On this episode of Ready, Set, Retina, Daniel Epshtein, OD, FAAO, sits down with Jessica Haynes, OD, FAAO, to review a case of retinal pigment epithelium (RPE) tear secondary to exudative age-related macular degeneration (AMD).
Dr. Haynes is a consulting faculty member at the Southern College of Optometry in Memphis, Tennessee, and a consultative optometrist at the Charles Retina Institute in Germantown, Tennessee.
What is an RPE tear?
RPE tears or rips are a rare complication of vascularized retinal pigment epithelial detachments (PEDs) that can be visually devastating.1 They occur when the RPE acutely tears and retracts—usually in the area overlying a PED—leaving the underlying Bruch’s membrane and choroid exposed.2
While various eye conditions can cause RPE tears, they classically occur in patients with neovascular AMD (nAMD) as part of the natural history of PEDs or in response to anti-vascular endothelial growth factor (VEGF) treatment, laser photocoagulation, transpupillary thermotherapy, or photodynamic therapy.1,3 Of note, reports of RPE tears have increased due to the widespread use of anti-VEGF drugs for nAMD.4
RPE tear case report
Baseline
A 76-year-old white male patient was referred to the clinic in 2021 for AMD in both eyes (OU). The patient reported vision loss in the left eye (OS) for ~1 month and drusen were observed in the right eye (OD).
The patient’s best-corrected visual acuity (BCVA) was 20/20 OD and 20/400 OS. Dr. Haynes noted that patients with RPE tears tend to have a poor visual prognosis—particularly for tears involving the fovea.3
Figure 1: Near-infrared reflectance (NIR) and optical coherence tomography (OCT) imaging OS at baseline. The OCT image highlights an RPE tear in which the RPE can be visualized as a highly reflective band at the top of the lesion.
Figure 1: Courtesy of Jessica Haynes, OD.
Anti-VEGF treatments OS
Subsequently, the patient received anti-VEGF injections in his left eye; his BCVA remained stable at 20/400 OS.
Figure 2: NIR and OCT imaging OS from 2022 (18 months after the baseline imaging). The OCT image shows improvements in the RPE tear and the yellow circle highlights the fovea, which is located on top of irregular RPE and is adjacent to atrophic tissue on the left. The NIR imaging highlights the atrophic tissue as a large hyperreflective area.
Figure 2: Courtesy of Jessica Haynes, OD.
Notable risk factors for RPE tears
Studies have suggested that serous PEDs that are large in height and diameter have a higher risk of developing into an RPE tear with foveal involvement, noted Dr. Haynes.3,4
In addition, the presence of subretinal or prechoroidal clefts has been associated with macular neovascularization (MNV) exudation and a high risk of RPE tear or subretinal hemorrhage.5,6 Prechoroidal clefts are defined as hyporeflective spaces on OCT located between the fibrovascular and Bruch’s membrane.7
Further, it has been suggested that vascularized PEDs with shorter durations (i.e., newer lesions with fresher vasculature) may be able to contract more rapidly and consequently have a higher risk for RPE tear in response to anti-VEGF therapy.8
Haunted by the fellow eye
In 2022, the patient presented for a routine follow-up visit, and OCT imaging demonstrated that he had developed a PED in the right eye as well. The patient was asymptomatic and had a BCVA of 20/20 OD.
Figure 3: NIR and OCT imaging OD that demonstrate a drusenoid PED.
Figure 3: Courtesy of Jessica Haynes, OD.
Figure 4: NIR and OCT imaging OD from 3 months later, showing a vascularized PED with adjacent subretinal fluid on both sides. This indicates that the patient developed exudative AMD in the fellow eye, noted Dr. Haynes.
Figure 4: Courtesy of Jessica Haynes, OD.
Anti-VEGF injections in the fellow eye
To manage the choroidal neovascularization, the patient received anti-VEGF injections in the right eye. After 6 months of treatment, the patient’s BCVA remained stable at 20/20 OD.
Figure 5: NIR and OCT imaging OD 6 months into anti-VEGF treatment. The lesion has become somewhat elongated, and there is still some subretinal fluid present adjacent to the lesion. A subretinal cleft can be visualized.
Figure 5: Courtesy of Jessica Haynes, OD.
Figure 6: NIR and OCT imaging OD from September 2024; the patient continues to receive anti-VEGF injections every other month and has a BCVA of 20/20 OD. There has been significant resolution of the vascularized PED.
Figure 6: Courtesy of Jessica Haynes, OD.
Treatment considerations for RPE tears
Dr. Haynes emphasized that it can be difficult to manage rarer conditions like RPE tears because there is no recommended treatment approach. For example, some studies have recommended delaying anti-VEGF treatment after an RPE tear, as anti-VEGF therapy may increase the tear area.9 However, unintended consequences can arise from waiting, as the lesion may grow and cause further problems, she added.
Conversely, another study found that continuing anti-VEGF therapy in patients with RPE tears and an active choroidal neovascular membrane is beneficial in suppressing the underlying disease.10 It is also worth noting that the strength of the anti-VEGF therapy may have an impact on the risk of RPE tear, Dr. Haynes remarked.
A retrospective study analyzing the incidence of RPE tears in ranibizumab-treated patients found no statistical differences in RPE tear incidence for 0.3 or 0.5mg ranibizumab.11 However, most (76%) of RPE tears in ranibizumab-treated patients were identified within 3 months of initiating treatment, while the majority (80%) of late-onset RPE tears occurred in control patients.11
Additionally, due to the fact that RPE tears can be serious vision-limiting events for most patients,2 it is critical to monitor the fellow eye for progression, especially considering that the majority of RPE tears are secondary to nAMD.1 Both Drs. Epshtein and Haynes recommended referring patients who develop poor vision in both eyes for low vision services to help manage their visual prognosis.
Comparing features of RPE tears and geographic atrophy
Dr. Haynes highlighted that while the lesions associated with RPE tears are atrophic, they do not occur due to a degenerative process (as seen in geographic atrophy [GA]), but because the RPE was ripped out from underneath the photoreceptors.
In fact, per Sadda et al., the classification system for OCT-defined atrophy caused by AMD specifically excludes findings of RPE tears.11 Consequently, although these patients may at first glance look like they have classic GA, they would not benefit from complement inhibition therapy, she added.
Conclusion
Although RPE tears are a relatively rare phenomenon, due to the potential for significant visual impairment, it is critical that eyecare practitioners understand how to identify and co-manage these patients with retina specialists as well as refer them to low vision services if necessary.
