Ron Gallemore, M.D.
RPE Changes in the Macula: Take a closer look
Pigment changes in the macula may be benign or signs of serious pathology. Pigmented cells in the eye come from the melanin-containing retinal pigment epithelium and hence the term “RPE changes”. Here we review the major disorders associated with pigmentary changes in the macula and provide some guidelines for work-up and referral.
Juvenile Macular Degeneration:
This is defined as any macular degeneration before age 50. Stargardt’s disease is the most common form. Differential includes prior CSR, cone dystrophy, and Best’s disease. It is often associated with bull’s eye maculopathy from atrophy secondary to loss of RPE cells and some hyperpigmentation at the borders of atrophy caused by hypertrophy or proliferation of RPE cells. Prompt referral is important since patient may benefit from low vision aids and vitamin supplementation. Family members with the disease can be identified and genetic testing may be indicated.
Age Related Macular Degeneration:
Pigment mottling in AMD is associated with a high risk of vision loss. These patients should have additional testing even in the absence of symptoms including a macular OCT. If irregularities are noted or the patient is symptomatic, referral for further testing and possible treatment is warranted.
Dry and wet myopic macular degeneration can lead to RPE changes in the macula. All high myopes with degenerative changes should be screened annually with OCT and referred for any edema or new Amsler changes.
Inflammation in the posterior pole can also cause RPE changes. The vitreous should always be checked for cells. Visual field defects may also signify inflammation. Consider Presumed Ocular Histoplasmosis Syndrome (POHS), Punctate Inner Choroidopathy (PIC) in young myopic females, Acute Multifocal Placoid Pigment Epitheliopathy (AMPPE), Multifocal Choroiditis with Panuveitis and toxoplasmosis.
RPE cells are liberated after a retinal tear and can settle on the macula as part of an epiretinal membrane. Careful peripheral exam with scleral depression is warranted in such cases.
Blunt trauma can cause bruising of the retina (commotio retinae) with subsequent pigmentation. This carries an increased risk of choroidal neovascularization and such changes warrant a retinal referral.
Prior laser scars:
Focal laser for DME, RVO and even AMD can leave pigmentated scars in the macula. This can be a sign of over-aggressive treatment and may increase the risk of secondary choroidal neovascularization. Screening with an OCT for overlying retinal edema and referral for angiographic studies may be warranted, particularly for those patients with Amsler changes.
New clumps of pigmentation may be a sign of cancer – Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP) is associated with cancers of the lung and elsewhere and results from systemic hormonal stimulation of RPE cell growth or melanin production. CHRPE lesions, in contrast, are congenital and associated with colon cancer in some families (Gardner’s syndrome).
RPE changes that have been documented before, appear stable and are found in asymptomatic patients with "normal" vision may not require a work up. New onset RPE changes, or old changes associated with visual symptoms warrant additional testing and retinal consultation should be considered.
Retina Macula Institute and the Retina Macula Research Center
Edmund Yong, B.A.
Ron P. Gallemore, M.D., Ph.D.
Founder and Director
South Bay 310-944-9393