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CONCENTRIC MACULAR RINGS SIGN IN COMBINED HAMARTOMA OF RETINA AND RETINAL PIGMENT EPITHELIUM
Lívia da Silva Conci1, Renata Martins Maia1, Rodrigo Pessoa Cavalcanti Lira2
1Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil.
Purpose:
The aim of this study was to describe two cases of concentric macular rings sign in patients with combined hamartoma of the retina and retinal pigment epithelium.
Methods:
Case report involving clinical examination, spectral domain optical coherence tomography, and optical coherence tomography angiography.
Results:
The first patient was a 26-year-old woman with clinical diagnosis of neurofibromatosis type 2. The best corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. She presented a subtle grayish lesion in the macular region of the right eye. On macular spectral domain optical coherence tomography, there was a thickened and disorganized retina, suggestive of combined hamartoma of the retina and retinal pigment epithelium. In addition, at the parafoveal region, there was a dentate pattern in the outer plexiform layer and Henle fiber layer on cross-sectional spectral domain optical coherence tomography. Optical coherence tomography angiography showed no foveal avascular zone in the right eye, and Optos ultra-widefield revealed a concentric macular rings sign in this eye. The second patient was a 14-year-old adolescent boy, with diabetes mellitus type 1. His best corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. He had a subtle whitish lesion in the macular region of the right eye, with spectral domain optical coherence tomography findings suggestive of combined hamartoma of the retina and retinal pigment epithelium. Similar to the first case, there was a dentate pattern in the outer plexiform layer and Henle fiber layer observed on spectral domain optical coherence tomography. In the right eye, blue reflectance images exhibited the concentric macular rings sign, and optical coherence tomography angiography showed absent foveal avascular zone.
Conclusion:
Combined hamartoma of the retina and retinal pigment epithelium may be related to changes in the outer plexiform layer and Henle fiber layer interface and may present the concentric macular rings sign.
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