Treatment for syringomyelia depends on what is causing the syrinx to form. Since the leading cause of syringomyelia is the Chiari malformation, proper surgery to decompress the Chiari malformation should lead to resolution of the syrinx cavity. 

Note the dark syrinx cavity in the spinal cord of this child (figure 1).  Also note the white-outline of the Chiari malformation. The syrinx cavity is caused by the Chiari malformation blocking the flow of cerebrospinal fluid (CSF).

Think of the Chiari as a "cork" that is blocking the flow of spinal fluid so that it has nowhere to go but inside the spinal cord itself. This is why surgeons aim to "decompress" the Chiari by removing part of the bone that surrounds that area. Once normal flow of spinal fluid is permitted, the patient should see their syrinx resolve. Please understand that we are only discussing the treatment of syringomyelia that is caused by a Chiari malformation.

Other disorders can cause syringomyelia and treatment of those should be discussed with an appropriate specialist. The best surgery for syringomyelia will aim to fix the cause of the syrinx and not to operate on the syrinx directly. 

We've included a chart showing some of the findings we've seen in Chiari patients that we have recently operated on.

The bottom-line is that every patient is different. Some have as little as one or two millimeters of herniation, others have more than twenty. Some have scar tissue, some don't. Can you see how successful treatment will really depend on whether the surgeon can figure out what exactly is causing the blockage of CSF? This is why experience pays in selecting a doctor.

Information on treating Chiari Malformations and Syringomyelia is available at the UW Medicine Chiari Malformation Web site.

The general "decompression" surgical procedure designed to treat a Chiari I malformation is covered in this section.

Even though this procedure has been widely adopted as the optimal form of treatment, many neurosurgeons still disagree on some of the specifics of this procedure. There is no single right way to perform this procedure.

The procedure

First, the patient is placed in the "prone" position, which simply means on your stomach. Don't worry, you are fast asleep by this point!

Next, the surgeon will cut through your neck muscles until your skull and vertebrae are exposed. (Figure 1)

As you can see, an arc approximately two centimeters (wide) by one centimeter (high) is removed from the base of your skull and the back of your top vertebrae is removed. This is done in an attempt to create room for your herniated brain to reside. It is hoped that it will "fall back" into this newly created space and that it will no longer put pressure on the brainstem. This should result in the normal flow of your Cerebrospinal Fluid (CSF) having been restored.

Once the appropriate bone has been removed, the dura layer will be opened to allow access to the brain matter. (Figure 2) The dura layer is a transparent, skin-like layer that surrounds the brain and spinal cord. Your spinal fluid flows between the dura and the brain and spinal cord, keeping the brain and spinal cord "buoyant." Surgeons will often-times have to open the dura layer in order to remove scar tissue, or to perform other additional steps such as cauterizing and shrinking the herniated tonsils so they do not obstruct CSF flow.

Remember, the goal of the surgery is to:

1. create more room for your brain, and
2. restore normal flow of the spinal fluid in this region and sometimes the dura needs to be opened in order to adequately accomplish this goal.

Finally, once proper flow of the spinal fluid has been established, the surgeon needs to sew a patch to cover the dura layer that has just been opened. (Figure 3)

This patch is sewn in “watertight” so CSF does not leak out. This patch is also known as a "dural graft" and can be made with different materials. For instance surgeons will use gore-tex, bovine material, or even your own pericranium for the “dural graft”. Once the graft has been successfully installed, the surgeon will sew your skin back together and you will soon wake up! Afterwords, you can consider yourself "decompressed."

This procedure is also known as a "Posterior-fossa decompression".

Below are signs and symptoms of Chiari I Malformation

Crainial/Brainstem Issues (headaches): Heavy, severe, suboccipital posterior with radiation to the vertex, exercise induced common finding.

Ocular disturbances: Eye pressure or pain, photophobia, field cuts.

Ocular findings: Decreased visual acuity, field cuts, extra-ocular muscle paresis

Otoneurologic Disturbances: Dizziness, disequilibrium, vertigo, tinnitus (ringing in the ear), ear pressure, hearing loss.

Otoneurologic Findings: Nystagmus (irregular eye movements) and hearing loss.

Crainial Nerve Disturbances: Dysphagia (difficulty swallowing), hoarseness, hiccups, facial pain, facial numbness, syncope (fainting) and aspiration.

Cranial Nerve Findings: Impaired gag reflex, CN V sensory loss, vocal cord, paralysis, apnea.

The prognosis of patients with syringomyelia is a tricky subject. The number one goal of treating syringomyelia is to get rid of the syrinx cavity in the spinal cord. Once this is done, the patient should be "good as new," right? Well, it's not that simple.

If a patient has numbness associated with a syrinx, there is a good chance it will go away if the operation is successful. If the patient has burning pain, this is most likely permanent and can be reduced but not resolved completely. Finally, if the patient has weakness in their extremities associated with the syrinx, this is also most likely permanent.

The best thing is to speak with an experienced neurosurgeon about your prognosis, since there are many factors that will determine it.

If not treated, the syrinx cavity can cause permanent nerve damage, which is why early treatment is essential.