Gaztanaga Wendy, Luther Evan, McCarthy David, Chamyan Gabriel, Wang Shelly, Ragheb John
Department of Neurosurgery, University of Miami Miller School of Medicine, Miami, FL, USA.
Department of Pathology, Nicklaus Children's Hospital, Miami, FL, USA.
Childs Nerv Syst. 2022 May;38(5):1005-1009. doi: 10.1007/s00381-021-05358-7. Epub 2021 Sep 30.
Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature.
颅内混合性血管畸形(MVMs)被定义为在单个连续病变内发育性静脉异常(DVA)、脑海绵状畸形(CCM)、毛细血管扩张症(CTG)或动静脉畸形(AVM)的任何组合。然而,文献中描述的大多数MVMs仅包含2种病理上不同的畸形;单个病变中并列存在3种或更多异常的情况仍然极为罕见。我们报告了一例19个月大的女性患者,其新发局灶性癫痫发作,右侧基底节有一个4厘米的病变,最初被认为是胚胎性肿瘤。随后,她通过经侧裂-经岛叶入路对病变进行了全切除(GTR)。术中,病变看起来质地不均且血管丰富,有紫灰色易碎组织区域。病理证实该病变为包含CCM、CTG和DVA的MVM。这似乎是文献中首例经病理证实的此类病变。