Concentric macular rings sign in combined hamartoma of retina and retinal pigment epithelium.

PURPOSE:To describe two cases of concentric macular rings (CMR) sign in patients with combined hamartoma of retina and retinal pigment epithelium (CHRRPE). METHODS:History and clinical examination, spectral-domain optical coherence tomography (SD-OCT), and optic coherence tomography angiography (OCTA). RESULTS:The first patient was a 26-year-old woman with clinical diagnosis of Neurofibromatosis type 2. The Best-Corrected Visual Acuity (BCVA) was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). She presented a subtle grayish lesion in the macular region of OD. On macular SD-OCT, there was a thickened and disorganized retina, suggestive of CHRRPE. In addition, at the parafoveal region, there was a dentate pattern in the outer plexiform layer (OPL) and Henle fiber layer (HFL) on cross-sectional SD-OCT. OCTA showed no foveal avascular zone (FAZ) in OD and Optos ultra-wide-field revealed CMR sign in this eye. The second patient was a 14-year-old boy, with diabetes mellitus type 1. His BCVA was 20/25 in OD and 20/20 in OS. He had a subtle whitish lesion in the macular region of OD, with SD-OCT findings suggestive of CHRRPE. Similar to the first case, there was a dentate pattern in the OPL and HFL, on SD-OCT. In OD, blue reflectance images exhibited the CMR sign, and OCTA showed absent FAZ. CONCLUSION:CHRRPE may be related to changes in the OPL and HFL interface and may present the CMR sign.