Tethered Spinal Cord Syndrome Health Article

Demographics

TSCS is a relatively rare disorder. Its exact frequency is unknown, mostly because of a general lack of research on the disorder, and because the mildest forms may never be detected. TSCS in all forms affect both sexes and all races and ethnic groups.

Causes and symptoms

Congenital TSCS is initiated by incomplete closure of the neural tube during embryonic development. During the eighteenth to twenty-second day of embryonic development, the beginning structure of the neural tube, which will become the spinal column and cord, is formed by ectodermal tissue on the back of the embryo that forms a groove, which deepens and forms into a hollow tube, still open dorsally along its length. The tube begins to close itself, starting in the thoracic region, then moving on toward the head and lumbar regions. During the twenty-eighth to forty-eighth day of development, ectodermal tissue in the tail area of the embryo forms a separate, short length of neural tube, the conus medullaris, whose anterior end meets and fuses with the main neural tube while the posterior forms the filum terminale. The conus medullaris also produces the cauda equinae nerves.

Symptoms of TSCS may be visible at birth or appear later, even in adulthood, but most often in childhood. The symptoms may be visible or behavioral. Various visible signs on the skin of the lower back, along and near the spinal cord, are:

• lipomas, or fatty tumors below the skin
• hairy patches
• spots of increased pigmentation
• dimples that may indicate dermal sinus tracts
• skin lesions
• skin tags or outgrowths
• angiomas, or port-wine stains

Behavioral symptoms manifest as:

• chronic lower back pains
• progressive scoliosis, or curvature of the spine
• foot deformities
• numbness and loss of sensation in the legs or feet
• awkward gait and stumbling
• weakness in legs or feet
• unequal growth in the legs or feet
• progressive loss of control over bladder and bowel functions (incontinence)
• urinary tract infections

Diagnosis

The initial indicators of TSCS are the physical and behavioral ones listed above. A newborn that carries any of the symptomatic skin defects should be diagnosed further for possible TSCS. Among the behavioral signs, a child will likely complain to parents of lower back pains, while other behavioral symptoms will become obvious to parents. An adult who shows any of the physical or behavioral symptoms should bring these to the attention of his family physician, who should suspect TSCS as the cause. Symptoms, physical or behavioral, may not appear until many years after birth, including well into adulthood, depending on the time of tethering, degree of stretching of the spinal cord, and severity of damage to the nerves of the cord.

The next steps in diagnosis of TSCS are taking x-ray images of the spine to detect bone abnormalities, followed by the application of diagnostic neuro-imaging by means of MRI (magnetic resonance imaging) to produce three-dimensional images of the spinal column and spinal cord. Since a defect in the spinal cord or column makes it likely that there are other defects in the cord, column, or brain, an entire imaging of the brain and spinal column are recommended. Electromyography (EMG) can be used to check for or assess damage to nerve conduction in the spinal cord and the nerves of the cauda equinae. Ultra-sound imaging can be used to monitor unborn infants for evidence of TSCS, should there be a reason to suspect it.

Since the muscles of the bladder are often affected by TSCS, urodynamics testing is recommended to discover the extent of the damage.

Treatment team

A family doctor is probably the person most likely to first link symptoms in a child or adult to TSCS, when parents bring in a child for a routine health check or because of the physical and behavioral signs and problems. Following the tentative diagnosis, the patient will be sent to neurologists, MRI imaging technicians, EMG technicians, urologists, surgeons and neurophysiologists if surgery is called for, and the personnel monitoring recovery.

Treatment

TSCS is corrected by surgery to detach the cord at its place of tethering. Follow-up examinations are necessary because the freed spinal cord sometimes becomes re-tethered to growing scar tissue.

In the case of tight filum terminale, the filum terminale is severed, allowing the cord to float freely.

Surgery for TSCS generally takes four to six hours, and is conducted according to the form of TSCS in the patient. The spinal column is opened from behind to reach the site of tethering. Neurophysiologists are present to monitor spinal cord and nerve functioning to reduce the risk of damage to nerves and other tissues.