Bursać Danijel, Stipanović Jasminka, Partl Jasenka Zmijanac, Lučić Dejana, Hadžić Daria, Bošnjak Diana Culej, Duić Željko
Department of Obstetrics and Gynecology, University Hospital Merkur, Zagreb, Croatia.
University of North, University Center Varaždin, Croatia.
Eur J Obstet Gynecol Reprod Biol X. 2024 Mar 26;22:100306. doi: 10.1016/j.eurox.2024.100306. eCollection 2024 Jun.
Vein of Galen aneurysmal malformation (VGAM) is a rare vascular anomaly originating during embryonic development, specifically between the 6th and 11th weeks of gestation. This malformation results from abnormal arteriovenous connections between primitive choroidal arteries and the median prosencephalic vein (MPV) of Markowski. Typically, the MPV regresses by the 11th week, but in VGAM, this regression is hindered, leading to persistent abnormal flow and the formation of arteriovenous shunts. We present a case of successful prenatal detection, as well as a comprehensive literature review that summarizes current knowledge, emphasizes the importance of prenatal detection, detailed imaging techniques, understanding clinical presentations, and outlines treatment options. Prenatal detection, crucial for early intervention, has become feasible through ultrasonography and MRI. Fetal MRI has emerged as the gold standard, offering detailed insights into arterial feeders, nidus presence, fistula position, venous drainage, and potential complications. The clinical presentation of VGAM varies with age, and neonates diagnosed in utero may exhibit signs of high-output cardiac failure. Early detection is critical for timely intervention, as untreated VGAMs often result in high mortality rates. Prognosis depends on the severity of heart failure, the number of arteriovenous shunts, and the presence of accompanying fetal abnormalities. Various imaging modalities, including CT angiography and digital subtraction angiography (DSA), aid in the assessment and treatment of VGAM. DSA remains the gold standard for evaluating angioarchitecture and guiding endovascular interventions. The optimal treatment for VGAM is transarterial embolization, offering significant improvements in prognosis. Surgical interventions are limited due to high morbidity and mortality. Management decisions should consider the balance between minimizing neurological damage and achieving maximum embolization effectiveness.
大脑大静脉动脉瘤样畸形（VGAM）是一种罕见的血管异常，起源于胚胎发育期间，具体在妊娠第6至11周。这种畸形是由于原始脉络膜动脉与马尔科夫斯基中脑前静脉（MPV）之间的动静脉连接异常所致。通常，MPV在第11周时退化，但在VGAM中，这种退化受到阻碍，导致持续的异常血流和动静脉分流的形成。我们报告一例成功的产前检测病例，并进行全面的文献综述，总结当前知识，强调产前检测的重要性、详细的成像技术、对临床表现的理解，并概述治疗选择。产前检测对于早期干预至关重要，通过超声检查和MRI已变得可行。胎儿MRI已成为金标准，可提供有关动脉供血、瘤巢存在、瘘管位置、静脉引流和潜在并发症的详细见解。VGAM的临床表现随年龄而异，宫内诊断的新生儿可能表现出高输出量心力衰竭的体征。早期检测对于及时干预至关重要，因为未经治疗的VGAM通常会导致高死亡率。预后取决于心力衰竭的严重程度、动静脉分流的数量以及是否存在伴随的胎儿异常。包括CT血管造影和数字减影血管造影（DSA）在内的各种成像方式有助于VGAM的评估和治疗。DSA仍然是评估血管结构和指导血管内介入治疗的金标准。VGAM的最佳治疗方法是经动脉栓塞，可显著改善预后。由于高发病率和死亡率，手术干预有限。管理决策应考虑在尽量减少神经损伤和实现最大栓塞效果之间取得平衡。
