Hosmann Arthur, El-Garci Ahmed, Gatterbauer Brigitte, Bavinzski Gerhard, Knosp Engelbert, Gruber Andreas
Department of Neurosurgery, Medical University of Vienna, Vienna, Austria.
Department of Neurosurgery, Medical University of Vienna, Vienna, Austria.
World Neurosurg. 2018 Apr;112:e837-e847. doi: 10.1016/j.wneu.2018.01.172. Epub 2018 Feb 3.
The vein of Galen aneurysmal malformation (VGM) is a rare intracranial arteriovenous fistula with a dramatic manifestation during infancy and 100% mortality without treatment. Therapeutic strategies for VGMs have changed over time as a result of advances in endovascular techniques. We present our experience and multimodality approach within the last 4 decades.
A retrospective analysis and angiographic review were performed of patients with VGM between 1975 and 2016 at our institution.
Eighteen consecutive patients were identified, including 10 with choroidal and 8 with mural VGMs. In 37 endovascular interventions, a transarterial approach was mostly performed (82.8%). One patient was initially treated surgically and received 2 Gamma Knife treatments after multiple embolizations. The preferred embolization agent was histoacryl for choroidal VGMs and a combination of coils and histoacryl for mural VGMs. Total occlusion was achieved in 87.5% of mural VGMs and 11.1% of choroidal VGMs. Cerebral hemorrhages and infarction occurred only in patients younger than 1 month. At a median follow-up interval of 4.6 years, good outcome was achieved in 53.8% and poor outcome in 46.2%, with an overall mortality of 16.7%. Poor outcome was significantly associated with choroidal-type VGMs, age <1 month at first embolization, and incomplete occlusion.
Endovascular embolization using a transarterial approach is the therapy of choice. Gamma Knife radiosurgery can be considered as second-line therapy in a multimodal approach. Whereas treatment within the first month of life is associated with the highest mortality and complications, we did not detect any severe adverse events thereafter.
大脑大静脉动脉瘤样畸形（VGM）是一种罕见的颅内动静脉瘘，在婴儿期表现显著，未经治疗死亡率达100%。由于血管内技术的进步，VGM的治疗策略随时间发生了变化。我们介绍过去40年里我们的经验和多模式治疗方法。
对1975年至2016年在我院诊断为VGM的患者进行回顾性分析和血管造影复查。
共确定18例连续患者，其中脉络丛型VGM 10例，壁型VGM 8例。在37次血管内介入治疗中，大多采用经动脉入路（82.8%）。1例患者最初接受手术治疗，在多次栓塞后接受了2次伽玛刀治疗。脉络丛型VGM首选的栓塞剂是组织黏合剂，壁型VGM则采用弹簧圈和组织黏合剂联合使用。壁型VGM的完全闭塞率为87.5%，脉络丛型VGM为11.1%。脑出血和梗死仅发生在1个月以下的患者中。中位随访期为4.6年，53.8%的患者预后良好，46.2%的患者预后不良，总死亡率为16.7%。预后不良与脉络丛型VGM、首次栓塞时年龄<1个月以及闭塞不完全显著相关。
经动脉入路的血管内栓塞是首选治疗方法。伽玛刀放射外科可作为多模式治疗方法中的二线治疗。虽然出生后第一个月内进行治疗与最高的死亡率和并发症相关，但此后我们未发现任何严重不良事件。
