A Case of Congenital Simple Hamartoma of the Retinal Pigment Epithelium and Coats' Disease in the Same Eye

Dear Editor, Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare retinal pigment epithelium (RPE) tumor, incidentally diagnosed in asymptomatic children and young adults. It usually appears as a small, dark pigmented, nodular mass within the macula [1]. Coats’ disease is an idiopathic, progressive retinopathy with retinal vascular telangiectasia, exudation, and frequent retinal detachment occurring more commonly in male children [2]. Herein, we describe a male diagnosed with CSHRPE and Coats’ disease simultaneously in the same eye, which has not been previously reported. A 23-year-old Korean male was referred to our hospital with a history of reduced vision in his right eye for 4 years. He had no medical or ophthalmic disease history. On examination, the best-corrected visual acuity was 20 / 63 in the right eye and 20 / 20 in the left eye. The left eye was unremarkable. Fundus examination of the right eye revealed a small, well-circumscribed, dark pigmented, minimally elevated nodular mass involving the fovea, as well as retinal vascular telangiectasia, multiple aneurysms, and tortuous dilated vessels with yellowish-white exudation in the temporal midperipheral retina (Fig. 1A). Fluorescein angiography (FA) revealed persistent blockage of fluorescence at the fovea due to the mass lesion, and subtle leakage from its surroundings in the late phase. Early hyperf luorescence due to retinal vascular telangiectasia and hypofluorescence due to exudation and capillary drop-out were present in the temporal midperipheral retina (Fig. 1B and 1C). Spectral domain optical coherence tomography (OCT) showed well-demarcated retinal elevation with intense hyper-reflectivity on its inner surface, and complete optical shadowing of the outer retina at the fovea (Fig. 1D). With the diagnosis of CSHRPE and Coats’ disease, focal argon laser photocoagulation was performed around the abnormal vascular lesion temporal to the macula. The best-corrected visual acuity of the right eye was 20 / 40 and both lesions were stable at the 24-month follow-up. CSHRPE is a rare RPE tumor which appears as a dark pigmented, nodular mass with well-circumscribed margins near the fovea. In previous cases, FA showed early nonfluorescence and a late halo of fluorescence at the border of the mass. OCT showed bright ref lectivity of the anterior Korean J Ophthalmol 2015;29(4):282-283 http://dx.doi.org/10.3341/kjo.2015.29.4.282