Syringomyelia (sih-ring-go-my-E-lee-uh) is the development of a fluid-filled cyst (syrinx) within your spinal cord. Over time, the cyst may enlarge, damaging your spinal cord and causing pain, weakness and stiffness, among other symptoms. If left untreated, syringomyelia symptoms may worsen, in some cases requiring surgery. Syringomyelia has several possible causes. The majority of syringomyelia cases are associated with Chiari malformation, a condition in which brain tissue protrudes into your spinal canal. Other causes of syringomyelia include spinal cord tumors, spinal cord injuries and damage caused by inflammation around your spinal cord. If syringomyelia isn't causing any problems, monitoring the condition may be all that's necessary. But if you're bothered by symptoms, you may need surgery.

Complications of Syringomyelia

In some people, syringomyelia can become a progressive disorder and lead to serious complications. In others, there may be no associated symptoms and no intervention necessary. Complications that may occur as a syrinx grows, or if it damages nerves within your spinal cord, include:

• Scoliosis — an abnormal curve of your spine
• Horner syndrome — a disorder that can occur when the nerves that run from your brain to your eye and face (sympathetic nerve fibers) are damaged, leading to decreased sweating on the side of your face that's affected, a drooping eyelid and a small (constricted) pupil
• Chronic pain

Causes of Syringomyelia

Although it's unclear exactly how and why it happens, when syringomyelia develops, cerebrospinal fluid — the fluid that surrounds, cushions and protects your brain and spinal cord — collects within the spinal cord itself, forming a fluid-filled cyst (syrinx).

The following conditions and diseases can lead to syringomyelia:

• Chiari malformation — a condition in which brain tissue protrudes into your spinal canal
• Meningitis — an inflammation of the membranes surrounding your brain and spinal cord
• Tethered spinal cord syndrome — a disorder caused when tissue attached to your spinal cord limits its movement
• A spinal cord tumor
• A spine injury

Signs & Symptoms of Syringomyelia

Symptoms of syringomyelia usually develop slowly over time. If your syringomyelia is caused by Chiari malformation — a condition in which brain tissue protrudes into your spinal canal — symptoms may begin during your teenage years or early adulthood. In some cases, a fall, minor trauma, coughing or straining may trigger symptoms of syringomyelia, although none of these causes syringomyelia.

The following early signs and symptoms of syringomyelia may affect the back of your neck, shoulders, arms and hands first:

• Muscle weakness and wasting (atrophy)
• Loss of reflexes
• Loss of sensitivity to pain and temperature

Other signs and symptoms of syringomyelia may include:

• Stiffness in your back, shoulders, arms and legs
• Pain in your neck, arms and back
• Bowel and bladder function problems
• Muscle weakness and spasms in your legs
• Facial pain or numbness
• A tingling sensation rapidly spreading down your trunk and into your legs when you flex your neck sharply (Lhermitte's sign).

Diagnosis of Syringomyelia

• To diagnose syringomyelia, your doctor will begin by asking about your medical history and doing a complete physical examination.
• If your doctor suspects syringomyelia, you'll likely undergo a magnetic resonance imaging (MRI) scan of your spine and spinal cord. An MRI is the most reliable tool for diagnosing syringomyelia.
• Using magnetic fields and radio waves, an MRI produces 3-D, high-resolution images of your spine and spinal cord. If a syrinx has developed within your spinal cord, your doctor will be able to see it on an MRI. A dye or contrast medium can be injected for the test, and once it travels to your spine, it can enhance the MRI images. An MRI is a safe and painless test. Over time, repeat MRIs can be used to monitor the progression of syringomyelia.
• In some cases, syringomyelia may be discovered incidentally when a spine MRI or computerized tomography (CT) scan is done for other reasons.

Treatments of Syringomyelia

Treatment for syringomyelia depends on the severity and progression of your signs and symptoms.

Monitoring

If syringomyelia is discovered on an MRI scan that's done for an unrelated reason, and it's not causing signs or symptoms, monitoring with periodic MRI and neurological exams may be all that's necessary. In rare cases, a syrinx may resolve on its own without treatment.

Surgery

If syringomyelia is causing signs and symptoms that interfere with your daily life, or if signs and symptoms rapidly worsen, surgery is usually recommended. The goal of surgery is to remove the pressure the syrinx places on your spinal cord and to restore the normal flow of cerebrospinal fluid. The type of surgery you'll need depends on the underlying cause of syringomyelia.

Typically, surgery for syringomyelia includes one or more of the following:

• Treating Chiari malformation. If syringomyelia is caused by Chiari malformation, your doctor may recommend surgery that involves enlarging the opening at the base of your skull (suboccipital craniectomy) and expanding the covering of your brain (dura). This surgery can reduce pressure on your brain and spinal cord, restore the normal flow of cerebrospinal fluid and, in most cases, resolve syringomyelia.
• Draining the syrinx. To drain the syrinx, your doctor may surgically insert a drainage system, called a shunt. It consists of a flexible tube with a valve that keeps fluid from the syrinx flowing in the right direction. One end of the tubing is placed in the syrinx, and the other is placed just outside your spinal cord. The shunt remains inside your spine after surgery. In some cases, your doctor may be able to drain the syrinx during surgery with a small tube (catheter), and a shunt is not required.
• Removing the obstruction. If something within your spinal cord is hindering the normal flow of cerebrospinal fluid, such as a tumor or a bony growth, surgically removing the obstruction may restore the normal flow and allow fluid to drain from the syrinx.
• Correcting the abnormality. If a spinal abnormality is hindering the normal flow of cerebrospinal fluid, surgery to correct it — such as a releasing a tethered spinal cord — may restore normal fluid flow and allow the syrinx to drain.

Surgery doesn't always effectively restore the flow of cerebrospinal fluid, and the syrinx may remain, despite efforts to drain the fluid from it.

Follow up

Follow-up care after surgery is critical because syringomyelia may recur. You'll need regular examinations with your doctor, including periodic MRI tests, to assess the outcome of surgery. Other syrinxes may form, requiring additional surgery. Even after treatment, some signs and symptoms of syringomyelia may remain, as a syrinx can cause permanent spinal cord and nerve damage.

When to seek Medical Advice

• If you have any of the signs or symptoms associated with syringomyelia, see your doctor. Because many signs and symptoms of syringomyelia can be associated with other disorders, a thorough medical evaluation is important for accurate diagnosis.
• If you've experienced a spinal injury, watch carefully for signs and symptoms of syringomyelia. Many months to several years may pass after an injury before syringomyelia develops. If you have syringomyelia symptoms, when you go for an evaluation make sure your doctor knows you had a spinal injury.