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| Spina Bifida |
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| SPINA BIFIDA - AN INTRODUCTION |
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| Spina bifida is the
most common of a group of birth defects called neural
tube defects (NTDs). The neural tube is the embryonic
structure that develops into the brain and spinal cord.
Often called open spine, spina bifida affects the backbone
and, sometimes, the spinal cord. 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. Spina bifida and other NTDs occur more frequently
among Hispanics and whites of European extraction and,
less commonly, among Ashkenazi Jews, most Asian ethnic
groups and African-Americans. |
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| Child
with Spina Bifida |
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HOW
DOES SPINA BIFIDA AFFECT A CHILD ? |
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In
the embryo, there is a tiny ribbon of tissue that folds
inward to form a tube. This structure, called the neural
tube, forms by the 28th day after conception. When this
process goes awry and the neural tube does not close completely,
defects in the spinal cord and in the vertebrae (small
bones of the spine) can result. There are three forms
of spina bifida: |
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Occulta
: In this usually symptomless form, there
is a small defect or gap in one or more of the vertebrae
of the spine. The spinal cord and nerves usually
are normal, and most affected individuals have no
problems. |
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Meningocele : In
this rarest form, a cyst or lump consisting of membranes
surrounding the spinal cord pokes through the open
part of the spine. The cyst, which can vary in size,
can be removed by surgery, allowing for normal development. |
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Myelomeningocele
: In this most severe form, the cyst holds
both the membranes surrounding the spinal canal
and nerve roots of the spinal cord and, often, the
cord itself. Or there may be no cyst, but only a
fully exposed section of the spinal cord and nerves.
Spinal fluid may leak out. Affected babies are at
high risk of infection until the back is closed
surgically, although antibiotic treatment may offer
temporary protection. In spite of surgery, some
degree of leg paralysis and bladder and bowel control
problems remain. |
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The
severity of paralysis is largely determined by the spinal
nerves involved. In general, the higher the cyst on the
back, the more severe the paralysis. About 80 percent
of spina bifida cysts are in the lower back's lumbar and
sacral regions. |
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WHAT
CAUSES SPINA BIFIDA ? |
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Spina
bifida usually is an isolated birth defect. Although scientists
believe that genetic and environmental factors may act
together to cause this and other NTDs, 95 percent of babies
with spina bifida and other NTDs are born to parents with
no family history of these disorders. While spina bifida
appears to run in certain families, it does not follow
any particular pattern of inheritance. If one child has
spina bifida, the risk of recurrence in any subsequent
pregnancy is greatly increased, to about one in 40. If
there are two affected children, the risk in any subsequent
pregnancy is about one in 20. Spina bifida also can occur
as part of a syndrome with other birth defects. Here,
inheritance patterns may differ from those of isolated
spina bifida. |
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Women
with certain chronic health problems, including diabetes
and seizure disorders (treated with certain anticonvulsant
medications), have an increased risk (approximately 1/100)
of having a baby with spina bifida. |
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HOW
IS SPINA BIFIDA TREATED ? |
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| Spina
bifida occulta usually requires no treatment. Meningocele,
which does not involve the spinal cord, can be repaired
surgically, usually with no paralysis. Most children with
meningocele develop normally. However, affected children
should be evaluated for hydrocephalus and bladder problems
so they can be treated promptly. A baby with the most
severe form of spina bifida, myelomeningocele, usually
requires surgery within 24 to 48 hours after birth. Doctors
surgically tuck exposed nerves and spinal cord back inside
the spinal canal and cover them with muscle and skin.
Prompt surgery helps prevent additional nerve damage from
infection or trauma. However, nerve damage that already
has occurred cannot be reversed and limb paralysis and
bladder and bowel problems usually remain. |
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soon after surgery as possible, a physical therapist teaches
parents how to exercise their baby's legs and feet to
prepare for walking with leg braces and crutches. Studies
show that about 70 percent of affected children can walk
with or without these devices, although many children
will require a wheelchair. |
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| About
90 percent of children with the most severe form of spina
bifida develop hydrocephalus, or fluid on the brain. When
the cerebrospinal fluid, which cushions and protects the
brain and spinal cord, is unable to drain normally, fluid
collects in and around the brain, causing the head to
be enlarged. Without treatment, mental retardation and
other neurologic damage may result. |
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| If
the child develops hydrocephalus, fluid can be drained
from the brain through surgical placement of a special
tube called a shunt. The shunt runs under the skin into
the chest or abdomen, and the fluid passes harmlessly
into the child's body. |
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| Most
children with severe spina bifida have a tethered spinal
cord, meaning that the spinal cord does not slide up and
down with movement as it should, because it is held in
place by surrounding tissue. While most children have
no symptoms from this, some suffer progressive loss of
function in their legs, and a few develop scoliosis (curvature
of the spine). If the spinal cord is surgically untethered
soon after these symptoms begin, a child should return
to his or her usual level of functioning. |
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| Other
chronic complications associated with severe spina bifida
include obesity, gut and urinary tract disorders, psychological
and sexual issues, and learning disabilities. |
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| According
to the Spina Bifida Association of America (SBAA), between
18 and 73 percent of children with spina bifida are allergic
to latex (natural rubber), possibly due to intense exposure
during surgeries and medical procedures. Symptoms may
include watery eyes, wheezing, hives, rash, and even life-threatening
anaphylactic reactions. Doctors should use only nonlatex
gloves and equipment during any procedures on individuals
with spina bifida. Affected individuals and their families
should avoid latex items often found in the home and community,
such as baby bottle nipples, pacifiers and balloons (a
list is available from the SBAA). |
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| With
treatment, children with spina bifida usually can become
active individuals. At least 70 percent of children with
spina bifida have normal intelligence, although some children
do have learning problems. Most affected women can have
children, but such pregnancies are considered "high
risk," as the risk of having a baby with spina bifida
is about 1 in 100. |
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