Department of Pediatrics, Ministry of National Guards-Health Affairs, Riyadh, Saudi Arabia.
King Abdullah International Medical Research Center, Riyadh, Saudi Arabia.
J Med Case Rep. 2021 Aug 6;15(1):418. doi: 10.1186/s13256-021-03005-y.
Elevated intracranial pressure is a potentially catastrophic complication of neurologic injury in children. Successful management of elevated intracranial pressure requires prompt recognition and therapy directed at both reducing intracranial pressure and reversing its underlying cause. A rare condition that causes elevated intracranial pressure is childhood primary angiitis of the central nervous system, which is a rare inflammatory central nervous system disease that poses diagnostic and therapeutic challenges. To our knowledge, this is the first reported case of angiography-positive progressive childhood primary angiitis of the central nervous system requiring decompressive hemicraniectomy for refractory elevated intracranial pressure in children.
We report the case of a 5-year-old Saudi girl who presented to the pediatric emergency department with fever and new-onset status epilepticus. She had elevated inflammatory markers with radiological and histopathological evidence of angiography-positive progressive childhood primary angiitis of the central nervous system, complicated by elevated intracranial pressure. Despite medical management for both childhood primary angiitis of the central nervous system and elevated intracranial pressure, her neurological status continued to deteriorate and the elevated intracranial pressure became refractory. She developed right uncal, right subfalcine, and tonsillar herniation requiring decompressive hemicraniectomy with a favorable neurological outcome.
Decompressive craniectomy might be considered in cases of angiography-positive progressive childhood primary angiitis of the central nervous system with elevated intracranial pressure refractory to medication. A multidisciplinary approach for the decision of decompressive craniectomy is advised to ensure patient safety and avoid possible morbidities and mortality.
颅内压升高是儿童神经损伤潜在的灾难性并发症。成功管理颅内压升高需要迅速识别和治疗，既要降低颅内压，又要逆转其根本原因。一种导致颅内压升高的罕见情况是儿童中枢性原发性血管炎，这是一种罕见的炎症性中枢神经系统疾病，具有诊断和治疗方面的挑战。据我们所知，这是首例报告的血管造影阳性进展性儿童中枢性原发性血管炎病例，需要行去骨瓣减压术治疗儿童难治性颅内压升高。
我们报告了一例 5 岁沙特女孩的病例，她因发热和新发性癫痫发作到儿科急诊就诊。她的炎症标志物升高，影像学和组织病理学均有血管造影阳性进展性儿童中枢性原发性血管炎的证据，并伴有颅内压升高。尽管针对中枢性原发性血管炎和颅内压升高进行了药物治疗，但她的神经状态仍继续恶化，颅内压升高变得难治。她出现右侧钩回、右侧小脑幕切迹下和小脑扁桃体疝，需要行去骨瓣减压术，神经功能预后良好。
对于药物治疗难治性血管造影阳性进展性儿童中枢性原发性血管炎合并颅内压升高的患者，可能需要考虑行去骨瓣减压术。建议采取多学科方法来决定是否行去骨瓣减压术，以确保患者安全并避免可能的并发症和死亡率。
