PMC5903099_01
Congenital simple hamartoma of retinal pigment epithelium (CSHRPE) in a 41-year-old Japanese woman. a Photograph of the fundus of the left eye at the initial examination shows a clear black lesion in the foveal region and a myelinated nerve fiber in the nasal area. b Spectral-domain optical coherence tomography (OCT) at the initial evaluation shows a hyperreflective lesion that spreads from the outer plexiform layer through the inner surface, protruding into the vitreous cavity, with deep optical shadowing at the first examination. c Photograph of the fundus 3 years after the first examination. d OCT 3 years after the first examination. e Photograph of the fundus 10 years after the first examination. f OCT 10 years after the first examination g Fundus autofluorescence showed hypoautofluorescence at the site corresponding to the lesion.
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F1
A black lesion was apparent in the foveal region, and a myelinated nerve fiber was identified in the nasal area of the left fundus (Fig. 1a). Spectral-domain OCT (Cirrus; Carl Zeiss Meditec, Inc., Dublin, CA, USA) revealed a hyperreflective lesion from the outer plexiform layer through the inner surface, protruding into the vitreous cavity, and producing deep optical shadowing (Fig. 1b). After her 10-year follow-up, the lesion remained same, and OCT showed no change since her initial evaluation (Fig. 1d, f). In addition, fundus autofluorescence showed hypoautofluorescence at the site corresponding to the lesion (Fig. 1g).
Multifocal electroretinography of both eyes (a right, b left) shows normal patterns.
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F2
B-scan did not show any specific findings such as nodular elevation in the retina, sclera, and orbita. mfERG of both eyes showed normal patterns (Fig. 2).
Optical coherence tomography angiography was used to analyze the vascular flow of the superficial plexuses (a), deep plexuses (b), outer retina (c), and choriocapillaris (d). Vascular networks appeared at the superficial and deep plexuses. No blood flow was detected in the tumor at the outer retina or choriocapillaris.
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F3
OCTA (Cirrus PLEX Elite 9000; Carl Zeiss Meditec, Inc., Dublin, CA, USA) showed vascular flow in the superficial plexuses (Fig. 3a), deep plexuses (Fig. 3b), outer retina (Fig. 3c), and choriocapillaris (Fig. 3d).