Cerebral venous sinus thrombosis and dural arteriovenous fistula associated with protein S deficiency: a case series study

Abstract

Objective: To describe the characteristics of patients with cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (AVF) associated with protein S (PS) deficiency.

Methods: We conducted a search of medical records in Hainan General Hospital from January 2000 to December 2020 for coexistence of CVST and dural AVF associated with PS deficiency and searched PubMed、Embase and Chinese biomedical databases (CBM) for all literature describing CVST and dural AVF with PS. We analyzed clinical characteristics, location, sequence of CVST and dural AVF, level of PS, therapeutic methods and prognosis.

Results: We presented 1 patient in our hospital's database combined CVST and dural AVF associated with PS, plus 5 cases reported in literature. The most common symptoms were headache, generalized seizure, disturbance of consciousness. The most frequent location of CVST was at internal cerebral vein, while transverse sinus, sigmoid sinus, parietal region in dural AVF. Two patients developed dural AVF several months or years after CVST. Clinical characteristics and level of PS were summarized.

Conclusion: These findings alert physicians to consider PS deficiency in patients who suffer from CVST, especially those combined with dural AVF.

Keywords: Cerebral venous sinus thrombosis; Clinical characteristics; Dural arteriovenous fistula; Protein S deficiency.

Fig. 1

Flow chart of article retrieval

Fig. 2

Cerebral MRI showed abnormal signal in left cerebellar hemisphere, vermis cerebelli, pontibrachium(A, B: T1W1 showed hypointensity, C: T2WI showed hyperintensity, D: Flair showed hyperintensity); MRV showed left sigmoid sinus was indistinct(E)

Fig. 3

DSA revealed a dural AVF fed by the left occipital artery(A, B), occlusion of left sigmoid, torcular sinus and internal cerebral vein(C)