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,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 骨皮质改变,女,10岁 进行性脑神经缺失(婴儿型DAVF),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: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,CVR=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,良性DAVF 搏动性杂音 眼眶充血 颅神经麻痹 慢性头痛 无症状,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 1 Cognard typeI/a,Borden type 2/3 Cognard 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,搏动性突眼 球结膜水肿和充血 眶周杂音 进行性视力下降 颅神经麻痹,杂音,耳鸣,头痛 眼部症状 颅内出血(少见),杂音,耳鸣 颅内出血 中枢神经缺失,头痛 颅内出血 中枢神经缺失,痴呆,颅内出血 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 DAVF(Cognard ) The left lateral ICA angiogram shows a tentorial DAVF fed by an inferior marginal tentorial artery draining into a cortical vein,L-ICA,Male,49 DAVF of anterior cranial fossa (Cognard ) The left lateral internal carotid arteriogram 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 hemorrhage,L-ICA,tentorial DAVF(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;左侧颈外动脉造影侧位,左侧横窦DAVF伴CVR,同侧乙状窦闭塞,女,37肾移植术后,左横窦DAVF(Cognard a+b),岩上窦DAVF(Cognard )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,58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 DAVF T2WI 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 voids,L-ECA,Left 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 middle cerebral vein .Althoughthe patients symptoms were unchanged, occlusion ofthe DAVF was indicated,TVE of DAVF via an occluded inferior petrosal sinus,L,Superselective venogram shows that the tip of the microcatheter has been introduced into the outlets to the superior ophthalmic vein Left CCA angiogram obtained after TVE shows complete occlusion of the DAVF,TVE 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,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 fistula,Conversion of an aggressive DAVF to a benign(G3),This parallel channel was embolized with a combination of platinum coils and Hydrocoil A control left ECA arteriogram shows that the CVR was eliminated, although the fistula persists,Conversion of an aggressive DAVF to a benign(G3),The venous phase of the lateral CCA angiograms before and after treatment, we see that these cortical veins can participate in the venous drainage of the brain after disconnection,难以完全治愈时,可将侵袭性DAVF转化为良性DAVF,Conversion of an aggressive DAVF to a benign(G3),Early arterial phase left CCA angiogram shows a transverse-sigmoid sinus DAVF. Late arterial phase left CCA angiogram shows that the left sigmoid sinus is occluded and the dural AVF drains mainly into cortical veins and the posterior condylar vein. Superselective venogram shows a microcatheter that has been advanced via the posterior condylar vein into the affected sinus,Recanalization of a transverse-sigmoid sinus DAVF after TVE,Left CCA angiogram
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