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Sir,

Retinal pigment epithelial (RPE) rips occur relatively uncommonly and are usually associated with serous detachments of the pigment epithelium, secondary to choroidal neovascularization. These typically occur at the macular and cause rapid visual loss. Once the pigment epithelial tear occurs, the RPE retracts from the outer portion of Bruch's membrane and scrolls up. Fluorescein angiography typically demonstrates a well-demarcated hyperfluorescence in the early phase of the angiogram. We describe a case of RPE tear, unusual in terms of its pathogenesis and size.

Case report

An 81-year-old lady was referred with deteriorating vision in her right eye over a period of weeks. Her left eye was blind due to an inoperable retinal detachment 60 years previously. Examination revealed acuity of 6/9 in her right eye and hand movements in her left. On fundoscopy, the left eye had a chronic inferior macular off retinal detachment. Above the superior arcade, there was a large area of absent RPE, exposing the underlying choriocapillaris. A retracted flap of RPE lay adjacent to a hypopigmented region of denuded Bruch's membrane (Figure 1). Inferior to the rip was an area of subretinal scarring consistent with subretinal proliferative vitreoretinopathy (PVR). Fluorescein angiography demonstrated hypofluorescence over the flap adjacent to diffuse hyperfluorescence (Figure 2), with a characteristic demarcation line between the two.

Figure 1
figure 1

A retracted flap of RPE lay adjacent to a hypopigmented region of denuded Bruch's membrane.

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Figure 2
figure 2

Fluorescein angiography demonstrated hypofluorescence over the flap adjacent to diffuse hyperfluorescence, with a characteristic demarcation line between the two.

Full size image

Comment

Tears in the RPE were first described in 1981 by Hoskins et al.1 They are usually associated with progressive serous pigment epithelium detachments in AMD, or with laser photocoagulation or photodynamic therapy.1, 2, 3 RPE rips, however, may also occur in patients with central serous chorioretinopathy, lupus, polypoidal vasculopathy, chorioretinal scarring, and presumed ocular histoplasmosis syndrome.4, 5, 6, 7 In each of these conditions, tears may be associated with increased hydrostatic pressure generated by damaged choriocapillaris.

In contrast, Gass5 proposed that in AMD choroidal, neovascularization directly separates the RPE from Bruch's membrane and contractile forces of the choroidal neovascular membrane tears the RPE.4 Supporting this, others have noted CNV in the bed of RPE rips, as well as at the site of the scrolled PRE through angiographic and histologic examination.8, 9, 10

The RPE tear in our case has occured due to traction from a proliferative vitreoretinopathy subretinal membrane and not due to hydrostatic or other mechanisms. Relatively small rips in RPE have been reported in other cases with PVR.4 Our case was unusual in that it covered 4 clock hours of mid-peripheral retina—being the first reported with a ‘giant RPE rip’.