Myelodysplasia

Question 1

types of meylodysplasia

Answer 1

• myelodysplasia
• spina bifida
• myelomeningocele
• meningeocele
• diastematomyelia
• lipoma

Question 2

myleodysplasia

Answer 2

• defective development of any part of the spinal cord

Question 3

spina bifida

Answer 3

• most commonly used to describe various forms of defects of neural tube closure
• aperta-visual or open lesion
• occulta-hidden or not visible lesion

Question 4

myelomeningocele

Answer 4

• external protrusion of the meninges and spinal cord. not covered by skin
• associated with spinal nerve paralysis

Question 5

meningeocele

Answer 5

• external protrusion of meninges

Question 6

diastematomyelia

Answer 6

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

Question 7

lipoma

Answer 7

• occulta, but usually visible subcutaneous fat masses

- classified by location

Question 8

spina bifida pathoembryology

Answer 8

• the upper end normally closes on the 25th day
• the bottom on the 7-28th day to form the SC

-the neural tube fails to develop or close properly, resulting in spina bifida

Question 9

Myelomeningocele: etiology

Answer 9

• teratogens: maternal alcohol consumpyion, valprouc acid, street drug abusers
• nutritional deficiencies: inadequate folic acid, begin supplements at least 3 months before conception

Question 10

Perinatal management

Answer 10

• Dx made by maternal alpha-fetoprotein screening, US, or amniotic fluid analysis
• prenatal dx allows for repairs in utero. fetal surgery can be performed in some conditions
• post natal closure of the tube requires surgery within 48 hours to avoid infection, and during of the nerve roots

Question 11

musculoskeletal deformities

Answer 11

• spinal and LE limb deformities and contractures
• restrictions in UE ROM due to overuse for WB and poor posture
• typical postural deficits: forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, anterior pelvic tilt, rotational deformities of hip or tibia, flexed hips and knees and pronated feet
• take caution to avoid habitual positions

Question 12

deformities at thoracic -L2

Answer 12

• hip flexion, abduction, and ER contractures
• knee flexion contrcatures
• ankle PF contrcatures
• lordotic lumbar spine

Question 13

deformities at L3-L5

Answer 13

• hip and knee flexion contractures
• increased lumbar lordosis
• genu and calcaneal valgus misalignment
• pronated feet
• often walk in crouched gait and bear weight through calcaneus

Question 14

deformities sacral level

Answer 14

• mild hip and knee flexion contractures
• increased lumbar lordosis
• ankle and foot in varus and valgus with pronated or supinated foot
• mild crouched gait

Question 15

deformities crouched standing

Answer 15

• persistent hip and knee flexion and increased lumbar lordosis

Question 16

deformities scoliosis

Answer 16

• congenital or acquired

Question 17

impairments osteoporosis

Answer 17

• decreased bone mineral density
• walking ability highly correlated to increased bone mineral density

-fractures may not be immediately apparent due to poor sensation

Question 18

impairment motor paralysis

Answer 18

• motor level- lowest intact, functional neuromuscular segment
• may be asymmetric

Question 19

impairments sensory deficits

Answer 19

• do not always correlated to motor levels. may skip areas
• evaluate vibration, light touch and pinprick
• patients to be educated on safety, skin inspection and pressure relief

Question 20

hydrocephalus

Answer 20

• present at birth in 25% or more of children with myelomeningocele
• additional 60% develop it after surgical closure of lesion.
• 80-90% requires a CSF shunt

Question 21

early sign of shunt dysfucntion

Answer 21

-changes in speech
-fever and malaise
recurring headache
-decr activity level
-decr school performance
- onset or worsening of scoliosis
-decreased visual acuity
-onset or increased freq of seizures
-etc…

Question 22

cognitive dysfunction with hydrocephalus

Answer 22

-without or with uncomplicated hydrocephalus, intelligence is typically avg

Question 23

cranial nerve palsies

Answer 23

• may result from Chiari malformation, hydrocephalus or dysplasia of the brains stem

Question 24

neurogenic bowel

Answer 24

• <5% of children with myelomeningocele have control of urinary or anal sphincter

Question 25

outcomes T10 or above

Answer 25

• strong UE and upper thoracic and aneck motions
• weak lower trunk
• difficulty with sitting and possible respiration

Question 26

outcomes for T12

Answer 26

• strong trunk and sitting balance
• may have weak hip hike from quadratus lumborum
• wheel chair for mobility in home and community
• tend to have greater CNS involvement
• supervision required in living situation
• may work in sheltered settings or volunteer work

Question 27

• high lumbar (L1-2) outcomes

Answer 27

• L1- weak hip flexion
• L2- hip flexors, adductors and rotators are grade 3 or more
• frequent hip dislocation due to unopposed hip flexion and adduction
• household ambulation for small children possible. with support and orthotics
• wheel chair for community
• ~50% live independently

Question 28

L3 level outcome

Answer 28

• strong hip flexion and weak rotation
• some knee extension
• KAFOs and crutche for household ambulation in childhood
• wheel chair for community
• ~60% live independently

Question 29

L4 level outcome

Answer 29

• calcaneal deformities common
• strong knee extension
• functional ambulators with AFOs and crutches
• focus on maintaining ankle joint alignment

Question 30

L5level outcome

Answer 30

• lateral hamstrings grade 3 or more and neither. grade 2 glute min, grade 3 post tib, grade 4 peroneus tertius
• antigravity knee flexion and weak hip extension
• require orthoses for alignment
• bilateral UE support recommended

Question 31

S1 level outcome

Answer 31

• gastroc/soleus grade 2
• gluteus medius grade 3
• gluteus max grade 2
• ambulate w/o orthotics or support

Question 32

S2,S3 and “no loss” levels

Answer 32

• S2 decreased push off and stride length with running
• S2-3 most LE muscle have grade 5, few with 4
• “no loss” - normal bowel and bladder function, normal strength

Question 33

Intervention strategies

– intervention approaches for developmental delay in myelomeningocele

Answer 33

• children encouraged by parent/teacher/therapist with “high dose” of normal developmental activities in “at risk” areas
• remediation- repetition of a graded task in area of concern
• teaching compensatory skills- increasing independence

Question 34

examination and intervention strategies for strength

Answer 34

• dynamometer of grip and pinch
• strengthening indicated if: function is present. weakness in muscle groups important for postural stability , ADL, mobility or balance of muscle forces around joint
• strengthen within functional ROM

Question 35

examination and intervention strategies for mobility

Answer 35

• provide options that allow for exploration of environment
• changes in body proportions can significantly alter mobility
• measures energy expenditure via HR

Question 36

examination and intervention strategies for gait

Answer 36

• expect delays in achieving ambulation
• gait anlysis to monitor function: deviations may be noted early
• indications for gait training: Orthoses, gait pattern, safety, efficiency
• equipment