第198课病例探析（046）-颅内海绵状血管畸形

上期试题：健康体检时，胸部X线片发现肺内靠近胸膜的孤立性小结节，此时应首先进行的检查是：D

53岁男性，新发癫痫发作就诊。

影像所见

CT平扫示右侧额叶囊性病灶，内可见实性高密度结节。

横断位T2WI示囊性病变可见中央低信号实性成分。

横断位T2-FLAIR示囊性部分信号抑制，呈低信号。

横断位T2*WI示病灶实性成分内没有明显的钙化。囊壁低信号提示含铁血黄素沉着。

横断位T1WI增强扫描病灶内实性部分轻度强化。

颅内海绵状血管畸形是一种良性血管错构瘤，由不规则扩张的血管组成。囊性海绵状血管畸形临床罕见，文献中仅有少量报道。与大多数颅内病变类似，囊性海绵状血管畸形的症状与病灶所在的位置相关，主要症状包括头痛（32%），小脑体征（32%），视乳头水肿（24%），轻偏瘫（20%），癫痫发作（20%）。

Intracranial cavernous vascular malformation is a benign hemangiotoma composed of irregular dilated vessels. Cystic cavernous vascular malformation is rare, and there are only a few reports in the literature. Like most of the intracranial lesions, cystic cavernous vascular malformation of symptoms associated with the lesion location, the main symptoms include headache (32%), cerebellar signs (32%), depending on the papillary edema (24%), hemiparesis (20%), seizures (20%).

疾病影像特点

囊性海绵状血管畸形囊变或血栓形成的病变过程不同，而出现不同的影像学表现。CT上，海绵状血管畸形表现为囊性病变伴高密度实性部分。MR通常表现为T1WI、T2WI混杂信号结节，囊性部分T1WI常呈等或高信号，T2WI高信号。由于病变影像学表现复杂，最后确诊需要依靠手术病理。

There are different imaging manifestations in cystic cavernous angiogenesis or thrombosis. On CT, cavernous vascular malformation is manifested as cystic lesion with high density. MR usually appears as T1WI, T2WI mixed signal nodules, cystic part T1WI is often present or high signal, T2WI high signal. Due to the complexity of the imaging findings, the final diagnosis depends on surgical pathology.

感谢肖老师

以下为惠州市第一人民医院肖老师的总结

脑内型海绵状血管瘤记忆要点：

脑内海绵状血管瘤发病率仅次于发育性静脉畸形，但是最常见的血管造影阴性的脑血管畸形。
The incidence of intracerebral cavernous hemangioma is second only to developmental venous malformation, but the most common angiographic negative cerebrovascular malformations.
脑血管畸形简单4分型：发育性静脉畸形，海绵状血管瘤，动静脉畸形，毛细血管扩张症。
Cerebrovascular malformation simple 4: developmental venous malformation, cavernous hemangioma, arteriovenous malformation, telangiectasia.
CT高密度（不同期龄的出血，血肿机化、钙化）。
CT high density (not concurrent age of hemorrhage, hematoma machine, calcification).
T2WI上爆米花样改变，病变周边低信号环（慢性出血含铁血黄素沉积）。
T2WI was modified with popcorn samples, with low signal rings around the lesion (chronic bleeding with hemosiderin deposition).
75%单发，10%～30%多发。
75% single hair, 10% ~ 30%.
孤立、分叶的桑椹样结节。
Mulberry nodules isolated and divided into leaves.
多无瘤周水肿。
There is no tumor edema.
多无占位效应。
There is no placeholder effect.
可伴发发育性静脉畸形（混合型脑血管畸形），薄壁衬有内皮细胞的血管组成。
It can be accompanied by developmental venous malformations (mixed cerebrovascular malformations), which are composed of blood vessels of endothelial cells.
不含正常脑组织。
No normal brain tissue.