What Is Syringomyelia?

Syringomyelia is a rare disease in which a fluid-filled cyst, or syrinx, forms in the spinal cord. Over time, the syrinx can grow and compress on the spinal cord, which can interfere with the body's ability to send information to and from the brain. Syringomyelia often causes neurologic symptoms, such as weakness or numbness in various areas of the body.

Regardless of the type of syringomyelia, symptoms might not occur for months or years. It is estimated that syringomyelia occurs in approximately 8 out of 100,000 people and appears slightly more in males than females.

This article will discuss the various types, symptoms, and treatment of syringomyelia, as well as other important information about this disorder.

Types of Syringomyelia

There are two main types of syringomyelia—congenital and acquired.

Congenital Syringomyelia

Sometimes referred to as communicating syringomyelia, this congenital condition is often caused by a rare birth defect known as Chiari malformation. This defect leads to syrinx development on the cervical spinal column located in the neck.

A person with this condition will typically develop symptoms between 25 and 40 years of age. The pressure on the spinal cord from the syrinx creates a build-up of cerebrospinal fluid, or the fluid that provides a cushion around our spinal cord and brain.

This fluid build-up creates a condition referred to as hydrocephalus and leads to increased pressure in the brain. Symptoms may worsen with activities like straining or coughing due to increased pressure.

Acquired Syringomyelia

Also known as noncommunicating syringomyelia, this form of the condition is caused by an injury or disease which leads to the development of a syrinx.

Spinal cord tumors, spinal cord injuries, or inflammation of the brain or spinal cord membranes often caused by an infection known as meningitis can potentially cause acquired syringomyelia.

Symptoms

The syrinx that causes syringomyelia can occur anywhere along the spinal cord, and the size of the fluid-filled cyst can vary. Depending on where the syrinx forms and how large it is, symptoms will vary, but typically can include:

• Pain
• Progressive weakness in the arms and legs
• Stiffness in the back, shoulders, neck, arms or legs
• Headaches
• Loss of sensitivity to pain or hot and cold, especially in the hands
• Numbness or tingling
• Balance issues
• Loss of bowel and bladder control
• Problems with sexual function
• Curvature of the spine, known as scoliosis, which might be the only symptom identified in children.

Diagnosis

Typically, a healthcare provider, such as a physician, will obtain a comprehensive history and perform a physical examination with particular emphasis on neurological function. If there is evidence to suggest further investigation of a possible syringomyelia diagnosis, medical testing might include:

• Magnetic resonance imaging (MRI): This noninvasive medical test can produce images of the structures inside your body to give detailed views of your organs, tissues, and skeleton.
• Computed tomography (CT) scan: Also known as a CAT scan, this noninvasive test involves using multiple X-ray images that help form a three-dimensional image of an organ, injury, or other internal issues, such as a syrinx.
• Myelography: This is a type of fluoroscopy where injected contrast material is used to evaluate the spinal cord, nerve roots, and spinal membranes.

Treatment

If you are diagnosed with syringomyelia, you should seek out a specialized healthcare provider, such as a neurologist or neurosurgeon. These specialists can accurately evaluate the most appropriate treatment option to ensure the proper flow of cerebrospinal fluid is maintained, or as much as possible:

Monitoring

If the diagnosis of syringomyelia is made when a person has few or no symptoms, close monitoring by a neurologist or neurosurgeon is recommended. For affected individuals being monitored, it is important to avoiding certain activities that involve straining, like lifting heavy objects or jumping, as this can increase pressure in the spinal column and brain.

Surgery

If a person with syringomyelia is experiencing harsh symptoms or notices a worsening of symptoms, surgery is the only definitive treatment. It is highly recommended to seek evaluation, and surgical intervention from a neurosurgeon skilled with syringomyelia as the type of surgery may vary depending on the root cause.

For example, treating a Chiari malformation will be a different procedure than those used to remove an obstruction like scar tissue, bone, or tumors. There are also cases in which the syrinx may be drained, or an expansive duraplasty may be used to prevent a syrinx from forming or growing. This technique takes scar tissue surrounding the spinal cord and re-applies it as a patch to expand the spinal cord membrane.

Prognosis

When identified early, particularly when symptoms are relatively mild, syringomyelia has a good outlook with minimal spinal cord damage.

Since very few individuals are diagnosed with syringomyelia and treatment routes vary based on the size and location of the syrinx, studies on the outcomes following surgical intervention are limited. However, some research indicates the majority of people affected by syringomyelia who undergo surgery have stabilized symptoms or see modest improvement.

Summary

Syringomyelia is a condition caused by a fluid-filled cyst, or syrinx, on the spinal cord. It can cause a multitude of neurological symptoms that will vary based on the location and size of the syrinx. Seeking specialized treatment from a neurologist or neurosurgeon is the best course of action in receiving the appropriate treatment and relieving symptoms.

A Word From Verywell

Since syringomyelia is a rare disease that can develop over the course of several years, it may be difficult to understand symptoms at first. Receiving a proper diagnosis may also be challenging, but finding the right healthcare provider who can closely monitor changes in your symptoms is essential. Any new or worsening neurological symptoms should be evaluated and surgery may be necessary, as it is the only way to definitively treat the condition. However, the outlook for people diagnosed with syringomyelia is positive, especially if diagnosed early.