Evaluation of Retinal Pigment Epithelial Hamartoma Using Oct – A Case Summary

We report a case of healthy sixteen year old girl who presented with left eye defective vision with a Snellen best corrected visual acuity of 6/36. Although the anterior segment was normal, a black retinal lesion at the fovea was detected by fundus examination. The right eye had 6/6 vision and fundus examination was normal. In the left eye the fundus fluorescein angiography features and spectral domain optical coherence tomography findings were compatible with a congenital simple hamartoma of retinal pigment epithelium (CSHRPE) at fovea. It is essential to detect this tumour and differentiate it from other pigmented retinal lesions. Received: 19 November, 2016; Accepted: 01 December, 2016; Published: 12 December, 2016 *Corresponding author: J. Kaliamurthy, Institute of Ophthalmology, Joseph Eye Hospital, Tiruchirappalli, Tamil Nadu, India, Tel: +91-431-2460622, Mob: +91-9894540875; E-mail: [email protected] gliosis. There was no associated RPE atrophy, macular edema, retinal exudation or subretinal fluid. Fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and ocular B Scan ultrasonography were performed. Fluorescein angiography showed blockage of fluorescence during all phases of FFA due to pigmented tumour and there was no leakage at fovea [Figure 2a & 2b]. The retinal vessels at macula were minimally dilated and there was no feeder vessels for the tumour mass. The temporal arcades were dragged towards the lesion. The macular OCT scan was performed using CirrusTM HDOCT Model 4000 (Carl-Zeiss Meditec, Inc, Dublin, California, USA). The High definition OCT image scan HD 5-line raster scan [Figure 3] of the left eye revealed an abruptly elevated hyper reflective mass with increased optical reflectivity on its inner surface with optical shadowing beneath and well-defined clear cut tumour margins. There was vitreo macular adhesion at the nasal margin of the lesion. The surrounding retina and choroid was normal. The retinal tumour thickness in SD-OCT was 1004 microns (1 mm). B-scan ocular ultrasonography of the left eye showed a nodular high-echogenic small mass at the macula [Figure 4]. Introduction Tumours of the Retinal Pigment Epithelium (RPE) are uncommon. They are classified into four groups including CSHRPE, congenital hypertrophy of the RPE, combined hamartoma of the retina and RPE and adenoma or adenocarcinoma of the RPE [1-2]. Congenital simple hamartoma of the RPE was first recognized by Laqua [3] in 1981. Gass [1] coined the term retinal hamartoma in 1989. Since then, very few cases are reported in the literature [3-4]. The largest case series has been reported by Shields et al. in 2003 and included 5 cases [5]. We report ophthalmoscopic and imaging findings (FFA, OCT, B Scan) of a case with CSHRPE at fovea.