双语病例——海绵状血管瘤

朗读老师：Jane   天津某医院

翻译老师：章维    昆明医科大学第二附属医院

审校老师：姜春雷   青岛市第九人民医院

History: A 33-year-old woman status post motor vehicle collision.

Head CT without IV contrast was performed.

rain MRI without IV contrast was performed. 

33岁女性车祸后

病史，CT及核磁影像

病史：33岁女性，在发生机动车辆碰撞事故后，接受了颅脑CT平扫检查及核磁检查，

Findings

• CT: There are multiple areas of intra-axial hyperattenuation with prominent lesions in the left posterior centrum semiovale and the left pons. The largest lesion is along the left frontal convexity and contains interspersed calcifications.

影像表现

CT：左侧半卵圆中心后份、桥脑左份可见多发明显高密度病灶。最大的病灶出现在左侧额叶凸面，其内多发点片状钙化。

• MRI: There are more than 20 supratentorial and infratentorial lesions of variable size and T1/T2 hyperintensities. All of the lesions exhibit increased susceptibility on GRE and are in keeping with cerebral cavernous malformations.

磁共振：幕上、幕下可见20多个大小不一的T1、T2高信号病灶。所有的病灶在梯度回波序列上都表现为磁敏感性增强，和脑海绵状血管瘤表现一致。

Differential diagnosis

• Cerebral cavernous malformations

• Cerebral amyloid angiopathy

• Diffuse axonal injury

• Hemorrhagic metastatic disease

Diagnosis: Cerebral cavernous malformations

鉴别诊断

脑海绵状血管瘤畸形

脑淀粉样血管病

脑实质弥漫性轴索损伤

出血性转移性疾病

影像诊断

脑海绵状血管瘤畸形

Cerebral cavernous malformations

海绵状血管瘤

Pathophysiology

Cerebral cavernous malformations (cavernomas) are clusters of 'low flow' vascular channels without brain parenchyma intervening between the sinusoidal vessels. Pathogenesis of cavernomas is not well established. A majority of cavernomas are sporadic, but up to 20% follow a familial, autosomal dominant inheritance pattern characterized by the presence of multiple cavernomas in a single patient. This has led to the identification of three homologically distinct genes responsible for cavernoma development.

病理生理

脑海绵状血管畸形(海绵状血管瘤)是成簇状的低流量血管管道，簇状血管间没有脑实质。海绵状血管瘤的发病机制尚不明确。大多数海绵状血管瘤是散发性的，但高达20%的患者遵循家族性、常染色体显性遗传模式，以单个患者存在多个海绵状血管瘤为特征。以上特征证明海绵状血管瘤的发生、发展是由三个同源的不同基因负责。

Epidemiology

Cavernous malformations are the second most common incidental vascular finding -- after aneurysms -- on brain MRI, with a prevalence of 1 in 625 neurologically asymptomatic people. Clinical presentation is bimodal with a significant number of cases detected in both adolescents and middle-aged adults. There is no discernible sex difference in prevalence; however, there is conflicting research as to whether prognosis is different among men and women.

流行病学 

海绵状血管瘤是颅脑磁共振检查中仅次于动脉瘤的第二大偶发的血管源性病变，625无神经症状人群中就有1例。该病在临床中呈双峰分布，即疾病集中发生于青少年和中年人。患者没有明显的性别差异，然而，关于男性和女性患者预后是否不同仍存在争执。

Clinical presentation

A great majority of cerebral cavernous malformations are incidentally noted on imaging. The presentation of symptomatic cavernous malformations varies by location in the brain, with common manifestations of seizures (50%), intracranial hemorrhage, and focal neurological deficits without radiographic evidence of recent hemorrhage.

临床表现

绝大多数的脑海绵状血管瘤在影像学检查中偶然发现。有症状性脑海绵状血管瘤的表现因病灶在脑内的部位不同而异，常见表现为癫痫发作(50%)、颅内出血和局灶性神经功能受损，而影像学上没有近期出血的证据。

Imaging features

On MRI, a key imaging characteristic is susceptibility of the lesion with susceptibility-induced signal loss of variable size, well seen on GRE sequences or even better on susceptibility-weighted imaging. There is variable appearance of cavernomas on T1- or T2-weighted imaging based on the four different types of lesions that have been classified.

On CT, cavernous malformations are best characterized as somewhat well-defined focal hyperattenuating lesions that may contain calcification. Commonly, many lesions are too small to be distinguished on CT.

影像特点

在磁共振影像上，一个关键的影像特点是病变的磁敏感性不同，因而产生不同磁敏感信号丢失，这一现象在梯度回波序列上也可以出现，而在磁敏感加权成像上可以显示得更好。根据已分类的四种表现，海绵状血管瘤可在T1、T2加权像表中表现出任意一类。

在CT图像上，脑海绵状血管瘤畸形的最具有特征的表现是边界清晰的局灶性高密度病变，其内可有钙化。通常，许多病变因为太小而无法区分。

Treatment

Most cavernous malformations are asymptomatic and do not require treatment or surveillance. If they are symptomatic, surgical or radiotherapy treatment are viable options.

治疗

大多数海绵状血管瘤无症状，不需要治疗或监测。如果有症状，可以考虑手术或放射治疗。