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Learn more about what Chiari is, what to do after getting a Chiari diagnosis and find more resources that may help.

basics of chiari-related diseases:

What is syringomyelia (SM)?

QUICK FACTS: Syringomyelia 1-Page Fact Sheet (PDF)

Syringomyelia (sear-IN-go-my-EEL-ya) (SM) occurs when a cavity, sometimes called a “syrinx”, is formed inside the spinal cord. The syrinx is caused by a build-up of fluid, resulting from a blockage.

That blockage can either be from a Chiari malformation, a spinal trauma, a tumor, or some other cause. Syringomyelia can cause stretching and, eventually, permanent injury to nerve fibers. Syringomyelia can sometimes be called something different, depending on the person—these other names may include hydromyelia, syringohydromyelia or spinal cord cyst.

WHAT ARE THE SYMPTOMS OF SYRINGOMYELIA?

Patients may have a range of symptoms, but the most common are:

  • Numbness
  • Tingling
  • Pain
  • Weakness

*These symptoms frequently occur in the arms and legs.

HOW IS SYRINGOMYELIA DIAGNOSED?

Syringomyelia is diagnosed by MRI. The primary cause of the syrinx must be discussed with your doctor. Sometimes, there is no clear reason for the SM—this is often called idiopathic syringomyelia.

There are three important components for appropriate diagnosis and treatment of a syrinx:

  1. Patient history and symptoms
  2. Clinical exam consistent of SM signs
  3. Brain and spinal MRI (and MRI of both will help determine the syrinx cause)

HOW IS SYRINGOMYELIA TREATED?

Syringomyelia is treated based on “etiology”, or the underlying cause of the syrinx. Patients with no symptoms and no clear etiology are best managed with watchful waiting and regular check-ups.

A symptomatic or large syringomyelia is treated by surgery. Different causes include:

  • Chiari I malformation
  • Spina bifida (related to Chiari II malformation)
  • Tethered cord (a.k.a. – spina bifida occulta)
  • Arachnoiditis (a.k.a. – spinal inflammation)
  • Spinal trauma
  • Spinal cord tumor
  • Idiopathic – unknown cause

Some patients are referred with what is called a residual central canal. This is a thin, fluid-filled structure within the spinal cord with no associated pathology. It is usually an incidental finding on MRIs obtained for other issues. These patients are usually neurologically normal on exams. In these patients who have back pain, other causes for the pain should be investigated.

WHAT IS THE PROGNOSIS FOR SYRINGOMYELIA?

Prognosis is largely dependent on the cause of the syrinx. Usually, if the cause is known and can be effectively treated, long-term improvement and disappearance of the syrinx is good. Some, however, are more complicated and depend on good patient-doctor communication.

OTHER RESOURCES

National Institutes of Neurological Disorders and Stroke (NINDS)/National Institutes of Health (NIH) – Syringomyelia Information Page