Chow Maggie L, Cooke Daniel L, Fullerton Heather J, Amans Matthew R, Narvid Jared, Dowd Christopher F, Higashida Randall T, Halbach Van V, Hetts Steven W
School of Medicine, University of California, San Francisco Medical School, San Francisco, California, USA.
Department of Interventional Neuroradiology, University of California, San Francisco, San Francisco, California, USA.
J Neurointerv Surg. 2015 Jun;7(6):443-8. doi: 10.1136/neurintsurg-2013-011005. Epub 2014 Apr 30.
Vein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized.
To examine the clinical and radiological features of VOGMs and the predictors of outcome in patients.
We retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ(2) analyses.
Forty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at <10 days of age and elective embolization after 1 year of age. Patients commonly presented with hydrocephalus (65.9%) and congestive heart failure (61.0%). Mixed-type (31.7%) VOGM was more common in our cohort than purely mural (29.3%) or choroidal (26.8%) types. The most common feeding arteries were the choroidal and posterior cerebral arteries. Transarterial embolization with coils was the most common technique used to treat VOGMs at our institution. Functional outcome was normal or only mildly disabled in 50% of the cases at last follow-up (median=3 years, range=0-23 years). Younger age at first diagnosis, congestive heart failure, and seizures were predictive of adverse clinical outcome. The survival rate in our sample was 78.0% and complete thrombosis of the VOGM was achieved in 62.5% of patients.
VOGMs continue to be challenging to treat and manage. Nonetheless, endovascular approaches to treatment are continuing to be refined and improved, with increasing success. The neurodevelopmental outcomes of affected children whose VOGMs are treated may be good in many cases.
大脑大静脉畸形（VOGM）是罕见且复杂的先天性动静脉瘘。VOGM的临床和影像学特征及其与临床结局的关系尚未完全明确。
研究VOGM的临床和影像学特征以及患者预后的预测因素。
我们回顾性分析了1986年至2013年在加利福尼亚大学旧金山分校接受治疗的所有VOGM患者的现有影像学和病历资料。确定了影像学和临床特征。我们应用改良Rankin量表通过病历审查确定功能结局。通过χ²分析评估预后的预测因素。
41例被确诊为VOGM。大多数患者（78%）在出生后第一年内被诊断为VOGM。治疗年龄呈双峰分布，主要是在<10日龄时进行紧急栓塞，1岁后进行择期栓塞。患者常见表现为脑积水（65.9%）和充血性心力衰竭（61.0%）。在我们的队列中，混合型（31.7%）VOGM比单纯壁型（29.3%）或脉络膜型（26.8%）更常见。最常见的供血动脉是脉络膜动脉和大脑后动脉。在我们机构，使用弹簧圈经动脉栓塞是治疗VOGM最常用的技术。在最后随访时（中位时间=3年，范围=0 - 23年），50%的病例功能结局正常或仅有轻度残疾。首次诊断时年龄较小、充血性心力衰竭和癫痫发作是不良临床结局的预测因素。我们样本中的生存率为78.0%，62.5%的患者实现了VOGM的完全血栓形成。
VOGM的治疗和管理仍然具有挑战性。尽管如此，血管内治疗方法仍在不断完善和改进，成功率不断提高。在许多情况下，接受治疗的患有VOGM的儿童的神经发育结局可能良好。
