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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
s/s of tethered cords
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
Seizure disorders at birth are associated with
myelomeningocele (15%) generalized, tonic-clonic new onset seizures = shunt infection or failure
27
musculoskeletal problems
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
scoliosis most common in lesions where?
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
Other problems
problems with attention and execution learning disabilities altered growth - precocious puberty latex allergy - multiple surgeries
30
strabismus in __ % of children with \_\_\_&\_\_\_?
20% with hydrocephalus and myelomeningoele
31
in utero surgery outcomes
decresed neuro deficits, hydrocephalus & Chiari 2 malformations doesnt change functions of walking or continence increased risk fetal mortality
32
Post natal surgery outcomes
must be done emergently with 24-48 hours to prevent infection delivery at tertiary care center with neurosurgeon
33
Hydrocephalus treatments
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
SB medications
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
neuro eval
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
Urologic eval
BUN, Creatnine, US to r/o hydronephrosis or renal anomalies voiding cystourethrography to eval for reflux or if atb prophylaxis is needed
37
PCP monitoring of shunt
HC, fevers - esp in first 6 months after surgery 10% develop infection in first 6 months often develop malfunction within 2 years
38
s/s of shunt malfunctioning or infection
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
Loss of sensation means we can miss what?
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
Bowel training
encourage early and develop routine to promote eventual independence and self confidence Goal of continence or regular elimination of stool
41
Urinary concerns
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
Growth and puberty.. early or late?
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
Foods high in folic acid
rice, pasta, bread, cereals, broccoli, asparagus, nuts, black beans, soy beans, avocadoes, turkey and beef liver
44
Driving
they can drive with hand modifications
45
exercise/sports to recommend
aquatic therapy, adaptive skiing, adaptive bikes,
46
resources
spina bifida association spina bifida CDC

Fall 2020 Exam 2 (7 decks)

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