Medical Problems Associated with Spina Bifida

Spina Bifida is a range of birth defects within the central nervous system. Many people have never heard of it, yet its occurrence rates are higher than many may think. In North America, Spina Bifida occurs in one out of every 700 birthdays to whites, and one in every 3,000 births to African Americans. In Great Britain, the occurrence rate is one in every 100 births leading scientists to believe that environmental factors influence the occurrence. (Carson-DeWitt, 1999)

Spina Bifida is divided into two smaller categories, Spina Bifida Occulta and Cystica. Spina Bifida Occulta occurs in only5% of the population affected with Spina Bifida. (Bloom & Selijeskog, 1998) It is often misdiagnosed as a fatty tumor and symptoms include a birthmark along the spine, muscle weakness, and poor reflexes. X-rays and CAT scans are used to diagnose Spina Bifida Occulta, as it is a defect in the lamina or the covering of the bones along the vertebrae. (Carson-DeWitt, 1999) Surgery can be performed to reverse the abnormality when performed at an early age, however after the child is 3 years old, it is likely the surgery will only slow or halt the progression. (Gregerson, 1997)

Spina Bifida Cystica is a slightly more complicated disorder. It is a common term for a condition know as myelomeninocell, meningomycele and myelodysoia. (Bloom & Selijeskog, 1998) These terms are used interchangeably, as different terms are used in different regions of the country. (Bloom & Selijeskog, 1998) This birth defect effects a section of the spinal cord, similar to Spina Bifida Occulta, but also effects the surrounding nerves, and prohibits them from developing normally. “Most often the areas of the maldeveloped spinal cord and nerves is enclosed in a thin-walled sac or cyst containing spinal fluid, hence the medical term ‘cystica.’ (Bloom & Selijeskog, 1998)” “Doctor’s… will usually recommend that the baby had surgery, often immediately, for repair and closing of the sac. (Bloom & Selijeskog, 1998) However, sometimes the doctor may deem the surgery too risky and supportive care only be recommended.

However, Spina Bifida is more than just a bone and nerve deformity. Due to skeletal abnormalities and nerve damage a whole variety of medical problems and infection can and often do occur. These infections and medical problems can sometimes prove fatal. Each of these secondary conditions is treated on an individual basis, as they are diagnosed.

One of the most frequent secondary conditions is associated with Spina Bifida is hydrocephalus. It affects 90% of children with Spins Bifida. (Yahoo Health, 2002) Hydro means water and cephalous means brain. This is an accurate description of this condition. Cerebral spinal fluid is produced in specialized areas of the brain known as choroids plexus, which are within the ventricular brain cavities. (Bloom & Selijeskog, 1998) “The average baby only needs a few tablespoons of cerebral spinal fluid to protect and bathe the brain. However, much more fluid is produced every day, the excess simply passes onto the brain’s surface where it is ultimately absorbed into the bloodstream. (Bloom & Selijeskog, 1998)” Babies with Spina Bifida, due to the open neural tube defect, have a drainage obstruction. This causes the cerebral spinal fluid to accumulate and therefore pressure on the brain will build. This pressure can cause the brain to rupture and the child to die.

The most general treatment for hydrocephalus is a narrow, soft tube called a shunt. This is place in one of the ventricular cavities in the brain and drain excess cerebral spinal fluid into another body cavity, relieving pressure. This tube goes under the scalp, behind the ear, down the neck, and to an area of the body where fluid is easily absorbed. The most common place for a shunt to run is the abdominal cavity outside the stomach and intestine. This is known as a peritoneal catheter. Less commonly, the shunt can run to the jugular vein and into the atrium of the heart where the fluid is drained away in the bloodstream. This is a ventriculoatrial shunt (Bloom & Selijeskog, 1998). Hydrocephalus is common in children with Spina Bifida due to the obstruction of the cerebral spinal fluid drainage system.

Orthopedic problems are also very common in children with Spina Bifida Cystica due to nerve damage. How bad the motor and sensory deficits are depends on how damaged nerves are. The most common orthopedic problem found in children with Spina Bifida is tallipes equinovans, more commonly know as clubfoot (Bloom & Selijeskog, 1998). This is when one of both feet point down and turn inward. This is commonly resolved by a series of casts changes weekly that move the foot into a more normal position (Gregerson, 1997). This will give the child a better chance of walking. If the child is able to walk, after the feet have been moved to a more normal position through serial casting, braces may be designed and worn to provide support (Bloom & Selijeskog, 1998).

Serial casting and braces sometimes fail to correct the foot’s position. A doctor may then recommend surgery. The major tendon may then be split allowing the foot to fall into a more normal position. Serial casting and braces usually follow (Bloom & Selijeskog, 1998).

Spinal deformities often occurs, kyphosis and scoliosis being the most common. Kyphosis is the condition where the spine is bent or curved forward and, when severe, can produce a hump. This can prevent a child from sitting up and surgery would be desirable (Bloom & Selijeskog, 1998).

Scoliosis is a spinal curvature that happens when muscles are paralyzed or weakened, making difficult for the spine to grow straight. “Children with Spina Bifida are more vulnerable to these problems because of the malformed vertebrae in the spine and because of weakened or paralyzed muscles controlling spinal muscles (Bloom & Selijeskog, 1998).”

It is easy to see how Spina Bifida is related to orthopedic disabilities and indirectly related to hydrocephalus. However, it is difficult for some people to understand why the bladder and kidneys are also affected. The nerves controlling the bladder are located in sacral portion of the spine, more specifically S2 to S5. Nearly all who are affected by Spina Bifida have involvement with the bladder nerves. Normally, when the bladder is full sensory nerves send a signal through the spinal cord that tell the bladder walls to contract and empty itself. The urine is then pushed through the urethra. Along the urethra are valves made of muscles called sphincters. There sphincters allow mature individuals to control when to urinate. Within children with Spina Bifida the sensory nerves of the bladder are impaired, so it is difficult to tell when the bladder is full. It is also difficult to empty the bladder, voluntarily or instinctively. The old urine in the bladder can result in chronic bladder infections or the urine may reflux back through the ureters into the kidneys causing infection there. “the Crede maneuver may be used to assist to empty (the bladder) more completely (Bloom & Selijeskog, 1998).” Nurses initially do it, but then parents soon learn how to perform this maneuver, where pressure is placed on the lower stomach and urine is pushed out of the bladder.

Similarly, the nerves controlling the rectal sphincters are also commonly affected. However, there is no concern of reflux, and therefore there is no concern of serious health problems. As the child gets older, regular bowel movement schedule should be used to avoid the need for diapers (Bloom & Selijeskog, 1998).

Children with Spina Bifida also experience disturbances in height. Very commonly, the children are short due to spine abnormalities within the spine. The only treatment currently known for this is hormone therapy, which is currently very controversial and the choice to use it must be made on an individual basis (Bloom & Selijeskog, 1998).

Seizures are also more common in children with Spina Bifida, and usually a diagnosis of epilepsy is made. Often the seizures are a result of a shunt malfunction or infection, but they can be also caused by “hydrocephalus, brain scarring from past infection, irritation around the shunt tube, or abnormal brain tissue arising from an associated birth defect (Bloom & Selijeskog, 1998).” If seizures occur, the shunt should be evaluated and repaired if necessary. Medication may then be used to control the seizures.

Nearsightedness and estrophia, or “crossed eyes” is also common as children with Spina Bifida are more prone to visual problems. An ophthalmologist can prescribe glasses to help these conditions or in severe conditions surgery may be necessary (Bloom & Selijeskog, 1998).

Spina Bifida is a defect that is more prominent than many people realize. While there really aren’t any cures for Spina Bifida, researchers are finding that folic acid and vitamin B taken during pregnancy can help prevent it (Probe, 2000). There is also no know cause for Spina Bifida, making it difficult to take any other precautions. It affects those who have it much more than what may be thought. Along with the surgeries to correct skeletal problems, there is a multiplicity of other medical problems as a result of the Spina Bifida. Many of these problems, such as hydrocephalus and infection can be fatal if they aren’t dealt with accordingly and quickly. Spina Bifida is more than just an imperfection in the spinal cord. It affects the child in many ways, and results in medical conditions that need to be watched over for the rest of the individual’s life.

Works Cited

Bloom, Beth-Ann & Selijeskog, M.D., Edwards. (1998). A parents guide to Spina Bifida. Minnesota: University of Minnesota Press

Carson-DeWitt, M.D., Rosalyn. (1999). Spina Bifida. Encyclopedia of Medicine,5

Gregerson, Douglas M. (1997). Clinical consequences of Spina Bifida Occulta. Journal of Manipulative & Physiological Theraputics.20(8), 546-550

Vitamin that prevents terrible birth defects is being ignored. (2000, January 31). Probe,8,2, 6-7

Yahoo Health. (2002). Myelomeningocele [Internet]. Retrieved February 6, 2002 from World Wide Wed: http://health.yahoo.com/health/dc/001558/o.html