主要表现为认知功能障碍的硬脑膜动静脉瘘的临床特点(附1例报告)

目的探讨主要表现为认知功能障碍的硬脑膜动静脉瘘(DAVF)的临床特点。方法回顾性分析1例主要表现为认知功能障碍的DAVF患者的临床资料。结果患者为中年女性,亚急性起病,表现为进行性加重的认知功能障碍,随后出现步态异常和尿失禁;发病1个多月后生活已不能自理。头颅MRI示两侧大脑、小脑半球表面多发异常迂曲、扩张的血管影,两侧大脑半球白质对称大片状T2WI、Flair高信号影。脑血管DSA示右侧颈外动脉动静脉瘘,左侧颈外动脉DAVF,右侧颈内静脉重度狭窄。经血管介入栓塞动静脉瘘及支架置入术治疗后,患者的病情显著改善。结论 DAVF的认知功能障碍起病较急,系颅内静脉高压引起的广泛脑白质病变所致,早期手术治疗的效果好。     

硬脑膜动静脉瘘的临床及影像学特征

目的研究硬脑膜动静脉瘘(duralarteriovenousfistula,DAVF)的临床表现和影像学特征。方法对8例DAVF患者的临床和神经影像学资料进行回顾性分析。结果DAVF临床表现复杂多样,以头痛(4例)和搏动性颅内血管杂音(5例)为主要临床表现,也可以癫痫为首发症状。头部CT和MRI可显示DAVF的一些继发性改变,如蛛网膜下腔出血、颅内出血、广泛的血管流空现象及迂曲扩张异常的血管。MRA、DSA可显示吻合动脉及引流静脉。结论DAVF的CT、MRI、MRA及MRV等具有一定的特征性改变。结合临床资料可提示DAVF,确诊依赖于脑血管造影(DSA)。     

表现为后循环缺血性病变的小脑幕硬脑膜动静脉瘘的临床和影像学特点(附3例报告)

目的 探讨表现为后循环缺血性病变的小脑幕硬脑膜动静脉瘘(DAVF)的临床和影像学特点.方法 回顾性分析3例表现为后循环缺血性病变的小脑幕DAVF患者的临床和影像学资料.结果 本组患者起病形式多样,均表现头晕、恶心、呕吐,病变侧共济失调、水平眼震;1例患者出现交叉性感觉障碍、轻度延髓麻痹和不完全Horner征;头颅MRI均示小脑病变,其中1例合并延髓病变,病灶处均出现长T1、长T2及弥散加权成像高信号;2例MRI增强见小脑病灶强化及多条弯曲血管影;3例头部数字减影血管造影(DSA)检查均发现小脑幕DAVF瘘口.结论 小脑幕DAVF可引起小脑及脑干缺血表现,MRI增强扫描可见病灶强化及异常血管影,DSA检查可明确诊断.     

与板障静脉沟通的硬脑膜动静脉瘘并发颅内出血1例报道及文献复习

目的 总结与板障静脉沟通的硬脑膜动静脉瘘（DAVF）并发颅内出血的诊治经验。方法 回顾性分析血管内介入治疗的1例与板障静脉沟通的DAVF并发颅内出血的临床资料,并结合相关文献分析。结果 62岁男性,左侧肢体肌力0级,头部CT示右侧额顶叶出血;DSA示右侧额顶部DAVF,由右侧脑膜中动脉额顶支供血,经板障静脉、大脑镰静脉引流并因压力增高导致皮层静脉逆流致静脉高压性脑出血;经脑膜中动脉额支注入Onyx胶闭塞瘘口,术后复查造影发现引流静脉消失;术后半年,左侧肢体肌力恢复至4级。结论 与板障静脉沟通的DAVF并发颅内出血为罕见疾病,血管内治疗可达到治愈。     

硬脑膜动静脉瘘的影像特征分析

目的 通过分析硬脑膜动静脉瘘（dural arteriovenous fistula,DAVF）患者的影像学检查结果,探索DAVF在不同影像学检查中的特征性表现,为临床诊断治疗提供依据。方法 对自2005年以来在我院治疗的32例DAVF患者的影像学资料进行回顾性分析。结果 32例DAVF患者,颅脑CT和磁共振成像（magnetic resonance imaging,MRI）检查能较好地显示因DAVF所致的颅内继发性病变,如扩张的引流静脉,继发性出血及静脉窦狭窄、闭塞等改变。数字减影血管造影（digital subtraction angiography,DSA）检查则能准确地显示瘘口部位、供血动脉、静脉引流情况及颅内血流分布情况,但无法显示继发的脑实质病变。 结论 DSA检查是诊断DAVF的金标准。CT、MRI检查亦能为诊断DAFV提供依据。     

以进行性下肢乏力伴大小便失禁、认知损害为主要表现的硬脑膜动静脉瘘(附1例报告及文献复习)

目的提高临床医生对以进行性下肢乏力伴大小便失禁、认知损害为主要表现的硬脑膜动静脉瘘(DAVF)的认知。方法回顾性分析1例以进行性下肢乏力伴大小便失禁、认知损害为主要表现的DAVF患者的临床资料、影像学表现等结果,结合文献复习进行讨论。结果患者男性,67岁。临床表现为进行性动作迟缓、行走不稳、少动-强直为主要特征,同时伴有反应迟钝、小便失禁。头颅MRI平扫示两侧脑白质含水量多,脑内少许缺血灶;增强扫描可见脑内强化血管影增多。脑血管DSA示双侧横窦区硬脑膜动静脉瘘,右侧明显,以双侧颈外动脉供血为主,颈内动脉也有少量供血。经血管介入行DAVF栓塞术后,患者病情显著改善。结论以进行性下肢乏力伴大小便失禁、认知损害为主要临床表现的DAVF易漏诊,DAVF具有可治性,及时行DSA检查可确诊,手术治疗后预后较好。     

特殊类型硬脑膜动静脉瘘临床及影像表现

目的：加深对硬脑膜动静脉瘘（DAVF）临床表现、影像学表现的认识。方法分析1例少见类型DAVF患者的临床资料。结果临床表现主要为头痛、头晕、血管杂音;DSA示：双侧颈外动脉、左侧大脑后动脉均可见动静脉瘘,左侧较重,致颈内动脉显影不佳,左侧椎动脉颅内段不显影,左侧椎动脉颅外段可见明显动静脉瘘。结论 DAVF的临床表现多样且不特异,影像学诊断至关重要,对于不明原因出现头痛、头晕、血管杂音等症状,应高度注意是否存在 DAVF ,尽早行DSA检查。     

硬脑膜动静脉瘘导致双侧丘脑病变(附2例报告及文献复习)

目的报道2例硬脑膜动静脉瘘(DAVF)导致的双侧丘脑病变病例。方法收集2例经数字减影血管造影(DSA)明确的DAVF导致的双侧丘脑病变患者的临床和影像学资料,结合文献复习进行分析。结果病例1,男性,63岁,因反应迟钝、懒言少动近2个月就诊,头颅MRI见双侧丘脑异常信号。DSA见直窦区域DAVF,行动静脉栓塞术,每年随访反应迟钝依然存在,但生活基本能自理。病例2,男性,63岁,因记忆力下降、反应迟钝1个月收治入院。头颅MRI见直窦内少许条状等信号,考虑直窦血栓,伴大脑深静脉梗死可能,继发左侧丘脑出血。DSA见直窦-窦汇区DAVF,予瘘口栓塞术,术后2个月随访,生活自理能力已完全恢复。结论 DAVF导致丘脑病变临床少见,起病隐匿,临床表现或影像学表现相似的疾病较多,诊断困难。遇有进行性认知功能下降,且影像学检查有双侧丘脑病变伴有肿胀出血者,应考虑DAVF可能。     

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。     

颅高压综合征中的硬脑膜动静脉瘘二例报道

目的 介绍2例硬脑膜动静脉瘘(DAVF)影像学特征及诊断过程,以期提高临床工作者对该病的认识及正确诊断率. 方法 回顾性分析厦门大学附属中山医院2009年及2011年收治的2例以颅高压为主要表现的DAVF患者的临床资料及影像学资料. 结果 2例患者均为Ⅱ型患者,临床表现以头痛为主(患者2还出现视力变化).头颅MRI、MRV检查均未见异常,DSA检查提示DAVF. 结论 DAVF可表现为单纯颅高压症状,DSA、MR数字减影血管成像技术可予以鉴别诊断.     

Diffusion-Weighted Magnetic Resonance Imaging of Spontaneous Venous Infarction and Cortical Hemorrhage Complicating Dural Arteriovenous Fistula

Dural arteriovenous fistula (DAVF) is an abnormal arteriovenous shunt that occurs in the dura matter within or near a dural sinus. The clinical manifestations vary. The authors report herein a rare case of type III DAVF presenting with coexisting intracranial hemorrhage (ICH) and venous infarction evidenced by computed tomography (CT) and diffusion-weighted magnetic resonance imaging. Conventional angiography proved that the right middle meningeal artery and the occipital artery supplied the DAVF, with direct drainage into the cortical veins and superior sagittal sinus.     

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.     

Paradoxical Exacerbation of Symptoms with Obstruction of the Venous Outflow after Gamma Knife Radiosurgery for Treatment of a Dural Arteriovenous Fistula of the Cavernous Sinus

A 59-year-old female presented with progressive right proptosis, chemosis and ocular pain. An imaging work-up including conventional catheter angiography showed a right-sided dural arteriovenous fistula of the cavernous sinus, which drained into the right superior petrosal sinus, right superior ophthalmic vein, and right inferior ophthalmic vein, and cortical venous reflux was seen via the right petrosal vein in the right posterior fossa. After failure of transvenous embolization, the patient underwent Gamma Knife radiosurgery (GKRS). At one month after GKRS, she developed increasing ocular pain and occipital headache. Repeat angiography showed partial obliteration of the fistula and loss of drainage via the superior and inferior ophthalmic veins with severe congestion, resulting in slow flow around the right cerebellar hemisphere. Prompt transarterial embolization relieved the patient''s ocular symptoms and headache. We report on a case of paradoxical exacerbation of symptoms resulting from obstruction of the venous outflow after GKRS for treatment of a dural arteriovenous fistula of the cavernous sinus.     

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。     

A Dural Arteriovenous Fistula of the Anterior Cranial Fossa Angiographically Mimicking an Anterior Ethmoidal Artery Aneurysm

Venous aneurysm or varix at the venous side of the fistula commonly exist in dural arteriovenous fistula (DAVF) of the anterior cranial fossa, which may be initially mistaken with aneurysm on computed tomography and magnetic resonance imaging, but always identified by angiography. We report a very unusual case of anterior cranial fossa DAVF angiographically mimicking an anterior ethmoidal artery aneurysm, which was ultimately corrected by surgery. A 41‐year‐old male presented with right frontal intraparenchymal hematoma with intraventricular extension. Angiography revealed a vascular lesion adjacent to the anterior fossa mimicking an anterior ethmoidal artery aneurysm, which was surgically proven to be a partially thrombosed venous varix of drainaging vein originated from the cribriform plate. A diagnosis of anterior cranial fossa DAVF was made, and venous varix was excised. Follow‐up angiography after the operation revealed complete disappearance of the lesion. Our case illustrates a unique occasion that a proximal venous varix without obvious outflow angiographically in DAVF might be mistaken with an aneurysm.     

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.     

3T三维时间飞越法磁共振血管成像在硬脑膜动静脉瘘诊断中的应用

目的探讨3T三维时间飞越法(3D-TOF)磁共振血管成像(MRA)对硬脑膜动静脉瘘(DAVF)的诊断价值。方法对18例DAVF患者的头颅MRI、3D-TOF-MRA及DSA检查结果进行回顾性对照分析。结果发生部位以后颅凹最为常见(15/18,其中天幕9例,乙状窦及横窦区4例,窦汇区及颈静脉孔区各1例),顶部2例,海绵窦区1例。临床表现呈多样性,以颅内高压(8例)和癫闇(4例)最为常见。MRI以脑出血样混杂信号多见(9/18),可伴周边粗大流空血管影、脑水肿及脑组织萎缩等。3D-TOF-MRA显示供血动脉由颈内外、椎基底动脉共同参与多见(13/18例),6例血管影增多增粗,与DSA所见皮层静脉逆行(CVR)引流相吻合。结论 3T 3D-TOF-MRA结合常规MR影像,能较为准确地确定病灶的部位、供血动脉及皮层引流静脉,同时清晰显示DAVF继发性的颅内改变,弥补了DSA的不足,可对DAVF病变进行无创全面的评价。     

Conus perimedullary arteriovenous fistula in a child: unusual angio-architectural features and pathophysiology

INTRODUCTION: The perimedullary arteriovenous fistulas are located on the pial surface and are usually supplied by spinal medullary arteries, that is, either by the anterior or posterior spinal arteries, with no intervening nidus between the feeder arteries and the venous drainage. The clinical findings are, more commonly, caused by progressive radiculomedullary ischemic processes secondary to steal vascular mechanism. As the vascular supply to the spinal cord and to the arteriovenous fistulas (AVF) is not shared with one another, the vascular steal phenomenon cannot be implicated in this case's physiopathology. Most probably, the mass effect caused by the giant venous dilatation was the pathophysiological mechanism involved in this lesion CASE REPORT: The authors describe the case of a 6-year-old girl with an intradural ventral arteriovenous fistula, with a giant venous dilatation, fed directly by L2 and L3 radiculomedullary arteries at the conus medullaris. There was no arterial supply to the fistula from the anterior or posterior spinal arteries. Selective spinal angiography showed an arteriovenous fistula supplied directly by two radiculomedullary arteries, with a large draining vein caudally. Interposing the arterial and venous vessels was a giant venous aneurysmal dilatation located ventral to the conus medullaris and extending from L3 to T6. The patient was successfully treated by a surgical approach through a laminotomy from L3 to T11. CONCLUSION: The type IV-C spinal arteriovenous malformations or perimedullary AVFs are rare lesions predominately described at the conus medullaris with various types of angio-architecture and controversial treatment.