Foveal congenital hypertrophy of the retinal pigment epithelium in the setting of geographic atrophy from age-related macular degeneration.
OBJECTIVE
To report a case of presumed congenital hypertrophy of the retinal pigment epithelium in the fovea of an 88-year-old woman in the setting of geographic atrophy from age-related macular degeneration.


METHODS
Observational case report.


METHODS
An 88-year-old woman was examined.


RESULTS
Best-corrected visual acuity was 20/63 in the right eye and 20/50 in the left eye. Multifocal areas of geographic atrophy and large-sized drusen were seen in the maculae of both eyes. Biomicroscopic examination of the right eye showed hyperpigmentation consistent with congenital hypertrophy of the retinal pigment epithelium through the center of the macula. No prior photographic documentation of the retina was available.


CONCLUSIONS
This case suggests that foveal congenital hypertrophy of the retinal pigment epithelium may be seen in the setting of macular geographic atrophy. Although it is theoretically possible that the hyperpigmentation is reactive rather than congenital, the pigmentation is typical for congenital hypertrophy and is unlike any reactive pigmentation in our experience or described in a MEDLINE search of features of age-related macular degeneration. The case suggests that a hypertrophic process of the retinal pigment epithelium may coexist within or immediately adjacent to the anatomic boundaries of an atrophic process such as geographic atrophy from age-related macular degeneration.
Apte
Rajendra S
RS
Retinal Vascular Center, The Wilmer Ophthalmological Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Bressler
Neil M
NM
D000368:Aged; D000369:Aged, 80 and over; D001284:Atrophy; D005260:Female; D006801:Humans; D006984:Hypertrophy; D008268:Macular Degeneration / Q000150:complications*; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012160:Retina / Q000473:pathology*; D012164:Retinal Diseases / Q000209:etiology*