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Multimodal imaging in congenital simple hamartoma of retinal pigment epithelium (CSHRPE): optical coherence tomography (OCT), autofluorescence and oct angiography, a review of the literature and case presentation


Purpose: To report a case of congenital simple Hamartoma of retinal pigment epithelium (CSHRPE) with avulsion of RPE without visual impairment.
Methods: A 11-years-old Caucasian male presented with full visual acuity in both eyes, and was made diagnosis of CSHRPE after a complete ophthalmological examination. Was also performed: fundus photographs, spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) and OCT angiography (OCT-A).
Results: Hepresented with full visual acuity in both eyes; fundus examination showed a small, darkly pigmented, well-circumscribed lesion involving the central fovea in the right eye.
Conclusion:This case shows a rare complication such as avulsion can be still associated with high visual acuity. Despite CSHRPE was storically defined as congenital pigment epithelial adenomas or primary pigment epithelial hyperplasia without vascular or glial components; the new sophisticated imaging techniques show the coexistence of a vascular and glial involvement. So CSHRPE can be defined as isolated RPE Hamartoma for the prevalence of the epithelial component, but this is not the only component. These new sofisticated techniques, especially OCT and OCT-A, should take us to consider Retinal Hamartomas like a broad spectrum.


optical coherence tomography, RPE hamartoma, angiography, retina