Myelomeningocele / Spina Bifida Flashcards
Development of Nervous System
- neural plate
- neural tube
- brain and SC
Neural tube closure is completed by
day 26
defect in neural tube closure and overlying posterior vertebral arches
spina bifida
potential cause of spina bifida
genetic predisposition that may enhanced factors
Genetic RF for spina bifida
- celtic origin
2. family hx spina bifida
Spina bifida:
environmental factors
- lower socioeconomic status
2. folic acid and Vit A deficiency
Spina Bifida Prenatal testing
- AFP abnormaly high in amniotic fluid past 14 weeks gestational age
- compare w/ ultrasound results
Spinda Bifida:
Occulta
- failure of vertebral arches to meet and fuse in 3rd month
- overlying skin dimple, pigmentation, or patch of hair
- lumbosacral area common site
- usually non neuro or msk dysfunction
- higher incidence of urinary tract disorder
Spinda Bifida:
Cystica
Failure of neural tube and vertebral arches to close w/cystic protrusion of meninges with or w/out spinal cord
A. Meningocele
B. Myelomeningocele
Meningocele
- spinal cord remains in vertebral canal but may be abnormal
- lipomeninggocele
Lipomeninggocele
superficial fatty mass in lower lumbar/sacral area. high incidence of B&B dysfunction, tethered cord
Myelomeningocele
- extensive SC abnormalities
- most common in thoracic and lumbosacral regions
- anencephaly
Anencephaly
cranial end of neural tube doesn’t fuse
Myelomeningocele: Clinical manifestation (9)
- loss of sensory/motor function
- orthopedic abnormalities
- hydrocephalus
- brain/SC abnormalities
- B&B dysfunction
- Cognitive impairment/Learning issues
- Integumentary impairment
- disturbed growth and development
- psychosocial issues
Myelomeningocele:
Orthopedic abnormalities due to…
lack of sensorimotor function in utero or lack of movement.
Myelomeningocele:
Loss of sensory and motor function dependent on:
- degree of SC abnormality
- Traction or stretch on SC
- Trauma to exposed neural tissue during delivery
- Postnatal damage resulting from dying/infection of neural plate
Myelomeningocele:
Sensory Impairment
- Sensation below level of lesion impaired may not exactly match level (incomplete lesion)
- includes kinesthetic, proprioceptive, somatosensory
Myelomeningocele:
2 main types of motor dysfunction
- complete loss of function below level of lesion
2. Mixture of flaccidity and spasticity
Myelomeningocele:
Orthopedic problems
- deformity
- contractures
- osteoporosis
Myelomeningocele:
deformity and contractures due to:
- muscle imbalance
- stress
- posture
- gravity
- congenital
- decreased sensation
- neuro complications
Myelomeningocele:
typical congenital malformations
- hemivertebrae
- deformed vertebral bodies/ribs
- missing ribs
- lumbar kyphosis
- club feet
- rocker bottom feet
Myelomeningocele:
Osteoporosis
secondary to lack of WB
Myelomeningocele:
Hydrocephalus
- present in 80-90% of cases
- blockage of normal CSF flow
Hydrocephalus signs
- increased head size
- bulging fontanelles
- irritability
- lethargic
- sunset eyes
Hydrocephalus interventions
- Ventroatrial shunt
2. Ventroperitoneal shunt
Signs of shunt malfunction:
Infants
- bulging fontanelle
- vomiting
- change in appetite
- “sunset” sign (eyes)
- edema/rednes
- thinning of skin
- high pitched cry
- irritability
- lethargy
- seizures
- excessive growth
Signs of shunt malfunction:
Toddlers
- vomiting
- irritability
- seizures
- headaches
- redness/edema
- lethargy
- new nystagmus/squint
Signs of shunt malfunction:
school aged children
- vomiting
- irritability
- seizures
- headaches
- redness/edema
- lethargy
- new nystagmus/squint
- memory changes
- handwriting changes
arnold chiari malformation
brainstem and cerebellum herniate through foramen magnum, usually into upper cervical canal
Arnold Chiari Malformation:
Symptoms
- respiration issues
- paralysis of vocal cords
- bulbar dysfunction
- apnea
- swallowing issues
- abnormal gag
- UE weakness
Hydromyelia
dilation of center of canal of SC. May be focal, multiple, or diffuse
Hydromyelia:
cause
consequence of untreated/inadequately treated hydrocephalus
Hydromyelia:
symptoms
- UE muscle weakness
- rapidly progressive scoliosis
- spasticity
- ascending motor loss in LE
Hydromyelia:
interventions
- shunt revision
- posterior cervical decompression
- flushing device (into pleural cavity)
Tethered Cord
Pathological fixation of SC in abnormal caudal location
causes mechanical stretch, distortion, and ischemia
Tethered cord symptoms
- decreased strength
- LE spasticity
- back p! at site of sac closure
- change in urological function
Tethered cord intervention
release
Myelomeningocele:
Cog / learning issues
- visual motor perceptual deficits
- difficulties w/spatial relations
- difficult development of hand dominance
- poor hand function
- “cocktail party speech”
Myelomeningocele:
Integumentary
- latex allergy
2. pressure sores
Myelomeningocele:
Bowel and Bladder maintenance
self cath taught early if possible
Myelomeningocele:
PT intervention
age appropriate assessment / interventions for:
- ROM / MMT
- sensory testing
- developmental testing
- positioning
- equipment needs
- education for family
- balance
- coordination
- reflexes
- address tone and skin condition
Myelomeningocele:
Age specific issues- Prenatal
- referral to EI
- possible sac closure in utero
- C section to prevent trauma to neural sac
Myelomeningocele:
Age specific issues- Newborn
- sac closure w/in 24-48 hrs
- shunt procedure
- assess function to determine baseline pre/post sx
- ID muscle imbalances/deforming forces
- ID need for positioning, bracing, taping
Myelomeningocele:
Age specific issues- Newborn MMT
observation
- state
- positions
- spontaneous movement in response to sensory input
- differentiate between reflex and voluntary movement
Myelomeningocele:
Age specific issues- infant/toddler
- facilitate developmental sequence
- prevent deformity
- home program
- early WB (watch alignment)
- skin care
- emphasize balance and equilibrium in standing/sitting/core str
- importance of fine motor skills
Myelomeningocele:
Age specific issues- Toddler/preschool
- emphasize progressive LE WB / gait training
- EI and school based programs
- adaptive equipment
- bladder training
Myelomeningocele:
MMT for toddler-preschool
antigravity control in developmental positions, during play, and weighted toys
Myelomeningocele:
Sensory for toddler-preschool
start assessing light touch and position changes as cognitive and language skills permit
Myelomeningocele:
Age specific issues- 6-adolescent
- by 6, aware of their differences
- constantly reevaluate due to rate of growth
- independent community mobility: WC
- prepare family for changes in adolescence.
Myelomeningocele:
thoracic level
- flaccid LE: frog leg position
- prone positioning, ROM, gentle wrapping
- surgical intervention
- total contact orthosis for standing
- resting AFO to maintain foot position
Myelomeningocele:
surgical intervention
IT band, hip ER, knee flexors
Myelomeningocele:
walking w/parapodium
learn to use head and upper trunk to shift weight
Myelomeningocele:
T12-L3
- strong hip flexors/ADD
- require bracing to stand
- hip subluxation and dislocation common
- parapodium/ swivel walker/ A frame
- RGO, HKAFO
Myelomeningocele:
L4-L5
- strong hip FLX/ADD
- some hip ABD
- strong quads, medial hamstrings
- anterior tib active, some foot intrinsics
- Use of floor reactive AFO
Myelomeningocele:
L4-L5 AD for rotation
Twister Cables
Myelomeningocele:
L4-5 AD for weak quads
KAFO
Myelomeningocele:
sacral level
- full or partial innervation of hip ext, lat hams, glut med, med hams, quads
- variation of innervation in foot/ankle
- gastroc may be weak
- watch for crouched gait w/weight gain
- aggressive trx for hip instability
Myelomeningocele:
Sacral. What to watch for w/weak gastroc
toe flexors may provide some plantar flexion, but not strong enough for gait.
AFO needed
Pschosocial Adjustment:
0-6 months
parents may be in shock/denial.
may misinterpret or reject info
Pschosocial Adjustment:
6-18 months
- stressful
- many appointments/hospitalizations
Pschosocial Adjustment:
2 years-school age
concerns about toilet training and social acceptability
Pschosocial Adjustment:
6 y.o.
- child more aware of disability
- family attitudes/ environment influence child’s adjustment
- important to have interaction w/peers and age appropriate responsibilites
Pschosocial Adjustment:
adolescence
- B&B management
- sexual issues
- females- potential for orgasm/fertility
- men usually sterile w/small testicles and penis. potential for erection/ejaculation depending on lesion
