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Pediatrics > Myelodysplasia Part 1 > Flashcards

Myelodysplasia Part 1 Flashcards

1
Q

Myelomeningocele (MM)

A

open spinal defect, dorsally protruding sac containing meninges, spinal fluid, and neural elements

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2
Q

Myelodysplasia

A

refers to defective development of any part of the spinal cord; the term Spina Bifida is the commonly used term referring to various forms of myelodysplasia

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3
Q

Physical therapy for myelodysplasia

A

prevent secondary impairments contributing to functional limitations and participation restrictions

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4
Q

What is neurological damage, loss of muscle activity and sensation determined by?

A

vertebral level

Expected mobility and independence affected by pattern of innervation

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5
Q

Who is incidence highest in?

A

highest in Hispanic, lowest in black populations

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6
Q

Etiology of myelodysplasia?

A

2-3% recurrence in siblings in US.
Fetal alcohol syndrome, valproic acid during pregnancy.
Inadequate dietary levels of folic acid.
Folic acid additive to enriched grain products
recommended dietary supplement for all women of childbearing age

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7
Q

What is maternal screening looking for?

A

alpha-fetoprotein at 18 weeks

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8
Q

What are the cranial signs in utero?

A

Lemon sign

Banana sign

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9
Q

Lemon sign

A

overlapping frontal bones caused by enlarged ventricles

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10
Q

Banana sign:

A

abnormally shaped midbrain and elongated cerebellum associated with Arnold Chiari malformation

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11
Q

Hydrocephalus

A

Abnormal accumulation of cerebral spinal fluid, CSF in cranial vault
80% of cases of MM

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12
Q

What is the primary cause of hydrocephaly in MM?

A

Arnold Chiari (Chiari II)

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13
Q

Arnold Chiari malformation

A

cerebellar hypoplasia with caudal displacement of brainstem into foramen magnum

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14
Q

What are red flags of shunt formation?

A

headache, fever/malaise, changes in appetite, weight.
altered speech, decreased activity level or school performance.
visual acuity, strabismus or diploplia; decreased visual-perceptual coordination.
seizures; increased spasticity, static or declining grip strength.
personality changes, e.g., irritability, difficult arousal

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15
Q

Progressive Neurologic Dysfunction

Red flags

A

Gait deterioration or
loss of sensation or strength in extremities or trunk.
pain - repair site or radiating along dermatome.
onset or worsening spasticity.
development or rapid progression of scoliosis.
development unexplained lower limb deformity.
change in bowel or bladder sphincter control

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16
Q

What are causes of progressive neurologic dysfunction?

A

shunt obstruction, lipoma, subarachnoid cysts of the cord.

tethering of spinal cord – traction on neural structures

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17
Q

What is goal of PT with symmetric alignment?

A

minimize joint stresses and deforming forces, optimize muscle length for function

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18
Q

What postures should be avoided?

A

habitual ‘frog-leg’, W-sitting, and crouch standing should be avoided

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19
Q

Innervated muscle below the level of the lesion:

A

flaccid; spasticity(25%)

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20
Q

When does positioning begin?

A

at birth: taught to parents, daily LE ROM exercises
splinting
manual exercise

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21
Q

Overall goal of PT in MM?

A

prevent joint contractures, correct existing deformities, prevent or minimize effects of sensory and motor deficiency, and optimize mobility within natural environments

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22
Q

What should be avoided:

A

excessive force causing fractures.

excessive hip adduction risking dislocation

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23
Q

Measure hip extension in:

A

prone

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24
Q

Measure ankle ROM:

A

subtalar neutral

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25
Q

What contractures do full term infants have

A 
hip flexion (30 degrees)
knee flexion (10-20 degrees)
ankle dorsiflexion (40-50 degrees)
26
Q

Pattern movements:

A

hip flexion, knee flexion with contralateral hip extension

hip flexion and abduction with holding at end ROM to increase ROM

27
Q

What can ROM restrictions result in?

A

ADLs; bed mobility, transfers
poor postural habits, gait deviations
risk for skin breakdown

28
Q

What should MMT look for on initial assessment?

A

level of muscle innervation
baseline muscle function
imbalances of power around joint
prediction of future deformity aids decision making about orthotic

29
Q

How is motor level assigned?

A

according to last intact nerve root or the lowest level of antigravity movement through available ROM

30
Q

What does MMT grade 3 or better indicate?

A

muscle innervation

31
Q

What does strengthening in upper limbs do?

A

prepare for transfers, w/c propulsion, use of assistive mobility devices (AMDs).

32
Q

What does strengthening in the lower limbs do?

A

function within functional ROM, e.g., sufficient quad/HS strength (L4) may allow progress from KAFOs to AFOs

33
Q

When is the child at risk for loss of strength and endurance?

A

during rapid growth periods

post surgery

34
Q

Motor assessments:

A

Alberta Infant Motor Scale (AIMS).
Peabody Developmental Motor Scales (PDMS-2).
Gross Motor Function Measure (GMFM).

35
Q

What positions are static and dynamic balance observed?

A

sitting, four-point, kneeling, half-kneeling, standing, transitions

36
Q

Sitting in alignment:

A

hips and knees at 90o, feet resting on floor
symmetrical weight-bearing,
neutral pelvic tilt with slight lumbar lordosis,
inclining seat backward 15 degrees minimizes lumbar stress, keeps pelvis properly seated

37
Q

Typical postural problems

A

Forward head, rounded shoulders, kyphosis.
Excessive lordosis, anterior pelvic tilt.
Rotation deformities of hip or tibia.
Windswept hips, flexed hips/knees, pronated feet

38
Q

Pressure relief in children using wheelchair

A

wheelchair push-ups every 20-30 min.
Seat cushion design.
50% of day in w/c in 4-5 year-olds

39
Q

Pressure relief in ambulatory children

A

Inspect lower limbs, skin under orthoses.

Regular orthotic checks/adjustments during growth spurts

40
Q

What can impairments in proprioception and kinesthesia affect?

A

balance and standing, more reliance on visual and vestibular inputs rather than information from feet and ankles for postural adjustments

41
Q

What do children rely on with a loss of distal balance control

A

reliance on support from stable objects, adaptive equipment

42
Q

Secondary Impairments of MM

A

MSK deformities
joint contractures
muscle imbalance
upper limb deformities

43
Q

Spinal deformities are more common in:

A

higher level lesions

44
Q

What can severe kyphosis and scoliosis limit?

A

exercise tolerance, chest wall expansion
restrict lung ventilation
frequent infections

45
Q

Congential scoliosis:

A

vertebral anomalies

inflexible curve

46
Q

Acquired scoliosis:

A

muscle imbalance, flexible curve until skeletal maturity with little further progression

47
Q

Kyphosis

A

10-15% congential

1/3 acquired by adolescence

48
Q

Thoracic to L2 Deviations:

A

hip flexion, abduction, ER contractures; knee flexion and ankle plantar flexion contractures; lumbar lordosis

49
Q

Low lumbar (L3-L5) Deviations:

A

hip and knee flexion contractures, increased lumbar lordosis, genu and calcaneal valgus, pronated foot position (WB); pronounced crouch gait, WB on calcaneus

50
Q

Sacral level deviations:

A

mild hip/knee flexion contractures, increased lumbar lordosis; varus or valgus ankle/foot with pronated or supinated forefoot; mild crouch gait, calcaneal WB’ing with plantar flexor weakness

51
Q

Crouch standing:

A

persistent hip/knee flexion

increased lumbar lordosis

52
Q

What is weak in crouch standing?

A

soleus weakness insufficient to maintain vertical tibia

orthopedic deformities-calcaneal valgus

53
Q

Secondary impairments to crouch standing:

A

secondary hip/knee flexion contractures in response to adaptive muscle shortening
decreased torque-generating capacity

54
Q

Surgery candidate for hip subluxation/dislocation:

A

L3 or below with quad function.
unilateral dislocation may interfere with sitting or standing posture, contribute to scoliosis, affect skin care.
Painful hip dysplagia in ambulators

55
Q

What foot position is a priority?

A

plantigrade foot position in subtalar neutral

56
Q

What ankle and foot deformities are most common?

A

plantar flexor contracture

torsional deformities

57
Q

What level are ankle and foot deformities common?

A

L5 and above

58
Q

What deformity is seen in thoracic and high lumbar lesions?

A

ankle equinovarus

clubfoot

59
Q

What deformity is seen in sacral lesions?

A

Forefoot varus/valgus, supination or pronation, calcaneal varus/ valgus, pes cavus/ pes planus; claw-toe deformities

60
Q

What torsional deformities are seen:

A

excessive foot progression angle, windswept hips from femoral or tibial torsion

Pediatrics (13 decks)

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