Abstract：
OBJECTIVE: To understand the etiology, work-up, and secondary systemic and ocular events of retinal artery occlusion (RAO) in young patients (≤ 45 years old) without typical cardiovascular risk factors.
METHODS: Retrospective longitudinal case series of 18 young patients with RAO and without typical cardiovascular risk factors evaluated at the University of Michigan Medicine Health System between the year 2000 and 2022. Laboratory and imaging studies performed at the time of RAO diagnosis, along with systemic and ocular events during follow-up, were recorded. These data were combined with data from a literature review of 74 similar patients experiencing a RAO.
RESULTS: Fifteen (83%) of patients were female and 10 (56%) suffered a branch retinal artery occlusion (BRAO). 56% of patients had one risk factor associated with cryptogenic stroke, most commonly a migraine history (33%). The most frequent etiology of RAO was vasculitis (28%), followed by idiopathic (22%) and patent foramen ovale (PFO, 17%). Three out of four patients with idiopathic RAOs developed new migraines around the time of RAO diagnosis, whereas none of the patients with a clear etiology had new onset migraines (n = 14). No patients suffered a stroke or myocardial infarction (MI) in the follow-up period (average 3.6 years ± 3.2 years). Two patients (11%) suffered a repeat RAO, both of whom were diagnosed with a vasculitis. Patients with isolated retinal vasculitis required repeat fluorescein angiograms for up to 2 years after the initial event to definitively identify the vasculitic etiology of the RAO. When our data are pooled with similarly healthy patients from previously published RAO series, structural/functional cardiac abnormalities and vasculitides are the most common identifiable etiologies for RAOs in this group.
CONCLUSIONS: The most common identifiable etiologies of RAO in young patients with low cardiovascular risk are structural/functional cardiac abnormalities and vasculitides, with a small range of additional causes/associations accounting for remaining cases. We suggest a focused work-up algorithm to rapidly identify etiologies in this group while minimizing unnecessary testing. The long-term risk of systemic or ocular secondary events in these patients is low regardless of the etiology of their RAO.
METHODS: Retrospective longitudinal case series of 18 young patients with RAO and without typical cardiovascular risk factors evaluated at the University of Michigan Medicine Health System between the year 2000 and 2022. Laboratory and imaging studies performed at the time of RAO diagnosis, along with systemic and ocular events during follow-up, were recorded. These data were combined with data from a literature review of 74 similar patients experiencing a RAO.
RESULTS: Fifteen (83%) of patients were female and 10 (56%) suffered a branch retinal artery occlusion (BRAO). 56% of patients had one risk factor associated with cryptogenic stroke, most commonly a migraine history (33%). The most frequent etiology of RAO was vasculitis (28%), followed by idiopathic (22%) and patent foramen ovale (PFO, 17%). Three out of four patients with idiopathic RAOs developed new migraines around the time of RAO diagnosis, whereas none of the patients with a clear etiology had new onset migraines (n = 14). No patients suffered a stroke or myocardial infarction (MI) in the follow-up period (average 3.6 years ± 3.2 years). Two patients (11%) suffered a repeat RAO, both of whom were diagnosed with a vasculitis. Patients with isolated retinal vasculitis required repeat fluorescein angiograms for up to 2 years after the initial event to definitively identify the vasculitic etiology of the RAO. When our data are pooled with similarly healthy patients from previously published RAO series, structural/functional cardiac abnormalities and vasculitides are the most common identifiable etiologies for RAOs in this group.
CONCLUSIONS: The most common identifiable etiologies of RAO in young patients with low cardiovascular risk are structural/functional cardiac abnormalities and vasculitides, with a small range of additional causes/associations accounting for remaining cases. We suggest a focused work-up algorithm to rapidly identify etiologies in this group while minimizing unnecessary testing. The long-term risk of systemic or ocular secondary events in these patients is low regardless of the etiology of their RAO.
摘要：
目的：了解病因，工作,以及无典型心血管危险因素的年轻患者（≤45岁）中视网膜动脉阻塞（RAO）的继发性全身和眼部事件。
方法：2000年至2022年在密歇根大学医学卫生系统评估的18名患有RAO且没有典型心血管危险因素的年轻患者的回顾性纵向病例系列。在RAO诊断时进行的实验室和影像学研究，随访期间的全身和眼部事件，被记录下来。这些数据与来自74例经历RAO的类似患者的文献综述的数据相结合。
结果：15例（83％）患者为女性，10例（56％）患有视网膜分支动脉阻塞（BRAO）。56%的患者有一个与隐源性卒中相关的危险因素，最常见的偏头痛病史（33%）。RAO最常见的病因是血管炎（28％），其次是特发性（22%）和卵圆孔未闭（PFO，17%）。特发性RAO患者中有四分之三在RAO诊断时出现了新的偏头痛，而没有明确病因的患者出现新发偏头痛（n=14）。随访期间（平均3.6年±3.2年），无患者发生中风或心肌梗死（MI）。两名患者（11％）反复出现RAO，两人都被诊断为血管炎。患有孤立性视网膜血管炎的患者在最初事件后需要重复荧光素血管造影长达2年，以明确确定RAO的血管病因。当我们的数据与以前发表的RAO系列中类似健康的患者合并时，结构/功能性心脏异常和血管炎是该组中最常见的可识别病因。
结论：在低心血管风险的年轻患者中，RAO最常见的可识别病因是结构性/功能性心脏异常和血管炎，与小范围的其他原因/关联占其余案件。我们建议采用集中的工作算法，以快速识别该组中的病因，同时最大程度地减少不必要的测试。这些患者的全身或眼部继发性事件的长期风险较低，无论其RAO的病因如何。
方法：2000年至2022年在密歇根大学医学卫生系统评估的18名患有RAO且没有典型心血管危险因素的年轻患者的回顾性纵向病例系列。在RAO诊断时进行的实验室和影像学研究，随访期间的全身和眼部事件，被记录下来。这些数据与来自74例经历RAO的类似患者的文献综述的数据相结合。
结果：15例（83％）患者为女性，10例（56％）患有视网膜分支动脉阻塞（BRAO）。56%的患者有一个与隐源性卒中相关的危险因素，最常见的偏头痛病史（33%）。RAO最常见的病因是血管炎（28％），其次是特发性（22%）和卵圆孔未闭（PFO，17%）。特发性RAO患者中有四分之三在RAO诊断时出现了新的偏头痛，而没有明确病因的患者出现新发偏头痛（n=14）。随访期间（平均3.6年±3.2年），无患者发生中风或心肌梗死（MI）。两名患者（11％）反复出现RAO，两人都被诊断为血管炎。患有孤立性视网膜血管炎的患者在最初事件后需要重复荧光素血管造影长达2年，以明确确定RAO的血管病因。当我们的数据与以前发表的RAO系列中类似健康的患者合并时，结构/功能性心脏异常和血管炎是该组中最常见的可识别病因。
结论：在低心血管风险的年轻患者中，RAO最常见的可识别病因是结构性/功能性心脏异常和血管炎，与小范围的其他原因/关联占其余案件。我们建议采用集中的工作算法，以快速识别该组中的病因，同时最大程度地减少不必要的测试。这些患者的全身或眼部继发性事件的长期风险较低，无论其RAO的病因如何。
