Clinical presentation,imaging findings,and prognosis of spinal dural arteriovenous fistula

Spinal dural arteriovenous fistula (SDAVF) is a relatively common acquired vascular malformation of the spinal cord. Assessment of a SDAVF is often difficult because of non-specific findings on non-invasive imaging modalities. Diagnosis of a SDAVF is often delayed, and some patients receive unnecessary treatment and treatment delays, often resulting in a poor outcome. The aim of this study was to characterize the clinical presentation, typical imaging findings, and long-term outcome of SDAVF. Forty patients (13 women, 27 men; mean age 58.18 ± standard deviation 14.75 years) who were treated at our hospital from June 1992 to March 2014 were retrospectively reviewed. We investigated the baseline characteristics, clinical presentation, imaging findings, treatment modalities, and outcome of the patients. The most common clinical presentation was a sensory symptom (80%), followed by motor weakness (70%), and sphincter dysfunction (62.5%). Roughly one-third (32.5%) of patients had a stepwise progression of fluctuating weakness and sensory symptoms, but the most common presentation was chronic progressive myelopathic symptoms (47.5%). Thirty-four patients (85%) had T2 signal change on the spinal cord MRI, indicative of cord edema. Thirty-eight patients had typical perimedullary vessel flow voids on T2-weighted MRI. Twenty-eight patients were treated with endovascular embolization, five patients underwent surgery, and four patients underwent both. Clinical outcome was determined by severity of initial deficit (p = 0.008), extent of cord edema (p = 0.010), treatment failure (p = 0.004), and a residual fistula (p = 0.017). SDAVF causes a treatable myelopathy, so early diagnosis and intervention is essential.     

Long-term surgical outcomes of patients with delayed diagnosis of spinal dural arteriovenous fistula

Spinal dural arteriovenous fistula (dAVF) is an extremely rare vascular entity that is usually misdiagnosed. We sought to determine the long-term clinical outcomes of patients undergoing microsurgical treatment for delayed diagnosis of spinal dAVF. This retrospective study identified patients with delayed diagnosed spinal dAVF at our institution from 2009 to 2018. Patients’ data, including demographics, imaging, and follow-up data, were evaluated. This cohort included 65 consecutive patients with 68 dAVFs and a male-to-female ratio of 4:1 and a mean age of 53.5 ± 13.7 years. The presenting symptoms consisted of limb weakness (n = 42, 64.6%), paraparesis (n = 34, 52.3%), sphincter disturbances (n = 8, 12.3%), and pain (n = 13, 20.0%). The proportion of patients with each symptom significantly increased and patients experienced increased disability when the diagnosis was finalized. The mean length of delay of diagnosis was 20.7 ± 30.0 months. Surgery resulted in complete occlusion of the fistula on the first attempt in all patients. Three patients developed recurrent fistulas, and three died in the follow-up period. Improved motor function was achieved in 38 patients (59.5%). Other symptoms, such as sensory disorders, sphincter dysfunction, and pain, improved by 37.3%, 32.3%, and 66.7%, respectively. Patients with spinal dAVF usually exhibit progressive ascending myelopathy and often remain misdiagnosed for months to years. Some patients’ increased disability cannot be reversed through surgery.     

应用复合手术平台治疗硬脊膜动静脉瘘

目的探讨复合手术平台在硬脊膜动静脉瘘(SDAVF)治疗中的应用价值。方法回顾性分析2018年1月至2019年10月复合手术治疗的18例SDAVF的临床资料。结果 18例手术时间3.1～4.6 h,平均(3.5±0.8)h。术后即刻造影未发现瘘口及异常引流静脉。无手术死亡病例,未发生与造影相关并发症。术后3个月复查脊髓造影均无复发,18例症状均明显改善;术后1年,造影复查未发现复发。结论利用复合手术平台治疗SDAVF,定位准确,手术损伤小,复发率低,疗效好。     

硬脊膜动静脉瘘误诊分析

目的 探讨如何避免误诊硬脊膜动静脉瘘。方法 回顾分析3例误诊的硬脊膜动静脉瘘的临床表现，误诊原因及治疗结果。结果 1例误诊为腰椎间盘突出，2例误诊为前列腺肥大，不仅延误了诊断，还因误治加重了神经功能损害或导致正常器官损害。结论 仔细询问病史和查体，适时行脊髓MRI检查是避免误诊的关键。     

硬脊膜动静脉瘘的手术治疗

目的探讨硬脊膜动静脉瘘的诊断及总结手术治疗的经验。方法回顾性分析13例经脊髓血管造影确诊的硬脊膜动静脉瘘患者的临床资料，均行手术夹闭瘘口，其中经全椎板切除入路9例，经半椎板切除入路4例。结果瘘口位于上胸段2例，中胸段3例，下胸段6例，腰段2例。全部病例手术后行脊髓血管造影复查，均未见异常瘘口及迂曲引流静脉。随访2—36个月，13例中症状基本消失、痊愈5例，症状改善、好转7例，无变化1例。结论脊髓血管造影可以准确定位瘘口位置，是诊断硬脊膜动静瘘的金标准。手术夹闭瘘口方法简单，夹闭瘘口确切可靠，效果肯定，可作为硬脊膜动静脉瘘的首选治疗方法。     

Primary conus medullaris arteriovenous shunt and secondary lumbo-sacral epidural arteriovenous fistula: One malformation can hide another

We report an anatomical-based association between conus medullaris pial arteriovenous shunt that drain caudally towards the lumbosacral area with very delayed onset of an acquired lumbar epidural shunt, draining secondarily towards intradural veins and responsible for a venous congestive myelopathy with identical clinical symptoms. These patients require close clinical and imaging follow-ups in order to propose adequate treatments before onset of irreversible neurological deficits. MRA should include the lumbo-sacral area in its field of view.     

硬脊膜动静脉瘘治疗预后相关因素分析——一种新的手术预后评分系统

目的硬脊膜动静脉瘘(Spinal dural arteriovenous fistulas,SDAVFs)患者预后因素分析。方法通过对中国医科大学附属第一医院2012年1月至2017年12月间收治的41例SDAVFs患者的流行病学特征、影像学特点、治疗方法及随访结果等资料进行回顾性分析。分为手术治疗组30例(SDAVFs切断术,不包括SDAVFs栓塞术)及非手术治疗组11例(拒绝手术,选择药物或物理治疗者),运用Aminoff and Logue评分(Aminoff and Logue Score,ALS)量化脊髓功能情况,分析SDAVFs患者治疗预后的相关因素。结果手术治疗组影像学治愈率100%,临床症状改善率36.7%(11/30),症状无变化者63.3%(19/30),临床症状加重者0%(0/30);非手术治疗组,临床症状改善率0%(0/11),症状无变化者27.3%(3/11),临床症状加重者72.7%(8/11);手术治疗能够明显改善SDAVFs患者功能预后(P=0.017);而患者治疗时年龄(P=0.020)、患病时间(P=0.037)、迂曲扩张的引流静脉长度(P=0.020)及患病当时临床症状严重程度(P=0.035)与患者手术预后有统计学意义,并提出全新的SDAVFs手术预后评分系统,7～8分为术后临床症状改善率高,而4～6分为术后临床症状改善不明显(P=0.000)。结论 SDAVFs切断术影像学治愈率极高,且相对于非手术治疗组的临床症状改善率亦较明显;根据相关预后因素(年龄、患病时间、迂曲扩张的引流静脉长度及患病当时的临床症状严重程度)提出的硬脊膜动静脉瘘手术预后评分系统,可用来评估预后。     

Double spinal dural arteriovenous fistulas

Background

Spinal dural arteriovenous fistulas (SDAVF) are usually solitary lesions. Synchronous and/or metachronous double SDAVF have rarely been reported in the literature. We report on three patients with double SDAVF and present our single center experience in the diagnostic and treatment management in these patients.

硬脊膜动静脉瘘的临床诊断与显微手术治疗分析

目的分析和总结硬脊膜动静脉瘘的临床诊断及显微手术切除治疗经验。 方法回顾性分析2010年1月至2014年1月在平煤神马医疗集团总医院确诊并行显微手术切除治疗的硬脊膜动静脉瘘患者8例,并从临床症状、影像学资料及显微手术切除治疗效果总结了临床诊断及治疗的经验。 结果所有病例均经半椎板入路行动静脉瘘显微手术切除术,术后复查脊髓血管造影,均未发现异常的瘘口及迂曲扩张的引流静脉显影。随访24个月,其中痊愈5例,好转2例,有效率87.5%。 结论MRI是筛选硬脊膜动静脉瘘的无创手段,脊髓血管造影是确定诊断的金标准。显微手术方法简单,疗效确切,可作为治疗硬脊膜动静脉瘘的首选方法。     

硬脊膜动静脉瘘治疗预后相关因素分析——一种新的手术预后评分系统

目的 硬脊膜动静脉瘘（Spinal dural arteriovenous fistulas，SDAVFs）患者预后因素分析。方法 通过对中国医科大学附属第一医院2012年1月至2017年12月间收治的41例SDAVFs患者的流行病学特征、影像学特点、治疗方法及随访结果等资料进行回顾性分析。分为手术治疗组30例（SDAVFs切断术，不包括SDAVFs栓塞术）及非手术治疗组11例（拒绝手术，选择药物或物理治疗者），运用Aminoff and Logue评分（Aminoff and Logue Score，ALS）量化脊髓功能情况，分析SDAVFs患者治疗预后的相关因素。结果 手术治疗组影像学治愈率100%，临床症状改善率36.7%（11/30），症状无变化者63.3%（19/30），临床症状加重者0%（0/30）；非手术治疗组，临床症状改善率0%（0/11），症状无变化者27.3%（3/11），临床症状加重者72.7%（8/11）；手术治疗能够明显改善SDAVFs患者功能预后（P=0.017）；而患者治疗时年龄（P=0.020）、患病时间（P=0.037）、迂曲扩张的引流静脉长度（P=0.020）及患病当时临床症状严重程度（P=0.035）与患者手术预后有统计学意义，并提出全新的SDAVFs手术预后评分系统，7~8分为术后临床症状改善率高，而4~6分为术后临床症状改善不明显（P=0.000）。结论 SDAVFs切断术影像学治愈率极高，且相对于非手术治疗组的临床症状改善率亦较明显；根据相关预后因素（年龄、患病时间、迂曲扩张的引流静脉长度及患病当时的临床症状严重程度）提出的硬脊膜动静脉瘘手术预后评分系统，可用来评估预后。     

硬脊膜动静脉瘘的早期诊断策略

目的硬脊膜动静脉瘘（SDAVF）是一种缓慢进展的脊髓血管性疾病,临床表现无特殊性,容易误诊。现分析3例得到早期诊断病例的资料,以期制定SDAVF早期诊断的策略。方法对近年来得到早期诊断的3例SDAVF患者诊断资料进行分析,总结其临床表现特征、诊断程序及内容。结果3例SDAVF患者均表现为双下肢无力或麻木,运动后加重等特点。磁共振检查出现脊髓周边血管流空影及局部脊髓水肿均高度提示SDAVF,均通过DSA获得确诊。结论对于临床表现疑似SDAVF患者,脊髓MRI扫描和进一步的DSA检查有助于早期诊断,提高治疗效果。     

复合手术治疗硬脊膜动静脉瘘31例分析

目的 探讨复合手术治疗硬脊膜动静脉瘘（SDAVF）的疗效。方法 回顾性分析2014年8月至2019年7月复合手术治疗的31例SDAVF的临床资料。术中DSA技术定位瘘口,据造影结果调整动脉夹,并验证瘘口闭塞的安全性和准确性。术后平均随访（28.9±2.7）个月,采用Aminoff-Logue评分（ALS）评价脊髓功能。结果 31例瘘口位置均准确定位,无扩大切口以暴露病灶,术后造影显示瘘口均消失。末次随访ALS[（3.3±0.5）分]较术前[（4.4±0.5）分]明显降低（P<0.05）;好转14例,无变化4例,加重2例,好转率为66.7%。随访期间无复发病例。结论 复合手术SDAVF安全、有效,与血管内栓塞相比,解决了其早期失败率及晚期复发率高的问题;与显微手术相比,实现了术中精准定位及实时效果评价。     

Long-term follow-up and outcome in patients treated for spinal dural arteriovenous fistula

Clinical outcome was examined in 21 patients treated for spinal dural arteriovenous fistula after 5–50 months. We compared the neurological condition (motor function, pain, sensory disturbance, vegetative dysfunction) and Barthel index before and after fistula occlusion. Neurological impairment was assessed as improved, unchanged, or deteriorated. All patients initially showed paraparesis of varying degree, and a sensory loss with a defined level in 81% before treatment. The greatest postoperative change that we measured was in motor activity (67% improved), and the most important deterioration was in male potency (28% deteriorated). We observed unchanged or absent symptoms in pain in 71% of our patients. We were thus able not only to stop but even to reverse the progression of symptoms and the degree of disability. On average, neurological symptoms stabilized within 1 year. The results of this study confirm that spinal dural arteriovenous fistula should be treated as early as possible after diagnosis. Received: 9 October 1997 Received in revised form: 17 July 1998 Accepted: 24 July 1998     

硬脊膜动静脉瘘五例误诊分析

目的探讨硬脊膜动静脉瘘的临床特点及误诊原因。方法分析5例确诊的硬脊膜动静脉瘘的临床、MRI及DSA表现。结果 5例中仅1例急性起病,表现为一侧肢体无力,误诊为急性脑梗死;余4例均慢性病程,表现为双下肢无力和麻木,部分伴有尿频和便秘。其中2例误诊为腰椎间盘突出,2例误诊为多发性硬化,1例误诊为脊髓炎,并予激素治疗后症状无明显好转,仍进行性加重。结论本病早期临床表现无特异性,容易误诊,脊髓MRI为首选,及时行此检查是避免误诊的关键;选择性的脊髓血管造影是诊断本病的金标准。     

硬脊膜动静脉瘘的显微手术与血管内栓塞治疗

目的 分析硬脊膜动静脉瘘的治疗效果,探讨其治疗方法.方法 回顾性分析近3年来收治的11例硬脊膜动静脉瘘患者的临床资料、治疗方式及术后转归.结果 11例患者中男10例,女1例.年龄33 ～ 67岁,平均53岁.所有患者均在脊髓血管造影后得到确诊.其中单纯手术治疗9例,栓塞治疗2例;所有患者术后症状得到不同程度的改善,改良Aminoff-Logue功能评分降低.11例随访6-24个月,未见复发症状.结论 显微手术和血管内栓塞治疗硬脊膜动静脉瘘可以达到良好效果,部分病例采用血管内治疗须栓塞引流静脉近端.     

Venous congestive myelopathy of the cervical spinal cord: An autopsy case showing a rapidly progressive clinical course

We report a rapidly progressive myelopathy in a 74‐year‐old Japanese man who was admitted to our hospital with a 4‐month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord. At autopsy, ischemic changes were observed in the upper‐to‐middle cervical cord segments, with so‐called arterialized veins in the subarachnoid space. No neoplastic lesions were found within or outside the brain and spinal cord. These pathological findings were essentially those of venous congestive myelopathy (VCM) associated with dural arteriovenous fistulae (AVF), formerly known as Foix–Alajouanine syndrome. VCM associated with dural AVF, which is now considered to be treatable in the early stages, is rare found in the cervical spinal cord. The present autopsy case, with MRI findings, provides further information that might be useful for recognition and diagnosis.     

表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘

目的对表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘发病机制进行分析,预测出血因素,避免漏诊,指导治疗。方法对5例表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘的临床资料进行总结。结果5例瘘口均位于枕大孔区～颈2节段,由椎动脉脊膜支供血．通过髓周静脉引流。所有5例引流静脉均向颅内引流,有不同程度扩张,3例伴有静脉瘤样改变。手术后复合瘘口均消火,4例治愈,1例死亡。结论高颈段硬脊膜动静脉瘘向颅内引流并伴有引流静脉扩张易出血,颅内压增高可能是诱发出血的因素。血管造影应全面,避免小部分蛛网膜下腔出血的患者造影假阴性。手术夹闭瘘口并切断近端引流静脉是可靠的治疗方法。     

Dual-lumen balloon to increase onyx venous penetration in the treatment of spinal dural arteriovenous fistulas

Purpose

Spinal dural arteriovenous fistulas (sDAVF) are the most common spinal vascular lesions. The arterialization of the recipient vein results in venous hypertension and chronic ischemia. Intravascular injection of acrylic glue in order to occlude the draining vein is the principle of endovascular treatment, but a significant portion of embolization procedures do not succeed. We present our initial experience of endovascular balloon augmented embolization of sDAVF using a dual-lumen balloon.

硬脊膜动静脉瘘血管内栓塞治疗效果评估

目的评价经动脉入路血管内栓塞治疗硬脊膜动静脉瘘（sDAvFs）的临床疗效。方法回顾性分析近6年来我中心采用经动脉栓塞治疗的24例SDAVFs患者的临床资料。结果术后造影显示79．16％（19／24）的患者实现完全闭塞。19例治愈患者临床随访1～12个月,症状改善（Aminoff-Logue评分降低1分以上）9例（47．05％）,稳定（Aminoff-Logue评分不变）7例（36．8％）,进展（Aminoff-Logue评分降提高1分以上）3例（15．7％）;其中16例术后6。12月复查脊髓血管造影均未见SDAVFs复发。结论经动脉入路血管内栓塞治疗SDAVFs是安全、可行、有效的方法。     

经静脉途径治疗海绵窦区硬脑膜动静脉瘘

目的 探讨经静脉途径栓塞治疗海绵窦区硬脑膜动静脉瘘的疗效. 方法 经全脑血管造影(DSA)确诊海绵窦区硬脑膜动静脉瘘9例.均经静脉途径予以栓塞治疗. 结果 本组9例中治愈8例,症状好转1例,无加重和死亡.随访2个月～3年,无复发及加重者. 结论 静脉途径栓塞治疗硬脑膜动静脉瘘是一种安全、有效的方法 .