Do you have a cyst inside your spine? (Syringomyelia) Let's learn about it.

Do you sometimes feel back pain, numbness in your hands, or difficulty walking? Some people think that these are normal things, but behind these symptoms there may be a serious illness. Today we are going to talk about a rare condition that affects our spinal cord. That is syringomyelia .

What is Syringomyelia?

Simply put, Syringomyelia is a condition where a fluid-filled sac (called a syrinx) forms inside your spinal cord . Think about it, the spinal cord runs through your spine. This spinal cord is like a 'messenger' that carries messages from your brain to the rest of your body, and from your body back to your brain. So, this 'syrinx' can get bigger and longer over time. What happens when that happens? The spinal cord starts to get damaged. The nerve fibers that carry those messages get pinched and damaged.

What is this liquid?

The fluid inside this cyst is called Cerebrospinal Fluid (CSF) . This fluid, called CSF, normally surrounds our brain and spinal cord to protect them. It's like the shock absorbers in a car. Not only that, but the cavities inside our brain (called Ventricles) are also filled with this CSF. These cavities are connected to a small channel (Central Canal) that runs along the spinal cord.

So, if you have `(Syringomyelia)`, what happens is that this `(CSF)` fluid collects inside the tissue of the spinal cord, widening that central canal `(Central Canal)` and creating a `(Syrinx)`. Most of the time, a `(Syrinx)` is formed when the `(CSF)` flow around the spinal cord or around the lower part of the brain (`(Brain Stem)`) is not happening normally, which means that there is something obstructing that flow. If this `(Syrinx)` affects the `(Brain Stem)`, that condition is called `(Syringobulbia)`.

Syringomyelia most often affects the upper parts of the spinal cord, that is, the cervical and thoracic regions.

Who is most likely to develop this condition? How common is it?

Syringomyelia is most commonly diagnosed in people between the ages of 20 and 50. However, it can also occur in children and the elderly. However, it is a very rare disease . It affects about 8 out of 100,000 people. It is also responsible for about 5% of cases of paraplegia, which is a condition in which the legs and lower body are paralyzed.

What are the symptoms of Syringomyelia?

Symptoms usually develop slowly, and may worsen over years.. Some people can have a small `(Syrinx)` and go for a long time without any symptoms. Symptoms also vary, depending on where the `(Syrinx)` is located in the spine, how large it is, and how long it is. Symptoms can occur on one side of the body or on both sides. While some people have very little, others can be severely affected.

Common symptoms that can be seen are:

• Headache .
• Pain that travels down the arms, down the neck, down the middle of the back, or down the legs (Radiculopathy).
• Progressive weakness and muscle wasting in the arms and/or legs (Muscle Atrophy).
• Spasms or Tightness in the muscles of the back, shoulders, neck, arms, or legs.
• Loss of sensitivity to pain or temperature , especially in the hands.
• Numbness or tingling in the hands and/or feet.
• Problems with balance and difficulty walking.
• Loss of bowel and bladder control.
• Problems related to sexual function.

In addition to these symptoms, there are also symptoms that particularly affect young children:

• Scoliosis.
• Toe walking.

The most important thing is that if you or your child has any of these symptoms, you should see a doctor as soon as possible.

Why does Syringomyelia develop? What are the causes?

In fact, scientists still haven't figured out exactly what the underlying cause of a syrinx is. But most theories suggest that the main cause is either a blockage or a problem with the flow of cerebrospinal fluid (CSF) that we talked about earlier.

The causes of syringomyelia can be divided into two main categories: congenital (meaning present at birth) and acquired (meaning developed later in life) .

Congenital Causes

Syringomyelia is often associated with a condition called Chiari Malformation Type 1. This is when the lower part of our cerebellum protrudes into the foramen magnum, a hole at the base of the skull. The spinal cord normally passes through this hole. This structural problem obstructs the normal flow of cerebrospinal fluid (CSF).

Often, this ``Chiari Malformation'' is something that is present at birth. However, some people with ``Chiari Malformation Type I'' may not have any symptoms at all, or they may not develop symptoms until they are young or old.

Other congenital causes are:

• Myelomeningocele: Also called Open Spina Bifida, this is a birth defect. This is where the spinal cord and spinal canal do not close properly before birth. This is a type of neural tube defect (NTD).
• Tethered Cord Syndrome:This is also a very rare neurological condition. What happens here is that the spinal cord becomes attached to the tissues around the spine (that's what it's called being tethered). This is often associated with ``Spina Bifida'' and ``Scoliosis''.

Acquired Causes

The factors that contribute to the development of ``Syringomyelia`` later in life are:

• Spinal Cord Injuries: This type of syringomyelia is sometimes called post-traumatic syringomyelia. It can occur months or years after the initial injury. It is often caused by scar tissue that blocks the flow of CSF.
• Spinal Cord Tumors: Tumors, especially hemangioblastomas and ependymomas, can obstruct the flow of cerebrospinal fluid (CSF), causing syringomyelia.
• Arachnoiditis: The arachnoid membrane is one of the membranes that surround and protect the spinal cord. If this membrane becomes inflamed (swelling), it can cause syringomyelia. This can be caused by diseases such as sarcoidosis, transverse myelitis, and multiple sclerosis (MS).
• Meningitis: Meningitis is an infection of the protective membranes (meninges) surrounding the brain and spinal cord. This can also cause syringomyelia.

Sometimes, syringomyelia can develop without any obvious cause. This is called idiopathic syringomyelia .

How to accurately diagnose this disease?

To find out for sure if you have `(Syringomyelia)`, your doctor will first take your medical history and then do a physical exam. He will pay special attention to nerve function. He may also refer you to a `(Neurologist)` - a doctor who specializes in diseases of the brain and nervous system.

Next, you will be asked to do imaging tests to take pictures of your spinal cord or brain. Sometimes, syringomyelia may be discovered by chance while doing these imaging tests for another condition.

What tests are done to diagnose this disease?

If your doctor suspects syringomyelia, he or she may order one or more of these tests:

• MRI (Magnetic Resonance Imaging) Scan: The most reliable way to diagnose `(Syringomyelia)` is an MRI scan.. This uses a large magnet and a computer to take detailed pictures of what's inside your body. With this test, your doctor can find out if you have a `(Syrinx)` in your spinal cord, or if there's any other abnormality, such as a tumor. An `(MRI)` can clearly show where the `(Syrinx)` is, its size, and how far it has spread.
• Dynamic MRI: This type of MRI shows how the cerebrospinal fluid (CSF) flows around the spinal cord and into the syrinx. This may require a contrast agent (a special liquid that makes the images clearer) to be injected into your vein.
• Myelogram with CT scan: If you can't have an MRI, the next option is a CT scan. A myelogram is a test that uses a special dye called a contrast dye and a CT scan (Computed Tomography) to look for problems in the spinal cord.

What are the treatments for Syringomyelia?

Treatment for syringomyelia depends on how severe your symptoms are and how quickly they progress.

If there are no symptoms , doctors usually do not treat `(Syringomyelia)`. However, they recommend that the `(Syrinx)` is carefully monitored by a `(Neurologist)` or `(Neurosurgeon)`. Because symptoms can appear or worsen over time. This monitoring usually includes periodic `(MRI)` scans and neurological tests.

For those with symptoms, the general goals of treatment for Syringomyelia are:

• If possible, treat the underlying cause of the syrinx.
• Controlling symptoms .
• Restoring the proper flow of cerebrospinal fluid (CSF) and preventing further damage to the spinal cord.

Some type of surgery is usually performed to treat the underlying cause and stop further damage to the spinal cord. Common treatments to control symptoms include:

• Painkillers and other medications.
• Physical Therapy and Rehabilitation.
• Limiting certain activities, especially those that put strain on the back (such as lifting weights, jumping).

What types of surgeries are performed for Syringomyelia?

If you have a condition called ``Syringomyelia'' that is causing symptoms, or if the ``Syrinx'' is getting bigger, your neurologist or neurosurgeon will likely recommend surgery. There are two main types of surgery: to normalize the flow of ``CSF'' around the spinal cord and to remove the fluid directly from the ``Syrinx''. The type of surgery you have will depend on the cause of your symptoms.

Surgeries include:

• Chiari Malformation Treatment: The goal of this surgery is to create more space at the base of the skull and the top of the neck. This reduces pressure on the brain and spinal cord, allowing the flow of cerebrospinal fluid (CSF) to return to normal. The most common surgery for this is called Posterior Fossa Decompression . In this procedure, the neurosurgeon removes small pieces of bone from the back of the skull to create space. After this surgery, the fluid in the Syrinx can drain, sometimes shrinking or disappearing completely. Even if the Syrinx remains the same size or is only slightly smaller, symptoms can improve.
• Treating or preventing post-traumatic syringomyelia: In syringomyelia caused by an accident, the main goal is to first stop a syrinx from forming or to stop an existing one from getting bigger. This surgery is called an expansive duraplasty . This involves removing scar tissue around the spinal cord and sometimes inserting a patch to expand the dura mater. This creates space around the spinal cord and cleans it out, allowing the cerebrospinal fluid (CSF) to flow normally.
• Removing the obstruction: Surgical removal of obstructions such as scar tissue, bone fragments in the spinal canal, or tumors can help restore the flow of CSF. If a tumor is causing the syringomyelia, removing the tumor will often resolve the syrinx. Sometimes, your doctor may also recommend radiation therapy to shrink the tumor.
• Syrinx drainage: If there is no obvious cause for the syrinx, especially if it is getting larger, your doctor may recommend draining the fluid. This involves a neurosurgeon placing a stent or shunt in the syrinx. A stent is a small tube that allows fluid to drain from the spinal cord to the outside. A shunt is a flexible tube with a valve that allows fluid to drain from the syrinx to another part of the body, usually the abdomen or chest. Both of these procedures can help drain the fluid from the syrinx, which can help stop symptoms from getting worse.

After surgery, the doctor will perform an MRI scan to see if the syrinx has stabilized or has decreased in size.

Surgery for `(Syringomyelia)` can improve symptoms and stabilize the condition in many people. However, `(Syringomyelia)` can recur even after successful treatment . In that case, more surgery may be required.

What are the possible complications of surgery?

The following complications may occur after surgery for Syringomyelia:

• Cerebrospinal fluid (CSF) leak.
• Infections.
• Hemorrhage.
• Further damage to the spinal cord.
• Rebuilding the `(Syrinx)`.

It is very important to talk to your neurosurgeon about these risks related to your surgery and how likely they are.

What is the outlook for Syringomyelia? (Prognosis)

It is important to remember that the symptoms, severity, and causes of Syringomyelia vary widely. Also, the rate at which the disease progresses can vary from person to person, or some people may not get better at all. Because of these factors, it is difficult for doctors to give an accurate prognosis for Syringomyelia.

Early surgery can usually reduce symptoms and have good outcomes . Although the progression of neurological deficits after surgical treatment is usually stable, and symptoms sometimes improve, many people remain with symptoms.

Generally speaking, if the diameter of the `(Syrinx)` is more than five millimeters `(5mm)` and there is swelling `(Edema)` with it, it indicates that the outlook is not very good.

Myelopathy (damage to the spinal cord due to severe compression) is a major complication of Syringomyelia and something to be concerned about. This can eventually lead to paralysis/quadriplegia, recurrent pneumonia, and problems with bowel and bladder function.

Can Syringomyelia be prevented?

Syringomyelia is usually not preventable. However, early detection and treatment can help prevent symptoms from getting worse .

If I have Syringomyelia, how should I take care of myself?

If you have Syringomyelia, consider these things to help manage your symptoms:

• Avoid activities that may make your symptoms worse , such as lifting, straining, or anything that puts pressure on your back (like jumping).
• Consider physical therapy to reduce walking difficulties, muscle weakness or stiffness, and pain.
• If you have chronic pain due to syringomyelia, talk to your doctor about treatment options.
• Living with Syringomyelia can be overwhelming and stressful. It's important to seek mental health support from friends, family, and/or a counselor. Joining a support group with other people who have Syringomyelia can also help you feel like you're not alone.

When should I see my doctor about Syringomyelia?

If you have been diagnosed with Syringomyelia, monitor your symptoms and the Syrinx, whether or not you have had surgery.You should meet with your medical team regularly.

Finding out you have a spinal cord injury can be overwhelming. But remember, you are not alone, and there are many resources to help you. It is important to talk to a doctor who is knowledgeable about Syringomyelia and learn more about how it may affect you. Don't be afraid to ask your medical team questions - they are there to help you .

Our Take-Home Message

Okay, so I hope you have understood something from what we have talked about `(Syringomyelia)`. Although this is a rare disease, it is very important to seek medical advice immediately if you have symptoms . Especially if you have persistent back pain, numbness in the limbs, or weakness, you should definitely see a doctor. If you recognize the disease early, you can seek appropriate treatment and maintain a good quality of life. Don't worry, there are solutions to everything, and if you have any questions, ask your doctors.

` Syringomyelia, spinal cord, back pain, syrinx, CSF, neurological disease, Chiari malformation