What is the management approach for patients with Retinal Pigment Epithelial Rip (RPER) or related conditions, such as Retinal Pigment Epithelial Detachment (RPED) or Tears (RPT), particularly those with a background of age-related macular degeneration (AMD)?

Management of Retinal Pigment Epithelial Rip/Tear (RPET) in Age-Related Macular Degeneration

Continue anti-VEGF therapy immediately after RPE tear diagnosis, as tears are not a contraindication to ongoing treatment and cessation leads to worse outcomes. 1

Understanding RPE Tears in AMD Context

RPE tears occur as a complication of neovascular AMD, typically in the setting of fibrovascular pigment epithelial detachment (FVPED), with an incidence ranging from 8-27% in patients receiving anti-VEGF therapy. 2, 3 The mechanism involves tangential forces on the RPE monolayer overlying a vascularized PED, creating a demarcation line between areas of RPE atrophy and RPE folds. 2

Risk Factors for RPE Tear Development

• Large PED height is the strongest predictor, with tears occurring when PED height increases significantly (mean increase of 363.8 µm before rupture in one cohort). 4
• Baseline leakage parameters and distorted RPE contour on OCT are associated with higher tear risk. 3
• Subretinal fluid presence on OCT correlates with tear occurrence. 3
• The choice of anti-VEGF agent (ranibizumab, aflibercept, or bevacizumab) does not affect tear incidence—all carry equal risk. 2

Treatment Algorithm After RPE Tear Occurs

Immediate Management

Do not discontinue anti-VEGF therapy. 1 The American Academy of Ophthalmology explicitly states that RPE tears may occur with or without treatment modalities, yet this is not a contraindication to continued anti-VEGF therapy. 1

• Continue the same anti-VEGF regimen (aflibercept, ranibizumab, or bevacizumab) that was being used. 1, 2
• Maintain treatment frequency or increase injection frequency as needed based on disease activity. 2, 5
• Eyes with RPE tears typically require more injections than control eyes after tearing. 5

Monitoring Protocol

• Perform OCT at each visit to assess for persistent or recurrent subretinal fluid and monitor RPE defect progression. 3, 4
• Fluorescein angiography may be used to evaluate baseline leakage parameters and ongoing CNV activity. 3
• Monitor for progressive RPE atrophy, which increases significantly over time (mean increase from 3.35 mm² to 6.81 mm² over 2 years). 4

Visual Prognosis and Timing Considerations

Early vs. Late Tears

The timing of RPE tear occurrence significantly impacts outcomes, with a critical breakpoint at approximately 6 months (182 days) of treatment. 5

Early tears (occurring before 6 months):

• Baseline VA is typically lower (mean 53.6 letters). 5
• Vision often improves before the tear occurs (+5.6 letters from baseline). 5
• Immediate VA loss after tear (-8.3 letters), but recovery occurs with continued treatment. 5
• Long-term outcomes at 12 and 24 months show no significant difference compared to matched controls without tears. 5

Late tears (occurring after 6 months):

• Baseline VA is higher (mean 63.4 letters). 5
• VA is already declining before the tear occurs (55.5 letters vs. 66.9 in controls). 5
• Continued VA decline after tear, with persistently worse outcomes compared to controls at all endpoints. 5
• Poor response to therapy before tearing predicts worse long-term outcomes. 5

Expected Outcomes

• Visual stabilization without significant improvement is the most realistic expectation in eyes with RPE tears. 3, 4
• In one series, 87.5% of patients with RPE tears achieved stabilization or improvement, though significant improvement (≥15 letters) was not observed. 3
• Mean VA decrease at 2-year follow-up is approximately 10.5 letters, though this did not reach statistical significance. 4
• The amount of anti-VEGF injections after rupture does not significantly affect visual outcome. 4

Critical Clinical Pitfalls to Avoid

Never discontinue anti-VEGF therapy after RPE tear diagnosis. 1, 2 Continuous monitoring and adherence to anti-VEGF therapy leads to better anatomical and functional results in the long term. 2

• Do not assume all RPE tears have the same prognosis—timing matters significantly. 5
• Do not expect visual improvement in most cases; stabilization is the goal. 3, 4
• Do not ignore the fellow eye, which remains at high risk for CNV development and requires AREDS2 supplementation. 6
• Recognize that RPE atrophy will progress despite treatment, implying ongoing contraction and/or macular atrophy. 4

Adjunctive Considerations

• Ensure patients are on AREDS2 supplementation (vitamin C, vitamin E, zinc, copper, lutein 10mg, zeaxanthin 2mg) to reduce progression risk in the fellow eye. 6
• Counsel patients that central vision loss is common but total blindness is extremely rare, as peripheral vision remains preserved. 6
• Refer to vision rehabilitation services for optical/electronic magnifying devices and reading aids. 6
• Address smoking cessation, as it remains the key modifiable risk factor for AMD progression. 6