두 자매에서 발생한 각막이영양증을 동반하지 않은 Bietti 결정망막병증

Two Female Siblings With Bietti Crystalline Retinopathy Without Corneal Dystrophy

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.