Ozanne A, Alvarez H, Krings T, Lasjaunias P
Service de neuroradiologie-diagnostique et thérapeutique, hôpital de Bicêtre, 78 rue du Général-Leclerc, Le Kremlin-Bicêtre, Paris, France.
J Neuroradiol. 2007 Jul;34(3):145-66. doi: 10.1016/j.neurad.2007.04.005.
The purpose of this article is to give an overview of the cerebral and spinal cord pediatric malformations; we particularly describe three of them: Vein of Galen arteriovenous Malformation, Pial Arteriovenous Malformation, and Dural Sinus Malformation. We report the experience of Bicêtre since 1981 to 2003, with 317 VGAM, 302 Pial AVM and 30 DSM. We describe natural history, clinical and imaging features, endovascular management and the clinical and morphologic results. The clinical consequences of the VGAM and Pial AVM are systemic or cerebral. When they are cerebral they are mainly from hydrovenous type, as melting brain, or chronic hydrocephalus with calcifications and seizures. Depending of their angioarchitecture and amount of arteriovenous shunt, their gravity and the time of revealing are variable. Pial AVM, they carry the risk of hemorrhage, unlike VGAM. The presence of a fistula type should lead to suspect hereditary hemorrhagic telangiectasia. The main risk of DSM is hemorrhage due to thrombosis of the malformation, more than hydrovenous disorders. In VGAM, we obtain the complete cure of the shunt in 55% of case, and reduction of more than half of the shunt in 93.8%. Seventy-four percent of treated cases had a normal neurologic examination. DSM have a worse prognostic, and mortality is 38% despite treatment.
本文旨在概述小儿脑和脊髓畸形；我们特别描述其中三种：大脑大静脉动静脉畸形、软膜动静脉畸形和硬脑膜窦畸形。我们报告了1981年至2003年比塞特尔医院治疗317例大脑大静脉动静脉畸形、302例软膜动静脉畸形和30例硬脑膜窦畸形的经验。我们描述了其自然史、临床和影像学特征、血管内治疗以及临床和形态学结果。大脑大静脉动静脉畸形和软膜动静脉畸形的临床后果为全身性或脑部性的。当为脑部性时，主要为静脉性脑积水类型，如脑软化，或伴有钙化和癫痫发作的慢性脑积水。根据其血管构筑和动静脉分流的程度，其严重程度和发现时间各不相同。与大脑大静脉动静脉畸形不同，软膜动静脉畸形有出血风险。存在瘘管型应怀疑遗传性出血性毛细血管扩张症。硬脑膜窦畸形的主要风险是畸形血栓形成导致的出血，而非静脉性脑积水疾病。对于大脑大静脉动静脉畸形患者，55%的病例实现了分流的完全治愈，93.8%的病例分流减少超过一半。74%接受治疗的病例神经检查结果正常。硬脑膜窦畸形的预后较差，尽管接受了治疗，死亡率仍为38%。
