Atypical Congenital Hypertrophy of the Retinal Pigment Epithelium in Gardner's Syndrome.
- Author:
Weon Seuk ROHR
1
;
Jun Keun YOON
;
Seok Joon LEE
;
Jong Hyuck LEE
Author Information
1. Department of Ophthalmology, Wonju Christian Hospital, Yonsei University Wonju College of Medicine #162 Ilsan-dong, Wonju, 220-701, Korea.
- Publication Type:Original Article
- Keywords:
Congenital hypertrophy of the retinal pigment epithelium (CHRPE);
Gardner's syndrome
- MeSH:
Colectomy;
Colon;
Epidermal Cyst;
Gardner Syndrome*;
Humans;
Hypertrophy*;
Ileostomy;
Osteoma;
Retinal Pigment Epithelium*;
Retinaldehyde*
- From:Journal of the Korean Ophthalmological Society
2000;41(11):2509-2513
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital hypertrophy of the retinal pigment epithelium (CHRPE)is a well circumscribed, flat, pigmented fundus lesion that have a variety of shapes. It is the most common extracolonic manifestation of Gardner's syndrome. The number and shape of CHRPE should arouse suspicion of increased risk of polyposis.We experienced one case of Gardner's syndrome in a family history of colonic carcinoma that showed atypical congenital hypertrophy of the retinal pigment epithelium, osteoma, epidermal cyst and polyposis combined with colonic carcinoma.A total colectomy and ileostomy were performed.So we reported it with the review of literature of clinical value of CHRPE as a predictive marker of Gardner's syndrome.
