Indian Journal of Ophthalmology

: 2018  |  Volume : 66  |  Issue : 7  |  Page : 999--1000

Foveolar simple retinal pigment epithelial hamartoma

Amar Pujari, Shreyas Temkar, Sahil Agarwal, Gaurav Garg, Rohan Chawla, Atul Kumar 
 Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Amar Pujari
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Room No 212, Second Floor, RPC-1, New Delhi - 110 029


How to cite this article:
Pujari A, Temkar S, Agarwal S, Garg G, Chawla R, Kumar A. Foveolar simple retinal pigment epithelial hamartoma.Indian J Ophthalmol 2018;66:999-1000

How to cite this URL:
Pujari A, Temkar S, Agarwal S, Garg G, Chawla R, Kumar A. Foveolar simple retinal pigment epithelial hamartoma. Indian J Ophthalmol [serial online] 2018 [cited 2020 Jul 10 ];66:999-1000
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 Case Report

A 28-year-old male patient underwent routine ophthalmic evaluation. Visual acuity was 20/20, and the anterior segment was unremarkable in both the eyes. Right eye retinal examination was normal whereas the left eye showed a healthy optic disc along with a well-defined circular pigmented lesion at the foveola [Figure 1]a and [Figure 1]b. The rest of the retinal examination was within normal limits. Swept-source optical coherence tomography (OCT) revealed a hyper-reflective lesion just inferior to the umbo [Figure 1]c. The hyperreflective lesion was seen just adjacent to the foveal dip in the inner retinal layers. The outer retinal and choroidal layers below the lesion were obscured because of the shadowing. However, the outer retinal and pigment epithelial layers surrounding the lesion appeared intact without any discontinuity or neurosensory detachment [Figure 1]d. OCT angiography revealed a mildly distorted foveal avascular zone at the location of the lesion. However, the adjacent superficial retinal plexus was intact and well preserved. No abnormal or intrinsic vasculature was noted in the lesion [Figure 1]e. The patient was counseled about the nature of the retinal lesion and advised for regular follow-up.{Figure 1}


Gass described three patterns of retinal involvement in congenital simple retinal pigment epithelial (RPE) hamartoma, (1) superficial retinal involvement, (2) full thickness retinal involvement with a preretinal extension, or (3) full thickness retinal involvement with a preretinal extension having intrinsic vascularization.[1] Similarly, shields elaborated five such cases focusing mainly on the dimensions of the tumor, distance from the foveola, and the disc and associated retinal changes. They described all the cases as involving the full thickness of retina however OCT features were not described.[2] The noted superficial retinal changes include dilated feeder retinal vessel, surrounding retinal traction, pigmented vitreous cells, and retinal exudations.[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


OCT-A is a better noninvasive tool in the evaluation of changes along the retinal vascular plexus in cases of RPE hamartoma.

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Conflicts of interest

There are no conflicts of interest.


1Gass JD. Focal congenital anomalies of the retinal pigment epithelium. Eye (Lond) 1989;3(Pt 1):1-8.
2Shields CL, Shields JA, Marr BP, Sperber DE, Gass JD. Congenital simple hamartoma of the retinal pigment epithelium: A study of five cases. Ophthalmology 2003;110:1005-11.