spina bifida

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spina bifida
2012-03-20 10:53:30
spina bifida

spina bifida
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  1. spina bifida
    defective embryonic neural tube closure during the 1st trimester of pregnancy results in various malformations of the spine.

    generally the defect occurs in the lumbosacral area but maybe found in the sacral, thorcic, and cervical areas
  2. 3 types of spina bifida
    spina bifida occulta

    spina bifida w/meningocele

    spina bifida w/ myelomeningocele
  3. spina bifida occulta
    most common/least severe spinal cord defect

    characterized by incomplete closure of one or more vertebrae w/o protrusion of the spinal cord or meninges
  4. spina bifida meningocele
    incomplete closure of one or more vertebrae causes protrusion of the meninges of CSF in an external sac or cystic lesion
  5. spina befida myelomeninglocele

    incomplete closure of the vertebrae causes protrusion of the meninges, CSF, and a portion of the spinal cord or nerve roots in an external sac or cystic lesion.
  6. prognosis
    varies w/ degree of accompanying neurologic deficit

    myelomeninocele-least favorable prognosis

    large open lesion, neurogenic bladders, increased infection, renal failure
  7. spina bifida occulta
    prognosis better

    many people may lead normal life w/ no complication
  8. causes and incidence
    normally-20 days after conception the embroyo develops a nerual groove in the dorsal ectoderm

    the groove deepens, edges fuse & become the neural tube

    by day 23, the tube is completely closed except for an opening at each end
  9. theoretically,
    if the posterior portion of the neural tube fails to close by the 4th week of gestation, or if it closes but then splits open from a cause such as an abnormal increase in CSF later in the first trimester

    a spinal defect results
  10. signs/symptoms
    spina bifida occulta
    • depression or dimple
    • tufts of hair
    • soft fatty deposits
    • port wine nevi

    any combination of these abnormalities on the skin cover the defect

    all signs may be absent

    usually does not cause neurological dysfunction

    occasionally associated with foot weakness and bowel and bladder disturbances
  11. sign/symptoms
    structure protrudes over spine

    rarely caused neurological deficts

    sac contains CSF and meninges
  12. signs/symptoms
    saclike structure over spine

    neurological deficits related to the level of the defect

    permanent neurological dysfunction (flaccid/spastic paralysis, bowel/bladder incontinence, etc)
  13. associated disorders
    tropic skin disturbances (ulcerations, cyanosis)

    mental retardation


    knee contractures

    hydrocephalus (in about 90%)
  14. diagnosis
    spina bifida occulta
    often overlooked

    x-rays can show the bone defect
  15. diagnosis
    pin prick examination of legs and trunk (sensory evaluation)

    need to identify sensory and motor levels

    skull x-rays, head measurements, CAT scan (hydrocephalus)


    can only detect open defects (myelomeningocele and meningocele)

    alpha-fetoprotein test-done at 14 weeks gestation-may indicate defects in the spinal cord (neural tube oopening)
  16. treatment
    spina bifida occulta
    usually requires no treatment

    if neuromuscular problems occur with growth, surgery may be required
  17. treatment
    surgical closure of the protruding sac

    continual assessment of growth and development
  18. treatment
    surgical repair of the sac-cover the flap

    supportive measures to promote independence and prevent further complicatons (therapies, adaptive equipment )

    parent/child bonding an issue

    surgery cannot reverse neurological deficits

    usually a shunt is necessary to relieve associated hydrocephalus rehabilitation

    orthopedic appliances (crutches, braces, walkers, wheelchairs)

    adaptive equipment

    diet and bowel training

    manage incontinence or colostomy

    neurologenic bladder-catheter, crede(pressure on the bladder to go to the bathroom)
  19. special considerations
    care of the child with severe spinal defects requires a team approach:

    • neurosurgeon
    • orthopedist
    • urologist
    • nurse
    • social worker
    • OT
    • PT
    • parents
    • teachers,etc
  20. immediate goals
    psychological support to help parents accept the diagnosis and the child

    positioning and handling

    sensory stimulation

    promote bonding
  21. long term goals

    working with the child and family

    prevent complications

    contractures-pressure ROM

    decubiti ulcers
  22. before surgery
    handle the infant carefully

    do not apply pressure to the defect
  23. contracture
    can be minimized by PROM exercises
  24. to prevent hip dislocation
    moderately abduct hips with a pad between knees
  25. work with parents
    to help them cope with the infant's physical problems and to successfully reach the long-term goals