Myles L M, Massicotte P, Drake J
Division of Neurosurgery, The Hospital for Sick Children, University of Toronto, Canada.
Pediatr Neurosurg. 2001 Feb;34(2):94-7. doi: 10.1159/000056001.
Hemophilia is a rare disorder, and an uncommon cause of intracranial hemorrhage in neonates. We present 2 patients with hemophilia A, who presented with massive subdural hemorrhages on day 5 and day 4 postpartum. Both were taken urgently to surgery without a diagnosis of hemophilia being established. Neither patient had a family history of hemophilia, and both were born following difficult deliveries. The activated partial thromboplastin time (APTT) was normal in patient No. 1 (subsequent factor VIII level 10%). In patient No. 2, the APTT was slightly prolonged, but initially interpreted as being within the normal range for age (subsequent factor VII level of < 1%). Patient 1 rebled, required a second operation, and had a poor outcome. Patient 2 was given prophylactic fresh frozen plasma, and made a good recovery. Factor VIII assay should be performed in all term male babies presenting with intracranial hemorrhage. In urgent circumstances, prophylactic clotting therapy should be administered during surgery to prevent postoperative bleeding in an undiagnosed hemophiliac.
血友病是一种罕见的疾病，也是新生儿颅内出血的不常见原因。我们报告2例甲型血友病患者，分别在产后第5天和第4天出现大量硬膜下出血。两人均在未确诊血友病的情况下紧急接受手术。两名患者均无血友病家族史，且均为难产出生。1号患者活化部分凝血活酶时间（APTT）正常（随后检测因子VIII水平为10%）。2号患者APTT略有延长，但最初被解释为在该年龄正常范围内（随后检测因子VII水平<1%）。1号患者再次出血，需要进行二次手术，预后不良。2号患者接受了预防性新鲜冰冻血浆治疗，恢复良好。对于所有出现颅内出血的足月男婴，均应进行因子VIII检测。在紧急情况下，手术期间应给予预防性凝血治疗，以防止未确诊血友病患者术后出血。
