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Syringomyelia (also known as hydromyelia and syringohydromyelia) occurs when fluid collects within the spinal cord and creates a cyst. This type of cyst, referred to as a syrinx, grows over time. It typically begins in the neck, but can extend down the length of the spinal cord. Without treatment, a syrinx can damage the center of the spinal cord, causing permanent injury.
In most cases, syringomyelia is considered a symptom of other conditions, including those listed below.
Chiari Malformation Type 1
The most common condition associated with syringomyelia is the Chiari malformation Type I. It is believed that crowding of tissue from the cerebellum located at the opening of the base of the skull can cause spinal fluid blockage. As a result, fluid builds up within the spinal cord. When Chiari malformation is associated with syringomyelia, surgery usually is recommended to create more space at the base of the skull and upper neck.
Spina Bifida
The second most common cause of a syrinx in the spinal cord is Spina Bifida (myelodysplasia). Tension related to tethering of the bottom or the middle of the spinal cord leads to a stretch injury that can then obstruct the flow of fluid within the central canal of the spinal cord. This produces the fluid backup seen in syringomyelia. This type of syrinx is best treated with surgery to untether the spinal cord.
Spinal Cord Trauma
Trauma to the spinal cord is the third most common cause of syringomyelia. When the trauma is low level (e.g., the mass of a bulging disk creating compression of the spinal cord which reacts by forming a syrinx), treatment involves surgery to remove the compressing mass. In the case of severe spinal cord injury leading to paralysis of the legs or arms and legs, there currently is no standard treatment specifically for the syrinx.
Spinal Tumor
In some cases, a tumor within the spinal cord can physically block the flow of fluid in the central canal. This type of syrinx generally is treated by surgical removal of the tumor.
Other Causes
In rare circumstances, physicians are unable to pinpoint the specific cause of syringomyelia. In this situation, neurosurgeons insert a small drainage tube into the syrinx cavity to allow the fluid to drain directly to a space outside of the spinal cord. While this treatment does not address the underlying cause of the condition, it usually relieves symptoms.
An interdisciplinary team of adult and pediatric neurosurgeons, neurologists and pain management specialists coordinates the care of adults and children who have syringomyelia. The same clinicians who treat patients also conduct research on Chiari malformation, tethered cord syndrome and other causes of syringomyelia. Their research is contributing to increased understanding of the condition and improved treatment strategies for our patients.
