RESUMO
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.
RESUMO
PURPOSE: To describe the sequential evolution of outer retinal tubulations (ORTs) in patients diagnosed with choroidal neovascularization and/or retinal pigment epithelium atrophy. METHODS: Retrospective evaluation of spectral domain optical coherence tomography of a consecutive cohort of patients with various retinal conditions. RESULTS: We reviewed the clinical findings of 238 eyes of 119 consecutive patients (54 men and 65 women) with a mean age of 76.2 ± 14.2 years (range: 57-90) and a mean follow-up of 3 ± 1.6 years (range 1-7). Over the follow-up period, ORTs were diagnosed in 67 of 238 eyes (28.1%), 9 of which were imaged with sequential, eye-tracked spectral domain optical coherence tomography dating from the beginning of ORT formation. The presence of geographic atrophy and subretinal hyperreflective material at baseline were found to be risk factors for ORT development (P < 0.001 and P < 0.001, respectively). Outer retinal tubulations were divided into forming versus formed morphologies. The latter was comprised open and closed ORTs of which the open subtype was the most common. The formation of ORTs was significantly associated with microcystic macular lesions in the inner nuclear layer and the downward displacement of the outer plexiform layer, referred to as the outer plexiform layer subsidence sign (P < 0.001). CONCLUSION: Outer retinal tubulation is a frequent optical coherence tomography finding in eyes with choroidal neovascularization and geographic atrophy. Open ORTs with progressive scrolled edges and shortened diameter were significantly associated with microcystic macular lesions in the inner nuclear layer and the outer plexiform layer subsidence sign.