Syringomyelia

What Is Syringomyelia?

Syringomyelia is a long-term condition that causes fluid-filled cysts, which doctors call “syrinx,” to form inside your spinal cord. You may also hear it called hydromyelia, syringohydromyelia, or Morvan disease.

You may not have symptoms, or even be aware that you have it. But a severe case can cause problems that need to be treated.

Syringomyelia is most common in adults ages 20 to 40, but it can happen to people of all ages. Men are more likely to get it than women.

Syringomyelia Symptoms

Symptoms depend on the location and size of the cyst. Over time, if it grows wider and longer, it can damage the nerves in the center of your spinal cord. This can lead to chronic pain that’s hard to treat.

Symptoms usually happen slowly over many years. But they can appear suddenly after an accident, like a fall.

Other symptoms you may have include:

  • Curving of the spine, called scoliosis
  • Changes in or loss of bowel and bladder function
  • Heavy sweating
  • Not being able to feel hot and cold in the fingers, hands, arms, and upper chest
  • Loss of reflexes
  • Muscle stiffness that may make it hard to walk
  • Muscle weakness, especially in the arms, hands, and shoulders
  • Feeling numbness, tingling, burning, or piercing
  • Pain in the neck, shoulders, and sometimes arms and hands
  • Paralysis (in severe cases)
  • Headaches
  • Sexual problems
  • Swings in blood pressure levels
  • Twitches, or involuntary muscle contractions
  • Uncoordinated movements

If the cyst damages a nerve that controls the muscles in your eyes and face, you may get what doctors call Horner syndrome. It causes droopy eyelids, narrowing of the opening between the eyelids, reduced pupil size, and decreased sweating on the affected side of the face.

Syringomyelia Causes

You get this condition when something blocks the normal flow of cerebrospinal fluid, which protects your spinal cord and brain. It enters the central canal of the spinal cord and can cause a cyst to form.

With syringomyelia, there are two main reasons this fluid can get blocked or rerouted: a birth defect or trauma.

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Congenital syringomyelia, also called communicating syringomyelia, usually happens when there’s a problem with the baby’s brain development during pregnancy. A defect that doctors call a “Chiari I malformation” causes the lower part of the baby’s brain to push downward into their spinal canal. This blocks the normal flow of fluid between the spine and the brain, and a cyst forms. But not everyone with a Chiari I malformation gets this disorder. Symptoms usually show up between ages 25 and 40.

Acquired syringomyelia, also called primary spinal or noncommunicating syringomyelia, is when a cyst forms in a damaged part of the spinal cord. It may happen when there is:

  • Swelling of the covering of the spinal cord
  • Meningitis
  • Spinal cord injury
  • Spinal tumor
  • Tethered spinal cord

Sometimes, it happens for reasons doctors don’t understand. They call this “idiopathic syringomyelia.”

Syringomyelia Diagnosis

Doctors use magnetic resonance imaging (MRI) scans of the brain and spine to see if you have syringomyelia. The MRI can show a cyst or other condition.

Sometimes, doctors discover you have this condition when doing an MRI for another health reason.

You might also have a kind of X-ray procedure called a CT scan of your spine.

Syringomyelia Treatment

What kind of treatment you get for syringomyelia depends on whether you have symptoms, and how serious they are.

Monitoring: You probably won’t need treatment if you don’t have symptoms or if they’ve mild. But you should have regular checkups to watch for cyst growth and changes in signs and symptoms. The doctor may tell you to avoid activities like heavy lifting that involve straining. They might also prescribe medicine for pain or refer you to physical therapy.

Surgery: If your symptoms cause problems, you may need surgery to restore the normal flow of the cerebrospinal fluid. The type of surgery you’ll have depends on what caused your syringomyelia. For example, if the problem is due to a Chiari I malformation, your doctor might do a procedure called posterior fossa decompression. They’ll remove the bone at the back of your skull and spine. It creates more space for your brainstem and cerebellum, the part of the brain that controls your movements and balance. Talk to the doctor about the risks and benefits of surgery.

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Syringomyelia Outlook

Surgery for syringomyelia doesn’t always fix the problem. Fluid can build up again. And sometimes, the damage to your nerves and spinal cord is permanent.

You’ll need regular follow-ups with your doctor, including MRIs, to check whether the cyst has come back.

WebMD Medical Reference Reviewed by Neha Pathak, MD on September 28, 2020

Sources

SOURCES:

National Organization for Rare Disorders: “Syringomyelia.”

American Syringomyelia & Chiari Alliance Project: “Syringomyelia.”

National Institute of Neurological Disorders and Stroke: “Syringomyelia Fact Sheet.”

Mayfield Brain & Spine: “Chiari I Malformation.”

Mayo Clinic: “Syringomyelia.”

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