Neurosurgical Diseases and Disorders (M-Z) / 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.
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