Spontaneous spinal cerebrospinal fluid‐venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging

Abstract

Objective: To determine the occurrence of cerebrospinal fluid (CSF)-venous fistulas, a type of spinal CSF leak that cannot be detected with routine computerized tomography myelography, among patients with orthostatic headaches but normal brain and spine magnetic resonance imaging. Background: Spontaneous spinal CSF leaks cause orthostatic headaches but their detection may require sophisticated spinal imaging techniques. Methods: A prospective cohort study of patients with orthostatic headaches and normal brain and conventional spine imaging who underwent digital subtraction myelography (DSM) to look for CSF-venous fistulas, between May 2018 and May 2020, at a quaternary referral center for spontaneous intracranial hypotension. Results: The mean age of the 60 consecutive patients (46 women and 14 men) was 46 years (range, 13-83 years), who had been suffering from orthostatic headaches between 1 and 180 months (mean, 43 months). DSM demonstrated a spinal CSF-venous fistula in 6 (10.0%; 95% confidence interval [CI]: 3.8-20.5%) of the 60 patients. The mean age of these five women and one man was 50 years (range, 41-59 years). Spinal CSF-venous fistulas were identified in 6 (19.4%; 95% CI: 7.5-37.5%) of 31 patients with spinal meningeal diverticula but in none (0%; 95% CI: 0-11.9%) of the 29 patients without spinal meningeal diverticula (p = 0.024). All CSF-venous fistulas were located in the thoracic spine. All patients underwent uneventful surgical ligation of the fistula. Complete and sustained resolution of symptoms was obtained in five patients, while in one patient, partial recurrence of symptoms was noted 3 months postoperatively. Conclusion: Concerns about a spinal CSF leak should not be dismissed in patients suffering from orthostatic headaches when conventional imaging turns out to be normal, even though the yield of identifying a CSF-venous fistula is low.

The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities

“…Similar to a recent study of patients suspected of SIH but with normal brain imaging, 42 the present study shows that the presence of spinal meningeal diverticula is an important predictor of detecting a spinal CSF‐venous fistula. This likely reflects the presence of a systemic underlying duropathy.…”

“…Identifying a CSF-venous fistula is important because it allows directed and effective treatments, such as surgical ligation of the fistula. The present study underscores the importance of being able to identify and treat the spinal CSF leak because all patients with behavioral variant frontotemporal brain sagging syndrome and a CSF-venous fistula had a good outcome compared to only one fourth of patients with frontotemporal brain sagging syndrome who did not have an identifiable CSF leak.Similar to a recent study of patients suspected of SIH but with normal brain imaging,42 the present study shows that the presence of…”

Imaging, Clinical, and Demographic Differences in Patients With Type III Spinal Cerebrospinal Fluid Leak (Cerebrospinal Venous Fistulas) Compared With Patients With Types I and II Spinal Cerebrospinal Fluid Leak

“…In this cohort, no patient with type III spinal CSF leak had a SLEC, whereas all patients with type I/II CSF leak had evidence of at least 1 SLEC on spine MRI. This is similar to previous studies demonstrating that SLECs are seen exclusively in the setting of dural tears (types I and II spinal CSF leaks) 1,8,15,18,20,21 . These findings are potentially important, as patients with suspected spinal CSF leak will frequently undergo brain and total spine MRI before myelography to find the potential site of spinal CSF leak.…”

CSF–Venous Fistulas: Anatomy and Diagnostic Imaging

“…Recently, two investigations have addressed the question of diagnostic yield of decubitus DSM in patients with orthostatic headaches but without clear evidence of SIH on brain or spine MRI, with conflicting conclusions. The first investigation found a CVF in 6 of 60 (10%) patients with a clinical suspicion for SIH but negative brain and spine MRI [25]. A second contemporaneous study did not find any CVF in patients with both normal brain MRI and normal spine MRI who underwent decubitus DSM, although the cohort of patients with negative imaging was smaller in this study compared to the first study [26].…”