2例罕见硬脊膜外动静脉瘘的诊治及误诊原因分析

目的 探讨硬脊膜外动静脉瘘的诊治方法，分析误诊原因和对策。方法 回顾性分析2017年9月至2018年12月收治的2例罕见硬脊膜外动静脉瘘的临床资料，总结临床特点，并分析其误诊原因。结果 2例均表现为进行性双下肢乏力伴大小便功能障碍，外院DSA无法确诊而转入我院。我们结合神经系统体征定位诊断，在病变定位部位采用延长脊髓血管造影时间的方法发现胸5节段硬脊膜外动静脉瘘，伴硬脊膜外静脉湖形成；运用介入栓塞供血动脉和瘘口，再行开放性手术灼闭硬脊膜外静脉湖；1例治愈，另1例因误诊2年余仅部分神经功能恢复。结论 硬脊膜外动静脉瘘是一种临床罕见的脊髓血管病，以椎管内静脉高压或脊髓压迫的形式造成脊髓功能障碍，因为临床表现缺乏特异性，容易误诊和漏诊；可通过结合神经系统体征定位、延长选择性脊髓动脉造影时间或进行脊髓主要供血动脉无关血管的造影，可提高确诊率；介入治疗和复合手术均能达到良好治疗效果，临床症状的改善程度与确诊时间呈负相关。

Treatment and diagnosis of spinal epidural arteriovenous fistula: report of 2 cases and review of literature

Objective To investigate the clinical characteristics, diagnosis and treatment of spinal epidural arteriovenous fistula (SEDAVF), and to analyze the the causes and countermeasures of misdiagnosis of rare SEDAVF. Methods The clinical data of 2 patients with SEDAVF who received interventional and surgical treatment in our department from September 2017 to December 2018 were retrospectively analyzed. The literatures related to SEDAVF were reviewed. Results Both 2 patients showed progressive weakness of lower extremities with defecation dysfunction, but they did not be difinitely diagnosed by DSA in external hospital and then were transferred to our hospital. Based on the neural location diagnosis, we found SEDAVF in the 5th thoracic segment of spine with the formation of epidural vein lake by prolonging the time of spinal cord angiography. Both 2 patients were treated using interventional embolization of the blood supply arteries and fistulas combined with operation to occlude the extradural vein lake. One patient was cured, and the other was only partially recovered due to the misdiagnosis for more than 2 years. Conclusions The SEDAVF is a rare spinal vascular disease which results in myelopathy by intraspinal venous hypertension or compression of spinal cord. The clinical manifestation of SEDAVF lacks specificity, resulting in misdiagnosis and missed diagnosis in clinc. The extension of exposure time during DSA or selective angiography of apparently irrelative artery combined with clinical symptoms may improve the accuracy of diagnosis. The fistula can be eliminated by interventional or hybrid surgery. The clinical symptom improvement rates are negatively correlated with the duration between the symptom onset and difinite diagnosis.