Ryu Bikei, Sato Shinsuke, Yamaguchi Koji, Abe Keiichi, Ottomo Daiki, Okada Yoshikazu
Department of Neurosurgery, Tokyo Women's Medical University.
No Shinkei Geka. 2013 Sep;41(9):773-7.
Noonan syndrome(NS)is a common, clinically and genetically heterogeneous condition characterized by distinctive facial features, short stature, congenital heart disease, and other comorbidities. However, there have been only a few case reports of cerebrovascular abnormalities in patients with NS. Especially, the cases of intracranial aneurysms with NS are very rare. In addition, comorbidities of azygos anterior cerebral artery(ACA)and NS have not been reported before now. We encountered such a rare case of azygos ACA unruptured aneurysm in patient with NS. The case was 64-year-old woman with NS complaining of gait disturbance. The three-dimensional CT angiography(3D-CTA)showed azygos ACA and an unruptured saccular aneurysm at the distal end of the A2 segment. An operation for this aneurysm was performed. We reviewed clinical features of NS with cerebrovascular disease such as aneurysm.
努南综合征（NS）是一种常见的疾病，在临床和基因方面具有异质性，其特征为独特的面部特征、身材矮小、先天性心脏病及其他合并症。然而，关于NS患者脑血管异常的病例报告仅有少数几例。特别是，NS合并颅内动脉瘤的病例非常罕见。此外，此前尚未有关于奇静脉大脑前动脉（ACA）与NS合并症的报道。我们遇到了这样一例罕见的NS患者合并奇静脉ACA未破裂动脉瘤的病例。该病例为一名64岁患有NS的女性，主诉步态障碍。三维CT血管造影（3D - CTA）显示奇静脉ACA以及A2段远端一个未破裂囊状动脉瘤。针对该动脉瘤进行了手术。我们回顾了NS合并脑血管疾病如动脉瘤的临床特征。
