Aras Mustafa, Oral Sukru
Department of Neurosurgery, Ondokuzmayis University, Samsun, Turkey.
Department of Neurosurgery,, Kayseri City Hospital, Kocasinan Kayseri, Turkey.
Childs Nerv Syst. 2020 Sep;36(9):2041-2046. doi: 10.1007/s00381-020-04528-3. Epub 2020 Feb 5.
Intracranial hemorrhage due to head trauma in patients with hemophilia is a major cause of mortality and morbidity. The incidence of central nervous system bleeding in these patients is between 3 and 8%. In this study, we shared our experience on the treatment and follow-up of seven pediatric patients with hemophilia A who had intracranial bleeding due to trauma.
Between 2010 and 2019, the clinical and radiological findings of seven pediatric patients with hemophilia A with intracranial hemorrhage were retrospectively evaluated in our clinic. One patient underwent operation owing to intracranial hemorrhage, and the other six underwent conservative treatment. Hemoglobin, aPTT (activated partial thromboplastin time), and factor VIII levels were measured at regular intervals. For intracranial hemorrhage follow-up, regular computed tomography (CT) was performed.
All patients visited the emergency department with initial neurological complaints. Further, two of the seven patients died, one was treated with sequelae (cerebral palsy), and the other four were treated without sequelae.
Early diagnosis and treatment of intracranial hemorrhage is very important in patients with bleeding disorders. Factor VIII replacement should be performed in such patients prior to radiological examinations and consultations. The main objective should be to bring factor VIII levels to normal limits during their treatment and follow-up.
血友病患者因头部外伤导致的颅内出血是死亡和发病的主要原因。这些患者中枢神经系统出血的发生率在3%至8%之间。在本研究中，我们分享了7例因外伤导致颅内出血的甲型血友病患儿的治疗和随访经验。
2010年至2019年期间，我们对本诊所7例甲型血友病合并颅内出血患儿的临床和影像学检查结果进行了回顾性评估。1例患者因颅内出血接受了手术，另外6例接受了保守治疗。定期测量血红蛋白、活化部分凝血活酶时间（aPTT）和凝血因子VIII水平。对于颅内出血的随访，定期进行计算机断层扫描（CT）。
所有患者均因最初的神经系统症状前往急诊科就诊。此外，7例患者中有2例死亡，1例有后遗症（脑瘫），另外4例无后遗症。
对于出血性疾病患者，颅内出血的早期诊断和治疗非常重要。此类患者应在进行影像学检查和会诊之前进行凝血因子VIII替代治疗。主要目标应是在治疗和随访期间将凝血因子VIII水平恢复到正常范围。
