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Fall 2020 Exam 2 > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Open neural tube defects

A

brian, spinal cord or spinal nerves are exposed through defects in the meninges and skull or vertebral collumn

(ancephaly, myelomeningocele, meningocele)

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2
Q

Closed neural tube defects

A

skin covered lesions under which the nervious system structures are abnormally formed

(spina bifida oculta, split cord malformation, derman sinus tract, tethered spinal cord, intraspinal lipoma)

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3
Q

Signs of a closed NTD

A

sacral dimple. gluteal cleft, tuft of hair or skin tag at sacral area

assess dimple with otoscope to see if there is skin at base, palpate the dimple for the coccyx

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4
Q

spina bifida

A

mildest form - often not diagnosed until adulthood

occulta means hidden

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5
Q

meningeocele

A

part of the spinal cord protrudes through the spine like a sac

there is no nerve damage

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6
Q

Myelomeningocele

A

Most common and most severe type (85%)

opening on the spine where both nerve roots and spinal cord tissues protrude unto a fluid-filled sac on the back

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7
Q

Upper lesions- thoracic and higher lumbar areas

A

associated with significant motor and sensory defecits and structural abnormalities in lower extremities

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8
Q

Lower lesions- lower lumbar and sacral regions

A

less likely to have defecits/structural anomalies

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9
Q

Functional defecits of urogenital and lower intestinal tract can occur with

A

lesions at all levels and are likely

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10
Q

prevalence is decreasing due to

A

improved prenatal nutrition, folic acid supplementation and mandatory enrichment of flour with folate

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11
Q

Ethnicity at higher risk

A

latino women

corn flour doesnt have folate mandate

and those who have recently moved here

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12
Q

occurs more freuently in

A

younger women

often in the first pregnancy

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13
Q

Maternal risk factors for SB

A

exposure to alcohol, valporic acid, carbamazepine (seizures/bipolar/nerve pain), isotretinoin (acne), hyperthermia from hot tubs/saunas, malnutrition (esp folate), diabetes, obesity

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14
Q

Family h/o NTD increases risk by

A

20-fold

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15
Q

SB genetic factors

A

irish, scottish, northern europeans possibly due to altered folate metabolism

chromosomal disorders like trisomy 13/18 and microdeletion 22q11 are linked to NTDs

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16
Q

How are open NTDs diagnosed?

A

Prenatally

ultrasound and serum markers

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17
Q

Serum marker for open NTDs

A

maternal serum alpha-fetoprotie (AFP)

evaluated between 16-18 weeks

elevated with open myelomeningocele

if abnormal US is used to eval head/back

amniocentesis can eval for AFP and acetylchoinesterase, an enzyme found in CSF

Chrom eval to r/o associated conditions

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18
Q

What do we do if an open NTD is discovered at birth?

A

cover the lesion

prone positioning, transfer to NICU with neurosurgeon for emergent repair

parenteral ATB until closure to prevent CNS infection

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19
Q

Sensory and motor defecits

A

lower extremities and genitals

sensory defecits - skin breakdown/ulcers - careful assessment is needed

decreased male or female sexual function - ED

20
Q

Bowel and bladder dysfunctions

A

60% have some degree

almost all myelomeningocele cases have neurogenic bladder

most have disorders with innervation of lower int tract and anus with lower tract dysmotility, poor or absent sphincter control, leads to fecal incontinence

may require cath and bowel programs

21
Q

ankle and foot orthotics

bracing

walkers or wheelchairs

A

encourage standing position for bone calcification- limited standing will lead to leg legnth discrepancy and increased risk of fractures

referral to PT/OT

22
Q

cognitive development

A

most have normal intelligence with severe learning disabilities, nonverbal disorder, poor executive skills

factors affecting cog dev: shunt infection, Chiari ll malformation, hydrocephalus with repeat shunt replacements, neuronal migration disorders,

DEVELOPMENTAL surveilence at all visits -dev and learning disorders can present at any age

23
Q

hydrocephalus

A

occurs in 85% of pts with myelomeningoceles

usually requires shunt placement

may be caused by closure of the defect with changes to CSF draining

24
Q

Chiari ll malformations s/s

A

associated with myelomeningeocele

occurs from herniation of cerebellar vermis, brainstem, and 4th ventricle

s/s: dysphagia, poor/prolonged feeding, pooling of oral secretions, cyanosis, choking, coughing, nasal regurgitation, hoarseness/stridor, aspiration, apnea, stiffness, weakness, decreased arm function

25
Q

s/s of tethered cords

A

back pain, weakness, worsening of scoliosis from muscle imbalance, changes to bowel and bladder (often during rapid growth bone legnthens and streches cord, ages 5-9 and adolescence)

26
Q

Seizure disorders at birth are associated with

A

myelomeningocele (15%)

generalized, tonic-clonic

new onset seizures = shunt infection or failure

27
Q

musculoskeletal problems

A

club feet, hip sublazation and dislocation, knee instability, contractures present at birth,

especially in lumbar/thoracic lesions

osteopenia - scoliosis

need specilaized evacuation plan at school

28
Q

scoliosis most common in lesions where?

A

most common in kids with lesions above the second lumbar vertebra

start monitoring for in preschoolers

any progression of scoliosis warrants a neuro eval and imaging to r/o tethered cord, hyrdomyelia or shunt malfunction

29
Q

Other problems

A

problems with attention and execution

learning disabilities

altered growth - precocious puberty

latex allergy - multiple surgeries

30
Q

strabismus in __ % of children with ___&___?

A

20% with hydrocephalus and myelomeningoele

31
Q

in utero surgery outcomes

A

decresed neuro deficits, hydrocephalus & Chiari 2 malformations

doesnt change functions of walking or continence

increased risk fetal mortality

32
Q

Post natal surgery outcomes

A

must be done emergently with 24-48 hours to prevent infection

delivery at tertiary care center with neurosurgeon

33
Q

Hydrocephalus treatments

A

15% with SB may not need shunting

may delay until need is evident (weeks)

after repair neuro will monior HC and ventricular size by Xray or MRI

34
Q

SB medications

A

used to treat symptoms and ass conditions

anticholinergics & antispasmotics - decrease spasticity help with sphincter control and decrease high muscle tone in the bladder

injections and surgeries can be done for sphincter control and incont,

fiber, stool softners and laxitives for constipation

35
Q

neuro eval

A

including anal wink, DTRs, movement and sensation of lower extremities

Daily HC, HUS or MRI needed

monitor for irritability

Infants with open fontanel may not be symptomatic of increased ICP, HC crossing percentiles on growth chart, MRI of enlarging ventricles determine need for shunt

genetic counseling provided

36
Q

Urologic eval

A

BUN, Creatnine, US to r/o hydronephrosis or renal anomalies

voiding cystourethrography to eval for reflux or if atb prophylaxis is needed

37
Q

PCP monitoring of shunt

A

HC, fevers - esp in first 6 months after surgery

10% develop infection in first 6 months

often develop malfunction within 2 years

38
Q

s/s of shunt malfunctioning or infection

A

fever, bulging anterior fontanel, apnea, vomiting, change in mental status, worsening b/b function, back pain or worsening scoliosis, head ache, change in function of upper extremities

need school emergency plan for ICP

39
Q

Loss of sensation means we can miss what?

A

fractures or scoliosis

no pain signs

families should monitor calcium and vit D intake

Xrays if concern for fracture, observe for swelling or worsening of contractures

40
Q

Bowel training

A

encourage early and develop routine to promote eventual independence and self confidence

Goal of continence or regular elimination of stool

41
Q

Urinary concerns

A

risk for UTIs and renal tract injury d/t neurogenic bladder, monitor BP - high pressure

CIC, private place at school or vesicostomy

All positive urine cultures in kids who CIC should not be treated unless + fever, abd/back pain, vomiting, or incont between cath times occurs

May also have low pressure- leaking- impaired sphincter control

medications to decrease tone or improve sphincter or surgery are possible

42
Q

Growth and puberty.. early or late?

A

precocious puberty common in girls with myelo and hydrocephalus

average start 2 years earlier

affects sexual maturation and linear growth

recomend folic acid to all sexually active females

43
Q

Foods high in folic acid

A

rice, pasta, bread, cereals, broccoli, asparagus, nuts, black beans, soy beans, avocadoes, turkey and beef liver

44
Q

Driving

A

they can drive with hand modifications

45
Q

exercise/sports to recommend

A

aquatic therapy, adaptive skiing, adaptive bikes,

46
Q

resources

A

spina bifida association

spina bifida CDC

Fall 2020 Exam 2 (7 decks)

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