School of Medicine, Koc University, Istanbul, Turkey.
Department of Neurosurgery, Korle Bu Teaching Hospital, Accra, Ghana.
Neurosurg Rev. 2024 Sep 11;47(1):587. doi: 10.1007/s10143-024-02851-w.
Vein of Galen malformations (VOGMs), also known as Vein of Galen Aneurysmal Malformations (VGAMs), are rare and complex cerebrovascular anomalies that pose significant diagnostic and therapeutic challenges. These malformations result from abnormal arteriovenous shunts during embryonic development, leading to a range of severe clinical manifestations, including high-output cardiac failure and hydrocephalus. Advances in prenatal imaging, particularly fetal MRI, have improved early detection, allowing for timely intervention. Endovascular techniques, especially transarterial embolization, have become the primary treatment modality, often preferred over surgical approaches due to their effectiveness and lower risk. However, challenges remain, particularly in managing these malformations in neonates and infants, where the risk of complications is high. Gamma Knife radiosurgery offers a non-invasive alternative for select cases, though its effects are gradual and may carry delayed risks. Despite advancements, the management of VOGMs continues to require a multidisciplinary approach, with ongoing research focused on improving outcomes through a better understanding of the genetic and molecular underpinnings of the disease. Future directions include the integration of genetic studies into clinical practice and the refinement of treatment strategies to optimize outcomes for this complex condition.
脑静脉瘤畸形(VOGM),又称脑静脉瘤样畸形(VGAM),是一种罕见且复杂的脑血管畸形,具有重大的诊断和治疗挑战。这些畸形是由于胚胎发育过程中的异常动静脉分流引起的,导致一系列严重的临床表现,包括高输出性心力衰竭和脑积水。产前成像技术的进步,特别是胎儿 MRI 的应用,提高了早期检测的能力,从而能够及时进行干预。血管内技术,特别是经动脉栓塞术,已成为主要的治疗方式,由于其有效性和较低的风险,通常优于手术方法。然而,挑战仍然存在,特别是在处理新生儿和婴儿的这些畸形时,并发症的风险很高。伽玛刀放射外科为特定病例提供了一种非侵入性的替代方法,但它的效果是渐进的,可能会带来延迟的风险。尽管取得了进展,VOGM 的管理仍需要多学科的方法,并且正在进行研究,通过更好地了解疾病的遗传和分子基础来改善治疗效果。未来的方向包括将遗传研究纳入临床实践,并改进治疗策略,以优化这种复杂疾病的治疗效果。