Myelodysplasia Flashcards
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 meninges and spinal cord
Not covered by skin
Meningeocele
External protrusion of meninges
Diastematomyelia
Fibrous, cartilaginous or bony band separating the spinal cord into hemicords each surrounded by dural sac
Lipoma
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
Upper end of neural tube usually closed on
25th day
Bottom of the neural tube usually closed
27/28 day
Spina bifida is the result of
The neural tube failing to develop or close properly
Myelomeningocele: genetics
Recurrence 2-3% risk in siblings
Associated w/ “syndromes”
High variability in incidence among various ethnic groups
Myelomeningocele: teratogens
-maternal alcohol consumption
Valproic acid
Street drug abusers
Myelomeningocele: nutritional deficiencies
-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
Internationally: % of pregnancies with prenatal dx of neural tube deficits are terminated
23%
Dx usually occurs at 18 weeks
Myelomeningocele DX:
Maternal alpha-fetoprotein screenings, US, amniotic fluid analysis
-lemon sign, banana sign
Myelomeningocele: perinatal management
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
Myelomeningocele musculoskeletal deformities
Spinal and LE limb deformations and contractures
Restriction in UE ROM due to overuse for WB and poor posture
Myelomeningocele typical postural deficits
Forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, ant pelvic tilt, rotational deformities of hip or tibia, flexed hips and knees, pronated feet
Myelomeningocele: thoracic to L2
Hip flex, AB, ER contracture
Knee flex contract
PF contract
Lordotic lumbar spine
Myelomeningocele: L3-L5
Hip and knee flex contracture Increased lumbar lordosis Genu and calc valgus Pronated feet Often walk in crouched gait and bear weight through calcaneous
Myelomeningocele sacral level
Mild hip and knee flexion contracture
Increased lumbar lordosis
Foot in varus or valgus w/ pronated and supinated foot
Mild crouching gait
Myelomeningocele osteoporosis
Decrease bone mineral density
- walking ability highly correlated to increased bone mineral density
- fax may not be immediately apparent due to poor sensation
Myelomeningocele motor paralysis
Motor level - lowest intact functional NM seg
May be asymmetric
Myelomeningocele sensory deficit
Don’t always correlated to motor levels
Eval
Pt ed
Myelomeningocele hydrocephalus
At birth 25% or more
Additional 60% develop after sx closure of lesion
80-90% require CSF shunt
Myelomeningocele cog function
W/ or w/out uncomplicated hydrocephalus, intellegence typically average
Myelomeningocele latex allergy
73%
Myelomeningocele seizures
10-30%
Myelomeningocele skin breakdown
Decubitus ulcer and other breakdown occur in 85-95%
Myelomeningocele cranial nerve palsy
May result from chiari malformation, hydrophealus, dysphasia of brain system
Myelomeningocele neurogenic bowel
<5% have control of urinary or anal sphincter
Myelomeningocele neurogenic bladder
Early catheterization will help avoid overstretching bladder wall
Can learn to self cath by 6-8yo
Myelomeningocele exam
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
Myelomeningocele common goals across life span
Prevent joint contracture
Correct existing deformities
Prevent/min effect of sensory and motor deficiency
Optimize mobility w/in natural enviro
Myelomeningocele outcome T10 above
Strong UE, upper TSpine, neck motions
Weak lower trunk, diff w/ sitting and resp
Myelomeningocele outcomes T12
Strong trunk and sitting balance
May have weak hip hike form QL
Myelomeningocele outcomes: general thoracic
W/c
Tend to have greater CNS involve- cog impairment
Supervision required in living situation
May work in sheltered settings or volunteer work
Myelomeningocele L1-L2
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)
Myelomeningocele L3
Strong hip flex, weak rotation Some knee ext KAFO and crutches for household in childhood W/c for community 60% (I)
Myelomeningocele L4
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
Myelomeningocele L5
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
Myelomeningocele S1
Gast/sol 2
Glut med 3
Glut max 2
Amb w/out orthotics for support
Myelomeningocele S2, S2-3, and “no loss”
S2- decreased push off and stride length w/ running
S2-3 more LE have 5, few 4
“No loss” normal BB function, normal strength
Myelomeningocele intervention
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
Myelomeningocele strengthening indicated if
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
Myelomeningocele mobility: measure energy expenditure via
HR
Myelomeningocele indications for gait training
Potential for progression to new orthosis or AD
Potential for improving pattern
Improve safety and confidence
Efficiency of gait and transfer
Myelomeningocele: key muscle groups for amb
Iliopsoas Glute med and max Quad Ant tib Hamstring
