Jones Blaise V, Ball William S, Tomsick Thomas A, Millard Justin, Crone Kerry R
Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
AJNR Am J Neuroradiol. 2002 Nov-Dec;23(10):1717-24.
We herein present our experience in diagnosing and treating 13 children with vein of Galen aneurysmal malformations (VGAM), with an emphasis on possible prognostic indicators, endovascular strategies, factors affecting treatment during the neonatal period, and long-term follow-up. With this review, we hope to identify those factors that have the most significant prognostic value in determining long-term outcomes in children with VGAM.
We retrospectively reviewed the radiology studies, hospital charts, and outpatient clinic chart notes (when applicable) of 13 children evaluated and treated for VGAM at a single tertiary care pediatric hospital. Clinical presentation, diagnostic methods, treatment strategies, and outcome were documented for each child. The present neurologic status and level of function of each patient was determined by review of the outpatient charts and direct contact with the clinicians who were conducting the follow-up. Outcome was graded on a 5-point scale, ranging from 0 (death) to 4 (normal), taking into account only neurologic and developmental characteristics.
Eight of 13 patients presented as neonates with congestive heart failure. The other five patients ranged in age from 4 months to 13 years at the time of presentation. The five patients presenting outside of the neonatal period achieved normal or near-normal outcomes. Two of the eight patients presenting during the neonatal period achieved normal or near-normal outcomes, one experienced significant impairment, and the other five died. We were unable to identify significant differences in outcome on the basis of differences in treatment strategies.
Our experience confirms that children with VGAM presenting during the neonatal period have a generally much worse prognosis than do those presenting later in childhood. Complicating factors in the management and treatment of these children are discussed in light of their impact on outcome.
本文介绍了我们诊断和治疗13例大脑大静脉动脉瘤样畸形（VGAM）患儿的经验，重点关注可能的预后指标、血管内治疗策略、影响新生儿期治疗的因素以及长期随访情况。通过本综述，我们希望确定那些在决定VGAM患儿长期预后方面具有最重要预后价值的因素。
我们回顾性分析了在一家三级儿科专科医院接受评估和治疗的13例VGAM患儿的放射学检查、医院病历以及门诊病历记录（如适用）。记录了每个患儿的临床表现、诊断方法、治疗策略和结果。通过查阅门诊病历并直接与进行随访的临床医生联系，确定了每位患者目前的神经状态和功能水平。仅考虑神经和发育特征，结果采用5分制进行分级，从0（死亡）到4（正常）。
13例患者中有8例在新生儿期表现为充血性心力衰竭。其他5例患者就诊时年龄在4个月至13岁之间。在新生儿期以外就诊的5例患者获得了正常或接近正常的结果。在新生儿期就诊的8例患者中有2例获得了正常或接近正常的结果，1例出现明显损伤，另外5例死亡。我们无法根据治疗策略的差异确定结果的显著差异。
我们的经验证实，新生儿期出现的VGAM患儿的预后通常比儿童期后期出现的患儿差得多。鉴于这些因素对结果的影响，讨论了这些患儿管理和治疗中的复杂因素。
