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February 10, 2012

Spina Bifida and Anencephaly (cont.)

How is spina bifida treated?

There is no cure for spina bifida. The nerve tissue that is damaged or lost cannot be repaired or replaced, nor can function be restored to the damaged nerves. Treatment depends on the type and severity of the disorder. Generally, children with the mild form need no treatment, although some may require surgery as they grow.

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. Typically, a child born with spina bifida will have surgery to close the defect and prevent infection or further trauma within the first few days of life.

Doctors have recently begun performing fetal surgery for treatment of myelomeningocele. Fetal surgery—which is performed in utero (within the uterus)—involves opening the mother's abdomen and uterus and sewing shut the opening over the developing baby's spinal cord. Some doctors believe the earlier the defect is corrected, the better the outcome is for the baby. Although the procedure cannot restore lost neurological function, it may prevent additional loss from occurring. However, the surgery is considered experimental and there are risks to the fetus as well as to the mother.

The major risks to the fetus are those that might occur if the surgery stimulates premature delivery such as organ immaturity, brain hemorrhage, and death. Risks to the mother include infection, blood loss leading to the need for transfusion, gestational diabetes, and weight gain due to bed rest.

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. As an added benefit, doctors have discovered that the procedure affects the way the brain develops in the uterus, allowing certain complications—such as Chiari II with associated hydrocephalus—to correct themselves, thus, reducing or, in some cases, eliminating the need for surgery to implant a shunt.

Many children with myelomeningocele develop a condition called progressive tethering, or tethered cord syndrome, in which their spinal cords become fastened to an immovable structure—such as overlying membranes and vertebrae—causing the spinal cord to become abnormally stretched and the vertebrae elongated with growth and movement. This condition can cause loss of muscle function to the legs, bowel, and bladder. 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, which can be outgrown or become clogged.

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. Children with a defect high on the spine and more extensive paralysis will often require a wheelchair, while those with a defect lower on the spine may be able to use crutches, bladder catherizations, leg braces, or walkers.

Treatment for paralysis and bladder and bowel problems typically begins soon after birth, and may include special exercises for the legs and feet to help prepare the child for walking with braces or crutches when he or she is older.



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