Cervical cerebrospinal fluid venous fistula with syringomyelia treated with suboccipital decompression: illustrative case
Journal
Journal of neurosurgery. Case lessons
Journal Volume
3
Journal Issue
15
Date Issued
2022-04-11
Author(s)
Abstract
Background: Cerebrospinal fluid (CSF) venous fistulas are a recently discovered and underdiagnosed cause of spontaneous spinal CSF leak, which may lead to spontaneous intracranial hypotension. Most cases occur in the thoracic spine, and only 2 cases were reported in the cervical spine. Treatments include the epidural blood patch, fibrin glue injection, and surgical ligation of the fistula.
Observations: The authors report the treatment of a C6-7 CSF venous fistula, for which direct ligation was not feasible, with suboccipital decompression, leading to the complete resolution of the symptoms. Based on the clinical course and outcome in our patient, the authors summarize the previous theory and propose a hypothesis for the pathophysiology of headache and other symptoms in patients with CSF venous fistulas.
Lessons: The symptoms of CSF venous fistulas may be linked not only to intracranial hypotension but also to the altered CSF dynamics induced by tonsillar herniation. Suboccipital decompression should be considered as a potential treatment option, especially in patients with Valsalva-induced headache who show a poor response to surgical ligation, patients in whom surgical ligation is not feasible, and patients with foramen magnum obstruction. Further investigation of the pathophysiology of CSF venous fistulas is warranted and should be performed in the future.
Subjects
cerebrospinal fluid venous fistula
spontaneous intracranial hypotension
spontaneous spinal cerebrospinal fluid leak
suboccipital decompression
syringomyelia
tonsillar herniation
SDGs
Publisher
American Association of Neurological Surgeons
Type
journal article