目的:颅内动静脉分流术(IAVS)是婴幼儿罕见的血管疾病。它们可以分为Galen动脉瘤畸形(VGAM)的静脉,静脉动静脉瘘(PAVF),与硬脑膜窦畸形(DAVF/DSM)相关的硬脑膜动静脉瘘。我们试图回顾临床表现,成像特性,血管内治疗(EVT),以及十年来到四级儿科转诊中心就诊的婴儿的IAVS结局。
方法:对2011年1月至2021年1月在四级儿科转诊中心诊断为IAVS的所有婴儿进行前瞻性维护数据库的回顾性回顾。对于每个病人来说,人口统计数据,临床表现,影像学发现,管理策略,并对结果进行了回顾和讨论。
结果:在研究期间,连续38名婴儿被诊断为IAVS。VGAM患者(23/38,60.5%)表现为先天性心力衰竭(CHF)(14/23),脑积水(4/23),和癫痫发作(2/23),三名患者无症状。18例VGAM患者接受了EVT。其中,13例患者(72.2%)成功通过血管造影治愈,3例患者(3/18,17%)死亡。PAVF(9/38,23.7%)患者出现CHF(5/9),颅内出血(2/9),和癫痫发作(2/9),他们都成功地进行了血管内治疗。I型DAVF/DSM患者(4/6,66.6%)表现为质量效应(2/4),脑静脉高压(1/4),CHF(1/4),和脑面部静脉同色异类综合征(1/4)。II型DAVF/DSM患者(2/6,33.3%)在耳后表现出兴奋感。DAVF/DSM患者接受血管内治疗,五名患者治愈,一个I型DAVF/DSM死亡。
结论:颅内动静脉分流是婴儿罕见但可能危及生命的神经血管病变。血管内治疗具有挑战性,但对精心选择的患者是可行的。
OBJECTIVE: Intracranial arteriovenous shunts (IAVS) are rare vascular diseases in infants. They can be categorized into vein of Galen aneurysmal malformation (VGAM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula associated with dural sinus malformation (DAVF/DSM). We sought to review the clinical presentation, imaging characteristics, endovascular treatment (EVT), and outcomes of IAVS in infants presenting to a quaternary pediatric referral center over one decade.
METHODS: A retrospective review of a prospectively maintained database was performed of all infants diagnosed with IAVS between January 2011 and January 2021 in a quaternary pediatric referral center. For each patient, demographic data, clinical presentation, imaging findings, management strategies, and outcomes were reviewed and discussed.
RESULTS: Over the study period, 38 consecutive infants were diagnosed with IAVS. Patients with VGAM (23/38, 60.5%) presented with congenital heart failure (CHF) (14/23), hydrocephalus (4/23), and seizures (2/23), and three patients were asymptomatic. Eighteen patients with VGAM underwent EVT. Among those, 13 patients (72.2%) were successfully treated with an angiographic cure and three patients (3/18, 17%) died. Patients with PAVF (9/38, 23.7%) presented with CHF (5/9), intracranial hemorrhage (2/9), and seizures (2/9), and all of them were successfully treated endovascularly. Patients with Type I DAVF/DSM (4/6, 66.6%) presented with mass effect (2/4), cerebral venous hypertension (1/4), CHF (1/4), and cerebrofacial venous metameric syndrome (1/4). Patients with type II DAVF/DSM (2/6, 33.3%) presented with a thrill behind the ear. Patients with DAVF/DSM were treated endovascularly, five patients were cured, and one with type I DAVF/DSM died.
CONCLUSIONS: Intracranial arteriovenous shunts are rare but potentially life-threatening neurovascular pathologies in infants. Endovascular treatment is challenging but feasible in carefully selected patients.