Topless optic disk syndrome without maternal diabetes mellitus.

Abstract

To describe four cases of topless optic disk syndrome without maternal diabetes mellitus. Four patients had incidentally discovered inferior visual field defects. Ophthalmoscopic examinations in all four patients disclosed superiorly displaced entrances of the central retinal artery and thinning of the superior peripapillary nerve fiber layers. One patient had a superior peripapillary crescent with pallor of the superior disk. These clinical findings were consistent with a diagnosis of superior segmental optic hypoplasia, the topless disk. None of the patients had mothers who had diabetes. The topless optic disk syndrome can occur in the absence of maternal diabetes mellitus.