Journal of the Korean Ophthalmological Society 2000;41(11):2509-2513.
Published online November 1, 2000.
Atypical Congenital Hypertrophy of the Retinal Pigment Epithelium in Gardner's Syndrome.
Weon Seuk Rohr, Jun Keun Yoon, Seok Joon Lee, Jong Hyuck Lee
Department of Ophthalmology, Wonju Christian Hospital, Yonsei University Wonju College of Medicine #162 Ilsan-dong, Wonju, 220-701, Korea.
가드너씨 증후군에 동반된 비전형적 선천성 망막색소상피비대증 1 례
노원석(Weon Seuk Rohr),윤준근(Jun Keun Yoon),이석준(Seok Joon Lee),이종혁(Jong Hyuck Lee)
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.
Key Words: Congenital hypertrophy of the retinal pigment epithelium (CHRPE);Gardner's syndrome


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