Giant Retinal Pigment Epithelium Tear Resulting in Neurosensory Retinal Detachment: An Atypical Case

Abstract

Purpose:

We report a rare case of neurosensory retinal detachment (RD) in the setting of a giant retinal pigment epithelium (RPE) tear.

Methods:

A 58-year-old man presented with a macula-involving RD in the left eye. Exam revealed a neurosensory detachment inferiorly and RPE abnormalities temporally. Optical coherence tomography showed a large RPE tear and detachment in the temporal macula contiguous with a neurosensory RD.

Results:

No clear etiology was identified and failure of conservative management led to vitrectomy with RD repair. Follow-up intravenous fluorescein angiography 3 months postoperatively showed a large RPE window defect.

Conclusions:

RPE tears are common; however, concomitant neurosensory RD is rare. A thorough workup to determine treatable causative factors is necessary; in the event of idiopathic diagnosis, close follow-up is necessary to determine the need for surgery. Pars plana vitrectomy, external drainage of subretinal fluid, endolaser, and 5000-centistoke silicone oil placement were successful in this patient.

Introduction

Retinal pigment epithelial (RPE) tears are a recognized and feared complication in the era of antivascular endothelial growth factor (anti-VEGF) therapy, often resulting in significant visual limitation. RPE tears can arise spontaneously or in the setting of treatment when a pigment epithelial detachment (PED) is present. Other ocular conditions associated with RPE tears include neovascular age-related macular degeneration (AMD), central serous chorioretinopathy (CSR), polypoidal choroidal vasculopathy, and retinal angiomatous proliferation. There are few reports in the literature, however, in which an RPE tear is directly associated with the development of a neurosensory retinal detachment (RD). We present a case of an idiopathic PED resulting in a giant RPE tear associated with a neurosensory RD.

Methods

Case Report

A 58-year-old man with a medical history of type 2 diabetes mellitus, ulcerative colitis (UC), stage 3 chronic kidney disease, hypertension, iron deficiency anemia, and grade 2 renal cell carcinoma postnephrectomy presented to the urgent care clinic with decreased vision and a nasal visual field defect in his left eye. Two days before presentation, the patient was seen by a glaucoma specialist for routine monitoring of his chronic narrow-angle glaucoma. At the time of that visit, the patient noticed a small “brown wedge” in the superonasal visual field of his left eye but did not mention it to the provider. At this visit, the best-corrected visual acuity (VA) was 20/30^-2 in the left eye and the intraocular pressure (IOP) was 21 mm Hg. The visual field change progressed over the next 2 days until his presentation to the urgent care clinic. The patient denied flashes, floaters, eye pain, redness, or a recent history of trauma in either eye on initial presentation.

On presentation to the urgent care clinic, the left eye had a VA of counting fingers vision at 2 feet (0.6 m), IOP of 13 mm Hg, and superior and nasal visual field deficit. Anterior segment evaluation was benign, with no signs of inflammation or neovascularization. A grade 1+ nuclear sclerotic cataract was present. Dilated fundus examination of the left eye was notable for trace pigmented cell in the vitreous cavity without PVD. A large, bullous, macula-involving RD extended from approximately 3 o’clock (temporally) to 7 o’clock (inferonasally).

A narrow, linear hyperpigmented lesion was noted running vertically, temporally to the foveola, varying from 0.5 to 1.0 disc diameters in width at the largest extent. This defect began superotemporally to the superotemporal arcade and extended inferiorly through the inferotemporal arcade (Figure 1). Multiple other smaller hyperpigmented foci were scattered, mostly temporally to this region in the temporal macula. On scleral-depressed examination, small vitreoretinal tufts were noted inferonasally without evidence of retinal tears or breaks. The inferior aspect of the detachment appeared clinically similar to a typical neurosensory detachment, whereas the entire temporal extent appeared to involve the RPE. No frank retinal tears or holes were found on extensive scleral-depressed examination.

Figure 1.

Color fundus photograph of the left eye shows an inferior neurosensory retinal detachment and retinal pigment epithelium (RPE) tear temporal to the fovea with elevation of the temporal RPE.

Thorough examination of the right eye did not reveal relevant findings. There was no evidence of anterior or posterior segment inflammation. The macula appeared flat with good contour on optical coherence tomography (OCT) imaging. There was no evidence of retinal breaks or tears, RPE, or choroidal pathology. Intravenous fluorescein angiography (IVFA) of the right eye was unremarkable.

At the patient’s glaucoma visit 2 days previously, a macula OCT scan of the left eye showed a large PED in the temporal macula without associated subretinal fluid (Figure 2). On his presentation to urgent care 2 days later, repeat OCT of the macula revealed a giant RPE tear associated with subretinal fluid into the fovea temporally (Figure 3). OCT showed a clear break in the RPE with mild scrolling and a contiguous track between the subretinal and sub-RPE space. The smaller, scattered, hyperpigmented foci noted on examination were small areas of RPE defects within the RPE detachment. OCT illustrated that the neurosensory detachment involving the macula and inferior retina was continuous, running through the giant RPE tear with the temporal RPE detachment. The choroid appeared to be of normal thickness without evidence of pachychoroid. IVFA/indocyanine green angiography (ICGA) imaging was notable for window defects in the region of the RPE tear and smaller RPE breaks temporally (Figure 4). Of note, no leakage, vasculitis, tumors/metastases, or inflammatory changes were noted in either eye. A B-scan did not show masses or inflammatory changes.

Figure 2.

Optical coherence tomography of the left eye macula 2 days before presentation shows large pigment epithelial detachment with preserved foveal contour and intact retinal pigment epithelium.

Figure 3.

Optical coherence tomography of the macula at presentation shows retinal pigment epithelium break with scrolling and neurosensory retinal detachment.

Figure 4.

Fluorescein angiography and indocyanine green angiography of the left eye at 1 minute on presentation show multiple window defects correlating with the large retinal pigment epithelium (RPE) tear and multiple scattered, smaller RPE breaks in the temporal retina. Of note, there is no evidence of vasculitis or leakage.

Considerations for the atypical presentation of this large RPE tear and subsequent neurosensory RD and PED include inflammatory, autoimmune, malignant, and metastatic processes. Prompt surgical intervention was delayed pending further workup to rule out the aforementioned etiologies. Magnetic resonance imaging (MRI) scans of the brain and orbits could not be performed because of the presence of a noncompatible Medtronic spinal cord stimulation device. A CT of the head and orbits was obtained without contrast because of renal function; it showed abnormal hyperattenuation in the left posterior globe and subtle enlargement with central hypoattenuation of the inferior rectus muscles.

Laboratory analysis was negative for infectious and inflammatory etiologies. A trial of high-dose oral prednisone (1 mg/kg) was initiated to treat potential underlying inflammatory etiologies. On day 4 of therapy, the patient became intolerant of medication side effects and further progression of subretinal fluid was noted on examination despite prednisone treatment. The patient was rapidly tapered off prednisone and subsequently taken to the operating room, where a pars plana vitrectomy with endolaser and 5000-centistoke silicone oil was performed.

Results

The patient remained attached under silicone oil throughout his postoperative course (Figure 5). At the most recent follow-up visit, his VA was 20/300, his IOP was 19 mm Hg, and the macula was attached (Figure 6). A repeat IVFA/ICGA was performed 3 months postoperatively (Figure 7). Notably, it appeared as though the entire temporal RPE was no longer present, most easily seen with the large temporal window defect on IVFA. This represented a more widespread disorder of the RPE than was previously appreciated. It is unclear whether the RPE that was detached had scrolled further temporally or possibly atrophied during the postoperative period.

Figure 5.

Color fundus photograph of the left eye 3 months postoperatively shows large temporal granular pigmentary changes consistent with the area of retinal pigment epithelium (RPE) loss/atrophy.

Figure 6.

Postoperative month 3 optical coherence tomography of the macula shows a good anatomical result and loss of ellipsoid zone.

Figure 7.

Fluorescein angiography and indocyanine green angiography of the left eye 3 months postoperatively shows a temporal patchy window defect and relative hypercyanescence. The hyperfluorescence inferior to the disc is the location of the drainage retinotomy.

Conclusions

RPE tears are most commonly the result of PED in patients with exudative AMD. ¹ The buildup of serous fluid in the sub-RPE space places pressure on the RPE cells, which cannot withstand the force required to separate from the Bruch membrane, leading to a rupture. ² In a study of patients with vascular PED, 9 of 72 eyes (12.5%) had an RPE tear. ³ Current literature suggests the incidence of these breaks ranges from 2.8% to 33% with the advent and growing application of anti-VEGF injections, a possible increase vs spontaneous occurrence; however, further research is needed. ¹ One mechanism behind this purported increase is that injections can cause contraction of choroidal neovascular membranes, placing the RPE under additional tension. A study by Doguizi and Ozdek ⁴ reported the occurrence of RPE tears in patients receiving anti-VEGF injections was 19.7% in vascular PED compared with 2.1% in avascular PED. Other etiologies of and risk factors for RPE for tears include CSR (rare), ⁵ polypoidal choroidal vasculopathy (8.3%), ⁶ angioid streaks, ⁷ pathologic myopia, ⁸ trauma, ⁹ tumors, ¹⁰ as well as other chorioretinopathies.

Detection of RPE tears can vary. Larger RPE tears can be visualized directly by dilated fundus examination, most commonly appearing as a depigmented, crescent-shaped area with hyperpigmented lines and underlying bare choroid. Tears might also be concentric with an island of scrolled RPE at the center. OCT imaging of RPE tears show irregular contours of hyperreflectivity with underside shadowing from a retracted RPE. Other characteristics include a hyperreflective bare choroid area beneath the window of missing RPE and an intact neurosensory retina with or without neurosensory detachment. Fundus autofluorescence shows remarkable hypoautofluorescence where the rip occurs with a central area of hyperautofluorescence, corresponding to RPE bunching. FA demonstrates a window defect over areas of RPE loss and blocked fluorescence over areas of RPE bunching.

Review of the literature found a correlation between hypotony and RPE tears.^11–14 In the same way that the choroidal neovascular membranes put strain and tension on the RPE, choroidal detachments under hypotony are thought to lead to RPE tears.^4,11 One notable distinction between the previous cases and ours is that there was no evidence of hypotony before our patient’s detachments. The additional component of a serous RD in our patient raises the question of whether a previous inflammatory condition increased his susceptibility for an RPE tear.

Harada et al ¹¹ reported a patient who had a history of uveitis, and others report patients with a history of blebitis and prior cyclocryotherapy treatment who had an inflammatory response. Our patient’s history of UC calls into question the integrity of his RPE. UC has been shown to have a prevalence of ocular symptoms of between 1.6% and 5.4%.^15,16 Despite our patient’s history of colectomy, previous inflammatory damage might have already altered the RPE’s stability. ¹⁷

Arroyo and Jaffe ¹⁸ reported a patient who developed an RPE tear and subsequent exudative RD in the setting of chronic idiopathic panuveitis and hypotony. They proposed 2 components that might be relevant when reflecting on our patient—exudative RD in the setting of the RPE tear might be related to a large amount of exposed choroid and/or exacerbated by increased choroidal hydrostatic pressure. The patient’s comorbid hypertension and chronic kidney disease might have contributed to his presentation.

Paraneoplastic syndromes are another consideration when assessing a patient with exudative RDs. However, given the rapid onset of the patient’s presentation, this appears less likely.^19–21 After removal of his Medtronic implant, the patient underwent an MRI scan of his brain, orbits, and abdomen that revealed previously known kidney changes postnephrectomy without frank evidence of a metastatic or inflammatory process.

There continues to be no obvious systemic correlation with the patient’s presentation. The temporal hyperfluorescent spots suggest an inflammatory etiology or one similar to multifocal CSR. To our knowledge, the first documentation of a patient with RPE tears in the setting of CSR was presented by Ishida et al, ²² who noted this in a 45-year-old man who was treated with steroids before his presentation. In their case report, they support the hypothesis by Guyer and colleagues ²³ that hyperpermeability of the choroidal vessels surpasses the RPE’s ability to maintain equilibrium and thus leads to a serous RPE detachment. Moreover, the mechanical stress exerted at the angle where the attached RPE meets the detached RPE results in an RPE tear. In our patient’s case, the significant extent and size of the RPE tear allowed for large direct communication between the sub-RPE space and the subretinal space that likely contributed to the degree of neurosensory RD that developed.

We report a patient with an atypical macula-off, serous RD with a giant RPE tear associated with an RPE detachment. At the time of this publication, the patient was doing well his retina was completely attached 360° under silicone oil.

This atypical case is the first of its kind to be documented in the literature. Although there was an idiopathic etiology, this case serves as a template to systematically approach a patient with an RPE tear. Multiple confounding variables in the patient’s medical history prevent the determination of a causative factor. Taken together, these variables might have contributed to the patient’s presentation.