Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE)

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Download a pamphlet on CHRPE

CHRPE is a flat, pigmented spot within the outer layer of the retina at the back of the eye.

The spot is congenital, meaning that patients who have it are typically born this way. Many CHRPE’s are in the retinal periphery and are difficult to detect without a full retinal exam by a retina specialist, so they often go undetected for some time and then are identified during adult life.

Many CHRPE’s do not change or grow over time. In some patients, the borders of the spot grow very slowly over time but the spot remains flat. In the majority of patients, CHRPE is a benign finding that never causes a problem with the patient’s vision or life.

If the CHRPE is located in the center of the retina (macula), it can cause patients to have poor vision. But if the spot is in the periphery of the retina as in most patients, it causes no functional vision loss.

Very very rarely, a true tumor called an adenoma of the retinal pigment epithelium or an adenocarcinoma of the retinal pigment epithelium can arise from a CHRPE. None of these rare patients have ever developed metastases (cancer cells that traveled outside the eye).

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Rarely, patients who have multiple CHRPE’s and/or bilateral (both eyes) CHRPE’s or CHRPE’s with certain characteristic features are found to have Gardner’s Syndrome (a genetic condition also called Familial Adenomatous Polyposis, or FAP). Patients with this syndrome can have colon cancer and skin tumors in addition to the retinal findings. If you are found to have these features, Dr. Schefler will refer you to a gastroenterologist and/or geneticist for further testing. If you have a family history of this disorder, it is important to have both a retinal examination as well as routine colonoscopies.

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