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[视神经发育不全与视隔发育不良]

[Optic nerve hypoplasia and septo-optic dysplasia].

作者信息

Lohmüller R, Gangloff A-S, Wenzel F, Lagrèze W A

机构信息

Molekulare und Technische Medizin, Hochschule Furtwangen University, Furtwangen, Deutschland.

Medizinische Diagnostik, Hochschule Furtwangen University, Furtwangen, Deutschland.

出版信息

Ophthalmologe. 2017 Aug;114(8):759-766. doi: 10.1007/s00347-017-0535-6.

Abstract

Optic nerve hypoplasia (ONH) is one of the most common causes of congenital visual impairment. It was first described in 1915 and represents a developmental disorder of the central nervous system. It is often associated with intracranial midline defects and is then referred to as septo-optic dysplasia (SOD). The symptoms of ONH range from minimal visual dysfunction to significant visual impairment with sensory defect nystagmus and even blindness. The ONH is often associated with further systemic, endocrinological and neurological abnormalities requiring a close interdisciplinary treatment of the patients.

摘要

视神经发育不全(ONH)是先天性视力障碍最常见的病因之一。它于1915年首次被描述,是一种中枢神经系统发育障碍。它常与颅内中线缺陷相关,此时被称为视隔发育不良(SOD)。视神经发育不全的症状从轻微的视觉功能障碍到伴有感觉缺陷性眼球震颤甚至失明的严重视力损害不等。视神经发育不全常与进一步的全身、内分泌和神经异常相关,需要对患者进行密切的多学科治疗。

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