Optical coherence tomography in presumed congenital simple hamartoma of retinal pigment epithelium

PURPOSE: To report the optical coherence tomography (OCT) findings in two cases of presumed congenital simple retinal pigment epithelial (RPE) hamartoma. DESIGN: Observational case report. METHODS: Fundus examination, fluorescein angiography, ultrasonography, and OCT were done on two cases of simple RPE hamartoma. RESULTS: A heavily pigmented solitary macular lesion was noted in the right eye of a 22-year,old man and a 55-year-old woman. The young man had a foveal lesion and a vision of 10/200; the woman with eccentric lesion had a vision of 20/20. The lesions blocked fluorescence on angiogram; sonography showed hyper-reflective nodules. OCT demonstrated full,thickness retinal replacement, complete optical shadowing, and abrupt margins of the lesion in both the cases, and vitreomacular adhesion at the temporal margin of the foveal lesion. CONCLUSIONS: Congenital simple RPE hamartoma may rarely occur at the foveal center, resulting in poor visual acuity. OCT is a useful non-invasive adjunct for diagnosis of this rare tumor and may reveal additional features like vitreoretinal adhesion. (c) 2005 by Elsevier Inc. All rights reserved.