나비모양 망막이상증의 빛간섭단층촬영 소견 1예

• 영문명: Spectral Domain Optical Coherence Tomography Findings of Butterfly Shaped Pigment Dystrophy
• 발행기관: 대한안과학회
• 저자명: 김경민 박한상 하승민 유용성 김시열,Kyoung Min Kim, MD, Han Sang Park, MD, Seung Min Ha, MD, Yong Sung You, MD, Si Yeol Kim, MD
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume53,number11, 1699~1703쪽, 전체 5쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2012.11.15

영문 초록

Purpose: The butterfly-shaped pigment dystrophy is an extremely rare autosomal dominant retinal disorder. The authors present a case of butterfly-shaped pigment dsytrophy not reported previously in Korea. Case summary: A 45-year-old man had bilateral blurred vision for 2 years. His visual acuity was 0.2 in the right eye, and 0.3 in the left and was uncorrected. Funduscopically, yellow pigment deposits were present at the level of retinal pigment epithelium (RPE) in the central macula of both eyes. Fluorescein angiography (FA) revealed a central, dark, butterfly- shaped lesion surrounded by a region of hyperfluorescence, Spectral domain optical coherence tomography (OCT) showed thick elevation of the RPE with hyperreflectivity and disruption of the inner and outer segment (IS/OS) interface of the photoreceptors. The patient had normal color vision, visual field and electroretinograms and reduced electrooculograms. Conclusions: In general, butterfly-shaped pigment dystrophy is known to have good visual prognosis. However, in some cases the disease can be a chronic progressive disorder with secondary involvement of the photoreceptors, as exemplified this patient. The authors anticipate more detail regarding the natural course of this disease will be obtained through spectral domain OCT.

키워드

Butterfly shaped pigment dystrophy, Spectral domain optical coherence tomography