Changes in the retinal pigment epithelium close to retinal vessels in familial adenomatous polyposis

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is known to occur in patients with familial adenomatous polyposis. Its relation to the course of the retinal blood vessels is emphasized in this publication. A 15-year-old girl with familial polyposis coli showed a longitudinal strip of whitish change following the course of a superior nasal artery of the left eye, falsely appearing to enclose the blood vessel like a sheath. A 34-year-old woman from another family with polyposis coli also showed a longitudinally orientated area of hypo- and hyperpigmentation close to temporal retinal veins. Two further patients revealed hyperpigmentation of the RPE under retinal vessels. It is hypothesized that these changes indicate an effect of the hypo- and hyperpigmentations of the RPE on the development of retinal vessels. In four patients, multiple dotlike hyperpigmentations were found in the extreme periphery of the retina; however, the bigger patchy hyperpigmentations were predominantly located in the midperiphery of the retina.