Syringomyelia is mainly treated with surgery that focuses on restoring the normal flow of cerebrospinal fluid (CSF) or draining fluid from the syrinx
Treatment largely depends on the severity of syringomyelia and the progression of symptoms, as well as whether the condition is the result of an underlying medical condition such as a tumor.
In people who do not have any symptoms, a neurosurgeon or neurologist may monitor disease progression to see whether symptoms worsen over time. Regular monitoring is essential for making sure treatment is timely.
What is syringomyelia?
Syringomyelia, also called hydromyelia or syringohydromyelia, is a condition where there is the formation of a fluid-filled sac or cyst within the spinal cord. This cyst is called syrinx and can grow with time, causing increased pressure on the spinal cord. If not treated, the syrinx can cause considerable nerve damage.
Syrinxes can form anywhere along the length of the spinal cord, causing symptoms that vary depending on its size and location. Syringomyelia is generally seen in individuals aged 25 to 40 years old and affects more males than females.
What causes syringomyelia?
In some people, there may be no identifiable cause of syringomyelia (idiopathic). However, syringomyelia may be caused by:
- Birth defects (namely Chiari malformation)
- Rheumatoid arthritis
- Infections or trauma to the spinal cord
What are the symptoms of syringomyelia?
Symptoms of syringomyelia depend on the size and location of the syrinx. Some people may not have any symptoms. When caused by congenital conditions, symptoms may not appear until the person is 25 to 40 years old. Following an injury, symptoms may take at least 2-3 months to appear.
Symptoms of syringomyelia include:
- Pain typically in the midline over the spine (trunk), shoulders, neck, and upper arms
- Sideways curvature of the spine (scoliosis)
- Weakness and wasting (shrinking) of muscles in the arms and legs
- Muscle spasms (tightness) in the legs, arms. or hands
- Numbness in the arms and hands
- Difficulty walking (toe walking may be seen in children)
- Total or partial loss of bowel and bladder control
- Sexual dysfunction
- Uncontrolled eye movements (nystagmus)
- Syncope (fainting)
- Drooping of one eyelid (ptosis)
- Trouble swallowing
What types of surgeries can treat syringomyelia?
In individuals who need surgery, intervention mainly focuses on restoring the normal flow of cerebrospinal fluid (CSF) or draining the fluid from the syrinx. Depending on what is causing symptoms, types of surgery include:
- Treating Chiari malformation: Chiari malformation is a developmental condition in which there is an abnormality in the manner the parts of the brain are shaped and positioned in the skull. This may lead to displacement (herniation) of parts of the brain through the opening at the base of the skull into the spinal canal. Surgery for Chiari malformation (suboccipital decompression) helps create more room for the brain, thereby reducing pressure on the brain. It helps attain normal CSF flow and may correct or improve syringomyelia.
- Relieving obstruction: Surgery may be done to remove the obstruction to the CSF flow caused by conditions, such as a tumor, abnormal bone growth, or scar tissue. For tumors, radiation therapy or chemotherapy may also be used to reduce their size.
- Draining the syrinx: Fluid collected in the syrinx may be drained through a shunt or stent surgery. These surgeries help relieve compression over the spinal cord by providing alternative pathways for drainage of the collected fluid.
- Treating a tethered spinal cord: Normally, the spinal cord floats in the spinal canal without any attachment. A tethered spinal cord occurs when the spinal cord is fixed or attached anywhere within the spinal canal. A surgery called laminectomy may be done to free the spinal cord and restore normal CSF flow.
- Preventing trauma-related syrinx formation or progression: For people who sustained an injury (trauma) to the spinal cord, preventive procedures (such as expansive duraplasty) may be needed to stop the development or progression of a syrinx.
Other supportive measures, such as avoiding straining (such as while jumping, pushing, or lifting heavy objects) and rehabilitative care may also be needed. Rehabilitation (particularly neurorehabilitation) helps preserve neurological functions and prevents complications in bedridden patients, such as infection and pressure sores (decubitus ulcers).