What is syringomyelia?

Syringomyelia is a rare disorder in which a fluid-filled cyst forms within your spinal cord. This cyst is referred to as a syrinx.

As the syrinx expands and lengthens over time, it compresses and damages part of your spinal cord from its center outward.

Damage to the spinal cord caused by a syrinx can lead to symptoms such as progressive pain, stiffness, and weakness in the:

  • back
  • shoulders
  • arms
  • legs

People with the disorder might lose the ability to feel cold and pain normally. Some people with this disorder won’t have any symptoms and won’t need treatment. For others, syringomyelia will cause symptoms and complications that worsen as the syrinx expands.

Treatment aims to relieve the pressure on your spinal cord. The treatment your doctor suggests for you will depend on the cause of your syringomyelia. Follow-up care after surgery is important because syringomyelia can reoccur.

Most cases of syringomyelia are caused by a malformation of the brain known as Chiari type 1 malformation (CM1).

CM1 occurs where the brain joins the spinal cord. In this malformation, the brainstem lies lower than normal. Located at the back of the brainstem is the cerebellum. Often with CM1, the bases of the lobes of the cerebellum, or the cerebellar tonsils, protrude from the skull and into the spinal canal.

Syringomyelia can develop as a complication of:

Arachnoiditis is a progressive inflammatory disorder that affects the arachnoid, the membrane that surrounds the brain and spinal cord. A primary arachnoid cyst is present at birth, but it may take years for symptoms to appear.

Symptoms of this disorder are caused by the pressure the syrinx puts on the spinal cord and the damage that follows. They may include:

You should visit your doctor if you have any of these symptoms. If you’ve had a spinal injury, it’s important to watch for these symptoms. It may take months or even years after your injury for syringomyelia to develop.

If your doctor suspects you have syringomyelia, you may be referred to a neurologist, a specialist in treating the nervous system.

To diagnose your condition, your neurologist will first take your complete medical history. A complete physical examination will also be performed. Be prepared to tell your neurologist about your symptoms and how long you’ve had them.

If your neurologist thinks you may have syringomyelia, they’ll order an MRI scan to look for a syrinx in your spinal cord. An MRI scan is the most reliable diagnostic tool for syringomyelia, and it’s considered the gold standard for diagnosing the condition.

Treatment depends on the progression of the disorder and whether you’re experiencing symptoms that disrupt your life. If you have no symptoms or mild symptoms, you may not need treatment. Your neurologist will monitor the progression of the disorder.

If your symptoms are negatively affecting your life, your neurologist will recommend medications or surgery.

Medications such as gabapentin (Neurontin) may help decrease the painful sensation of the shoulders and arms that frequently occur with syringomyelia.

The goal of surgery is to correct the underlying cause of the syrinx and relieve the pressure on your spinal cord. The type of surgery will depend on the cause of your syringomyelia.

If you have CM1, your surgeon may suggest surgery to expand the base of your skull and the covering of your brain. This will take pressure off your spinal cord and your brain. The normal flow of cerebrospinal fluid should be restored. For most people, this surgery resolves their syringomyelia.

If you have a tumor or bony growth that’s causing syringomyelia, removal of the growth frequently resolves the syringomyelia.

In some cases, your surgeon will use a small, flexible tube called a shunt to drain the syrinx. They’ll place the shunt in the syrinx to drain the excess fluid. Sometimes the surgeon can completely drain the syrinx during surgery. If that isn’t possible, the shunt will remain in place after your surgery.

After surgery, you may be prescribed a course of antibiotics to prevent complications from infection. Your doctor may also recommend physical therapy, which can help strengthen muscles in limbs that have progressive weakness.

The outlook of those who undergo treatment and have a successful surgery varies. Damage to the spinal cord may be severe enough to cause permanent neurological issues. Some people may struggle with walking or have permanent weakness in their limbs. After the syrinx has been treated, the hope is that these conditions will slowly improve with physical therapy and time.

It’s important to attend follow-up appointments with your doctor. You’ll need periodic MRI scans because syringomyelia can reoccur.