WHAT IS SYRINGOMYELIA MALFORMATION? 

Learn more about how syringomyelia (a syrinx in the spinal cord) is identified, diagnosed and treated.

basics of chiari-related diseases:

What is Syringomyelia?

SYRINGOMYELIA INFORMATION PAGE (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
  • Paralysis

The above symptoms typically 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 3 important components for appropriate diagnosis and treatment of a syrinx:

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

HOW IS SYRINGOMYELIA TREATED?

Syringomyelia is treated based on its 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 of syringomyelia 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. A residual central canal is a thin, fluid-filled structure within the spinal cord that has 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 syrinxes, however, are more complicated and depend on good patient and doctor communication.

RESOURCES

Print-Out for Use at Appointments + To Share

Syringomyelia Fact Sheet (PDF). Syringomyelia is a difficult, complex and fairly rare disorder. It is not uncommon that some medical professionals need their memory jogged to appropriately treat their patients. Sometimes, the most experience a clinician will have with rare disorders is a single paragraph in a textbook from medical school. We encourage you print out this fact sheet and bring it to your appointment if your physician, nurse, PA or therapist does not see syrinx patients all the time. Foster a dialogue and talk about what symptoms and concerns on the sheet that you have. It may even help them make better recommendations and suggestions for your treatment!

You can also use this print-out at any events that you are hosting to raise awareness.

Other Reputable Sites with Information

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

 

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Revised on 05/2022