Background: Cerebral venous sinus thrombosis (CVST) is a rare manifestation of thrombosis commonly caused by thrombophilia, hormonal-related factors, non-cerebral malignancy, and hematologic diseases. The aim of this review was to identify and summarize rare CVST cases. Methods: A literature search of the Medline database was performed in November 2022. CVST cases of a common cause were excluded. Demographic and clinical data were extracted. Eligible cases were categorized into inflammatory, primary CNS tumors, post-operative/traumatic, and idiopathic groups to allow statistical group comparisons. Results: 76 cases were analyzed. Idiopathic CVST was most frequently reported followed by inflammatory, post-traumatic/operative and primary CNS tumor causes. The intracranial hemorrhage rate was 23.7% and it was found to increase in the inflammatory group (45.8%). Anticoagulation was used in the majority of cases and it was significantly related to better outcomes. A low rate of anticoagulation use (43.8%) was found among CVST cases in the post-operative/traumatic group. The overall mortality rate was 9.8%. 82.4% of patients showed significant early improvement. Conclusions: Most rare CVST cases were either of idiopathic or inflammatory origin. Interestingly, hemorrhage occurred often he idiopathic CVST cases. A low rate of anticoagulation use in neurosurgical CVST cases after trauma or head surgery was observed.
Keywords: cerebral venus sinus thrombosis; idiopathic; inflammatory; rare cause; systematic review.