H35.371-373 Macular Pucker

Corneal edema is a clinical sign of corneal disease.  The condition is characterized by an increase in corneal thickness secondary to an abnormal accumulation of fluid.  The excess fluid produces a swelling of the corneal tisssue and can result in a loss of stromal transparency that produces blurred vision or visual impairment.

Macular pucker is a clinico-anatomic description of structural damage to the retina caused by epimacular proliferation or vitreomacular traction.

• Epimacular proliferation is characterized by the formation of collagenous membranes on the surface of the retina  
• As the membranes grow, they can develop contractile properties that produce traction forces on the surface of the macula 
• Traction on the macula can result in traction maculopathies such as macular pucker, cellophane maculopathy, vitreomacular traction syndrome or macular hole

Common Names for Epimacular Proliferation Epiretinal membrane Cellophane maculopathy Macular pucker Preretinal macular gliosis Preretinal macular fibrosis Surface wrinkling maculopathy Epiretinal astrocytic membrane Preretinal vitreous membrane Silk-screen retinopathy

The histopathological changes needed to create epiretinal membranes usually begins with a posterior vitreous detachment.  In some people, it’s believed that the separation of the vitreous membrane from the retinal surface damages the retina structurally.

Structural Damage to the Eye

• Posterior vitreous detachment produces focal areas of damage on the inner retinal surface
• Immune system response to damaged areas results in inflammatory reaction on the inner retinal surface
• Glial cells from the neurosensory retina grow through breaks in the internal limiting membrane of the damaged retina
• Glial cells combine with inflammatory cells and collagen cells to form membranes
• Membranes form attachments to the inner retinal surface
• Membranes proliferate in the central retina and macula
• Membranes contract over time and create traction forces on retinal tissue
• Underlying retina becomes structurally damaged secondary to traction forces

Functional Damage to the Eye

• Traction on the macula produces decreased visual acuity based on the amount of force and its duration of action
• Vascular incompetence secondary to macular traction (especially in diabetic patients) results in progressive loss of acuity
• Diffuse macular edema usually results in decreased vision
• Foveal cysts usually results in decreased vision
• Macular detachment results in decreased vision

The main goal of the diagnostic evaluation in a patient with macular pucker is to accomplish the following:

• Evaluate epiretinal membrane density, thickness, and location in relation to the retina
• Determine the presence or absence of vascular incompetence secondary to the macular traction
• Determine the presence or absence of macular edema secondary to the traction 
• Prescribe a treatment program to manage the traction maculopathy and prevent permanent vision loss 

Patient History

The symptoms of traction maculopathy vary from no symptoms to severe visual impairment.  Patients with early disease may report blurred vision, decreased vision, or mild visual distortion.  More advanced presentations often produce metamorphopsia, micropsia, or other abnormal visual distortions of shape and size. 

Patients with traction maculopathy usually present with the following range of decreased visual acuity. 

• 20/25 acuity or better is present in 56-67% of patients
• 20/40 acuity or better is present in 75-85% of patients  
• 20/400 acuity or worse is present in 2-5% of patients

Clinical Appearance of the Retina

• A broad, glial epiretinal membrane adheres to the retinal surface and is anchored in the vascular arcades of the right eye

Macular pucker in in the right eye of a 78-year-old with 20/40 visual acuity

Norma macular appearance in the left eye of a 78-year-old with 20/20 visual acuity

DIAGNOSTIC TESTS

Retinal Laser Scan

Optical Coherence Tomography OCT testing can determine the presence or absence of an epiretinal membrane OCT testing canectively measure the effects of the epiretinal membrane on the macular surface Sector plot analysis on the right eye is abnormal- increased retinal thickness superiorly in the right macula Sector plot analysis on the left eye is normal High resolution scan reveals deformation of the retinal surface in the right eye High resolution scan reveals normal foveal contour in the left eye

Classification of epiretinal membranes is based on the apperance of the membrane and the underlying retina and blood vessels.

		Grade 0 membranes Translucent membranes not associated with any retinal distortion Known as cellophane maculopathy because of cellophane-like sheen reflected from the retinal surface as the membrane is visualized ophthalmoscopically. Patient is 70-year-old white female with 20/25 visual acuity
Patient in the following images is a 42-year-old black woman  Grade 1 membranes 20/30 visual acuity Vitreous membrane causes a wrinkling or puckering of the retinal surface via macular traction Macular traction force is tangential to the macular surface Traction on macular tissues produces gradual structural damage to the tissue Functional loss of vision secondary to macular damage is related to the intensity of the traction forces and their duration of action
Optical Coherence Tomography Shallowing of foveal depression in the left macula No cystoid macular edema No surgical treatment at this time unless patient is intolerant of visual blur or distortion
		Patient in the following images is a 72-year-old black woman Grade 2 membranes 20/50 visual acuity Vitreous membrane has a thick, opaque appearance Retinal edema Occasional small hemorrhages Occasional cotton-wool spots
Bilateral Presentation of Epiretinal Membrane 20/25 visual acuity in the left eye Grade 1 membrane in the left eye Grade 2 membrane in the right eye
		Optical Coherence Tomography OCT scan reveals epiretinal membrane overlying the right macula Sector plot analysis is abnormal — diffuse increase in macular thickness measured in microns High resolution scan is abnormal — deformation of the retinal profile with a loss of the foveal depression Macular thickness map is abnormal — increased probability of pathology based on comparison to normative database
Optical Coherence Tomography OCT scan reveals an epiretinal membrane surrounding the left macula Sector plot analysis is normal Profile of the retinal surface is normal Macular thickness map is normal

Differential diagnoses would include other diseases that share the clinical signs of macular pucker.  This would include diseases or conditions that produce abnormal vitreomacular interfaces

• Cystoid macula edema
• Posterior uveitis 
• Retinal vascular occlusive disease
• Diabetic retinopathy

Palliative Treatment

• Observation in the early stages of the disease and when there is minimal traction on the macula

Pharmacologic Treatment

On January 1, 2013, the FDA approved JETREA (ocriplasmin) for the treatment of symptomatic vitreomacular adhesion.  JETREA is a proteolytic enzyme delivered in a single 3.5mg/mL dose that alters the biochemistry of the vitreous to produce a pharmacologic vitreolysis.

JETREA Intravitreal Injection

• Liquefies vitreous
• Produces a separation between the vitreous cortex and the internal limiting membrane
• Resolution of vitremacular traction in 26% of treated group during Phase III clinical studies

Potential side effects include the following

• Intraocular inflammation
• Intraocular infection
• Intraocular hemorrhage
• Increased intraocular pressure
• Lens subluxation
• Floaters
• Retinal detachment
• Changes in color vision

Surgical Treatment

Pars Plana Vitrectomy with Membrane Peel

Potential side effects include the following

• Intraocular inflammation
• Intraocular infection
• Intraocular hemorrhage
• Retinal detachment
• Extended recovery period

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5.  Morris R, Witherspoon CD, Kuhn F, Nelson S, Priester B, Mayne R. Traction maculopathy.  Retinology Today. 
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