The Management of Idiopathic and Refractory Syringomyelia

Abstract

Idiopathic syringomyelia (IS) and refractory syringomyelia (RS) are types of syringomyelia that often pose a management challenge and are associated with long-term clinical sequela. They are usually an epiphenomenon reflecting an underlying pathology where the treatment of the primary cause should be the aim for any surgical intervention. In the case of IS, the initial step is agreeing on the definition of the terms idiopathic and syringomyelia. After a rigorous exhaustive clinic-radiological workup, only IS patients with progressive neurology are treated, usually unblocking subarachnoid cerebrospinal fluid (CSF) pathway obstruction somewhere in the thoracic spinal canal and reserving shunting techniques to nonresponsive cases. Similar to IS, also RS is multifactorial, and its management varies based on the initial pathology, strongly supported by radiological and clinical features. We aim to address this topic focusing on the etiopathology, investigation paradigm, and surgical pathway, formulating algorithms of management with available evidence in literature. Surgical techniques are discussed in detail.

Keywords: Arachnoidolysis; Chiari 1 malformation; Duroplasty; Hydromyelia; Idiopathic syringomyelia; Refractory syringomyelia; Syringo-subarachnoid shunt; Syringomyelia.