Syringomyelia and Tethered Cord

In post-traumatic syringomyelia (sear-IN-go-my-EE-lia) a cyst or fluid-filled cavity forms within the cord. This cavity can expand over time, extending two or more spinal segments from the level of SCI.

The clinical symptoms for syringomyelia and tethered spinal cord are the same and can include:

• Progressive deterioration of the spinal cord
• Progressive loss of sensation or strength,
• Sweating
• Spasticity
• Pain
• Autonomic dysreflexia (AD)

What is a Tethered Spinal Cord?

Tethered spinal cord is a condition where scar tissue forms and holds the spinal cord itself to the dura, the soft tissue membrane that surrounds it. This scarring prevents the normal flow of spinal fluid around the spinal cord and impedes the normal motion of the spinal cord within the membrane.

Tethering causes cyst formation. It can occur without evidence of syringomyelia, but post-traumatic cystic formation does not occur without some degree of cord tethering.

Detection and Treatment

Syringomyelia and tethered spinal cord can occur from months to many decades after spinal cord injury.

Magnetic resonance imaging (MRI) detects cysts in the spinal cord, unless rods, plates or bullet fragments are present.

Syringomyelia can also be associated with spina bifida, spinal cord tumors, arachnoiditis, and idiopathic (cause unknown) syringomyelia. MRI has significantly increased the number of diagnoses in the beginning stages of syringomyelia. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining.

Tethered cord and syringomyelia are treated surgically. Untethering involves a delicate surgery to release the scar tissue around the spinal cord to restore spinal fluid flow and the motion of the spinal cord.

In addition, a small graft may be placed at the tethering site to fortify the dural space and decrease the risk of re-scarring.

If a cyst is present, a shunt may be placed inside the cavity to drain fluid from the cyst.

Surgery usually leads to improved strength and reduced pain. However, it does not always bring back lost sensory function.While surgery results in stabilization or modest improvement in symptoms for most people although, a delay in treatment may result in irreversible spinal cord injury.

Recurrence of syringomyelia after surgery may result in additional operations necessary. These operations may not be completely successful over the long term. Up to one half of those treated for syringomyelia have symptoms return within five years.

Chiari Malformation

Syringomyelia also occurs in people who have a congenital abnormality of the brain called a Chiari malformation. During development of the fetus, the lower part of the cerebellum protrudes from the base of the head into the cervical portion of the spinal canal.

Symptoms usually include vomiting, muscle weakness in the head and face, difficulty swallowing, and varying degrees of mental impairment.

Paralysis of the arms and legs may also occur. Adults and adolescents with Chiari malformation who previously showed no symptoms may show signs of progressive impairment, such as involuntary, rapid, downward eye movements.

Other symptoms may include dizziness, headache, double vision, deafness, an impaired ability to coordinate movement, and episodes of acute pain in and around the eyes.

Webcast: SCI & Syringomyelia

In this recording of an online discussion, Nurse Linda discusses the treatment and prevention of syringomyelia.

Resources

If you are looking for more information on syringomyelia and tethered spinal cord or have a specific question, our Information Specialists are available business weekdays, Monday through Friday, toll-free at 800-539-7309 from 9:00 am to 8:00 pm ET.

Additionally, the Reeve Foundation maintains a syringomyelia fact sheet with additional resources from trusted Reeve Foundation sources. Check out our repository of fact sheets on hundreds of topics ranging from state resources to secondary complications of paralysis.

We encourage you to reach out to syringomyelia support groups and organizations, including:

• American Syringomylia Association Project (ASAP) offers a nationwide clearinghouse for information on Chiari/syringomyelia.
• Conquer Chiari provides extensive resources and education on chiari and syringomyelia.

Sources: National Institute of Neurological Disorders and Stroke,American Syringomyelia & Chiari Alliance Project