Syringomyelia

Syringomyelia (sih-ring-go-my-E-lee-uh) is the development of a fluid-filled cyst within the spinal cord. The cyst, which is sometimes called a syrinx, can grow larger over time. When it does, it can damage the spinal cord and cause pain, weakness and stiffness.

Syringomyelia has several possible causes. Many cases are associated with a Chiari malformation. This is a condition in which brain tissue pushes into the spinal canal.

Other causes of syringomyelia include spinal cord tumors, spinal cord injuries and damage caused by swelling around the spinal cord.

If syringomyelia doesn't cause problems, monitoring the condition might be all that's necessary. But if the symptoms are bothersome, surgery might be needed.

Syringomyelia symptoms usually develop slowly over time. If syringomyelia is caused by brain tissue pushing into the spinal canal, it is called a Chiari malformation. Symptoms generally begin between ages 25 and 40.

In some cases, coughing or straining can trigger symptoms of syringomyelia, although neither causes syringomyelia.

Syringomyelia might affect the back, shoulders, arms or legs. Symptoms can include:

• Muscle weakness and muscle wasting.
• Loss of reflexes.
• Loss of sensitivity to pain and temperature.
• Headaches.
• Stiffness in the back, shoulders, arms and legs.
• Pain in the neck, arms and back.
• Scoliosis. This is when the spine curves sideways.

When to see a doctor

If you have any of the symptoms associated with syringomyelia, see your healthcare professional.

If you've had a spinal cord injury, watch for symptoms of syringomyelia. It may take months to years after an injury before syringomyelia develops. Make sure your healthcare professional knows you had a spinal cord injury.

It's not clear how and why syringomyelia happens. When it develops, the fluid that surrounds, cushions and protects the brain and spinal cord collects within the spinal cord itself. This fluid is called cerebrospinal fluid. If it collects and forms a fluid-filled cyst, it is called a syrinx.

Several conditions and diseases can lead to syringomyelia, including:

• Chiari malformation, a condition in which brain tissue pushes into the spinal canal and blocks the regular flow of cerebrospinal fluid.
• Meningitis, which is swelling of the membranes surrounding the brain and spinal cord.
• Spinal cord tumor, which can interfere with the regular flow of cerebrospinal fluid.
• Conditions present at birth, such as a tethered spinal cord. A tethered spinal cord is a condition caused when tissue attached to the spinal cord limits its movement.
• Spinal cord injury, which can cause symptoms months or years later.

In some people, syringomyelia can progress and lead to serious complications. Other people have no symptoms.

A syrinx can cause complications if it grows or damages nerves within the spinal cord. Complications include:

• Scoliosis. This is when the spine curves sideways.
• Chronic pain resulting from damage to the spinal cord.
• Motor difficulties that can affect walking. Examples include weakness and stiffness in the leg muscles.
• Paralysis.

Your healthcare professional asks about your medical history and do a complete physical exam. In some cases, syringomyelia might be discovered during a spine MRI or CT scan conducted for other reasons.

If your healthcare professional suspects you may have syringomyelia, you may need to undergo testing. Tests may include:

• MRI. An MRI scan of the spine and spinal cord is the most reliable tool for diagnosing syringomyelia.

  An MRI uses radio waves and a strong magnetic field to produce detailed images of the spine and spinal cord. If a syrinx has developed within the spinal cord, it is visible on the MRI scan.

  The MRI might be repeated over time to monitor the progression of syringomyelia.

• CT scan. A CT scan uses a series of X-rays to create a detailed view of the spine and spinal cord. It can reveal tumors or other spine conditions.

Treatment for syringomyelia depends on how bad the symptoms are and the size of the syrinx.

Monitoring

If syringomyelia isn't causing symptoms, it may not need treatment. Your healthcare professional may check it from time to time with MRI and neurological exams.

Surgery

Surgery may be needed if syringomyelia is causing symptoms that get in the way of your life, or if symptoms rapidly worsen.

The goal of surgery is to remove the pressure the syrinx places on the spinal cord and to restore the regular flow of cerebrospinal fluid. This can help improve symptoms and nervous system function. The type of surgery needed depends on the cause of syringomyelia.

To reduce pressure on the brain and spinal cord, surgery options include:

• Treating Chiari malformation. If syringomyelia is caused by a Chiari malformation, surgery might involve removing a small section of bone at the back of the skull. This surgery can reduce pressure on the brain and spinal cord and restore the regular flow of cerebrospinal fluid. Surgery may improve or clear syringomyelia.
• Draining the syrinx. Your healthcare professional inserts a drainage system, called a shunt. It consists of a flexible tube that keeps fluid from the syrinx flowing in the desired direction. One end of the tubing is placed in the syrinx, and the other is placed in another area of the body such as the abdomen.
• Removing the obstruction. Sometimes something within the spinal cord is interfering with the flow of cerebrospinal fluid. Examples include a tumor or a bone spur. Surgically removing the obstruction might clear the flow. This can allow fluid to drain from the syrinx.
• Correcting the irregularity. If a spinal irregularity is hindering the flow of cerebrospinal fluid, surgery may be needed. For example, surgery can release a tethered spinal cord and restore fluid flow. This allows the syrinx to drain.

Surgery doesn't always fix the syrinx. Sometimes the syrinx remains even after efforts to drain the fluid from it.

Follow-up care

Syringomyelia can come back after surgery. You'll need regular exams with your healthcare team. You might need an MRI now and then to check the outcome of surgery.

The syrinx can grow over time and may require more treatment. Even after treatment, some symptoms of syringomyelia can remain. This is because a syrinx can cause permanent damage to the spinal cord and nerves.

The following steps might help reduce the effects of syringomyelia.

Avoid activities that can make symptoms worse

Avoid activities that involve heavy lifting, straining or putting force on the spine.

Consider physical therapy and occupational therapy

A syringomyelia sometimes causes neurological conditions that decrease the ability to move. For instance, it may cause muscle weakness, pain, fatigue or stiffness. Physical and occupational therapy can help to improve muscle function. A physical therapist can create an exercise program that may help reduce these symptoms. An occupational therapist can teach you how to function better in your daily tasks.

Talk to your healthcare team about physical and occupational therapists in your area who have expertise in neurological conditions.

Manage chronic pain

If you have chronic pain from syringomyelia, talk to your healthcare team about treatment options. Many medical centers have doctors who specialize in pain management.

Living with syringomyelia and its complications can be challenging. Having someone to talk with, whether a friend, counselor or therapist, can be invaluable. Or you might find the support and encouragement you need in a syringomyelia support group.

Ask your healthcare professional to recommend a local group or look for groups online. A support group provides a place for sharing experiences. It also can be a good source of information and offer helpful tips for people with syringomyelia.

You're likely to start by seeing your family healthcare professional. You might get a referral to see a neurologist. A neurologist is a doctor trained in brain and nervous system conditions.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance. If you have past medical reports, MRI scans or CT scans that might relate to your condition, bring them to your appointment.

Take a family member or friend to your appointment, if possible, to help you remember the information you receive.

Make a list of:

• Your symptoms and when they began
• Key personal information, including spinal or back surgeries or injuries you've had and family history of syringomyelia
• All medications, vitamins or other supplements you take, including doses
• Questions to ask your healthcare professional.

For syringomyelia, questions to ask your healthcare professional include:

• What is the likely cause of my symptoms or condition?
• Are there other possible causes?
• Is it possible my symptoms will get better on their own?
• What tests do I need?
• What is the best course of action?
• Can exercise help?
• I have other health conditions. How can I best manage them together?
• Are there brochures or other printed material I may have? What websites do you recommend?

What to expect from your doctor

Your healthcare professional is likely to ask you questions, including:

• Have your symptoms been ongoing, or do they come and go?
• How bad are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, seems to worsen your symptoms?

What you can do in the meantime

Avoid doing anything that worsens your symptoms. For many people with syringomyelia, heavy lifting and straining can trigger symptoms, so avoid these activities. Also, avoid flexing the neck.