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Peds > Myelodysplasia > Flashcards

Myelodysplasia Flashcards

1
Q

Myleodysplasia

A

Defective development of any part of the spinal cord

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2
Q

Spina bifida

A

Most commonly used to describe various forms of defects of neural tube closure

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3
Q

Aperta

A

Visual or open lesion

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4
Q

Occulta

A

Hidden or not visible lesion

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5
Q

Myelomeningocele

A

External protrusion of meninges and spinal cord

Not covered by skin

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6
Q

Meningeocele

A

External protrusion of meninges

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7
Q

Diastematomyelia

A

Fibrous, cartilaginous or bony band separating the spinal cord into hemicords each surrounded by dural sac

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8
Q

Lipoma

A

Occulta, but usually visible subcutaneous fat mass
Classified by location
Lipomyelomeningocele w/ paralysis
Lipomeningocele w/ no paralysis
Lipoma of the filum terminal
Lipoma of the Claudia equina or conus medullaris

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9
Q

Upper end of neural tube usually closed on

A

25th day

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10
Q

Bottom of the neural tube usually closed

A

27/28 day

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11
Q

Spina bifida is the result of

A

The neural tube failing to develop or close properly

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12
Q

Myelomeningocele: genetics

A

Recurrence 2-3% risk in siblings
Associated w/ “syndromes”
High variability in incidence among various ethnic groups

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13
Q

Myelomeningocele: teratogens

A

-maternal alcohol consumption
Valproic acid
Street drug abusers

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14
Q

Myelomeningocele: nutritional deficiencies

A 
-inadequate folic acid!! 
Begin supp at least 3m before conception
-reduce risk 70%
-w/ fam history/1st deg relative - 4mg/day
W/out fhx 0.4mg/day
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15
Q

Internationally: % of pregnancies with prenatal dx of neural tube deficits are terminated

A

23%

Dx usually occurs at 18 weeks

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16
Q

Myelomeningocele DX:

A

Maternal alpha-fetoprotein screenings, US, amniotic fluid analysis
-lemon sign, banana sign

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17
Q

Myelomeningocele: perinatal management

A

Prenatal dx allows for repairs in utero

Post natal closure of tube requires sx w/in 48 hours to avoid infection and drying of nerve roots

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18
Q

Myelomeningocele musculoskeletal deformities

A

Spinal and LE limb deformations and contractures

Restriction in UE ROM due to overuse for WB and poor posture

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19
Q

Myelomeningocele typical postural deficits

A

Forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, ant pelvic tilt, rotational deformities of hip or tibia, flexed hips and knees, pronated feet

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20
Q

Myelomeningocele: thoracic to L2

A

Hip flex, AB, ER contracture
Knee flex contract
PF contract
Lordotic lumbar spine

21
Q

Myelomeningocele: L3-L5

A 
Hip and knee flex contracture
Increased lumbar lordosis 
Genu and calc valgus
Pronated feet
Often walk in crouched gait and bear weight through calcaneous
22
Q

Myelomeningocele sacral level

A

Mild hip and knee flexion contracture
Increased lumbar lordosis
Foot in varus or valgus w/ pronated and supinated foot
Mild crouching gait

23
Q

Myelomeningocele osteoporosis

A

Decrease bone mineral density

  • walking ability highly correlated to increased bone mineral density
  • fax may not be immediately apparent due to poor sensation
24
Q

Myelomeningocele motor paralysis

A

Motor level - lowest intact functional NM seg

May be asymmetric

25
Q

Myelomeningocele sensory deficit

A

Don’t always correlated to motor levels
Eval
Pt ed

26
Q

Myelomeningocele hydrocephalus

A

At birth 25% or more
Additional 60% develop after sx closure of lesion
80-90% require CSF shunt

27
Q

Myelomeningocele cog function

A

W/ or w/out uncomplicated hydrocephalus, intellegence typically average

28
Q

Myelomeningocele latex allergy

A

73%

29
Q

Myelomeningocele seizures

A

10-30%

30
Q

Myelomeningocele skin breakdown

A

Decubitus ulcer and other breakdown occur in 85-95%

31
Q

Myelomeningocele cranial nerve palsy

A

May result from chiari malformation, hydrophealus, dysphasia of brain system

32
Q

Myelomeningocele neurogenic bowel

A

<5% have control of urinary or anal sphincter

33
Q

Myelomeningocele neurogenic bladder

A

Early catheterization will help avoid overstretching bladder wall
Can learn to self cath by 6-8yo

34
Q

Myelomeningocele exam

A 
ROM WNL
Strength, 
GM, FM, ADL
Sensation
Home and school enviro
*delays in GM, use of AD to facilitate exploration, social, ADLS
*monitor joint alignment, strength, contracture, posture
*tethered cord
35
Q

Myelomeningocele common goals across life span

A

Prevent joint contracture
Correct existing deformities
Prevent/min effect of sensory and motor deficiency
Optimize mobility w/in natural enviro

36
Q

Myelomeningocele outcome T10 above

A

Strong UE, upper TSpine, neck motions

Weak lower trunk, diff w/ sitting and resp

37
Q

Myelomeningocele outcomes T12

A

Strong trunk and sitting balance

May have weak hip hike form QL

38
Q

Myelomeningocele outcomes: general thoracic

A

W/c
Tend to have greater CNS involve- cog impairment
Supervision required in living situation
May work in sheltered settings or volunteer work

39
Q

Myelomeningocele L1-L2

A

L1 weak hip flex
L2 hip flex, add, rotators 3/5 or higher
-frequent dislocation of hip. Due to unopposed flex or ad
Household amb for small children poss w/ support/orthotics
W/c for community
50% live (I)

40
Q

Myelomeningocele L3

A 
Strong hip flex, weak rotation
Some knee ext
KAFO and crutches for household in childhood
W/c for community 
60% (I)
41
Q

Myelomeningocele L4

A

Calc deformities common - unopposed ant tib
Strong knee ext
Funct amb w/ AFO and crutches (20% amb as adult)
Focus on maintaining ankle joint alignment

42
Q

Myelomeningocele L5

A 
Lateral hamstring 3/5 and either
-glut min 2, post tib 3, peroneus tertius 4
Antigravity knee flex and weak hip ext
Require orthoses for alignment
Bilateral UE support recommended
80% achieve (I) living
43
Q

Myelomeningocele S1

A

Gast/sol 2
Glut med 3
Glut max 2
Amb w/out orthotics for support

44
Q

Myelomeningocele S2, S2-3, and “no loss”

A

S2- decreased push off and stride length w/ running
S2-3 more LE have 5, few 4
“No loss” normal BB function, normal strength

45
Q

Myelomeningocele intervention

A

Children encouraged w/ high dose or normal developmental activities in at risk areas
Remediation - rep of graded task in area of concern
Reach compensatory skills - inc independence

46
Q

Myelomeningocele strengthening indicated if

A

Function is present
Weakness in muscle group important for postural stability, ADL, mobility or balance of muscle forces around joint
Strengthen w/in functional ROM

47
Q

Myelomeningocele mobility: measure energy expenditure via

A

HR

48
Q

Myelomeningocele indications for gait training

A

Potential for progression to new orthosis or AD
Potential for improving pattern
Improve safety and confidence
Efficiency of gait and transfer

49
Q

Myelomeningocele: key muscle groups for amb

A 
Iliopsoas
Glute med and max
Quad
Ant tib
Hamstring

Peds (18 decks)

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