Giant retinal pigment epithelium rip in a patient with peripheral exudative hemorrhagic chorioretinopathy.

A 74-year-old lady presented with complaints of floaters in both eyes since 1 month. Best-corrected visual acuity was 6/12 in the right eye and 6/9 in the left eye. Fundus examination showed regions of subretinal bleed and a large retinal pigment epithelium (RPE) rip with folded edges in the right eye [Fig. 1a] and subretinal bleed in the left eye [Fig. 1b]. Fundus autofluorescence showed the RPE rip [Fig. 1c] and subretinal bleed [Fig. 1c and d]. Fundus fluorescein angiography also confirmed the RPE rip [Fig. 2, top left] and subretinal bleed and also showed peripheral retinal degenerations [Figs. 2 and 3, top left]. Indocyanine green angiography (ICGA) was suggestive of polyps in both the eyes [Figs. 2 and 3, top right]. Spectral domain optical coherence tomography (SD-OCT) showed thinned out choroid in both eyes [Figs. 2 and 3, bottom] and a fine epiretinal membrane in the right eye [Fig. 2, bottom]. Reticular drusen were also noted on infrared images and SD-OCT in the left eye [Fig. 3, bottom]. In view of good visual acuity, no intervention was sought. The patient has been on follow-up with maintained visual acuity.

Figure 1

(a) Fundus image of the right eye shows subretinal bleed (white arrowhead) and a giant RPE rip (black arrowheads). (b) The left eye shows peripheral subretinal bleed (white arrowhead). (c) FAF of the right eye shows the RPE rip (black arrowhead) and the subretinal bleed (white arrowhead). (d) FAF of the left eye shows the subretinal bleed (white arrowhead)

Figure 2

FFA of the right eye (top left) shows a hyperfluorescent giant RPE rip (black arrowheads) and peripheral retinal degenerations (green arrowhead). Subretinal bleed (hypo-fluorescent – white arrowhead) is also noted. ICG (top right) shows a peripheral polyp (yellow arrowhead) and the RPE rip (black arrowheads). SD OCT shows thinned out choroid (white arrowheads) and an epiretinal membrane on the surface

Figure 3

FFA of the left eye (top left) shows subretinal bleed (hypo-fluorescent – white arrowhead) and peripheral retinal degenerations (green arrowhead). ICG (top right) shows peripheral polyps (yellow arrowhead). Infra-red image (bottom left) shows reticular pseudo-drusen (red arrowhead), also seen on SD-OCT (red arrowhead, bottom right) along with thinned out choroid (white arrowhead)

Discussion

Our case presented with regions of peripheral subretinal bleed and exudation, a classic finding of peripheral exudative hemorrhagic chorioretinopathy (PEHCR), in both eyes. ICGA showed presence of distinct hypercyanescent “hot-spots” that indicate polyps. Presence of a giant RPE rip in the right eye of the patient was a novel finding in our case. An RPE rip is a tear in the RPE that exposes the underlying large choroidal vessels.

PEHCR has been postulated to be a peripheral variant of AMD[1] as well as PCV[2] with multiple reports of concurrent occurrence. The presence of such a giant RPE tear in the periphery may point toward the coexistence of a large serous pigment epithelial detachment (PED) in the past. This finding along with presence of peripheral polyps adds credibility to the hypothesis of PEHCR being a peripheral variant of PCV as proposed by Mashayekhi et al.,[2] who documented a case with peripheral mounds of subretinal and sub-RPE bleed that showed polyp-like hotspots on ICGA.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.