Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a well circumscribed, flat, pigmented tumor that can occur anywhere in the fundus . It often shows depigmented lacunae within the lesion and a surrounding pale halo. It can occur as a solitary lesion or it can be multiple as part of congenital grouped pigmentation lesion (Fig. 8.22). Similar but distinct multifocal pigmented lesions may be a marker for familial adenomatous polyposis and Gardner's syndrome in which patients have a high likelihood of developing colonic cancer .
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