Center for Endovascular Surgery, Hyman Newman Institute for Neurology and Neurosurgery, St. Luke's Roosevelt Hospital Center, New York, New York 10019, USA.
Neurosurgery. 2012 May;70(5):1207-13; discussion 1213-4. doi: 10.1227/NEU.0b013e3182417be3.
Untreated patients with symptomatic neonatal presentation of vein of Galen aneurismal malformations (VGAMs) carry almost 100% morbidity and mortality. Medical management and endovascular techniques for neonatal treatment have significantly evolved.
To evaluate the clinical and angiographic outcomes of modern management of neonates with refractory heart failure from VGAMs.
From 2005 to 2010, 16 neonatal patients with VGAM presented to our institution. Medical care from the prenatal to perinatal stages was undertaken according to specified institutional guidelines. Nine patients with refractory heart failure required neonatal endovascular intervention. All patients were treated by transarterial deposition of n-butyl cyanoacrylate into fistula sites. Short- and long-term angiographic studies and clinical outcomes were reviewed.
Control of heart failure was achieved in 8 patients. One premature baby died shortly after treatment. Long-term angiographic follow-up shows total or near-total angiographic obliteration in all 8 patients. One patient has a mild hemiparesis from treatment. Another has a mild developmental delay. One patient developed a severe seizure disorder and developmental delay. Overall, 66.7% patients have normal neurological development with near-total or total obliteration of the malformation.
Treatment of refractory heart failure in neonatal VGAM with modern prenatal, neurointensive, neuroanesthetic, and pediatric neuroendovascular care results in significantly improved outcomes with presumed cure and normal neurological development in most.
未经治疗的有症状新生儿静脉窦瘤样畸形（VGAMs）患者几乎 100%会出现发病率和死亡率。针对新生儿的医学治疗和血管内技术已经得到了显著的发展。
评估现代治疗方法对伴有难治性心力衰竭的新生儿静脉窦瘤样畸形的临床和血管造影结局。
2005 年至 2010 年，我们机构收治了 16 例新生儿 VGAM 患者。根据特定的机构指南，对围产前和围产期的患者进行了医疗护理。9 例有难治性心力衰竭的患者需要新生儿血管内介入治疗。所有患者均通过动脉内注射正丁基氰丙烯酸酯到瘘口部位进行治疗。回顾了短期和长期的血管造影研究和临床结果。
8 例患者的心力衰竭得到了控制。1 例早产儿在治疗后不久死亡。长期血管造影随访显示，所有 8 例患者的血管均完全或接近完全闭塞。1 例患者因治疗导致轻度偏瘫。另 1 例患者有轻度发育迟缓。1 例患者出现严重癫痫发作和发育迟缓。总体而言，66.7%的患者在畸形完全或接近完全闭塞的情况下，神经发育正常。
对新生儿 VGAM 伴难治性心力衰竭采用现代的围产前、神经重症、神经麻醉和儿科神经血管内治疗，可显著改善预后，大多数患者得到治愈且神经发育正常。
