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Classically, optic nerve hypoplasia is viewed as a congenital anomaly with a small disc and poor vision. However, recently "segmental" hypoplasia, with good visual acuity and localized visual field defect (VFD), have often been encountered. The most frequent abnormality is lower wedge-shaped VFD oriented to the blind spot. As this indicates that the superior part of the optic nerve is defective, it is termed ,'superior segmental optic nerve hypoplasia' (SSOH). 'Topless disc', in which the upper part of the optic disc looks hypoplastic, was once considered to be a characteristic of SSOH. In fact, the optic disc in SSOH is, variable, e.g., it contains a small disc, topless disc, large cupping or double ring sign. However, in all cases nerve fiber layer defect corresponding to the VFD exists. In the West, many authors have suggested that SSOH is a sign of gestational diabetes, but the data in Japan does not support this view. Currently in Japan, the differential diagnosis between SSOH with large cupping and normal-tension glaucoma is under debate, given the recent popularity of screening examination for glaucoma. While VFD does not progress during patients' youth, there is nothing known about the course in elderly patients. Thus, for the time being, long-term follow-ups of patients with segmental optic nerve hypoplasia are recommended. LA: Japanese
Dr. M. Takagi, Divisions of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Japan
10 Differential diagnosis e.g. anterior and posterior ischemic optic neuropathy
6.6.2 Automated (Part of: 6 Clinical examination methods > 6.6 Visual field examination and other visual function tests)