Aneurysmal Malformations of the Vein of Galen
Aneurysmal Malformations of the Vein of Galen
复制标题
盖伦静脉动脉瘤畸形
DOI：
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发表时间：
1996
影响因子：
1.7
通讯作者：
W. Taylor
中科院分区：
文献类型：
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作者：
P. Lasjaunias;H. Alvarez;G. Rodesch;R. Garcia;K. T. Brugge;Burrows Pe;W. Taylor
Galen静脉动脉瘤畸形（VGAM）被认为是一种自发性和治疗中发病率和死亡率较高的病变，部分原因是对病变对幼儿特定生理学的影响认识不完全。此外，在短时间内已经应用了各种技术来治疗病变，而不是它所造成的疾病。我们报告的经验，120例连续VGAM管理在过去十年中：24个产前诊断; 50在新生儿期出现血流动力学障碍; 35在婴儿出现继发性静脉积水疾病（大颅，室管膜下萎缩和脑室扩张）; 12个被认为是在儿童。我们无法跟踪10%的患者，因为转诊团队决定不遵循我们的治疗建议。17%的病例禁忌治疗（早期脑损伤和快速致死性结局）。有5例（4%）自发血栓形成（但其中只有2例神经功能正常）。78例患者通过经动脉股动脉入路使用胶水进行栓塞。53%的患者已经实现了解剖治愈。在接受治疗的患者中，即使治疗尚未完成，80%的患者的神经功能正常。8.5%的患者存在与病变自然史相关的永久性神经功能缺损或不可逆的神经认知延迟。总死亡率为9%。这些数字证实，以前对VGAM预后的看法应予以修订。动脉内栓塞是迄今为止的首选治疗。
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 (macrocrania, 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.