Spina Bifida Flashcards

(59 cards)

1
Q

what is spina bifida?

A

neural tube defect which occurs in utero and is present at the time of birth

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2
Q

how does anormal neural tube develop?

A

nervous system develops from a portion of the embryonic ectoderm called the neural plate. During gestation, the neural plate develops folds that begin to close, forming the neural tube

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3
Q

what is Spina Bifida Occulta?

A

Cosmetic impairment no physical disability
Lack of closure between vertebral arches
Spinal cord and meninges are unimpaired

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4
Q

what does Spina Bifida Occulta occur?

A

3rd month of development

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5
Q

in what portion of the spinal cord does spina bifida occulta usually occur?

A

lumbo-sacral area

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6
Q

what does spina bifida occulta look like?

A

Covered with skin and marked with a dimple or hair

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7
Q

what is spina bifida cystica (meningocele)?

A

Protrusion of the meninges and CSF only into a cystic sac
Spinal cord remains in the vertebral canal
(uncommon)

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8
Q

what is the prognosis of spina bifida cystica (meningocele)?

A

If spinal cord isn’t injured there aren’t abnormalities

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9
Q

what is spina bifida cystica (myelocele)

A

Dilation of the central canal of spinal cord producing a large covered cyst
Neural tube appears closed but is distended due to swelling
(rare)

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10
Q

what is spina bifida cystica (Myelomeningocele)?

A

Both spinal cord and meninges are herniated through the defective vertebral arches into a sac
Extensive abnormalities of spinal cord and neural tissue
(common)

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11
Q

what are the impairments seen for Myelomeningocele?

A

sensory and motor impairment at the level of the lesion and below

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12
Q

what can cause spina bifida?

A

Nutritional deficiencies (folic acid, vit A)
Environmental factors
Amniocentesis: high levels of alpha-phetoprotein

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13
Q

what are the clinical manifestations of Myelomeningocele?

A

Flaccid paralysis
loss of sensation
absent reflexes
bowel and bladder incontinence

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14
Q

what are the orthopedic problems of myelomeningocele?

A

Contractures of hip, knee, ankle and foot

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15
Q

what is muscle imbalance in meningomyocele caused by?

A

L4-L5 lesion will have inactive extensors and active flexors

no balance

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16
Q

what is the common position for myelomeningocele?

A

bilateral hip flexion and abduction that becomes contracted

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17
Q

what causes contractures in myelomeningocele?

A

muscle imbalance
Stress, posture and gravity
Associated congenital malformations
Abnormal positioning in utero

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18
Q

what Malformations of the vertebral bodies might require surgical reconstruction for myelomeningocele?

A

Hemivertebrae and various deformities
Lumbar deformities
Stress and gravity cause need for surgery

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19
Q

what can cause scoliosis for kids with myelomeningocele?

A

Congenital or acquired
Neurogenic due to weakness or asymmetrical spasticity
Lordoscoliosis is common in teens and associated hip flexion contractur

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20
Q

what deformities of the foot can occur to kids with myelomeningocele?

A

Equinovarus deformity (club foot)
Calcaneovalgus: calcaneus tipped
Vertical talus: talus tipped

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21
Q

what hip deformities occur for kids with myelomeningocele?

A

Hip dislocation and subluxation

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22
Q

what is the most common neurogentic bladder dysfx for kids with myelomeningocele?

A

Hypotonic bladder resulting in retention, incontinence and frequent urination with incomplete emptying

Bacterial growth causing UTI
OT teaches self-catheterization

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23
Q

what % of children with spina bifida develop hydrocephalus?

A

80-90%

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24
Q

what does hydrocephalus cause?

A

thinning of white matter and must be managed immediately

Uncontrolled can result in increased brain damage + death

25
what is Arnold Chiari Malformation Type II?
Brain structures herniated into neck (usually between C1-C4) not all symptomatic 99% chance Chiari malformation with myelomeningocele
26
what is Arnold Chiari Malformation Type II associated with?
paralysis of the vocal cords, respiratory difficulties and apnea
27
what is Hydromyelia?
Central portion of spinal cord swells
28
what can cause hydromyelia?
May be a consequence of untreated or poorly treated hydrocephalus
29
what are symptoms of hydromyelia?
rapidly progressive scoliosis, weakness and spasticity
30
how is hydromyelia txed?
with shunt revision or posterior cervical decompression
31
what causes tethered spinal cord?
scar tissue or lipoma (fatty tissue tumor) at the repair site
32
how do you tx tethered spinal cord?
Surgical intervention is performed to untethered the cord
33
what are the Cognitive and Perceptual Deficits of myelomeningocele?
Intellectual fxing vary: profound mental retardation to normal intelligence. (caused by hydrocephalus) Perceptual and visual-motor deficits area common Speech and language deficits Cocktail party speech
34
what is the medical management of myelomeningocele?
Closure of the sac takes place within24 to 48 hours after delivery. Neural tissue is placed back into vertebral canal Spinal defect covered for a water-tight sac closure
35
what is Hydrocephalus?
ventricles in brain increase in size due to reduction of flow of CSF
36
how do you tx hydrocephalus?
Placement of a ventricular shunt (ventricles to abdomen) for life
37
what are the types of ventricular shunts?
ventriculoperitoneal shunt (VP shunt): most common. composed of proximal catheter, distal catheter and 1 way valve. ventriculoatrial shunt (VA shunt) drains into heart b/c of abnormalities
38
how do you tx arnold chiari malformation?
Surgery – posterior cervical decompression to relieve pressure Respiration is affected neurologically Requires aggressive medical management since it can be life-threatening
39
how is bladder management controlled?
Clean intermittent catheterization (every 3-4 hrs) Urinary diversion through the abdominal wall Cutaneous vesicostomy Monitoring of bowel evacuation
40
what is the OT assessment for spina bifida?
Complete eval of functional motor skills (Orthopedics, ROM, MMT) Sensory testing with attention below level of lesion Perceptual testing (from hydrocephaly) Visual-motor testing ADL-functioning Evaluation of seating and positioning needs
41
what is muscular dystrophy?
group of hereditary myopathies characterized by progressive muscular weakness
42
what is duchenne muscular dystrophy?
Characterized by the replacement of muscle tissue with fibrous and fatty tissue (psedohypertrophy)
43
what does duchenne MD lead to?
total paralysis and early death in the late teens or young adulthood No cure
44
what is the incidence of duchenne MD?
X-linked and recessive pattern (only males affected)
45
what are the clinical features of duchenne MD?
Between ages 2 and 5 years symmetrical weakness proximal to distal first: weakness of shoulder girdle and pelvis next: Weakness of extremities and trunk Symptoms rarely before 2
46
what is the most common cause of death in duchenne MD?
respiratory failure
47
what occurs in lower extremities for duchenne MD?
1. Pseudohypertrophy 2. Development of hip, knee and ankle contractures 3. muscle imbalance + lordotic posture (pregos) + plantarflexion 4. loss of independt ambulation
48
what is duchenne MD Common compensation boys use to get off floor?
gowers maneuver: Maintaining hip flexion entire time and using arms to push up b/c of weak lower extremities
49
what occurs in upper extremities for duchenne MD?
Functional UE loss lags behind LE weakness (manual wheelchair) Early weakness at scapula and shoulder girdle (Meryon’s sign) Gradual loss at biceps, brachioradialis, triceps and distal musculature
50
how can you test for Meryon's sign?
Slip through hold at shoulders | due to weak shoulder muscles
51
what are the medical txs of duchenne MD?
Genetic counseling Corticosteroids and growth hormone no cure
52
what are the orthopedic problems with duchenne MD?
Scoliosis occurs in at least half of all cases
53
what are the onsets for duchenne scoliosis?
Early onset – develops prior to wheelchair age and tends to be most severe Late onset – develops typically 4 years after wheelchair dependence and is milder
54
what are surgical management options for duchenne scoliosis?
Spinal fusion with instrumentation (Harrington rods) Segmental fixation systems Surgical management can improve comfort in positioning
55
what are LE orthopedic problems with duchenne?
Hip and knee flexion contractures. | Equinus deformity
56
what are nutritional concerns for duchenne MD?
Excessive weight gain due to decreasing calorie requirement Nutritional assessment to prevent obesity that compromises fx, makes care more difficult Swallowing difficulties may require alternative feeding through nasogastric tube or gastrostomy (later)
57
what is OT assessment for duchenne MD?
Manual muscle testing ROM evaluation ADL assessment Mobility and seating needs
58
what are tx goals for duchenne MD?
Maintain ROM and prevent contractures Maintain strength / prevent atrophy from disuse Maximize ADL independence Maintain participation in school related-related tasks
59
what are the psychosocial considerations for duchenne MD?
Psychological support for the family and child Support groups Individual counseling for the child Peer support groups and community organizations