Atypical Presentation of Chorioretinal Folds-Related Maculopathy.

PURPOSE: Chorioretinal folds are undulations that anatomically include the inner choroid, Bruch's membrane, and the retinal pigment epithelium, and secondarily affect the overlying neurosensory retina. We analyzed clinical data and management of six patients diagnosed with chorioretinal folds-related maculopathy with atypical presentations. CASE REPORT: The mean age of the six patients (five women) was 77 years. Best-corrected visual acuity (BCVA) ranged between 20/200 and 20/80. None of the patients had history of hypertension, cardiovascular diseases, or autoimmune disease, and they were all diagnosed with idiopathic chorioretinal folds. Case 1, 2, and 3 received intravitreal antivascular endothelial growth factor (VEGF) therapy; case 4 received intravitreal anti-VEGF and photodynamic therapy; case 5 received only photodynamic therapy; and case 6 received intravitreal injections of sustained-release dexamethasone implant (Ozurdex). In case 1 and 2, the use of ranibizumab resulted in BCVA improvement and resolution of sub-/intraretinal exudation. In case 3, ranibizumab led to a mild reduction of the intraretinal exudation but no changes in BCVA. In case 4 and 5, six intravitreal injections of ranibizumab with two photodynamic therapies and three photodynamic therapies, respectively, led to a mild reduction of the sub-/intraretinal exudation but no changes in BCVA. In case 6, five intravitreal injections of Ozurdex in both eyes led to reduction of the subretinal or intraretinal fluid accumulation and BCVA improvement. DISCUSSION: Choroidal vessel dilation and hyperpermeability may be involved in atypical presentations of chorioretinal folds-related maculopathy characterized by sub-/intraretinal fluid accumulation. Dilated and hyperpermeable choroidal vessels may result in focal retinal pigment epithelium alterations that can progress to choroidal neovascularization or chronic central serous chorioretinopathy-like maculopathy with or without telangiectatic retinal capillaries. Intravitreal anti-VEGF administration seems effective to treat choroidal neovascularization in stage 3 chorioretinal folds-related maculopathy, both anti-VEGF and photodynamic therapy seem to have only limited efficacy on chronic central serous chorioretinopathy-like maculopathy (and telangiectatic retinal capillaries), whereas intravitreal injection of Ozurdex seems efficacious to treat chronic central serous chorioretinopathy-like maculopathy.