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Spina Bifida

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Spina bifida is one of the more common birth defects in the United States. ... This form of spina bifida rarely causes disability or symptoms. ... – PowerPoint PPT presentation

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Title: Spina Bifida


1
Spina Bifida
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Case-basic data
  • Name???
  • Age 34 y/o
  • G2P1( twins)
  • Previous pregnant history
  • Pregnancy 34 weeks with twins s/p C/S
  • 2000g and 1700 g
  • No other systemic disease.

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Current condition
  • Pregnancy 354 weeks with twins
  • Transfer from VGHTC
  • One fetusspina bifida

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Spina Bifida
  • The human nervous system develops from a small,
    specialized plate of cells along the back of an
    embryo.
  • Early in development, the edges of this plate
    begin to curl up toward each other, creating the
    neural tubea narrow sheath that closes to form
    the brain and spinal cord of the embryo.
  • the top of the tube becomes the brain and the
    remainder becomes the spinal cord.

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  • This process is usually complete by the 28th day
    of pregnancy.
  • But if problems occur during this process, the
    result can be brain disorders called neural tube
    defects, including spina bifida.

16
What is spina bifida?
  • Spina bifida, which literally means cleft
    spine, is characterized by the incomplete
    development of the brain, spinal cord, and/or
    meninges.
  • Spina bifida is one of the more common birth
    defects in the United States.
  • It is among the most common severe birth defects
    in the United States, affecting 1,500 to 2,000
    babies (one in every 2,000 live births) each
    year.

17
the different types of spina bifida
  • Occulta is the mildest and most common form in
    which one or more vertebrae are malformed.
  • The name occulta, which means hidden,
    indicates that the malformation, or opening in
    the spine, is covered by a layer of skin. 
  • This form of spina bifida rarely causes
    disability or symptoms. 

18
  • Closed neural tube defects make up the second
    type of spina bifida.
  • This form consists of a diverse group of spinal
    defects in which the spinal cord is marked by a
    malformation of fat, bone, or membranes.
  • In some patients there are few or no symptoms in
    others the malformation causes incomplete
    paralysis with urinary and bowel dysfunction.

19
  • meningocele the meninges protrude from the
    spinal opening, and the malformation may or may
    not be covered by a layer of skin.
  • Some patients with meningocele may have few or no
    symptoms while others may experience symptoms
    similar to closed neural tube defects.

20
  • Myelomeningocele is the most severe and occurs
    when the spinal cord is exposed through the
    opening in the spine, resulting in partial or
    complete paralysis of the parts of the body below
    the spinal opening. 
  •  The paralysis may be so severe that the affected
    individual is unable to walk and may have urinary
    and bowel dysfunction. 

21
What causes spina bifida?
  • The exact cause of spina bifida remains a
    mystery. 
  • No one knows what disrupts complete closure of
    the neural tube, causing a malformation to
    develop.
  • Scientists suspect genetic, nutritional, and
    environmental factors play a role. 
  • it appears to result from a combination of
    genetic and environmental risk factors, such as a
    family history of neural tube defects, folic acid
    deficiency and medical conditions such as
    diabetes and obesity.

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Risk factors
  • Race. Spina bifida is more common among Hispanics
    and whites of European descent.
  • Family history of neural tube defects. Couples
    who've had one child with a neural tube defect
    have a slightly higher chance of having another
    baby with the same defect. That risk increases if
    two previous children have been affected by the
    condition. In addition, a woman who was born with
    a neural tube defect, or who has a close relative
    with one, has a greater chance of giving birth to
    a child with spina bifida. However, most babies
    with spina bifida are born to parents with no
    known family history of the condition.

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  • Folic acid deficiency. This vitamin is important
    to the healthy development of a fetus. Lack of
    folic acid (vitamin B-9) increases the risk of
    spina bifida and other neural tube defects.
  • Some medications. Anti-seizure medications, such
    as valproic acid (Depakene), seem to cause neural
    tube defects when taken during pregnancy, perhaps
    because they interfere with the body's ability to
    use folic acid.

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  • Diabetes. The risk of spina bifida increases with
    diabetes, especially when the mother's blood
    sugar is elevated early in her pregnancy. Much of
    this risk is preventable by careful blood sugar
    control and management.
  • Obesity. There's a link between pre-pregnancy
    obesity and neural tube birth defects, including
    spina bifida. Obese women may have more babies
    with spina bifida possibly because of nutritional
    deficits from poor eating habits or because they
    may have diabetes.

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  • Increased body temperature. Some evidence
    suggests that increased body temperature
    (hyperthermia) in the early months of pregnancy
    may increase the risk of spina bifida.

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Complications
  • Factors that affect the severity of complications
    include
  • The size and location of the neural tube defect .
  • Whether skin covers the affected area
  • Whether spinal nerves come out of the affected
    area of the spinal cord.
  • Children with myelomeningocele may experience
    physical and neurological problems, including
    lack of normal bowel and bladder control, and
    partial or complete paralysis of their legs.

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  • Babies born with myelomeningocele also commonly
    experience accumulation of fluid in the brain, a
    condition known as hydrocephalus.
  • Most babies with myelomeningocele will need a
    shunt .
  • The majority of newborns with myelomeningocele
    survive.
  • some may develop meningitis, an infection in the
    tissues surrounding the brain

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  • Children with myelomeningocele may develop
    learning disabilities.
  • Children with spina bifida may also suffer from
    latex allergies, skin problems, urinary tract
    infections, gastrointestinal disorders, seizure
    disorders, depression, and social and emotional
    problems.

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Screening and diagnosis
  • (1)Blood tests
  • second trimester maternal serum alpha fetoprotein
    (MSAFP)
  • alpha-fetoprotein (AFP) is made naturally by the
    fetus and placenta. 
  • But if abnormally high levels of this protein
    appear in the mothers bloodstream it may
    indicate that the fetus has a neural tube defect.
  • The MSAFP test, however, is not specific for
    spina bifida.

30
  • Ultrasound An advanced ultrasound can also
    detect signs of spina bifida.
  • AmniocentesisAn analysis indicates the level of
    AFP present in the amniotic fluid.
  • A small amount of AFP is normally found in
    amniotic fluid.
  • when an open neural tube defect is present, the
    amniotic fluid contains an elevated amount of AFP
    because the skin surrounding the baby's spine is
    gone and AFP leaks into the amniotic sac.
  • MRI

31
Treatment
  • There is no cure for spina bifida.
  • The nerve tissue that is damaged or lost cannot
    be repaired or replaced.
  • Treatment depends on the type and severity of the
    disorder.
  • children with the mild form need no treatment .

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  • The key priorities for treating myelomeningocele
    are to prevent infection from developing through
    the exposed nerves and tissue of the defect on
    the spine, and to protect the exposed nerves and
    structures from additional trauma.
  • Doctors have recently begun performing fetal
    surgery for treatment of myelomeningocele.

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  • Fetal surgery involves opening the mothers
    abdomen and uterus and sewing shut the opening
    over the developing babys spinal cord. 
  • They believe the earlier the defect is corrected,
    the better the outcome is for the baby.
  • Still, the benefits of fetal surgery are
    promising, and include less exposure of the
    vulnerable spinal nerve tissue and bones to the
    intrauterine environment, in particular the
    amniotic fluid, which is considered toxic.

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  • Early surgery on the spinal cord may allow the
    child to regain a normal level of functioning and
    prevent further neurological deterioration.
  • Some children will need subsequent surgeries to
    manage problems with the feet, hips, or spine.
  • Individuals with hydrocephalus generally will
    require additional surgeries to replace the shunt
  • Some individuals with spina bifida require
    assistive devices such as braces, crutches, or
    wheelchairs.  The location of the malformation on
    the spine often indicates the type of assistive
    devices needed. 

35
  • Treatment for paralysis and bladder and bowel
    problems typically begins soon after birth

36
Prevention
  • Folic acid is an important vitamin in the
    development of a healthy fetus.
  • Recent studies have shown that by adding folic
    acid to their diets, women of childbearing age
    significantly reduce the risk of having a child
    with a neural tube defect, such as spina bifida.
     
  • Dosage400 micrograms of folic acid daily
  • Foods high in folic acid include dark green
    vegetables, egg yolks, and some fruits.

37
prognosis
  • Prognosis depends on the number and severity of
    abnormalities and associated complications.
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