Addis Ababa University College of Heath Science, Addis Ababa, Ethiopia.
Addis Ababa University College of Heath Science, Addis Ababa, Ethiopia.
World Neurosurg. 2020 Jan;133:112-120. doi: 10.1016/j.wneu.2019.09.059. Epub 2019 Sep 25.
Retroclival hematomas are a rare entity and may occur in 3 compartments, namely the epidural, subdural, and subarachnoid spaces. They are frequently secondary to trauma. Hemophilia is a clinical syndrome affecting usually men and characterized by the inherited tendency to bleed excessively after slight injury. Hemophilia is caused by a specific defect of coagulation factor VIII. The main concern associated with the disease is bleeding, especially after trauma and surgeries. The most serious site of bleeding is the central nervous system.
An 11-year-old boy diagnosed with hemophilia presented after sustaining a fall. On arrival to the emergency department, his vitals where within normal range and he was fully conscious. Neurologic examination was significant for bilateral abducens nerve palsy; the rest of the examination was unremarkable. Imaging studies with computed tomography (CT) scan and brain magnetic resonance imaging showed subacute retroclival subdural hemorrhage with left cerebellar and upper cervical spine extension. Follow-up imaging with CT scan showed progressive resolution of the hematoma, and the patient had a stable clinical course while receiving factor VII replacement.
Retroclival subdural hematomas are rare and may present either spontaneously or after trauma. Conservative treatment is the usual course of treatment. Patients with hemophilia A are under a constant threat from bleeding, either spontaneous or after trivial injury. The most common cause of death in this patient population is intracranial hemorrhage. The most important aspect of intracranial hematoma management is the early replacement therapy of deficient coagulation factors in patients with hemophilia.
颅后窝硬膜外血肿是一种罕见的病症,可能发生在 3 个部位,即硬膜外、硬膜下和蛛网膜下腔。它们通常是外伤的结果。血友病是一种影响男性的临床综合征,其特征是轻微损伤后过度出血的遗传倾向。血友病是由凝血因子 VIII 的特定缺陷引起的。与该疾病相关的主要关注点是出血,尤其是在创伤和手术后。最严重的出血部位是中枢神经系统。
一名被诊断为血友病的 11 岁男孩在摔倒后就诊。到达急诊室时,他的生命体征在正常范围内,意识完全清醒。神经检查双侧展神经麻痹显著;其余检查无明显异常。计算机断层扫描(CT)和脑磁共振成像显示亚急性颅后窝硬膜下血肿,伴有左侧小脑和颈上段延伸。CT 扫描随访显示血肿逐渐吸收,患者在接受因子 VII 替代治疗时临床状况稳定。
颅后窝硬膜下血肿罕见,可自发性或外伤后出现。保守治疗是常见的治疗方法。甲型血友病患者持续面临出血的威胁,无论是自发性还是轻微损伤后。该患者人群中死亡的最常见原因是颅内出血。颅内血肿管理最重要的方面是对血友病患者及时补充缺乏的凝血因子进行替代治疗。