Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.
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