Retinal pigment epithelium tear following central serous chorioretinopathy

Editor, Central serous chorioretinopathy (CSC) is characterized by the formation of a localized neurosensory retinal detachment caused by fluid leakage from the retinal pigment epithelium (RPE). In most cases, the detachment resolves spontaneously and visual function returns (Wang et al. 2007). However, a few patients may have a severe, permanent loss of central vision because of multiple recurrences of retinal detachment in one or both eyes or because of complications. Retinal pigment epithelium tears are most often reported in age-related macular degeneration. They are also known to occur spontaneously, traumatically, or following treatment with anti-VEGF (vascular endothelial growth factor) therapies or thermal laser treatment (Kiss et al. 2007). We describe a patient who experienced RPE tear following a fibrotic scar associated with CSC. A 30-year-old man with a 2-year history of bilateral CSC (Fig. 1) complained of a sudden reduction in vision in his left eye. His corrected visual acuity (VA) was 20/200 in the left eye. He had not been treated with photocoagulation. Fundus examination (Fig. 2) showed an RPE tear, subretinal fibrosis and retinal striae at the posterior pole. Fluorescein angiography (Fig. 3) revealed a well circumscribed area of staining, corresponding with lost RPE, with a narrow portion of blocked fluorescence on the nasal margin, consistent with retracted RPE. There was no RPE leakage and no subretinal neovascularization from the area of the RPE rip. Optical coherence tomography (OCT) showed an area devoid of the reflective RPE/choriocapillaris band and a focal area of intense hyper-reflectivity corresponding to scrolled RPE (Fig. 4A). A further OCT scan showed contracted subretinal fibrosis as a highly reflective yellow–red band between the less reflective outer retina and the prominent red band representing the RPE/choriocapillaris (Fig. 4B). Within 3 months, VA improved to 20/40 without treatment. Baseline fluorescein angiogram of the left eye showing a hyperfluorescent area corresponding to a retinal pigment epithelium detachment and demonstrating the typical smokestack leakage. Colour fundus photograph of the left eye showing a retinal pigment epithelium tear. Note subretinal fibrosis and retinal striae below the macula area. Fluorescein angiogram of the left eye revealing a well circumscribed area of staining with blocked fluorescence along the nasal margin. (A) Optical coherence tomography (OCT) through the posterior pole (white line in Fig. 2) displaying absence of the retinal pigment epithelium (RPE)/choriocapillaris band and scrolled RPE. (B) The OCT image obtained through the fibrosis (black line in Fig. 2) shows the contracted subretinal fibrosis. Note retinal striae. The patient reported here had a form of CSC that is far more severe than the typical type. The presence of the RPE tear and subretinal fibrosis may suggest an association. Fibrin can be found in the subretinal space in CSC, but it is unusual for deposition of fibrin to be followed by fibrotic scar formation. An RPE tear is a very unusual complication of subretinal fibrosis associated with CSC (Schatz et al. 1995). Presumably, after the deposition of subretinal fibrin, subretinal fibrosis developed. The fibrotic scar cells were connected to the RPE and, in a process of scar contraction, pulled and ripped the RPE (Schatz et al. 1995). Tears in the RPE following CSC without subretinal fibrosis were reported (Shanmugam & Bhende 2000; Ishida et al. 2004). These were probably caused by a large detachment of RPE. Increased fluid beneath the RPE may cause tangential stress which leads to the formation of a tear; this process has been proposed as a mechanism. Our patient did not receive steroid therapy, which is known to severely exacerbate retinal detachment (Ishida et al. 2004). This case provides further evidence that subretinal fibrosis following CSC can lead to this very unusual complication.