海绵状血管瘤.ppt

颅内海绵状血管瘤(intracranialcavernousangiomas,ICA)
起源与病变部位：研究证明海绵状血管瘤是一种不完全外显性的常染色体显性遗传性疾病，基因位于第7条染色体上
发病率：占脑血管畸形的25.1%；人群发生率约为0.4%~0.8%
分类：脑内型与脑外型
脑内型发生在大脑半球占77%~90%，病灶主要位于皮质下区
脑外型海绵状血管瘤较少见,常见于中颅窝底、鞍旁等部位,尤其海绵窦区。由于与硬膜关系密切,又称为硬膜型海绵状血管瘤
Zabramski的分型标准临床特点影像表现病理表现治疗Figure1.Type1cavernousangiomainasymptomatic8-year-oldgirl.
(a)TransverseT1-weightedSEMRimageshowsalargelesionthatincludesahigh-signal-intensityarea(arrow)andalow-signal-intensityarea(arrowhead)suggestiveofrecentbleedingintheleftcentrumovale.
(b)Transverseintermediate-weightedSEMRimageconfirmsthepresenceofalargehemorrhagiclesion(arrow)intheleftcentrumovale.Thelesionissurroundedbyedema(arrowhead).Thediagnosisofacutehemorrhagerelatedtoatype1cavernousangiomawasconfirmedatsurgeryandpathologicanalysis.Figure2.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,Figure3.Type3cavernousangiomainanasymptomatic43-year-oldman.TransverseT2-weightedSEMRimage(3,000/98)showsasmallcavernousangioma(arrow)intherightcerebralhemisphere,closetothethirdventricleandcharacterizedbyhomogeneouslylowsignalintensity.Figure4.Type4cavernousangiomainanasymptomatic40-year-oldwoman.TransverseT2-weightedGREMRimageshowsasmalllow-signal-intensitylesion(arrow)intherightcerebellarhemisphere..Imaging:Thespinechoimagesdemonstrateawellcircumscribedberrylikelesionintheposteriormidpons,whichischaracterizedbymixedsignalintens