幕下硬脑膜动静脉瘘三例临床分析

Clinical analysis of three cases of infratentorial dural arteriovenous fistula

目的:提高临床对幕下硬脑膜动静脉瘘(DAVF)的认识。方法:对2017年9月至2019年9月苏州大学附属第一医院收治的3例DAVF患者进行回顾性分析,包括临床特征、脑脊液检查、头颅影像学及治疗等,并电话随访预后。结果:例1为女性,43岁,慢性病程,急性加重,临床表现为双下肢乏力及大小便障碍,头颅磁共振成像(MRI)可见延髓异常信号,脑脊液水通道蛋白4抗体阳性,以“视神经脊髓炎谱系病”治疗无效,后行数字减影血管造影(DSA)检查示左侧小脑幕下动静脉瘘,给予血管内栓塞治疗,随访2年病情无复发。例2为女性,57岁,慢性进展性病程,主要表现为记忆力下降,后出现吞咽困难、高热以及意识障碍,以“中枢神经系统感染”治疗未见好转,后行DSA示双侧横窦-乙状窦区动静脉瘘,给予血管内栓塞治疗,栓塞部分瘘口,术后2个月患者因肺部感染死亡。例3为男性,52岁,亚急性病程,主要表现为顽固性恶心呕吐,多次于消化科治疗,头颅MRI示延髓斑片状异常信号,其中可见血管流空影,完善DSA检查,示颅颈交界区动静脉瘘,给予血管内栓塞治疗,术后半年未见复发。结论:DAVF是一组异质性较大的疾病,幕下DAVF可表现为脑干、小脑、脊髓受累的急性神经功能障碍,临床极易误诊,MRI可见颅内异常信号,对这类患者要考虑到DAVF的可能,及时行DSA检查是确诊的关键。血管内栓塞治疗是目前主要的治疗手段,预后取决于临床表现及瘘管分级。

Objective To improve awareness about infratentorial dural arteriovenous fistula(DAVF).Methods Three cases of DAVF in the First Affiliated Hospital of Soochow University from September 2017 to September 2019 were retrospectively analyzed in terms of clinical features,cerebrospinal fluid(CSF)analysis,brain imaging and treatment,and followed up through telephone call.Results Case 1:A 43-year-old woman,in chronic but acute aggravated course,presented with weakness of both lower limbs and urination and defecation dysfunction.Brain magnetic resonance imaging(MRI)revealed abnormal signal in medulla.CSF analysis demonstrated aquaporin-4 antibodies positive.Misdiagnosed as neuromyelitis optica spectrum disorders,the treatment was poor.Then digital subtraction angiography(DSA)showed DAVF at the left infratentorial area,and endovascular treatment was operated.Relapse was not observed in two-year follow up.Case 2:A 57-year-old woman,in chronic progressive course,mainly manifested as memory loss,but progressed with dysphagia,fever,coma.Treatment as“central nervous infection”was poor.Then DSA showed DAVF at the bilateral transverse-sigmoid sinus area,and endovascular treatment was operated with embolized partial fistulas.The patient died from lung infection within two months.Case 3:A 52-year-old man,in subacute course,was treated in the Gastroenterology Department with clinical manifestion of stubborn nausea and vomiting.Brain MRI revealed abnormal signal in medulla,with prominent vessel flow voids nearby.Then DSA showed DAVF at the craniocervical junction,and endovascular treatment was operated.Relapse was not observed in six-month follow up.Conclusions DAVF has a variety of clinical manifestations,and infratentorial DAVF can manifest as acute neurological dysfunction involving the brain stem,cerebellum,spinal cord,which may be easily misdiagnosed.When brain MRI showed intracranial abnormal signal,the possibility of DAVF should be considered.DSA remains the gold standard to diagnose DAVF.Endovascular embolization is the main treatment of infratentorial DAVF at present.Prognosis depends on clinical presentation and fistula classification.