SYRINGOMYELIA: 

Questions and Answers

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. 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.
• Early diagnosis and treatment are crucial to prevent the worsening of symptoms and potential complications.

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 point 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.

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 and surrounding structures 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 and determining the appropriate course of treatment. Early diagnosis and intervention can help prevent further neurological damage and improve patient outcomes.

Nonsurgical Treatment:

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:

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

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.

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.

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, 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 and allow 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, surgical drainage may be performed to remove fluid from the cyst and alleviate pressure on the spinal cord. This may involve creating a small opening in the cyst wall (fenestration) or inserting a catheter to drain fluid externally or into the subarachnoid space.

Spinal Fusion:

In some instances, particularly when syringomyelia is associated with spinal instability or deformity, spinal fusion surgery may be recommended. 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.

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.

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, with ongoing monitoring to 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.

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National Organization of Rare Diseases: Syringomyelia