Background

To assess patients with indirect carotid-cavernous fistulas (CCF) for evidence of hypercoagulable state (HS) by combination of comprehensive medical questionnaire and laboratory testing.

Methods

Patients with confirmed diagnosis of CCF treated between 2003 and 2019 were included and administered a questionnaire screening for HS risk factors and undergone laboratory investigations which included complete blood count (CBC), prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody titres), Factor V Leiden, prothrombin, protein C, protein S, antithrombin III, homocysteine, prothrombin G20210, CALR and JAK2 mutation screening. Participants with abnormal laboratory testing and/or past history of ischemic stroke, atrial fibrillation, cancer or hypercoagulability-associated hereditary disorders were deemed to have HS.

Results

Twenty-two patients were enrolled. Seventeen were women and the mean age at diagnosis was 60. Fourteen (64%) had evidence of HS: six on medical history, three with laboratory evidence and five with both. Eight (36%) had current abnormal hypercoagulability markers. One had a diagnosis of Klippel–Trenaunay Syndrome, but no others had evidence of hereditary thrombophilia. Nine were on anti-coagulation initiated after diagnosis of stroke or atrial fibrillation discovered on average 5.5 years after the diagnosis of CCF.

Conclusion

A total of 64% percent of patients with previous indirect CCF had evidence of underlying HS indicating that hypercoagulability might play a role in the pathogenesis of CCF. The results support need for comprehensive testing for underlying HS in patients with indirect CCFs to better identify, manage, and prevent further thromboembolic events.

中文翻译：

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间接颈动脉海绵窦瘘患者的高凝状态

背景

通过综合医学问卷和实验室检测相结合，评估间接颈动脉海绵窦瘘（CCF）患者是否存在高凝状态（HS）的证据。

方法

纳入 2003 年至 2019 年间接受治疗的确诊为 CCF 的患者，对 HS 危险因素进行问卷筛查，并进行实验室检查，包括全血细胞计数 (CBC)、凝血酶原时间 (PT)、部分凝血活酶时间 (PTT)、纤维蛋白原、抗磷脂抗体（狼疮抗凝剂和抗心磷脂抗体滴度）、V因子Leiden、凝血酶原、蛋白C、蛋白S、抗凝血酶III、同型半胱氨酸、凝血酶原G20210、CALR和JAK2突变筛查。实验室检测异常和/或既往有缺血性中风、心房颤动、癌症或高凝相关遗传性疾病病史的参与者被视为患有 HS。

结果

共有 22 名患者入组。17 名女性，诊断时的平均年龄为 60 岁。14 名 (64%) 有 HS 证据：6 名有病史，3 名有实验室证据，5 名两者都有。八人 (36%) 目前存在异常高凝标志物。其中一名患者被诊断为 Klippel-Trenaunay 综合征，但没有其他人有遗传性血栓形成倾向的证据。9 名患者在诊断出中风或心房颤动后开始接受抗凝治疗，平均在 CCF 诊断后 5.5 年发现。

结论

总共 64% 的既往间接 CCF 患者有潜在 HS 的证据，表明高凝状态可能在 CCF 的发病机制中发挥作用。结果支持需要对间接 CCF 患者的潜在 HS 进行全面检测，以更好地识别、管理和预防进一步的血栓栓塞事件。