Syringomyelia

What is syringomyelia?

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.

The upper portions of your spinal cord (cervical and thoracic) are often affected by syringomyelia.

Who does syringomyelia affect?

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.

How common is syringomyelia?

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.

What are the symptoms of syringomyelia?

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:

• Headaches.
• Pain down your arms, neck, into the middle of your back or your legs (radiculopathy).
• Progressive weakness and muscle atrophy in your arms and/or legs.
• Muscle spasms or tightness in your back, shoulders, neck, arms or legs.
• Loss of sensitivity to pain or temperature, especially in your hands.
• Numbness or tingling in your arms and/or legs.
• Balance problems and difficulty walking.
• Loss of bowel and bladder control.
• Issues with sexual function.

In addition to the symptoms above, symptoms of syringomyelia that are specific to children include:

• Scoliosis (curvature of the spine).
• Toe walking.

If you or your child are experiencing these symptoms, it’s important to see your healthcare provider as soon as possible.

What causes syringomyelia?

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).

Congenital causes of syringomyelia

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:

• Myelomeningocele: Myelomeningocele (also known as open spina bifida) is a birth defect in which your backbone (spine) and spinal canal don’t close before birth. It’s a type of neural tube defect (NTD).
• Tethered cord syndrome: Tethered cord syndrome is a rare neurological condition in which your spinal cord is attached (tethered) to the surrounding tissues of your spine. It’s often associated with spina bifida and scoliosis.

Acquired causes of syringomyelia

Acquired (developed later in life) causes of syringomyelia include:

• Spinal cord injuries: Syringomyelia that results from a spinal cord injury is sometimes called post-traumatic syringomyelia. The development of post-traumatic syringomyelia may occur many months or years after the initial traumatic injury. It’s usually due to a buildup of scar tissue that blocks the flow of CSF.
• Spinal cord tumors: Spinal cord tumors, especially hemangioblastomas and ependymomas, can interfere with the normal circulation of CSF, resulting in syringomyelia.
• Inflammation of the arachnoid membrane (arachnoiditis): The arachnoid membrane is one of the membranes that surrounds and protects your spinal cord. Inflammation of this membrane can result in syringomyelia. Inflammation could be due to conditions such as sarcoidosis, transverse myelitis and multiple sclerosis (MS).
• Meningitis: Meningitis is an infection of the meninges, which are a protective lining around your brain and spinal cord. This can lead to syringomyelia.

In some cases, syringomyelia can develop without any known cause. This is called idiopathic syringomyelia.

How is syringomyelia diagnosed?

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.

What tests will be done to diagnose this condition?

If your healthcare provider suspects you have syringomyelia, they may recommend one or more of the following tests:

• MRI (magnetic resonance imaging) scan: An MRI scan is the most reliable way to diagnose syringomyelia. An MRI takes detailed pictures of structures inside your body using a large magnet and a computer. Using this test, your provider will be able to determine if there’s a syrinx in your spine or another abnormality, such as a tumor. An MRI readily reveals the location, size and extent of the syrinx.
• Dynamic MRI: This type of MRI can show the flow of cerebrospinal fluid (CSF) around your spinal cord and within the syrinx. It may require a contrast agent, which is injected into one of your veins, to enhance the MRI images.
• Myelogram with CT scan: If you’re unable to undergo an MRI, a CT scan is the next choice. A myelogram is a diagnostic imaging test that uses a contrast dye and computed tomography (CT) to look for issues in your spinal canal.

How is syringomyelia treated?

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:

• Treating the underlying cause of the syrinx, if possible.
• Managing the symptoms.
• Preventing further damage to your spinal cord by restoring the proper flow of cerebrospinal fluid.

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:

• Pain management and medications.
• Physical therapy and rehabilitation.
• Limiting certain activities, especially those that cause strain on your spine, such as lifting heavy options and jumping.

What are the types of surgery for syringomyelia?

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.

Surgeries include:

• Treating Chiari malformation: The goal of Chiari malformation surgery is to provide more space at the base of your skull and upper neck, which reduces pressure on your brain and spinal cord and restores the normal flow of CSF. The most common surgery for this is called posterior fossa decompression. With this procedure, a neurosurgeon creates room by removing small pieces of bone in the back of your skull. Surgery can allow the syrinx to drain, sometimes becoming smaller or even disappearing entirely. Symptoms may improve even if the syrinx remains the same size or is reduced only slightly.
• Treating or preventing post-traumatic syringomyelia: In the case of trauma- or injury-related syringomyelia, the main goal is to prevent a syrinx from developing or growing in the first place. This surgery is called expansive duraplasty and involves removing scar tissue around your spinal cord and sometimes adding a patch to expand the membrane that surrounds your spinal cord (the dura mater). By clearing and expanding the space around your spinal cord, CSF may be able to flow as it normally does.
• Removing the obstruction: Surgical removal of obstructions such as scar tissue, bone from your spinal canal or tumors can help restore the normal flow of CSF. If a tumor is causing syringomyelia, removing it almost always eliminates the syrinx. In some cases, your provider may recommend radiation therapy to shrink the tumor.
• Draining the syrinx: If there’s no identifiable cause of the syrinx, your provider may recommend draining it, especially if it’s growing. To perform this procedure, a neurosurgeon inserts a drain called a stent or shunt into the syrinx. A stent (a small tube) allows fluid from within your spinal cord to drain into the space just outside of it. Similarly, a shunt consists of a flexible tube and a valve that drains the syrinx fluid into another part of your body, usually your abdomen or chest. By draining the syrinx, both of these procedures may stop the progression of 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.

What are the possible complications of surgery to treat syringomyelia?

Complications following surgery to treat syringomyelia can include:

• Cerebrospinal fluid (CSF) leaks.
• Infection.
• Hemorrhage (bleeding).
• Further spinal cord injury.
• Recurrence of the syrinx.

It’s important to talk to your neurosurgeon about these risks and their likelihood of happening in your specific surgery.

Can syringomyelia be prevented?

While syringomyelia generally can’t be prevented, early diagnosis and treatment may help prevent the worsening of symptoms.

What is the prognosis (outlook) for syringomyelia?

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.

How do I take care of myself if I have syringomyelia?

If you have syringomyelia, consider the following actions to help manage your symptoms:

• Avoid activities that can make your symptoms worse, such as anything that involves heavy lifting or straining or putting force on your spine (like jumping).
• Consider physical therapy to help with mobility issues, muscle weakness or stiffness and pain.
• If you have chronic pain from syringomyelia, talk to your provider about treatment options.
• It can be daunting and stressful to live with syringomyelia, so seeking mental health support from friends, family and/or a therapist is important. A support group consisting of people who also have syringomyelia may also help you feel less alone.

When should I see my healthcare provider about syringomyelia?

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.