A coordinated team approach is the most successful treatment method for children with spina bifida. At the Spina Bifida Center, our renowned experts in Neurosurgery, Orthopaedic Surgery, and Urology collaborate with teams from Rehabilitative Services (including Physical and Occupational Therapy, Speech Pathology, Audiology, and Orthotics), Clinical Nutrition, and Family Services to provide innovative and comprehensive clinical care for our patients and their families.
Clearly the major physical problems of the newborn period are related to the nervous system. They are directly related because of the open spine and hydrocephalus and indirectly because of bladder and bowel dysfunction and bone deformity resulting from disconnection of the nervous system.
Download a document that suggests which agencies might be most helpful at different times in a child's life.
The Neurosurgeon's Role
After the obstetrician and pediatrician have made their initial evaluation of the child, the next step in the sequence of events is the involvement of a pediatric neurosurgeon, who manages treatment related to the brain and spinal cord. The neurosurgeon's initial goal is to prevent infection in the nervous system.
The secondary aims of the neurosurgeon are preservation of all nervous tissue and to create an environment in which function is preserved. Occasionally nerves which are intact at birth can repair themselves, and some improvement in the child's ability to move may be seen. To attain these goals, early closure of the back is absolutely essential.
Many neurosurgeons prefer to close the back within the first 24 hours of life. Modern techniques in anesthesia now make surgery possible on newborns with minimal risk. The coverings of the nervous system are reconstructed and reinforced, and the skin closed over the involved area.
Within the first few months of life, 80 to 90 percent of children born with myelomeningocele develop progressive hydrocephalus. Consequently, the neurosurgeon observes the child closely, watching for an increase in the child's head circumference. Initially, the child's head size may appear normal or even small. However, if the child's head circumference increases progressively after the child's back is closed, a shunt will eventually become required.
Thus a period of observation is necessary during which the neurosurgeon will evaluate measurements of the head size, and perform studies to determine the size and changes in compartments at the center of the brain. CT scans or MRIs are used to create images of the structures under examination. Today, effective methods for the drainage of CSF from the ventricular system also are available. A system of tubes and valves are used to carry the fluid to another body cavity where it can be returned to the blood either directly or by absorption through the lining of the body cavity.
This system of tubes is called a shunt and usually passes from the brain to the abdominal cavity (peritoneal cavity) or the heart (atrial chamber). The shunts are thus named by the cavities they interconnect; ventriculoperitoneal (V-P) and ventriculoatrial (V-A). A functioning shunt establishes normal growth of the head, ensures maintenance of the brain function and allows development of the intellect. However, occasionally shunts can malfunction, a situation that requires surgery and a short hospitalization.
The neurosurgeon evaluates the shunt function (if hydrocephalus is present) by following the head circumference, obtaining occasional CT scans and monitoring for any neurological problems. The parents can expect at least three shunt revisions in the first ten years of the child's life.
Many children will have more, and some will have fewer revisions. The state of the back closure is evaluated and the function in the legs is determined. If the child is problem-free and making good progress, the clinic visits will become less and less frequent as the child grows older.
Learn more about Neurosurgery at Lurie Children’s.
The Orthopaedic Surgeon's Role
The orthopaedic surgeon sees the child during his initial admission to evaluate deformities already present and to determine if problems will develop. Some orthopaedic deformities are present at birth, such as clubfoot or hip dislocation, but the great majority develop during growth because of the unequal or imbalanced muscle groups.
The degree of muscle paralysis and of muscle imbalance is not the same in every child. It varies according to the level of the spina bifida defect. Of course, the degree of muscle paralysis, in association with the child's balance and motor coordination, are major factors in determining the child's ability to walk with braces. A major goal of orthopaedic care is to correct deformities that are present at birth and to prevent more deformities from developing during the growing years.
Spina bifida also causes varying degrees of muscle weakness. Decreased mobility and decreased time spent in the standing position leads to bones that are often fragile and prone to fractures. Fractures can occur even with minimal trauma, but with appropriate care these fractures heal quickly requiring only a short period in a cast. In fact, bone healing in spina bifida is quite different from other children. Bones usually heal quite fast and only require a short time in the cast or even the child's brace.
Unequal or unbalanced muscle groups can lead to deformities as the child grows. These deformities are accentuated by the position of the legs during uterine development. As a result, splinting and range of motion exercises are prescribed. Early therapy may consist of bandaging, casting or bracing to slowly bring the deformed extremity into the proper plane of function to maximize available motor function. This may be done with or without braces.
Other deformities may arise because of lying or sitting in a certain position for a prolonged period of time. Night splints are often used to help prevent these problems. Sometimes, however, surgery is necessary. Short periods in the hospital are required, and immobilization in a cast is frequent, but rarely exceeds four to six weeks.
Two major types of curvature of the spine are seen in spina bifida. The first is lumbar kyphosis, a marked forward curvature of the spine in the area of the opening on the child's back. This forward curvature results in a hump on the child's back, which makes closure of the back difficult. Lumbar kyphosis is a progressive deformity, which often requires surgery. The bony prominence on the back requires skin care to prevent sores and infection of the back closure.
The second curvature of the spine usually comes later when the child spends more time in the erect position. The curvature — scoliosis — is usually more prominent in the side-to-side direction and is often progressive and requires surgical stabilization.
Several surgical procedures and continual bracing may be used to maintain the extremity function. Contractures (shortening of tendons from disuse) are a major threat to the child's ability to walk. A joint fixed in one position may prevent standing, sitting or both. Surgery is often necessary to release these contractures, but prevention, by good physical therapy, is obviously the better choice.
Ultimately, all children will stand and most will walk. The majority will require braces and/or crutches to walk. Some of the children who require braces to keep their knees locked and their hips straight will walk as children, but as adolescents may find a wheelchair more convenient and efficient.
Learn more about Orthopaedics at Lurie Children’s.
The Urologist's Role
The urologist looks for problems in the urinary system, such as infection, continual dribbling of urine or deterioration of kidney function. Urine is collected and examined for bacteria and signs of infection. A special kidney x-ray known as an intravenous pylogram (IVP) or ultrasound is repeated periodically to determine the status of the urinary system. Early detection of problems is very important. The goal of the urologist is a dry child with normal kidney function.
Learn more about Urology at Lurie Children’s.
Other Team Members
Physical therapy and occupational therapy staff members play a major role in the child's development of the skills needed to walk and carry out those social activities of daily living. An early and aggressive physical therapy program is directed not only toward range of joint motion but also toward the promotion of normal motor development.
The aim is to have children standing by the age of 10 months in a device called a standing A-frame. And if the child has good sitting balance, usually between the ages of 16 to 18 months a walking brace will be fitted to provide joint stabilization. The type of brace will depend on the child's motor level. In most cases, the lower the functional level, the less bracing is required.
Social workers also play a vital role in the care of the child with spina bifida. During the first weeks after the child's birth when parents are under severe pressure, parents can be helped to understand the effects of stress on themselves and on the total family system and can develop skills to deal with this stress.
Later as the child develops and particular issues arise, e.g. hospitalization, schooling, a new pregnancy, vocational guidance, illness of a parent, etc., parents may wish to consult with a social worker who can counsel them or direct them to a service in the community that can meet their particular needs.
Very often, the pediatrician is the direct liaison between the patients and the staff of the clinic team. In other cases, the pediatrician assumes the role of general medical provider for problems as they relate to spina bifida. Since a child with spina bifida has all the childhood illnesses that a child without the condition has, a family pediatrician should get to know your child and his problems.
Dieticians also are a great help to families. The first concern that the dietitian has is to assure that the patient is receiving minimal and adequate caloric intake. This is to allow for healthy growth and development. Furthermore, since mobility is sometimes a problem with children born with spina bifida, obesity can be a hindrance to the child's mobility. A dietitian working in conjunction with the other members on the team can assess and follow the body growth rates of patients with spina bifida.
The psychologist on the clinic team is concerned with the psycho-developmental aspects of a patient's life. Developmental levels and intellectual testing scores can point out both medical and educational problems. Protocols for routine developmental testing are often used to pick up subtle changes in function, which could indicate a shunt malfunction.
The psychologist often works with professionals who are responsible for educating the child. The physical problems that a child might have can result in a subtle learning disability being overlooked. Not all children with spina bifida have learning disabilities, but a good working relationship between the medical and educational system is vital to ensure the child's maximum achievement.
Sometimes, an ophthalmologist is needed. Of interest is the fact that a number of children with spina bifida develop crossed eyes (strabismus) during the first few years of life. This may appear as part of a shunt malfunction, but more often it is without apparent cause. At that point an ophthalmologist is brought into the team, and either glasses or surgical correction may be indicated.
With the birth of a child with spina bifida, the anticipated joy may suddenly turn into a confusing nightmare. Bewildered parents are bombarded with facts and pushed to make decisions of great consequence to the child's future. Bewilderment fades into shock and anger. Typically, they ask "Why? What caused this to happen?"
Someday we may know the answers to some of those questions, which may lead to prevention. Intrauterine repair of the back and treatment of hydrocephalus may arrest progression of this disease and allow the birth of minimally affected children. Electrical stimulation of paralyzed legs, bladder and bowel may return voluntary function. In the distant future, repair of the nervous system itself may be possible.
Clearly, research and the support of an informed public are necessary for continuing progress. Spina bifida is a life-long condition like diabetes and arthritis. Adults with spina bifida must know their own problems and must be followed by physicians knowledgeable in each of the systems involved.
A major concern of those who care about these children is habilitation. We must ensure the opportunity to develop to full potential and full independence and insist on a barrier-free society for the individual with spina bifida.
Families who live in large cities often can find a team of specialists in this condition. In rural areas, family doctors can refer patients to the nearest available help. In almost every community, agencies and resources are available to families and to children with special needs.
The hospital team can usually put families in touch with the visiting nurses, the infant stimulation program, vocational guidance programs, the Easter Seal program, special and regular schools, counseling programs for parents and for children, special recreational programs, rehabilitation programs and genetic counseling. Furthermore, financial aid is available at both the state and federal levels.
Illinois' Division of Specialized Care for Children (DSCC) will make this information available. The myelomeningocele team will direct the family to educational and rehabilitation facilities available. When a child is born with spina bifida, many parents and families feel that they are the only ones in the world experiencing this birth defect. Nothing could be further from the truth. In fact, every hour 24 hours a day, another child is born with spina bifida.
The Spina Bifida Association of America was formed to address the needs of those with spina bifida. It is the only national organization totally devoted to this birth defect. The Spina Bifida Association is composed of parents, professionals, adults with spina bifida, and interested members of the general public. Check your phone book or their Website for further information, and get involved.
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