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[大脑大静脉动脉瘤样畸形:一例具有不寻常病程的新生儿病例]

[Vein of Galen aneurysmal malformation: a neonatal case with unusual evolution].

作者信息

Cherif A, Néji K, Sebaï L, Ben Jemaa W, Bellagua I, Jebnoun S, Khrouf N

机构信息

Service de néonatologie, centre de maternité et de néonatologie, Tunis, Tunisie.

出版信息

Arch Pediatr. 2007 Jul;14(7):893-6. doi: 10.1016/j.arcped.2007.01.019. Epub 2007 Apr 23.

Abstract

We report on a neonatal patient case with a cerebral vascular dilation consistent with a vein of Galen malformation diagnosed on an antenatal doppler ultrasound examination. Antenatal diagnosis was confirmed by fetal MRI scan. After delivery, cerebral MRI scan found the same dilatation and showed no cerebral parenchymal damage. The infant's condition was stable and particularly there was no congestive cardiac failure. Before discharge at ten days of age, clinical examination and particularly cardiac examination was normal. At 14 days of age, symptoms in relation to a congestive cardiac failure appeared. Echocardiography showed a high output cardiac failure with no cardiac malformation. Infectious laboratory tests were normal. Evolution was initially favourable after digitalo-diuretic treatment. Unfortunately, death occurred at 36 days of age due to intractable cardiac failure. This case highlights that, unusually, heart failure can occur long after the first days of life in cases of Galen vein aneurysmal malformation. The optimal time for vascular embolization in neonatal cases of Galen vein aneurysmal malformation is discussed.

摘要

我们报告了一例新生儿病例,该患儿在产前多普勒超声检查中被诊断为符合大脑大静脉畸形的脑血管扩张。胎儿MRI扫描证实了产前诊断。分娩后,脑部MRI扫描发现了相同的扩张,且未显示脑实质损伤。婴儿状况稳定,尤其没有充血性心力衰竭。在出生十天出院前,临床检查尤其是心脏检查正常。在14日龄时,出现了与充血性心力衰竭相关的症状。超声心动图显示为高输出量心力衰竭,无心脏畸形。感染性实验室检查正常。在使用洋地黄利尿剂治疗后,病情最初进展顺利。不幸的是,患儿在36日龄时因顽固性心力衰竭死亡。该病例突出表明,在大脑大静脉动脉瘤畸形病例中,不同寻常的是,心力衰竭可能在出生后的头几天很久之后才出现。本文讨论了新生儿大脑大静脉动脉瘤畸形病例中血管栓塞的最佳时机。

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