1、 起源与病变部位：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：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：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海绵状血管瘤是一种较常见的先天性血管畸形海绵状血管瘤是一种较常见的先天性血管畸形Zabramski的分型标准 I型为亚急性出血者 II型为病灶反复出血及血栓形成者 III型为慢性出血者 IV型可能为微小ICA
2、或毛细血管扩张症临床特点临床特点 见于各年龄组,然以3050岁居多,男女比例大致相等 临床上常表现为反复出血和癫痫,进而可引起神经功能缺失,甚至危及生命 该瘤通常单发，单发病灶无症状患者占11%44 多发者仅6%13.4%,且多见于女性或有家族史者。多发性ICA临床上往往出现神经系统多个部位受损的症状和体征，而病程较单发ICA者短,而癫痫、颅高压及神经系统局灶症状体征多见,并可出现皮质功能减退的表现 可伴发血管皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤影像表现影像表现 脑内CA直径多较小，在23cm左右，脑外病灶直径相对较大 CTCT平扫平扫表现为高密度,病灶中心常可见斑点状钙化，增强扫描病
3、灶多呈轻至中度强化,如有血栓形成也可以不强化 MRIMRI表现表现具有一定的特异性，边界清晰,病灶周围无或轻度水肿,无或有轻微占位效应。T1WI以等信号为主，T2WI以高信号为主的混杂信号，周边有环形或弧形低信号带环绕，混杂信号与病变内钙化、含铁血黄素沉积及不同时期出血有关（与动静脉畸形的鉴别:后者病灶多较边界不清，形态不规则，密度或信号不均匀，MRI示灶内常见流空的血管）病理表现病理表现 海绵状血管瘤为红色圆形或分叶状血管团,形似草莓或桑葚,切面如海绵状。与AVM不同,海绵状血管瘤血流速度不快,缺乏明显的供血动脉及引流静脉 光镜下,病灶由窦状扩张的畸形静脉血管组成,血管壁薄,有单层的内皮细胞
4、和较薄的外膜,缺乏肌层和弹力纤维,管腔内充满血液，病灶内见不同时期的出血、血栓、钙化,出血腔的再上皮化，血管的增生和肉芽组织的增生可以使海绵状血管瘤增大。病灶周边也常可见含铁血黄素沉着及反应性胶质增生，病灶内不含神经组织且分界清晰而有别于其它血管畸形。它可发生于中枢神经系统的任何部位,以颅内幕上多见 治疗治疗 理想的治疗方法尚未确定。有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。手术死亡率为2.7%7.5%，手术方法及效果取决于病灶位置。海绵状血管瘤为红色圆形或分叶状血管团,形似草莓或桑葚,切面如海绵状。病灶周边也常可见含铁血黄素沉着及反
5、应性胶质增生，病灶内不含神经组织且分界清晰而有别于其它血管畸形。The lesion is surrounded by edema(arrowhead).Type 1 cavernous angioma in a symptomatic 8-year-old girl.4%,且多见于女性或有家族史者。5%，手术方法及效果取决于病灶位置。可伴发血管皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。发病率：占脑血管畸形的25.5%，手术方法及效果取决于病灶位置。Transverse T2-weighte
6、d SE MR image(3,000/98)shows a small cavernous angioma(arrow)in the right cerebral hemisphere,close to the third ventricle and characterized by homogeneously low signal intensity.4%,且多见于女性或有家族史者。脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区它可发生于中枢神经系统的任何部位,以颅内幕
7、上多见The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.(a)Transverse T1-weighted SE MR image shows a cavernous angioma in the right cingulate gyrus.与AVM不同,海绵状血管瘤血流速度不快,缺乏明显的供血动脉及引流静脉(a)
8、Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.Figure 1.Type 1 cavernous angioma in a symptomatic 8-year-old girl.(a)Transverse T1-weighted SE M
9、R image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centru
10、m ovale.The lesion is surrounded by edema(arrowhead).The diagnosis of acute hemorrhage related to a type 1 cavernous angioma was confirmed at surgery and pathologic analysis.Figure 2.Type 2 cavernous angioma in an asymptomatic 24-year-old man.(a)Transverse T1-weighted SE MR image shows a cavernous a
11、ngioma in the right cingulate gyrus.The lesion includes a central reticulated core(arrow)and a peripheral low-signal-intensity rim(arrowhead).(b)Transverse T2-weighted fast SE MR image helps confirm the presence of a type 2 cavernous angioma in the right cingulate gyrus.The lesion has a core of hete
12、rogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.A second cavernous angioma(curved arrow)with the same MR imaging features is clearly demonstrated in the left frontal ascending gyrus.The surrounding rim is bette
13、r demonstrated with a T2-weighted sequence,as in b,than with a T1-weighted sequence,Figure 3.Type 3 cavernous angioma in an asymptomatic 43-year-old man.Transverse T2-weighted SE MR image(3,000/98)shows a small cavernous angioma(arrow)in the right cerebral hemisphere,close to the third ventricle and
14、 characterized by homogeneously low signal intensity.Figure 4.Type 4 cavernous angioma in an asymptomatic 40-year-old woman.Transverse T2-weighted GRE MR image shows a small low-signal-intensity lesion(arrow)in the right cerebellar hemisphere.Imaging:The spin echo images demonstrate a well circumscr
15、ibed berrylike lesion in the posterior midpons,which is characterized by mixed signal intensity,rim of hemosiderin,inhomogenous gadolinium enhancement,and absence of surrounding edemaHistory:This 53-year-old male truck driver presented with disabling rotatory nystagmus,which had gradually progressed
16、 over a 6-year period.大脑半球海绵状血管瘤大脑半球海绵状血管瘤脑干海绵状血管瘤脑干海绵状血管瘤脊髓海绵状血管瘤脊髓海绵状血管瘤治疗治疗 理想的治疗方法尚未确定。有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。手术死亡率为2.7%7.5%，手术方法及效果取决于病灶位置。Figure 1.Type 1 cavernous angioma in a symptomatic 8-year-old girl.(a)Transverse T1-weighted SE MR image shows a large lesion th
17、at includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.The lesion is surrounde
18、d by edema(arrowhead).The diagnosis of acute hemorrhage related to a type 1 cavernous angioma was confirmed at surgery and pathologic analysis.它可发生于中枢神经系统的任何部位,以颅内幕上多见该瘤通常单发，单发病灶无症状患者占11%44Type 1 cavernous angioma in a symptomatic 8-year-old girl.脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区Type 3 cavernous angioma
19、in an asymptomatic 43-year-old man.(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)
20、related to hemosiderin deposition.5%，手术方法及效果取决于病灶位置。Zabramski的分型标准有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。Type 3 cavernous angioma in an asymptomatic 43-year-old man.Type 1 cavernous angioma in a symptomatic 8-year-old girl.脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区(a)Transverse T1-weighted SE MR image
21、 shows a cavernous angioma in the right cingulate gyrus.理想的治疗方法尚未确定。History:This 53-year-old male truck driver presented with disabling rotatory nystagmus,which had gradually progressed over a 6-year period.4%,且多见于女性或有家族史者。有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。Transverse T2-weighted SE
22、MR image(3,000/98)shows a small cavernous angioma(arrow)in the right cerebral hemisphere,close to the third ventricle and characterized by homogeneously low signal intensity.理想的治疗方法尚未确定。The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal i
23、ntensity(arrowhead)related to hemosiderin deposition.Type 3 cavernous angioma in an asymptomatic 43-year-old man.The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.可伴发血管皮肤和其它脏器血管畸形及颅内和
24、椎管内其它肿瘤The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.发病率：占脑血管畸形的25.(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the
25、 left centrum ovale.5%，手术方法及效果取决于病灶位置。可伴发血管皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤多发性ICA临床上往往出现神经系统多个部位受损的症状和体征，而病程较单发ICA者短,而癫痫、颅高压及神经系统局灶症状体征多见,并可出现皮质功能减退的表现脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区病灶周边也常可见含铁血黄素沉着及反应性胶质增生，病灶内不含神经组织且分界清晰而有别于其它血管畸形。(b)Transverse T2-weighted fast SE MR image helps confi
26、rm the presence of a type 2 cavernous angioma in the right cingulate gyrus.(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.The lesion has a core of heterogeneously high signal intensity(straight arrow)and a periphera
27、l rim of low signal intensity(arrowhead)related to hemosiderin deposition.(a)Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.与AVM不同,海绵状血管瘤血流速度不快,
28、缺乏明显的供血动脉及引流静脉IV型可能为微小ICA或毛细血管扩张症The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区Type 3 cavernous angioma in an asymptomatic 43-year-old man.理想的治疗方法尚未确定。
29、它可发生于中枢神经系统的任何部位,以颅内幕上多见(a)Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.The lesion is surrounded by edema(arrowhead).4%,且多见于女性或有家族史者。可伴发血管皮肤和其
30、它脏器血管畸形及颅内和椎管内其它肿瘤脑内CA直径多较小，在23cm左右，脑外病灶直径相对较大IV型可能为微小ICA或毛细血管扩张症有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.理想的治疗方法尚未确定。可伴发血管
31、皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.II型为病灶反复出血及血栓形成者脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区理想的治疗方法尚未确定。Type 1 cavernous angioma in a symptomatic 8-year-old gi
32、rl.A second cavernous angioma(curved arrow)with the same MR imaging features is clearly demonstrated in the left frontal ascending gyrus.病灶周边也常可见含铁血黄素沉着及反应性胶质增生，病灶内不含神经组织且分界清晰而有别于其它血管畸形。5%，手术方法及效果取决于病灶位置。理想的治疗方法尚未确定。有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。Type 3 cavernous angioma in an as
33、ymptomatic 43-year-old man.The lesion is surrounded by edema(arrowhead).The surrounding rim is better demonstrated with a T2-weighted sequence,as in b,than with a T1-weighted sequence,脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区(b)Transverse intermediate-weighted SE MR image confirms the
34、 presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.CT平扫表现为高密度,病灶中心常可见斑点状钙化，增强扫描病灶多呈轻至中度强化,如有血栓形成也可以不强化MRI表现具有一定的特异性，边界清晰,病灶周围无或轻度水肿,无或有轻微占位效应。The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)rela
35、ted to hemosiderin deposition.Zabramski的分型标准(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.Type 1 cavernous angioma in a symptomatic 8-year-old girl.可伴发血管皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤The lesion is surrounded by edema(arrow
36、head).History:This 53-year-old male truck driver presented with disabling rotatory nystagmus,which had gradually progressed over a 6-year period.History:This 53-year-old male truck driver presented with disabling rotatory nystagmus,which had gradually progressed over a 6-year period.The lesion has a
37、 core of heterogeneously high signal intensity(straight arrow)and a peripheral rim of low signal intensity(arrowhead)related to hemosiderin deposition.IV型可能为微小ICA或毛细血管扩张症MRI表现具有一定的特异性，边界清晰,病灶周围无或轻度水肿,无或有轻微占位效应。(a)Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-int
38、ensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of recent bleeding in the left centrum ovale.5%，手术方法及效果取决于病灶位置。有认为对无症状的较小CA可在临床及CT或MR监护下行保守治疗，而多数认为手术切除是治疗CA的主要手段。4%,且多见于女性或有家族史者。The lesion has a core of heterogeneously high signal intensity(straight arrow)and a peripheral rim
39、of low signal intensity(arrowhead)related to hemosiderin deposition.脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区MRI表现具有一定的特异性，边界清晰,病灶周围无或轻度水肿,无或有轻微占位效应。脑内型发生在大脑半球占77%90%，病灶主要位于皮质下区Zabramski的分型标准Type 1 cavernous angioma in a symptomatic 8-year-old girl.Transverse T2-weighted SE MR image(3,000/98)shows a small cavern
40、ous angioma(arrow)in the right cerebral hemisphere,close to the third ventricle and characterized by homogeneously low signal intensity.(a)Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)suggestive of
41、recent bleeding in the left centrum ovale.（与动静脉畸形的鉴别:后者病灶多较边界不清，形态不规则，密度或信号不均匀，MRI示灶内常见流空的血管）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(a)Transverse T1-weighted SE MR image shows a large lesion that includes a high-signal-intensity area(arrow)and a low-signal-intensity area(arrowhead)sugges
42、tive of recent bleeding in the left centrum ovale.可伴发血管皮肤和其它脏器血管畸形及颅内和椎管内其它肿瘤The lesion includes a central reticulated core(arrow)and a peripheral low-signal-intensity rim(arrowhead).病灶周边也常可见含铁血黄素沉着及反应性胶质增生，病灶内不含神经组织且分界清晰而有别于其它血管畸形。(b)Transverse intermediate-weighted SE MR image confirms the presence of a large hemorrhagic lesion(arrow)in the left centrum ovale.
