What is the treatment for syringomyelia?

Treatment of Syringomyelia

The treatment of syringomyelia primarily involves surgical intervention targeting the underlying cause, with posterior fossa decompression being the main approach for Chiari malformation-associated syringomyelia, which is the most common etiology. 1

Diagnosis and Classification

Syringomyelia is characterized by a fluid-filled cyst (syrinx) forming within the spinal cord, resulting in myelopathy. Proper diagnosis requires:

• MRI of the complete spine (without and with IV contrast) is the gold standard for diagnosis 1
• Clinical symptoms may include:
  • Sensory loss (particularly pain and temperature)
  • Motor weakness and wasting
  • Autonomic dysfunction (rarely including syncope) 1
  • Neck or back pain
  • Progressive neurological deterioration

Classification by Etiology

1. Foraminal syringomyelia: Associated with Chiari malformation (most common)
2. Non-foraminal syringomyelia: Related to arachnoiditis (infection, inflammation, trauma)
3. Tumor-associated syringomyelia: Secondary to intramedullary tumors 2

Treatment Algorithm

1. Chiari Malformation-Associated Syringomyelia

• First-line treatment: Posterior fossa decompression (PFD) with or without duraplasty 1

  • Both PFD and posterior fossa decompression with duraplasty (PFDD) may be utilized to improve symptoms (Grade C recommendation) 1
  • Cerebellar tonsil reduction may be performed during surgery to improve syrinx resolution (Grade C recommendation) 1

• Monitoring after surgery:

  • Follow for 6-12 months to evaluate syrinx reduction
  • Additional neurosurgical intervention may be considered if no radiographic improvement after this period (Grade B recommendation) 1

2. Post-traumatic or Arachnoiditis-Related Syringomyelia

• Surgical options:
  • Arachnoidolysis to restore CSF flow
  • Syringosubarachnoid or syringoperitoneal shunting 2

3. Tumor-Associated Syringomyelia

• Treatment: Tumor resection (syrinx typically resolves spontaneously after tumor removal) 2

Important Considerations

• Surgical treatment aims to halt disease progression but typically does not reverse existing neurological damage 3
• The correlation between symptom relief and syrinx resolution is not strong 1
• Asymptomatic syringomyelia (incidental finding) generally does not require surgical intervention 2
• Pain management is an essential component of treatment as chronic pain is often a persistent symptom 2

Pitfalls and Caveats

• Delaying treatment in progressive syringomyelia can lead to permanent neurological deficits
• Not all symptoms improve equally after surgery - upper motor neuron weakness, joint position sense, and central neck pain are most likely to improve 4
• Patients should be monitored long-term as syringomyelia can recur
• Idiopathic syringomyelia is rare but may require syringoarachnoid shunting if symptomatic 5

Follow-up Management

• Regular clinical neurological assessments
• Follow-up MRI imaging to monitor syrinx size
• Pain management for persistent symptoms
• Physical therapy to maintain function and prevent complications

For patients with syringomyelia associated with Chiari malformation and no improvement after initial surgery, additional intervention should be considered after 6-12 months, as this represents the strongest evidence-based recommendation (Grade B) in the current literature 1.