Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management

A syrinx is a fluid-filled cavity within the spinal cord that can be an incidental finding or it can be accompanied by symptoms of pain and temperature insensitivity. Although it is most commonly associated with Chiari malformation Type I, the advancement of imaging techniques has resulted in more incidental idiopathic syringes that are not associated with Chiari, tumor, trauma, or postinfectious causes. The authors present a comprehensive review and management strategies for the idiopathic variant of syringomyelia.

The authors retrospectively identified 8 idiopathic cases of syringomyelia at their institution during the last 6 years. A PubMed/Medline literature review yielded an additional 38 articles.

Two of the authors' patients underwent surgical treatment that included a combination of laminectomy, lysis of adhesions, duraplasty, and syrinx fenestration. The remaining 6 patients were treated conservatively and had neurologically stable outcomes. Review of the literature suggests that an etiology-driven approach is essential in the diagnosis and management of syringomyelia, although conservative management suffices for most cases. In particular, it is important to look at disturbances in CSF flow, as well as structural abnormalities including arachnoid webs, cysts, scars, and a diminutive posterior fossa.

The precise etiology for idiopathic syringomyelia (IS) is still unclear, although conceptual advances have been made toward the overall understanding of the pathophysiology of IS. Various theories include the cerebellar piston theory, intramedullary pulse pressure theory, and increased spinal subarachnoid pressure. For most patients with IS, conservative management works well. Continued progression of symptoms, however, could be approached using decompressive strategies such as laminectomy, lysis of adhesions, and craniocervical decompression, depending on the level of pathology. Management for patients with progressive neurological dysfunction and the lack of flow disturbance is unclear, although syringosubarachnoid shunting can be considered.