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What

The spinal cord extends from the base of the brain down through the spinal column into the lower back. Shortly after conception, special cells come together to form a neural tube that will become the spinal cord. If this tube does not close completely, the spinal cord can become abnormally attached within the spine, or tethered. Normally, the spinal cord is able to move freely when a child bends or stretches, but with Tethered Cord Syndrome, the cord is not able to freely move, so it can stretch itself. This tension can cause permanent damage to the nerves of the legs, feet, bowel and bladder.

Who

This condition is associated with children who are born with spina bifida, a separate condition known as a neural tube defect in which the spinal column does not close properly and the spinal cord is exposed. The incidence of spina bifida is approximately 6 per 100,000 babies yet Tethered Cord Syndrome does not occur in each of these cases. However, it is rather a common complication in patients with spina bifida. Exact incidence is unknown because many cases are not diagnosed until adulthood, if ever. The number of affected males is equal to females and this disease does not favor any particular ethnic group.

Signs and Symptoms

A person with this syndrome may have one or more of the following signs and symptoms: Skin findings on the lower back: o Fatty mass o Hairy patch or discoloration o Skin tags o Dimples (These findings suggest spina bifida occulta, an abnormality of the lower spine and a condition that predisposes people to develop Tethered Cord Syndrome. More information below under “Possible Causes”.) Orthopedic Problems: o Persistent back pain o Scoliosis (curvature of the spine) o Loss of sensation in the legs or feet o Unequal changes in the size of the legs or feet o Stumbling or walking problems o Weakness in legs or feet Bowel or bladder problems: o Changes in bowel control o Incomplete emptying of the bladder o Frequent urinary tract infections o Changes in bladder pressure, measured by special testing referred to as urodynamics o Difficulty in toilet training younger children o Diapers always wet in-between changes

Possible Causes

As mentioned earlier, this disease is a common complication associated with spina bifida. A major cause of spina bifida is folate deficiency during pregnancy and the risk of spina bifida will drop dramatically if the mother takes prenatal vitamins. There are two ways the spinal cord can become tethered from spina bifida. First, if a child is born with the regular form of spina bifida, in which the defect is readily visible in the lower back of the newborn, corrective surgery to close the spinal cord within the spinal column is done right away. The surgery, although necessary, can lead to Tethered Cord Syndrome because of the build up of scar tissue that may later restrict the movement of the spinal cord within the spinal column. Spina bifida occulta is a form of the disease in which the defect in the lower spine is much smaller and not visible on the exterior. It can elude diagnosis for several decades or for a patient’s entire life. This form can also lead to Tethered Cord Syndrome, and affected patients usually present with the symptoms listed above for “Skin findings on the lower back”. This form of spina bifida is also responsible for the adult-onset symptoms of Tethered Cord Syndrome, since these patients have a very small defect that slowly accumulates injury over the years.

Diagnosis

Early diagnosis is very important in order to surgically correct the tethering before further damage occurs to the spinal cord caused by repetitive stretching. If a child has any combination of the symptoms mentioned above, x-rays of the spine will usually be ordered to look for any bony abnormalities that may be pinching on the nerves as they exit from the spinal cord. An MRI is a much better diagnostic test as it produces a 3-dimensional image of the spine and spinal cord which can allow for a definitive diagnosis. Some patients go undiagnosed until adulthood because their symptoms are either mild or even absent for decades before they become severe enough to necessitate a visit to the physician for an evaluation.

Treatment

If a child is diagnosed with this condition it is recommended that the child have the condition corrected by surgically releasing the spinal cord so it can hang freely within the spinal column. The procedure usually lasts between 4 and 6 hours and will require several days of hospitalization to ensure the surgical site heals properly. Depending on the extent of neurological effects, patients may undergo extensive physical therapy to restore strength and function in the lower limbs.

Prognosis

The goal of the surgery is to prevent further deterioration and hopefully alleviate the symptoms that were present at diagnosis. With treatment, patients have a normal life expectancy. If not treated, 90% of patients will develop irreversible neurological problems. If repaired, there is less than a 2% chance that new symptoms will appear as a result of the surgical procedure. Back pain usually improves but bladder dysfunction usually does not. Leg strength may improve, and there is a 50% chance that sensation and motor problems will return to normal.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Spina Bifida Association
Great source for up-to-date information.

University of Missouri Health Care: Family Guide to Neuromedicine
about the surgical procedure and the recovery process.

Lucile Packard Children’s Hospital at Stanford
Information about this disorder and neurological disorders as a whole.

BrainTalk Communities
A message board with helpful advice and questions to ask your physician.

Google Search for Tethered Cord Syndrome

References and Sources

University of Missouri Health Care: Family Guide to Neuromedicine www.muhealth.org/~neuromedicine/tetheredcord.shtml Lucile Packard Children’s Hospital at Stanford www.lpch.org/clinicalSpecialtiesServices/COE/BrainBehavior/Neurosurgery/tetheredCord.html Wrong Diagnosis.com www.wrongdiagnosis.com/t/tethered_spinal_cord_syndrome/intro.htm