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Int J Retina Vitreous. 2019 Jan 15;5:5. doi: 10.1186/s40942-018-0154-7. eCollection 2019.

Retinal sensitivity and photoreceptor arrangement changes secondary to congenital simple hamartoma of retinal pigment epithelium.

Author information

1Department of Ophthalmology, Ribeirão Preto School of Medicine, University of São Paulo, 3900, Bandeirantes Avenue, Ribeirão Prêto, SP 14049-900 Brazil.
Department of Ophthalmology, São José do Rio Preto School of Medicine, São José do Rio Preto, Brazil.
Department of Ophthalmology, D'Olhos, São José do Rio Preto, Brazil.
4Ocular Oncology Service, Wills Eye Hospital, Philadelphia, USA.



The congenital simple hamartoma of the retinal pigment epithelium is a benign lesion and previous observations with noninvasive imaging have detected potential photoreceptor abnormalities and retinal function interplay.

Case presentation:

A 35-year-old woman was found to have an asymptomatic, solitary, circumscribed, pigmented lesion in her left eye. The patient underwent ophthalmic examination including multimodal evaluation with fluorescein angiography, near-infrared reflectance scanning laser ophthalmoscopy, blue autofluorescence, enhanced-depth imaging spectralis B-scan optical coherence tomography (EDI-SBOCT), en face OCT angiography (OCT-A) and microperimetry plus adaptive optics imaging. Ophthalmoscopic examination revealed a juxtafoveolar pigmented lesion with feeding retinal arteriole, consistent with congenital simple hamartoma of RPE. There was no macular edema, exudation, hemorrhage, traction or subretinal fluid. Multimodal imaging of the mass using fluorescein angiography revealed intra-lesion late staining, near-infrared reflectance imaging demonstrated intrinsic hyperreflectivity, short-wavelength autofluorescence and red-free filter photography revealed blocked signal, and SBOCT showed abrupt shadowing. On OCT-A, an exclusive ring-shaped vascular circuit with increased foveal avascular zone was noted. Adaptive optics revealed cell density arrangement and retinal sensitivity correlations on microperimetry.


These findings suggest that this hamartomatous lesion might cause specific cellular changes that impact retinal sensitivity response and potentially result from vasculature malnourishment to the outer retinal layers.


Adaptive optics; Hamartoma; Multimodal imaging; OCT-A; RPE; Tumor

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