Prevalence, clinical presentation, and treatment-management of cerebral venous thrombosis associated with spontaneous intracranial hypotension: A combined case-series and systematic literature review approach

Cerebral venous thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). Therefore, its correct diagnosis and the corresponding optimal treatment-management identification remains challenging. Over the last 10 years, 300 patients received a definite SIH diagnosis at our stroke center. Through thorough review of the database, we identified all patients with SIH-related CVT. In addition, we performed a systematic literature review including all publications on SIH-related CVT. Five out of our 300 SIH patients showed CVT (F/M:2/3, mean age: 51.8 ± 15.7). Through the literature search, 72 additional cases were identified. Overall, the prevalence was 1.3% and main clinical presentations were orthostatic headache, nausea, and vomiting. The CVT was predominantly located at the superior sagittal sinus. Treatment strategies included anticoagulants (ACs) (43%), epidural blood patch (EBP) (19.4%), and combined AC + EBP (33.3%). In our cohort, all but one patient received combined EBP and AC. The mean clinical and radiological follow-up were 2 years and 1.5 years, respectively. Complete clinical recovery was reported in 96% of the cases, whereas 56% showed complete radiological CVT resolution. Among patients without radiological resolution (26.4%), 57% received AC-only, while 43% received combined AC + EBP. Of our five cases, all but one patient received combined AC + EBP. The overall prevalence of SIH-related CVT was 1.3%. AC and combined AC + EBP were the most used treatment-management strategies. CVT resolution was more commonly achieved after the combined strategy. Overall, the rate of complete clinical recovery was 96%.