Spina Bifida and Anencephaly (cont.)
In this Article
- Introduction to spina bifida
- What is spina bifida?
- What are the different types of spina bifida?
- What causes spina bifida?
- What are the signs and symptoms of spina bifida?
- What are the complications of spina bifida?
- How is spina bifida diagnosed?
- How is spina bifida treated?
- Can spina bifida be prevented?
- What is the prognosis for spina bifida?
- What research is being done on spina bifida?
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 early priorities for treating myelomeningocele are to prevent infection from developing through the exposed nerves and tissue through the spine defect, 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 minimize the risk of infection or further trauma within the first few days of life.
Selected medical centers continue to perform fetal surgery for treatment of myelomeningocele through a National Institutes of Health protocol (Management of Myelomeningocele Study, or MOMS). Fetal surgery is performed in utero (within the uterus) and involves opening the mother's abdomen and uterus and sewing shut the abnormal opening over the developing baby's spinal cord. Some doctors believe the earlier the defect is corrected, the better the baby's outcome. Although the procedure cannot restore lost neurological function, it may prevent additional losses from occurring.
Originally planned to enroll 200 expectant mothers carrying a child with myelomeningocle, the Management of Myelomeningocele Study was stopped after the enrollment of 183 women, because of the benefits demonstrated in the children who underwent prenatal surgery.
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 -- including 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 fetal hindbrain develops in the uterus, allowing certain complications -- such as Chiari II and hydrocephalus -- to correct themselves, thus, reducing or, in some cases, eliminating the need for surgery to implant a shunt.
Twenty to 50 percent of children with myelomeningocele develop a condition called progressive tethering, or tethered cord syndrome. A part of the spinal cord becomes fastened to an immovable structure (such as overlying membranes and vertebrae). This causes the spinal cord to become abnormally stretched and the vertebrae elongated with growth and movement. This condition can cause change in the muscle function of the legs, as well as changes in bowel and bladder function. 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 mobility 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. Beginning special exercises for the legs and feet at an early age may help prepare the child for walking with braces or crutches when he or she is older.
Treatment of bladder and bowel problems typically begins soon after birth, and may include bladder catheterizations and bowel management regimens.
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