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PT 641 Peds > Myelodysplasia > Flashcards

Myelodysplasia Flashcards

1
Q

neural tube defect

A

myelodysplasia

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2
Q

when can myelodysplasia be diagnosed

A

prenatally via US or postnatally as a sac present on the back at birth

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3
Q

what is contained in the sac

A

meninges and spinal cord tissue

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4
Q

since there is no definitive cause of spina bifida, what are some causes

A

genetics, environment, dietary (folic acid), chromosomal abnormalities

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5
Q

when should mother begin taking folic acid to help prevent spina bifida due to low levels

A

prior to conception

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6
Q

what is the recommended amount of folic acid intake for mothers

A

400ug before conception and 600ug during first trimester

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7
Q

what other factors can lead to spina bifida

A

maternal use of valproic acid (anticonvulsant), maternal use of antidepressants

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8
Q

at what day in embryo do cells of the neural plate differentiate into neural tube and neural crest

A

18

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9
Q

peripheral nervous system (cranial N, spinal N, autonomic N, ganglia)

A

neural crest

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10
Q

CNS, brain, and spinal cord (open at both ends)

A

neural tube

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11
Q

when does the superior end of neural tube close and what does it lead to if failure to close

A

~24/25 and anencephaly

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12
Q

when does the caudal end of neural tube close and what can it lead to if fails to close

A

~26/27 and spina bifida cystica or myelomeningeocele

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13
Q

motor and sensory is lost ____ the level of the spinal defect and loss of neural control of bowel and bladder

A

below

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14
Q

Infants present with sacral dimple
Red marks or hyperpigmentation on the back or tufts of hair
The spinal cord may not grow correctly, could cause issues later in life
Newborns with these physical signs are referred for further testing

A

Occult Spinal Dysraphism

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15
Q

where is spina bifida occulta most common

A

lumbar and sacral spine

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16
Q

Non-fusion of the halves of the vertebral arches, but normal neural tissue

A

Spina Bifida Occulta

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17
Q

Part of the spinal cord comes through the spine (pushed out sac)
Nerve fluid is in the sac
Not typically nerve damage
Less severity of impairments

A

meningocele

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18
Q

what may you see with spina bifida occula

A

tuft of hair along the spine, with or without sacral dimple

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19
Q

21-26% of parents of patients with spina bifida will have

A

spina bifida occulta

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20
Q

motor and sensory function thought to be normal in spina bifida occulta but will have increased incidence of what

A

UTI or tethered cord

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21
Q

what is Myelominigocele/ Meningomyelocele also called

A

spina bifida cystica

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22
Q

what is the most severe form of spina bifida and why

A

Myelominigocele/ Meningomyelocele because the spinal cord and nerves come through the opening

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23
Q

70-90% of infants born with Myelominigocele/ Meningomyelocele will also have

A

hydrocephalus

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24
Q

Superficial fatty mass in the low lumbar or sacral region with high incidence of bowel/bladder dysfunction

A

Lipomeningocele

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25
Q

by 5 mo gestation, what level is the spinal cord at; full term birth; adulthood

A

S1, L3, L1-2

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26
Q

happens when adhesions anchor to the spinal cord at the site of the original lesion so the spinal cord is unable to slide upward and reposition at typical location

A

tethered cord

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27
Q

increased stretch of the spinal cord caused metabolic changes and ischemia of the neural tissue and leads to

A

decreased muscle function

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28
Q

May see rapid scoliosis, increased tone, changes in gait pattern, changes in urological function

A

tethered cord

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29
Q

what are ways that are used to help with spina bifida prognosis

A

Antibiotics - help prevent infection at lesion
Ventriculoperitoneal (VP) Shunt - for hydrochepalus
Clean Intermittent catherization (CIC) - empty bladder and control UTI and renal deterioration

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30
Q

if spina bifida is present at birth, how early can they preform surgery to place the neural tissue into the vertebral column

A

72 hours after birth

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31
Q

medical complications of spina bifida

A
  • hydrocephalus
  • chiari II
  • urological conditions
  • latex precautions
32
Q

abnormal accumulation of CSF in cranial vault

A

hydrocephalus

33
Q

obstruction of CSF may be due to what

A

arnold chiari II - deformity of cerebellum (posterior cerebellum herniated downward through foramen magnum)

34
Q

possibly due to mistimed steps of the ventricular system development

35
Q

symptoms associated with chiari II

A

apnea, reflux, paralysis of vocal cords, facial palsy, poor feeding, hypotonia, UE weakness, seizures, nystagmus

36
Q

most children with chiari II have a smaller what

A

posterior fossa

37
Q

ways to monitor hydrocephalus

A

head circumference, head US, head CT or MRI

38
Q

what may you see in children with hydrocephalus due to increased sizes of ventricles

A

sunsetting or downward deviation of the eyes and a bulging anterior fontanelle

39
Q

used to relieve intracranial pressure and is a thin, flexible tube that runs from the lateral ventricles and either to the right atrium (VA) or peritoneal cavity (VP - more common)

40
Q

signs and symptoms of shunt malfunction in infants

A

building fontanelle, vomiting, change in appetite, sunsetting eyes, edema/redness along shunt tract

41
Q

signs and symptoms of shunt malfunction in toddlers

A

vomiting, irritability, HA, edema/redness along shunt line

42
Q

signs and symptoms of shunt malfunction in school-aged kids

A

HA, lethargy, irritability, edema/redness along shunt line, handwriting changes, high-pitched cry, seizures, rapid growth of head circumference, thinning of skin over scalp, newly noted nystagmus, newly noted eye squint, vomiting, decreased school performance, personality changes, memory changes

43
Q

what is used to empty the bladder if needed in kids with spina bifida

A

clean intermittent catheterization

44
Q

all pts with spina bifida are considered to have what type of sensitivity or outright allergy

45
Q

reactions to latex sensitivity/allergy in kids with spina bifida

A

itchy/watery eyes, sneezing, coughing, rash at site of contact, swelling of trachea, changes in BP, anaphylactic shock

46
Q

L1 possible muscle function

A

Upper/lower trunk (abdominals)
Hip flexors weak (2/5)

47
Q

L2 possible muscle function

A

Hip flexors 3/5
Hip adductors 3/5

48
Q

L3 possible muscle function

A

Knee extensors 3/5

49
Q

L4 possible muscle function

A

Medial knee flexors 3/5
Ankle dorsiflexors 3/5

50
Q

L5 possible muscle function

A

Hip abductors (weak) 2/5
Lateral knee flexors 3/5
Ankle inverters 3/5
Long toes extensors/EHL 3/5

51
Q

S1 possible muscle function

A

Hip abductors 3/5
Hip extensors (weak) 2/5
Plantar flexors (weak) 2/5

52
Q

S2 possible muscle function

A

Hip extensors 4/5
Plantar flexors 3/5
Toe flexors 3/5

53
Q

according to the spina bifida association, you must be a clear ___ to be classified with that motor level

54
Q

newborns with spina bifida typically have flexor contractures where

A

hips, knees, ankles

55
Q

orthopedic concerns for spina bifida

A

pes calcaneus/calcaneovarus, pes equinus (flat foot), convex pes valgus (rocker bottom foot), pes cavus, clubfoot, hip dislocation, scoliosis

56
Q

complete trunk flexion, exercise ambulation, sometimes household ambulation

57
Q

hip flexor muscles present, exercise ambulation, household ambulation

58
Q

knee extensors or quad muscles present, household ambulation, possible community ambulation

59
Q

medial knee flexors present, ankle Df, 3/5 strength

60
Q

community ambulation

61
Q

may walk with or without crutches in home

62
Q

hip abductors 3/5 strength

63
Q

hip extensors 4/5 strength, ankle PF 3/5, may walk with or without crutches

64
Q

bowel and bladder function varies; bladder parasympathetic input from pelvic N, somatic input from pudendal N to urethral sphincter

65
Q

all muscle activity may be within normal limits

66
Q

no motor control below the thorax and flaccid LE

A

thoracic level and high lumbar lesion

67
Q

early orthotic used for thoracic level/high lumbar lesion

A

A frame - standing frame –> allows pt to support to stand without use of UE to allow for play/feeding

68
Q

L1, L2, L3 do not have what muscles and would benefit from what device

A

HS or glutes, HKAFO

69
Q

possible community ambulation with KAFO

70
Q

uses a system of cables with dual action hip joints to flex one hip while maintain the opposite hip in extension during stance

A

reciprocating gait orthosis (RGO)

71
Q

what muscles are strong, weak, and absent in level L4/5 lesions

A

strong: hip flexors and adductors
weak: glut med and TFL
absent: hip ext/glut max

72
Q

what muscles are strong and absent in L4 lesion

A

strong quads and medial HS
absent lateral HS

73
Q

if have L4/5 lesion but are able to stand they can use what orthosis

74
Q

what is the tendency of gait when using AFO in L4/5 lesion

A

crouched position because unmatched force from gastroc/soleus

75
Q

muscle activation in S1/2

A

strong knee flexion, glut med, glut max
present but weak gastroc/soleus

PT 641 Peds (16 decks)

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