Paumier A, Winer N, Joubert M, Yvinec M, Aubron F, Sagot S, Quere M P, Boog G
Service d'Obstétrique et de Médecine Foetale, Hôpital Mère et Enfant, CHU, Nantes.
J Gynecol Obstet Biol Reprod (Paris). 1998 Dec;27(8):814-20.
Aneurysm of the vein of Galen is a rare congenital disease, with a generally poor prognosis. It represents less than 1% of cerebral arteriovenous malformations. The intrauterine diagnosis of this vascular anomaly has been facilitated by vascular exploration with pulsed and color Doppler sonography. According to some studies focused on prognostical criteria, the perinatal prognosis seems to be invariably compromised when signs of cardiac decompensation develop prenatally, knowing that early delivery may generate additional complications in the very premature infant. Medical treatment is inefficient, because of the large, persisting systemic shunt, so that pregnancy termination may be performed, after parental consent, in some cases demonstrating all signs of cardiac decompensation. On the other hand, infants with normal velocity waveforms and a low extent of the systemic shunt have a good extrauterine adaptation. A full clinical evaluation of the affected neonates at the end of the first week of live is now the best way to select the infants for intravascular embolization.
大脑大静脉动脉瘤是一种罕见的先天性疾病,预后通常较差。它占脑动静脉畸形的比例不到1%。通过脉冲和彩色多普勒超声进行血管探查有助于在子宫内诊断这种血管异常。根据一些关注预后标准的研究,当产前出现心脏代偿失调迹象时,围产期预后似乎总是受到影响,因为早产能给极早产儿带来更多并发症。由于存在大的、持续的体循环分流,药物治疗无效,因此在某些出现心脏代偿失调所有迹象的病例中,经父母同意后可终止妊娠。另一方面,具有正常速度波形且体循环分流程度低的婴儿宫外适应良好。对存活第一周末的患病新生儿进行全面临床评估,是现在选择婴儿进行血管内栓塞的最佳方法。