congenital hypertrophy of the retinal pigment epithelium


congenital hypertrophy of the retinal pigment epithelium

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CHRPE

A thickening of the retinal pigment epithelium present at birth. The thickening appears on funduscopic examination as an oval, black, clearly demarcated lesion within which there are atrophic areas surrounded by a halo that has no pigment. It has been associated with and has been considered to be an ocular marker of familial adenomatous polyposis.

retinal pigment epithelium, congenital hypertrophy of the (CHRPE) 

A benign congenital proliferation of the retinal pigment epithelium which may appear unilaterally as a dark-grey or black, round or oval lesion in the fundus (typical form) or in other cases as multiple smaller lesions grouped together and resembling 'bear tracks'. In atypical form the lesions are bilateral. The latter is associated with familial adenomatous polyposis (an inherited condition characterized by neoplasms derived from epithelial tissue which appear as polyps throughout the rectum and colon and may become malignant) and its variants, Turcot syndrome (neuroepithelial tumours in the central nervous system) or Gardner's syndrome (soft tissue tumours).