Syringomyelia: Symptoms, Causes, Treatment, Complications

Overview

Syringomyelia develops when a fluid-filled cyst forms within the spinal cord. Progressively, the cysts enlarge, damaging the spinal cord and leading to pain, weakness, stiffness, and other symptoms. It is also known as hydromyelia, syringohydromyelia, or Morvan disease. 

Understanding and knowing how to manage the condition is crucial. Therefore, the blog explains syringomyelia, its symptoms, causes, and treatment options. 

What is Syringomyelia?

It is a rare, long-term disorder developed due to a fluid-filled cyst in the spinal cord. The cyst is called a syrinx, and is made up of Cerebrospinal luid. Gradually, as the cyst enlarges, it compresses and damages the parts of the spinal cord from the centre outwards. It results in pain, stiffness, and weakness in various body parts, such as the back, shoulders, arms, and legs.

People with this disorder may not show signs and symptoms. The disease generally affects the upper part of the spinal cord, particularly the cervical and thorax.

There are several causes for syringomyelia, and it is mainly associated with Chiari malformation – a protrusion of brain tissue into the spinal canal. Spinal cord injury, spinal cord tumour, and spinal cord damage are other causes of this condition. 

It is essential to note that severe cases of syringomyelia may cause permanent problems. But a mild condition is manageable with constant monitoring and medical intervention – based on the cause of the disease. Patients undergoing surgery also need regular follow-ups as it may reoccur. 

Who is at risk of developing syringomyelia?

Syringomyelia can develop among young children or older adults. But it develops usually among people between 20 to 50 years. It is also important to note that men are more likely to develop than women.

What are the causes of syringomyelia?

The causes of syringomyelia are unclear. It develops due to cerebrospinal fluid collection inside the spinal cord, forming a fluid-filled cyst. The cyst is known as syrinx. 

With syringomyelia, there are two main reasons the fluid gets rerouted or blocked. 

They are congenital and acquired causes. 

Congenital Syringomyelia

It is also known as communicating syringomyelia. During pregnancy, the lower part of the baby’s brain gets pushed downwards towards the spinal cord, causing congenital syringomyelia. The brain defect is called Chiari I malformation. 

It blocks the usual fluid flow between the spine and the brain, causing a cyst to form. But not everyone with Chiari I malformation develops congenital syringomyelia. The symptoms start to appear between 25 to 40 years. The following are other reasons for congenital syringomyelia:

• Myelomeningocele: It occurs before a baby is born. It is also known as open spina bifida. Myelomeningocele is where the backbone and the spinal canal do not close before the child’s birth. It is one of the types of neural tube defects (NTD). 
• Tethered cord syndrome: A rare neurological condition where the spinal cord gets tied or attached to the surrounding spinal tissues. Tethered cord syndrome is often associated with other neurological disorders, such as spina bifida and scoliosis. 

Acquired Syringomyelia

It is also known as primary spinal or noncommunicating syringomyelia. In acquired syringomyelia, the cyst forms in the damaged spinal cord. Typically occurs when there are:

• Arachnoiditis: The arachnoid membrane surrounds and protects the spinal cord. When there is an inflammation of this membrane, it results in syringomyelia. The inflammation may be due to several conditions, including sarcoidosis, transverse myelitis, and multiple sclerosis.
• Meningitis: The infection of the protective layer of the spinal cord and brain is called meningitis. The protective layer is known as the meninges.  
• Spinal cord injury: Syringomyelia due to a spinal cord injury is post-traumatic syringomyelia. It may occur months or years after the initial traumatic injury. It develops due to scar tissue blocking the flow of the CSF. 
• Spinal tumour: Tumours such as hemangioblastomas and ependymomas interrupt the CSF’s flow, resulting in syringomyelia. 

Sometimes, acquired syringomyelia occurs without a cause, known as idiopathic syringomyelia. 

What are the symptoms of syringomyelia?

The syringomyelia symptoms gradually develop over time, depending on the size and location. If syringomyelia is due to Chiari malformation, the symptoms appear between ages 25 to 40. In certain instances, coughing and straining trigger the symptoms of the condition, but neither causes the development of syringomyelia. It can occur suddenly, too, due to accidents or falls. 

The symptoms that can affect the different body parts, such as the back, shoulders, arms, and legs, are as follows:

• Weakness in the muscle
• Muscle atrophy 
• Loss of reflexes
• Decreased or loss of sensitivity to pain and temperature
• Headaches
• Stiffness in the neck, back, arms, and legs
• Pain in the neck, back, and shoulders
• Curvature in the spine called scoliosis
• Change or loss of bowel and bladder control
• Sweating profusely 
• Numbness, tingling, burning, or piercing sensation
• Paralysis, in extreme cases
• Sexual issues
• Irregular blood pressure levels
• Involuntary and uncoordinated muscle movements

A patient suffering facial nerve damage can develop eye and face issues, which may lead to Horner syndrome. It leads to droopy eyelids, a narrow opening between the eyelids, reduced pupil size, and decreased sweating in the affected facial area.

When to seek medical help?

A person notices the signs and symptoms of the condition, and visiting a doctor is crucial in managing the condition efficiently. But a person with a spinal cord injury should keep a close watch on any symptoms that may develop after years. In such cases, the doctor should be aware of the medical history. 

If a person is diagnosed with syringomyelia, constantly visiting the healthcare team is essential to monitor the cyst. It also ensures if the patient needs surgical treatment or not.

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How is syringomyelia diagnosed?

The healthcare provider notes down the patient’s medical and family history. Afterwards, a physical examination focuses on the patient’s neurological abilities. 

To confirm the diagnosis, the neurologist may prescribe one or more of the following tests:

• Magnetic resonance imagining (MRI) scan: It is one of the most reliable forms of diagnosing the condition .The test reveals the syrinx as well as its location, size, and severity.
• Dynamic MRI: The tests clearly show the flow of the cerebrospinal fluid around the spinal cord and the cyst. The doctor uses a contrast agent while conducting the test. The contrast agent is injected into the patient’s veins to improve the MRI images. 
• Myelogram with CT scan: A CT scan is the next best option if a patient cannot undergo an MRI. 

In rare cases, a doctor may accidentally discover syringomyelia during a tests ordered for other medical reasons. 

What are the treatment options for syringomyelia?

The treatment of syringomyelia is based on the symptoms and the severity of the condition. The following are some of the treatment options recommended by the doctor:

1. Monitoring: A patient suffering from mild syringomyelia may not need treatment. But, the doctors may have to monitor the progress of the cyst and its symptoms regularly. The patient should avoid heavy lifting and other activities involving straining. The doctor may prescribe pain medication and refer the patient to physical therapy. 
2. Surgery: If symptoms that cause issues in daily life are severe, surgery is considered a good option. The main goal of the surgery is to relieve pain and restore the CSF’s flow to the brain and the spinal cord. Based on the cause and the severity of the condition, the doctor may perform any one or more of the following surgeries 
3. Treating Chiari malformation: If Chiari malformation is the cause of syringomyelia, the surgery removes a small part of the spinal cord present at the back of the skull. The surgery assists in reducing the pressure in the brain and efficiently restores the cerebrospinal fluid flow, thus improving or eliminating the symptoms of the condition. 
4. Syrinx drainage: The doctor inserts a shunt to drain the syrinx. One part is inserted in the syrinx and another in a different part of the body, such as the abdomen. 
5. Clearing obstruction: A tumour or a bone protruding needs immediate surgical removal as it limits the flow of cerebrospinal fluid. The surgery may restore the flow and lets the fluid drain out of the syrinx. 
6. Correcting abnormalities: Abnormalities such as tethered spinal cord need immediate surgical correction to restore the flow and syrinx to drain. 
   1. Before a patient considers the surgical option, it is crucial to know that the surgery may not successfully restore the CSF’s flow, and the cyst may reoccur with the best efforts of draining the fluid. 
7. Follow-up care: After surgery, syringomyelia may occur again. Therefore, the patient should regularly meet the doctor and undergo regular tests to assess the outcome of the surgery. Gradually, the patient may need additional treatment. In severe cases, it may cause permanent spinal cord and nerve damage.
8. Clinical trials: The patient with syringomyelia can opt and explore various clinical trials to detect, prevent, treat, and manage the condition. 
9. Lifestyle and home remedies: The following are specific steps that may assist in reducing the effect of syringomyelia:
   1. Avoid activities, such as heavy lifting, straining, or applying force on the spine that can worsen the symptoms
   2. Consider physical therapy that helps reduce muscle stiffness, weakness, pain, and fatigue
   3. Manage chronic pain with various treatment options. Talk to the doctor about medical centres that specialize in pain management
   4. Coping and support: It is not easy to live with syringomyelia and its complication. Having a family member or friend to talk to can be invaluable. If not, joining a support group helps. Such groups are a good source of valuable information that offers practical tips for patients with syringomyelia.

What are the complications of syringomyelia?

In a few patients, the cyst can increase in size, causing severe and permanent damage to the spinal cord. It can lead to numerous complications, such as the following:

• Chronic and intense pain
• The onset of paralysis of the hands and arms
• Stiffness in the legs and uncoordinated movement
• Uncontrolled muscle contractions and twitches
• Abnormal curvature of the spine
• No feeling in the shoulder or back

A patient may also experience complications from the surgery intended to treat syringomyelia. Therefore it is crucial to discuss the risks and possibility of its occurrence. The following are the complications of the surgery: 

• CSF leaks
• Infection 
• Bleeding
• Additional spinal cord injury
• Reccurrence of the syrinx

Conclusion

Trying and learning to live with syringomyelia can be daunting. Understanding and knowing that a person is not alone is crucial for mental and physical well-being. Talking to the healthcare provider, learning about the disease, joining a support group, and getting regular check-up help in managing the symptoms better. 

Frequently Asked Questions (FAQs)

Is syringomyelia preventable?

Unfortunately, syringomyelia is not preventable. But early diagnosis and treatment help manage and minimize the condition from worsening. 

Is syringomyelia common?

It is a rare condition that affects nearly every 8 out of 10,000 individuals. It accounts for close to 5% of paraplegia cases.