Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation

Abstract

Introduction: Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. We conducted a literature review to summarise the most accepted and widespread ideas about the pathophysiology, management and other aspects of syringomyelia unrelated to Chiari malformation.

Development: We reviewed the most relevant literature on this condition, focusing on the pathophysiology, clinical presentation, diagnosis, and treatment.

Conclusions: Syringomyelia unrelated to Chiari malformation is a distinct entity that must be well understood to guarantee correct diagnosis, monitoring, and management. When the disease is suspected, a thorough study should be conducted to identify its aetiology. Treatment must aim to eliminate the cause of the disease; symptomatic treatment should remain a second-line option.

Keywords: Arachnolysis; Aracnólisis; CINE-MRI; Chiari; Chiari malformation; Cine-RM; Derivación cisto-subaracnoidea; Derivación cistopleural; Siringomielia; Syringomyelia; Syringopleural shunting; Syringosubarachnoid shunting.