Syringomyelia

Syringomyelia is a condition where a fluid-filled cyst, known as a syrinx, forms within your spinal cord, potentially causing harm over time. This cyst, filled with cerebrospinal fluid (CSF), can grow and stretch, leading to damage to the spinal cord and compression of nerve fibers that transmit signals between your brain and body.

Usually, CSF envelops and safeguards your brain and spinal cord. It also occupies interconnected spaces within your brain called ventricles, which extend into a narrow canal running along your spinal cord.

In syringomyelia, CSF accumulates within the spinal cord tissue, enlarging the central canal and forming a syrinx. This condition often arises due to disruptions in the normal CSF flow around the spinal cord or lower brain stem. When the syrinx affects the brain stem, it’s termed syringobulbia.

Typically, syringomyelia impacts the upper parts of the spinal cord, particularly the cervical and thoracic regions.

Causes of Syringomyelia

The formation of a syrinx, or fluid-filled cyst within the spinal cord, is not fully understood by scientists yet. However, most theories suggest that it’s likely linked to the obstruction or disruption of the flow of cerebrospinal fluid (CSF).

There are two main categories of causes associated with syringomyelia: congenital (present at birth) and acquired (developed later in life).

Congenital causes of syringomyelia:

• Chiari malformation type 1: This condition occurs when the lower part of the cerebellum extends into the opening at the base of the skull, known as the foramen magnum, where the spinal cord normally passes through. This structural abnormality hampers the normal flow of CSF. Although often present at birth, symptoms may not arise until adolescence or adulthood.
• Myelomeningocele: Also called open spina bifida, this birth defect occurs when the backbone and spinal canal fail to close before birth, leading to a type of neural tube defect.
• Tethered cord syndrome: In this rare neurological condition, the spinal cord is abnormally attached to the surrounding tissues of the spine. It’s commonly associated with conditions like spina bifida and scoliosis.

Acquired causes of syringomyelia:

• Spinal cord injuries: Syringomyelia resulting from spinal cord injuries, termed post-traumatic syringomyelia, can develop months or even years after the initial trauma due to scar tissue buildup blocking CSF flow.
• Spinal cord tumors: Tumors such as hemangioblastomas and ependymomas can disrupt CSF circulation, leading to syringomyelia.
• Arachnoiditis: Inflammation of the arachnoid membrane, one of the protective membranes around the spinal cord, can cause syringomyelia. Conditions like sarcoidosis, transverse myelitis, and multiple sclerosis (MS) can lead to this inflammation.
• Meningitis: Infection of the meninges, the protective lining around the brain and spinal cord, can result in syringomyelia.

In some instances, syringomyelia may develop without a known cause, termed idiopathic syringomyelia.

Who is prone to syringomyelia?

This condition is commonly diagnosed in individuals aged 20 to 50, though it can also emerge in young children or older adults.

How prevalent is syringomyelia?

Syringomyelia is uncommon, affecting about 8 in every 100,000 people. It contributes to approximately 5% of cases involving paralysis of the legs and lower body, known as paraplegia.

Symptoms of Syringomyelia

The symptoms of syringomyelia typically develop gradually over many years and can vary depending on the location, size, and extent of the syrinx along the spine. Some individuals may have a small syrinx for an extended period without experiencing any symptoms. These symptoms may affect one or both sides of the body and can range from mild to severe. Here are the common symptoms:

1. Headaches: Persistent headaches may occur.
2. Pain: Pain can radiate down the arms, neck, into the middle of the back, or down the legs (known as radiculopathy).
3. Weakness and muscle atrophy: Progressive weakness and wasting of muscles in the arms and/or legs.
4. Muscle spasms or tightness: Spasms or tightness may be experienced in the back, shoulders, neck, arms, or legs.
5. Loss of sensation: Decreased sensitivity to pain or temperature, particularly in the hands.
6. Numbness or tingling: Sensations of numbness or tingling in the arms and/or legs.
7. Balance and walking difficulties: Problems with balance and coordination, leading to difficulty walking.
8. Loss of bowel and bladder control: Incontinence or difficulty controlling bowel and bladder functions.
9. Sexual dysfunction: Issues with sexual function may arise.

For children, additional symptoms may include:

1. Scoliosis: Curvature of the spine may occur.
2. Toe walking: Walking on tiptoes may be observed.

If you or your child are experiencing any of these symptoms, it is crucial to seek medical attention promptly.

When to Seek Medical Attention

If you experience any worsening of symptoms, new neurological deficits, or significant changes in your condition, promptly consult your healthcare provider. Additionally, adhere to your scheduled follow-up appointments to ensure comprehensive management of your syringomyelia.

By actively managing your condition and collaborating closely with your healthcare team, you can optimize your well-being and minimize the impact of syringomyelia on your daily life.

Diagnosis

Diagnosing syringomyelia involves a thorough examination of medical history and neurological function, often conducted by a neurologist. Imaging tests are crucial for confirmation, and they may be ordered even if syringomyelia is not initially suspected.

Diagnostic Tests for Syringomyelia:

1. MRI (Magnetic Resonance Imaging) Scan: This imaging test provides detailed pictures of internal structures using a powerful magnet and computer. It’s the most reliable method for diagnosing syringomyelia, enabling visualization of the syrinx, as well as any other abnormalities like tumors. MRI reveals the location, size, and extent of the syrinx accurately.
2. Dynamic MRI: This specialized MRI variant tracks the flow of cerebrospinal fluid (CSF) around the spinal cord and within the syrinx. Contrast dye may be injected into a vein to enhance image quality.
3. Myelogram with CT Scan: In cases where MRI isn’t feasible, a myelogram using contrast dye and computed tomography (CT) can be performed to examine the spinal canal for abnormalities.

Treatments

Treatment for syringomyelia depends on the presence and severity of symptoms. If the condition is asymptomatic, regular monitoring by a neurologist or neurosurgeon is advised. However, if symptoms are present, treatment aims to address the underlying cause, manage symptoms, and prevent further spinal cord damage.

General Treatment Goals:

1. Addressing the underlying cause: Treatment focuses on correcting the condition causing syringomyelia, if possible.
2. Symptom management: Managing pain and other symptoms associated with syringomyelia.
3. Restoring CSF flow: Preventing further spinal cord damage by restoring proper cerebrospinal fluid (CSF) flow.

Non-Surgical Management:

1. Pain management: Medications may be prescribed to alleviate pain.
2. Physical therapy: Exercises and rehabilitation programs can help improve mobility and function.
3. Activity modification: Limiting activities that strain the spine, such as heavy lifting, may be advised.

Surgical Options:

1. Treatment of Chiari malformation: Posterior fossa decompression surgery aims to create more space at the base of the skull and upper neck to relieve pressure on the brain and spinal cord. This procedure can facilitate syrinx drainage and symptom improvement.
2. Management of post-traumatic syringomyelia: Expansive duraplasty involves removing scar tissue around the spinal cord and enlarging the membrane surrounding it. This helps restore normal CSF flow and prevents syrinx formation or growth.
3. Removal of obstructions: Surgical removal of scar tissue, bone fragments, or tumors obstructing CSF flow can alleviate syringomyelia. Radiation therapy may be considered for tumor shrinkage.
4. Syrinx drainage: Inserting a drain (stent or shunt) into the syrinx allows fluid to be redirected outside the spinal cord. Stabilizing or reducing the syrinx size can halt symptom progression.

Post-Surgery Follow-up:

After surgery, regular MRI scans are conducted to monitor syrinx stability or reduction in size. Although surgery can improve symptoms and stabilize syringomyelia, recurrence may occur, necessitating additional procedures.

Overall, treatment approaches are tailored to individual circumstances, aiming to alleviate symptoms, prevent complications, and improve quality of life for individuals with syringomyelia.

Complications with the surgery

Complications can arise following surgery to treat syringomyelia, including:

1. Cerebrospinal Fluid (CSF) leaks: Leakage of CSF can occur, leading to complications such as headaches and infection.
2. Infection: Surgical site infections may develop, requiring antibiotics or additional treatment.
3. Hemorrhage (Bleeding): Excessive bleeding during or after surgery is a potential complication.
4. Further Spinal Cord Injury: Despite surgical intervention, there’s a risk of additional damage to the spinal cord.
5. Recurrence of the Syrinx: In some cases, the syrinx may return, necessitating further treatment.

Discussing these risks with your neurosurgeon and understanding their likelihood in your specific case is crucial.

Prevention

While syringomyelia typically cannot be prevented, early diagnosis and treatment may help prevent symptom progression.

Outlook/Prognosis

The prognosis for syringomyelia varies widely due to differences in symptoms, severity, and causes among individuals. Factors such as the rate of progression and response to treatment further complicate prognostication.

Early surgical intervention often results in symptom improvement and better outcomes. However, neurologic deficits may stabilize rather than fully resolve after surgery. A syrinx diameter exceeding five millimeters and associated swelling generally indicate a poorer prognosis.

Myelopathy, characterized by spinal cord injury due to severe compression, is a significant concern. It can lead to complications like paralysis, recurrent pneumonia, and bowel and bladder dysfunction.

Living With syringomyelia

If you’re living with syringomyelia, taking proactive steps to manage your condition can help alleviate symptoms and improve your quality of life. Here are some tips to consider:

1. Avoid Aggravating Activities: Refrain from activities that may exacerbate your symptoms, such as heavy lifting, straining, or putting undue pressure on your spine, like jumping.

2. Physical Therapy: Consider enrolling in a physical therapy program tailored to address mobility issues, muscle weakness or stiffness, and pain associated with syringomyelia. Physical therapists can provide exercises and techniques to improve your overall function and alleviate discomfort.

3. Pain Management: Chronic pain is a common symptom of syringomyelia. Discuss with your healthcare provider about available treatment options to manage your pain effectively. This may include medications, physical therapy modalities, or other interventions.

4. Mental Health Support: Coping with syringomyelia can be challenging, both physically and emotionally. Seek support from friends, family, or a therapist to help you navigate the emotional aspects of living with a chronic condition. Joining a support group comprising individuals with syringomyelia can also provide valuable peer support and a sense of community.

5. Regular Follow-up: It’s essential to maintain regular follow-up appointments with your healthcare team, whether you’ve undergone surgical treatment or not. Regular monitoring allows for the assessment of symptoms and the syrinx’s progression, facilitating timely interventions as needed.

Sources

• National Institute of Neurological Disorders and Stroke: “Syringomyelia Fact Sheet.”
• Mayo Clinic: “Syringomyelia.”
• Mayfield Brain & Spine: “Chiari I Malformation.”
• National Organization for Rare Disorders: “Syringomyelia.”
• American Syringomyelia & Chiari Alliance Project: “Syringomyelia.”
• Clevland Clinic: “Syringomyelia.”