| What is Spina Bifida?
Spina Bifida is one of the most common birth defects compatible with life.
Technically it means an open spinal column, but has come to specifically relate
to those instances when not only is the spinal column open, but the spinal cord
is incompletely formed and is exposed to the air. The much more common form is
the closed or occulta form which is probably of little significance and seen
widely in population, The spinal cord forms in the first few weeks of gestation.
In addition to genetic and environmental associations, it is felt that foliate
deficiency may be an underlying factor in its development. Besides the failure
of the spinal cord to form and close properly, other defects can occur in the
development of the nervous system. These include hydrocephalus,
syringohydromyelia and Chiari malformations. In addition there are potential
disturbances with the bowel and bladder function, both extremity and spine
related problems and respiratory and swallowing difficulties. These problems are
due wholly or in part to the disturbance in the nervous system.
The diagnosis can often be made on the basis of a high resolution ultrasound
or elevation of maternal alpha protein levels. Other times it is not recognized
until birth. It is widely accepted that most children born with Spina Bifida
should be aggressively treated both medically and surgically. Recommendations
are for early closure of the back and spinal cord as management of hydrocephalus
if indicated. The important thing to realize is that the operation is designed
specifically to prevent infection or ongoing trauma to the exposed spinal cord
and not to return function that is not present at birth. This is not an
insignificant distinction. Hydrocephalus is usually present in 80-90% of
children with Spina Bifida and should be treated when determined, usually before
discharge from the hospital.
As many different parts of the body and nervous system are potentially
involved, the child will need to see numerous physicians and therapists for
follow up. In addition to physical therapy and early intervention, the child may
need treatment for incontinence as well as bracing for the legs or spine.
Another entity which is common in children with Spina Bifida is known as
Chiari Malformation. There are different forms of Chiari Malformation, but most
are either type I or type II. All children with Spina Bifida have
some degree of type II malformation. The thing to realize, is that while
it is present raidographically it may not be causing the child any symptoms and therefore does not necessarily need to be treated. The types of symptoms that
occur with Chiari Malformation include repeated aspirations, apnea, neck pains,
and complaints of weakness or funny sensations in the arms. Usually, surgical
treatment for Chiari Malformation is reserved for those children with clear
evidence of progressive symptoms not for findings on a MRI.
There are three types of Spina Bifida:
- Myelomeningocele - the severest form in which the spinal cord and its
protective covering, the meanings, protrude from the opening in the spine.
- Meningocele - the spinal cord develops normally but the meanings protrude
from the opening created by damaged or missing vertebrae and may be exposed.
- Occulta - which means "hidden", indicates that a layer of skin covers the
defect, where one or more vertebrae are malformed. Occulta in the mildest form
of Spina Bifida.
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