Syringomyelia

Syringomyelia is a neurological disorder where a fluid-filled cyst, called a syrinx, forms inside your spinal cord. The cavity fills with cerebrospinal fluid (CSF). The cyst can press on the nerves in your spine and cause problems over time. It often targets the upper parts of your spinal cord near your neck and upper back.

The condition may develop slowly over many years. Symptoms can affect how you feel sensations or move. In some cases, your provider may discover a syrinx incidentally on an MRI. It may stay the same size and not cause symptoms as time progresses (remain stable).

This condition can happen for different reasons. It’s often linked to a Chiari malformation (when part of your brain sits lower than usual) or it can happen months to years after a traumatic spinal cord injury.

Syringomyelia (pronounced “sr-in-jow-mai-EH-lee-uh”) is rare. Research suggests that it affects about 8 people per 100,000.

Your provider can help you plan the best care to relieve symptoms if you experience them.

Types of syringomyelia

There are a couple types of syringomyelia. They differ by how the cyst forms and what it connects to:

• Communicating syringomyelia: This syrinx connects to the fourth ventricle in your brain. It often appears with hydrocephalus (extra fluid around your brain).
• Non-communicating syringomyelia: This type doesn’t connect to the fourth ventricle. It usually happens when something blocks or changes the flow of spinal fluid, like a Chiari malformation, past injury or inflammation.

Your provider may describe syringomyelia by its underlying cause (like Chiari-related).

Syringomyelia symptoms

Symptoms usually develop slowly and may get worse over many years. They depend on where the syrinx sits in your spine, how big it is and how far it stretches. You might not have any symptoms for a while if the syrinx is small.

Common symptoms include:

• Balance problems or trouble walking
• Headaches
• Muscle spasms or tightness
• Numbness or tingling
• Pain in your neck, arms, back or legs
• Issues with sexual function
• Weakness or muscle wasting (atrophy) in your arms or legs

A classic neurological finding is loss of pain and temperature sensation. While vibration and position senses stay normal, you might feel a “cape-like” pattern across your shoulders and upper arms.

Symptoms can affect one or both sides of your body and may be mild or severe.

Children may have symptoms like:

• Scoliosis (a curve in their spine)
• Toe walking

If you or your child has these symptoms, it’s important to see a healthcare provider as soon as possible. Early treatment may prevent permanent nerve damage.

Syringomyelia causes

Healthcare providers don’t know the exact reason a syrinx forms. Most theories say it happens when the flow of cerebrospinal fluid (CSF) changes or gets blocked.

Syringomyelia has two main types of causes:

• Congenital (from birth)
• Acquired (develops later in life)

Sometimes, a syrinx forms with no known cause. This is called idiopathic syringomyelia.

Congenital causes of syringomyelia

These conditions change the structure of your brain or spine. They can block CSF flow. Congenital causes include:

• Chiari malformation type I: The lower part of your brain sits lower than usual and blocks normal CSF movement. This is the most common congenital cause. Symptoms may not appear until your teen or adult years.
• Myelomeningocele: This is a type of spina bifida where your spine doesn’t close before birth.
• Tethered cord syndrome: Your spinal cord attaches to nearby tissues and can’t move freely.

Acquired causes of syringomyelia

These causes happen after birth. They change how CSF moves around your spine:

• Spinal cord injury: Scar tissue from an injury can block CSF flow months or years later.
• Spinal cord tumors: Tumors can press on your spinal cord and disrupt CSF flow.
• Arachnoiditis: This is inflammation of the membrane around your spinal cord. It’s sometimes caused by infections or autoimmune conditions.
• Meningitis: This is an infection of the protective lining around your brain and spine.

Risk factors

This condition can affect anyone. Most people get a diagnosis between the ages of 20 and 50. But it can also develop in children or adults after age 50.

Complications of syringomyelia

Syringomyelia can slowly damage your spinal cord over time. In late-stage and severe cases, complications may include:

• Long-lasting nerve pain (burning, tingling or shooting pain)
• Loss of bowel or bladder control
• Paralysis in your arms or legs
• Pressure sores (bedsores) from reduced movement
• Repeated lung infections, like pneumonia

How doctors diagnose syringomyelia

To diagnose syringomyelia, a provider will review your medical history and ask about your symptoms. They’ll also do a physical and neurological exam to check your strength, feeling, reflexes, balance and coordination.

Imaging tests help confirm the diagnosis. The most important test is an MRI. This can show the syrinx (a fluid-filled cyst) in your spinal cord. The MRI also helps your provider see where the syrinx is, how large it is and how far it extends. It can also show possible causes, like a Chiari malformation or a tumor. Your provider may use a cine flow (dynamic) MRI to evaluate how cerebrospinal fluid flows around your brain and spinal cord.

Your provider may order other tests if they need to rule out conditions with similar symptoms. There are other testing options available if you can’t have an MRI.

Syringomyelia treatment

Treatment focuses on reducing pressure in your spine and improving the flow of cerebrospinal fluid. Your provider might recommend:

• Observation: Some syrinxes, especially in children, can shrink or disappear over time as CSF flow improves. A provider may recommend regular check-ups and repeat MRI scans to watch for changes, unless symptoms get worse.
• Routine changes: If symptoms are mild, your provider may ask you to avoid activities that increase pressure in your head or neck. This may include heavy straining or holding your breath. You should avoid high-impact activities, like riding roller coasters, using a trampoline and playing contact sports.
• Symptom management: Physical therapy, occupational therapy, pain medication or other supportive treatments may ease discomfort and improve movement.
• Surgery: Some adults may need surgery to treat the underlying cause. For example, surgery can create more space for CSF to flow if you have a Chiari malformation. Surgery is often helpful. But a syrinx can sometimes return after treatment. You may need another procedure if that happens.

When should I see my healthcare provider?

If you have this condition, you’ll need regular visits with your care team. These check-ins monitor your symptoms and track any changes in the syrinx. They help whether you’ve had surgery or not.

Symptoms that need immediate medical attention include:

• Loss of bowel or bladder control or new bowel or bladder dysfunction
• Sudden or new weakness in your arms or legs
• Trouble walking or frequent falls

If you had surgery, your provider will tell you when to return for follow-up visits. Contact your care team right away if you notice any signs of a problem, like:

• Fever
• Pus or drainage
• Severe pain
• Swelling

What is the life expectancy of someone with syringomyelia?

Most people with syringomyelia live a normal lifespan. Treatment can help manage the cause and lower the chance of serious problems. This helps keep you safer over time.

Your outlook can depend on:

• What caused the syrinx
• How much nerve damage you have
• Where the syrinx is
• Whether your spinal cord has swelling

The condition can change slowly for some. Others may see symptoms get worse more quickly.

Early diagnosis and treatment may help slow these changes. Many people improve with surgery, especially when the syrinx is in your neck area. Strength and movement may get better, but pain or numbness may not fully go away.

A syrinx can return, so regular follow-up visits are important. Your provider will let you know how often these visits should be so you can stay on top of your care.