Skip to main content
Top
Published in: BMC Neurology 1/2022

Open Access 01-12-2022 | Cerebral Sinus Venous Thrombosis | Research

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

Authors: Hui Liang, Congjie Xu, Jiyi Xu

Published in: BMC Neurology | Issue 1/2022

Login to get access

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.
Literature
1.
go back to reference Behrouzi R, Punter M. Diagnosis and management of cerebral venous thrombosis. Clin Med (Lond). 2018;18(1):75–9.CrossRef Behrouzi R, Punter M. Diagnosis and management of cerebral venous thrombosis. Clin Med (Lond). 2018;18(1):75–9.CrossRef
2.
go back to reference Ropper AH, Klein JP. Cerebral venous thrombosis. N Engl J Med. 2021;385(1):59–64.CrossRef Ropper AH, Klein JP. Cerebral venous thrombosis. N Engl J Med. 2021;385(1):59–64.CrossRef
3.
go back to reference Yang S, You R, Wu W, et al. Dural arteriovenous fistula complicated with cerebral venous sinus thrombosis. World Neurosurg. 2020;134:348–52.CrossRef Yang S, You R, Wu W, et al. Dural arteriovenous fistula complicated with cerebral venous sinus thrombosis. World Neurosurg. 2020;134:348–52.CrossRef
4.
go back to reference Castoldi E, Hackeng TM. Regulation of coagulation by protein S. Curr Opin Hematol. 2008;15(5):529–36.CrossRef Castoldi E, Hackeng TM. Regulation of coagulation by protein S. Curr Opin Hematol. 2008;15(5):529–36.CrossRef
5.
go back to reference Engesser L, Broekmans AW, Briet E, et al. Hereditary protein S deficiency: clinical manifestations. Ann Intern Med. 1987;106(5):677–82.CrossRef Engesser L, Broekmans AW, Briet E, et al. Hereditary protein S deficiency: clinical manifestations. Ann Intern Med. 1987;106(5):677–82.CrossRef
6.
go back to reference Gupta A, Tun A M, Gupta K, et al. Protein S deficiency. 2022. Gupta A, Tun A M, Gupta K, et al. Protein S deficiency. 2022.
7.
go back to reference Wagh SB, Anadure R, Dutta V, et al. Isolated protein S deficiency presenting as catastrophic systemic arterial and subsequently venous thrombosis. Australas Med J. 2012;5(8):424–8.CrossRef Wagh SB, Anadure R, Dutta V, et al. Isolated protein S deficiency presenting as catastrophic systemic arterial and subsequently venous thrombosis. Australas Med J. 2012;5(8):424–8.CrossRef
8.
go back to reference Rezende SM, Simmonds RE, Lane DA. Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex. Blood. 2004;103(4):1192–201.CrossRef Rezende SM, Simmonds RE, Lane DA. Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex. Blood. 2004;103(4):1192–201.CrossRef
9.
go back to reference Izumi T, Miyachi S, Hattori K, et al. Thrombophilic abnormalities among patients with cranial dural arteriovenous fistulas. Neurosurgery. 2007;61(2):262–8.CrossRef Izumi T, Miyachi S, Hattori K, et al. Thrombophilic abnormalities among patients with cranial dural arteriovenous fistulas. Neurosurgery. 2007;61(2):262–8.CrossRef
10.
go back to reference Kim CK, Shin JY, Chang JY, et al. Spontaneous carotid cavernous fistula in a case with protein S deficiency that newly developed Ophthalmoplegia after embolization. J Clin Neurol. 2011;7(3):164–7.CrossRef Kim CK, Shin JY, Chang JY, et al. Spontaneous carotid cavernous fistula in a case with protein S deficiency that newly developed Ophthalmoplegia after embolization. J Clin Neurol. 2011;7(3):164–7.CrossRef
11.
go back to reference Sugiura Y, Miyamoto T, Takehara S, et al. Multiple dural arteriovenous fistulas following extensive sinus thrombosis: a case report. No Shinkei Geka. 1996;24(4):379–83.PubMed Sugiura Y, Miyamoto T, Takehara S, et al. Multiple dural arteriovenous fistulas following extensive sinus thrombosis: a case report. No Shinkei Geka. 1996;24(4):379–83.PubMed
12.
go back to reference Matsubara S, Satoh K, Satomi J, et al. Acquired pial and dural arteriovenous fistulae following superior sagittal sinus thrombosis in patients with protein S deficiency: a report of two cases. Neurol Med Chir (Tokyo). 2014;54(3):245–52.CrossRef Matsubara S, Satoh K, Satomi J, et al. Acquired pial and dural arteriovenous fistulae following superior sagittal sinus thrombosis in patients with protein S deficiency: a report of two cases. Neurol Med Chir (Tokyo). 2014;54(3):245–52.CrossRef
13.
go back to reference Yassari R, Jahromi B, Macdonald R. Dural arteriovenous fistula after craniotomy for pilocytic astrocytoma in a patient with protein S deficiency. Surg Neurol. 2002;58(1):59–64.CrossRef Yassari R, Jahromi B, Macdonald R. Dural arteriovenous fistula after craniotomy for pilocytic astrocytoma in a patient with protein S deficiency. Surg Neurol. 2002;58(1):59–64.CrossRef
14.
go back to reference Pasi M, Nappini S, Salvadori E, et al. Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency. J Clin Neurosci. 2014;21(9):1654–6.CrossRef Pasi M, Nappini S, Salvadori E, et al. Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency. J Clin Neurosci. 2014;21(9):1654–6.CrossRef
15.
go back to reference Witt O, Pereira PL, Tillmann W. Severe cerebral venous sinus thrombosis and dural arteriovenous fistula in an infant with protein S deficiency. Childs Nerv Syst. 1999;15(2–3):128–30.CrossRef Witt O, Pereira PL, Tillmann W. Severe cerebral venous sinus thrombosis and dural arteriovenous fistula in an infant with protein S deficiency. Childs Nerv Syst. 1999;15(2–3):128–30.CrossRef
16.
go back to reference Borden JA, Wu JK, Shucart WA. Correction: dural arteriovenous fistulous malformations. J Neurosurg. 1995;82(4):705–6.PubMed Borden JA, Wu JK, Shucart WA. Correction: dural arteriovenous fistulous malformations. J Neurosurg. 1995;82(4):705–6.PubMed
17.
go back to reference Abecassis IJ, Meyer RM, Levitt MR, et al. Assessing the rate, natural history, and treatment trends of intracranial aneurysms in patients with intracranial dural arteriovenous fistulas: a consortium for Dural arteriovenous fistula outcomes research (CONDOR) investigation. J Neurosurg. 2021;136(4):971-80. Abecassis IJ, Meyer RM, Levitt MR, et al. Assessing the rate, natural history, and treatment trends of intracranial aneurysms in patients with intracranial dural arteriovenous fistulas: a consortium for Dural arteriovenous fistula outcomes research (CONDOR) investigation. J Neurosurg. 2021;136(4):971-80.
18.
go back to reference Goddard AJ, Khangure MS. Multiple dural arteriovenous fistulas. Radiologic progression and endovascular cure. Case report. Interv Neuroradiol. 2002;8(2):183–91.CrossRef Goddard AJ, Khangure MS. Multiple dural arteriovenous fistulas. Radiologic progression and endovascular cure. Case report. Interv Neuroradiol. 2002;8(2):183–91.CrossRef
19.
go back to reference Gerlach R, Boehm-Weigert M, Berkefeld J, et al. Thrombophilic risk factors in patients with cranial and spinal dural arteriovenous fistulae. Neurosurgery. 2008;63(4):693–8.CrossRef Gerlach R, Boehm-Weigert M, Berkefeld J, et al. Thrombophilic risk factors in patients with cranial and spinal dural arteriovenous fistulae. Neurosurgery. 2008;63(4):693–8.CrossRef
20.
go back to reference Song Y, Han S, Kim BJ, et al. Feasibility of low-dose digital subtraction angiography protocols for the endovascular treatment of intracranial dural arteriovenous fistulas. Neuroradiology. 2021;63(2):267–73.CrossRef Song Y, Han S, Kim BJ, et al. Feasibility of low-dose digital subtraction angiography protocols for the endovascular treatment of intracranial dural arteriovenous fistulas. Neuroradiology. 2021;63(2):267–73.CrossRef
21.
go back to reference Schuchardt FF, Demerath T, Elsheikh S, et al. Dural arteriovenous fistula formation secondary to cerebral venous thrombosis: longitudinal magnetic resonance imaging assessment using 4D-combo-MR-venography. Thromb Haemost. 2021;121(10):1345–52.CrossRef Schuchardt FF, Demerath T, Elsheikh S, et al. Dural arteriovenous fistula formation secondary to cerebral venous thrombosis: longitudinal magnetic resonance imaging assessment using 4D-combo-MR-venography. Thromb Haemost. 2021;121(10):1345–52.CrossRef
22.
go back to reference Ishii M, Yamanouchi T, Oshima R, et al. A case of dural arteriovenous fistula that developed 21 months after cerebral venous sinus thrombosis. No Shinkei Geka. 2017;45(10):889–95.PubMed Ishii M, Yamanouchi T, Oshima R, et al. A case of dural arteriovenous fistula that developed 21 months after cerebral venous sinus thrombosis. No Shinkei Geka. 2017;45(10):889–95.PubMed
23.
go back to reference Huang Q, Chai X, Xiao C, et al. A case report of oral contraceptive misuse induced cerebral venous sinus thrombosis and dural arteriovenous fistula. Medicine (Baltimore). 2019;98(33):e16440.CrossRef Huang Q, Chai X, Xiao C, et al. A case report of oral contraceptive misuse induced cerebral venous sinus thrombosis and dural arteriovenous fistula. Medicine (Baltimore). 2019;98(33):e16440.CrossRef
24.
go back to reference Pierot L, Chiras J, Duyckaerts C, et al. Intracranial dural arteriovenous fistulas and sinus thrombosis. Report of five cases. J Neuroradiol. 1993;20(1):9–18.PubMed Pierot L, Chiras J, Duyckaerts C, et al. Intracranial dural arteriovenous fistulas and sinus thrombosis. Report of five cases. J Neuroradiol. 1993;20(1):9–18.PubMed
25.
go back to reference Sakaki T, Morimoto T, Nakase H, et al. Dural arteriovenous fistula of the posterior fossa developing after surgical occlusion of the sigmoid sinus. Report of five cases. J Neurosurg. 1996;84(1):113–8.CrossRef Sakaki T, Morimoto T, Nakase H, et al. Dural arteriovenous fistula of the posterior fossa developing after surgical occlusion of the sigmoid sinus. Report of five cases. J Neurosurg. 1996;84(1):113–8.CrossRef
26.
go back to reference Lawton MT, Jacobowitz R, Spetzler RF. Redefined role of angiogenesis in the pathogenesis of dural arteriovenous malformations. J Neurosurg. 1997;87(2):267–74.CrossRef Lawton MT, Jacobowitz R, Spetzler RF. Redefined role of angiogenesis in the pathogenesis of dural arteriovenous malformations. J Neurosurg. 1997;87(2):267–74.CrossRef
27.
go back to reference Hanaoka M, Matsubara S, Satoh K, et al. Dural arteriovenous fistulae after cerebral infarction: report of two cases. Neurosurgery. 2011;68(2):E575–9.CrossRef Hanaoka M, Matsubara S, Satoh K, et al. Dural arteriovenous fistulae after cerebral infarction: report of two cases. Neurosurgery. 2011;68(2):E575–9.CrossRef
28.
go back to reference Shinozaki N, Ebina Y, Deguchi M, et al. Protein S deficiency complicated pregnancy in women with recurrent pregnancy loss. Gynecol Endocrinol. 2016;32(8):672–4.CrossRef Shinozaki N, Ebina Y, Deguchi M, et al. Protein S deficiency complicated pregnancy in women with recurrent pregnancy loss. Gynecol Endocrinol. 2016;32(8):672–4.CrossRef
30.
go back to reference Fearon A, Pearcy P, Venkataraman S, et al. Protein S deficiency and arterial thromboembolism: a case report and review of the literature. J Hematol. 2019;8(1):37–9.CrossRef Fearon A, Pearcy P, Venkataraman S, et al. Protein S deficiency and arterial thromboembolism: a case report and review of the literature. J Hematol. 2019;8(1):37–9.CrossRef
31.
go back to reference Castoldi E, Maurissen LF, Tormene D, et al. Similar hypercoagulable state and thrombosis risk in type I and type III protein S-deficient individuals from families with mixed type I/III protein S deficiency. Haematologica. 2010;95(9):1563–71.CrossRef Castoldi E, Maurissen LF, Tormene D, et al. Similar hypercoagulable state and thrombosis risk in type I and type III protein S-deficient individuals from families with mixed type I/III protein S deficiency. Haematologica. 2010;95(9):1563–71.CrossRef
32.
go back to reference Wang T, Zhao XJ, Zhu HD, et al. Clinical characteristics, genes identification and follow-up study of a patient with central venous thrombosis from a protein S deficiency pedigree. Eur Rev Med Pharmacol Sci. 2021;25(1):353–61.PubMed Wang T, Zhao XJ, Zhu HD, et al. Clinical characteristics, genes identification and follow-up study of a patient with central venous thrombosis from a protein S deficiency pedigree. Eur Rev Med Pharmacol Sci. 2021;25(1):353–61.PubMed
Metadata
Title
Cerebral venous sinus thrombosis and dural arteriovenous fistula associated with protein S deficiency: a case series study
Authors
Hui Liang
Congjie Xu
Jiyi Xu
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-022-02693-3

Other articles of this Issue 1/2022

BMC Neurology 1/2022 Go to the issue