What is Spina Bifida
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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:

  1. Myelomeningocele - the severest form in which the spinal cord and its protective covering, the meanings, protrude from the opening in the spine.
  2. Meningocele - the spinal cord develops normally but the meanings protrude from the opening created by damaged or missing vertebrae and may be exposed.
  3. 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.