congenital hypertrophy of the retinal pigment epithelium

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congenital hypertrophy of the retinal pigment epithelium



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).
References in periodicals archive ?
Upon discovering a single, unilateral CHRPE on examination, the clinician should photograph and document the location, size, colour, and number of lacunae associated with the CHRPE.
CHRPEs appear as single, flat, round lesions with sharply demarcated smooth or scalloped margins (see Figure 1).
On autofluorescence, CHRPEs will hypofluoresce and can be differentiated from choroidal naevi by the lack of fluorescent lipofuscin.
The well-defined and deeply pigmented lesions of CHRPE may occur as a single tiny or large patch, or it may emprise a number of patches, clustered or widely scattered.
However, given the limited resources available to the NHS, it is not appropriate to refer every patient with a choroidal naevus or CHRPE (> 10% of the population) for assessment in secondary care.
Which of the following is the MOST appropriate management of CHRPE?
As CHRPE are such a common finding I think it's important to have a clear understanding.
Thank you for your enquiry, we are glad to have the opportunity to expand a little on the topic of CHRPE. The vast majority of these pigmented lesions are innocuous and do not require referral.
d) Multiple CHRPE can be associated with an inherited bowel condition
Which of the following statements about congenital hypertrophy of the retinal pigment epithelium (CHRPE) is FALSE?
Atypical CHRPE lesions are associated with cardiomyopathy