Disc excavation in dominant optic atrophy

Objective: In patients with dominant optic atrophy (DOA, Kjer type), excava tion of the optic nerve develops, and these patients may be misdiagnosed as having normal tension glaucoma (NTG). This study examined disc morphologic features in patients with DOA and explored features that help distinguish this condition from NTG. Design: Noncomparative, observational case series. Participants: Patients with DOA who were seen at the Duke University Eye Ce nter between 1987 and 1996 and who had bilateral optic nerve photographs. Methods. Retrospective chart review of the results of visual acuity testing , visual field testing by Goldmann perimetry, color vision testing, intraoc ular pressure measurement, and observation of bilateral optic nerve photogr aphs. Main Outcome Measures: Appearance of the optic disc and peripapillary zone in patients with DOA. Results. Nine patients were identified. The mean age at the time of evaluat ion was 28 years (range, 11-62 years). Most patients had a mild to moderate reduction in visual acuity. Color vision as tested with Hardy-Rand-Rittler plates was reduced (4.0/10 +/- 4.2/10). A cup-to-disc ratio of more than 0 .5 was observed in at least one eye of eight patients. A temporal wedge-sha ped area of excavation was observed in 14 of the 18 eyes studied. Moderate to severe temporal pallor was observed in all of the eyes. Pallor of the re maining (noncupped) neuroretinal rim was also observed consistently, rangin g from mild to moderate. A gray crescent and some degree of peripapillary a trophy were noted in all eyes. Conclusions. Several clinical features, including early age of onset, prefe rential loss of central vision, sparing of the peripheral fields, pallor of the remaining neuroretinal rim, and a family history of unexplained visual loss or optic atrophy, help to distinguish patients with DOA from those wi th NTG. (C) 2001 by the American Academy of Ophthalmology.