Two Female Siblings With Bietti Crystalline Retinopathy Without Corneal Dystrophy.

Ha, Seung Min; Yu, Hyeong Gon

doi:2009.50.7.1120

J Korean Ophthalmol Soc > Volume 50(7); 2009 > Article

Case Report

Journal of the Korean Ophthalmological Society 2009;50(7):1120-1127.
DOI: https://doi.org/10.3341/jkos.2009.50.7.1120 Published online July 15, 2009.

Two Female Siblings With Bietti Crystalline Retinopathy Without Corneal Dystrophy.

Seung Min Ha, Hyeong Gon Yu

¹Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea. hgonyu@snu.ac.kr
²Sensory Organ Institute, Medical Research Center, Seoul National University, Seoul, Korea.

두 자매에서 발생한 각막이영양증을 동반하지 않은 Bietti 결정망막병증

하승민¹ㆍ유형곤^1,2

Department of Ophthalmology, Seoul National University College of Medicine¹, Seoul, Korea / Sensory Organ Institute, Medical Research Center, Seoul National University², Seoul, Korea

Abstract

PURPOSE
To report clinical and functional results in two female siblings with Bietti crystalline retinopathy. CASE SUMMARY: Recently, a 48-year-old female with bilateral intraretinal depositions presented with a complaint of decreased visual acuity and night blindness in both eyes. Several tiny glistening yellow intraretinal crystalline depositions were observed. Fluorescein angiography showed a well-demarcated choriocapillaris filling defect and pigment epithelial window defect. Electrophysiologic tests showed decreased amplitude and OCT scans showed fine intraretinal lesions with increased signal intensity. In addition, a 50-year-old female sibling presented with bilateral yellow, intraretinal crystalline depositions. A choriocapillaris filling defect and pigment epithelial window defect in a fluorescein angiography was observed. Electrophysiologic tests showed severely decreased amplitude. CONCLUSIONS: Two female siblings with Bietti crystalline retinopathy are reported.

Key Words: Bietti crystalline retinopathy;Siblings