新生儿盖伦动脉瘤样静脉畸形的影像学和临床特征及血管内介入治疗的结果:10年经验

Sinan Tufekci, Z. Ince, B. Yaşa, Meltem Bor, M. Barburoglu, S. Sencer, A. Coban
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引用次数: 2

摘要

目的:探讨新生儿盖伦静脉畸形(VGAM)的临床特点、诊断、治疗及预后。方法:回顾性分析8例新生儿期VGAM患者的临床表现、诊断、治疗策略及随访情况。幸存的4名患者中有3名接受了神经学评估,而1名由于搬到另一个城市而失去了随访。结果:8例患者中有7例获得产前诊断。在所有病例中,从出生的第一天起就出现严重的心力衰竭和肺动脉高压,随后几天出现低血压、多器官衰竭、脑积水和癫痫发作。应用颅磁共振成像和磁共振血管造影对VGAM及其供血动脉进行定位。经动脉栓塞治疗7例,其中4例存活,3例死亡,1例在干预前死亡。结论:VGAM因其复杂的解剖、病理生理和特点,致死率和发病率高,存活者可留下严重的神经系统后遗症。通过积极的医疗支持和早期血管内栓塞治疗,可以改善高危新生儿的预后。
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Radiological and Clinical Features of Vein of Galen Aneurysmal Malformation in Newborn Infants and, the Results of Endovascular Interventional Treatment: 10-Years Experience
Aim: To assess the clinical features, diagnosis, treatment and prognosis of newborn infants with a diagnosis of Vein of Galen Malformation (VGAM)during a 10-year period. Method: Eight patients with a diagnosis of VGAM in the neonatal period were assessed retrospectively in terms of clinical signs, diagnosis, treatment strategies and follow-up. Three of four patients who survived had neurological assessment whereas one was lost to follow-up because of moving to another city. Results: Seven of 8 patients had an antenatal diagnosis. In all cases, severe heart failure and pulmonary hypertension were present from the first day of life and hypotension, multiorgan failure, hydrocephaly and seizures developed in the following days. VGAM and its feeder arteries were mapped by cranial magnetic resonance imaging and magnetic resonance angiography. Transarterial embolization therapy was performed on 7 patients, of whom four babies survived and three babies died, while one patient died before any intervention. Conclusion: The mortality and morbidity rates of VGAM is high because of its mixed anatomy, pathophysiology and characteristic features leading to severe neurological sequelae in the survivors. Prognosis in high risk neonates can be improved with aggressive medical support and early endovascular embolization therapy.
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