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Tethered Spinal Cord

Under normal circumstances, the bottom of the spinal cord floats freely within the bones of the vertebral column so that growth and movement will not damage the nerves. In some children, improper development resulting in a bony protrusion, tumor, fatty deposit, or scar tissue, may pin the spinal cord somewhere in the lower back. This results in a condition called tethered spinal cord, in which the spinal cord is tugged between the lower back and the brain stem. Because the vertebral column grows more quickly than the nerves that make up the spinal cord, tethering will cause increased tension on the cord as a child grows. In addition, activity and scoliosis (a curvature of the spine) will cause additional abnormal stretching of the spinal cord, leading to a wide range of neurological symptoms. Unless treated, this condition will worsen over time. Children with spina bifida, a birth defect in which the spine column does not close, often have tethered spinal cord.

Symptoms

Children with tethered spinal cord can experience a wide range of symptoms. The condition can be identified by a lesion, discoloration, patch of hair, deep dimple, or fatty tumor on the lower back. This usually is accompanied by low-back pain; deformities of the leg, hip, and foot; problems walking; weakness and loss of sensation in the legs; scoliosis; and incontinence. The symptoms may increase gradually, and therefore may not be recognized until significant nerve problems appear. Some of these neurological deficits may be permanent. If the condition continues into adulthood, the strain on the spinal cord will increase, leading to increasing sensory and motor problems, as well as loss of bladder and bowel control.

Diagnosis

Tethered spinal cord often is first noticed by telltale signs on the lower back, such as a lesion, discoloration, patch of hair, deep dimple, or fatty tumor. If tethered spinal cord is suspected, patients will undergo magnetic resonance imaging (MRI) scans, spine x-rays, and computed tomography (CT) scans to identify the cause and site of the tethering, as well as electromyography, an evaluation of nerve function. In infants, the condition may be recognized on an ultrasound.

Treatment

Surgery is the primary treatment for tethered spinal cord, and early surgery is recommended to prevent deterioration of nerve function. For this procedure, neurological surgeons open the lower back to expose the site where the spinal cord is pinned, and to free it by removing a protrusion or fixing a developmental abnormality. The surgery, which may be difficult, always is accompanied by careful neurophysiological monitoring to minimize damage to nerves. After surgery, there is a risk that the spinal cord may become retethered, so ongoing monitoring is necessary.

In cases in which open surgery is not an option, surgeons may cut nearby spinal cord nerve roots to ease the pain associated with the condition. Other treatment for tethered spinal cord is limited to addressing specific symptoms with medication or rehabilitation. If a child with tethered spinal cord has reached adult height without experiencing symptoms, immediate treatment may not be necessary. However, because movement gradually will increase wear on a tethered spinal cord, surgery may be required even after growth has stopped.

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