頚椎術後硬膜外血腫を契機に発見された第Ⅹ・XIII凝固因子欠乏症の1例

Congenital Factor Ⅹ・XIII Deficiency Diagnosed as a Result of the Postoperative Epidural Hematoma of the Cervical Spine. Case Report

　今回われわれは，術前スクリーニング検査に異常がなく特異的な頚椎術後硬膜外血腫を起こし，初めて凝固因子欠乏症と判明した症例を経験した．症例は69歳の男性で過去に出血傾向はなかった．問題なく椎弓形成術を行ったが，術後に異常な後出血を起こした末，硬膜外血腫を形成し神経症状が悪化したため，血腫除去術を行った．術後精査で先天性第Ⅹ・XIII凝固因子欠乏症と判明した．術後異常な後出血を認めた場合，本症を疑う必要がある．本症は新鮮凍結血漿が有効で，診断がつく前に緊急回避的処置として投与を考慮すべきである．

　We report a rare case of congenital factor Ⅹ・XIII deficiency diagnosed as a result of the development of a postoperative epidural hematoma of the cervical spine. A 69-year-old man with cervical spondylotic myelopathy underwent laminoplasty. The estimated intraoperative blood loss was only 130ml, and the postoperative course was uneventful, but the surgical wound started to bleed five hours after the operation and was difficult to control. The neurological manifestations eventually grew more as the result of the formation of an epidural hematoma, and we had to re-operate. Although the results of the routine preoperative examination were all within normal limits, detailed testing of the coagulation system revealed congenital factor XIII deficiency.