海綿狀血管瘤課件

起源與病變部位：研究證明海綿狀血管瘤是一種不完全外顯性的常染色體顯性遺傳性疾病，基因位于第7條染色體上發(fā)病率：占腦血管畸形的25.1%；人群發(fā)生率約為0.4%~0.8%分類：腦內(nèi)型與腦外型腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦外型海綿狀血管瘤較少見,常見于中顱窩底、鞍旁等部位,尤其海綿竇區(qū)。由于與硬膜關(guān)系密切,又稱為硬膜型海綿狀血管瘤海綿狀血管瘤是一種較常見的先天性血管畸形海綿狀血管瘤是一種較常見的先天性血管畸形1Zabramski的分型標(biāo)準(zhǔn)I型為亞急性出血者II型為病灶反復(fù)出血及血栓形成者III型為慢性出血者IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥Zabramski的分型標(biāo)準(zhǔn)I型為亞急性出血者2臨床特點(diǎn)見于各年齡組,然以30~50歲居多,男女比例大致相等臨床上常表現(xiàn)為反復(fù)出血和癲癇,進(jìn)而可引起神經(jīng)功能缺失,甚至危及生命該瘤通常單發(fā)，單發(fā)病灶無癥狀患者占11%~44％多發(fā)者僅6%~13.4%,且多見于女性或有家族史者。多發(fā)性ICA臨床上往往出現(xiàn)神經(jīng)系統(tǒng)多個(gè)部位受損的癥狀和體征，而病程較單發(fā)ICA者短,而癲癇、顱高壓及神經(jīng)系統(tǒng)局灶癥狀體征多見,并可出現(xiàn)皮質(zhì)功能減退的表現(xiàn)可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤臨床特點(diǎn)見于各年齡組,然以30~50歲居多,男女比例大致相等3影像表現(xiàn)腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相對(duì)較大CT平掃表現(xiàn)為高密度,病灶中心?？梢姲唿c(diǎn)狀鈣化，增強(qiáng)掃描病灶多呈輕至中度強(qiáng)化,如有血栓形成也可以不強(qiáng)化MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有輕微占位效應(yīng)。T1WI以等信號(hào)為主，T2WI以高信號(hào)為主的混雜信號(hào)，周邊有環(huán)形或弧形低信號(hào)帶環(huán)繞，混雜信號(hào)與病變內(nèi)鈣化、含鐵血黃素沉積及不同時(shí)期出血有關(guān)（與動(dòng)靜脈畸形的鑒別:后者病灶多較邊界不清，形態(tài)不規(guī)則，密度或信號(hào)不均勻，MRI示灶內(nèi)常見流空的血管）影像表現(xiàn)腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相4病理表現(xiàn)海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如海綿狀。與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈光鏡下,病灶由竇狀擴(kuò)張的畸形靜脈血管組成,血管壁薄,有單層的內(nèi)皮細(xì)胞和較薄的外膜,缺乏肌層和彈力纖維,管腔內(nèi)充滿血液，病灶內(nèi)見不同時(shí)期的出血、血栓、鈣化,出血腔的再上皮化，血管的增生和肉芽組織的增生可以使海綿狀血管瘤增大。病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見

病理表現(xiàn)5海綿狀血管瘤課件6治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。手術(shù)死亡率為2.7%~7.5%，手術(shù)方法及效果取決于病灶位置。治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床7海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如海綿狀。病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。Thelesionissurroundedbyedema(arrowhead).Type1cavernousangiomainasymptomatic8-year-oldgirl.4%,且多見于女性或有家族史者。5%，手術(shù)方法及效果取決于病灶位置。可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。發(fā)病率：占腦血管畸形的25.5%，手術(shù)方法及效果取決于病灶位置。TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.4%,且多見于女性或有家族史者。腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.Figure1.Type1cavernousangiomainasymptomatic8-year-oldgirl.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).Thediagnosisofacutehemorrhagerelatedtoatype1cavernousangiomawasconfirmedatsurgeryandpathologicanalysis.海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如8Figure2.Type2cavernousangiomainanasymptomatic24-year-oldman.

(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.Thelesionincludesacentralreticulatedcore(arrow)andaperipherallow-signal-intensityrim(arrowhead).(b)TransverseT2-weightedfastSEMRimagehelpsconfirmthepresenceofatype2cavernousangiomaintherightcingulategyrus.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.Asecondcavernousangioma(curvedarrow)withthesameMRimagingfeaturesisclearlydemonstratedintheleftfrontalascendinggyrus.ThesurroundingrimisbetterdemonstratedwithaT2-weightedsequence,asinb,thanwithaT1-weightedsequence,

Figure2.Type2cavernousang9Figure3.Type3cavernousangiomainanasymptomatic43-year-oldman.TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.Figure3.Type3cavernousang10Figure4.Type4cavernousangiomainanasymptomatic

40-year-oldwoman.TransverseT2-weightedGREMRimageshowsasmalllow-signal-intensitylesion(arrow)intherightcerebellarhemisphere.

Figure4.Type4cavernousang11.

Imaging:Thespinechoimagesdemonstrateawellcircumscribedberrylikelesionintheposteriormidpons,whichischaracterizedbymixedsignalintensity,rimofhemosiderin,inhomogenousgadoliniumenhancement,andabsenceofsurroundingedema

History:

This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.

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Imaging:Thespinechoimages12海綿狀血管瘤課件13大腦半球海綿狀血管瘤大腦半球海綿狀血管瘤14腦干海綿狀血管瘤腦干海綿狀血管瘤15脊髓海綿狀血管瘤脊髓海綿狀血管瘤16治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。手術(shù)死亡率為2.7%~7.5%，手術(shù)方法及效果取決于病灶位置。治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床17Figure1.Type1cavernousangiomainasymptomatic8-year-oldgirl.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).Thediagnosisofacutehemorrhagerelatedtoatype1cavernousangiomawasconfirmedatsurgeryandpathologicanalysis.Figure1.Type1cavernousang18它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見該瘤通常單發(fā)，單發(fā)病灶無癥狀患者占11%~44％Type1cavernousangiomainasymptomatic8-year-oldgirl.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)Type3cavernousangiomainanasymptomatic43-year-oldman.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.5%，手術(shù)方法及效果取決于病灶位置。Zabramski的分型標(biāo)準(zhǔn)有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Type3cavernousangiomainanasymptomatic43-year-oldman.Type1cavernousangiomainasymptomatic8-year-oldgirl.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.理想的治療方法尚未確定。History:This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.4%,且多見于女性或有家族史者。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.理想的治療方法尚未確定。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.Type3cavernousangiomainanasymptomatic43-year-oldman.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.發(fā)病率：占腦血管畸形的25.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.5%，手術(shù)方法及效果取決于病灶位置。可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤多發(fā)性ICA臨床上往往出現(xiàn)神經(jīng)系統(tǒng)多個(gè)部位受損的癥狀和體征，而病程較單發(fā)ICA者短,而癲癇、顱高壓及神經(jīng)系統(tǒng)局灶癥狀體征多見,并可出現(xiàn)皮質(zhì)功能減退的表現(xiàn)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。(b)TransverseT2-weightedfastSEMRimagehelpsconfirmthepresenceofatype2cavernousangiomaintherightcingulategyrus.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)Type3cavernousangiomainanasymptomatic43-year-oldman.理想的治療方法尚未確定。它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).4%,且多見于女性或有家族史者?？砂榘l(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相對(duì)較大IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.理想的治療方法尚未確定?？砂榘l(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.II型為病灶反復(fù)出血及血栓形成者腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)理想的治療方法尚未確定。Type1cavernousangiomainasymptomatic8-year-oldgirl.Asecondcavernousangioma(curvedarrow)withthesameMRimagingfeaturesisclearlydemonstratedintheleftfrontalascendinggyrus.病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。5%，手術(shù)方法及效果取決于病灶位置。理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Type3cavernousangiomainanasymptomatic43-year-oldman.Thelesionissurroundedbyedema(arrowhead).ThesurroundingrimisbetterdemonstratedwithaT2-weightedsequence,asinb,thanwithaT1-weightedsequence,腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.CT平掃表現(xiàn)為高密度,病灶中心?？梢姲唿c(diǎn)狀鈣化，增強(qiáng)掃描病灶多呈輕至中度強(qiáng)化,如有血栓形成也可以不強(qiáng)化MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有輕微占位效應(yīng)。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.Zabramski的分型標(biāo)準(zhǔn)(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Type1cavernousangiomainasymptomatic8-year-oldgirl.可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionissurroundedbyedema(arrowhead).History:This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.History:This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有輕微占位效應(yīng)。(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.5%，手術(shù)方法及效果取決于病灶位置。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。4%,且多見于女性或有家族史者。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有輕微占位效應(yīng)。腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)Zabramski的分型標(biāo)準(zhǔn)Type1cavernousangiomainasymptomatic8-year-oldgirl.TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.（與動(dòng)靜脈畸形的鑒別:后者病灶多較邊界不清，形態(tài)不規(guī)則，密度或信號(hào)不均勻，MRI示灶內(nèi)常見流空的血管）起源與病變部位：研究證明海綿狀血管瘤是一種不完全外顯性的常染色體顯性遺傳性疾病，基因位于第7條染色體上(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionincludesacentralreticulatedcore(arrow)andaperipherallow-signal-intensityrim(arrowhead).病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見Type319起源與病變部位：研究證明海綿狀血管瘤是一種不完全外顯性的常染色體顯性遺傳性疾病，基因位于第7條染色體上發(fā)病率：占腦血管畸形的25.1%；人群發(fā)生率約為0.4%~0.8%分類：腦內(nèi)型與腦外型腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦外型海綿狀血管瘤較少見,常見于中顱窩底、鞍旁等部位,尤其海綿竇區(qū)。由于與硬膜關(guān)系密切,又稱為硬膜型海綿狀血管瘤海綿狀血管瘤是一種較常見的先天性血管畸形海綿狀血管瘤是一種較常見的先天性血管畸形20Zabramski的分型標(biāo)準(zhǔn)I型為亞急性出血者II型為病灶反復(fù)出血及血栓形成者III型為慢性出血者IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥Zabramski的分型標(biāo)準(zhǔn)I型為亞急性出血者21臨床特點(diǎn)見于各年齡組,然以30~50歲居多,男女比例大致相等臨床上常表現(xiàn)為反復(fù)出血和癲癇,進(jìn)而可引起神經(jīng)功能缺失,甚至危及生命該瘤通常單發(fā)，單發(fā)病灶無癥狀患者占11%~44％多發(fā)者僅6%~13.4%,且多見于女性或有家族史者。多發(fā)性ICA臨床上往往出現(xiàn)神經(jīng)系統(tǒng)多個(gè)部位受損的癥狀和體征，而病程較單發(fā)ICA者短,而癲癇、顱高壓及神經(jīng)系統(tǒng)局灶癥狀體征多見,并可出現(xiàn)皮質(zhì)功能減退的表現(xiàn)可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤臨床特點(diǎn)見于各年齡組,然以30~50歲居多,男女比例大致相等22影像表現(xiàn)腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相對(duì)較大CT平掃表現(xiàn)為高密度,病灶中心常可見斑點(diǎn)狀鈣化，增強(qiáng)掃描病灶多呈輕至中度強(qiáng)化,如有血栓形成也可以不強(qiáng)化MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有輕微占位效應(yīng)。T1WI以等信號(hào)為主，T2WI以高信號(hào)為主的混雜信號(hào)，周邊有環(huán)形或弧形低信號(hào)帶環(huán)繞，混雜信號(hào)與病變內(nèi)鈣化、含鐵血黃素沉積及不同時(shí)期出血有關(guān)（與動(dòng)靜脈畸形的鑒別:后者病灶多較邊界不清，形態(tài)不規(guī)則，密度或信號(hào)不均勻，MRI示灶內(nèi)常見流空的血管）影像表現(xiàn)腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相23病理表現(xiàn)海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如海綿狀。與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈光鏡下,病灶由竇狀擴(kuò)張的畸形靜脈血管組成,血管壁薄,有單層的內(nèi)皮細(xì)胞和較薄的外膜,缺乏肌層和彈力纖維,管腔內(nèi)充滿血液，病灶內(nèi)見不同時(shí)期的出血、血栓、鈣化,出血腔的再上皮化，血管的增生和肉芽組織的增生可以使海綿狀血管瘤增大。病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見

病理表現(xiàn)24海綿狀血管瘤課件25治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。手術(shù)死亡率為2.7%~7.5%，手術(shù)方法及效果取決于病灶位置。治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床26海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如海綿狀。病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。Thelesionissurroundedbyedema(arrowhead).Type1cavernousangiomainasymptomatic8-year-oldgirl.4%,且多見于女性或有家族史者。5%，手術(shù)方法及效果取決于病灶位置?？砂榘l(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。發(fā)病率：占腦血管畸形的25.5%，手術(shù)方法及效果取決于病灶位置。TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.4%,且多見于女性或有家族史者。腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.Figure1.Type1cavernousangiomainasymptomatic8-year-oldgirl.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).Thediagnosisofacutehemorrhagerelatedtoatype1cavernousangiomawasconfirmedatsurgeryandpathologicanalysis.海綿狀血管瘤為紅色圓形或分葉狀血管團(tuán),形似草莓或桑葚,切面如27Figure2.Type2cavernousangiomainanasymptomatic24-year-oldman.

(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.Thelesionincludesacentralreticulatedcore(arrow)andaperipherallow-signal-intensityrim(arrowhead).(b)TransverseT2-weightedfastSEMRimagehelpsconfirmthepresenceofatype2cavernousangiomaintherightcingulategyrus.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.Asecondcavernousangioma(curvedarrow)withthesameMRimagingfeaturesisclearlydemonstratedintheleftfrontalascendinggyrus.ThesurroundingrimisbetterdemonstratedwithaT2-weightedsequence,asinb,thanwithaT1-weightedsequence,

Figure2.Type2cavernousang28Figure3.Type3cavernousangiomainanasymptomatic43-year-oldman.TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.Figure3.Type3cavernousang29Figure4.Type4cavernousangiomainanasymptomatic

40-year-oldwoman.TransverseT2-weightedGREMRimageshowsasmalllow-signal-intensitylesion(arrow)intherightcerebellarhemisphere.

Figure4.Type4cavernousang30.

Imaging:Thespinechoimagesdemonstrateawellcircumscribedberrylikelesionintheposteriormidpons,whichischaracterizedbymixedsignalintensity,rimofhemosiderin,inhomogenousgadoliniumenhancement,andabsenceofsurroundingedema

History:

This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.

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Imaging:Thespinechoimages31海綿狀血管瘤課件32大腦半球海綿狀血管瘤大腦半球海綿狀血管瘤33腦干海綿狀血管瘤腦干海綿狀血管瘤34脊髓海綿狀血管瘤脊髓海綿狀血管瘤35治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。手術(shù)死亡率為2.7%~7.5%，手術(shù)方法及效果取決于病灶位置。治療理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床36Figure1.Type1cavernousangiomainasymptomatic8-year-oldgirl.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).Thediagnosisofacutehemorrhagerelatedtoatype1cavernousangiomawasconfirmedatsurgeryandpathologicanalysis.Figure1.Type1cavernousang37它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見該瘤通常單發(fā)，單發(fā)病灶無癥狀患者占11%~44％Type1cavernousangiomainasymptomatic8-year-oldgirl.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)Type3cavernousangiomainanasymptomatic43-year-oldman.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.5%，手術(shù)方法及效果取決于病灶位置。Zabramski的分型標(biāo)準(zhǔn)有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Type3cavernousangiomainanasymptomatic43-year-oldman.Type1cavernousangiomainasymptomatic8-year-oldgirl.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)(a)TransverseT1-weightedSEMRimageshowsacavernousangiomaintherightcingulategyrus.理想的治療方法尚未確定。History:This53-year-oldmaletruckdriverpresentedwithdisablingrotatorynystagmus,whichhadgraduallyprogressedovera6-yearperiod.4%,且多見于女性或有家族史者。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.理想的治療方法尚未確定。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.Type3cavernousangiomainanasymptomatic43-year-oldman.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.發(fā)病率：占腦血管畸形的25.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.5%，手術(shù)方法及效果取決于病灶位置?？砂榘l(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤多發(fā)性ICA臨床上往往出現(xiàn)神經(jīng)系統(tǒng)多個(gè)部位受損的癥狀和體征，而病程較單發(fā)ICA者短,而癲癇、顱高壓及神經(jīng)系統(tǒng)局灶癥狀體征多見,并可出現(xiàn)皮質(zhì)功能減退的表現(xiàn)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。(b)TransverseT2-weightedfastSEMRimagehelpsconfirmthepresenceofatype2cavernousangiomaintherightcingulategyrus.(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.與AVM不同,海綿狀血管瘤血流速度不快,缺乏明顯的供血?jiǎng)用}及引流靜脈IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)Type3cavernousangiomainanasymptomatic43-year-oldman.理想的治療方法尚未確定。它可發(fā)生于中樞神經(jīng)系統(tǒng)的任何部位,以顱內(nèi)幕上多見(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).4%,且多見于女性或有家族史者?？砂榘l(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤腦內(nèi)CA直徑多較小，在2~3cm左右，腦外病灶直徑相對(duì)較大IV型可能為微小ICA或毛細(xì)血管擴(kuò)張癥有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.理想的治療方法尚未確定。可伴發(fā)血管皮膚和其它臟器血管畸形及顱內(nèi)和椎管內(nèi)其它腫瘤Thelesionhasacoreofheterogeneouslyhighsignalintensity(straightarrow)andaperipheralrimoflowsignalintensity(arrowhead)relatedtohemosiderindeposition.II型為病灶反復(fù)出血及血栓形成者腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)理想的治療方法尚未確定。Type1cavernousangiomainasymptomatic8-year-oldgirl.Asecondcavernousangioma(curvedarrow)withthesameMRimagingfeaturesisclearlydemonstratedintheleftfrontalascendinggyrus.病灶周邊也?？梢姾F血黃素沉著及反應(yīng)性膠質(zhì)增生，病灶內(nèi)不含神經(jīng)組織且分界清晰而有別于其它血管畸形。5%，手術(shù)方法及效果取決于病灶位置。理想的治療方法尚未確定。有認(rèn)為對(duì)無癥狀的較小CA可在臨床及CT或MR監(jiān)護(hù)下行保守治療，而多數(shù)認(rèn)為手術(shù)切除是治療CA的主要手段。Type3cavernousangiomainanasymptomatic43-year-oldman.Thelesionissurroundedbyedema(arrowhead).ThesurroundingrimisbetterdemonstratedwithaT2-weightedsequence,asinb,thanwithaT1-weightedsequence,腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)腦內(nèi)型發(fā)生在大腦半球占77%~90%，病灶主要位于皮質(zhì)下區(qū)(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.CT平掃表現(xiàn)為高密度,病灶中心常可見斑點(diǎn)狀鈣化，增強(qiáng)掃描病灶多呈輕至中度強(qiáng)化,如有血栓形成也可以不強(qiáng)化MRI表現(xiàn)具有一定的特異性，邊界清晰,病灶周圍無或輕度水腫,無或有