Jugular venous narrowing and spontaneous spinal cerebrospinal fluid leaks: A case-control study exploring association and proposed mechanism.

Background: Spontaneous skull base cerebrospinal fluid leaks (CSFLs) are associated with increased intracranial pressure in idiopathic intracranial hypertension (IIH) and hypothesized to relate to skull base erosions due to increased CSF pressure. Given the increasing recognition of internal jugular venous stenosis (IJVS) as a cause of intracranial hypertension (IH), we evaluated the relationship between spinal CSFL and venous causes of IH.

Methods: The spinal CSFL database at a single institution was assessed to identify 12 consecutive spontaneous, non-traumatic spinal CSFL patients with CTV data. Exclusion criteria included documented IIH and iatrogenic CSFL. Demographics, clinical parameters, imaging characteristics, and IJV manometry results were recorded. Internal jugular venous stenosis was graded as: none (0-10%), mild (10-50%), moderate (50-80%), severe (>80-99%), and occluded (100%). Twelve consecutive patients who presented with cerebrovascular accidents without CSFL, matched by age and sex, were similarly analyzed as a control group. STROBE guidelines were used in reporting results.

Results: All CSFL patients had IJVS (83.3% bilateral, 33.3% severe) compared to 41.7% of the control group (33.3% bilateral, 16.7% severe-occluded); p = 0.04. All CSFL patients with available venogram manometry data had at least unilateral IJV gradients. Most patients presented with modified Rankin score (mRS) of 1 (66.7%), but in those with higher mRS, medical and/or surgical interventions were associated with decreased morbidity.

Conclusion: Spontaneous spinal CSFL was associated with IJVS in patients not meeting IIH criteria. Persistently high CSF pressure resulting in CSFL may cause opening pressure to be falsely normal or low. Internal jugular venous stenosis may be a viable target in recurrent CSFL management and improve morbidity.