SYRINGOMYELIA: 

Questions and Answers

What Factors Affect the Prognosis of Syringomyelia?

The outlook for syringomyelia can vary widely from person to person, and several factors influence how the condition progresses. The underlying cause—whether it stems from a Chiari malformation, spinal tumor, trauma, or another source—plays a significant role in determining both the severity and the pace at which symptoms develop.

Early detection and treatment are crucial. Surgical intervention, when performed before significant nerve damage occurs, often leads to better outcomes and may help stabilize or even improve symptoms. However, it’s important to note that even after successful surgery, some people may continue to experience lingering symptoms.

Other factors include:

• Size and Location of the Syrinx: Larger Syrinxes (especially those wider than 5 millimeters) and those causing swelling in the spinal cord tend to be associated with a greater risk of complications.
• Extent of Spinal Cord Involvement: Syrinxes that damage critical areas can result in neurological issues such as weakness, loss of sensation, or impaired coordination.
• Duration of Symptoms Before Treatment: The longer the spinal cord is compressed by the syrinx, the higher the risk of lasting nerve damage.
• Presence of Complications: Conditions like myelopathy (spinal cord injury due to compression), scoliosis, and problems with bladder or bowel function can all affect the overall prognosis.

Ultimately, regular follow-up with specialists and prompt attention to new or worsening symptoms play a critical role in managing this complex condition. Early action can help limit permanent damage and improve long-term quality of life.

Who is Most Likely to Be Affected by Syringomyelia?

Syringomyelia can appear at almost any age, but it is most commonly diagnosed in adults between 20 and 50 years old. That said, children and older adults are not immune—cases can and do occur outside this typical age range. No matter the age, recognizing early warning signs and seeking medical advice is crucial for managing the condition and reducing the risk of long-term complications.

Can Syringomyelia Be Prevented?

Currently, there is no guaranteed way to prevent syringomyelia, as many causes—such as congenital abnormalities or spinal cord injuries—are beyond our control. However, recognizing symptoms early and seeking prompt medical care can make a significant difference. Early intervention can slow the progression of symptoms and reduce the risk of long-term complications. Regular monitoring, particularly for individuals with underlying risk factors like Chiari malformation or a history of spinal trauma, is key to catching changes before they become serious. As always, maintaining a healthy spine and promptly addressing any spinal injuries can support your overall neurological health.

Syringomyelia Causes:

While the exact cause of syrinx formation remains elusive, medical professionals have identified several factors that contribute to its development. Here’s an in-depth exploration of the potential causes of syringomyelia, combined with an understanding of how a syrinx forms within the spinal cord:

Formation of a Syrinx:

Cerebrospinal fluid (CSF) normally circulates around the brain and spinal cord, playing a crucial role in cushioning and protecting these vital structures. However, when the flow of CSF is obstructed, it can lead to the formation of a syrinx—a fluid-filled cyst—within the delicate tissue of the spinal cord. This obstruction causes the CSF to be redirected and accumulate, gradually forcing fluid into the cyst due to pressure differences between the brain and spinal cord. Over time, this cyst grows larger, exerting pressure on the surrounding spinal cord nerves, potentially leading to nerve damage. Syringomyelia typically progresses slowly, but symptoms can sometimes advance rapidly after actions that increase CSF pressure, such as straining or coughing.

Congenital Abnormalities (Communicating Syringomyelia):

• Chiari Malformation:  This is the most frequent cause of syringomyelia, particularly the communicating type. In Chiari malformation, part of the brain tissue protrudes through the skull’s base into the spinal canal. This can obstruct the flow of CSF, the fluid that surrounds and cushions the brain and spinal cord. The disrupted CSF flow can then put pressure on the spinal cord and potentially contribute to syrinx formation.

Acquired Causes (Non-Communicating Syringomyelia):

• Spinal Cord Injuries: Trauma to the spinal cord can disrupt the delicate balance of CSF flow and potentially lead to syrinx development. Scarring at the injury site can also contribute to this process.
• Meningitis and Arachnoiditis: Inflammatory conditions affecting the meninges (membranes surrounding the brain and spinal cord) or arachnoid (one of the meninges) can cause scarring and adhesions that obstruct CSF flow, potentially leading to syrinx formation.
• Spinal Cord Tumors: Tumors within the spinal cord can directly compress the spinal cord tissue or disrupt CSF flow. Both of the aforementioned conditions can cause syrinx development.
• Tethered Cord Syndrome: A congenital condition where the spinal cord is attached too tightly to the spinal canal, tethered cord syndrome can put abnormal tension on the spinal cord and disrupt CSF flow, potentially leading to syrinx formation.

Understanding both the causes and the mechanisms of syrinx formation provides a comprehensive view of syringomyelia, guiding both diagnosis and treatment strategies for affected individuals.

Idiopathic Cases:

In a significant portion of syringomyelia cases, doctors cannot identify a cause. These are termed idiopathic syringomyelia. Researchers believe that unidentified genetic or environmental factors are associated with these cases.

Overview of Syringomyelia Types:

To summarize, syringomyelia can be broadly categorized into three main types:

• Congenital Brain Defects: Such as Arnold-Chiari malformation, where brain tissue lies in the upper neck area instead of the skull, obstructing CSF flow and possibly linking to hydrocephalus.
• Spinal Cord Injuries: Includes trauma, tumors, infections (like meningitis, HIV), and severe cord compression, leading to syrinx formation.
• Idiopathic Syringomyelia: Cases where the cause remains unknown, potentially involving unidentified genetic or environmental factors.

This breakdown not only highlights the specific conditions associated with syringomyelia but also provides a comprehensive understanding of the different types, making it easier to grasp how each category contributes to the development of this condition.

• Chiari Malformation: This is the most frequent cause of syringomyelia, particularly the communicating type. In Chiari malformation, part of the brain tissue protrudes through the skull’s base into the spinal canal. This can obstruct the flow of cerebrospinal fluid (CSF), the fluid that surrounds and cushions the brain and spinal cord. The disrupted CSF flow can then put pressure on the spinal cord and potentially contribute to syrinx formation.

Acquired Causes (Non-Communicating Syringomyelia):

• Spinal Cord Injuries: Trauma to the spinal cord can disrupt the delicate balance of CSF flow and potentially lead to syrinx development. Scarring at the injury site can also contribute to this process.
• Meningitis and Arachnoiditis: Inflammatory conditions affecting the meninges (membranes surrounding the brain and spinal cord) or arachnoid (one of the meninges) can cause scarring and adhesions that obstruct CSF flow, potentially leading to syrinx formation.
• Spinal Cord Tumors: Tumors within the spinal cord can directly compress the spinal cord tissue or disrupt CSF flow.  Both of the aforementioned conditions can cause syrinx development.
• Tethered Cord Syndrome: A congenital condition where the spinal cord is attached too tightly to the spinal canal, tethered cord syndrome can put abnormal tension on the spinal cord and disrupt CSF flow, potentially leading to syrinx formation.

Idiopathic Cases:

In a significant portion of syringomyelia cases, doctors cannot identify a cause. These are termed idiopathic syringomyelia. Researchers believe that unidentified genetic or environmental factors are associated with these cases.

Important Considerations:

• The specific cause of syringomyelia can influence the location and size of the syrinx. For example, syrinxes associated with Chiari malformation are typically located in the lower cervical (neck) region of the spinal cord.
• In some cases, multiple factors might contribute to syringomyelia development. For instance, someone with a Chiari malformation could also experience a spinal cord injury, further increasing the risk of syrinx formation.

Ongoing Research:

While significant progress has been made in understanding the potential causes of syringomyelia, research is ongoing to identify the exact mechanisms behind syrinx development. This will hopefully lead to improved diagnostic methods, more targeted treatment options, and potentially even preventive strategies.

Syringomyelia Symptoms:

Syringomyelia, a condition characterized by fluid-filled cavities (syrinx) within the spinal cord, can cause a wide range of symptoms depending on the location, size, and growth rate of the syrinx. Here’s an in-depth exploration of the potential symptoms of syringomyelia:

Pain:

One of the most common symptoms of syringomyelia is chronic pain. The nature of the pain can vary depending on the location of the syrinx. The pain appears as:

• Burning or lancing
• Aching
• Numbness with a pins-and-needles sensation (dysesthesia)
• The pain often affects one side of the body more than the other (unilateral).

Sensory Loss:

Damage to the spinothalamic tract in the spinal cord, responsible for carrying sensory information to the brain, can lead to a variety of sensory disturbances. These can include:

• Loss of sensation to pain and temperature, particularly in the hands and arms (thermal-pain dissociation)
• Numbness and tingling
• Reduced ability to feel light touch or vibration

Motor Impairment:

• Depending on the location and size of the syrinx, progressive muscle weakness and wasting (atrophy) can occur. This can affect the hands, arms, legs, or even the muscles involved in breathing.
• In severe cases, syringomyelia can lead to paralysis.

Other Potential Symptoms:

• Balance problems and gait abnormalities: These can occur if the syrinx affects the areas of the spinal cord responsible for coordination and balance.
• Scoliosis: A curvature of the spine can develop in some cases of syringomyelia, particularly those associated with Chiari malformation.
• Bowel and bladder dysfunction: Syrinxes in the lower spinal cord can affect nerves controlling bladder and bowel function.  This leads to incontinence or difficulty with urination and bowel movements.
• Headaches: Some people with syringomyelia, especially those with Chiari malformation, may experience headaches that worsen with coughing, straining, or bending forward.
• Hearing loss and dizziness: These symptoms are less common but can occur if the syrinx affects the brainstem.

Progression of Symptoms:

• The symptoms of syringomyelia often develop gradually over time. However, the rate of progression can vary significantly from person to person. In some cases, the syrinx may remain stable or even shrink on its own.
• This condition usually progresses slowly over the years, but it’s important to note that symptoms can sometimes advance rapidly. This rapid change often occurs after actions like straining or coughing. These activities can temporarily increase cerebrospinal pressure, forcing fluid into the cyst and exacerbating symptoms.
• Early diagnosis and treatment are crucial to prevent the worsening of symptoms and potential complications. Understanding these dynamics can help in managing the condition effectively and improving the quality of life.

Important Considerations:

• The specific combination of symptoms a person experiences will depend on the location and size of the syrinx. For example, a syrinx in the cervical spine (neck) will cause pain and weakness in the arms and hands However, a syrinx in the lower spine (lumbar region) might affect the legs and bladder function.
• It’s important to note that the cause of some of these symptoms points to other medical conditions. A thorough evaluation by a healthcare professional is necessary for an accurate diagnosis.

By understanding the spectrum of symptoms associated with syringomyelia, individuals and healthcare professionals can work together to achieve earlier diagnosis.  This will lead to more effective management of this complex condition.

How Symptoms Differ in Adults and Children

While syringomyelia shares many hallmark symptoms across all ages, there are subtle differences in how this condition typically manifests in adults compared to children.

Adults
Adults with syringomyelia often experience symptoms that reflect the disruption of normal spinal cord function, such as:

• Persistent headaches, especially at the back of the head or neck
• Pain radiating along the arms, neck, back, or legs (sometimes described as shooting or burning)
• Gradual muscle weakness or wasting in the arms, hands, or legs, which might make everyday tasks more challenging
• Stiffness or spasticity in the back, shoulders, or limbs
• Reduced ability to perceive pain or temperature—this is classically most noticeable in the hands
• Episodes of numbness, tingling, or a pins-and-needles sensation in the limbs
• Difficulty maintaining balance and unsteady gait
• In some cases, problems with bowel or bladder function, or sexual dysfunction

Children
In children, syringomyelia can present differently, sometimes making diagnosis more challenging. Beyond the symptoms seen in adults, children may develop:

• Scoliosis (an abnormal curvature of the spine), which can be the first sign that draws attention to an underlying spinal problem
• Toe walking or other unusual gait patterns, reflecting mild muscle weakness or tightness

Children may not always be able to describe subtle changes in sensation or pain, so close attention to changes in posture, walking, or spinal alignment is important.

Understanding these age-related differences not only aids in recognizing syringomyelia early but also highlights the importance of tailored evaluation. Prompt identification of these symptoms—whether in adults or children—can make a significant difference in management and quality of life.

Syringomyelia Diagnosis:

Diagnosing syringomyelia typically involves a combination of clinical evaluation, imaging studies, and neurological tests. The following identify diagnostic tools:

• MRI Scan: Magnetic resonance imaging (MRI) is the gold standard for diagnosing syringomyelia. It allows for detailed visualization of the spinal cord and any associated abnormalities, including the presence of a syrinx.
• Neurological Examination: A thorough neurological examination helps assess muscle strength, sensation, reflexes, and coordination, which may be affected by syringomyelia.
• Electromyography (EMG): EMG testing evaluates the electrical activity of muscles and nerves, helping to identify areas of dysfunction or damage.
• Cerebrospinal Fluid Analysis: In some cases, analysis of cerebrospinal fluid obtained through a lumbar puncture will rule out other potential causes of symptoms.

Diagnosis of syringomyelia typically begins with a thorough medical history review and physical examination by a healthcare professional. During the physical examination, the doctor may assess neurological functions, such as muscle strength, reflexes, sensation, and coordination. However, due to the subtle nature of some symptoms, the condition may go undetected during routine examinations.

Imaging Studies

Medical imaging plays a crucial role in diagnosing syringomyelia. The most common imaging tests used are magnetic resonance imaging (MRI) and computed tomography (CT) scans. These imaging techniques allow healthcare providers to visualize the spinal cord and detect any abnormalities, such as the presence of a syrinx (fluid-filled cavity) within the spinal cord.

MRI is the preferred imaging modality for diagnosing syringomyelia as it provides detailed images of the spinal cord. Additionally, it does so without exposing the patient to radiation. During an MRI scan, a strong magnetic field and radio waves are used to create detailed cross-sectional images of the spinal cord and brain. These images help identify the location, size, and extent of the syrinx, as well as any underlying causes, such as Chiari malformation or spinal cord tumors.

Additional Tests

In some cases, doctors will perform additional tests to evaluate spinal cord function and assess the severity of neurological impairment. These tests may include electromyography (EMG), nerve conduction studies (NCS), and somatosensory evoked potentials (SSEPs). EMG and NCS evaluate the electrical activity of muscles and nerves, while SSEPs measure the speed of nerve signals traveling along the spinal cord.

Overall, a combination of clinical evaluation and diagnostic imaging is essential for accurately diagnosing syringomyelia. In addition, it will aid in determining the appropriate course of treatment. Early diagnosis and intervention can help prevent further neurological damage and improve patient outcomes.

Nonsurgical Treatment of Syringomyelia:

Syringomyelia is a neurological condition characterized by the formation of a fluid-filled cyst within the spinal cord, known as a syrinx. While doctors may recommend surgery in some cases, non-surgical treatments play a crucial role in managing symptoms and improving the quality of life for individuals with syringomyelia. Therefore, these non-surgical approaches aim to alleviate symptoms, slow the progression of the syrinx, and address underlying contributing factors. Here, we explore various non-surgical treatments for syringomyelia:

Medication Management:

Medications cannot cure syringomyelia, but they play a vital role in symptom management. Their primary function is to relieve pain and improve comfort for patients. Here are the key types of medications used:

• Pain Management: Medications such as nonsteroidal anti-inflammatory drugs may help alleviate pain associated with syringomyelia.
• Muscle Relaxants: Muscle spasms and stiffness commonly occur in individuals with syringomyelia. Muscle relaxants can help reduce muscle tightness and discomfort.
• Neuropathic Medications: Doctors may prescribe anticonvulsant drugs or tricyclic antidepressants to manage neuropathic pain and sensory symptoms.

While medications can’t treat the underlying cause of syringomyelia, they are essential in managing the symptoms effectively.

Physical Therapy:

• Physical therapy plays a crucial role in managing syringomyelia-related symptoms by improving strength, flexibility, posture, and overall mobility.
• Therapeutic exercises tailored to the individual’s needs can help strengthen muscles, improve range of motion, and alleviate discomfort.
• Modalities such as heat therapy, cold therapy, ultrasound, and transcutaneous electrical nerve stimulation (TENS) will enter the treatment plans to relieve pain and promote healing.

Occupational Therapy:

• Occupational therapists work with individuals with syringomyelia to address activities of daily living (ADLs) and optimize functional independence.
• Doctors may recommend adaptive equipment and techniques to help individuals overcome challenges related to fine motor skills, self-care tasks, and vocational activities.
• Environmental modifications and ergonomic assessments can also help create a safe and supportive living and work environment.

Pain Management Interventions:

• In addition to medication management, doctors will use interventional pain management techniques to provide targeted relief for localized pain.
• Doctors will recommend epidural injections or nerve blocks to reduce inflammation and alleviate pain associated with nerve compression or irritation.
• Radiofrequency ablation (RFA) or spinal cord stimulation (SCS) may be considered for individuals with refractory pain who have not responded to conservative measures.

Monitoring and Follow-Up:

• Regular monitoring and follow-up with healthcare providers are essential for individuals undergoing non-surgical treatment for syringomyelia.
• Close monitoring of symptoms, disease progression, and treatment response allows for timely adjustments to the treatment plan and optimization of outcomes.
• Healthcare providers may also provide education and support to help individuals effectively manage their condition and make informed decisions about their care.

Consistent check-ins are not only important for those managing syringomyelia without surgery, but also for anyone diagnosed with the condition, regardless of their treatment plan. Whether you’ve had surgical intervention or are following a conservative approach, regular follow-up appointments and imaging studies help keep track of changes in your symptoms and the size or impact of the syrinx. This proactive care ensures that any new symptoms or complications are identified early, and your care team can adjust your treatment as needed to maintain your quality of life.

In conclusion, although doctors may recommend surgical intervention in some cases of syringomyelia, non-surgical treatments play a crucial role in symptom management and improving quality of life. By utilizing a comprehensive approach that incorporates medication management, physical and occupational therapy, pain management interventions, and close monitoring, individuals with syringomyelia can effectively manage their condition and optimize long-term outcomes.

Tips for Managing Syringomyelia in Daily Life

Living with syringomyelia requires mindful adjustments and a proactive approach to daily routines. Here are some practical strategies to help manage symptoms and maintain overall well-being:

• Modify Activities: Be mindful to avoid activities that could strain the spine or worsen symptoms. This might include steering clear of heavy lifting, intense physical exertion, or sudden, jarring movements. When in doubt, consult your healthcare team about safe activity levels.
• Prioritize Physical Therapy: Make physical therapy a regular part of your routine if recommended. Consistent guided exercises can help you maintain strength, flexibility, and balance, all while easing discomfort and improving function.
• Be Proactive with Pain Management: If chronic pain becomes an issue, open communications with your healthcare provider. This will allow you to explore various pain management options. These could include medications, interventional procedures, or non-pharmacological techniques as part of a personalized plan.
• Create an Ergonomic Environment: Ensure your home and workspace are set up to support your physical needs. Simple modifications like ergonomic chairs, supportive mattresses, or assistive devices can make daily tasks safer and more comfortable.
• Prioritize Emotional Well-Being: Living with a chronic condition can understandably take a toll on your mental health. Don’t hesitate to reach out for support.  Whether that’s through family, friends, professional counseling, or connecting with a support group of others facing similar challenges.

By incorporating these self-care strategies, individuals with syringomyelia can better manage daily challenges.  They can also minimize discomfort, and maintain as active and fulfilling a lifestyle as possible.

Conservative (Wait-and-See) Approach

When encountering syringomyelia, a wait-and-see approach might be suitable under specific circumstances. This strategy is often considered when the cyst, or syrinx, is relatively small and does not cause noticeable symptoms or complications. In such scenarios, healthcare providers may choose regular monitoring over immediate intervention. This involves scheduling periodic check-ups and imaging tests to ensure the cyst does not enlarge or begin to exert pressure on the spinal cord.

This conservative approach can be beneficial in avoiding unnecessary procedures if the condition remains stable. It allows patients to maintain their current quality of life while closely observing for any changes that might necessitate future treatment.

Syringomyelia Surgical Treatment:

Surgical intervention is often necessary to address the underlying cause of syringomyelia and decompress the spinal cord. The specific surgical approach depends on the underlying condition. Still, it may include procedures such as posterior fossa decompression for Chiari malformation, tumor resection for spinal cord tumors, or untethering surgery for tethered cord syndrome. In some cases, surgeons may also perform syrinx drainage or shunting to divert CSF and reduce pressure within the syrinx.

Doctors use various surgical treatments to address syringomyelia, depending on the underlying cause and severity of the condition. Some common surgical procedures include:

Posterior Fossa Decompression:

This procedure is typically performed to address syringomyelia associated with Chiari malformation. Chiari malformation is a condition where the lower part of the cerebellum extends into the spinal canal. During posterior fossa decompression, the surgeon removes a small portion of the skull and/or the first cervical vertebra (C1) to create more space and relieve pressure on the spinal cord. By restoring normal cerebrospinal fluid (CSF) flow, this surgery aims to prevent further progression of syringomyelia.

Laminectomy:

Laminectomy involves the removal of a portion of the vertebral bone (lamina) to decompress the spinal cord. This allows for the removal of any obstructions causing syringomyelia, such as tumors or bone spurs. This procedure may be performed in cases where syringomyelia is secondary to spinal stenosis or other structural abnormalities.

Shunt Placement:

In some cases, particularly when syringomyelia is associated with hydrocephalus or other conditions causing cerebrospinal fluid (CSF) buildup, a shunt may be placed to divert excess fluid away from the spinal cord. A shunt is a small tube-like device inserted into the brain ventricles or spinal canal, allowing CSF to drain into another part of the body, such as the abdomen, where it can be reabsorbed.

Syrinx Drainage:

In cases where the syrinx is large or causing significant neurological symptoms, surgeons may perform surgical drainage to remove fluid.  The fluid is removed from the cyst and alleviates pressure on the spinal cord. This may involve creating a small opening in the cyst wall or inserting a catheter to drain fluid.

Spinal Fusion:

In some instances, particularly when syringomyelia is associated with spinal instability or deformity, doctors may recommend spinal fusion. Spinal fusion involves fusing two or more vertebrae using bone grafts, rods, screws, or other instrumentation to stabilize the spine and prevent further progression of the condition.

These surgical treatments aim to alleviate symptoms, prevent neurological deterioration, and improve overall quality of life for patients with syringomyelia. The choice of procedure depends on factors such as the underlying cause of syringomyelia, the extent of spinal cord compression, and the patient’s overall health and treatment goals.

Potential Complications of Surgical Treatment

As with any surgical procedure, there are certain risks associated with operations for syringomyelia. While these complications are not guaranteed, it’s important for patients and families to be aware of them so they can make informed decisions alongside their neurosurgeon.

Some potential complications following syringomyelia surgery include:

• Cerebrospinal Fluid (CSF) Leaks: Sometimes, the fluid that bathes the brain and spinal cord may leak from the surgical site, requiring additional intervention.
• Infection: As with other surgeries, there is always a risk of infection at the incision site or deeper within the spinal tissues.
• Bleeding (Hemorrhage): Excess bleeding can occur during or after surgery and may require further treatment.
• Neurological Injury: Any spinal surgery carries a risk of further injury to the spinal cord or nerves, which could impact motor skills or sensation.
• Recurrence of Syrinx: In some cases, the syrinx (fluid-filled cavity) can return, requiring additional monitoring or further surgical intervention.

It’s crucial to discuss these risks in detail with your surgical team, as the likelihood and severity of complications can vary depending on your overall health, the specific procedure performed, and the underlying cause of syringomyelia.

Benefits of Surgical Treatment:

Surgical treatment aims to alleviate symptoms, halt disease progression, and prevent further neurological damage. By addressing the underlying cause of syringomyelia and restoring normal CSF flow, surgery can improve neurological function, relieve pain, and enhance the quality of life for affected individuals.

Syringomyelia Recovery Period and Rehabilitation:

The recovery period following surgery for syringomyelia varies depending on the complexity of the procedure and the individual patient’s condition. Patients may require a period of immobilization and rehabilitation to regain strength and mobility.  Doctors will monitor the progress and assess surgical outcomes and detect any complications.

Reasons to Choose The Southwest Scoliosis and Spine Institute:

Finally, patients with syringomyelia can benefit from the expertise of the renowned spine surgeons at The Southwest Scoliosis and Spine Institute. Led by Doctors Richard A. Hostin, Devish Ramnath, and Ishaq Syed, the institute offers state-of-the-art diagnostic and treatment options for complex spinal conditions. With offices in Dallas, Plano, and Frisco, Texas, the institute provides personalized care and comprehensive support to help patients achieve optimal outcomes and improve their quality of life.

____________________

National Organization of Rare Diseases: Syringomyelia