Tethered spinal cord syndrome

Alternate names

Occult spinal dysraphism sequence; Tethered spinal cord syndrome; Segmental vertebral anomalies; Occult spinal dysraphism

Definition

Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.

Cause

• Tethered cord syndrome occurs when tissue attachments limit the movement of the spinal cord within the spinal column.
• In some cases, it may be the result of improper growth of the neural tube during fetal development, which is closely linked to spina bifida.
• Other potential causes include narrowing of the spinal column (with age), spinal cord injury, tumors, and infection.

Onset

In some cases, symptoms may be present at birth (congenital), while others may not experience symptoms until later in adulthood.

Signs and symptoms

• In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence.
• This type of tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida.
• Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge.
• This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time and may be exacerbated during sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age.
• Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord.
• Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia.
• This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.

Diagnosis

• For children younger than eight weeks of age , a tethered cord may be observed by using ultrasonography. Ultrasonography may still be useful through age 5 in limited circumstances.
• MRI imaging is often used to evaluate individuals with these symptoms, and can be used to diagnose the location of the tethering, lower than normal position of the conus medullaris, or presence of a tumor or fatty mass (lipoma).

Treatment

• In children, early surgery is recommended to prevent further neurological deterioration.
• Regular follow-up is important: retethering may occur in some individuals during periods of rapid growth and may be seen between five to nine years of age. If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain.
• In adults, surgery to free (detether) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms.
• Other treatment is symptomatic and supportive.

Prognosis

• With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy.
• However, some neurological and motor impairments may not be fully correctable.
• Surgery soon after symptoms emerge appears to improve chances for recovery and can prevent further functional decline.

NIH genetic and rare disease info

• NIH info on Tethered spinal cord syndrome

Tethered spinal cord syndrome is a rare disease.

Tethered spinal cord syndrome Resources
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