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甲型血友病患者后颅窝急性硬膜外血肿——2例手术治疗病例报告

[Acute epidural hematoma in the posterior fossa in patients with hemophilia A--report of two surgically treated cases].

作者信息

Ohishi H, Onuma T, Mizoi K, Mori K

出版信息

No Shinkei Geka. 1983 Jun;11(6):659-64.

PMID:6413874
Abstract

Case I: A 9-year-old boy, diagnosed as having hemophilia A at 8 months, was admitted complaining of slight headache and nausea one day after a minor head trauma. Neurological deficits were absent but CT scan revealed an epidural hematoma in the posterior fossa. Shortly afterwards, he lapsed into coma with apnea and dilated pupils. Following resuscitation, emergency suboccipital craniectomy and total removal of the bilateral supra- and infratentorial extradural hematoma was performed under AHG administration. The patient gradually regained consciousness, but during the subsequent nine weeks he underwent three major operations (laparotomy): the first two for hemostasis of gastrointestinal bleeding, and the last one for strangulated intestinal obstruction. Although this patient necessitated 16 weeks of AHG administration, he was discharged without any side effects after 4 months of hospitalization. Case II: A 10-year-old boy, diagnosed earlier as having hemophilia A, experienced a minor head trauma and was admitted because of headaches and nausea. CT scan revealed an epidural hematoma in the posterior fossa. Removal of the hematoma was successfully completed under AHG administration. The patient was discharged without any neurological deficits. In the above hemophilic cases, we used a high concentrated AHG and maintained at 70% of the plasma concentration of the VIII factor during the first 14 postoperative days. The high concentrated AHG was safe for long term administration, so one should not hesitate operation even in the case of intracranial hemorrhage of hemophilic patients. CT scan should be recommended to the patient of hemophilia A even in minor head trauma.

摘要

病例一

一名9岁男孩,8个月时被诊断为甲型血友病,在一次轻微头部外伤一天后因轻微头痛和恶心入院。无神经功能缺损,但CT扫描显示后颅窝硬膜外血肿。不久后,他陷入昏迷,伴有呼吸暂停和瞳孔散大。复苏后,在输注抗血友病球蛋白(AHG)的情况下进行了紧急枕下颅骨切除术,并完全清除了双侧幕上和幕下硬膜外血肿。患者逐渐恢复意识,但在随后的九周内他接受了三次大手术(剖腹手术):前两次是为了治疗胃肠道出血的止血,最后一次是为了治疗绞窄性肠梗阻。尽管该患者需要输注AHG 16周,但住院4个月后出院时没有任何副作用。病例二:一名10岁男孩,早些时候被诊断为甲型血友病,经历了一次轻微头部外伤,因头痛和恶心入院。CT扫描显示后颅窝硬膜外血肿。在输注AHG的情况下成功完成了血肿清除术。患者出院时没有任何神经功能缺损。在上述血友病病例中,我们使用了高浓度的AHG,并在术后的前14天将其维持在VIII因子血浆浓度的70%。高浓度的AHG长期使用是安全的,因此即使是血友病患者发生颅内出血也不应犹豫进行手术。即使是轻微头部外伤的甲型血友病患者也应建议进行CT扫描。

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