What is the initial management for a patient with syringomyelia at the T6 (thoracic 6) level?

Initial Management of Syringomyelia at T6 Level

The initial management for a patient with syringomyelia at the T6 level should include MRI of the complete spine to evaluate for associated conditions such as Chiari malformation, which is the most common cause of syringomyelia. 1

Diagnostic Evaluation

• Complete spine MRI without IV contrast is essential as the first imaging study to:

  • Determine the extent of the syrinx
  • Identify underlying causes (especially Chiari malformation)
  • Evaluate for other associated conditions like hydrocephalus 2, 1

• Brain MRI should be performed concurrently to:

  • Evaluate for Chiari malformation (the most common cause)
  • Assess for other potential causes of syringomyelia 1

Management Algorithm Based on Etiology

1. If Chiari Malformation is Present:

• Posterior fossa decompression (PFD) or posterior fossa decompression with duraplasty (PFDD) is the first-line surgical treatment 2, 1
• Cerebellar tonsil reduction may be performed during surgery to improve syrinx and symptoms (Grade C recommendation) 2
• After surgery, wait 6-12 months before considering additional intervention if the syrinx persists (Grade B recommendation) 2, 1

2. If Spinal Arachnoiditis is Present:

• Surgical decompression of the spinal subarachnoid space may be indicated 3
• Shunting procedures (syringo-peritoneal or syringo-subarachnoid) may be necessary 3

3. If Idiopathic Syringomyelia:

• Surgical decompression via laminectomy and syringoarachnoid shunt may be effective 4
• Early surgical intervention is recommended before gross neurological deficits develop 5

Clinical Considerations

• Symptoms requiring intervention include:

  • Pain (often neuropathic)
  • Paresthesia
  • Progressive weakness
  • Bladder/bowel dysfunction 6, 4

• Monitor for signs of syringomyelia-induced disruption of sympathetic fibers in the thoracic spinal cord, which can rarely cause syncope 2

Important Caveats

• Timing is critical: Early intervention before establishment of gross neurological deficits leads to better outcomes 5
• Conservative treatment is generally not recommended as the disease typically progresses without intervention 5
• Post-surgical follow-up: MRI should be performed to assess syrinx size reduction, but allow 6-12 months before considering additional procedures 2, 1
• Shunting procedures should be considered when reestablishment of normal CSF pathways proves impossible or as a second procedure 5

Emerging Treatments

While not yet standard of care, recent research suggests that uncultured umbilical cord-derived mesenchymal stem cells may offer a potential alternative treatment for syringomyelia, particularly for pain relief and cavity reduction 6.