Purpose: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare fundus condition. Two cases of CSHRPE are reported.
Methods: The clinical findings and biomicroscopy, fluorescein angiography, optical coherence tomography (OCT), and ultrasonography features differentiating this pigmented macular tumor from uveal melanoma or retinal pigment epithelium (RPE) anomalies and neoplasms are discussed.
Results: Two patients were observed with a pigmented macular lesion. Sharply demarcated transretinal lesions were seen in the macula of both patients, arising from the RPE. The periphery of the lesion in Case 1 was flat and black; the central portion was elevated and fibrotic with a central vitreous strand. A narrow crescent of RPE atrophy was noted temporally. Optical coherence tomography of both lesions showed the typical findings of CSHRPE: high reflectivity, irregular surface, abrupt borders, and full-thickness retinal shadowing. The decision was made to observe the lesions. During follow-up, the lesions remained unchanged.
Conclusion: The unusual finding of CSHRPE associated with RPE atrophy makes the differentiation between CSHRPE and hyperplasia of the RPE even more difficult. Noninvasive OCT has a major role in the diagnosis of CSHRPE.