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731: Pediatrics > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Spina bifida is the ____ most common birth defect, occurring __ per 10,000 live births

A

second

3.4

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2
Q

What is the proposed etiology of spina bifida?

A

maternal lack of folic acid during the first trimester

also associated with the seizure medication valproic acid

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3
Q

It is suggested that the pregnant woman takes ___ μg of folic acid during the months prior to conception and ___ μg through the first trimester

A

400

600

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4
Q

The survival rate of spina bifida is __% with early treatment

A

90

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5
Q

Excellent prognosis is due to what 3 things?

A
  • he use of antibiotics to limit infection
  • The surgical insertion of shunts to manage hydrocephalus
  • Early and consistent use of clean, intermittent catheterization to completely empty the bladder
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6
Q

What is spina bifida aka?

A

Myelomeningocele

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7
Q

Describe the spinal defect that is present in spina bifida patients

A

There is an external sac on the infant’s back that contains meninges and spinal cord tissue that protrudes through a dorsal defect in the vertebrae

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8
Q

Where does the defect typically occur?

A

in the lumbar region

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9
Q

What is spina bifida occulta?

A

o A condition involving nonfusion of the halves of the vertebral arches, but without disturbance of the underlying neural tissue

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10
Q

How can spina bifida occulta be distinguished externally?

A

by a midline tuft of hair

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11
Q

There is a high rate of what 2 things in spina bifida occulta patients?

A

tethered cord and urinary tract disorders

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12
Q

What is myelocele?

A

A protruding sac containing meninges and CSF, but the spinal cord and nerve roots remain intact and in their normal positions which means there typically is no motor or sensory deficits, hydrocephalus, or other CNS problems

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13
Q

There is a high incidence of ______ and _____ dysfunction resulting from a tethered spinal cord

A

bladder and bowel

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14
Q

What is lipomeningocele?

A

A superficial fatty mass in the low lumbar or sacral level of the spinal cord

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15
Q

The caudal end of the neural tube closes on approximately day __ of gestation

A

26

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16
Q

What are the 4 clinical signs of spina bifida?

A
  • Absence of motor and sensory function (usually bilateral) below the level of the spinal defect
  • Loss of neural control of bowel and bladder function
  • Higher motor or sensory level on one side than on the other
  • Functional deficits may be partial or complete
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17
Q

What can be defined as an abnormal accumulation of CSF in the cranial vault?

A

Hydrocephalus

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18
Q

What is the keystone of Hydrocephalus?

A

bulging fontanels

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19
Q

What are the 4 causes of hydrocephalus?

A
  • Overproduction of CSF
  • A failure in absorption of CSF fluid
  • Obstruction in the normal flow of CSF through the brain structures and spinal cord
  • Arnold-Chiari malformation
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20
Q

What is considered to be the primary cause of hydrocephalus in children with spina bifida?

A

Arnold-Chiari malformation

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21
Q

What is Arnold-Chiari malformation?

A

It is a deformity of the cerebellum, medulla, and cervical spinal cord in which the cerebellum sinks into the posterior aspect of the cranium which results in the posterior cerebellum being herniated downward through the foramen magnum displacing brainstem structures caudally

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22
Q

Management of the neonate with spina bifida typically depends on the extent of what 3 things?

A
  • neurologic impairment
  • hydrocephalus
  • kyphoscoliosis/scoliosis
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23
Q

Early and aggressive surgical intervention is supported and it is now acceptable for the child’s back to be closed within __ hours of birth

A

72

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24
Q

Prior to surgery is PT intervention necessary?

A

An evaluation may not be imperative, but an assessment can offer insight into the severity of any ortho problems present at birth

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25
Q

Hydrocephalus is a common side effect that occurs in __% of children with spina bifida

A

90

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26
Q

Typically how is hydrocephalus managed?

A

via shunting

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27
Q

What are the 2 types of shunts?

A
  • Ventriculoatrial (VA) shunt

- Ventriculoperitoneal (VP) shunt

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28
Q

The VA shunt moves excess CSF from one lateral ventricle to where?

A

the right atrium of the heart

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29
Q

The VP shunt moves excess CSF from one lateral ventricle to where?

A

free abdominal space

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30
Q

Which shunting technique is preferred?

A

VP

31
Q

When should MMT be performed before and after surgery?

A
  • Before surgery
  • 10 days after
  • at 6 months
  • yearly thereafter
32
Q

What is the goal of early MMTs?

A

to assist the medical staff to identify the level of the back lesion by assessing LE movement or lack thereof

33
Q

What is a motor level assigned according to?

A

The last intact nerve root found (lowest level)

34
Q

What movements can be mistaken as voluntary in infants with thoracic level spina bifida?

A

Reflex movements at the knee (flexion) or ankle (PF/DF)

35
Q

Why is this reflex movement a concern?

A

It is usually unopposed by an active antagonist at the same joint and can therefore cause deformity

36
Q

Extreme tightness of which muscle group may be evident in the child with a motor level at L2-L3 or L3-L4?

A

hip flexors

37
Q

Extreme tightness of which muscle group may be evident in the child with a motor level at L5?

A

dorsiflexors

38
Q

Typically what position are ROM exercises performed in?

A

prone or side-lying

39
Q

Post-op ROM exercises should be performed 2-3 times per day with the PT’s hands close to the joint being moved which will make a ____ lever arm

A

short

40
Q

True or False

Aggressive stretching should always be avoided

A

True

41
Q

True or False

You should stroke the plantar surface of the baby’s foot in order to get them to react

A

False

They have no sensation on the bottom aspect of their feet

42
Q

Where should sensory stimulation be done at?

A

thigh or trunk

43
Q

How can you tell if a child’s shoe or orthosis does not fit correctly?

A

When areas of redness last longer than 30 minutes

44
Q

What is the earliest problems noted in a developing infant with spina bifida?

A

Instability of the head and upper body with delayed or weak acquisition of antigravity movement in all positions, balance, and equilibrium responses

45
Q

Children with spina bifida who demonstrate poor neck stability may retain what reflex longer than a typically developing infant?

A

Moro (startle)

46
Q

What are 4 reasons why upright positions are emphasized to parents?

A
  • They learn best in these positions through environmental interaction
  • Lung function is optimized
  • Abdominal activation
  • Vestibular Input
47
Q

Why is early weight bearing essential?

A

Promotes bone health and joint formation

48
Q

Describe the child with thoracic level paralysis

A

They have flaccid lower extremities and at risk for developing a frog-legged deformity (legs are abducted, ER, and flexed at the hips and knees with the feet in PF)

49
Q

What type of orthosis should be worn in a child with thoracic level spina bifida?

A

total contact body brace

50
Q

Describe the child with high lumbar level paralysis

A

Usually exhibit some active hip flexion and adduction, but usually no other strong movements at the knees or hips are present

51
Q

What defect/problem is common in children with high lumbar level paralysis?

A

hip dislocations

52
Q

True or False

ROM exercises should be discontinued following hip dislocation

A

False

53
Q

Describe the child with low lumbar level paralysis

A

Usually have strong hip flexors and adductors and poor to good hip abduction strength.

54
Q

What ____ deformity is common in low lumbar level paralysis?

A

clubfoot

55
Q

What is the expected functional level in children with low lumbar level paralysis?

A

Ambulatory in life unless increased body weight, flexion contractures, poor CNS status, or further complications exist

56
Q

What type of orthotics are required for ambulation in a child with low lumber level paralysis?

A
  • RGO (if CNS deficit present)
  • KAFO (weak quads)
  • AFO
57
Q

Describe muscle involvement in a child with sacral level paralysis

A

Retain use of all muscles with exception of glute max, gastroc, and foot intrinsics

58
Q

What is the expected functional level in children with sacral level paralysis?

A

Independent gait with moderate to miimal deviations based on patterns of weakness

59
Q

What type of orthotics are required for ambulation in a child with sacral level paralysis?

A

AFOs

60
Q

What provides the best assessment of bracing needs?

A

3D Gait Analysis with MMT

61
Q

What are 6 signs of CNS deterioration?

A
  • Nausea/Vomiting
  • Increased spasticity and tone
  • Sunsetting/Doll eyes (unable to attain vertical gaze)
  • Decreased balance
  • Complaints of headaches
  • Excessive crying
62
Q

What are 2 conditions that may indicate CNS deterioration?

A
  • hydromelia

- tethered spinal cord

63
Q

What is hydromelia?

A

A collection of excess CSF in pockets down the spinal cord that created areas of pressure and necrosis of the surrounding peripheral nerves

64
Q

What does hydromelia lead to?

A

scoliosis

65
Q

What are the characteristics of hydromelia?

A

Progressive upper extremity weakness and hypertonus

66
Q

How is hydromelia managed?

A

shunting

67
Q

What is tethered spinal cord?

A

Adhesions that are anchored to the spinal cord at the site of the original lesion which does not allow the spinal cord to move resulting in excessive stretch and ischemia of neural tissue

68
Q

How is tethered spinal cord managed?

A

surgically reparied

69
Q

Children with spina bifida have an increased incidence of ____ allergy

A

latex

70
Q

Children with spina bifida tend to have a wide variety of cognitive levels which seem to depend on what 3 things?

A
  • treatment of hydrocephalus
  • episodes of cerebral infection
  • the presence of other CNS abnormalities
71
Q

What type of special PT is recommended in children with spina bifida and why?

A

Helps to reduce tone and increase sensory involvement

72
Q

What are the most common complications in the young adult with spina bifida?

A
  • Obesity
  • Incontinence
  • Recurrent urinary tract infections
  • Chronic decubiti
  • Joint pain
  • Hypertension
  • Neurologic deterioration
  • Depression
73
Q

In conclusion what is the most common cause of morbidity in children with spina bifida?

A

urinary tract issues

731: Pediatrics (19 decks)

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