Mustafa Mohammed, Amawi Muhanned, Altoonisi Mustafa M, Soliman Wessam, Kamal Mohamed, Asaad Ziad, Albalawi Aseel, Alharbi Joud, Awadalla Akram M, A-Azim Ahmed Mohamed, Hanafy Ehab
Prince Sultan Oncology Center, King Salman Armed Forces Hospital, Tabuk, SAU.
Pediatrics, King Salman Armed Forces Hospital, Tabuk, SAU.
Cureus. 2024 Oct 5;16(10):e70871. doi: 10.7759/cureus.70871. eCollection 2024 Oct.
Sickle cell anemia (SCA) is a genetic disorder characterized by the production of abnormal hemoglobin S, leading to red blood cell sickling and subsequent vaso-occlusive events. Neurological complications, particularly strokes, significantly contribute to the morbidity and mortality associated with SCA. While ischemic strokes are more common, hemorrhagic strokes, though less frequent, present significant challenges, especially in the pediatric population. Understanding the complex interplay of genetic, environmental, and hematological factors is crucial for managing these cases. We report two cases of pediatric patients with SCA who experienced rare hemorrhagic strokes. The first case involves a nine-year-old male presenting with a subarachnoid hemorrhage, revealing cortical ischemia and multiple cerebral artery strictures. Early supportive measures resulted in a good clinical improvement, after which the patient underwent bone marrow transplantation. The second case describes a seven-year-old male who developed an epidural hematoma during a vaso-occlusive crisis, necessitating emergency surgical intervention. After initial persistent neurological deficits, the patient began to show gradual improvement with ongoing management, reflecting the complexity and severity of such events. Hemorrhagic strokes in pediatric SCA patients, though rare, represent significant clinical challenges due to their multifactorial etiology and complex management needs. These cases underscore the importance of a multidisciplinary approach and advanced diagnostic tools in managing hemorrhagic complications in SCA. Further research is essential to unravel the pathophysiological mechanisms and develop targeted prevention strategies to improve outcomes for this vulnerable population.
镰状细胞贫血(SCA)是一种遗传性疾病,其特征是产生异常血红蛋白S,导致红细胞镰变并随后发生血管闭塞事件。神经并发症,尤其是中风,是导致SCA相关发病率和死亡率的重要因素。虽然缺血性中风更为常见,但出血性中风尽管发生率较低,但带来了重大挑战,尤其是在儿科人群中。了解遗传、环境和血液学因素之间的复杂相互作用对于处理这些病例至关重要。我们报告了两例患有SCA的儿科患者发生罕见出血性中风的病例。第一例涉及一名9岁男性,表现为蛛网膜下腔出血,伴有皮质缺血和多条脑动脉狭窄。早期支持性措施使临床状况得到良好改善,之后患者接受了骨髓移植。第二例描述了一名7岁男性,在血管闭塞危机期间发生硬膜外血肿,需要紧急手术干预。在最初持续存在神经功能缺损后,随着持续治疗,患者开始逐渐好转,这反映了此类事件的复杂性和严重性。儿科SCA患者的出血性中风虽然罕见,但由于其多因素病因和复杂的管理需求,代表了重大的临床挑战。这些病例强调了多学科方法和先进诊断工具在管理SCA出血性并发症中的重要性。进一步的研究对于阐明病理生理机制和制定有针对性的预防策略以改善这一弱势群体的预后至关重要。