Description

Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE) is a disorder of the retinal pigment that may be associated with familial adenomatous polyposis (FAP).


 

CHRPE:

(1) are ovoid or round discrete pigmented lesions

(2) >= 150 microns in diameter

(3) may have a depigmented halo around them

(4) histologically are choristomas or hamartomoas

 

They need to be differentiated from smaller (< 150 micron) pigmented lesions that can be found in normal patients without FAP.

 

The lesions of CHRPE are usually bilateral but can also be unilateral.

 

Not everyone with FAP has CHRPE, and not everyone with CHRPE has FAP. The correlation for each depends on the physician performing the exam, the population and other factors.

 

Indications to work the patient up for FAP (Meyer et al):

(1) family history of FAP and one or more CHRPE

(2) more than 3 CHRPE in one eye

(3) bilateral CHRPE

 


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