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醫(yī)學(xué)影像服務(wù)中心國(guó)內(nèi)最大的醫(yī)學(xué)影像專(zhuān)業(yè)平臺(tái)，唯一擁有500例病例+征象及專(zhuān)題講座。
來(lái)源：熊貓放射
譯者：花人青
History: An elderly woman presents with memory loss and confusion.
病史：老年女性，記憶力減退，精神混亂。
CT images are shown below.
CT圖像如下所示
Here are some panels of fluid-attenuated inversion-recovery (FLAIR) and gradient-recalled echo (GRE) images.
水抑制圖像及GRE序列圖像如下所示。
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.
影像表現(xiàn)：
CT：右小腦半球偏上部可見(jiàn)一直徑約5mm高密度灶，周?chē)h(huán)繞低密度影。腦溝、腦室廣泛增寬，腦體積縮小。雙側(cè)側(cè)腦室旁白質(zhì)可見(jiàn)片狀較低密度影，為腦小血管缺血性疾病表現(xiàn)。
MRI：GRE序列軸位圖像示小腦、右側(cè)腦橋近中心處、左側(cè)丘腦后側(cè)及腹側(cè)、放射冠可見(jiàn)多發(fā)小類(lèi)圓形低信號(hào)灶。
Differential diagnosis
Multiple cavernous malformations
Multiple hypertensive hemorrhages
Sequelae of prior trauma
Diffuse axonal injury
Parenchymal contusions
Amyloid angiopathy
Multiple hemorrhagic telangiectasia
鑒別診斷：
多發(fā)海綿狀腦血管畸形
多發(fā)高血壓性腦出血
腦外傷后遺癥
彌漫性軸索損傷
腦挫傷
淀粉樣腦血管病
多發(fā)出血性毛細(xì)血管擴(kuò)張癥
Diagnosis: Multiple cavernous malformations (presumed, given the distribution and lack of inciting trauma or hypertension)
診斷：多發(fā)海綿狀腦血管畸形
（此為假設(shè)性診斷，結(jié)合病變分布特征、缺乏外傷及高血壓病史）
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.
海綿狀血管畸形（也稱(chēng)作海綿狀血管瘤），是一種良性的血管錯(cuò)構(gòu)瘤，由內(nèi)襯上皮的血管竇及不成熟的血管組成；可緩慢進(jìn)展、消退或增大；是最常見(jiàn)的中樞神經(jīng)系統(tǒng)血管畸形，發(fā)病率約0.5%。
約75%的病例為單發(fā)，10%~30%為多發(fā)或家族性。平均發(fā)病年齡為40~60歲，偶見(jiàn)于兒童。男女發(fā)病率相同，可見(jiàn)于各種族人群。
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.
海綿狀血管畸形大小不等，從微小的到巨大的（> 6 cm），平均0.5-4cm。病變包含不同時(shí)期的血液產(chǎn)物，?？梢?jiàn)一完整的含鐵血黃素環(huán)。
三個(gè)基因位點(diǎn)與海綿狀血管畸形相關(guān): CCM1, CCM2, CCM3。有一種病為：多發(fā)性家族性腦海綿狀血管畸形綜合癥。
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.
海綿狀血管畸形與下列病變有關(guān)：發(fā)育性靜脈異常、表面含鐵血黃素沉積癥、皮膚異常（包括咖啡斑、角化過(guò)度海綿狀靜脈畸形）。
臨床上常表現(xiàn)為癲癇（50%），25%的患者有神經(jīng)系統(tǒng)癥狀，值得注意的是20%的患者完全無(wú)癥狀。
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平掃：30%~50%為陰性，典型表現(xiàn)為邊界清楚的圓形或類(lèi)圓形高密度灶，一般小于3cm。40%~60%可見(jiàn)鈣化；無(wú)占位效應(yīng)，除非腫瘤近期出血。鄰近腦實(shí)質(zhì)正常。
CT增強(qiáng)：病變輕度強(qiáng)化。除非合并發(fā)育性靜脈異常，CTA一般為陰性。
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是評(píng)估海綿狀血管畸形的最好影像檢查方法。
T1WI：根據(jù)腫瘤內(nèi)血液產(chǎn)物成分的不同而表現(xiàn)不一，大部分表現(xiàn)為“爆米花樣”的不均勻高低混合信號(hào)，少數(shù)為急性期出血表現(xiàn)。
T2WI：呈網(wǎng)紋狀、爆米花樣，信號(hào)不均，周?chē)梢?jiàn)完整的含鐵血黃素環(huán)。偶爾可見(jiàn)液液平面。腫瘤呈圓形。偶爾在T2WI上呈低信號(hào)。
FLAIR：急性出血期病灶周?chē)梢?jiàn)少量周?chē)[。
T2* GRE：最優(yōu)序列，表現(xiàn)為明顯磁敏感偽影，如果超過(guò)三個(gè)病變，大部分表現(xiàn)為多發(fā)的點(diǎn)狀低信號(hào)灶。
增強(qiáng)：輕度強(qiáng)化；除非合并發(fā)育性靜脈異常，MRA一般為陰性。
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