Wong Georgia, Gurevich Stephanie, Teti Saige, Guerrera Michael F, Zelleke Tesfaye, Gaillard William D, Oluigbo Chima O
Georgetown University School of Medicine, Washington, District of Columbia, USA.
Department of Neurosurgery, Children's National Hospital, Washington, District of Columbia, USA.
Pediatr Neurosurg. 2025;60(1-2):38-44. doi: 10.1159/000543194. Epub 2024 Dec 31.
Hereditary bleeding disorders stem from the absence or insufficient levels of particular clotting proteins, essential for facilitating coagulation in the clotting cascade. Among the most prevalent are hemophilia A (deficiency of factor VIII), hemophilia B (deficiency of factor IX), and von Willebrand disease (VWD). Management of pharmacoresistant epilepsy is more difficult in a patient with bleeding disorder due to increased risk of bleeding during surgery. While patients who have both a bleeding disorder and epilepsy are rare, reporting on the management of these patients who may require intracranial monitoring for pharmacoresistant epilepsy offers valuable insights into the challenges and considerations necessary for safely navigating the complex intersection of bleeding risk and seizure control.
Two patients with bleeding disorders (VWD and factor XI deficiency) underwent invasive intracranial monitoring for medical refractory epilepsy followed by epilepsy focus resection surgery. Both patients were found to have a bleeding disorder during their preoperative laboratory work. After abnormal laboratories were reported, both patients were referred to hematology for further evaluation and surgical planning. The first patient was a 10-year-old boy with medically refractory focal epilepsy who was found to have type IIM VWD. He underwent surgery for subdural grid placement followed by resection on postoperative day 6. He required wilate® (human von Willebrand factor/coagulation factor VIII complex) infusions from the day of surgery prior to surgery through postoperative day 14. The second case was a 2-year-old boy with a history of tuberous sclerosis and medically refractory epilepsy who was found to have factor XI deficiency (hemophilia C) who required fresh frozen plasma and platelet transfusions throughout his hospitalization. He underwent surgery for sEEG followed by resection of the tubers. Both patients remained stable throughout their invasive monitoring and completed epilepsy resection surgeries without reported complications. Both patients achieved seizure freedom after surgery since their most recent follow-up of 1 month and 13 months.
The two patients successfully underwent invasive neuromonitoring with subdural grids and sEEG for seizure focus identification followed by resective epilepsy surgery without bleeding complications while achieving seizure freedom. While epilepsy patients with a bleeding disorder should not automatically be denied surgery due to the increased risk of hemorrhage, it is crucial that any decision is based on a comprehensive, multidisciplinary evaluation. This case report highlights the potential for future meta-analysis and further conversations regarding improved protocols for patients with bleeding disorders.
遗传性出血性疾病源于特定凝血蛋白的缺乏或水平不足，这些蛋白对于在凝血级联反应中促进凝血至关重要。其中最常见的是血友病A（因子VIII缺乏）、血友病B（因子IX缺乏）和血管性血友病（VWD）。由于手术期间出血风险增加，对于患有出血性疾病的患者，药物难治性癫痫的管理更加困难。虽然同时患有出血性疾病和癫痫的患者很少见，但报告这些可能需要进行颅内监测以治疗药物难治性癫痫的患者的管理情况，对于安全应对出血风险和癫痫控制这一复杂交叉点所需的挑战和考虑因素提供了有价值的见解。
两名患有出血性疾病（VWD和因子XI缺乏）的患者接受了有创颅内监测以治疗药物难治性癫痫，随后进行了癫痫病灶切除术。两名患者在术前实验室检查中均被发现患有出血性疾病。在报告异常实验室检查结果后，两名患者均被转诊至血液科进行进一步评估和手术规划。第一名患者是一名10岁男孩，患有药物难治性局灶性癫痫，被发现患有IIM型VWD。他接受了硬膜下格栅置入手术，术后第6天进行了切除术。从手术当天到术后第14天，他需要输注威尔特（人血管性血友病因子/凝血因子VIII复合物）。第二例是一名2岁男孩，有结节性硬化症病史且患有药物难治性癫痫，被发现患有因子XI缺乏（血友病C），在住院期间需要输注新鲜冰冻血浆和血小板。他接受了立体定向脑电图（sEEG）检查，随后切除了结节。两名患者在整个有创监测过程中均保持稳定，完成了癫痫切除手术，且未报告并发症。自最近一次随访1个月和13个月以来，两名患者术后均实现了无癫痫发作。
两名患者成功接受了硬膜下格栅和sEEG有创神经监测以确定癫痫病灶，随后进行了切除性癫痫手术，未出现出血并发症，同时实现了无癫痫发作。虽然患有出血性疾病的癫痫患者不应因出血风险增加而自动被拒绝手术，但任何决定都必须基于全面的多学科评估，这一点至关重要。本病例报告强调了未来进行荟萃分析以及就改善出血性疾病患者治疗方案展开进一步讨论的可能性。
