BACKGROUND Moyamoya Disease (MMD) and moyamoya Syndrome (MMS) have been reported to be associated with pro-thrombotic states in some patients. To date, however, such reports have been limited to case reports or small case series. We sought to determine the prevalence of pro-thrombotic states among a large cohort of both MMD and MMS patients. METHODS We retrospectively reviewed the medical records of all adult patients who were diagnosed with MMD or MMS from our institution. In addition to basic demographic and clinical data, the presence of certain pro-thrombotic conditions was noted. The presence of such conditions was determined based on review of clinical diagnoses and laboratory reports. The length of follow-up and the presence of interval ischemic events were obtained. RESULTS Out of a total of 180 patients diagnosed with MMD or MMS, 33 were found to have a concomitant pro-thrombotic condition (18.3%). Of 147 patients with MMD, 23 were found to have a pro-thrombotic condition (15.6%). There were 10 out of 33 total patients (30.3%) in the MMS cohort that had a concomitant pro-thrombotic condition. There were no differences in specific pro-thrombotic conditions between MMD and MMS cohorts. There were no differences in future ischemic events between moyamoya patients with pro-thrombotic tendencies compared to those without (13.3% versus 23.7%, respectively, P = 0.32), and this was also true for patients who underwent revascularization procedures (22.6% versus 29.4%, P = 0.76). There was also no difference in prevalence of revascularization graft occlusion between moyamoya patients with pro-thrombotic tendencies and those without at follow-up (1.2% versus 5.9%, P = 0.31). CONCLUSIONS It is likely that both MMD and MMS are associated with or predispose to pro-thrombotic conditions in adult patients, although the clinical and surgical significance is uncertain. Further study is needed in order to further elucidate and characterize this potential association.

中文翻译：

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成人烟雾病和烟雾综合症患者的血栓形成前病患高发：单中心研究。

背景技术据报道，在一些患者中，烟雾病（MMD）和烟雾病综合症（MMS）与血栓形成前状态有关。但是，迄今为止，此类报告仅限于案例报告或小案例系列。我们试图确定大量MMD和MMS患者的血栓形成前状态的患病率。方法我们回顾性研究了我院诊断为MMD或MMS的所有成年患者的病历。除了基本的人口统计学和临床​​数据，还注意到存在某些促血栓形成情况。根据对临床诊断和实验室报告的审查，确定是否存在此类疾病。获得随访的时间和间隔性缺血事件的存在。结果在总共180名被诊断为MMD或MMS的患者中，发现33例患者同时有血栓形成前症状（18.3％）。在147名MMD患者中，发现有23名具有血栓形成前症状（15.6％）。在MMS队列中，共有33例患者中有10例伴有血栓形成前状态。MMD和MMS队列之间在特定的血栓形成前状况无差异。有血栓形成倾向的moyamoya患者与没有血栓形成倾向的moyamoya患者之间的未来缺血事件没有差异（分别为13.3％和23.7％，P = 0.32），接受血运重建手术的患者也是如此（22.6％和29.4） ％，P = 0.76）。在有血栓形成倾向的moyamoya患者和没有随访的moyamoya患者之间，血运重建移植物阻塞的发生率也没有差异（1.2％vs 5.9％，P = 0.31）。结论尽管临床和手术意义尚不确定，但MMD和MMS都可能与成年患者的血栓形成相关或易患。为了进一步阐明和表征这种潜在的关联，需要进一步的研究。