25例硬脊膜动静脉瘘患者的临床特征分析

Clinical Characteristics of 25 Cases Series with Spinal Dural Arteriovenous Fistula

目的分析硬脊膜动静脉瘘(spinal dural arteriovenous fistula,SDAVF)的临床特征,提高临床医师对SDAVF的认识,减少误诊、漏诊。方法回顾性收集2015年1月-2020年12月北京丰台右安门医院收治的SDAVF患者的病历资料,描述性分析其一般资料、临床表现、脑脊液检查、影像学特点、治疗及预后。结果共纳入25例患者,其中男性21例(84.0%),发病年龄40~75岁,中位发病年龄61岁。临床表现为运动障碍25例(100.0%),感觉障碍22例(88.0%),尿便功能障碍16例(64.0%),腰背部疼痛3例(12.0%),呼吸费力、构音障碍、吞咽困难2例(8.0%)。脑脊液检查示白细胞计数升高2例(18.2%,2/11),蛋白水平升高8例(72.7%,8/11)。MRI均表现为长节段脊髓病变,19例(76.0%)位于胸腰段,2例(8.0%)位于骶段,4例(16.0%)位于颈段及颅颈交界区。19例(76.0%,19/25)髓周可见血管流空影,11例(61.1%,11/18)髓质不规则片状强化或伴脊髓表面线样强化,其中5例(45.5%,5/11)呈现“断层征”。CTA检查的瘘口检出率为93.3%(14/15),供血动脉检出率为80.0%(12/15)。DSA检查示23例(92.0%)为单瘘口,2例(8.0%)为双瘘口。所有患者均行瘘口切除术或血管内介入栓塞治疗,中位随访5(3~6)个月,4例(16.0%)失访,完成随访的21例患者采用改良Aminoff-Logue评分评价预后,其中17例(81.0%)改善,3例(14.3%)无缓解,1例(4.8%)恶化。结论SDAVF多发生于脊髓胸腰段,以进行性运动、感觉及尿便功能障碍为主要临床表现,颅颈交界区SDAVF可出现脑干功能障碍甚至危及生命。脑脊液检查可呈类炎症反应,脊髓增强MR“I断层征”有助于SDAVF的早期识别,CTA检查可作为SDAVF的常规筛查手段。SDAVF容易误诊,早期诊治可改善预后。

Objective To analyze the clinical characteristics of spinal dural arteriovenous fistula(SDAVF).Methods The clinical data of patients with SDAVF admitted to Beijing Fengtai You'anmen Hospital from January 2015 to December 2020 were collected and retrospectively analyzed.The general information,clinical manifestations,cerebrospinal fluid(CSF)examination,imaging features,treatment and prognosis were analyzed.The prognosis was evaluated using modified Aminoff and Logue score(ALS).Results A total of 25 eligible patients were included,with 21 males(84.0%)and a median age of 61 years(range:40-75 years).The clinical manifestations included 25 cases(100.0%)of motor dysfunction,22 cases(88.0%)of sensory dysfunction,16 cases(64.0%)of urination and defecation dysfunction,3 cases(12.0%)of back pain,2 cases(8.0%)of dyspnea,dysarthria and dysphagia.CSF leukocytes was increased in 2/11(18.2%)cases,and the protein was increased in 8/11(72.7%)cases.MRI showed long-segment lesions,which were located in thoracolumbar segment in 19cases(76.0%),in sacral segment in 2 cases(8.0%),in cervical and craniocervical junction in 4 cases(16.0%).Flow void phenomena was found in 19/25(76.0%)cases.Patchy or accompanied with linear enhancement was found in 11/18(61.1%)cases,5 of whom(45.5%)showed"piecemissing sign".The detection rate of fistula on CTA was 93.3%(14/15),and that of feeding artery was 80.0%(12/15).DSA showed single fistula in 23 cases(92.0%)and two fistulas in 2 cases(8.0%).All patients underwent fistulectomy or intravascular embolization.During a median follow-up of 5 months(range:3-6 months),4 cases(16.0%)were lost to follow-up.For 21 followed-up cases,17 cases(81.0%)relieved,3 cases(14.3%)did not improve,1 case(4.8%)died.Conclusions SDAVF mostly occurred in thoracolumbar spinal cord,with the main clinical manifestations of progressive motor,sensory,urination and defecation dysfunction.SDAVF at the craniocervical junction can cause brain stem dysfunction,even life-threatening.The changes of CSF can be similar to inflammation.The"piece-missing sign"on contrast-enhanced MRI is helpful to the early identification of SDAVF.CTA can be used as a tool for screening SDAVF.The misdiagnosis rate of SDAVF is high.Early diagnosis and treatment can improve the prognosis.