Syringomyelia associated with arachnoid septum at the craniovertebral junction, contradicting the currently prevailing theory of syringomyelia formation

Despite a number of various hypotheses in the literature, the pathophysiology of syringomyelia is still not well understood. In this article, we report two cases of cervical syringomyelia not associated with Chiari I malformation. Both cases had a septum-like structure in the subarachnoid space on the dorsal side of the cord at the craniovertebral junction. Cardiac-gated phase-contrast cine-mode magnetic resonance imaging (MRI) demonstrated decreased cerebrospinal fluid (CSF) flow on the dorsal side of the spinal cord. Surgical excision of this septum, restoring the CSF flow, resulted in a prompt reduction of the syrinx size in both cases. Findings in these cases contradict the currently prevailing hypothesis of syrinx formation that postulate that the piston-like movement of the cerebellar tonsils enhance the pulsatile CSF flow in the spinal subarachnoid space, driving the CSF into the syrinx through the perivascular space of Virchow and Robin. The authors propose that a mechanism based on the decreased pulsatile CSF flow in the spinal subarachnoid space will be more suitable as a hypothesis in studying the pathophyisiology of syringomyelia. These cases also provide an important lesson in managing the patients with syringomyelia not associated with Chiari I malformation.