Syringomyelia is a neurological disorder characterized by the formation of fluid-filled cavities, called syrinxes, within the spinal cord. These syrinxes are due to abnormalities in cerebrospinal fluid (CSF) circulation.
Over time, these syrinxes can expand, leading to compression and damage of the surrounding nerve tissue. Most syringomyelia cases are associated with a structural brain abnormality known as Chiari malformation type 1, In this condition, brain tissue extends into the spinal canal through the skull opening.
Treatment may not be necessary for individuals with mild symptoms. However, in cases where symptoms are moderate to severe, surgical intervention may be recommended.
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What Is Syringomyelia?
Syringomyelia is a condition where cerebrospinal fluid (CSF) accumulates within the spinal cord, forming a syrinx. Normally, CSF circulates through the brain and spinal cord to protect and cushion them. However, in syringomyelia, CSF becomes trapped within the spinal cord tissue, expanding and exerting pressure on nerve pathways. This compression disrupts nerve signal transmission between the brain and body, leading to neurological symptoms.
Types of syringomyelia
Syringomyelia can be classified into different types based on its cause:
Congenital syringomyelia (communicating syringomyelia)
- Frequently linked to Chiari Malformation Type 1.
- Often associated with hydrocephalus (excess CSF in the brain) and enlarged brain ventricles.
- Usually caused by an abnormal expansion of the central spinal canal that communicates with the fourth ventricle of the brain.
Acquired syringomyelia (noncommunicating or primary spinal syringomyelia)
Develops due to obstruction of CSF flow from various conditions, such as:
- Arachnoiditis — Inflammation of the arachnoid membrane surrounding the spinal cord.
- Meningitis — Infection-induced inflammation of the brain and spinal cord membranes.
- Spinal cord injury.
- Spinal cord tumors.
- Tethered cord syndrome — A congenital condition that restricts spinal cord movement.
Extracanalicular syringomyelia
In this condition:
- Syrinx formation occurs outside the central spinal canal within the spinal cord tissue itself.
- Often linked to spinal trauma, stroke, or degenerative conditions like myelomalacia — spinal cord tissue softening due to poor blood flow.
What causes syringomyelia?
While the exact cause of syrinx formation is unknown, many cases are associated with Chiari malformation.
Other potential causes include:
- Arachnoiditis.
- Meningitis.
- Spinal cord trauma.
- Tethered cord syndrome.
- Tumors within the spinal cord.
Additional contributing factors may include:
- Fetal brain development abnormalities.
- Genetic predisposition.
- Maternal folic acid deficiency during pregnancy.
In some cases, syringomyelia is idiopathic, meaning no clear cause can be identified.
Risk factors and complications of syringomyelia
Risk factors
You may be at higher risk for syringomyelia if you:
- Are between the ages of 20 and 40.
- Have Chiari malformation.
- Have had a spinal cord injury or tumor.
- Have had an infection like meningitis.
- Were assigned male at birth.
Potential complications
Without treatment, syrinx expansion can lead to:
- Bowel and bladder dysfunction.
- Chronic nerve pain in the neck, back, arms, or legs.
- Coordination and balance difficulties.
- Loss of temperature and pain sensation, especially in the hands.
- Muscle weakness or paralysis.
- Scoliosis — spinal curvature, particularly in children.
How common is syringomyelia?
Syringomyelia is a rare condition, affecting approximately eight in 100,000 people. However, it accounts for about 5% of cases of paraplegia — paralysis affecting the lower body and legs.
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What Are the Symptoms of Syringomyelia?
Symptoms vary depending on the syrinx’s size and location and often develop gradually over time.
They may be unilateral (affecting one side) or bilateral (affecting both sides) and include:
- Ataxia — muscle weakness or loss of coordination.
- Bowel and bladder dysfunction.
- Chronic pain in the shoulders, neck, arms, or legs.
- Headaches.
- Neck, back, or limb stiffness.
- Paralysis in severe cases.
- Progressive arm and leg weakness.
- Scoliosis — which may be the only symptom in children.
- Sensory loss — particularly to temperature and pain.
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How Do You Diagnose Syringomyelia?
If syringomyelia is suspected, a doctor will conduct a physical and neurological examination, followed by imaging tests, such as:
- MRI (magnetic resonance imaging) — The primary tool for diagnosing syringomyelia, showing syrinx location and size.
- Dynamic MRI — Captures multiple images in succession to assess CSF flow abnormalities.
- Electromyography (EMG) — Evaluates nerve function and helps differentiate syringomyelia from peripheral neuropathies.
- CT myelography — Detects CSF leakage into the syrinx; used when MRI cannot be done due to metal body implants.
Sometimes, syringomyelia is discovered incidentally on imaging conducted for unrelated reasons.
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How Do You Treat Syringomyelia?
Treatment depends on symptom severity and progression. Regular monitoring is recommended for asymptomatic cases. If symptoms worsen, surgical intervention may be necessary.
Surgical treatment
If surgical intervention is necessary, the options include:
- Chiari malformation surgery — Enlarges the skull base and upper spinal canal to restore normal CSF flow and reduce syrinx size.
- Syrinx drainage (shunting) — Involves inserting a catheter to drain excess CSF from the syrinx into another part of the body, where it can be absorbed.
- Obstruction removal — If caused by a tumor, scar tissue, or bone fragments, surgical removal can restore CSF circulation.
- Syrinx Prevention Surgery — Also called expansive duraplasty, this procedure restores CSF flow after traumatic injuries to prevent syrinx formation.
Treatment outcomes
Surgical success depends on the underlying cause. If the source of obstruction is addressed, symptoms often stabilize or improve significantly. However, syrinx recurrence may require additional interventions
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Why Choose UPMC for Syringomyelia Care?
For expert evaluation and personalized syringomyelia care, schedule a consultation today.
We offer:
- Access to neurology and neurosurgery specialists.
- Advanced imaging and surgical techniques.
- Comprehensive diagnostic and treatment services.
- Multidisciplinary treatment plans.
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By UPMC Editorial Staff. Last reviewed on 2025-06-26.
