INCIDENCE AND PREDICTIVE VALUE OF CONGENITAL HYPERTROPHY OF RETINAL-PIGMENT EPITHELIUM IN CHINESE FAMILIAL ADENOMATOUS POLYPOSIS PATIENTS

Objective To investigate the incidence and predictive value of congeni tal hypertrophy of retinal pigment epithelium (CHRPE) in the Chinese f amiliar adenomatous polyposis (FAP) patients. Methods Eleven FAP patie nts and 28 at risk relatives from 7 families were examined for CHRPE l esions. Results CHRPE was present in all 11 FAP patients. Nine CHRPE l esions were found in 9 of the 28 at risk relatives. There were great i ntra and inter-familial variations of the CHRPE lesions. Two at risk r elatives were diagnosed to have FAP only after our screening and subse quent proctosigmoidoscopy. Nine at risk relatives were put under high surveillance because of the presence of CHRPE. Conclusion The incidenc e of CHRPE in the FAP patients in our study is 100%. Eye examination f or CHRPE for FAP patients and their at risk relatives is of very high value. A central FAP registry is thus recommended.