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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骨皮质改变女，女，1
2、010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：硬脑膜动静脉之间存“生理性动静脉交通”（dormant c
3、hannels)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按静脉引流方向分型Borden classificationBorden classification1 Venous drainage directly into dural venous sinus or meningeal vein2 Venous drainage
4、 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 drainage into dural venous
5、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 plexusCVR=cortical ven
6、ous 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
7、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 tentorial DAVFDAVF（Cognard Cognard ）The left
8、 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 demonstrates a DAVF supplied by
9、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；左侧颈外动脉造影侧位，左侧横窦DAVF伴CVR，同侧乙状窦闭塞 女，女，3737
10、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N
11、BCA/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TVETAENBCA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 regression of a cavernous sinus DAVFDAVFT2WI imag
12、e 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 ECA angiogram shows a cavernous sinus DAVF dra
13、ining 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 sympto
14、ms 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 to the superior ophthalmic vein Left CCA an
15、giogram 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DAVFTVE（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DAVFTVE（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
16、横窦乙状窦DAVFTVE（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 parall
17、el channel. Venography in this parallel channel shows the veins that were draining the fistulaConversion of an aggressive DAVF to a benign(G3) 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 ar
18、teriogram shows that the CVR was eliminated, although the fistula persistsConversion of an aggressive DAVF to a benign(G3) 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 t
19、he 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DAVFConversion of an aggressive DAVF to a benign(G3) 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 t
20、hat 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 sinusRecanalization of a transverse-sigmoid sinus DAVF af
21、ter TVERecanalization of a transverse-sigmoid sinus DAVF after TVELeft CCA angiogram obtained after TVE shows disappearance of the AVF. CT scan obtained 2 months after TVE shows a massive hemorrhage in the left temporal lobe. Left common carotid angiogram shows recanalization of the dural AVF at the
22、 retrograde cortical drainage outlet Recanalization of a transverse-sigmoid sinus DAVF after TVERecanalization of a transverse-sigmoid sinus DAVF after TVE可能与栓塞不致密有关小脑幕DAVF只经软脑膜静脉引流Cognard III /IV，; Borden 3侵袭性DAVF，颅内出血风险大治疗难度大老年及一般状况差的患者可考虑放射治疗Treatment Options for Tentorial Dural AVFsTreatment Opt
23、ions for Tentorial Dural AVFsTreatment Option*Results Radiation therapy Complete occlusion (50%60%) Intervention TAE with n-butyl-2-cyanoacrylate Complete occlusion (50%100%)TVE Complete occlusion (90%100% in few case reports) Surgery (disconnection of Complete occlusion (100%)leptomeningeal venous
24、drainage) *Surgery and TAE with n-butyl-2-cyanoacrylate are equal in terms of potential risk and technical difficulty; they are more potentially risky and technically difficult than radiation therapy and less so than TVE.tentorial dural AVFtentorial dural AVF （Cognard IVCognard IV）Left ECA angiogram
25、 shows a tentorial dural AVFwith leptomeningeal-cortical venous drainage and venous ectasia Lateral radiograph shows the plannedradiation field Left CCA angiogram obtained 8 months after radiation therapyshows complete obliteration of the tentorial dural AVF Male,62, presented with a brain stem hemo
26、rrhage Male,62, presented with a brain stem hemorrhageThe left ICA angiogram shows a DAVF fed by an inferior marginal tentorial artery draining into a cortical vein. Using a transvenous approach catheterization of the venous pouch was feasible. Coils were deposited within the cortical vein and the v
27、enous pouch 上矢状窦DAVF发生与上矢状窦血栓形成密切相关经静脉途径栓塞困难，常需经手术入路静脉窦栓塞或手术治疗部分病例（瘘口较大）可经动脉行静脉窦栓塞（静脉窦无正常静脉引流）Treatment Options for Superior Sagittal Sinus Dural AVFsTreatment Options for Superior Sagittal Sinus Dural AVFsTreatment Option* Results Radiation therapy Unknown Intervention TAE with particles Complete o
28、cclusion (rare) TVE Complete occlusion (90%100%) TAE with n-butyl-2-cyanoacrylate Complete occlusion (90%100%) Transarterial sinus catheterization Complete occlusion (100% in case and coil embolizatio reports) Surgery (sinus isolation or resection) Complete occlusion (90%100%) combined with interven
29、tion *Treatment options in decreasing order of potential risk and technical difficulty are TAE with n-butyl-2-cyanoacrylate,surgery, TVE, and radiation therapy.Superior sagittal sinus dural AVF Superior sagittal sinus dural AVF Right ECA angiogram shows a dural AVF with cortical reflux and occlusion
30、 of the superior sagittal sinus Right ECA angiogram obtained during transarterial sinus embolization shows a microcatheter that has been advanced into the superior sagittal sinus via the right middle meningeal artery Right ECA angiogram obtained after embolization shows obliteration of the AVF前颅窝DAV
31、F多由双侧眼动脉的筛动脉供血经软脑膜静脉引流Cognard III /IV; Borden 3侵袭性DAVF，颅内出血风险大外科手术相对安全，疗效好Treatment Options for Anterior Fossa Dural AVFsTreatment Options for Anterior Fossa Dural AVFsTreatment Option* ResultsRadiation therapy UnknownIntervention TAE with n-butyl-2-cyanoacrylate Complete occlusion (90%100% in a few
32、 case reports) TVE with a retrograde cortical Complete occlusion (90%100% in venous approach a few case reports)Surgery (disconnection of Complete occlusion (100%)leptomeningeal venous drainage) *TVE and TAE with n-butyl-2-cyanoacrylate are equal in terms of potential risk and technical difficulty;
33、they are more potentially risky and technically difficult than surgery, which in turn is more so than radiation therapy. Anterior fossa dural AVFAnterior fossa dural AVFUnenhanced CT scan shows intracranial hemorrhage at the frontal base Left ICA angiogram shows a dural AVF that is fed by the ethmoi
34、dal artery and drains into theleptomeningeal vein, which demonstrates varices Left ICA angiogram obtained after clipping of the draining vein shows disappearance of the AVFL-ICAL-ICA男，男，39 39 前颅窝前颅窝DAVFDAVF右颈内动脉造影：前颅窝DAVF，由增粗的筛前动脉供血，向前引流至上矢状窦，向深部引流至岩上窦左颈内动脉造影：左侧筛前动脉参与供血R-ICAR-ICAL-ICAL-ICA男，男，39 39
35、前颅窝前颅窝DAVFDAVF经上矢状窦置入微导管，颈内动脉证实微导管头位于引流静脉瘤样扩张处，应用两枚电解弹簧圈栓塞R-ICAR-ICA男，男，39 39 前颅窝前颅窝DAVFDAVFR-ICAR-ICAL-ICAL-ICA参考文献Hiro Kiyosue, Yuzo Hori, Mika Okahara,et al. Treatment of Intracranial Dural Arteriovenous Fistulas: Current Strategies Based on Location and Hemodynamics, and Alternative Techniques of Transcatheter Embolization1. RadioGraphics 2004; 24:16371653.Robert W.Hurst, Robert H.Rosenwasser. INTERVENTIONAL NEURORADIOLOGY.335-351
