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1、硬脑膜动静脉瘘的介入诊断及治疗硬脑膜动静脉瘘（DAVF)发生于硬脑膜及其附属结构如静脉窦、大脑镰、小脑幕上的异常动静脉分流 约占颅内动静脉畸形的10%-15%可见于任何年龄，成人多见硬脑膜动静脉瘘（DAVF)硬脑膜窦畸形伴动静脉瘘 新生儿或婴儿，常为巨大囊袋或硬膜湖，与其它窦或大脑静脉以缓流交通，多累及上矢状窦，常伴栓塞、闭锁或一侧颈内静脉球发育低下 婴儿型DAVF 高流速，高流量，多灶性，表现为大的窦及多发的局部动静脉瘘和大的供血血管，常继发引起皮层软膜分流，直窦常缺如；静脉出口闭塞可引起颅压增高，脑室积水 成人型DAVF婴儿型DAVF 多支供血动脉 静脉窦瘤样扩张 梗塞性脑积水 直窦缺如
2、骨皮质改变女，女，1010岁岁 进行性脑神经缺失（婴儿型进行性脑神经缺失（婴儿型DAVFDAVF）CT强化：上矢状窦扩张，脑皮质钙化，白质变薄MR T1WI：上矢状窦及窦汇巨大流空影，小脑扁桃体下移成人型DAVF硬脑膜动脉 前颅窝 脑膜中动脉前支 筛前、后动脉 脑膜返动脉 蝶腭动脉 中颅窝 脑膜中/副动脉 颈内动脉下外侧干 咽升动脉脑膜支 后颅窝 椎动脉脑膜支 脑膜垂体干 枕动脉脑膜支 脑膜中动脉后支 咽升动脉脑膜支 大脑后动脉分支 小脑上动脉分支 小脑下后动脉分支发病机制 DAVF与手术、头外伤、感染、硬脑膜窦血栓形成、雌激素等因素有关，但确切发病机制不明 两种假说“生理性动静脉交通”开放：
3、硬脑膜动静脉之间存“生理性动静脉交通”（dormant channels)或“裂隙样血管”（crack-like vessels），某些病理状态使其开放，形成DAVF 新生血管：某些血管生长因子异常释放促使硬脑膜新生血管形成，致使DAVF形成分型 按静脉引流方向分型：与临床表现及预后密切相关 按DAVF部位分型：与血供来源及治疗途径密切相关 静脉引流方向与病变部位相结合分型按静脉引流方向分型Borden classificationBorden classification1 Venous drainage directly into dural venous sinus or meninge
4、al vein2 Venous drainage into dural venous sinus with CVR3 Venous drainage directly into subarachnoid veins(CVR only)Cognard classificationCognard classificationI Venous drainage into dural venous sinus with antegrade flowIIa Venous drainage into dural venous sinus with retrograde flowIIb Venous dra
5、inage into dural venous sinus with antegrade flow and CVRIIa+b Venous drainage into dural venous sinus with retrograde flow and CVRIII Venous drainage directly into subarachnoid veins(CVR only)IV Type III with venous ectasias of the draining subarachnoid veinsV Venous drainage into the perimedullary
6、 plexusCVR=cortical venous reflux（可能与静脉窦闭塞有关）按DAVF部位分型 海绵窦DAVF 横窦乙状窦DAVF 小脑幕DAVF 上矢状窦DAVF 前颅窝DAVF 边缘窦DAVF 岩上/下窦DAVF 舌下神经管DAVF临床表现v良性DAVF搏动性杂音眼眶充血颅神经麻痹慢性头痛无症状v侵袭性DAVF颅内出血颅内高压非出血局部神经缺失血管性痴呆死亡Borden type 1Cognard typeI/aBorden type 2/3Cognard type IIb-皮层静脉返流（CVR)或深静脉引流是预后不良的重要因素v搏动性突眼v球结膜水肿和充血v眶周杂音v进行性
7、视力下降v颅神经麻痹v杂音，耳鸣，头痛v眼部症状v颅内出血（少见）v杂音，耳鸣v颅内出血v中枢神经缺失v头痛v颅内出血v中枢神经缺失，痴呆v颅内出血v头痛诊断 经颅多普勒：可探测血流动力学改变，特异性较低 CT与MRI:对良性DAVF敏感性较低；对侵袭性DAVF，可显示异常血管，颅内出血，局部占位效应，脑水肿，脑积水，静脉窦血栓形成及颅骨骨质异常等征象 CTA与MRA：可清楚显示异常增粗的供血动脉和扩张的引流静脉及静脉窦，对瘘口位置及“危险吻合”显示欠佳诊断 DSA 供血动脉 瘘口位置 引流静脉 静脉窦扩张与闭塞 脑循环异常 Male,62 tentorial Male,62 tentoria
8、l DAVFDAVF（Cognard Cognard）The left lateral ICA angiogram shows a tentorial DAVF fed by an inferior marginal tentorial artery draining into a cortical veinL-ICA Male,49 DAVF of anterior cranial fossa Male,49 DAVF of anterior cranial fossa（Cognard Cognard）The left lateral internal carotid arteriogram
9、 demonstrates a DAVF supplied by the anterior ethmoidal branches of the ophthalmic artery and the draining intracranial vein with a focal aneurysmal dilatation at the site of parenchymal hemorrhageL-ICAtentorialtentorial DAVFDAVF（Cognard Cognard）R-ICA术后1年MR示上矢状窦血栓形成，3年后自感颅内杂音，MR示脑表多发迂曲血管流空影；左侧颈外动脉造影
10、侧位，左侧横窦DAVF伴CVR，同侧乙状窦闭塞 女，女，3737肾移植术后，左横窦肾移植术后，左横窦DAVFDAVF（Cognard a+bCognard a+b）岩上窦岩上窦DAVFDAVF（Cognard)Cognard)向脊髓静脉引流向脊髓静脉引流右脑膜中动脉后支，右枕动脉脑膜支及右侧脑膜垂体干供血RECA造影：右侧海绵窦DAVF，引流至眼上静脉及皮层静脉男，男，5858右眼球结膜充血水肿右眼球结膜充血水肿治疗 保守治疗 立体定向放射治疗 血管内介入治疗 外科手术介入治疗策略 经动脉微粒栓塞（TAE-微粒）：难以达到完全栓塞，通常用于缓解症状或辅助治疗 经静脉弹簧圈栓塞(TVE)：治愈性
11、手段，必须致密栓塞，否则可使症状恶化；可并发静脉壁损伤，颅内出血 经动脉NBCA/Onyx栓塞(TAE)：用于复杂DAVF不能通过静脉途径栓塞时，完全栓塞率较高；可造成异位栓塞，对操作技术要求高 支架植入：其支撑力可恢复静脉窦正常引流并可封闭位于静脉窦壁上的瘘口；远期效果待进一步观察海绵窦DAVF 保守 放疗 TAE微粒 TVE TAENBCA海绵窦DAVF 经静脉途径是首选的治愈性的方法 经岩下窦入路（闭塞时亦可通过）经眼上静脉入路 其它入路：岩上窦、对侧海绵窦、基底静脉丛 Spontaneous regression of a cavernous sinus Spontaneous reg
12、ression of a cavernous sinus DAVFDAVFT2WI image shows multiple flow voids in the posterior cavernous sinus Left ECA angiogram shows a cavernous sinus dural AVF with posterior drainage into the inferior and superior petrosal sinuses Follow-up MR image shows resolution of the flow voidsL-ECAL-ECALeft
13、ECA angiogram shows a cavernous sinus DAVF draining mainly into the inferiorpetrosal sinus and pterygopharyngeal plexus Follow-up angiogram obtained 3 monthslater shows that the inferior petrosal sinus is occluded,and the dural AVFnow drains into the superior ophthalmic vein and the superficial midd
14、le cerebral vein.Althoughthe patients symptoms were unchanged,occlusion ofthe DAVF was indicatedTVE of DAVF via an occluded inferior petrosal sinusTVE of DAVF via an occluded inferior petrosal sinusLSuperselective venogram shows that the tip of the microcatheter has been introduced into the outlets
15、to the superior ophthalmic vein Left CCA angiogram obtained after TVE shows complete occlusion of the DAVFTVE of DAVF via an occluded inferior petrosal sinusTVE of DAVF via an occluded inferior petrosal sinus横窦乙状窦DAVF 放疗+TAE-微粒横窦乙状窦DAVF TVE（可先栓塞供血动脉）放疗+TAE-微粒 支架植入+TAE-微粒+放疗TVE避免栓塞正常皮层静脉引流系统横窦乙状窦DAVF
16、 TVE（可先栓塞供血动脉）支架植入受累静脉窦及返流皮层静脉近端必须致密栓塞，以防再通致脑出血横窦乙状窦DAVF TVE（手术入路、经闭塞静脉窦入路、经皮层静脉入路）TAE-NBCA 手术切除（可先栓塞供血动脉）操作难度大，要求技术高The lateral left ECA angiogram shows a DAVF of the transverse sinus with CVR and occlusion of the ipsilateral sigmoid sinus.A transvenous approach via the contralateral transverse sinus allowed selective catheterization of a parallel channel.Venography in this parallel channel shows the veins that were draining the fistulaConversion of an aggressive DAVF to a benign(G3)Conversion
