血液凝固第XIII因子補充療法が著効を示した硬膜外自己血パッチ無効のPDPH

The Effect of Intravenous Factor XIII Administration on Intractable Postdural Puncture Headache：A Case Report

要旨

　硬膜穿刺後頭痛（postdural puncture headache：PDPH）は，硬膜穿刺部位からの髄液漏出に起因する合併症である。今回，保存療法および硬膜外自己血パッチ（EBP）を複数回実施したにもかかわらず，PDPHが1カ月以上遷延した血液凝固第XIII因子欠乏症患者に対して，補充療法（適用外使用）が著効を示した症例を経験した。

　No definite treatment recommendations have been established for an intractable postdural puncture headache（PDPH）that resists repeated epidural blood patches（EBPs）. We describe the case details of a 45-year-old Japanese woman with an unrevealed Factor XIII deficiency who suffered from a prolonged PDPH that was not resolved by two consecutive EBPs. A thorough patient interview led to the suspicion of coagulopathy, and blood tests revealed a low factor XIII value（62％, normal range70-130％）. Referring to the literature concerning spontaneous intracranial hypotension, we administered an intravenous infusion of factor XIII（Fibrogamin^® P, 240 unitsvial, 5 vialsday for 5 days）. Lator rhat dat, her her headache gradually inproved, after 7 day, she was dischaged, and 12 days after treatment, her headache disappeared completely. Although an intravenous administration of factor XIII is an off-label use in Japan at this time, our patient's case indicates that the use of an intravenous infusion of factor XIII might be useful for treating intractable PDPHs.