Myelodysplasia Part 1 Flashcards
Myelomeningocele (MM)
open spinal defect, dorsally protruding sac containing meninges, spinal fluid, and neural elements
Myelodysplasia
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
Physical therapy for myelodysplasia
prevent secondary impairments contributing to functional limitations and participation restrictions
What is neurological damage, loss of muscle activity and sensation determined by?
vertebral level
Expected mobility and independence affected by pattern of innervation
Who is incidence highest in?
highest in Hispanic, lowest in black populations
Etiology of myelodysplasia?
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
What is maternal screening looking for?
alpha-fetoprotein at 18 weeks
What are the cranial signs in utero?
Lemon sign
Banana sign
Lemon sign
overlapping frontal bones caused by enlarged ventricles
Banana sign:
abnormally shaped midbrain and elongated cerebellum associated with Arnold Chiari malformation
Hydrocephalus
Abnormal accumulation of cerebral spinal fluid, CSF in cranial vault
80% of cases of MM
What is the primary cause of hydrocephaly in MM?
Arnold Chiari (Chiari II)
Arnold Chiari malformation
cerebellar hypoplasia with caudal displacement of brainstem into foramen magnum
What are red flags of shunt formation?
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
Progressive Neurologic Dysfunction
Red flags
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
What are causes of progressive neurologic dysfunction?
shunt obstruction, lipoma, subarachnoid cysts of the cord.
tethering of spinal cord – traction on neural structures
What is goal of PT with symmetric alignment?
minimize joint stresses and deforming forces, optimize muscle length for function
What postures should be avoided?
habitual ‘frog-leg’, W-sitting, and crouch standing should be avoided
Innervated muscle below the level of the lesion:
flaccid; spasticity(25%)
When does positioning begin?
at birth: taught to parents, daily LE ROM exercises
splinting
manual exercise
Overall goal of PT in MM?
prevent joint contractures, correct existing deformities, prevent or minimize effects of sensory and motor deficiency, and optimize mobility within natural environments
What should be avoided:
excessive force causing fractures.
excessive hip adduction risking dislocation
Measure hip extension in:
prone
Measure ankle ROM:
subtalar neutral
What contractures do full term infants have
hip flexion (30 degrees) knee flexion (10-20 degrees) ankle dorsiflexion (40-50 degrees)
Pattern movements:
hip flexion, knee flexion with contralateral hip extension
hip flexion and abduction with holding at end ROM to increase ROM
What can ROM restrictions result in?
ADLs; bed mobility, transfers
poor postural habits, gait deviations
risk for skin breakdown
What should MMT look for on initial assessment?
level of muscle innervation
baseline muscle function
imbalances of power around joint
prediction of future deformity aids decision making about orthotic
How is motor level assigned?
according to last intact nerve root or the lowest level of antigravity movement through available ROM
What does MMT grade 3 or better indicate?
muscle innervation
What does strengthening in upper limbs do?
prepare for transfers, w/c propulsion, use of assistive mobility devices (AMDs).
What does strengthening in the lower limbs do?
function within functional ROM, e.g., sufficient quad/HS strength (L4) may allow progress from KAFOs to AFOs
When is the child at risk for loss of strength and endurance?
during rapid growth periods
post surgery
Motor assessments:
Alberta Infant Motor Scale (AIMS).
Peabody Developmental Motor Scales (PDMS-2).
Gross Motor Function Measure (GMFM).
What positions are static and dynamic balance observed?
sitting, four-point, kneeling, half-kneeling, standing, transitions
Sitting in alignment:
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
Typical postural problems
Forward head, rounded shoulders, kyphosis.
Excessive lordosis, anterior pelvic tilt.
Rotation deformities of hip or tibia.
Windswept hips, flexed hips/knees, pronated feet
Pressure relief in children using wheelchair
wheelchair push-ups every 20-30 min.
Seat cushion design.
50% of day in w/c in 4-5 year-olds
Pressure relief in ambulatory children
Inspect lower limbs, skin under orthoses.
Regular orthotic checks/adjustments during growth spurts
What can impairments in proprioception and kinesthesia affect?
balance and standing, more reliance on visual and vestibular inputs rather than information from feet and ankles for postural adjustments
What do children rely on with a loss of distal balance control
reliance on support from stable objects, adaptive equipment
Secondary Impairments of MM
MSK deformities
joint contractures
muscle imbalance
upper limb deformities
Spinal deformities are more common in:
higher level lesions
What can severe kyphosis and scoliosis limit?
exercise tolerance, chest wall expansion
restrict lung ventilation
frequent infections
Congential scoliosis:
vertebral anomalies
inflexible curve
Acquired scoliosis:
muscle imbalance, flexible curve until skeletal maturity with little further progression
Kyphosis
10-15% congential
1/3 acquired by adolescence
Thoracic to L2 Deviations:
hip flexion, abduction, ER contractures; knee flexion and ankle plantar flexion contractures; lumbar lordosis
Low lumbar (L3-L5) Deviations:
hip and knee flexion contractures, increased lumbar lordosis, genu and calcaneal valgus, pronated foot position (WB); pronounced crouch gait, WB on calcaneus
Sacral level deviations:
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
Crouch standing:
persistent hip/knee flexion
increased lumbar lordosis
What is weak in crouch standing?
soleus weakness insufficient to maintain vertical tibia
orthopedic deformities-calcaneal valgus
Secondary impairments to crouch standing:
secondary hip/knee flexion contractures in response to adaptive muscle shortening
decreased torque-generating capacity
Surgery candidate for hip subluxation/dislocation:
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
What foot position is a priority?
plantigrade foot position in subtalar neutral
What ankle and foot deformities are most common?
plantar flexor contracture
torsional deformities
What level are ankle and foot deformities common?
L5 and above
What deformity is seen in thoracic and high lumbar lesions?
ankle equinovarus
clubfoot
What deformity is seen in sacral lesions?
Forefoot varus/valgus, supination or pronation, calcaneal varus/ valgus, pes cavus/ pes planus; claw-toe deformities
What torsional deformities are seen:
excessive foot progression angle, windswept hips from femoral or tibial torsion
