Lasjaunias P L, Alvarez H, Rodesch G, Garcia-Monaco R, Ter Brugge K, Burrows P, Taylor W
Neuroradiologie Vasculaire Diagnostique et Thérapeutique, Hôpital de Bicêtre; Le Kremlin Bicêtre Cedex, France.
Interv Neuroradiol. 1996 Mar 30;2(1):15-26. doi: 10.1177/159101999600200102. Epub 2001 May 15.
The Vein of Galen Aneurysmal Malformation (VGAM) is regarded as a lesion with high morbidity and mortality, both spontaneously and under treatment, in part due to an incomplete appreciation of the effects of the lesion on the specific physiology of young children. In addition, various techniques have been applied over a short period of time to treat the lesion rather than the disorders it creates. We report experience with 120 consecutive cases of VGAM managed over the past ten years: 24 were diagnosed antenatally; 50 presented in the neonatal period with haemodynamic disturbance; 35 in infants presented with secondary hydrovenous disorders (macro crania, subependymal atrophy and ventricular dilatation); 12 were seen in children. We were unable to follow 10% of the patients because the referring teams decided not to follow our therapeutic advice. Treatment was contra-indicated in 17% of cases (with early brain damage and a rapidly fatal outcome). There were five (4%) which thrombosed spontaneously (but only two of these were neurologically normal). Embolisation was performed in 78 patients via a transarterial femoral approach using glue. Anatomical cure has already been achieved in 53%. Of the treated patients, even when treatment has not been completed, 80% are growing neurologically normal. Permanent neurological deficit or irreversible neurocognitive delay related to the natural history of the lesion were evident in 8.5%. Overall mortality was 9%. These figures confirm that previous views on the prognosis of VGAM should be revised. Endoarterial embolisation, is the preferred treatment to date.
大脑大静脉动脉瘤样畸形（VGAM）被认为是一种发病率和死亡率都很高的病变，无论是在自然状态下还是接受治疗时都是如此，部分原因是对该病变对幼儿特定生理机能的影响认识不足。此外，在短时间内应用了各种技术来治疗该病变，而不是治疗其引发的病症。我们报告了过去十年中连续治疗的120例VGAM病例的经验：24例在产前被诊断出；50例在新生儿期出现血流动力学紊乱；35例婴儿出现继发性静脉积水病症（巨头症、室管膜下萎缩和脑室扩张）；12例在儿童期被发现。由于转诊团队决定不遵循我们的治疗建议，我们无法跟踪10%的患者。17%的病例存在治疗禁忌（伴有早期脑损伤和快速致命的结果）。有5例（4%）自发血栓形成（但其中只有2例神经功能正常）。78例患者通过经股动脉途径使用胶水进行了栓塞治疗。53%的患者已实现解剖学治愈。在接受治疗的患者中，即使治疗尚未完成，80%的患者神经发育正常。与病变自然病程相关的永久性神经功能缺损或不可逆的神经认知延迟在8.5%的患者中明显。总体死亡率为9%。这些数据证实，之前关于VGAM预后的观点应该修正。动脉内栓塞是目前首选的治疗方法。
