About Syringomyelia

General Information

• Syringomyelia is a condition in which a cyst (pocket of fluid) forms within the spinal cord.
• This cyst, called a syrinx, can expand over time, damaging the center of the spinal cord. Since the spinal cord connects the brain to nerves in the arms and legs, this damage can result in pain, weakness and stiffness in the back, shoulders, arms or legs.

What causes syringomyelia?

• Syringomyelia is a frequent finding in patients with Chiari I malformation.
• Syringomyelia may occur as a complication of spine trauma, meningitis, hemorrhage, a tumor or arachnoiditis. These cases are sometimes termed “primary spinal syringomyelia.”

Symptoms

• Highly variable presentation with a slowly progressive course, often over years. The characteristic findings include:
  • Sensory loss in a "cape" distribution (over the tops of the shoulders); loss of pain and temperature sensation with preserved touch and position sense.
  • Cervical and occipital pain, extremity pain.
  • Hand and arm weakness and atrophy.
  • Spasticity.
• In primary spinal syringomyelia, symptoms may appear months or even years after the initial injury, starting with pain, weakness and sensory impairment often originating at the site of trauma.

Diagnosis

• Magnetic resonance imaging (MRI) is the diagnostic study of choice since it will accurately depict the structure of the spinal cord. In some cases, this should include a contrast injection to look for a spinal cord tumor.
• Computed Tomography (CT)-myelogram is often very helpful in cases of primary spinal syringomyelia.

Treatment

• Syringomyelia nearly always requires treatment.
• In cases associated with Chiari malformation, surgery for the Chiari malformation usually reduces in the size of the syrinx cavity.
• Primary spinal syringomyelia is difficult to treat, and options include:
  • Laminectomy and duraplasty in order to re-establish free cerebrospinal fluid flow across an area of scarring.
  • Thecoperitoneal shunting, a procedure pioneered by UCLA neurosurgeons.
  • Direct shunting of the syrinx cavity.