Komatsu Y, Narushima K, Kobayashi E, Nose T, Maki Y
Department of Neurosurgery, Tsukuba Memorial Hospital.
No Shinkei Geka. 1988 Oct;16(11):1281-5.
A case of ruptured cerebral aneurysm with hemophilia B is reported, and discussion is made concerning the management of mild type hemophilia in surgical operations. A 41-year-old male came to our hospital with complaints of severe headache, vomiting, and transient consciousness disturbance. His dentist said the patient had a mild bleeding tendency when he was 30 years old, however no postoperative hemorrhage was repeated in appendectomy in his childhood. He also had had no episodes of spontaneous bleeding. CT scan on admission showed subarachnoid hemorrhage, and angiography revealed a ruptured aneurysm at the trifurcation of the left middle cerebral artery. His coagulation screening tests (bleeding time, clotting time, prothrombin time, and activated partial thromboplastin time) were normal. An aneurysmal neck clipping was carried out, and operators did not detect any bleeding tendency during the surgery. CT scan on the next day showed no remarkable finding. On the third postoperative day, right hemiparesis occurred. Left putaminal hemorrhage took place. His coagulation tests and FDP were also normal. The hematoma was partially evacuated. After the second operation his condition was good, and rehabilitation program started. On the 15th hospital day his consciousness deteriorated suddenly, and CT scan showed a massive epidural hematoma on the left. His prothrombin time elongated mildly, but other tests were normal. Coagulation factors VIII and IX were examined and the factor IX was 22.5% of control. He was thought to be a patient with mild type hemophilia B. Despite a third operation for hematoma removal he died on the 20th hospital day. Mild type hemophilia B does not bleed spontaneously.(ABSTRACT TRUNCATED AT 250 WORDS)
报告了一例乙型血友病合并脑动脉瘤破裂的病例,并对手术中轻型血友病的处理进行了讨论。一名41岁男性因严重头痛、呕吐和短暂意识障碍前来我院就诊。他的牙医称其在30岁时有轻度出血倾向,但童年时阑尾切除术后未反复出血。他也没有自发性出血史。入院时CT扫描显示蛛网膜下腔出血,血管造影显示左大脑中动脉分叉处有一破裂动脉瘤。他的凝血筛查试验(出血时间、凝血时间、凝血酶原时间和活化部分凝血活酶时间)均正常。进行了动脉瘤夹闭术,术中术者未发现任何出血倾向。次日CT扫描未见明显异常。术后第三天,出现右侧偏瘫,左侧豆状核出血。他的凝血试验和纤维蛋白降解产物也正常。血肿部分清除。第二次手术后他情况良好,开始了康复治疗。住院第15天,他的意识突然恶化,CT扫描显示左侧有巨大硬膜外血肿。他的凝血酶原时间轻度延长,但其他检查正常。检测了凝血因子VIII和IX,因子IX为对照值的22.5%。他被认为是轻型乙型血友病患者。尽管进行了第三次血肿清除手术,他仍在住院第20天死亡。轻型乙型血友病不会自发性出血。(摘要截短至250字)