Acute zonal occult outer retinopathy. Donders Lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992.

PURPOSE: This report describes 13 patients, predominantly young women, with a syndrome characterized by rapid loss of one or more large zones of outer retinal function, photopsia, minimal funduscopic changes, and electroretinographic abnormalities affecting one or both eyes. All patients on follow-up examination had persistent visual field defects, and most had chronic photopsia and zones of pigment epithelial atrophy. Evidence is presented that these patients probably represent part of the spectrum of a single disorder that includes the multiple evanescent white-dot syndrome (MEWDS), acute idiopathic blind-spot-enlargement syndrome (AIBSES), acute macular neuroretinopathy (AMN), and the pseudo-presumed ocular histoplasmosis syndrome (P-POHS).

METHODS: The medical records of these 13 patients and 2 additional patients, who developed, in addition to the features of this syndrome, funduscopic changes typical of MEWDS, AMN, and P-POHS, were reviewed and follow-up obtained.

RESULTS: These patients had extensive unrewarding medical and neurological investigations because of suspected diagnoses, including central nervous system disorders, cancer-associated retinopathy, retinal vasculitis, diffuse unilateral subacute neuroretinitis, and tapetoretinal degenerations. Although most patients retained good visual acuity, all had permanent visual field loss that in some cases was severe. The cause of the disorder was not determined. No effective treatment was found.

CONCLUSIONS: Acute visual loss and photopsia in these patients is probably caused by damage to large zones of the outer retina that appears unaffected ophthalmoscopically. Electroretinography is important in early diagnosis. Future investigations probably will reveal further evidence linking this disorder to MEWDS, AIBSES, AMN, and P-POHS.