血友病A型硬膜下出血(SDH)钻孔减压术的围手术期处理

A. Praniarda, Buyung Hartiyo Laksono
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摘要

血友病是一种严重的遗传性血液疾病,由女性传播,主要影响男性,并持续一生。A型血友病是最常见的形式。如果凝血所需的任何因素缺失或不足,凝血机制就会受到干扰,导致无法满足的出血。血友病患者最常见的死亡原因是头部外伤引起的脑出血。颅内出血应立即手术治疗,以获得较好的预后。一名17岁男性,诊断为左侧额颞顶区颅内硬膜下出血(SDH)导致2x4意识丧失,第4天脑水肿伴右侧镰下疝合并血友病A,计划钻孔减压以排出SDH。患者术前接受4000单位凝血因子VIII注射液。术中出血1100cc,输血1940cc血液制品直至血流动力学稳定。术后入住ICU 8天,病情好转后拔管。对于血友病患者,应立即进行出血清除,但再次出血的风险很高。对于严重A型血友病患者急性出血的治疗,应立即给予重组因子VIII替代品。麻醉维持应包括降低高血压和心动过速的风险,以尽量减少出血。
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Perioperative Management of Subdural Hemorrhage (SDH) Trepanation Decompression with Hemophilia A
Hemophilia is a serious inherited blood disease, transmitted by women, that affects mainly men and lasts for a lifetime. Hemophilia A is the most common form. If any of the factors necessary for blood clotting are absent or insufficient, the clotting mechanism is disturbed, causing insatiable bleeding. The most common cause of death in hemophilia patients is cerebral hemorrhage due to head trauma. In cases of intracranial hemorrhage, surgery should be performed immediately to obtain a better prognosis. A 17-year-old man diagnosed with a 2x4 loss of consciousness due to intracranial subdural hemorrhage (SDH) in the left frontotemporoparietal region and cerebral edema on day 4 accompanied by subfalcine herniation to the right with hemophilia A, planned trepanation decompression for SDH evacuation. The patient received 4000 units of factor VIII injection before surgery. Bleeding during surgery was 1100cc and he received a transfusion of 1940cc blood products until hemodynamically stable. In the postoperative phase, he was admitted to the ICU for 8 days, extubation was performed after the condition improved. In patient with hemophilia, evacuation of bleeding should be performed immediately, but there is a high risk of rebleeding. A recombinant factor VIII substitute should be administered immediately for the treatment of acute bleeding in patients with severe haemophilia A. Anesthetic maintenance should include reducing the risk of hypertension and tachycardia to minimize bleeding.
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