Our spina bifida program
It is now evident that a coordinated team approach to spina bifida is the most successful method to treat the growing number of children and to ensure that they can function at their optimal level. Frequent clinic visits based on need — a time in which each of the specialists on the team evaluates the child — leads to a unified approach and early detection of problems.
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.
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.