Spontaneous giant retinal pigment epithelial rip with retinal and choroidal detachment

Hoskin and colleagues described retinal pigment epithelial (RPE) tears or rips in 1981.[1] They are classified according to the size of the tear as microrips, conventional RPE tears, and giant tears.[2] Giant RPE tears are often extramacular and occasionally associated with hypotony following glaucoma surgery.[3] Here, we describe a case of primary open angle glaucoma (POAG) that presented with spontaneous bullous retinal detachment and choroidal detachment secondary to a giant extramacular RPE rip in the left eye. Such an occurrence of retinal detachment and choroidal detachment secondary to an RPE tear has not been reported to the best of our knowledge. Case History A 62-year-old lady, on treatment with maximal medical therapy for POAG for the past 12 years, presented with a sudden loss of vision in the left eye. She had undergone phacoemulsification with intraocular lens (IOL) implantation in the left eye 10 years ago. Her right eye underwent trabeculectomy many years ago, and cataract surgery was done 2 years ago. Intraocular pressure (IOP) was stable in the left eye, while there were occasional fluctuations in the IOP in the right eye. The spikes of IOP in the right eye were controlled with the addition of oral acetazolamide. On examination, her best corrected visual acuity was 6/6 and counting fingers close to face in the right and left eyes, respectively. The left eye was quiet with a well-centered IOL. Fundus examination showed total bullous retinal detachment with 360-degree choroidal detachment. No peripheral degeneration or breaks were detected on scleral depression. B-scan of the left eye corroborated the clinical findings and ruled out any choroidal mass. The right eye was unremarkable. The intraocular pressures were 17 mm Hg and 10 mm Hg in the right and left eyes, respectively. The left eye pseudophakic retinal detachment was presumed to be rhegmatogenous, and the patient was offered vitreoretinal surgery. Before the surgery, the patient was started on oral steroids (1 mg/Kg body weight) for a week. The choroidal detachment resolved during this period, but the bullous retinal detachment remained status quo. The left eye underwent 23 G pars plana vitrectomy and subretinal fluid drainage through a superotemporal drainage retinotomy. The subretinal fluid was thick, and a fluid–fluid exchange followed by fluid–air exchange was done. After the retina was reattached, a large hypopigmented orangish lesion was noted temporal to the fovea extending inferiorly along the arcade, measuring approximately 5 to 6 DD. The surgeon suspected choroidal hemangioma and proceeded with peripheral cryotherapy and endolaser photocoagulation over the lesion. No peripheral breaks were noted on inspection. Silicone oil (5000 cS) was injected with a planned underfill of oil because of advanced glaucoma, and the retina was well attached. At 1 week postop, a multimodal imaging of the left eye was done. Ultrawide field imaging confirmed good retinal reattachment with a large RPE tear temporal to the fovea [Fig. 1]. Autofluorescence showed a large area of hypoautofluorescence corresponding to the orangish lesion [Fig. 2]. Optical coherence tomography (OCT) through the tear confirmed the presence of rolled edges of the RPE [Fig. 3]. After 2 months, she underwent silicone oil removal. The visual acuity in the left eye improved to 6/9 unaided, with a best corrected near vision of N6, and the intraocular pressure was well controlled with topical medications.Figure 1: Ultrawide field imaging shows a well-attached retina under oil and the giant RPE ripFigure 2: Autofluorescence imaging showing a large area of hypoautofluorescence corresponding to the area of giant RPE rip, after retinal reattachment under silicone oilFigure 3: OCT over the area of the giant RPE tear showing the rolled edge of the tearDiscussion Retinal detachment with choroidal detachment following a spontaneous giant RPE tear or rip is rare. Giant RPE tears are usually associated with large fibrovascular pigment epithelial detachments in neovascular age-related macular degeneration or hypotony and inflammation after intraocular surgery, especially trabeculectomy. In most cases, the RPE tear is visible even in the presence of retinal detachment.[2,3] In our case, the tear was not seen until after retinal reattachment. This was because of the extensive bullous nature of the detachment and the thick subretinal fluid obscuring the view of the RPE. Even after the reattachment, the RPE tear did not have the typical appearance, with its orangish hue masquerading as a choroidal hemangioma. The pathophysiology of such giant RPE tears after trabeculectomy seems to be related to hypotony and choroidal edema or detachment, leading to a rip in the vulnerable RPE layer.[3] Chronic intraocular pressure fluctuations in our case would have led to a weak RPE layer, and the same fluctuations with resultant choroidal fluid build-up, eventually causing a giant RPE rip. Whether conservative management would have resulted in a spontaneous resolution is a matter of debate in this case. Though the choroidal detachment resolved with oral steroids, the bullous nature of the retinal detachment did not change. Moreover, there were no inciting events such as intraocular surgery or hypotony to precipitate such a giant RPE tear. The presence of choroidal detachment, which has been hitherto unreported in the literature in cases of giant RPE rip and retinal detachment, further favored the diagnosis of a rhegmatogenous retinal detachment and early intervention. The presence of subretinal fluid of such colloidal nature would have taken several days to resolve, endangering the health of the central RPE and photoreceptors. We believe that prompt surgery and reattachment of the retina helped achieve good visual results for this patient. In conclusion, giant RPE tears can occur spontaneously, leading to retinal and choroidal detachment in susceptible eyes. While most of them resolve with reversal of hypotony, a few with significant subretinal fluid may need surgical intervention. Multimodal imaging techniques play an important role in diagnosing these giant RPE tears.