Bullous Central Serous Chorioretinopathy Associated with Retinal Pigment Epithelial Tear.

Bullous retinal detachment (RD) is a rare manifestation of chronic central serous chorioretinopathy and may be associated with an underlying retinal pigment epithelial (RPE) tear. It may be confused with rhegmatogenous RD or serous RD from conditions such as Harada disease. Appropriate diagnosis is essential as this may prevent unnecessary surgery or treatment with medication that may worsen the condition. We present the case of a young male Indian patient with unilateral bullous RD and a tentative diagnosis of rhegmatogenous RD. He underwent RD surgery, however a large RPE tear was discovered intraoperatively. Copyright:

Introduction

Bullous retinal detachment (RD) is a rare manifestation of chronic central serous chorioretinopathy (CSCR).[1] This atypical presentation is thought to be an exaggerated form of typical CSCR as there are large, single or multiple, leaking pigment epithelial detachments (PEDs) which are often hidden underneath the cloudy subretinal exudation.[2] Retinal pigment epithelial (RPE) tears may rarely occur in CSCR, particularly when the neurosensory detachment is associated with an underlying large PED.[3] Due to its peculiar clinical findings, it can present a diagnostic dilemma and lead to inappropriate management.[2] We report a patient with a bullous RD due to CSCR with a tentative diagnosis of rhegmatogenous RD. He was managed surgically, however no retinal breaks were discovered intraoperatively, rather a large RPE tear was observed at the macular area.

Case Report

A 34-year-old male Indian patient presented to our clinic with a 1-week history of sudden, painless loss of vision in his right eye (RE). There was no history of trauma to the eye or steroid use. On examination, the RE had a visual acuity (VA) of hand movement and did not improve with correction, whereas the left eye (LE) had a VA of 6/6. Anterior segment examination was within normal limits in both eyes. Posterior segment examination revealed a bullous subtotal RD involving the macula in the RE [Figure 1]. There was no underlying retinal break on indirect ophthalmoscopy with scleral indentation. The only significant finding in the posterior segment of the LE was a small PED temporal to the macula. A tentative diagnosis of rhegmatogenous RD was made. He subsequently underwent scleral buckling along with pars plana vitrectomy, internal drainage through drainage retinotomy, endolaser photocoagulation, and fluid–gas exchange with silicone oil as the tamponading agent. After settling the retina, a well-demarcated area of bare choroid at the macular area was noted, suggestive of RPE rip. No retinal breaks were found during the surgery. On the 1st postoperative day, he underwent fundus fluorescein angiography (FFA) (Topcon TRC-50 DX Type IA by Topcon Medical Systems Inc., Oakland, NJ, USA) and time-domain optical coherence tomography scan (Cirrus HD OCT Model 4000 by Carl Zeiss Meditec Inc., Dublin, CA, USA). FFA showed a large area of window defect with hyperfluorescence corresponding to the area of bare choroid and adjacent areas of hypofluorescence corresponding to the area of redundant RPE [Figure 2]. On OCT, the RPE rip could be clearly depicted as an area of bare choroid with redundant RPE just nasal to the fovea, while the LE had a small PED temporal to the fovea [Figure 3]. A definitive diagnosis of CSCR with RPE rip was therefore made. The postoperative period was uneventful, and best-corrected visual acuity was 3/60 at 3 months post surgery.

Figure 1

Preoperative retinal drawing showing subtotal retinal detachment. Attached retina superiorly (red color)

Figure 2

Postoperative day 1 images of the right eye: (a) fundus photograph showing area of bare choroid and redundant retinal pigment epithelium (white circle), (b) hyperfluorescence of the areas of bare choroid seen clearly as a window defect on fundus fluorescein angiography, (c) optical coherence tomography showing absent subfoveal retinal pigment epithelial and redundant retinal pigment epithelial nasal to the fovea (white arrow)

Figure 3

Images of the left eye: (a) fundus photograph of the left eye showing a serous pigment epithelial detachment, (b) optical coherence tomography showing pigment epithelial detachment

Discussion

This report describes a young male Indian patient with CSCR who developed an RPE tear with a bullous RD. This is a severe and atypical presentation of CSCR and may be inappropriately diagnosed as serous RD from conditions such as Harada disease or a rhegmatogenous RD.[2] Spontaneous large RPE tear is an unusual manifestation most commonly described in association with choroidal neovascularization from age related macular degeneration.[4] However, it may also occur in CSCR and following ocular trauma.[56] In CSCR, RPE tears are more likely to occur in the bullous variant of the disease.[1] Focal hyperpermeability of the choriocapillaris overwhelms the RPE initially, leading to serous RPE and neurosensory detachments.[3] The sub-RPE fluid causes tangential stress, leading to formation of a tear.[36] Acute RPE tears lead to leakage of sub-PED fluid into the subretinal space as well as escape of fluid from the choriocapillaris, thereby producing a bullous RD.[6] In a series of 136 eyes with CSCR by Balaratnasingam et al., RPE tears were seen in 95% of eyes in the bullous group and none of the eyes in the nonbullous group.[1]

Resolution of serous RD in eyes with RPE tears sparing the fovea is associated with favorable visual outcomes.[7] Our patient had a subfoveal RPE tear with a poor visual outcome. There is no specific treatment for an RPE tear and subretinal fluid tends to resolve with healing of the tear.[7] Eyes with this condition, however, need to be monitored closely as choroidal neovascularization is an important but rare complication.[7]

Conclusion

Bullous RD is a rare manifestation of CSCR and may be associated with an RPE tear. A high index of suspicion is required to distinguish this atypical variant of CSCR from rhegmatogenous RD or serous RD from other causes.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.