背景:Galen静脉畸形(VOGM)是一种罕见的先天性颅内血管畸形。这种病变自发血栓形成是非常罕见的。患者的临床表现可以从无症状到危重。引起自发性血栓形成的潜在病理生理机制仍然知之甚少。
结果:系统综述了关于VOGM自发性血栓形成的文献,分析,并重点总结了其病理生理学,类型,临床表现,诊断,管理,和结果。还通过案例介绍来说明。该病例在一个成功接受手术切除治疗的2岁男孩中表现出不寻常的VOGM表现和位置。
结论:世界范围内报道了少数的VOGM血栓形成病例,其中手术被用于治疗。壁式低流量瘘容易发生自发性血栓形成,延迟了临床表现,通常诊断为幼儿。在许多可能的表现中,脑积水是迄今为止最常见的。在没有血液流动的情况下,MRI是首选的诊断测试。根据病人的症状,切除动脉瘤或转移脑脊液的手术通常会导致良好的预后。
Vein of Galen malformation (VOGM) is an exceptionally uncommon form of congenital intracranial vascular malformations. It is highly unusual for this lesion to spontaneously thrombose. The clinical presentation of a patient may range from being asymptomatic to critically ill. The underlying pathophysiological mechanisms that cause spontaneous thrombosis are still poorly understood.
The literature on spontaneous thrombosis of VOGM was systematically reviewed, analyzed, and summarized with a focus on its pathophysiology, types, clinical presentations, diagnosis, management, and outcomes. It was also illustrated with a case presentation. The case presents an unusual presentation and location of a VOGM in a 2-year-old boy who was successfully treated with surgical resection.
A handful of cases of thrombosed VOGM have been reported worldwide where surgery was used to treat the condition. Low-flow fistulas of the mural type are prone to spontaneous thrombosis, have delayed clinical presentations, and are typically diagnosed in young children. Among the many possible manifestations, hydrocephalus is by far the most common. In the absence of blood flow, MRI is the diagnostic test of choice. Depending on the patient\'s symptoms, surgery to either remove the aneurysm or divert the cerebrospinal fluid usually results in a good prognosis.