocated hips, club feet

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20
Q

myelomeningocele: spinal deformities

A

scoliosis (more profound vs scoliotic patient with closed neural tube), kyphosis

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21
Q

spina bifida management: initial care*

A
  • PREVENTION OF INFECTION!
  • sac exposed to air
  • monitor closely
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22
Q

spina bifida management: neuro assessment

A
  • daily head circumference
  • assess fontanels for tension, bulging
  • check for associated anomalies
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23
Q

anterior fontanel closes

A

15-18 mo

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24
Q

posterior fontanel closes

A

1 month

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25
Q

spina bifida management: early closure of defect

A
  • within 24-72 hours after birth
  • increased chance of most favorable outcome – decreases morbidity/mortality especially from serious infection
  • decreases trauma to tissues
  • decreases stretching of nerve root
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26
Q

spina bifida care: gu functioning

A
  • urinary diversion

- often need to be intermittently catheterized on regular schedule: parents taught, child taught around 6 yo

27
Q

spina bifida care: bowel control

A

diet modification, regular toilet habits = decreases constipation and impaction

28
Q

acquired hydrocephalus

A

infection, neoplasm, hemorrhage

29
Q

communicating hydrocephalus

A

impaired absorption

30
Q

non-communicating hydrocephalus

A

impaired/obstructed flow of csf through ventricle system

31
Q

hydrocephalus: clinical manifestations in infancy

A

CUSHING’S TRIAD

  • bulging fontanel
  • dilated scalp veins
  • head growth abnormal rate
  • frontal bossing
  • sunsetting eyes
  • irritable, lethargic, change in loc, paradoxical cry, high pitched “neuro” cry, poor feeder
32
Q

cushing’s triad*

A

classic s/s increased icp

  • increased blood pressure, widened pulse pressure
  • bradycardia
  • abnormal or decreased RR

look at vitals TREND!

33
Q

hydrocephalus: clinical manifestations in child

A
  • irritable, lethargic, confused, apathetic
  • headache on awakening: improves after emesis or sitting up
  • ataxia
  • CUSHING’S TRIAD
34
Q

hydrocephalus: diagnosis

A

antenatal: fetal us @ 14 weeks
- increasing head circumference
- associated neurologic signs
- primary diagnostic tools: CT, MRI

35
Q

hydrocephalus: shunt procedure (2)

A

provides primary drainage of csf from ventricles to extracranial compartment, usually peritoneum

  • ventriculoperitoneal shunt (VP)
  • ventriculoatrial shunt (VA)
36
Q

ventriculoperitoneal shunt (VP)

A

surgical management of hydrocephalus - most common

37
Q

ventriculoatrial shunt (VA)

A

surgical management of hydrocephalus - rare

38
Q

VP shunt complications: infection

A

MOST SERIOUS! greatest risk 1-2 months after placement, may be sepsis or wound infection

39
Q

VP shunt complications: infection - s/s

A

shunt malfunction due to increased icp, fever, wound/shunt inflammation, abdominal pain

40
Q

VP shunt complications: infection - treatment

A

IV antibiotics for 7-10 days

- often requires removal of shunt and placement of extraventricular drainage system (EVD)

41
Q

extraventricular drainage system

A

possible procedure necessary after IV antibiotics for VP shunt infection

  • externalizes shunt until infection resolved
  • allows for removal of CSF to external collection
  • otherwise, shunt removed = increased icp
42
Q

VP shunt complications: shunt malfunction

A
  • typically mechanical obstruction

- most common reason for revision = displacement for growth

43
Q

VP shunt complications: shunt malfunction - s/s

A

also increased icp s/s!

older child: typical + personality change, neck pain, deterioration in school performance, decrease in sensory or motor functions

44
Q

have a shunt and vomiting? then…

A

SHUNT SERIES! CHECK FOR MALFORMATION! always, even if suspected stomach bug etc

45
Q

important nursing care s/p shunt malfunction

A

do NOT sedate child to reduce irritability - potentially masking s/s of increased icp

46
Q

hydrocephaly: prognosis - untreated

A

50-60% mortality if left untreated

- survivors have high incidence of subnormal intellectual capacity

47
Q

hydrocephaly: prognosis - surgical treatment

A

90% survival, 2/3 intellectually normal

48
Q

meningitis + types (3)

A

inflammatory process of meninges and csf

  • bacterial (pyogenic)
  • viral (aseptic)
  • tuberculous
49
Q

meningitis: bacterial - etiology > 2 mo

A

H flu, strep pneumo, n meningitidis

50
Q

meningitis: bacterial - etiology neonates

A

gbs, e coli, listeria

51
Q

HIB vaccine

A

decreased incidence of meningitis!!!!

52
Q

bacterial meningitis*

A

most common route of infection*

53
Q

bacterial meningitis: pathophysiology

A
  • vascular dissemination from a focus of infection elsewhere*
  • post lp, surgical procedures
  • anatomic abnormalities (spina bifida!)
  • foreign body (VP shunt!)
  • infective process (exudate, wbc accumulation, tissue damage
  • brain: hyperemic, edematous, covered with purulent exudate, can lead to obstructive hydrocephalus
54
Q

hyperemic

A

increased blood flow to certain part of body

55
Q

bacterial meningitis: clinical manifestations in children/adolescents

A

abrupt or gradual onset:

  • fever, chills, headache, v, change in sensorium, irritable, agitated, photophobia, confusion, hallucinations, drowsiness, stupor, coma
  • SEIZURE can be initial manifestation!
  • nuchal rigidity! NOT CONSISTENTLY PRESENT IN > 18 MO
  • kernig sign, brudzinski sign
  • meningococcal purpuric/petechial rash
56
Q

kernig sign

A

clinical manifestation of bacterial meningitis in children/adolescents

flex thigh on body and extend leg
- positive: resistance and pain in hamstring

NOT CONSISTENTLY PRESENT IN > 18 MO

57
Q

brudzinski sign

A

clinical manifestation of bacterial meningitis in children/adolescents

pt supine: flex head
- positive: pain or hips/knees flex involuntarily

NOT CONSISTENTLY PRESENT IN > 18 MO

58
Q

meningococcal-purpuric/petechial rash clinical manifestation of…

A

bacterial meningitis

59
Q

bacterial meningitis: clinical manifestations in infants/young children

A

3 mo - 2 yo

  • bulging fontanel MOST SIGNIFICANT
  • fever, poor feeding, vomiting, irritable, restless, high pitched cry
  • nuchal rigidity, brudzinski, kernig NOT CONSISTENTLY PRESENT IN > 18 MO
60
Q

bacterial meningitis: clinical manifestations in neonate

A

difficult to diagnose! vague like ALL neonatal sepsis

- neck is usually supple

61
Q

bacterial meningitis: csf diagnosis

A

lumbar puncture (DEFINITIVE TEST)

  • increased spinal fluid pressure
  • elevated wbc, protein concentration
  • glucose DECREASED
  • cloudy
  • gram stain reactive
62
Q

menectra vaccine

A

prevents bacterial meningitis, for ages 11-55

63
Q

viral meningitis: csf diagnosis

A

lumbar puncture

  • wbc slightly elevated
  • protein WNL or slight increase
  • glucose WNL
  • clear/slightly cloudy
  • gram stain: not reactive
64
Q

viral meningitis: manifestations & tx

A

clinical manifestations: ha, fever, malaise, gi symptoms

treatment: if symptomatic, hydration, pain management, positioning

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