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Syringomyelia is a rare condition characterized by the formation of a fluid-filled cyst, known as a syrinx, within the spinal cord, which disrupts the normal flow of cerebrospinal fluid (CSF). This disruption can lead to damage to the spinal cord and compression of nerve fibers, resulting in symptoms such as pain, weakness, and stiffness. When the brain stem is affected, it is referred to as syringobulbia.
Typically, syringomyelia affects the upper regions of the spinal cord, including the cervical and thoracic areas. One common cause of syringomyelia is Chiari malformation, where brain tissue protrudes into the spinal canal. Other factors contributing to syringomyelia include spinal cord tumors, injuries, and inflammation around the spinal cord.
Treatment for syringomyelia, especially when symptoms are severe, often involves surgery. However, in cases where syringomyelia is not causing significant issues, regular monitoring may be all that is necessary.
Symptoms
Syringomyelia is a condition characterized by the development of fluid-filled cysts within the spinal cord, with symptoms that generally emerge gradually and may worsen over time. Although some individuals with a small syrinx may not immediately experience symptoms, the severity and nature of these symptoms can vary based on the syrinx’s location, size, and how far it extends along the spine, affecting either one or both sides of the body. Symptoms can range from mild to severe. In cases where syringomyelia is caused by brain tissue protruding into the spinal canal, it is known as a Chiari malformation, with symptoms typically starting between the ages of 25 and 40. It’s also noted that actions like coughing or straining might trigger symptoms but are not causes of the condition.
Common symptoms:
- Muscle weakness and wasting
- Loss of reflexes
- Reduced sensitivity to pain and temperature
- Headaches
- Stiffness in the back, shoulders, arms, and legs
- Pain in the neck, arms, and back
- Scoliosis, which involves a sideways curvature of the spine
Symptoms Specific to Children:
- Scoliosis
- Toe walking
If you experience any of the above symptoms, consult a healthcare professional. For those with a history of spinal cord injury, it is crucial to monitor for symptoms of syringomyelia, as they may develop months to years after the injury. Ensure your healthcare provider is aware of any past spinal injuries.
Causes
Syringomyelia occurs when the fluid that typically envelops and safeguards the brain and spinal cord accumulates inside the spinal cord itself. While the precise reason for this buildup remains elusive, theories propose it may stem from an obstruction or disturbance in the flow of cerebrospinal fluid (CSF).
The causes of syringomyelia can be broadly categorized into two groups: congenital, meaning present at birth, and acquired, which develop later in life. Occasionally, syringomyelia can manifest without a discernible cause, termed idiopathic syringomyelia.
Generally, common causes include:
- Chiari malformation: The majority of cases are linked with Chiari malformation type 1. While this is typically present at birth, symptoms may not appear until adolescence or adulthood, or some individuals may never experience symptoms at all. This disorder causes brain tissue to protrude into the spinal canal and obstructs the normal passage of CSF fluid.
- Open spina bifida: Also known as myelomeningocele, is a birth defect where the backbone and spinal canal fail to close before birth, classified as a type of neural tube defect (NTD).
- Tethered cord syndrome: Commonly linked with conditions like spina bifida and scoliosis, this rare neurological condition involves the spinal cord being attached to the surrounding spine tissues.
- Meningitis: This is an infection of the protective lining around the brain and spinal cord which can cause syringomyelia.
- Spinal cord tumor: This include hemangioblastomas and ependymomas, which can disrupt CSF circulation, leading to syringomyelia.
- Spinal cord injury: This is termed post-traumatic syringomyelia and typically arises months or years after the initial injury due to scar tissue blocking cerebrospinal fluid (CSF) flow.
Risk factor
Most individuals diagnosed with syringomyelia are between the ages of 20 and 50, although the condition can also manifest in young children and older adults.
Diagnosis
The following will assist the healthcare provider in diagnosing syringomyelia:
- Physical examination: Diagnosing syringomyelia involves a review of one’s medical history, a neurological-focused physical examination, and imaging tests. In some cases, syringomyelia may be detected during a test performed for other reasons.
- Magnetic resonance imaging (MRI): MRI scans can identify if there is a syrinx or any other abnormalities like tumors in your spine. It clearly shows the location, size, and severity of the syrinx.
An MRI scan of the spine and spinal cord is the most reliable method for diagnosing syringomyelia as it provides detailed images using radio waves and a magnetic field. - Myelogram with computed tomography (CT) scan: A myelogram is a test that uses a contrast dye and computed tomography to examine your spinal canal for any issues. This may be recommended for people who cannot have an MRI.
Treatment
Treatment for syringomyelia depends on symptom severity and progression. Generally, if it is causing symptoms, treatment typically involves surgery aimed at addressing the underlying cause and preventing further spinal cord damage.
Treatment options include:
- Periodic monitoring: This involves going through periodic MRI scans and neurological exams to check the syringomyelia. This may be recommended if syringomyelia is asymptomatic.
- Surgery: There are two main types of surgery: one focuses on restoring normal cerebrospinal fluid (CSF) flow around the spinal cord, while the other involves directly draining the syrinx. The choice of surgical approach depends on the underlying cause of the symptoms.
The aim of surgery is to alleviate pressure on the spinal cord caused by the syrinx and restore normal cerebrospinal fluid flow. This can lead to symptom improvement and better nervous system function.
Surgical treatments include:- Treating Chiari malformation: This surgery can help drain a syrinx, which may shrink or vanish completely. Even if the syrinx does not change much, surgery can still improve symptoms. The procedure involves removing a small section of bone at the back of the skull.
- Removing the blockage: Surgery to remove blockages like scar tissue, bone, or tumors from your spinal canal can fix the normal flow of fluid in your brain and spine. In some cases, if a tumor is causing the syrinx, radiation therapy may be suggested to make the tumor smaller.
- Draining the syrinx: If the cause of the syrinx is unknown and it is getting bigger, draining it might be recommended. In this procedure, a drain called a stent or shunt is placed into the syrinx. The tubing has two ends: one is inserted into the syrinx and the other into a different part of the body, such the abdomen. Both of these methods can help prevent symptoms from getting worse.
- Fixing the irregularity: Surgery to help the fluid flow properly and drain a syrinx may be recommended, if the problem is a tethered spinal cord, or the spine blocking the flow of cerebrospinal fluid.
- Follow-up care: Following surgery, healthcare providers will utilize MRI scans to assess the syrinx’s condition, observing whether it is improving or maintaining its size. Additional MRIs may be necessary to evaluate the effectiveness of the surgery. Despite treatment, certain symptoms may persist due to the lasting damage inflicted by the syrinx on the spinal cord and nerves. Furthermore, the syrinx may enlarge over time, necessitating further intervention. Regular check-ups are essential to monitor for any recurrence of syringomyelia.
