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CC BY-NC-ND 4.0 · Asian J Neurosurg
DOI: 10.1055/s-0045-1809910
Case Report

Associations of Refractory Spontaneous Intracranial Hypotension and Chronic Subdural Hematoma with Factor XIII Deficiency: A Case Report

Takahiro Tsuchiya
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Satoru Miyawaki 
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Keita Saito
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Shoko Yoshimoto
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Masafumi Segawa
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Kazutoshi Ebisawa
2   Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Masako Nishikawa
3   Department of Clinical Laboratory, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Masahiko Sumitani
4   Department of Pain and Palliative Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
,
Mineo Kurokawa
2   Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
3   Department of Clinical Laboratory, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
4   Department of Pain and Palliative Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
5   Department of Cell Therapy and Transplantation Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
,
Yutaka Yatomi
3   Department of Clinical Laboratory, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
,
Nobuhito Saito
1   Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
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Abstract

The co-occurrence of spontaneous intracranial hypotension (SIH) due to thoracic dural tear and factor XIII deficiency (FXIIID) is rare and may result in chronic subdural hematoma (CSDH). CSDH often recurs and is difficult to treat, despite appropriate treatment. However, there is no definitive knowledge on the optimal timing of factor XIII (FXIII) supplementation or therapeutic interventions, such as epidural blood patch (EBP) and burr hole drainage (BHD). We present a case of refractory SIH and CSDH associated with FXIIID. Considering an ineffective initial EBP, we performed a second EBP after observing sufficiently high FXIII activity; SIH was cured subsequently. The patient experienced SIH recurrence after 14 months and was treated with a combination of EBP and BHD, with sufficient FXIII supplementation. CSDH disappeared and did not recur for more than 12 months. During the treatment of SIH and CSDH associated with FXIIID, surgical treatment such as EBP appeared ineffective due to low FXIII activity. This necessitates intravenous supplementation of FXIII to maintain sufficient FXIII activity. Regular monitoring of FXIII activity is also necessary to prevent CSDH recurrence.

Keywords

spontaneous intracranial hypotension - chronic subdural hematoma - factor XIII deficiency - factor XIII activity

Authors' Contributions

S.M. designed the study, the main conceptual ideas, and the proof outline. T.T., S.Y., K.S., and M.S. collected the data. K.E., M.N., M.K., and Y.Y. aided in interpreting the results and worked on the manuscript. M.S. performed EBP. N.S. supervised the project. T.T. wrote the manuscript with support from S.M. All authors discussed the results and commented on the manuscript.


Patients' Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.




Publication History

Article published online:
23 June 2025

© 2025. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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