Clinical characteristics of dural arteriovenous fistula.

Intracranial dural arteriovenous fistula (DAVF) is an uncommon neurosurgical condition; in particular, it has been infrequently reported in Korea. To understand the general clinical characteristics of DAVFs, the authors reviewed 53 cases and analyzed factors affecting DAVF hemorrhage of and treatment outcome. Since 1980 we have encountered 480 pial and 53 DAVFs, a ratio of 9.1 to 1. The age of these patients ranged from 1 month to 71 years, the most common being in the 6th decade, and females exceeded males by 1.65 to 1. All lesions except three were single, and symptoms were related to location and the venous drainage pattern. The most common location was the cavernous sinus, accounting for about 64% of cases, with the result that the most common clinical symptoms of DAVFs were ocular, namely proptosis and chemosis. The next was tinnitus also found in transverse-sigmoid sinus DAVFs. Intracranial hemorrhage was seen in eight cases,(15%) the primary cause of hemorrhage was retrograde intracranial venous drainage (P=0.017), and one hemorrhage was observed in cases with no intracranial venous drainage. Intracranial hemorrhage was more frequently in transverse-sigmoid than cavernous sinus DAVFs (P=0.049), and this proved to be so even where there was intracranial venous drainage. However, two of 34 patients with cavernous DAVFs became blind in one eye, demonstrating that in such patients, the clinical course could be aggressive. Thirteen patients were treated conservatively. The conservative treatment group was comprised of 13 patients, two of three patients with transverse-sigmoid sinus DAVF expired, and 7 of 10 with cavernous sinus DAVF experienced a clinical improvement or cure. Surgical excision was performed in only two patients. A total of 39 patients underwent embolization; clinical cure was achieved in 13, improvement of symptoms in 12, an unchanged or aggravated result occurred in 9, one died, and four were lost to follow up. During intervention, there was one hemorrhagic complication, owing to obstruction of the venous outflow with embolic materials.In this study, the most common location of DAVFs was the cavernous sinus. The cortical venous drainage remains the primary determinant of intracranial hemorrhage. Common indications for treatment include hemorrhage and neurological deficit. Endovascular treatment is preferred in the majority of cases except tentorial DAVF. The goal of embolization in cavernous DAVF is the alleviation of symptoms, not angiographic cure. But transverse-sigmoid sinus DAVF with venous restriction and leptomeningeal drainage should be treated aggressively.     

硬脑膜动静脉瘘侵袭性表现的危险因素分析

目的目前除了合并皮层静脉引流被认为是硬脑膜动静脉瘘(DAVF)的侵袭性表现的高危因素之外,尚无其他危险因素被大家所公认。本研究的目的在于通过单因素和多因素分析,阐明DAVF侵袭性表现的危险因素。方法回顾分析2007年1月至2012年12月第二军医大学长海医院临床神经医学中心收治的190例DAVF患者。其中侵袭性表现112例,非侵袭性表现78例。收集临床和影像学资料,提取人口学特征、临床表现类型、血管构筑学特征等参数,对比不同表现DAVF在上述参数上的差异,并进行Logistic回归分析。结果 Logistic回归分析发现侵袭性表现发病的独立危险因素为瘘口部位位于大静脉窦区(P0.001),引流方式为Borden II型(P0.001)和III型(P=0.002)。结论瘘口位于大静脉窦区,引流方式为Borden II型和III型是DAVF发生侵袭性表现的独立危险因素。     

硬脑膜动静脉瘘的治疗分析（附15例报告）

目的探讨硬脑膜动静脉瘘的治疗方法及其效果。方法回顾性分析2010年9月到2012年9月收治的15例硬脑膜动静脉瘘病人的临床资料。手术治疗4例,血管内治疗7例,伽玛刀治疗2例,保守治疗2例。结果手术治疗的4例患者均恢复良好。血管内治疗的7例患者中,5例术后即刻行造影显示硬脑膜动静脉瘘消失,未发生并发症;另2例栓塞后有少量残留,通过压迫供血的颈总动脉治愈。伽玛刀治疗2例,随访1年症状好转。保守治疗的2例患者,通过压迫供血的颈总动脉,其中1例成功治愈,另1例好转。15例患者出院后随访6～30个月,7例血管内治疗患者中2例复发,1例再次栓塞后治愈,1例通过压迫供血的颈总动脉及伽玛刀治疗后好转;随访期间15例患者均无神经功能缺失表现。结论硬脑膜动静脉瘘应针对其部位及类型,选择相应的治疗方法;血管内治疗安全、有效,是其首选方法。     

Bilateral ethmoidal dural arteriovenous fistula: Unexpected surgical diagnosis

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.     

经动脉途径应用Onyx胶栓塞治疗硬脑膜动静脉瘘(附16例报告)

目的探讨经动脉途径应用Onyx胶栓塞治疗颅内硬脑膜动静脉瘘(DAVF)的可行性及疗效。方法回顾性分析2007年3月至2010年10月收治的16例DAVF患者的临床资料及随访结果。经动脉途径采用Onyx胶栓塞治疗15例,动静脉联合途径1例。结果 16例患者共栓塞17次,栓塞结束即时解剖治愈14例(87.5%),另2例栓塞不全者术后辅以手术治疗。栓塞后出现动眼神经麻痹3例,面部感觉麻木3例,随访1月后症状消失;口咽部疼痛2例,应用糖皮质激素治疗后症状消失。本组无死亡病例。10例患者术后2～24个月复查DSA,1例DAVF复发,9例完全闭塞;6例门诊随访2～24个月均未见复发。结论经动脉途径应用Onyx胶栓塞治疗颅内DAVF是可行、有效的方法,效果良好。     

Onyx结合微弹簧圈经静脉入路治疗硬脑膜动-静脉瘘

研究背景采取单纯微弹簧圈栓塞供血动脉姑息治疗硬脑膜动静脉瘘的方法,在栓塞血管巢近端供血动脉后,可出现新的供血动脉并可能改变静脉引流途径,从而增加颅内出血风险。闭塞引流静脉是一种十分有效的治疗方法,且经静脉途径闭塞引流静脉成功率较高,本研究尝试经静脉入路注射液体栓塞剂Onyx结合微弹簧圈栓塞治疗硬脑膜动静脉瘘,并探讨其疗效。方法经静脉入路栓塞治疗12例硬脑膜动静脉瘘患者(海绵窦区8例、横窦乙状窦区4例),通过脑血管造影检查及临床随访评价疗效。结果术后即刻全脑血管造影检查瘘口完全闭塞者11例、瘘口处血流速度明显减慢者1例。随访3个月至3年,临床症状完全消失者11例、明显缓解者1例。结论应用液体栓塞剂Onyx结合微弹簧圈经静脉入路栓塞治疗硬脑膜动静脉瘘安全有效。     

Tips and tricks in the diagnosis of intracranial dural arteriovenous fistulas: A pictorial review

Dural arteriovenous fistulas (DAVFs) are complex vascular abnormalities that account for 10-15% of intracranial vascular malformations. DAVFs are typically encountered in middle-aged adults, with a slightly female predominance. The causative factors are still uncertain; however, abnormal local hemodynamics and neoangiogenesis related to dural sinus or venous thrombosis can contribute to DAVF occurrence. The diagnosis is dependent on a high level of clinical suspicion and high-resolution imaging techniques. Computed tomography and/or magnetic resonance imaging aid in the diagnosis, but conventional angiography remains the most accurate method for the complete characterization and classification of DAVFs. The therapeutic approach can be conservative or more aggressive, based on symptom severity, sequelae risk and patient characteristics. This article is a pictorial review of adult intracranial DAVFs that highlights some tips and tricks for recognizing useful red flags in the suspicion of DAVFs.     

硬脑膜动静脉瘘血管内栓塞治疗的实验和临床

目的 通过动物实验揭示ＤＡＶＦ的发病机理；临床中探索最佳治疗方法。方法 对３组狗作不同处理，诱发ＤＡＶＦ；临床中病情采用不同的栓塞方法，以临床治愈。结果 实验组中共有４只动物发生ＤＡＶＦ；３９例病人中，临床治愈２３例，好转１２例，死亡１例。结论 静脉窦内压增高是诱发ＤＡＶＦ病因之一；血管内栓塞对ＤＡＶＦ的治疗效果可靠，但必须针对病情采用不同的栓塞方法，或是联合治疗。     

Endovascular angioplasty and stent placement in venous hypertension related to dural arteriovenous fistulas and venous sinus thrombosis

Venous hypertension in lateral sinuses resulting from dural arteriovenous fistula (DAVF) and venous sinus thrombosis (VST) can manifest with severe neurological deficits, such as infarction or intracerebral hemorrhage. It has been proved that venous hypertension plays a significant role in the evolution and progression of DAVF and VST. The definite treatment in complicated conditions such as multiple DAVFs or multiple sinus occlusions is still unknown. Traditional transarterial embolization, transvenous embolization or radiosurgery alone has limited effects on these conditions. We reported one case with venous hypertension presenting with severe neurological symptoms. The case had quick clinical recovery after correction of venous hypertension by endovascular angioplasty and stent placement in occluded lateral sinuses. Accordingly, we propose this method can be an ideal treatment option either in single or staged therapy of venous hypertension related to DAVFs and VST.     

MRI and SPECT studies of dural arteriovenous fistulas presenting as pure progressive dementia with leukoencephalopathy: a cause of treatable dementia

We report two patients with dural arteriovenous fistulas (DAVFs) who presented with pure progressive dementia. Both patients showed only slowly progressive dementia, without headache, papilledema and other neurologic signs associated with diffuse white matter changes in MRI. MR cerebral angiography showed sigmoid sinus DAVFs that were mainly supplied by the occipital artery, together with retrograde filling of the superior sagittal and straight sinus and dilated cortical veins. SPECT studies showed extensive blood flow reduction in the occipital and parieto-occipital areas and right temporal lobe in one patient. Selective embolization for treatment of the DAVF improved cognitive function associated with the abnormal white matter MRI signal. MRI and SPECT showed that severity of dementia correlated with diffuse white matter changes and regional cerebral blood flow. Our cases suggest that gradually impaired cerebral circulation due to venous hypertensive encephalopathy could be involved in slowly progressive dementia with leukoencephalopathy resulting from a DAVF. DAVFs may be particularly important for differential diagnosis in elderly patients with pure progressive dementia. Thus, early diagnosis of DAVFs and treatment by endovascular surgery is important as treatable or reversible dementia.     

Surgical Obliteration in Superior Petrosal Sinus Dural Arteriovenous Fistula

Superior petrosal sinus (SPS) dural arteriovenous fistula (DAVF) is one of tentorial DAVFs with significant morbidity, which usually drains into the petrosal vein and its tributaries. Unless there is a connection with venous sinus, surgical obliteration is required. We present two cases of SPS DAVF which were successfully treated with the presigmoid retrolabyrinthine approach.     

Incidence of hereditary thrombophilia in patients with cranial dural arteriovenous fistulae

Dural arteriovenous fistulas (DAVFs) can develop as consequence of prior venous sinus thrombosis. However, the prevalence of prothrombotic conditions in patients with intracranial DAVFs is unknown and there is no consensus on the indications to screen for procoagulable conditions in these patients. We performed a retrospective review of patients presenting to our institution for management of cranial DAVF. Patient and DAVF characteristics were noted, in particular the incidence of hereditary thrombophilia (HT) detected by laboratory evaluation. Respective comparisons of patients with and without HT were performed using comparative statistics. There were 165 patients presenting for management of 171 DAVFs. A large proportion of DAVF were located at a large dural sinus (42.7%), which included superior sagittal, transverse, and sigmoid sinuses. On multivariate logistic regression analysis cortical venous drainage was independently associated with hemorrhagic presentation (OR 8.23, 95% CI 1.78–38.08; p = 0.007). A HT was detected in 18 of 61 patients (29.5%) who underwent a thrombophilia work-up. Location at a large dural sinus was not more common in patients with a HT (55.0% vs 54.5%; p = 0.97). There was also no difference in the presence of cortical venous drainage (56.5% vs 38.6%; p = 0.22) or hemorrhage on presentation (15.9% vs 5.0%; p = 0.22) in patients with and without a HT. In HT were found to be highly prevalent in our cohort, though our results do not suggest their presence greatly influences DAVF pathophysiology. Screening for procoagulable states is indicated in selected patients with an intracranial DAVF.     

A dural arteriovenous fistula in cavernous sinus developed from viral meningitis

Although hormonal influences, inflammation, trauma, sinus thrombosis, venous hypertension, and congenital origin have been proposed as sources of dural arteriovenous fistulas (DAVFs) in cavernous and sigmoid sinuses, the etiology of these lesions remains controversial. We present a case with a cavernous sinus DAVF developed from viral meningitis which has not been previously described. A 24-year-old male was admitted to our institute because of periorbital pain, decreased vision, pulsatile tinnitus, chemosis, and exophthalmos on the right side after he had suffered viral meningitis four months before. Cerebral angiography demonstrated a cavernous sinus DAVF, which was successfully obliterated with several platinum coils using a transvenous approach. The viral meningitis most likely caused the inflammation, that may be responsible for the occurrence of the cavernous sinus DAVF. Prompt treatment for inflammation may help to prevent the development of DAVFs.     

前颅窝底硬脑膜动静脉瘘的外科治疗（附3例报告及文献复习）

目的探讨前颅窝底硬脑膜动静脉瘘（DAVF）的临床表现及外科治疗方法和效果。方法对2011年9月至2012年7月我科收治的3例前颅窝底DAVF患者的临床资料进行回顾性分析并结合相关文献进行复习。结果3例患者均为男性,年龄分别为52、56和23岁,头昏1例,额叶血肿1例,硬脑膜下出血1例,3例均经全脑血管造影证实为前颅窝底DAVF,3例均经开颅手术灼闭瘘口,全部治愈,无术后并发症。结论前颅窝底DAVF较为少见,手术灼闭瘘口安全、有效,是首选治疗方法。     

前颅窝底硬脑膜动静脉瘘的经动脉入路治疗

目的评价经动脉入路栓塞前颅窝底硬脑膜动静脉瘘(DAVF)的临床疗效。方法分析6例有症状前颅窝底硬脑膜动静脉瘘(DAVFs)经动脉血管内治疗技术和结果。结果 6例经动脉入路插入导管用Onyx-18胶栓塞。本组病例在治疗过程中都能接近瘘口处并栓塞动静脉分流区域。最后血管造影显示DAVF完全闭塞。治疗后没有观察到相关后遗症,6例均进行临床随访,GOS评分均为5。6例获脑血管造影随访,均未见瘘口显影。结论经动脉入路栓塞前颅窝底DAVFs是可行的。     

小脑幕硬脑膜动静脉瘘临床及影像学特点

目的总结小脑幕硬脑膜动静脉瘘（DAVF）的临床表现及影像学特点。方法回顾分析11例小脑幕DAVF病例的临床及影像学资料。结果本组男7例,女4例,平均年龄56．2岁;急性起病6例,亚急性起病2例,慢性起病3例;临床主要表现头痛、头晕、恶心、呕吐、眼震、共济失调等;头颅CT联合MRI检查可提示颅内出血性病变、非出血性病变、血管流空或异常血管影;DSA检查可清楚显示小脑幕DAVF瘘口、供血动脉和引流静脉。结论小脑幕DAVF临床表现多变,一些MRI特征性改变可提供小脑幕DAVF诊断线索,但确诊依赖DSA检查。     

Dyna CT及吲哚箐绿造影在硬脊膜动静脉瘘手术治疗中的应用

目的探讨应用旋转断层重组技术获得CT样图像(Dyna CT)及吲哚箐绿(ICG)造影对定位、评估硬脊膜动静脉瘘(SDAVF)及手术治疗的指导作用及临床意义。方法对10名符合手术切除SDAVF的患者,术前行Dyna CT三维重建瘘口血流、评估瘘口与脊柱骨性标志的位置关系并设计手术预案,术中行ICG造影准确定位瘘口,辨别供血动脉及引流静脉后切除瘘口,并再次行ICG造影确认引流静脉不显影。结果所有10例患者术前造影及Dyna CT均可辨明瘘口位置及与脊柱的关系,术中均根据ICG造影中的血管显影顺序判断血流方向及定位瘘口,瘘口阻断后再次ICG造影见引流静脉均无显影。术后10例患者神经功能均有明显改善,1 w后所有患者复查DSA均见瘘口完全消失。临床随访8例均未见症状复发结论术前Dyna CT瘘口血流重建结合术中ICG造影可以准确定位瘘口,良好地区分供血动脉及引流静脉,可增加手术安全性。     

Angio-architecture of complex cranial dural arteriovenous fistulas: A single centre retrospective review of treatment modalities and outcomes

IntroductionCranial dural arteriovenous fistulas (DAVFs) are rare vascular lesions that often harbour complex angio-architectural features. This subtype of DAVF may require multiple, multimodality, or hybrid treatments. In this paper we aim to identify specific angio-architectural features that are present in complex cranial DAVFs and we report our series with respect to treatment modalities and outcomes.MethodsTwenty-five cranial Borden type II and III cranial DAVFs were treated at our Institution from 2013 to 2017. We classified nine (36%) as complex based on specific angio-architectural features. Treatment strategies were based on fistula location, angiographic features and patient's presenting condition. Phone interviews were used to confirm outcome at 6 and 12 months.ResultsFour patients (45%) presented with acute hydrocephalus, and 3 (33%) with intracranial hemorrhage. Multiple and combined treatment sessions were needed for all complex DAVFs. Five patients required 2 endovascular procedures each. One patient had 2 surgeries. The first line of treatment was endovascular in 6 cases (67%) and surgery in 3 (33%). Two treatment-related (22%) complications occurred. Complete disconnection was achieved in 5 out of 9 patients (55%). Two patients with an incomplete disconnection refused further treatment and were well at last follow up, with a partially treated fistula and persistent CVR. The other 3 patients concluded treatment after the end of our data collection period. At 1 year, 7/9 patients had stable or improved clinical symptoms, and 8/9 patients had GOS of 4 or 5.ConclusionsComplex cranial DAVF often require a multidisciplinary approach and multiple treatment sessions should be expected. Specific angio-architectural features that increase DAVF complexity include multiple arterial feeders, especially transosseous or pial, reflux into multiple cortical veins, sinus occlusion/entrapment, venous aneurysms, segmental stenosis, medial or deep location, and association with the deep venous system.     

Anterior Cranial Fossa Dural Arteriovenous Fistulae Presenting as Subdural Hematoma

Anterior cranial fossa dural arteriovenous fistulae (DAVFs) are very rare and the bleeding rate is very high, especially in the presence of leptomeningeal draining vein and aneurysmal varix formation. A 85-year-old male patient presented with subdural hematoma (SDH). Magnetic resonance image (MRI) and transfemoral carotid angiography (TFCA) disclosed DAVF at the anterior cranial fossa with bilateral arterial feeders and leptomeningeal draining vein with varix formation. The lesion was treated by simple ligation of pial connecting vein using low frontal craniotomy. In comparison with DAVFs of the other sites, the anterior cranial fossa DAVF is difficult to manage by endovascular treatment due to not only the difficulty of transvenous access but the risk of visual impairment when using transarterial route. Surgical ligation of pial connecting vein is feasible and effective treatment.     

Dural arteriovenous fistula at the foramen magnum presenting with subarachnoid hemorrhage: case reports and literature review

Background and purpose: Spinal dural arteriovenous fistulas (DAVFs) may arise at any level from the foramen magnum to the sacrum. Only a few case series of DAVFs at the foramen magnum have been reported, especially with patients presenting with subarachnoid hemorrhage (SAH). We performed a retrospective study of four such cases and summarize experiences in the diagnosis and surgical treatment of a DAVF at the foramen magnum. Methods: Four male patients, aged from 35 to 51 years, were admitted with severe headache. The cranial computerized tomography scans of all four patients showed SAH, with hemorrhage in the fourth ventricle with or without hemorrhage in the occipital horns of the lateral ventricles. Pre‐operative digital subtraction cerebral angiography showed a DAVF at the foramen magnum draining to medullary veins and/or the straight sinus and the confluence of sinuses. Two DAVFs were fed by the vertebral artery, whilst the others were fed by dural branches of the occipital artery and/or the ascending pharyngeal artery. Results: Three patients underwent direct microsurgical electrocoagulation and disconnection of the arteriovenous shunt via an enlargement of the foramen magnum and a hemilaminectomy at C1 by the far lateral suboccipital approach. Post‐operative angiography confirmed complete obliteration of the fistula. Conclusions: Cerebral digital subtraction angiography is an effective and accurate method for examination of a DAVF at the foramen magnum. It can be treated effectively and with minimal surgical trauma by microsurgical electrocoagulation and disconnection of the shunt.