Lasjaunias P, Hui F, Zerah M, Garcia-Monaco R, Malherbe V, Rodesch G, Tanaka A, Alvarez H
Unité de Neuroradiologie Vasculaire Diagnostique et Thérapeutique, Centre Hospitalier de Bicêtre, Le Kremlin Bicêtre, France.
Childs Nerv Syst. 1995 Feb;11(2):66-79; discussion 79. doi: 10.1007/BF00303807.
Over the past 10 years (1982-1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed (n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment (n = 48), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group (n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result.
在过去10年（1982 - 1992年）中，我们积极参与了179例儿童和婴儿脑动静脉畸形（CAVMs）的治疗。其中77例为真正的大脑大静脉畸形（VGAMs），102例为软膜动静脉畸形（PAVMs），即在软膜下间隙形成。出血是50%的软膜动静脉畸形患儿的首发症状，但在所有大脑大静脉畸形病例中均未出现。仅31例儿童被认为不适合血管内治疗，124例中栓塞被作为主要治疗方法（进行了104次栓塞）。仅21例儿童接受了直接手术治疗（大脑大静脉畸形组无）。在已完成治疗的栓塞组（n = 56）中，8例儿童死亡，39例达到解剖学治愈，34例临床正常。在治疗中的组（n = 48）中，16例不正常。问题在于治疗程序的时机和目标（完全或部分治疗）。在未栓塞组（n = 31）中，13例软膜病变进行了手术（无死亡，2例有中度神经功能缺损）。在大脑大静脉畸形组中，18例中有13例死亡，4例发生自发性血栓形成（仅1例神经功能正常）。在未栓塞组中，13个病变已完全排除，但仅5例神经功能正常。这一事实再次强调了治疗前进行预后评估以及明确治疗目标的必要性。对大量已发表的关于儿童动静脉畸形治疗系列（1017例）的分析显示存在不一致性，这妨碍了正确的评估和比较。根据我们的经验，血管内治疗似乎始终是大脑大静脉畸形和软膜动静脉畸形的最佳初始治疗方法。然而，治疗这些病变的儿童需要一个庞大的多学科团队，这是提供最合适、最有效治疗的唯一途径，也是取得良好结果的唯一保证。
