Belzic I, Yaseen H, Voirin J, Bonte J B, Laloum D
Service de Néonatologie, CHU Clemenceau, Caen.
Ann Pediatr (Paris). 1992 May;39(5):301-4.
Cerebral arteriovenous malformations with neonatal manifestations are infrequent and virtually always fatal. Heart failure with an intracranial bruit is the most common presentation. Exceptionally, the aneurysm is a manifestation of Rendu-Osler-Weber syndrome which is inherited on an autosomal dominant basis. Development of cerebral arteriovenous malformations occurs very early as demonstrated by the discovery of two aneurysms with major repercussions on the cerebral parenchyma in a female with severe prematurity. Pregnant women with suspected Rendu-Osler-Weber syndrome should undergo ultrasound studies targeted at identifying untreatable cerebral lesions antenatally.
具有新生儿表现的脑动静脉畸形并不常见,且几乎总是致命的。伴有颅内杂音的心力衰竭是最常见的表现。极少数情况下,动脉瘤是常染色体显性遗传的遗传性出血性毛细血管扩张症(Rendu-Osler-Weber综合征)的一种表现。正如在一名严重早产的女性中发现两个对脑实质有重大影响的动脉瘤所表明的那样,脑动静脉畸形在很早的时候就会发生。疑似患有遗传性出血性毛细血管扩张症的孕妇应接受超声检查,以便在产前识别无法治疗的脑部病变。