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.
Subject(s)
Female , Humans , Middle Aged , Crystallins , Eye , Fluorescein Angiography , Night Blindness , Siblings , Visual AcuityABSTRACT
Crystalline retinopathy is a tapetoretinal degeneration characterized by glistening yellow intraretinal crystals in the posterior pole, chorioidal sclerosis, and corneal crystalline dystrophy. We have recently encountered a 36 year-old female patient with bilateral intraretinal crystalline depositions who presented with a complaint of visual disturbance in her right eye for the last six months. Typical fundus findings of crystalline deposition in the posterior pole, tapetoretinal dysfunctions in the electrophysiologic tests and choriocapillaris filling defects in the fluorescein angiogram suggest that this the disorder is a widespread choriocapillaris and tapetoretinal disorder. There are no significant abnormal findings in serologic and radiologic examinations.
Subject(s)
Adult , Female , Humans , Crystallins , Fluorescein , Retinitis Pigmentosa , SclerosisABSTRACT
A 41-year-old woman showed a bilateral chorioretinal dystrophy with retinal crystal deposits, geographic areas of atrophy of the retinal pigment epithelium, and choriocapillaris in the posterior pole. Crystals were observed in the sensory retina and on the retinal vessels. But the patient did not have corneal crystals. There was no visual symptom, however, the results of electrophysiologic tests were subnormal. To our knowledge, this is the first report of Bietti's crystalline retinopathy in Korea.