硬腦膜動靜脈瘺的介入診斷及治療硬腦膜動靜脈瘺（DAVF)發生于硬腦膜及其附屬結構如靜脈竇、大腦鐮、小腦幕上的異常動靜脈分流約占顱內動靜脈畸形的10%-15%可見于任何年齡，成人多見硬腦膜動靜脈瘺（DAVF)硬腦膜竇畸形伴動靜脈瘺新生兒或嬰兒，常為巨大囊袋或硬膜湖，與其它竇或大腦靜脈以緩流交通，多累及上矢狀竇，常伴栓塞、閉鎖或一側頸內靜脈球發育低下嬰兒型DAVF高流速，高流量，多灶性，表現為大的竇及多發的局部動靜脈瘺和大的供血血管，常繼發引起皮層－軟膜分流，直竇常缺如；靜脈出口閉塞可引起顱壓增高，腦室積水成人型DAVF嬰兒型DAVF多支供血動脈靜脈竇瘤樣擴張梗塞性腦積水直竇缺如骨皮質改變女，10歲進行性腦神經缺失（嬰兒型DAVF）CT強化：上矢狀竇擴張，腦皮質鈣化，白質變薄MRT1WI：上矢狀竇及竇匯巨大流空影，小腦扁桃體下移成人型DAVF硬腦膜動脈前顱窩腦膜中動脈前支篩前、后動脈腦膜返動脈蝶腭動脈中顱窩腦膜中/副動脈頸內動脈下外側干咽升動脈腦膜支后顱窩椎動脈腦膜支腦膜垂體干枕動脈腦膜支腦膜中動脈后支咽升動脈腦膜支大腦后動脈分支小腦上動脈分支小腦下后動脈分支發病機制DAVF與手術、頭外傷、感染、硬腦膜竇血栓形成、雌激素等因素有關，但確切發病機制不明兩種假說“生理性動靜脈交通”開放：硬腦膜動靜脈之間存“生理性動靜脈交通”（dormantchannels)或“裂隙樣血管”（crack-likevessels），某些病理狀態使其開放，形成DAVF新生血管：某些血管生長因子異常釋放促使硬腦膜新生血管形成，致使DAVF形成分型按靜脈引流方向分型：與臨床表現及預后密切相關按DAVF部位分型：與血供來源及治療途徑密切相關靜脈引流方向與病變部位相結合分型按靜脈引流方向分型Bordenclassification1Venousdrainagedirectlyintoduralvenoussinusormeningealvein2VenousdrainageintoduralvenoussinuswithCVR3Venousdrainagedirectlyintosubarachnoidveins(CVRonly)CognardclassificationIVenousdrainageintoduralvenoussinuswithantegradeflowIIaVenousdrainageintoduralvenoussinuswithretrogradeflowIIbVenousdrainageintoduralvenoussinuswithantegradeflowandCVRIIa+bVenousdrainageintoduralvenoussinuswithretrogradeflowandCVRIIIVenousdrainagedirectlyintosubarachnoidveins(CVRonly)IVTypeIIIwithvenousectasiasofthedrainingsubarachnoidveinsVVenousdrainageintotheperimedullaryplexusCVR=corticalvenousreflux（可能與靜脈竇閉塞有關）按DAVF部位分型海綿竇DAVF橫竇－乙狀竇DAVF小腦幕DAVF上矢狀竇DAVF前顱窩DAVF邊緣竇DAVF巖上/下竇DAVF舌下神經管DAVF臨床表現良性DAVF搏動性雜音眼眶充血顱神經麻痹慢性頭痛無癥狀侵襲性DAVF顱內出血顱內高壓非出血局部神經缺失血管性癡呆死亡Bordentype1CognardtypeI/ⅡaBordentype2/3CognardtypeIIb-Ⅴ皮層靜脈返流（CVR)或深靜脈引流是預后不良的重要因素搏動性突眼球結膜水腫和充血眶周雜音進行性視力下降顱神經麻痹雜音，耳鳴，頭痛眼部癥狀顱內出血（少見）雜音，耳鳴顱內出血中樞神經缺失頭痛顱內出血中樞神經缺失，癡呆顱內出血頭痛診斷經顱多普勒：可探測血流動力學改變，特異性較低CT與MRI:對良性DAVF敏感性較低；對侵襲性DAVF，可顯示異常血管，顱內出血，局部占位效應，腦水腫，腦積水，靜脈竇血栓形成及顱骨骨質異常等征象CTA與MRA：可清楚顯示異常增粗的供血動脈和擴張的引流靜脈及靜脈竇，對瘺口位置及“危險吻合”顯示欠佳診斷DSA供血動脈瘺口位置引流靜脈靜脈竇擴張與閉塞腦循環異常Male,62tentorial

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

DAVF（CognardⅢ）R-ICA術后1年MR示上矢狀竇血栓形成，3年后自感顱內雜音，MR示腦表多發迂曲血管流空影；左側頸外動脈造影側位，左側橫竇DAVF伴CVR，同側乙狀竇閉塞

女，37腎移植術后，左橫竇DAVF（CognardⅡa+b）巖上竇DAVF（CognardⅤ)向脊髓靜脈引流右腦膜中動脈后支，右枕動脈腦膜支及右側腦膜垂體干供血R－ECA造影：右側海綿竇DAVF，引流至眼上靜脈及皮層靜脈男，58右眼球結膜充血水腫治療保守治療立體定向放射治療血管內介入治療外科手術介入治療策略經動脈微粒栓塞（TAE-微粒）：難以達到完全栓塞，通常用于緩解癥狀或輔助治療經靜脈彈簧圈栓塞(TVE)：治愈性手段，必須致密栓塞，否則可使癥狀惡化；可并發靜脈壁損傷，顱內出血經動脈NBCA/Onyx栓塞(TAE)：用于復雜DAVF不能通過靜脈途徑栓塞時，完全栓塞率較高；可造成異位栓塞，對操作技術要求高支架植入：其支撐力可恢復靜脈竇正常引流并可封閉位于靜脈竇壁上的瘺口；遠期效果待進一步觀察海綿竇DAVF保守放療TAE－微粒TVETAE－NBCA海綿竇DAVF經靜脈途徑是首選的治愈性的方法經巖下竇入路（閉塞時亦可通過）經眼上靜脈入路 其它入路：巖上竇、對側海綿竇、基底靜脈叢

SpontaneousregressionofacavernoussinusDAVFT2WIimageshowsmultipleflowvoidsintheposteriorcavernoussinusLeftECAangiogramshowsacavernoussinusduralAVFwithposteriordrainageintotheinferiorandsuperiorpetrosalsinusesFollow-upMRimageshowsresolutionoftheflowvoidsL-ECALeftECAangiogramshowsacavernoussinusDAVFdrainingmainlyintotheinferior

petrosalsinusandpterygopharyngealplexusFollow-upangiogramobtained3months

latershowsthattheinferiorpetrosalsinusisoccluded,andtheduralAVF

nowdrainsintothesuperiorophthalmicveinandthesuperficialmiddlecerebralvein.Although

thepatient’ssymptomswereunchanged,occlusionof

theDAVFwasindicatedTVEofDAVFviaanoccludedinferiorpetrosalsinusLSuperselectivevenogramshowsthatthetipofthemicrocatheterhasbeenintroducedintotheoutletstothesuperiorophthalmicveinLeftCCAangiogramobtainedafterTVEshowscompleteocclusionoftheDAVFTVEofDAVFviaanoccludedinferiorpetrosalsinus橫竇－乙狀竇DAVF放療+TAE-微粒橫竇－乙狀竇DAVFTVE（可先栓塞供血動脈）放療+TAE-微粒支架植入+TAE-微粒+放療TVE避免栓塞正常皮層靜脈引流系統橫竇－乙狀竇DAVFTVE（可先栓塞供血動脈）支架植入受累靜脈竇及返流皮層靜脈近端必須致密栓塞，以防再通致腦出血橫竇－乙狀竇DAVFTVE（手術入路、經閉塞靜脈竇入路、經皮層靜脈入路）TAE-NBCA手術切除（可先栓塞供血動脈）操作難度大，要求技術高ThelateralleftECAangiogramshowsaDAVFofthetransversesinuswithCVRandocclusionoftheipsilateralsigmoidsinus.Atransvenousapproachviathecontralateraltransversesinusallowedselectivecatheterizationofaparallelchannel.VenographyinthisparallelchannelshowstheveinsthatweredrainingthefistulaConversionofanaggressiveDAVFtoabenign(G3)ThisparallelchannelwasembolizedwithacombinationofplatinumcoilsandHydrocoilAcontrolleftECAarteriogramshowsthattheCVRwaseliminated,althoughthefistulapersistsConversionofanaggressiveDAVFtoabenign(G3)ThevenousphaseofthelateralCCAangiogramsbeforeandaftertreatment,weseethatthesecorticalveinscanparticipateinthevenousdrainageofthebrainafterdisconnection難以完全治愈時，可將侵襲性DAVF轉化為良性DAVFConversionofanaggressiveDAVFtoabenign(G3)EarlyarterialphaseleftCCAangiogramshowsatransverse-sigmoidsinusDAVF.LatearterialphaseleftCCAangiogramshowsthattheleftsigmoidsinusisoccludedandtheduralAVFdrainsmainlyintocorticalveinsandtheposteriorcondylarvein.SuperselectivevenogramshowsamicrocatheterthathasbeenadvancedviatheposteriorcondylarveinintotheaffectedsinusRecanalizationofatransverse-sigmoidsinusDAVFafterTVELeftCCAangiogramobtainedafterTVEshowsdisappearanceoftheAVF.CTscanobtained2monthsafterTVEshowsamassivehemorrhageinthelefttemporallobe.LeftcommoncarotidangiogramshowsrecanalizationoftheduralAVFattheretrogradecorticaldrainageoutletRecanalizationofatransverse-sigmoidsinusDAVFafterTVE可能與栓塞不致密有關小腦幕DAVF只經軟腦膜靜脈引流CognardIII/IV，Ⅴ;Borden3侵襲性DAVF，顱內出血風險大治療難度大老年及一般狀況差的患者可考慮放射治療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

上矢狀竇DAVF發生與上矢狀竇血栓形成密切相關經靜脈途徑栓塞困難，常需經手術入路靜脈竇栓塞或手術治療部分病例（瘺口較大）可經動脈行靜脈竇栓塞（靜脈竇無正常靜脈引流）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前顱窩DAVF多由雙側眼動脈的篩動脈供血經軟腦膜靜脈引流CognardIII/IV;Borden3侵襲性DAVF，顱內出血風險大外科手術相對安全，療效好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