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Special Populations > Myelomeningocele > Flashcards

Myelomeningocele Flashcards

1
Q

what is the etiology of Myelomeningocele?

A

Nervous system develops from a portion of the ectoderm called the neural plate

  • Neural plate → neural tube → brain and spinal cord
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2
Q

what is the incidence of myelomeningocele?

A

2/1000 births

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3
Q

when and where does the neural tube close?

A

begins in cervical region and proceeds cranially and caudally

completed by 26th day

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4
Q

what is spina bifida?

A

defect in neural tube closure and the overlying prosterior vertebral arches

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5
Q

what is the cause of spina bifida?

A
  1. no definitive cause, but there may be genetic predisposition that is enhanced by environmental factors
  2. genetic risk factors:
    • celtic orgin
    • previous spina bifida births
  3. environmental risk factors:
    • lower SES
    • deficiences
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6
Q

prenatal testing that indicates spina bifida

A
  1. AFP at abnormally high level in amniotic fluid past 14 weeks gestational age, compared with US results
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7
Q

list the types of spina bifida

A
  1. Occulta
  2. Cystica
    • Meningocele or myelocele
    • Myelomeningocele
    • Anencephaly
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8
Q

What is Occulta Spina Bifida

A

failure of vertebral arches to meet and fuse in 3rd month

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9
Q

describe Occula Spina Bifida

A
  1. overlying skin may be marked by dimble, pigmentation, or patch of hair
  2. lumbosacral area a common site
  3. usually associated with no neurological or musculoskeletal dysfunction
  4. however, there in a higher incidence than normal of urinary tract disorders
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10
Q

what is Cystica Spina Bifida

A

failure of neural tube and vertebral arches to close with cystic protrusion of meninges with or without spinal cord

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11
Q

describe meningocele/myelocele

A
  1. spinal cord remains in vertebral canal but it may be abnormal
  2. lipomeningocele
    • superficial fatty mass in low lumbar or sacral area
    • high incidence of B&B dysfunction due to tethered cord
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12
Q

describe myelomeningocele

A
  1. extensive spinal cord abnormalities
  2. most common in thoracic and lumbosarcal regions
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13
Q

describe anencephaly

A

cranial end of neural tube does not fuse

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14
Q

Clinical manifestation of Myelomeningocele

A
  1. loss of sensory and motor function
  2. orthopedic abnormalities
  3. hydrocephalus
  4. brain/spinal cord abnormalities
  5. B&B dysfunction
  6. cog impairments/learning issues
  7. integumentary impairment
  8. disturbed growth and development
  9. psychosocial issues
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15
Q

Loss of sensory and motor function in myelomeningocele is dependent on what?

A
  1. degree of spinal cord abnormality
  2. traction or stretch on spinal cord
  3. trauma to exposed neural tissue during delivery
  4. postnasal damage resulting from drying or infection of the neural plate
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16
Q

describe sensory impairment in spina bifida

A
  1. sensation below level of lesion is impaired
  2. may not exactly match level of lesion
  3. includes:
    • kinesthetic
    • proprioceptive
    • somatosensory
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17
Q

what are the two main types of motor dysfunction in spina bifida?

A
  1. Type 1 → complete loss of function below level of lesion resulting in flaccid paralysis, loss of sensation, and absent reflexes
  2. Type 2 → mixture of flaccidity and spasticity
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18
Q

List several orthopedic problems that can result from spina bifida

A
  1. deformity/contractures
  2. osteoporosis
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19
Q

what can be the cause of deformities in spina bifida?

A

due to

  1. muscle imbalance
  2. effect of stress
  3. posture and gravity
  4. congenital malformations
  5. decreased sensation
  6. neurological complications
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20
Q

list typical congenital malformations found in spina bifida

A
  1. hemivertebrae
  2. deformed vertebral bodies and ribs
  3. missing ribs
  4. lumbar kyphosis
  5. club feet
  6. rocker bottom feet
  7. gibbus deformity
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21
Q

describe hydrocephalus

A
  1. present in 80-90%
  2. blockage of normal flow of CSF
  3. signs
    • increased head size
    • bulging fontanelles
    • irritability
    • lethargic
    • sunset eyes
  4. typically develops following closure of sack
  5. watch for signs of shunt malfunction in child w/shunt
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22
Q

interventions for hydrocephalus

A

VA/VP shunt

shunt revision

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23
Q

Signs of shunt malfunction in infants

A
  1. bulging fontanelle
  2. vomiting
  3. change in appeptite
  4. sunset sign
  5. edema, redness along shunt
  6. thinning of skin over scalp
  7. high-pitched cry
  8. irritability
  9. lethargy
  10. seizures
  11. excessive growth of head
24
Q

Signs of shunt malfunction in toddlers

A
  1. vomiting
  2. irritability
  3. HAs
  4. edema, redness along shunt tract
  5. lethargy
  6. seizures
  7. new nystagmus
  8. new squint
25
Q

Signs of shunt malfunction in school-aged children

A
  1. HAs
  2. Lethargy
  3. Irritability
  4. Edema, redness along shunt tract
  5. handwriting changes
  6. vomiting
  7. seizures
  8. decreased school performance
  9. personality changes
  10. memory changes
26
Q

List several brain and spinal cord abnormalities

A
  1. Arnold Chiari Malformation
  2. Hydromyelia
  3. Tethered Cord
27
Q

what is an Arnold Chiari Malformation?

A

brainstem (medulla, possibly pons and 4th ventricle) and cerebellum herniate through foramen magnum usually into upper cervical canal (C1-C4)

28
Q

Symptoms of arnold chiari malformation

A
  1. respiration
  2. paralysis of vocal chords
  3. bulbar dysfunction
  4. apnea
  5. swallowing
  6. abnormal gag
  7. UE weakness
29
Q

what is hydromyelia?

A

dilation of the center canal of spinal cord, may be focal, multiple, or diffuse

30
Q

what are some causes of hydromyelia?

A
  1. may be a consequence of untreated or inadequately treated hydrocephalus
    • due to increased hydrostatic pressure from above
  2. may cause pressure necrosis of spinal cord, leading to muscle weakness and scoliosis
31
Q

List symptoms of hydromyelia

A
  1. rapidly progressive scoliosis
  2. UE weakness
  3. spasticity
  4. ascending motor loss in LE
32
Q

Interventions for hydromelia

A
  1. shunt revision
  2. posterior cervical decompression
  3. central canal to pleural cavity with a flushing device
33
Q

what is a tethered cord?

A

pathological fixation of the spinal cord in an abnormal cuadal location

  • causes a mechanical stretch, distortion, and ischemia
  • suspect in any pt w/abnormal neurulation
34
Q

List symptoms of a tethered cord

A
  1. decreased strength
  2. development of LE spasticity
  3. back pain at sit of sac closure
  4. change in urological function
35
Q

Intervention for a tethered cord

A

release

36
Q

Cognitive and learning issues with spina bifida

A
  1. visual motor perceptual deficits
  2. difficulties with spatial relations, body image, development of hand dominance
  3. poor hand function
  4. “cocktail party speech”
37
Q

Integumentary issues in spina bifida

A
  1. Latex allergy → use latex free gloves, therapy balls, theraband, tape
  2. Pressure sores
    • parental training
    • child training
38
Q

Bowel and bladder issues in spina bifida

A
  1. self cath taught early if possible
  2. body image, fine motor skills, perceptual skills impacted
39
Q

PT Interventions for spina bifida

A
  • appropriate assessment and interventions to include:
    1. ROM
    2. MMT
    3. Sensory testing
    4. Developmental testing (possibly all areas)
    5. Positioning and equipment needs
    6. Education needs for family and child
    7. Balance
    8. Coordination
    9. Reflexes
    10. Muscle tone
    11. Skin condition
40
Q

age-specific prenatal isues in spina bifida

A
  1. possible diagnosis, referral to EI
  2. possible sac closure in utero
  3. C section to prevent trauma to neural sac
  4. PT intervention → edu, assist with referrals
41
Q

age-specific newborn issues in spina bifida

A
  1. sac closure within 24-48 hours
  2. shunt procedure
42
Q

PT concerns in newborns with spina bifida

A
  1. Assess function to determine baseline pre/post surgery
  2. ID muscle imbalances, possible deforming forces
  3. ID need to positioning, bracing, taping
  4. address special concerns
43
Q

Special concerns in newborns with spina bifida

A
  1. MMT
  2. state → alert or crying
    • may need to use tactile or vestibular to alert child
  3. positions → sidelying or prone
  4. observe spontaneous movements, response to sensory input
  5. differentiate between reflex and voluntary movement
44
Q

PT concerns in Infant/Toddlers with spina bifida

A
  1. Facilitate developmental sequence
  2. Prevent deformity
  3. Home program
  4. Early weight bearing → watch alignment
  5. Skin care
  6. Emphasize balance and equilibrium in sitting/standing/core strength
  7. Importance of fine motor skills
45
Q

PT concerns in Toddlers-Preschoolers with spina bifida

A
  1. Emphasize progressive LE weight bearing/gait training activities, self care
  2. EI and school-based programs, community programs, adapt normal equipment/activities as necessary
  3. Bladder training
  4. MMT
    • antigravity control in developmental positions
    • during play
    • weighted toys
  5. Sensory
    • can start assessing light touch and position changes as cognitive and language skills permit
46
Q

PT concerns in primary grades - adolescent

A
  1. By 6, children become aware of their differences
  2. constantly reevaluate
    • body grows, muscle strength and length does not keep up, equipment needs change, skin and ROM issues may increases as more time is spent sitting
  3. Independent community mobility a necessity → wheelchair prescriptions if not already
  4. great changes in adolescence → prepare family
47
Q

how does Spina Bifida present if impacting the Thoracic Levels

A
  1. Flaccid LE → “frog leg” position
  2. Prone positioning, daily ROM, gentle wrapping
  3. Surgical intervention → IT band, hip ER, knee flexors
  4. Total contact orthosis for standing
  5. With age and increased sitting → resting AFO
48
Q

How would a high Lumbar T12-3 spina bifida present?

A
  1. strong hip flexors and adductors
  2. require bracing to stand
  3. hip subluxation and dislocation common
  4. parapodium/swivel walker/A frame
  5. RGO
  6. HKAFO
49
Q

what is a parapodium?

A

a full contact orthosis that allows you stand

50
Q

how would a low lumbar L4-L5 spina bifida present?

A
  1. hip → strong hip flexion and adduction
  2. some hip abduction (glut med or TFL)
  3. strong quads, medial hamstrings
  4. anterior tib, some foot intrinsics
  5. twister cables due to rotation, KAFO is quad weak, floor reaction AFOs
51
Q

Sacral level spina bifida

A
  1. better muscle balance at hips and kees with full or partial innervation of hip extensors and lateral hams, stronger glut med hams, quads, considerable variation in foot ankle
  2. gastroc may be weak
    • toe flexors may provide some PF but usually not strong enough for gait → AFO needed
  3. watch out for crouched gait with increased weight gain
  4. aggressive trx for hip instability
52
Q

0-6 month psychological adjustment

A

parents may be in shock, denial

may misinterpret or reject info

53
Q

6-18 month psychological adjustment

A

very stressful, lots of doctor’s appointments, hospitalizations

54
Q

2 years through school age psychological adjustments

A

concerns about toilet training, social acceptability

55
Q

6 years psychological adjustments

A

child more aware of disability

  • family attitudes and environmental conditions will influence child’s adjustment
  • important to be given opportunities to interact with peers, age-appropriate responsibilities
56
Q

adolescence psychological adjustments

A
  1. B&B management
  2. sexual issues
  3. females → potential for orgasm and fertility
  4. males → frequently sterile with small testicles and penises
  5. potential for erection and ejaculation depends on lesion

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