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Ⅰ.はじめに
血友病は,男子出生人口あたり5~10人/10万人程度の稀な先天性出血性疾患である5).脳神経外科領域では頭蓋内出血を起こし得ることが知られているが,家族歴があるものや小児期に異常出血で発症するなどするために,既に診断されている症例が多い.
今回われわれは,診断前の血友病を合併していた急性硬膜下血腫の手術症例を経験した.このような症例に遭遇することは稀であると考えられるが,出血傾向のある症例に対しては考慮に入れておくべきと思われたので報告する.
A 54-year-old man presented with unconsciousness. Computed tomography revealed acute subdural hematoma. Emergency evacuation of hematoma was performed showing any excessive tendency to bleed or difficulty to stop bleeding during the operation. However transfusion of fresh frozen plasma was needed to stop continuous bleeding from the surgical wound after the operation. The patient underwent craniotomy again 18 days after the operation because he suffered hemorrhagic infarction and recurrence of acute subdural hematoma. After the second operation,a coagulability examination revealed that his activated partial thromboplastin test was prolonged (74.5 seconds) and his plasma factor VIII level was 20% of normal,so he was diagnosed as having mild hemophilia A. Cranioplasty was accomplished with replacement therapy,and he was discharged with mild recent memory disturbance and homonymous hemianopsia.
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