What are the primary causes of retinal pigment epithelial layer atrophy in a 60-year-old male with a history of myopia?

Causes of Retinal Pigment Epithelial Layer Atrophy in a 60-Year-Old Male with Myopia

In a 60-year-old male with myopia, the primary causes of RPE atrophy are age-related macular degeneration (most common at this age), pathologic myopia with associated macular complications, and central serous chorioretinopathy with chronic RPE changes. 1, 2

Age-Related Macular Degeneration (AMD)

AMD is the leading cause of RPE atrophy in this age group and must be considered first. 1

• Geographic atrophy represents the extreme form of RPE abnormalities, characterized by alterations of the RPE-Bruch's membrane complex leading to hypopigmentation and/or hyperpigmentation 1
• The pathogenic sequence involves RPE dysfunction, accumulation of intracellular material, abnormal extracellular deposits (basal laminar and linear deposits), changes in Bruch's membrane composition and permeability, ultimately leading to either RPE atrophy or choroidal neovascularization 3
• If the patient has been treated with anti-VEGF therapy for neovascular AMD, this itself increases atrophy risk—the number of anti-VEGF injections is statistically significantly associated with progression of atrophy (odds ratio 1.35,95% CI 1.05-1.73) 4

Pathologic Myopia-Related Atrophy

Myopia is a critical risk factor that independently causes RPE atrophy through multiple mechanisms. 1, 2

• Myopic macular neovascularization leads to macular atrophy—all 25 eyes with extreme macular schisis in one series had macular atrophy caused by myopic CNV 2
• High myopia causes mechanical stretching and thinning of the RPE-Bruch's membrane-choroid complex, leading to progressive atrophy 1
• Myopic eyes are at increased risk for retinal detachment (10% risk in fellow eyes), and chronic detachment can cause secondary RPE atrophy 1
• Lacquer cracks and posterior staphyloma in pathologic myopia directly damage the RPE layer 2

Central Serous Chorioretinopathy (CSC)

Chronic or recurrent CSC leads to progressive RPE atrophy, particularly relevant in this demographic. 1

• CSC typically affects individuals between 35-50 years but can occur up to age 83, making it relevant for a 60-year-old 1
• The disease course can be complicated by atrophy of the RPE and/or photoreceptors as a consequence of chronic subretinal fluid 1
• Defects in the RPE outer blood-retina barrier occur above choroidal abnormalities, and persistent fluid leads to RPE decompensation and atrophy 1

Secondary Causes to Consider

Pigment epithelial detachments (PEDs) can lead to RPE atrophy regardless of underlying etiology. 1, 5

• Serous, hemorrhagic, or fibrovascular PEDs separate the RPE from Bruch's membrane and can result in atrophy upon resolution 5
• PEDs are common manifestations in both dry and wet AMD 5

Inflammatory chorioretinopathies should be considered if there are additional clinical features suggesting inflammation 6

• Multifocal choroiditis can cause RPE atrophy, though it typically affects younger patients 6

Ocular trauma history should be elicited, as blunt or penetrating injuries alter vitreoretinal structure and can lead to chronic RPE changes 1

Critical Diagnostic Algorithm

Perform multimodal imaging to differentiate these causes:

• Optical coherence tomography (OCT) to characterize RPE morphology, assess for sub-RPE deposits, measure choroidal thickness, and identify macular schisis in myopic eyes 1, 7, 2
• Fundus autofluorescence to map areas of RPE atrophy and dysfunction 4
• OCT angiography to detect occult choroidal neovascularization 1
• Fluorescein angiography and ICG if neovascularization is suspected 1

Key Clinical Pitfalls

Do not assume all RPE atrophy in myopic patients is purely degenerative—actively search for treatable neovascular components that may be contributing to atrophy progression 4, 2

Recognize that anti-VEGF treatment itself accelerates atrophy, so the presence of treatment history is both diagnostic (suggests prior CNV) and prognostic (predicts faster atrophy progression) 4

In highly myopic eyes, extreme macular schisis can simulate retinal detachment on examination—OCT is essential to identify thin remnants of outer retinal tissue above the RPE that distinguish schisis from true detachment 2