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Photo Essay
February 1998

Progression of Papillomacular Congenital Hypertrophy of the Retinal Pigment Epithelium Associated With Impaired Visual Function

Author Affiliations


Arch Ophthalmol. 1998;116(2):256. doi:10.1001/archopht.116.2.256

A 48-YEAR-OLD man visited our clinic with decreased vision in his left eye. His best-corrected visual acuity was 6/20 OS on the Snellen chart. Fundus examination revealed a large papillomacular pigmented lesion (Figure 1) with a 1-mm elevation on a standardized A- and B-mode ultrasonographic scan. The hyperpigmented lesion showed several punched-out lacunae of depigmentation and was surrounded by a marginal halo of depigmentation with a hyperpigmented rim; this appears as a double outline.1 Fluorescein angiography demonstrated a sharp delineated lesion with hypofluorescence and window defects, with no signs of choroidal or retinal vessel leakage (Figure 2). Microaneurysmal capillary ecstasies could be seen in the papillomacular region.2 Additional vision field examination (using Octopus 101, Interzeag, Haag Streit, Switzerland) showed a large, deep cecocentral scotoma that did not correspond completely with the extension of the ophthalmoscopic lesion (Figure 3). An ophthalmic examination of the patient's right eye yielded no significant information.