硬脑膜动静脉瘘的介入诊断及治疗

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MRT1WI：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”（dormantchannels)或“裂隙样血管”（crack-likevessels），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Bordenclassification1Venousdrainagedirectlyintoduralvenoussinusormeningealvein2VenousdrainageintoduralvenoussinuswithCVR3Venousdrainagedirectlyintosubarachnoidveins(CVRonly)CognardclassificationIVenousdrainageintoduralvenoussinuswithantegradeflowIIaVenousdrainageintoduralvenoussinuswithretrogradeflowIIbVenousdrainageintoduralvenoussinuswithantegradeflowandCVRIIa+bVenousdrainageintoduralvenoussinuswithretrogradeflowandCVRIIIVenousdrainagedirectlyintosubarachnoidveins(CVRonly)IVTypeIIIwithvenousectasiasofthedrainingsubarachnoidveinsVVenousdrainageintotheperimedullaryplexusCVR=corticalvenousreflux（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Bordentype1CognardtypeI/ⅡaBordentype2/3CognardtypeIIb-Ⅴ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,62tentorial

DAVF（CognardⅣ）TheleftlateralICAangiogramshowsatentorialDAVFfedbyaninferiormarginaltentorialarterydrainingintoacorticalveinL-ICAMale,49DAVFofanteriorcranialfossa（CognardⅣ）TheleftlateralinternalcarotidarteriogramdemonstratesaDAVFsuppliedbytheanteriorethmoidalbranchesoftheophthalmicarteryandthedrainingintracranialveinwithafocalaneurysmaldilatationatthesiteofparenchymalhemorrhageL-ICAtentorial

DAVF（CognardⅢ）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（CognardⅡa+b）岩上窦DAVF（CognardⅤ)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R－ECA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TVETAE－NBCA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

SpontaneousregressionofacavernoussinusDAVFT2WIimageshowsmultipleflowvoidsintheposteriorcavernoussinusLeftECAangiogramshowsacavernoussinusduralAVFwithposteriordrainageintotheinferiorandsuperiorpetrosalsinusesFollow-upMRimageshowsresolutionoftheflowvoidsL-ECALeftECAangiogramshowsacavernoussinusDAVFdrainingmainlyintotheinferior

petrosalsinusandpterygopharyngealplexusFollow-upangiogramobtained3months

latershowsthattheinferiorpetrosalsinusisoccluded,andtheduralAVF

nowdrainsintothesuperiorophthalmicveinandthesuperficialmiddlecerebralvein.Although

thepatient’ssymptomswereunchanged,occlusionof

theDAVFwasindicatedTVEofDAVFviaanoccludedinferiorpetrosalsinusLSuperselectivevenogramshowsthatthetipofthemicrocatheterhasbeenintroducedintotheoutletstothesuperiorophthalmicveinLeftCCAangiogramobtainedafterTVEshowscompleteocclusionoftheDAVFTVEofDAVFviaanoccludedinferiorpetrosalsinus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ThelateralleftECAangiogramshowsaDAVFofthetransversesinuswithCVRandocclusionoftheipsilateralsigmoidsinus.Atransvenousapproachviathecontralateraltransversesinusallowedselectivecatheterizationofaparallelchannel.VenographyinthisparallelchannelshowstheveinsthatweredrainingthefistulaConversionofanaggressiveDAVFtoabenign(G3)ThisparallelchannelwasembolizedwithacombinationofplatinumcoilsandHydrocoilAcontrolleftECAarteriogramshowsthattheCVRwaseliminated,althoughthefistulapersistsConversionofanaggressiveDAVFtoabenign(G3)ThevenousphaseofthelateralCCAangiogramsbeforeandaftertreatment,weseethatthesecorticalveinscanparticipateinthevenousdrainageofthebrainafterdisconnection难以完全治愈时，可将侵袭性DAVF转化为良性DAVFConversionofanaggressiveDAVFtoabenign(G3)EarlyarterialphaseleftCCAangiogramshowsatransverse-sigmoidsinusDAVF.LatearterialphaseleftCCAangiogramshowsthattheleftsigmoidsinusisoccludedandtheduralAVFdrainsmainlyintocorticalveinsandtheposteriorcondylarvein.SuperselectivevenogramshowsamicrocatheterthathasbeenadvancedviatheposteriorcondylarveinintotheaffectedsinusRecanalizationofatransverse-sigmoidsinusDAVFafterTVELeftCCAangiogramobtainedafterTVEshowsdisappearanceoftheAVF.CTscanobtained2monthsafterTVEshowsamassivehemorrhageinthelefttemporallobe.LeftcommoncarotidangiogramshowsrecanalizationoftheduralAVFattheretrogradecorticaldrainageoutletRecanalizationofatransverse-sigmoidsinusDAVFafterTVE可能与栓塞不致密有关小脑幕DAVF只经软脑膜静脉引流CognardIII/IV，Ⅴ;Borden3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TreatmentOptionsforTentorialDuralAVFs

TreatmentOption*

Results※Radiationtherapy

Completeocclusion(50%–60%)※Intervention

●TAEwithn-butyl-2-cyanoacrylate

Completeocclusion(50%–100%)

●TVE

Completeocclusion(90%–100%in

fewcasereports)※Surgery(disconnectionof

Completeocclusion(100%)

leptomeningealvenousdrainage)*SurgeryandTAEwithn-butyl-2-cyanoacrylateareequalintermsofpotentialriskandtechnicaldifficulty;theyaremorepotentiallyriskyandtechnicallydifficultthanradiationtherapyandlesssothanTVE.

tentorialduralAVF

（CognardIV）LeftECAangiogramshowsatentorialduralAVF

withleptomeningeal-corticalvenousdrainageandvenousectasiaLateralradiographshowstheplanned

radiationfieldLeftCCAangiogramobtained8monthsafterradiationtherapyshowscompleteobliterationofthetentorialduralAVFMale,62,presentedwithabrainstemhemorrhageTheleftICAangiogramshowsaDAVFfedbyaninferiormarginaltentorialarterydrainingintoacorticalvein.Usingatransvenousapproachcatheterizationofthevenouspouchwasfeasible.Coilsweredepositedwithinthecorticalveinandthevenouspouch

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）TreatmentOptionsforSuperiorSagittalSinusDuralAVFs

TreatmentOption*

Results※RadiationtherapyUnknown※Intervention

●TAEwithparticlesCompleteocclusion(rare)

●TVECompleteocclusion(90%–100%)

●TAEwithn-butyl-2-cyanoacrylateCompleteocclusion(90%–100%)

●TransarterialsinuscatheterizationCompleteocclusion(100%incaseandcoilembolizatioreports)※Surgery(sinusisolationorresection)Completeocclusion(90%–100%)combinedwithintervention*TreatmentoptionsindecreasingorderofpotentialriskandtechnicaldifficultyareTAEwithn-butyl-2-cyanoacrylate,surgery,TVE,andradiationtherapy.

SuperiorsagittalsinusduralAVFRightECAangiogramshowsaduralAVFwithcorticalrefluxandocclusionofthesuperiorsagittalsinusRightECAangiogramobtainedduringtransarterialsinusembolizationshowsamicrocatheterthathasbeenadvancedintothesuperiorsagittalsinusviatherightmiddlemeningealarteryRightECAangiogramobtainedafterembolizationshowsobliterationoftheAVF前颅窝DAVF多由双侧眼动脉的筛动脉供血经软脑膜静脉引流CognardIII/IV;Borden3侵袭性DAVF，颅内出血风险大外科手术相对安全，疗效好TreatmentOptionsforAnteriorFossaDuralAVFs

TreatmentOption*

Results※RadiationtherapyUnknown※Intervention

●TAEwithn-butyl-2-cyanoacrylateCompleteocclusion(90%–100%inafewcasereports)

●TVEwitharetrogradecorticalCompleteocclusion(90%–100%invenousapproachafewcasereports)※Surgery(disconnectionof

Completeocclusion(100%)

leptomeningealvenousdrainage)*TVEandTAEwithn-butyl-2-cyanoacrylateareequalintermsofpotentialriskand