【病例】多发海绵状脑血管畸形1例CT及MR

History: An elderly woman presents with memory loss and confusion.

病史:老年女性,记忆力减退,精神混乱。

CT images are shown below.

CT图像如下所示

【病例】多发海绵状脑血管畸形1例CT及MR

【病例】多发海绵状脑血管畸形1例CT及MR

MR images

Here are some panels of fluid-attenuated inversion-recovery (FLAIR) and gradient-recalled echo (GRE) images.

水抑制图像及GRE序列图像如下所示。

【病例】多发海绵状脑血管畸形1例CT及MR

【病例】多发海绵状脑血管畸形1例CT及MR

【病例】多发海绵状脑血管畸形1例CT及MR

【病例】多发海绵状脑血管畸形1例CT及MR

Findings

CT: CT shows a 5-mm focus of hyperattenuation with surrounding hypoattenuation within the superior right cerebellar hemisphere. Diffuse sulcal and ventricular prominence, compatible with diffuse cerebral volume loss. Scattered geographic regions of relative hypoattenuation within the bilateral periventricular white matter, compatible with small-vessel ischemic disease.
MRI: MRI shows numerous small, rounded areas of signal dropout, best appreciated on axial GRE, within the cerebellum, right paracentral pons, left posterior and ventral thalamus, and corona radiata.

影像表现:

CT:右小脑半球偏上部可见一直径约5mm高密度灶,周围环绕低密度影。脑沟、脑室广泛增宽,脑体积缩小。双侧侧脑室旁白质可见片状较低密度影,为脑小血管缺血性疾病表现。

MRI:GRE序列轴位图像示小脑、右侧脑桥近中心处、左侧丘脑后侧及腹侧、放射冠可见多发小类圆形低信号灶。

Differential diagnosis

  • Multiple cavernous malformations
  • Multiple hypertensive hemorrhages
  • Sequelae of prior trauma
    • Diffuse axonal injury
    • Parenchymal contusions
  • Amyloid angiopathy
  • Multiple hemorrhagic telangiectasia

鉴别诊断:

多发海绵状脑血管畸形

多发高血压性脑出血

脑外伤后遗症

  • 弥漫性轴索损伤
  • 脑挫伤

淀粉样脑血管病

多发出血性毛细血管扩张症

Diagnosis: Multiple cavernous malformations (presumed, given the distribution and lack of inciting trauma or hypertension)

诊断:多发海绵状脑血管畸形

(此为假设性诊断,结合病变分布特征、缺乏外伤及高血压病史)

Discussion

Cavernous malformations

Cavernous malformations (also known as cavernomas) are benign vascular hamartomas that are comprised of sinusoids of endothelial-lined, immature blood vessels without intervening normal brain tissue. They demonstrate a wide range of behavior and may progress, regress, or enlarge. Cavernous malformations are the most common angiographically occult CNS vascular malformation, with a prevalence of approximately 0.5%. About 75% of cases are solitary (sporadic), with 10% to 30% of cases being multiple or familial. The average age of onset is age 40 to 60, but they can be seen in children. They occur in equal frequency in men and women, and they are seen in all ethnicities.

海绵状血管畸形(也称作海绵状血管瘤),是一种良性的血管错构瘤,由内衬上皮的血管窦及不成熟的血管组成;可缓慢进展、消退或增大;是最常见的中枢神经系统血管畸形,发病率约0.5%。

约75%的病例为单发,10%~30%为多发或家族性。平均发病年龄为40~60岁,偶见于儿童。男女发病率相同,可见于各种族人群。

Cavernous malformations vary in size and may range from microscopic to giant (> 6 cm), averaging 0.5 to 4 cm. They frequently contain blood products of varying ages and often demonstrate a complete hemosiderin rim. Three gene loci are associated with cavernous malformations: CCM1, CCM2, and CCM3. There is a disease entity known as multiple familial cavernous malformations syndrome.

海绵状血管畸形大小不等,从微小的到巨大的(> 6 cm),平均0.5-4cm。病变包含不同时期的血液产物,常可见一完整的含铁血黄素环。

三个基因位点与海绵状血管畸形相关: CCM1, CCM2, CCM3。有一种病为:多发性家族性脑海绵状血管畸形综合症。

Cavernous malformations are associated with developmental venous anomaly (DVA), superficial siderosis, and cutaneous abnormalities, including café-au-lait spots and hyperkeratotic capillary venous malformations (“cherry angiomas”). They most commonly present clinically with seizures (50%), and 25% of patients with cavernous malformations will have neurologic deficits. However, it is important to note that 20% of these patients will be completely asymptomatic.

海绵状血管畸形与下列病变有关:发育性静脉异常、表面含铁血黄素沉积症、皮肤异常(包括咖啡斑、角化过度海绵状静脉畸形)。

临床上常表现为癫痫(50%),25%的患者有神经系统症状,值得注意的是20%的患者完全无症状。

Radiologic overview of the diagnosis

In terms of radiologic workup of cavernous malformations, noncontrast-enhanced CT (NECT) is frequently the first imaging modality employed. Interestingly, 30% to 50% of the time, NECT is negative. When demonstrated on NECT, cavernomas are typically well-delineated round to ovoid, hyperdense lesions, typically less than 3 cm. Approximately 40% to 60% of these lesions demonstrate calcification. They do not demonstrate mass effect, unless there has been recent hemorrhage. It is important to note that the adjacent brain parenchyma appears normal. On contrast-enhanced CT (CECT), cavernomas demonstrate minimal, if any, enhancement. The exception arises in the setting of associated DVA. CT angiography is typically negative.

CT平扫:30%~50%为阴性,典型表现为边界清楚的圆形或类圆形高密度灶,一般小于3cm。40%~60%可见钙化;无占位效应,除非肿瘤近期出血。邻近脑实质正常。

CT增强:病变轻度强化。除非合并发育性静脉异常,CTA一般为阴性。

The single best imaging modality for evaluating cavernous malformations is MRI. On T1-weighted imaging, cavernous malformations demonstrate a variable appearance, which depends on the stage of blood products. Most commonly, they have a “popcorn ball” appearance of heterogenous hyper- and hypointense blood-containing locules. Uncommonly, acute, nonspecific hemorrhage is identified. On T2-weighted imaging, cavernomas most commonly demonstrate a reticulated, “popcorn” lesion of heterogenous signal with complete, hypointense hemosiderin rim. They may or may not demonstrate locules of blood with fluid-fluid levels. These lesions have a rounded appearance, with a matrix preventing collapse. Uncommonly, these lesions are hypointense on T2-weighted imaging. FLAIR may show a small geographic region of surrounding edema in acute lesions. The single best sequence for evaluation of cavernous malformations is the T2* GRE sequence, which is characterized by its marked susceptibility artifact, or “blooming.” If more than three lesions are present, the most common presentation is numerous, punctuate hypointense foci on GRE. Contrast-enhanced T1-weighted imaging demonstrates minimal, if any, enhancement, but it is useful in evaluating for associated DVA. MRA is typically unremarkable, except in cases of associated DVA.

MRI是评估海绵状血管畸形的最好影像检查方法。

T1WI:根据肿瘤内血液产物成分的不同而表现不一,大部分表现为“爆米花样”的不均匀高低混合信号,少数为急性期出血表现。

T2WI:呈网纹状、爆米花样,信号不均,周围可见完整的含铁血黄素环。偶尔可见液液平面。肿瘤呈圆形。偶尔在T2WI上呈低信号。

FLAIR:急性出血期病灶周围可见少量周围水肿。

T2* GRE:最优序列,表现为明显磁敏感伪影,如果超过三个病变,大部分表现为多发的点状低信号灶。

增强:轻度强化;除非合并发育性静脉异常,MRA一般为阴性。

【病例】多发海绵状脑血管畸形1例CT及MR

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