Bullous variant of idiopathic central serous chorioretinopathy

Abstract

Background: Spontaneous bullous serous retinal detachment (RD) with subretinal exudation complicating idiopathic central serous chorioretinopathy (ICSC) is a rare and infrequently described clinical entity. Clinical observations are described on this variant form in 11 patients, the largest series reported to date.

Methods: 13 eyes of 11 Indian patients having this entity were followed up clinically and angiographically for 12-24 months (retrospective, longitudinal). None of the patients had any previous history of other diseases nor were they on any medications. Four eyes received laser treatment (group A); nine eyes were not treated (group B).

Results: All 11 patients were male, aged 23-49 years (median 37 years). The clinical and photographic records revealed subretinal exudation and inferior bullous serous RD complicating ICSC with evidence of large, single or multiple, leaking retinal pigment epithelial detachments (PEDs) in all the cases. In group A, resolution of serous RD occurred in 12 weeks (median) with a visual recovery of >/=20/30 in three out of four eyes while in group B resolution of serous retinal detachment was observed in 14 weeks (median) with eight out of nine eyes achieving a visual acuity of >/=20/30. Subretinal fibrosis developed in two eyes in group A and none of the eyes in group B.

Conclusion: The disease is an exaggerated form of ICSC and can occur spontaneously without any history of corticosteroid therapy. Recognition of this atypical presentation is important to avoid inappropriate treatment. These observations suggest that with respect to the duration of the disease and the final visual outcome laser therapy offers no additional benefit over the natural course of this variant form of ICSC.

Figure 1

Case 4 right eye. (A) Fundus appearance at presentation showing two pigment epithelium detachments (PEDs) outside the superior temporal arcade (thin solid arrows). Notice a subtle PED nasal to the fovea (thick solid arrow) in the vicinity of subretinal exudation (open arrow). (B) Midphase fluorescein angiogram demonstrating multiple PEDs (solid arrows). Hypofluorescent areas are due to subretinal exudation (open arrow) and a hidden PED underneath the subretinal exudation (open arrowhead). (C) Late phase fluorescein angiogram showing multiple PEDs (thick solid arrows), a leaking PED (thin solid arrow), staining of subretinal exudation (open arrow), and persistent hypofluorescence due to a PED (open arrowhead) hidden under subretinal exudation. (D) Fundus appearance 14 weeks after onset (non-laser treated) showing near total resolution of subretinal exudation temporal to the disc (open arrow).

Figure 2

Case 4 left eye. (A) Fundus appearance at presentation demonstrating massive subretinal exudation and serous retinal detachment in the posterior pole. Notice hidden PEDs (solid arrows) in the vicinity of subretinal exudation (open arrows). (B) Midphase fluorescein angiogram demonstrating multiple PEDs (solid arrows), and hypofluorescent areas are due to a hidden PED (open arrowhead) and subretinal exudation (open arrow). (C) Late phase fluorescein angiogram showing leakage from the edges of PEDs (solid arrows), and staining of the subretinal exudation (open arrow). (D) Fundus appearance 12 weeks after laser photocoagulation showing subretinal fibrotic membrane around the fovea (open arrows). (E) Late phase angiogram 16 weeks after laser photocoagulation showing substantial amount of RPE degeneration (solid arrows) and subretinal fibrotic membranes (open arrows).