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はじめに
Silverstein14は1960年に血友病患者の2.2〜7.8%に頭蓋内出血が合併し,その70%は死の転帰をとると報告している。
1960年以前の血友病に合併した頭蓋内出血に対する開頭術,穿頭術の報告は13例みとめられるが,そのうち救命しえた症例は3例に過ぎない。しかしその後1965年Pool&Shannon12)が寒冷抽出による抗血友病性グロブリン(第VIII因子)濃縮蛋白を報告し,1967年Prentice等13)がこれを臨床に応用し,開頭血腫除去に成功したのを始めとし,以来Brown1)(1967),Moody&Mullan7)(1968)等,次々とこの寒冷抽出による抗血友病性グロブリン濃縮蛋白使用による手術成功例を報告し,その後も同製剤使用による血友病患者の開頭術穿頭術成功例の報告が急速に増加しつつある。
Surgical experiences of two cases of subdural hematoma in infancy with congenital hemorrhagic diathesis were reported.
It has been considered that intracranial surgical approach to the patient with congenital hemorrhagic diathesis is contraindicated. However, recent ac-cumulation of knowledge about the mechanism of blood coagulation and use of cryoprecipitate (Factor VIII concentrate) made a surgical treatment possible in these cases. It is advocated with the presenta-tion of two cases that the surgical treatment should be considered even in patients with congenital hemorrhagic diathesis, when surgery is indicated. Case 1 : The patient was a 18 month old male with classical hemophilia who had an acute subdural hematoma. Carotid angiography and craniotomy were performed after the injection of Factor VIII concentrate. There was no unusual bleeding through-out the surgery. He died because of the delayed operation.
Case 2 : A 22 month old female with a parahemo-philia (Factor V level : 10%) was hospitalized several times to some clinic for bleeding episodes. Nine days before admission in our clinic, she had a minor injury on the head. Left carotid angiography re-vealed the presence of a subdural hematoma in the left temporooccipital region. The partially liquefied hematoma was evacuated through two burr holes made in the left temporal and occipital regions after fresh blood transfusion. There was no unusual bleeding during the surgery and the postoperative period. She made uneventful recovery and was dischargod on the 21st postoperative day.
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