Sir,

Retinal pigment epithelial (RPE) rips have been reported in association with age-related macular degeneration and laser photocoagulation. Rarely, they occur in central serous chorioretinopathy (CSCR), particularly when the neurosensory detachment is associated with an underlying large pigment epithelial detachment (PED). We documented with fundus imaging, a patient with idiopathic CSCR who developed an RPE rip.

Case report

A 62-year-old man presented with blurring of vision associated with micropsia and metamorphopsia in his left eye for two weeks. Best-corrected visual acuity was 6/12. There was a large neurosensory elevation over the left posterior pole (Figure 1a). Optical coherence tomography (OCT) imaging showed a large PED under the neurosensory detachment (Figure 1b). Four months later, his vision spontaneously recovered to 6/6. There was now a large RPE rip through the inferior half of the macula, sparing the fovea (Figure 2a). Fluorescein angiography demonstrated a well-demarcated crescenteric area of hyperfluorescence corresponding to the RPE rip, surrounded by a rim of hypofluorescence caused by the retracted RPE (Figure 2b). There was a smoke-stack leak in a residual superior blister of neurosensory elevation. The rest of the macula had flattened clinically and on OCT imaging.

Figure 1
figure 1

(a) Large left central serous chorioretinopathy of approximately five disc diameters. (b) Optical coherence tomography showing neurosensory detachment with underlying retinal pigment epithelial detachment.

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Figure 2
figure 2

(a) Fundus photograph of the retinal pigment epithelial (RPE) rip surrounded by retracted RPE (white arrows). (b) Fluorescein angiography showing hyperfluorescence in the area of the rip and blocked fluorescence in the area of scrolled RPE.

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Comment

RPE rips have been postulated to develop secondary to a build-up of hydrostatic pressure within a PED, with the weakening of intercellular connections between RPE cells. The break usually occurs at the margin of the detachment, presumably the area of greatest strain.1, 2 Expanding PEDs may eventually exert sufficient tangential stress to result in a rip.3

Reports of RPE defects in association with CSCR are rare.4, 5 In CSCR, focal hyperpermeability of the choriocapillaris overwhelms the RPE initially, leading to serous RPE and neurosensory detachments. The sub-RPE fluid causes tangential stress, leading to formation of a tear. One report described a male patient with CSCR who developed a retinal pigment epithelial tear after inappropriate treatment with oral and subconjunctival steroids.4 Another report documented two men with severe CSCR, one of whom was on systemic steroids, with RPE ‘blow-outs’ occurring at the dome of the detachment.5 Steroid usage is an exacerbating factor. Our patient developed a spontaneous RPE rip with CSCR with no history of steroid use.