Saxena Parima, Alkaissi Hussam, Chauhan Riddhi, Muthu John
Internal Medicine, State University of New York Downstate Medical Center, Brooklyn, USA.
Internal Medicine, Kings County Hospital Center, Brooklyn, USA.
Cureus. 2023 Jan 28;15(1):e34314. doi: 10.7759/cureus.34314. eCollection 2023 Jan.
The association between Moyamoya syndrome (MMS) and sickle cell disease (SCD) has been well-established in pediatric populations; however, limited literature exists documenting the characteristics and management of MMS in adult SCD patients. Studies have indicated the role of endovascular management in secondary stroke prevention for pediatric populations, with no current guidelines available for adult populations. Here, we describe a unique case of MMS in a 30-year-old patient with SCD and incidental protein S deficiency. Our unique case highlights a patient at high risk for neurosurgical intervention due to her hypercoagulable state who has benefitted from medical management. We also discuss current literature for the prevention of secondary cerebral vascular events and the role of further studies involving adult populations with MMS and SCD.
烟雾病综合征(MMS)与镰状细胞病(SCD)之间的关联在儿科人群中已得到充分证实;然而,关于成年SCD患者中MMS的特征和管理的文献有限。研究表明血管内治疗在儿科人群继发性卒中预防中的作用,目前尚无针对成年人群的指南。在此,我们描述了一名30岁患有SCD且伴有蛋白S缺乏症的MMS独特病例。我们的独特病例突出了一名因高凝状态而有神经外科干预高风险的患者,她从药物治疗中获益。我们还讨论了预防继发性脑血管事件的当前文献以及涉及成年MMS和SCD患者的进一步研究的作用。