Abstract
Of 237 patients seen in the Neuro-Ophthalmology Division of the Jules Stein Eye Institute 1986 2009, coded as "congenital optic nerve anomaly", 13 demonstrated focal superonasal flattening of the optic disc margin and nerve fibre layer thinning, with corresponding inferotemporal arcute visual field defect. In 11 cases, an unusual finding of margination of the visual field defects along the temporal horizontal midline was detected. In only two cases was a history of maternal diabetes positive. Magnetic resonace scans showed no additional brain abnormalities. A syndrome of focal superonasal optic nerve hypoplasia is identified, distinct from the superior segmental optic nerve hypoplasia syndrome.