Syringomyelia

Definition

Syringomyelia is damage to the spinal cord due to a fluid-filled hole that forms in the 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 cavity 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)
• Muscle function loss, loss of ability to use arms or legs
• Numbness or decreased feeling
  • Decreased feeling of pain or temperature
  • Less ability to feel when the skin is being touched
  • Neck, shoulders, upper arms, trunk -- in a cape-like pattern
  • 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:

• Spasms in, or tightness of the leg or hand and arm muscles
• Uncoordinated movement

Exams and Tests

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

Other tests that may be done include:

• MRI of the spine
• MRI of the head
• Spinal CT scan with myelogram (rarely)

Treatment

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

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

The health care provider may need to drain the fluid buildup. See: Ventriculoperitoneal shunting

Outlook (Prognosis)

Without treatment, the disorder gets worse very slowly. Over time it will cause severe disability.

Surgery usually stops the condition from getting worse. About 50% of people will have improvements in nervous system  function after surgery.

Possible Complications

Without treatment, the condition will 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: Saunders Elsevier; 2011:chap 598.

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