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Syringomyelia

Definition

Syringomyelia is a fluid-filled hole that forms in the spinal cord. Over time, it damages the spinal cord.

Alternative Names

Syrinx

Causes

The fluid buildup in syringomyelia may be caused by:

  • Birth defects (especially "chiari malformation," in which part of the brain pushes down onto the spinal cord at the base of the skull)
  • Spinal cord trauma
  • Tumors of the spinal cord

The fluid-filled hole usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.

Symptoms

There may be no symptoms, or symptoms may include:

  • Headache
  • Loss of muscle mass (wasting, atrophy), often in the arms and hands
  • Muscle function loss, loss of ability to use arms or legs
  • Numbness that decreases the feeling of pain or temperature; lowers the ability to feel when the skin is being touched; occurs in the neck, shoulders, upper arms, and trunk in a cape-like pattern; and slowly gets worse over time
  • Pain down the arms, neck, or into the middle back or legs
  • Weakness (decreased muscle strength) in the arms or legs

Other symptoms that may occur with this disease are:

Exams and Tests

A nervous system (neurologic) exam may show a loss of feeling or movement caused by pressure on the spinal cord.

Other tests that may be done include:

Treatment

The goals of treatment are to stop the spinal cord damage from getting worse and to improve function.

Surgery may be needed to relieve pressure in the spinal cord. Physical therapy may be needed to improve muscle function.

A person with syringomyelia may need to have ventriculoperitoneal shunting, in which a catheter (thin, flexible tube) is inserted to drain the fluid buildup.

Outlook (Prognosis)

Without treatment, the disorder may get worse very slowly. Over time, it may cause severe disability.

Surgery usually stops the condition from getting worse. Nervous system function will improve in about half the people who have surgery.

Possible Complications

Without treatment, the condition may lead to:

  • Loss of nervous system function
  • Permanent disability

Possible complications of surgery include:

  • Infection
  • Other complications of surgery

When to Contact a Medical Professional

Call your health care provider if you have symptoms of syringomyelia.

Prevention

There is no known way to prevent this condition, other than avoiding injuries to the spinal cord. Getting treated right away slows the disorder from getting worse.

References

Rekate HL. Spinal cord disorders. In: Kliegman RM, Stanton BF, St. Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 598.


Review Date: 5/28/2014
Reviewed By: Joseph V. Campellone, MD, Division of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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