Myelodysplasia Flashcards
Pathophysiology
degree of dysfunction depends on the anatomical level of lesion
motor and sensory loss below level of lesion
Etiology
deficient folic acid within first 6 weeks of pregnancy lower SES teratogenic agent hyperthermia during pregnancy rh factor vitamin A deficiency alcohol usage during pregnancy genetic (if had one child with it, 50% likely)
Diagnosing
Prenatally:
Ultrasound
AFP
if found prenatally, prep for c-section to avoid further damage
Postnatally:
via observation except occulta
Myelodysplasia occulta
failure of at least 1 or more vertebral arches failing to close within the first 3 mth of development
usually content stays within vertebral canal=not NM/MS symptoms
midline area, covered by skin
most commonly lumbosacral region
tuft of hair, cafe au lait spot, or dimple
Myelodysplasia asperta cystic:
failure of neural tube and neural arche to close
Meningiocele
protrusion of meninges and CSF
SC stays within vertebral column
Myelomeningiocele
protrusion of meninges/CSF/ SC
closed: skin membrane covered
commonly in the T-L or L-S junction
open: nerve roots, cord, and dura mater is apparent
2/3 in the T-L junction
Other myelodysplasia
Lipoda- fatty tumor on SC
Myelocystocele- cystic tumor on SC
anencephaly- no forebrain development, no sustainable life
Clin pic of meningiocele
usually no NM/MS signs
occasional bowel/bladder dysfunction
possible foot weakness due to location of L-S area
Clin pic of myelomeningiocele
motor loss (myotomal) sensory loss (dermatomal) 90% present with hydrocephalus skeletal deformity
MR- low percentage
LD- higher percentage
presents as SCI
Management of myelomeningiocele
prenatally: surgical= 90% hydrocephalus complication, 10% may d/c without complications
postnatally: closure within 72 hours, replace the neural contents within the vertebral canal, repair the skeletal deformity, achieve a water tight thecal sac
Hydrocephalus etiology/pathophy
overproduction of CSF
lack of absorption of CSF
obstruction between the brainstem and SC
leads to increased ICP= cerebral cellular damage
Hydrocephalus signs and symptoms
buldging of the fontanel large vein on top of head lethargy/irritability seizures high pitch cry change in appetite/vomiting
Management of Hydrocephalus
ventriculoperitoneal shunt
ventriculoatrial shunt
Shunt malformation signs/symptoms
firmness of fontanels
change in appetite/vomitting
increased head circumference size
seizures
trunk swelling
bowel/bladder dysfunction
sensory/motor/school performance decrease
blurred vision and headache
