Definition

Syringomyelia is a rare neurological disease characterized by the formation of a fluid-filled cyst in the spinal cord. This fluid is cerebrospinal fluid (CSF), which surrounds and protects the brain and spinal cord. When the flow of CSF is blocked, excess fluid can enter the spinal cord, leading to cyst formation.

Over time, the cyst may enlarge and damage the spinal cord, though some cysts may stabilize or even disappear. If the spinal cord is damaged, it can cause pain, weakness, and stiffness.

Syringomyelia has several possible causes, most cases are associated with the condition where brain tissue protrudes into the spinal canal (Chiari malformation). Other causes include spinal cord tumors, spinal injuries, or inflammation around the spinal cord.

If syringomyelia causes no symptoms, monitoring the patient's condition is sufficient. However, if it causes disturbing symptoms, surgery may be necessary.

Causes

Syringomyelia forms when cerebrospinal fluid accumulates to create a cyst within the spinal cord. Several conditions and diseases can cause syringomyelia, including:

• Chiari Malformation: A congenital condition where brain tissue protrudes into the spinal canal.
• Meningitis: Inflammation of the membranes surrounding the brain and spinal cord.
• Spinal Cord Tumors: These can disrupt the normal circulation of cerebrospinal fluid.
• Congenital Conditions: For instance, when tissue adheres to the spinal cord, limiting its movement.
• Spinal Cord Injury: Symptoms may appear months or years later.

Risk factor

The main risk factors for syringomyelia relate to its underlying causes. These include:

• Iatrogenic Factors: Risks arising from medical interventions, such as significant bleeding at surgery sites or sharp injuries, which can increase the risk of fibrosis (scar tissue) obstructing CSF circulation.
• Other Risk Factors: Spinal canal stenosis (>25%) and post-injury kyphosis (>15%).
• Congenital Malformations: Conditions such as Chiari malformation, Klippel Feil syndrome, or congenital scoliosis.
• Post-infection: Formation of arachnoiditis (inflammation of the arachnoid layer) and dura abscesses.
• Post-injury: Spinal injuries are significant risk factors.
• Post-inflammation: Conditions like multiple sclerosis, neurosarcoidosis, and amyotrophic lateral sclerosis (ALS).
• Neoplasms or Tumors: Ependymoma and hemangioblastoma are tumors often associated with syringomyelia.

Symptoms

Symptoms of syringomyelia usually develop slowly over time. They appear when the fluid-filled cyst presses on spinal cord nerves. Symptoms indicate the cyst's location and the degree of pressure it exerts. If caused by Chiari malformation, symptoms typically begin between ages 25 and 40.

In some cases, coughing or straining may trigger syringomyelia symptoms, though these actions do not cause the condition. Symptoms affecting the back, shoulders, arms, or legs include:

• Muscle Weakness and Atrophy
• Loss of Reflexes
• Loss of Sensation to Pain and Temperature
• Headache
• Stiffness in Back, Shoulders, Arms, and Legs
• Pain in Neck, Arms, and Back
• Abnormal Spine Curvature (Scoliosis)
• Digestive and Urinary Issues like Incontinence

Diagnosis

To diagnose syringomyelia, doctors will inquire about symptoms, health history, and perform a complete physical examination. Sometimes, syringomyelia is discovered incidentally during MRI or CT scans of the spine for other reasons.

If syringomyelia is suspected, the following tests may be recommended:

• MRI: The best test for diagnosing syringomyelia. It uses radio waves and strong magnets to create detailed images of the spine and spinal cord. If a cyst is present, it will be visible. Contrast dye may be injected to identify tumors or other abnormalities. Multiple MRIs may be conducted to monitor the condition.
• CT Scan: Uses X-rays to create detailed images of the spine and spinal cord, detecting tumors or other spinal conditions.

Management

Treatment for syringomyelia depends on the severity and progression of symptoms.

Monitoring: If there are no symptoms, regular neurological exams and MRIs are sufficient.

Surgery: If symptoms are disruptive or worsen quickly, surgery may be recommended to relieve spinal cord pressure caused by the cyst and restore normal CSF flow. Types of surgery depend on the cause of syringomyelia and include:

• Treating Chiari Malformation: Involves removing a small section of bone from the back of the skull to reduce pressure on the brain and spinal cord, restoring normal CSF flow, and alleviating symptoms.
• Draining the Cyst: Inserting a shunt (flexible tube) to drain fluid from the cyst to another body area, like the abdomen.
• Removing Obstructions: Surgery to remove tumors or abnormal bone growths blocking CSF flow.
• Correcting Abnormalities: Surgery to correct spinal abnormalities that block CSF flow and drain the cyst.

Surgery may not always restore CSF flow, and the cyst may persist, but efforts to drain it can delay worsening symptoms.

Follow-Up

Syringomyelia can recur post-surgery. Patients need regular check-ups to assess surgical outcomes, as cysts may regrow and require additional treatment. Some symptoms may persist due to permanent nerve and spinal cord damage.

Living with syringomyelia and its complications can be challenging. Having a support system, such as friends, counsellors, or therapists, is beneficial. Support groups for syringomyelia patients provide valuable resources, shared experiences, and tips.

Complications

Syringomyelia can develop into serious complications or remain asymptomatic. Complications arise when the cyst enlarges or damages spinal cord nerves, potentially leading to:

• Abnormal Spine Curvature (Scoliosis)
• Chronic Pain
• Motor Impairments like Muscle Weakness and Stiffness
• Paralysis

Prevention

There is no known effective way to prevent syringomyelia. However, these steps can help reduce symptoms and prevent worsening:

• Avoid activities that exacerbate symptoms, such as heavy lifting or straining.
• Consider physical therapy for mobility and activity impairments like muscle weakness, pain, fatigue, or stiffness. A physical therapist can design an exercise program to alleviate symptoms. Consult a doctor for recommendations.
• Manage chronic pain: Seek medical advice for pain management options. Many health centers have pain management specialists.

When to see a doctor?

If you experience any syringomyelia symptoms, consult your doctor. Be vigilant for signs of syringomyelia if you've had a spinal injury, as symptoms can appear months or years later. Discuss your spinal injury with your doctor.

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