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. 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 monitoring for signs of spinal cord dysfunction and surgery if it occurs. Infants born with meningocele usually need surgical removal of the cyst and go on to live with no or little impairment.
However, the situation is different for babies born with myelomeningocele. They require treatment that begins in a few cases before birth (see below) and in many cases immediately after birth. Medical and surgical management will be important throughout the individual’s life. Their well-being may depend on how fast and how well the treatment is delivered. For that reason, a woman who knows that her baby will be born with spina bifida should seek evaluation at a center with expertise in management of spina bifida early in pregnancy and 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 delivering babies with spina bifida. 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:
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 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 around and in the ventricular spaces of 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 exits the skull and 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 and can be complicated by malfunction of the shunt or infection of the shunt. A shunt may be needed for an entire lifetime and may need to be replaced as the child grows.
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.
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.
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.
However, follow-up of infants who had prenatal surgery is still not long-term, fetal surgery requires a high level of training and skill for the surgeon and the medical center where the surgery occurs. In the study looking at the impact of fetal surgery, certain condition precluded participation (such as uterine abnormalities, maternal obesity, prior delivery of a premature infant and fetuses with other malformations). It is not known if outcomes from in utero surgery would be as optimal in these situations.
Subsequent Treatment and 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 treated surgically.
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 in the spine. 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 their prior level functioning and prevent further nerve deterioration.
Children with hydrocephalus will usually need additional surgeries over time to replace the ventricular cerebrospinal fluid shunt, which can become outgrown or clogged.
Treatment for paralysis usually begins soon after birth. The goal of treatment is to increase mobility, strength and independence. Working with a physical therapist, parents can learn how to exercise the baby’s legs. They should start these exercises early on – not long after the initial surgery. In addition to physical therapy, children with spina bifida should be given physical education or adaptive training in school. Some people with spina bifida need adaptive equipment 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 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. A trunk 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.
Impaired nerve function can result in an inability to voluntarily empty the bowel or bladder. Inability to empty the bladder effectively can result in infections and kidney damage. Management of the bladder is critical since poor management can lead to kidney failure impacting health and lifespan. 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. Inability to effectively empty the bowels results in chronic severe constipation, most patients with bowel involvement will benefit from a bowel program to stimulate emptying the rectum regularly. Bowel and bladder problems may require surgery to enhance the function of the bladder or bowels.
Education regarding latex and other allergies and preventing skin break down in insensate areas should occur early in the child’s life. Issues to be addressed as the child grows include learning concerns, optimal nutrition including avoidance of obesity, monitoring for and if needed treatment to halt early puberty and treatment for adjustment and mental health concerns (including depression and anxiety).
Children with spina bifida should be involved in medical decision making and self-care consistent with their cognitive development throughout childhood. As children transition to adulthood, higher educational/vocational guidance is important, planning for transition to adult living and the adult health care system should be completed, discussion regarding family planning and recurrence risk should occur and guidance and support of sexual function should be offered.
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. These specialists will collaborate with your primary care clinician. Professionals within the education system will be important to develop a health plan at school, ensure appropriate physical supports in the school setting including mobility, access to the classroom, and adapted physical education, provide specialized educational supports when needed, and plan for the transition to adulthood.
What can I expect now that my child has been diagnosed with spina bifida?
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