Myelodysplasia Flashcards
what is myelodyspasia
defective development of any part of the spinal cord
what part of the spinal cord is most affected by myelodyspasia
lower segments (thoracic into lumbar)
mechanism of myelodyspasia
congenital tube defect (NTD)
for myelodyspasia, what is the degree of resulting dysfunction related to
anatomic level of the defect
what accompanies myelodyspasia
loss of neurologic function (sensory and motor) below the level of the lesion
what does multifactorial mean
possible environmental and genetic factors affecting
what deficiency is present in myelodyspasia patients
folic acid
what type of SES is present in myelodyspasia
lower SES
what type of agents are present with myelodyspasia patients
teratogenic
what vitamin deficiency is present in myelodyspasia patients
Vitamin A
what factor is present in myelodyspasia patients
Rh factor
what might be ingested in the womb of myelodyspasia patients
alcohol
what is the genetic link of myelodyspasia patients who are prgenant
parents with one child with myelodyspasia have 50x higher chance of having another sibling
what is the normal process of development from the embryo
embryo (20 days after conception)
–>
neural groove
–>
neural crest
–>
closure of neural tube
when does the closure occur for the embryo
day 23
typically, the embryo is completely closed except…
the hole at the top and hole at the bottom
what does the hole at the top represent in the embryo
brain
what does the hole at the bottom represent in the embryo
spinal cord
where else might there be errors in development
vertebral architecture
incidence of neural tube defects
1-2 in 1000
are males or females more affected by neural tube defects
females
what are two methods of pre-natal detection of neural tube defects
- ultrasonic scanning
2. serum AFP testing
what birthing process should be planned for children with neural tube defects
c-section
why is a c-section common in neural tube defect patients
to avoid trauma to the neural sac
what is a method of post-natal diagnosis for neural tube defects
observation
what are the main types of myelodyspasia
occulta and aperta
what does occulta mean
hidden, not visible
mechanism of occulta
failure of one or more vertebral arches to meet and fuse in 3rd month of development
are spinal cord and meninges harmed in occulta
no.. they remain in the vertebral canal unharmed
what happens to the bony defect and skin in occulta
it is covered by skin
where is occulta most common
lumbosacral
any neurologic or musculoskeletal issues in occulta
no.. both systems are fine
what may be present in occulta patients (4)
- depression/dimple
- cafe au lait spot
- soft fatty deposit
- tuft of hair
another word for aperta
cystica
mechanism of aperta
neural tube and vertebral arches fail to close appropriately
what is present in aperta
cystic protrusion of material through defective arches
two types of aperta
- meningocele
2. myelomeningocele
what happens to the meninges and CSF in meningocele
protrusion of meninges and CSF into cystic sac
what happens to the spinal cord in meningocele
spinal cord remains within the vertebral column
can abnormalities be present in meningocele
yes but less common
mechanism of myelomeningocele
protrusion of both spinal cord and meninges into cystic sac
generic types of myelomeningocele
open and closed
how can closed myelomeningocele be described
covered with a combination of skin and membranes
where is closed myelomeningocele most common
thoracic and lumbosacral areas
what is an open myelomeningocele
nerve roots and spinal cord may be exposed with dura and skin at edge of lesion
where does 2/3 of open myelomeningocele occur
thoracolumbar junction
what imaging techniques can be used for myelomeningocele
MRI and CT
three other types of myelodysplasia
- lipoma
- myelocystocele
- anencephaly
what is lipoma
fatty tumor on spinal cord
what is myelocystocele
cystic like tumor of spinal cord
what is anencephaly
failure of closure of cranial end of neural tube
what may be evident and absent in anencephaly
some brain tissue evident, forebrain usually absent
does occulta usually cause neurologic dysfcuntion
no, usually no neurologic symptoms
what are symptoms for occulta
- occasional disturbances in bowel and bladder function
- foot weakness
where is occulta most common
lumbosacral area
what is motor function like in myelomeningocele
myotomal loss
what is sensory function like in myelomeningocele
dermatomal loss
what type of deformity may be present in myelomeningocele
skeletal deformity
what do 90% of myelomeningocele patients develop
hyrdocephalus
do alot of myelomeningocele patients have MR
no, low percentage
do alot of myelomeningocele have a learning disability
yes, high percentage
motor function of myelomeningocele patients
depends on level of lesion
what type of patients do myelomeningocele patients have similar symptoms as
spinal cord injury
when does post-natal closure of myelodysplasia have to occur
within 72 hours
what is the surgical procedure of closure of myelodysplasia patients
place neural tissue into vertebral canal
what is the goal of the thecal sac in surgical repair of myelodysplasia patients
achieve a flat, watertight closure of thecal sac
percentage of myelodysplasia of recover after surgery and do not have further medical complications
10%
what do 90% of myelodysplasia patients after surgery develop
hydrocephalus
what else can myelodysplasia develop after surgery
Tethered cord and symptoms of Arnold Chiari
what is hydrocephalus
abnormal accumulation of CSF in cranial vault
what is hydrocephalus caused by (3)
- overproduction of CSF
- failure of absorption of CSF
- obstruction in normal flow of CSF through brain and spinal cord
what does accumulation of CSF (hydrocephalus) cause
increased ICP
what can hydrocephalus lead to
cerebral damage and cellular death
what may be present with someone with hydropcephalus regarding their head
full, bulging, tense soft spot (fontanel) on top of the head
what may be noted on the scalp of someone with hydropcephalus
large prominent veins on the scalp
what sign might be present in someone with hydropcephalus
setting sun sign
what is the setting sun sign
- child appears to only look down
- the whites of the eyes are obvious above the colored portion of the eyes
what changes may be noted in someone with hydropcephalus
behavioral changes (irritability, lethargy)
what type of cry might be noted with someone with hydrocephalus
high pitched cry
what medical condition might be noted in someone with hydropcephalus
seizures
what may be noted in appetite regarding someone with hydrocephalus
vomiting or change in appetite
what can be done medically done for someone with hydrocephalus
ventriculoperitoneal shunt
what is a ventriculoperitoneal shunt
thin tube with a small pump attached which diverts CSF from lateral ventricles to a location where the fluid can be managed
what is the most common type of shunt
ventriculoperitoneal
what is an alternate to ventriculoperitoneal shunt
ventriculo-atrial shunt
QUIZ MATERIAL ENDS HERE
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for shunt malfunction, what may the fontanels feel like
firm fontanels
what is an arnold-chiari malformation
defect in formation of the brainstem
mechanism of arnold-chiari malformation
hindbrain is displaced through the foramen magnum
frequency of arnold-chairi malformation contributes to…
development of hydrocephalus
arnold-chiari: ___ dysfunction and ____ involvement
hindbrain dysfunction and cranial nerve involvement
for arnold-chiari malformations, how is feeding
difficult
for arnold-chiari malformations, there is pooling of…
secretions
for arnold-chiari malformations, there is ____ breathing
stridor
what is stridor breathing
heavy breathing
is there scoliosis for tethered cord?
yes
what happens to spasticity for tethered cord
increased
describe the postures and movements for tethered cord
asymetrical
is the gait pattern altered for tethered cord
yup
where is there pain present for tethered cord
back
