How Is Spina Bifida Treated?

^{Reviewed by: My Child Without Limits Advisory Committee November 2009}

There is no cure for spina bifida. The nerve tissue that is damaged or lost cannot be repaired or replaced. However, certain treatments are effective. The aim of treatment is to enable the child to reach the highest degree of functioning and independence. The type of treatment required depends on the type and severity of the disorder. Generally, children born with the mild form of spina bifida (spina bifida occulta) need no immediate treatment, although some may require surgery over time. Infants born with meningocele usually need surgical removal of the cyst and go on to live with little impairment, if any.

Early intervention

However, the situation is different for babies born with myelomeningocele. They require treatment that begins almost immediately after birth and may last a lifetime. Their well-being may depend on how fast and how well that treatment is delivered. For that reason, a woman who knows that her baby will be born with spina bifida may decide to have her child in a large medical center where specialized surgery on her newborn can be performed. Her doctor also may recommend that she have a cesarean section (C-section), rather than deliver vaginally. By delivering the baby before labor begins, this approach may minimize the amount of damage to the infant's exposed nerves. That is why many specialists now recommend a C-section as the safest means of deliver babies with spina bifida. And because C-sections also tend to be scheduled in advance, this type of birth alerts the pediatric neurosurgical team so that they can be on site at the appointed time -- allowing them to perform surgery shortly after the baby is born.

The two most important goals in treating myelomeningocele are:

• to prevent infection from developing and affecting the exposed nerves and tissue of the spinal defect
• to protect the exposed nerves and tissue from additional damage

Typically, a child born with spina bifida will have surgery very soon after birth to close the defect and prevent infection or further damage. Doctors generally begin treatment with antibiotics as soon as possible in order to avoid infection of the exposed spinal cord. This could lead to encephalitis or meningitis -- both very serious, even fatal, infections. If the birth has taken place elsewhere, the baby should be transferred immediately to a medical center where surgery can be performed. The operation usually is performed within 36 to 48 hours after birth. Although prompt surgery is the ideal, it may have to be delayed for up to six weeks if the baby's health is in jeopardy. During the procedure, a neurosurgeon (a surgeon who specializes in operations on the brain, nerves, and spinal cord) puts the exposed spinal cord and tissue inside the spinal canal in the baby's body and then covers the opening with muscle and skin taken from either side of the back. If the area in question is very large and hard to close, a plastic surgeon may be called in to accomplish this part of the procedure.

Many infants with spina bifida also have hydrocephalus. Although the word literally means "water on the brain," it is, in fact, a build-up of cerebrospinal fluid in the brain. In some cases it is caused by an abnormality of the brain called the Chiari II malformation. With this malformation, one portion of the brain is displaced from the back of the skull down into the upper neck. That interrupts the normal flow of cerebrospinal fluid, resulting in an accumulation of fluid, or hydrocephalus. This condition requires surgery, in which a shunt -- or drainage tube -- is placed inside the head. It runs under the skin and down into the chest or abdomen. The shunt relieves pressure on the brain by removing the excess fluid from the brain and draining it into the abdomen, where it can be eliminated easily. The procedure is a fairly simple one, but it is essential to prevent swelling that may damage the brain. A shunt may be needed for an entire lifetime.

Earlier intervention (prenatal surgery)

Recently, doctors have begun performing fetal surgery to treat myelomeningocele. Fetal surgery, which takes place before birth, is performed in utero (within the uterus). This kind of surgery 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 it will be for the baby. Apparently, the more time that the spinal cord is exposed to substances outside the fetus's body -- even those within the uterus -- the greater the chance for damage to the cord and nerves. The hope is that by repairing the defect between the 19th and 25th weeks of pregnancy damage to exposed spinal nerves may be averted and the likelihood of paralysis and other problems may be lessened. Although the procedure cannot bring back lost nerve function, it may prevent additional loss from happening. However, the surgery is considered experimental and there are risks for the unborn child as well as for the mother.

One problem is that prenatal surgery greatly increases the risk of premature birth, which poses its own assortment of risks for the baby. If the surgery causes the baby to be born too early, there can be numerous complications: organs that are not mature, bleeding in the brain, and even death. Risks for the mother include infection, blood loss, gestational diabetes, and weight gain due to prolonged bed rest.

Another benefit that doctors have discovered is that the procedure positively affects the way the brain develops in the uterus. Certain complications -- such as the Chiari II malformation with associated hydrocephalus -- actually correct themselves when surgery is performed. This can reduce, and sometimes, eliminate the need for surgery after birth to implant a shunt to drain excess brain fluid.

Subsequent surgeries

Surgery on the newborn with spina bifida may only be the first of many operations that the child will need. Following the first surgery, the baby should have regular evaluations to assess any developmental issues or complications that may call for further procedures. For example, surgery may be required to correct any deformities. Orthopedic (bone- and joint-related) problems may include hip dislocations, curvatures in the back, ankle and foot deformities, and contracted muscles, tendons, and ligaments that may have to be surgically released. It is not uncommon for nerve damage to lead to clubfoot and other foot deformities. Placing the affected foot in a cast for the first several months of the child's life may work to straighten the foot. When the child reaches one year of age, corrective surgery may be performed on the foot. Similarly, hip deformities also may be corrected surgically -- allowing the child to walk.

Many children with myelomeningocele develop a complication called progressive tethering, or tethered cord syndrome. This is a condition in which the spinal cord is bound to the scar from the earlier surgery to close the opening. Because it is attached to something that does not move, the spinal cord is not able to grow in length and keep up with the growth of the child. As a consequence, it stretches abnormally. Tethered cord syndrome may result in a loss of nerve and muscle function in the legs, feet, bowel, and bladder and cause permanent damage. In addition to producing deformities of the feet and legs, this syndrome also may produce incontinence and paralysis. Another surgery to detach the scar tissue and release the end of the spinal cord may allow the child to regain a normal level of functioning and prevent further nerve deterioration.

Children with hydrocephalus will usually need additional surgeries over time to replace the shunt, which can become outgrown or clogged. Bowel and bladder problems may require surgery to enhance function in elimination, for constipation, or incontinence. Nerve damage may prevent complete emptying of the bladder. This can result in kidney damage and even kidney failure if left untreated. To deal with this problem, it may be necessary to use a urinary catheter several times a day to make sure that the bladder is completely emptied. This is known as clean intermittent catheterization, or CIC.

Some people with spina bifida need medical devices such as braces, crutches, or wheelchairs. The location of the malformation on the spine often indicates the type of devices needed. Children with a defect high up on the spine and more paralysis will often require a wheelchair, while those with a defect lower on the spine may be able to use crutches, bladder tubes, or walkers. When the child is old enough to walk, leg braces may be required. In addition to helping the child walk, they prevent joint damage as well. A brace also may help if the child has a curvature of the spine -- such as scoliosis (a sideways, or lateral curving of the spine) or kyphosis (forward curving of the spine, causing a hunchback) or both. However, the curvature may worsen as the child grows.Severe curvature must be corrected surgically.

Treatment for paralysis and bladder and bowel problems usually 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.

The care team

Every child with severe spina bifida will need extensive and intricate care. This requires the involvement of a specially trained team of professionals. Included in this care team are pediatricians, neurosurgeons, orthopedic surgeons, neurologists, endocrinologists, urologists, physical medicine specialists, physical therapists, orthotics specialists, occupational therapists, psychologists, nurses, dietitians, and social workers -- among others. Ideally, the child with spina bifida should receive care at a specialized multidisciplinary spina bifida setting where all the necessary specialists are and services can be delivered in a coordinated fashion. These clinics exist all over the U.S., and a list can be obtained from the Spina Bifida Association.

Children with spina bifida need to be seen frequently by their doctor. During the first six months of life, those visits may be once or twice each month. From six months to one year of age, the visits become more numerous -- two to three times a month. This is to monitor how well treatment is working and to see if further surgeries are needed. Once the child reaches the first birthday, doctor visits are advised two to four times per year.

The goal of treatment is to gain and increase mobility, strength, and independence. Working with a physical therapist, parents can learn how to exercise the baby's legs to increase strength and movement. They should start these exercises early on -- not long after the initial surgery. Children with spina bifida should be given extensive physical therapy, as well as physical education or adaptive training in school.

Next:

What Does the Future Hold for Children WIth Spina Bifida?

Download the Introduction to Spina Bifida.