Myelodysplasia Flashcards
types of meylodysplasia
- myelodysplasia
- spina bifida
- myelomeningocele
- meningeocele
- diastematomyelia
- lipoma
myleodysplasia
- defective development of any part of the spinal cord
spina bifida
- most commonly used to describe various forms of defects of neural tube closure
- aperta-visual or open lesion
- occulta-hidden or not visible lesion
myelomeningocele
- external protrusion of the meninges and spinal cord. not covered by skin
- associated with spinal nerve paralysis
meningeocele
- external protrusion of meninges
diastematomyelia
- fibrous, cartilaginous or bony band separating the spinal cord into hemicords each surrounded by a dural sac
lipoma
- occulta, but usually visible subcutaneous fat masses
- classified by location
spina bifida pathoembryology
- 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
Myelomeningocele: etiology
- teratogens: maternal alcohol consumpyion, valprouc acid, street drug abusers
- nutritional deficiencies: inadequate folic acid, begin supplements at least 3 months before conception
Perinatal management
- 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
musculoskeletal deformities
- 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
deformities at thoracic -L2
- hip flexion, abduction, and ER contractures
- knee flexion contrcatures
- ankle PF contrcatures
- lordotic lumbar spine
deformities at L3-L5
- 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
deformities sacral level
- mild hip and knee flexion contractures
- increased lumbar lordosis
- ankle and foot in varus and valgus with pronated or supinated foot
- mild crouched gait
deformities crouched standing
- persistent hip and knee flexion and increased lumbar lordosis
deformities scoliosis
- congenital or acquired
impairments osteoporosis
- decreased bone mineral density
- walking ability highly correlated to increased bone mineral density
-fractures may not be immediately apparent due to poor sensation
impairment motor paralysis
- motor level- lowest intact, functional neuromuscular segment
- may be asymmetric
impairments sensory deficits
- 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
hydrocephalus
- 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
early sign of shunt dysfucntion
-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…
cognitive dysfunction with hydrocephalus
-without or with uncomplicated hydrocephalus, intelligence is typically avg
cranial nerve palsies
- may result from Chiari malformation, hydrocephalus or dysplasia of the brains stem
neurogenic bowel
- <5% of children with myelomeningocele have control of urinary or anal sphincter
outcomes T10 or above
- strong UE and upper thoracic and aneck motions
- weak lower trunk
- difficulty with sitting and possible respiration
outcomes for T12
- 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
- high lumbar (L1-2) outcomes
- 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
L3 level outcome
- strong hip flexion and weak rotation
- some knee extension
- KAFOs and crutche for household ambulation in childhood
- wheel chair for community
- ~60% live independently
L4 level outcome
- calcaneal deformities common
- strong knee extension
- functional ambulators with AFOs and crutches
- focus on maintaining ankle joint alignment
L5level outcome
- 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
S1 level outcome
- gastroc/soleus grade 2
- gluteus medius grade 3
- gluteus max grade 2
- ambulate w/o orthotics or support
S2,S3 and “no loss” levels
- 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
Intervention strategies
– intervention approaches for developmental delay in myelomeningocele
- 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
examination and intervention strategies for strength
- 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
examination and intervention strategies for mobility
- provide options that allow for exploration of environment
- changes in body proportions can significantly alter mobility
- measures energy expenditure via HR
examination and intervention strategies for gait
- 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
