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,62tentorialDAVF（CognardⅣ）TheleftlateralICAangiogramshowsatentorialDAVFfedbyaninferiormarginaltentorialarterydrainingintoacorticalveinL-ICAMale,49DAVFofanteriorcranialfossa（CognardⅣ）TheleftlateralinternalcarotidarteriogramdemonstratesaDAVFsuppliedbytheanteriorethmoidalbranchesoftheophthalmicarteryandthedrainingintracranialveinwithafocalaneurysmaldilatationatthesiteofparenchymalhemorrhageL-ICAtentorialDAVF（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•TVE•TAE－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-ECALeftECAangiogramshowsacavernoussinusDAVFdrainingmainlyintotheinferiorpetrosalsinusandpterygopharyngealplexusFollow-upangiogramobtained3monthslatershowsthattheinferiorpetrosalsinusisoccluded,andtheduralAVFnowdrainsintothesuperiorophthalmicveinandthesuperficialmiddlecerebralvein.Althoughthepatient’ssymptomswereunchanged,occlusionoftheDAVFwasindicatedTVEofDAVFviaanoccludedinferiorpetrosalsinusLSuperselectivevenogramshowsthatthetipofthemicrocatheterhasbeenintroducedintotheoutletstothesuperiorophthalmicveinLeftCCAangiogramobtainedafterTVEshowscompleteocclusionoftheDAVFTVEofDAVFviaanoccludedinferiorpetrosalsinus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.Superselectivevenogramshowsamicrocatheterthathasbeenadvancedviatheposteriorcondylarveinintotheaffe