What is macular puckering of the retina?

What is Macular Puckering of the Retina?

Macular puckering, also known as epiretinal membrane (ERM), is a thin, translucent, cellophane-like membrane that forms on the inner surface of the retina over the macula, which can contract and cause wrinkling, distortion, and visual symptoms. 1

Definition and Pathophysiology

Macular pucker represents fibrocellular proliferation on the neurosensory retinal surface, comprising reactive cellular elements, vitreous structures, and fibrotic components that develop through a wound-healing response. 2

The condition develops when:

• The posterior vitreous detaches from the retina but remains adherent to the macula, creating conditions for abnormal cellular proliferation at the vitreomacular interface. 1, 2
• Retinal pigment epithelial (RPE) cells and/or retinal glial cells proliferate in response to this abnormal adhesion. 2
• The membrane adheres tightly to the internal limiting membrane of the retina and can contain native vitreous collagen. 1

Clinical Appearance and Types

Thin (Cellophane) Membranes

• Appear as thin, translucent, cellophane-like membranes on the retinal surface that may not initially cause tractional changes. 1
• The underlying neurosensory retina may appear relatively normal. 1

Thick (Fibrotic) Membranes

• Have a thicker, white, fibrotic appearance that obscures the underlying retina and are more likely to become symptomatic. 1
• These membranes are more likely to displace the macula than thinner, translucent ERMs. 1

Structural Changes to the Macula

When epiretinal membranes contract, they cause:

• Folds in the retina and distortion of the inner and outer macular layers. 1
• Traction on retinal vessels and displacement of the macula (ectopia). 1
• Loss or distortion of the normal foveal depression. 1
• Development of cystoid spaces, lamellar macular holes, or even full-thickness macular holes. 1

Common Symptoms

Patients experience metamorphopsia (distorted vision), decreased visual acuity, and difficulty using both eyes together, which can significantly impact reading, driving, and binocular vision. 1, 3

Specific symptoms include:

• Visual distortion and wavy lines (metamorphopsia). 1
• Blurred or decreased central vision. 1
• Diplopia (double vision) in some cases. 1
• Patients commonly report closing one eye while reading to eliminate distortion from the affected eye. 1

Epidemiology and Risk Factors

Prevalence

• Affects approximately 30 million adults in the United States aged 43 to 86 years. 1
• Prevalence ranges from 2.2% to 34.1% depending on the population studied and diagnostic method used. 1
• Can be bilateral in 20% to 35% of cases. 1, 2

Key Risk Factors

• Increasing age is the single most consistent and important risk factor across all population studies. 2
• Female gender significantly confers higher risk based on systematic review. 2
• Posterior vitreous detachment (PVD) is a major associated factor. 2

Classification: Idiopathic vs. Secondary

Idiopathic ERM

• The majority of cases are idiopathic and related to age-related posterior vitreous detachment. 2
• Develops without any identifiable underlying ocular pathology. 2

Secondary ERM

Secondary ERMs occur after specific retinal or ocular pathologies, including:

• Retinal breaks, tears, or detachments. 2, 4, 5, 6
• Diabetic retinopathy and retinal vein occlusions. 2
• Uveitis and ocular inflammatory diseases. 2
• Intraocular surgery (particularly cataract surgery) and ocular trauma. 2
• Retinal laser or cryotherapy treatment. 2

Diagnostic Approach

Spectral domain optical coherence tomography (OCT) is the highly sensitive and routine method used to diagnose and characterize epiretinal membranes. 1

On OCT imaging:

• ERM appears as a hyper-reflective layer on the inner surface of the retina. 1
• Shows a corrugated appearance with pegs emanating from the inner retinal surface. 1
• Demonstrates elevation of the normal foveal depression with inner retinal folds. 1
• Reveals thickening of the macula with associated cystoid spaces in various retinal layers. 1

Clinical Course

Many patients have stable vision with few symptoms, while others experience progressive loss of visual function. 1

Important considerations:

• Visual acuity often does not change dramatically during short-term follow-up. 1
• The presence of ERM does not automatically indicate need for surgery—symptoms drive treatment decisions. 2
• Distinguishing idiopathic from secondary ERM is critical, as secondary forms require addressing the underlying condition. 2

Common Pitfalls

• Do not confuse vitreopapillary traction with optic nerve disorders such as papilledema, as raised edges of adherent vitreous around the optic nerve head can mimic these conditions. 1
• Recognize that ERM and vitreomacular traction often occur together, with vitreous adherent to the macula in up to 57-65% of cases. 1
• In younger patients, macular pucker tissue is typically more fibrous and contractile compared to the thin cellophane appearance in elderly patients, reflecting more rapidly changing tissue. 7