Syringomyelia is a condition in which a cyst filled with cerebrospinal fluid (CSF) called a syrinx forms within your spinal cord. The syrinx can compress your spinal cord and cause neurological symptoms.
Syringomyelia is a condition in which a fluid-filled cyst called a syrinx forms within your spinal cord. The syrinx can get bigger and elongate over time, resulting in damage to your spinal cord and compression and injury of the nerve fibers that carry information from your brain to the rest of your body and vice versa.
The fluid in the cyst is cerebrospinal fluid (CSF). This fluid normally surrounds and protects your brain and spinal cord. CSF also fills connected cavities within the center of your brain called the ventricles, which continue to a small central canal that runs the length of your spinal cord.
If you have syringomyelia, CSF collects within the tissue of your spinal cord, expands the central canal and forms a syrinx. Generally, a syrinx develops when the normal flow of CSF fluid around your spinal cord or lower brain stem is disturbed. When syrinxes affect your brain stem, the condition is called syringobulbia.
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The majority of people with syringomyelia are diagnosed between the ages of 20 and 50, but it can also develop in young children or older adults.
Syringomyelia is rare. It affects approximately every 8 out of 100,000 people and accounts for up to 5% of paraplegia (paralysis of your legs and lower body) cases.
Symptoms of syringomyelia usually appear slowly and worsen over many years. Some people may have a small syrinx for a long time without having any symptoms. Symptoms also vary based on where the syrinx forms along your spine, how large it is and how long it extends. Symptoms can occur on one or both sides of your body and be mild or severe.
Symptoms of syringomyelia include:
In addition to the symptoms above, symptoms of syringomyelia that are specific to children include:
If you or your child are experiencing these symptoms, it’s important to see your healthcare provider as soon as possible.
Scientists don’t yet know the exact, underlying reason for the formation of a syrinx. Most theories suggest that the obstruction or disruption of the flow of cerebrospinal fluid (CSF) is the most likely cause.
There are two main groups of associated causes of syringomyelia: congenital (from birth) and acquired (developed later in life).
The majority of syringomyelia cases are associated with Chiari malformation type 1, which occurs when the lower part of your cerebellum extends into the hole (the foramen magnum) at the base of your skull. The spinal cord normally passes through this hole. This structural issue obstructs the normal flow of CSF.
In most cases, Chiari malformation is present at birth (congenital), but people with Chiari malformation type I may not have any symptoms or they may not develop symptoms until they’re adolescents or adults.
Other congenital causes of syringomyelia include:
Acquired (developed later in life) causes of syringomyelia include:
In some cases, syringomyelia can develop without any known cause. This is called idiopathic syringomyelia.
To diagnose syringomyelia, your healthcare provider will review your medical history and perform a physical exam focusing on neurological function. They may refer you to a neurologist, a provider who specializes in disorders of the brain and nervous system.
Your provider will likely order imaging tests of your spine or brain. In some cases, healthcare providers discover syringomyelia accidentally on imaging tests that were ordered for another medical reason.
If your healthcare provider suspects you have syringomyelia, they may recommend one or more of the following tests:
The treatment for syringomyelia depends on the severity and progression of your symptoms.
Healthcare providers usually don’t treat syringomyelia if it isn’t causing any symptoms. However, providers recommend having the syrinx carefully monitored by a neurologist or neurosurgeon since symptoms can develop or worsen over time. Monitoring typically involves periodic MRI scans and neurological exams.
For people who are experiencing symptoms, the general goals of treatment for syringomyelia include:
Treatment to fix the underlying cause and prevent further damage to your spinal cord usually involves some type of surgery. General therapies to manage symptoms include:
If you have syringomyelia that’s causing symptoms or the syrinx is getting bigger, your neurologist or neurosurgeon will likely recommend surgery. There are two general forms of surgery: restoration of normal CSF flow around your spinal cord and direct drainage of the syrinx. The type of surgical treatment depends on what’s causing the symptoms.
After surgery, your provider will evaluate the syrinx to make sure that it stabilizes or decreases in size by using an MRI scan.
Surgery for syringomyelia can often lead to an improvement of symptoms and stabilization of the condition for many people. However, syringomyelia can recur (come back) after successful treatment, which may require more procedures.
Complications following surgery to treat syringomyelia can include:
It’s important to talk to your neurosurgeon about these risks and their likelihood of happening in your specific surgery.
While syringomyelia generally can’t be prevented, early diagnosis and treatment may help prevent the worsening of symptoms.
It’s important to remember that syringomyelia has a wide range of symptoms, severity and causes. In addition, people can have different rates of progression of the condition or no progression at all. These factors make it difficult for healthcare providers to predict the prognosis (outlook) for syringomyelia.
Early surgery usually minimizes symptoms and has better outcomes. Although the progression of neurologic deficits usually stabilizes after surgical treatment, and the symptoms sometimes improve, people often remain symptomatic.
In general, a syrinx diameter of more than five millimeters (mm) and associated swelling (edema) generally indicate a worse prognosis.
Myelopathy (injury to your spinal cord due to severe compression) is a major complication and concern of syringomyelia. It can eventually lead to paraplegia/quadriplegia, recurrent pneumonia and bowel and bladder dysfunction.
If you have syringomyelia, consider the following actions to help manage your symptoms:
If you’ve been diagnosed with syringomyelia, you’ll need to see your healthcare team regularly to monitor your symptoms and the syrinx — whether you’ve had surgical treatment or not.
A note from Cleveland Clinic
Learning that you have a spinal cord condition can be overwhelming. Know that you're not alone and many resources are available to help you. It’s important that you speak with a healthcare provider who’s very familiar with syringomyelia so you can learn more about how you may be affected by it. Don’t be afraid to ask your healthcare team questions — they’re available to help you.
Last reviewed by a Cleveland Clinic medical professional on 04/20/2022.
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