Neurology:牵牛花视盘发育异常

2018-11-07 zyx整理 神经科病例撷英拾粹

7个月大女婴,表现为斜视。眼底镜检查(图A)提示右眼典型的牵牛花视盘发育异常(morning glory disc anomaly,MGDA),包括视神经盘扩大的漏斗样凹陷,视乳头周围色素环和视网膜血管辐射状分布。

7个月大女婴,表现为斜视。眼底镜检查(图A)提示右眼典型的牵牛花视盘发育异常(morning glory disc anomaly,MGDA),包括视神经盘扩大的漏斗样凹陷,视乳头周围色素环和视网膜血管辐射状分布。脑MRI(图B)显示视神经附着处凹陷伴异常组织,符合胶质簇。由MGDA引起的症状是多种多样的且并不总是存在:受累眼睛视敏度异常(通常较差),视野缺损和盲点扩大是最常见的症状。可出现浆液性视网膜脱落。通常为单侧。MRI有助于诊断和发现相关的颅内异常,特别是中线部位发育异常,如经蝶窦基底脑膨出和moyamoya综合征。MGDA没有理想的治疗方法;然而,提升视力对于预防弱视非常重要。


(图:A:右眼眼底检查可见视盘[*]漏斗状凹陷,视乳头周围区域[箭头]的色素环以及视网膜血管辐射状分布[箭头];B:T2WI可见视神经附着处凹陷[箭]和胶质簇形成[箭头])

原始出处:Poillon G, Gillard P, Lecler A. Teaching NeuroImages: Morning glory disc anomaly. Neurology. 2018 Oct 9;91(15):e1457-e1458. 

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    2019-03-23 yinhl1978
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    2018-11-08 neurowu