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

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p></jats:sec>

Syrinx location and size according to etiology: identification of Chiari-associated syrinx

“…16,24,26,28,30 Some syringes are not associated with any of these conditions and may be considered idiopathic. 3,14,18,19,23 It is well established that large cervical cord syringes in patients with typical-appearing CM-I are causally linked in many cases. Decompression of the CM-I leads to a reduction in syrinx size in a significant majority of cases.…”

Case Report of Two Level Thoracic Disc Herniation with Syringomyelia

“…Bu nedenle hastalığı atlamamak için biz klinisyenler, konjenital siringomiyeli gelişimi ve klinik bulguları konusunda yeterli bilgiye sahip olmalıyız. 1,2,5 Klinikte özellikle ciddi spinal kord hasarı sonrası posttravmatik siringomiyeli vakalarına daha sık rastlanmaktadır. Bu hastalarda siringosubaraknoid şant yanında duraplasti, araknolizis tercih edilen cerrahi tedavi seçeneklerindendir.…”

“…İlerleyici semptomların varlığı hâlinde siringosubaraknoid şant, laminektomi, adezyolizis, kraniyoservikal dekompresyon gibi dekompresyon tekniklerine başvurulmaktadır. [1][2][3] Torakal disk hernileri sıklıkla asemptomatik olmakla birlikte, uzamış açıklanamayan duysal semptomlar ile de başlayabilir ve zamanla motor değişiklikler de eklenerek Brown-sequard sendromu gelişimine neden olabilirler. 11 Olgumuzda olduğu gibi torakal disk hernisi atipik göğüs ve sırt ağrısı bulguları ile de ortaya çıkabilir.…”

Incidental Findings on Brain and Spine Imaging in Children

“…150,151,156 Although most cases of clinically relevant syringomyelia are associated with CM, it may also be seen in patients with spinal cord tumor, tethered cord, or arachnoiditis. 157,158 In general, the management of syringomyelia should be directed at the primary disorder. When no primary disorder is identified, the syrinx is considered idiopathic.…”