Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia

Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group; Katarzyna Krzywicka; Diana Aguiar de Sousa; Charlotte Cordonnier; Felix J. Bode; Thalia S. Field; Dominik Michalski; Johann Pelz; Mona Skjelland; Markus Wiedmann; Julian Zimmermann; Matthias Wittstock; Bruno Zanotti; Alfonso Ciccone; Monica Bandettini di Poggio; Afshin Borhani-Haghighi; Sophie Chatterton; Avinash Aujayeb; Annemie Devroye; Vanessa Dizonno; Thomas Geeraerts; Fabrizio Giammello; Albrecht Günther; Nasli R. Ichaporia; Timothy Kleinig; Espen S. Kristoffersen; Robin Lemmens; Emmanuel De Maistre; Zahra Mirzaasgari; Jean Francois Payen; Jukka Putaala; Marco Petruzzellis; Nicolas Raposo; Elyar Sadeghi-Hokmabadi; Silvia Schoenenberger; Meenakshisundaram Umaiorubahan; Padmavathy N. Sylaja; Anita van de Munckhof; Mayte Sánchez van Kammen; Erik Lindgren; Katarina Jood; Adrian Scutelnic; Mirjam R. Heldner; Sven Poli; Marieke J.H.A. Kruip; Antonio Arauz; Adriana B. Conforto; Sanjith Aaron; Saskia Middeldorp; Turgut Tatlisumak; Marcel Arnold

doi:10.1111/ene.15735

Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia

Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group, Katarzyna Krzywicka, Diana Aguiar de Sousa, Charlotte Cordonnier, Felix J. Bode, Thalia S. Field, Dominik Michalski, Johann Pelz, Mona Skjelland, Markus Wiedmann, Julian Zimmermann, Matthias Wittstock, Bruno Zanotti, Alfonso Ciccone, Monica Bandettini di Poggio, Afshin Borhani-Haghighi, Sophie Chatterton, Avinash Aujayeb, Annemie Devroye, Vanessa DizonnoThomas Geeraerts, Fabrizio Giammello, Albrecht Günther, Nasli R. Ichaporia, Timothy Kleinig, Espen S. Kristoffersen, Robin Lemmens, Emmanuel De Maistre, Zahra Mirzaasgari, Jean Francois Payen, Jukka Putaala, Marco Petruzzellis, Nicolas Raposo, Elyar Sadeghi-Hokmabadi, Silvia Schoenenberger, Meenakshisundaram Umaiorubahan, Padmavathy N. Sylaja, Anita van de Munckhof, Mayte Sánchez van Kammen, Erik Lindgren, Katarina Jood, Adrian Scutelnic, Mirjam R. Heldner, Sven Poli, Marieke J.H.A. Kruip, Antonio Arauz, Adriana B. Conforto, Sanjith Aaron, Saskia Middeldorp, Turgut Tatlisumak, Marcel Arnold

Hematology

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Abstract

Background and purpose: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. Methods: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680–1689), were included. Results: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. Conclusions: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.

Original language	English
Pages (from-to)	1335-1345
Number of pages	11
Journal	European Journal of Neurology
Volume	30
Issue number	5
Early online date	11 Feb 2023
DOIs	https://doi.org/10.1111/ene.15735
Publication status	Published - May 2023

Bibliographical note

Funding Information:
This research was funded by the Netherlands Organization for Health Research and Development (ZonMw, grant number 10430072110005) and the Dr C.J. Vaillant Foundation. The funding organizations had no role in gathering, analysing or interpreting the data.

Publisher Copyright:
© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

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Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopeniaFinal published version, 713 KBLicence: CC BY-NC-ND

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Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group, Krzywicka, K., Aguiar de Sousa, D., Cordonnier, C., Bode, F. J., Field, T. S., Michalski, D., Pelz, J., Skjelland, M., Wiedmann, M., Zimmermann, J., Wittstock, M., Zanotti, B., Ciccone, A., Bandettini di Poggio, M., Borhani-Haghighi, A., Chatterton, S., Aujayeb, A., Devroye, A., ... Arnold, M. (2023). Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia. European Journal of Neurology, 30(5), 1335-1345. https://doi.org/10.1111/ene.15735

@article{95373b06e8a945f294660f60a4123d45,

title = "Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia",

abstract = "Background and purpose: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. Methods: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680–1689), were included. Results: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. Conclusions: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.",

author = "{Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group} and Katarzyna Krzywicka and Diana Aguiar de Sousa and Charlotte Cordonnier and Bode, {Felix J.} and Field, {Thalia S.} and Dominik Michalski and Johann Pelz and Mona Skjelland and Markus Wiedmann and Julian Zimmermann and Matthias Wittstock and Bruno Zanotti and Alfonso Ciccone and Monica Bandettini di Poggio and Afshin Borhani-Haghighi and Sophie Chatterton and Avinash Aujayeb and Annemie Devroye and Vanessa Dizonno and Thomas Geeraerts and Fabrizio Giammello and Albrecht G{\"u}nther and Ichaporia, {Nasli R.} and Timothy Kleinig and Kristoffersen, {Espen S.} and Robin Lemmens and Emmanuel De Maistre and Zahra Mirzaasgari and Payen, {Jean Francois} and Jukka Putaala and Marco Petruzzellis and Nicolas Raposo and Elyar Sadeghi-Hokmabadi and Silvia Schoenenberger and Meenakshisundaram Umaiorubahan and Sylaja, {Padmavathy N.} and {van de Munckhof}, Anita and Mayte S{\'a}nchez van Kammen and Erik Lindgren and Katarina Jood and Adrian Scutelnic and Heldner, {Mirjam R.} and Sven Poli and Kruip, {Marieke J.H.A.} and Antonio Arauz and Conforto, {Adriana B.} and Sanjith Aaron and Saskia Middeldorp and Turgut Tatlisumak and Marcel Arnold",

note = "Funding Information: This research was funded by the Netherlands Organization for Health Research and Development (ZonMw, grant number 10430072110005) and the Dr C.J. Vaillant Foundation. The funding organizations had no role in gathering, analysing or interpreting the data. Publisher Copyright: {\textcopyright} 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.",

year = "2023",

month = may,

doi = "10.1111/ene.15735",

language = "English",

volume = "30",

pages = "1335--1345",

journal = "European Journal of Neurology",

issn = "1351-5101",

publisher = "Wiley-Blackwell Publishing Ltd",

number = "5",

}

Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group, Krzywicka, K, Aguiar de Sousa, D, Cordonnier, C, Bode, FJ, Field, TS, Michalski, D, Pelz, J, Skjelland, M, Wiedmann, M, Zimmermann, J, Wittstock, M, Zanotti, B, Ciccone, A, Bandettini di Poggio, M, Borhani-Haghighi, A, Chatterton, S, Aujayeb, A, Devroye, A, Dizonno, V, Geeraerts, T, Giammello, F, Günther, A, Ichaporia, NR, Kleinig, T, Kristoffersen, ES, Lemmens, R, De Maistre, E, Mirzaasgari, Z, Payen, JF, Putaala, J, Petruzzellis, M, Raposo, N, Sadeghi-Hokmabadi, E, Schoenenberger, S, Umaiorubahan, M, Sylaja, PN, van de Munckhof, A, Sánchez van Kammen, M, Lindgren, E, Jood, K, Scutelnic, A, Heldner, MR, Poli, S, Kruip, MJHA, Arauz, A, Conforto, AB, Aaron, S, Middeldorp, S, Tatlisumak, T & Arnold, M 2023, 'Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia', European Journal of Neurology, vol. 30, no. 5, pp. 1335-1345. https://doi.org/10.1111/ene.15735

Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia. / Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group; Krzywicka, Katarzyna; Aguiar de Sousa, Diana et al.
In: European Journal of Neurology, Vol. 30, No. 5, 05.2023, p. 1335-1345.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia

AU - Cerebral Venous Sinus Thrombosis with Thrombocytopenia Syndrome Study Group

AU - Krzywicka, Katarzyna

AU - Aguiar de Sousa, Diana

AU - Cordonnier, Charlotte

AU - Bode, Felix J.

AU - Field, Thalia S.

AU - Michalski, Dominik

AU - Pelz, Johann

AU - Skjelland, Mona

AU - Wiedmann, Markus

AU - Zimmermann, Julian

AU - Wittstock, Matthias

AU - Zanotti, Bruno

AU - Ciccone, Alfonso

AU - Bandettini di Poggio, Monica

AU - Borhani-Haghighi, Afshin

AU - Chatterton, Sophie

AU - Aujayeb, Avinash

AU - Devroye, Annemie

AU - Dizonno, Vanessa

AU - Geeraerts, Thomas

AU - Giammello, Fabrizio

AU - Günther, Albrecht

AU - Ichaporia, Nasli R.

AU - Kleinig, Timothy

AU - Kristoffersen, Espen S.

AU - Lemmens, Robin

AU - De Maistre, Emmanuel

AU - Mirzaasgari, Zahra

AU - Payen, Jean Francois

AU - Putaala, Jukka

AU - Petruzzellis, Marco

AU - Raposo, Nicolas

AU - Sadeghi-Hokmabadi, Elyar

AU - Schoenenberger, Silvia

AU - Umaiorubahan, Meenakshisundaram

AU - Sylaja, Padmavathy N.

AU - van de Munckhof, Anita

AU - Sánchez van Kammen, Mayte

AU - Lindgren, Erik

AU - Jood, Katarina

AU - Scutelnic, Adrian

AU - Heldner, Mirjam R.

AU - Poli, Sven

AU - Kruip, Marieke J.H.A.

AU - Arauz, Antonio

AU - Conforto, Adriana B.

AU - Aaron, Sanjith

AU - Middeldorp, Saskia

AU - Tatlisumak, Turgut

AU - Arnold, Marcel

N1 - Funding Information: This research was funded by the Netherlands Organization for Health Research and Development (ZonMw, grant number 10430072110005) and the Dr C.J. Vaillant Foundation. The funding organizations had no role in gathering, analysing or interpreting the data. Publisher Copyright: © 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

PY - 2023/5

Y1 - 2023/5

N2 - Background and purpose: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. Methods: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680–1689), were included. Results: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. Conclusions: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.

AB - Background and purpose: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. Methods: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680–1689), were included. Results: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. Conclusions: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.

UR - http://www.scopus.com/inward/record.url?scp=85150518544&partnerID=8YFLogxK

U2 - 10.1111/ene.15735

DO - 10.1111/ene.15735

M3 - Article

C2 - 36773014

AN - SCOPUS:85150518544

SN - 1351-5101

VL - 30

SP - 1335

EP - 1345

JO - European Journal of Neurology

JF - European Journal of Neurology

IS - 5

ER -

Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia

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