Spina Bifida
Spina bifida is a birth
defect that happens when a baby's backbone (spine) does not form normally. As
a result, the spinal cord and the nerves that branch out of it may be damaged.
The term spina bifida comes from Latin and literally means "split" or "open" spine.
This defect happens at the end of the first month of pregnancy, when a baby's spine
and spinal cord (a bundle of nerves that runs down the center of the spine)
are developing.
Sometimes, the defect causes an opening in the back, which is visible. The spinal
cord and its coverings sometimes push through this opening. Other times, there is
no opening and the defect remains hidden under the skin.
Depending on the severity of the defect and where it is on the spine, symptoms
vary. Mild defects may cause few or no problems, while more severe defects can cause
serious problems, including weakness, loss of bladder control, or paralysis.
Children with an exposed opening on the back will need surgery to close it.
Causes
Low levels of the vitamin folic
acid during pregnancy are linked to spina bifida. Folic acid plays a large role
in cell growth and development, as well as tissue formation. Not having enough folic
acid in the diet before and during early pregnancy can increase a woman's risk of
spina bifida and other neural tube defects.
The causes of spina bifida in pregnancies where mothers took prenatal vitamins
and got enough folic acid are largely unknown. Some evidence suggests that genes may
play a role, but most babies born with spina bifida have no family history of the
condition.
A high fever during pregnancy may increase a woman's chances of having a baby with
spina bifida. Women with epilepsy who have taken the drug valproic acid to control
seizures also are at an increased risk of having a baby with spina bifida.
Types of Spina Bifida
The two forms of spina bifida are spina bifida occulta and spina bifida aperta:
- Spina bifida occulta is the mildest form of the condition and
can go unnoticed. "Occulta" means "hidden" in Latin, which in this case means that
the defect is covered by skin. The spinal cord does not stick out through the skin,
although the skin over the lower spine may have a patch of hair, a birthmark, or a
dimple above the groove between the buttocks. Inside, the cord may be tethered (attached)
to surrounding tissue instead of floating loosely in the spinal column.
Most babies born with spina bifida occulta do not have long-term health problems.
- Spina bifida aperta ("aperta" means "open" in Latin) includes
two types of spina bifida:
- Meningocele (meh-NIN-guh-seel) involves the meninges, the membranes
that cover and protect the brain and spinal cord. If the meninges push through the
hole in the skull or the vertebrae (the small, ring-like bones that make up the spinal
column), it creates a fluid-filled sac called a meningocele. This sac is visible on
a baby's head, neck, or back. The sac can be as small as a grape or as large as a
grapefruit, and usually is covered by a thin layer of skin. Meningoceles can happen
anywhere along the spinal column or at the base of the skull.
Babies
with this condition can have health problems if the nerves around the spine are damaged.
For example, if the nerves that control the release of the bowels or bladder are affected,
it may be difficult for a child to control these body functions. They also might have
trouble moving certain muscles (paralysis). The degree of paralysis depends on where
the meningocele is in the spine. The higher the opening is on the back, the more severe
the paralysis can be.
- Myelomeningocele
(my-uh-low-meh-NIN-guh-seel) is the most severe form of spina bifida. It happens when
both the meninges and the bottom end of the spinal cord push through the hole in the
spine, forming a large fluid-filled sac that bulges out of a baby's back. Sometimes
the sac bursts during childbirth and the spine and nerves are exposed at birth.
A baby with this type of spina bifida usually has some paralysis, and muscle
or bone problems as a result of the paralysis. This is due to the abnormal development
of nerves in the spine, or to nerves being stretched as a result of the defect. It's also common for babies to have hydrocephalus,
a buildup of cerebrospinal fluid in and around the brain. This causes the baby to
have an enlarged head or bulging soft spot at birth, which is the result of too much
fluid and pressure inside the skull.
Diagnosis
Expectant parents may be able to find out if a baby has spina bifida by taking
certain prenatal tests. The alpha-fetoprotein (AFP) test is a blood test
done between the 16th and 18th weeks of pregnancy. This test measures how much AFP,
which the fetus produces, has passed in the mother's bloodstream. If the amount is
high, a repeat test may be done to make sure that the result is correct. If the second
result is high, it could mean that a baby has spina bifida. In this case, other tests
will be done to double-check and confirm the diagnosis.
In most cases of spina bifida aperta, doctors can see the defect on a prenatal
ultrasound. Amniocentesis also can help determine whether a baby has spina bifida.
A needle is inserted through the mother's belly and into the uterus to collect fluid
that is tested for AFP.
Usually, spina bifida occulta is not found until after a baby is born. To diagnose
the condition in these cases, doctors may do an ultrasound on younger babies (less
than 3 months old). For older babies, and to confirm results in younger babies, doctors
may rely on a magnetic resonance imaging
(MRI) scan or computed tomography (CT) scan.
Treatment
Treatment for spina bifida depends on its severity. Because spina bifida can involve
many different body systems, like the nervous and skeletal systems, children may need
support from a team of medical professionals. This team may include doctors (such
as neurosurgeons, urologists, and orthopedic surgeons), physical and occupational
therapists, and social workers.
Babies with spina bifida occulta might not need any treatment,
unless their spinal cord is tethered. Tethering can lead to problems later in life
(during growth spurts) so it's necessary to surgically detach the spinal cord from
surrounding tissue. After surgery, babies usually have no long-term health problems,
but may need surgery again later in childhood if the spinal cord reattaches.
Babies with a meningocele need surgery to push the meninges back
into the body and close the hole in the vertebrae or skull. This is usually done in
the first few months of life.
Babies with a myelomeningocele need surgery 1 to 2 days after
birth to protect the exposed area and central nervous system, and to prevent these
areas from becoming infected. If a myelomeningocele is detected early enough during
a woman's pregnancy, the fetus can be operated on to correct the defect during the
25th week of pregnancy. During surgery, doctors detach the spinal cord from the skin,
push the spinal cord back into place, and close the opening.
Babies who have hydrocephalus also need surgery to ease the buildup of fluid around
the brain. This may require an endoscopic third ventriculostomy procedure or a shunt
procedure:
- In a ventriculostomy, a small opening is made in the bottom of the third ventricle
(one of four ventricles in the brain) to allow fluid to exit the brain.
- In a shunt procedure, a thin tube is placed within the brain to drain extra fluid
down to the belly, where the body can absorb it.
Outlook
After recovery from surgery, babies born with a meningocele or myelomeningocele
may need long-term care to help treat any underlying conditions that result from their
spina bifida. Those with paralysis may eventually need walking aids like leg braces,
walkers, or a wheelchair. Children with myelomeningocele who also have hydrocephalus
will need the continuing care of a neurosurgeon, and they may have learning difficulties
in school that require special services.
With the right medical care, kids can go on to lead normal, active lives. The goal
is to create a lifestyle for them and their families in which their disability interferes
as little as possible with normal everyday activities.
Prevention
Many cases of spina bifida can be prevented if women of childbearing age take 0.4
milligrams (400 micrograms) of folic acid every day before pregnancy and continue
to take it throughout the first trimester. Some women may have to take more folic
acid, especially if they are taking the medicine valproic acid for epilepsy or depression.
Because many women don't find out that they're pregnant until 4 to 5 weeks into
the pregnancy, it is important to start taking folic acid before becoming pregnant.
This provides the best protection for an unborn baby. Good sources of folic acid include
eggs, orange juice, and dark green leafy vegetables. Many multivitamins contain the
recommended dose of folic acid, too.
Date reviewed: October 2016
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