Syringomyelia

Introduction

Syringomyelia is a condition in which fluid-filled cavities originate in the spinal cord or the central canal. Previously, it was considered a rare condition, but it is now a relatively common neurologic diagnosis and typically a chronic condition. The cavity can be formed by dilating the central canal, and its fluid is identical or like cerebrospinal (CSF) and extracellular fluid (ECF). A careful clinical analysis of patients with syringomyelia will always reveal an associated disorder characterized by either obstruction of CSF flow or a spinal tumor. The etiology of the condition remains enigmatic, and treatment results are unsatisfactory.

• Clinical Signs

The location of functional impairment in syringomyelia depends on the site of neuronal damage. Symptoms include chronic burning, progressive muscle weakness and/or stiffness, numbness, and autonomic dysfunctions. Pain is most commonly localized to the cervical region but is intermittent and difficult to localize.

• Etiology

Congenital syringomyelia is referred to as an abnormality of primary neurulation. A disturbance causes acquired syringomyelia in normal CSF circulation. This condition is caused by various primary problems, including the caudal fossa (Chiari malformations), spinal trauma, arachnoiditis, and tumors. The primary causative mechanism is increased pulse pressure in the spinal cord compared to the nearby subarachnoid space.

Posttraumatic Syringomyelia

This disease causes neurological deterioration after spinal cord injury (SCI) and represents a potentially severe complication with devastating consequences for the affected patients. The clinical signs include sensory disturbance, gradual loss of function, and increased spasticity.

Treatment of Syringomyelia

The disease is associated with several different pathologies. The etiology of posttraumatic syringomyelia remains poorly understood, and its treatment produces unsatisfactory results. Invariably, progressive loss of function often necessitates surgical intervention. The surgical strategy varies according to the etiological cause of the syringomyelia, and this treatment is limited to surgical interventions that aim to reduce fluid pressure or improve freedom of flow in the surrounding subarachnoid space. It is achieved by variously removing bone, draining the syrinx, inserting a shunt, and/or enlarging the enveloping soft tissue sheath. The benefits of stem cell implantation remain to be demonstrated.

Fig.1 Pathways of CSF flow. (Elliott, 2013)

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Syringomyelia is a chronic and progressive disease with remissions and exacerbations. Treatment needs to primarily be directed at restoring the normal CSF dynamics to stabilize the neurologic deficits. However, further research is still needed to understand the pathophysiology of this disease and identify better ways of treating the condition. Improvements in MRI, animal models and mathematical modeling can all play a role in this investigation.

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Reference

1. Elliott, N. S. J.; et al. Syringomyelia: a review of the biomechanics. Journal of Fluids and Structures. 2013, S 40, 1-24.