What is the management and treatment of Syringomyelia (a disorder in which a fluid-filled cavity forms within the spinal cord)?

Management and Treatment of Syringomyelia

The primary treatment for symptomatic syringomyelia is surgical intervention, with posterior fossa decompression with or without duraplasty being the first-line treatment option for patients with Chiari malformation-associated syringomyelia. 1

Etiology and Classification

Syringomyelia is characterized by the development of a fluid-filled cavity (syrinx) within the spinal cord. The condition can be classified into several types based on etiology:

• Chiari malformation-associated syringomyelia: Most common cause, detected in 25-50% of patients with Chiari malformation 1
• Post-traumatic syringomyelia: Develops following spinal cord injury 2
• Non-communicating syringomyelia: Associated with extramedullary compressive lesions, intramedullary tumors, or infections 3
• Communicating syringomyelia: Anatomically continuous with the 4th ventricle 3

Clinical Presentation

Symptoms of syringomyelia typically develop during the third or fourth decades of life and may include:

• Pain (often chronic and difficult to manage) 2
• Sensory loss (particularly pain and temperature sensation) 2
• Progressive weakness and muscle wasting 2
• Neurological deterioration over many years 2
• In cases associated with Chiari malformation: occipital/neck pain exacerbated by Valsalva maneuvers, peripheral motor/sensory defects, and lower cranial nerve dysfunction 1

Diagnostic Approach

• MRI of brain and spine: Gold standard for diagnosis, should include:

  • T1 and T2-weighted sequences 1
  • FLAIR imaging 1
  • T2*-weighted gradient echo or susceptibility-weighted imaging 1
  • Pre and post-contrast T1-weighted acquisitions 1
  • High-resolution heavily T2-weighted 3D sequences for the spine 1

• Dynamic MRI: Useful for evaluating CSF flow dynamics 2

Surgical Management

For Chiari Malformation-Associated Syringomyelia

1. First-line treatment: Posterior fossa decompression (PFD) with or without duraplasty (PFDD) 1, 4

   • Both PFD and PFDD show benefit for symptom relief and syrinx reduction 1
   • Recent evidence suggests improved outcomes with dural patch grafting 4

2. Cerebellar tonsil reduction: May be performed during PFD surgery to improve syrinx and/or symptoms (Grade C recommendation) 1

3. Follow-up and additional intervention:

   • Monitor for syrinx reduction for 6-12 months after initial surgery 1
   • Consider additional neurosurgical intervention if no radiographic improvement after 6-12 months (Grade B recommendation) 1, 4

For Other Types of Syringomyelia

• Tumor-associated syringomyelia: Resection of the tumor typically leads to resolution of the syrinx 2
• Post-traumatic syringomyelia: Spinal or syringocisternal shunting may be effective 3
• Syringomyelia with extramedullary obstruction: Excision of the obstruction or syringocisternal shunting 3
• Communicating syringomyelia with hydrocephalus: Ventriculoperitoneal shunting 3

Combined Surgical Approaches

Some evidence supports a combined approach for syringomyelia-Chiari complex:

• Foramen magnum decompression combined with syringosubarachnoid shunting performed in the same operation 5
• This approach has shown effectiveness in decompressing the syrinx cavity with maintained collapse on follow-up MRI 5

Treatment Decision Algorithm

1. Determine if intervention is needed:

   • Symptomatic and progressive syringomyelia requires treatment 2, 6
   • Incidental asymptomatic syringomyelia or hydromyelia generally does not require surgical intervention 2

2. Identify underlying cause:

   • Chiari malformation → Posterior fossa decompression with/without duraplasty 1, 4
   • Hydrocephalus → Ventriculoperitoneal shunt 3
   • Intramedullary tumor → Tumor resection 3, 2
   • Extramedullary compression → Remove compressive lesion 3
   • Post-traumatic → Syrinx shunting procedures 3

3. Evaluate response:

   • Monitor with MRI and clinical assessment 1
   • If no improvement after 6-12 months, consider additional intervention 1, 4

Pitfalls and Considerations

• Symptom resolution and syrinx resolution do not always correlate directly 1
• Early surgical intervention is recommended before the establishment of gross neurological deficits 6
• Conservative treatment is generally not recommended as surgical procedures can stop disease progression 6
• Surgical treatment aims to halt destruction of the nervous system but will not reverse damage that has already occurred 7
• Chronic pain often persists even after successful surgical management and requires dedicated medical treatment 2