Paternoster Delia M, Manganelli Francesca, Moroder Wolfgang, Nicolini Umberto
Department of Gynaecology and Human Reproduction, University of Padova, Padova, Italy.
Fetal Diagn Ther. 2003 Nov-Dec;18(6):408-11. doi: 10.1159/000073133.
Vein of Galen aneurysmal malformations are rarely seen intracranial malformations. They represent less than 1% of the cerebral arteriovenous malformations. Prenatal diagnosis of an arteriovenous fistula malformation may be achieved by real-time and Doppler sonography with color flow imaging, by identifying dilated veins and arteriovenous shunts with turbulent flow. In addition, an elevated cardiac output may be observed and correlated with the magnitude of the cerebral arteriovenous shunt. The high incidence of cardiomegaly in neonates with arteriovenous malformations also suggests that high-output cardiac failure is already present in a significant number of cases during the 3rd trimester, and, therefore, treatment in utero may need to be considered to improve the hemodynamic status. In our case, all the above cardiovascular features were present during prenatal ultrasonography. The information on outcome compiled from the literature suggests that when an arteriovenous malformation is large enough to be detected prenatally, as in our case, it is likely to lead to cardiac failure either during the antenatal period or soon after birth. In this paper, we present the management of a case of 3rd-trimester diagnosis of a vein of Galen aneurysm associated with cardiac decompensation.
大脑大静脉动脉瘤样畸形是罕见的颅内畸形。它们占脑动静脉畸形的比例不到1%。动静脉瘘畸形的产前诊断可通过实时和彩色多普勒超声检查以及彩色血流成像来实现,通过识别扩张的静脉和伴有湍流的动静脉分流。此外,可能观察到心输出量增加,并与脑动静脉分流的程度相关。患有动静脉畸形的新生儿中心脏肥大的高发生率也表明,在妊娠晚期的相当多病例中已经存在高输出量心力衰竭,因此,可能需要考虑在子宫内进行治疗以改善血流动力学状态。在我们的病例中,产前超声检查时出现了上述所有心血管特征。从文献中收集的关于预后的信息表明,当动静脉畸形大到足以在产前被检测到时,如我们的病例,它很可能在产前或出生后不久导致心力衰竭。在本文中,我们介绍了一例妊娠晚期诊断为大脑大静脉动脉瘤并伴有心脏代偿失调的病例的处理情况。