Syringomyelia

What is it?

Syringomyelia is characterized by developing a syrinx, a fluid-filled cystic cavity, within the spinal cord’s central canal or parenchyma. This pathological cavity can expand and elongate over time, exerting pressure on the surrounding neural tissue. The precise etiopathogenesis of syrinx formation remains a subject of research, but several mechanisms have been proposed, including:

  • Cerebrospinal Fluid Dynamics Disruption: Altered cerebrospinal fluid flow, often associated with Chiari malformation, spinal cord injury, or meningitis, leading to fluid accumulation within the spinal cord.
  • Hemodynamic Changes: Alterations in spinal cord blood flow and venous hypertension.
  • Arachnoiditis: Inflammatory scarring of the arachnoid layer of the meninges, impeding cerebrospinal fluid flow.

Symptoms

Syringomyelia’s clinical manifestations are diverse and can be insidious in onset:

  • Segmental loss of pain and temperature sensation, usually preserving light touch
  • Motor Deficits: Weakness and atrophy, particularly of the intrinsic hand muscles.
  • Autonomic Dysfunction: Including Horner’s syndrome, sweating abnormalities, and bowel/bladder disturbances.

Diagnostics

The advanced diagnostic approach includes:

  • High-Resolution MRI
  • CSF Flow Studies
  • Neurophysiological Testing (electromyography and nerve conduction studies)

Treatment

Management of syringomyelia is multidisciplinary and depends on the underlying cause, symptomatology, and syrinx progression:

  • Surgical Intervention: The mainstay for symptomatic syringomyelia, mainly when associated with structural abnormalities. Options include:
    • Posterior Fossa Decompression: For Chiari-associated syringomyelia.
    • Untethering Procedures: In cases of tethered cord syndrome.
    • Syrinx Shunting: To drain the syrinx with variable success rates.
    • Tumor Resection: If a neoplasm is the causative factor.
  • Pharmacotherapy: Aimed at symptom control, such as neuropathic pain management.
  • Rehabilitation Therapy: Physical and occupational therapies are critical for managing disability and improving functional outcomes.