Autofluorescence and OCT features of Bietti’s crystalline dystrophy
- A Ayata1,
- S Tatlıpınar2,
- M Ünal1,
- D Erşanlı1,
- A H Bilge1
- 1Department of Ophthalmology, GATA Haydarpasa Training Hospital, Istanbul, Turkey
- 2Yeditepe University School of Medicine, Istanbul, Turkey
- A Ayata, GATA Haydarpasa Egitim Hastanesi Lojmanlari E Blok, Daire:7, Uskudar, Istanbul, Turkey; ali_ayata{at}yahoo.com
- Accepted 13 February 2008
Bietti’s crystalline dystrophy (BCD) is an autosomal recessive retinal degeneration characterised by innumerable glistening intraretinal dots scattered over the fundus. This retinal degeneration was first described in three patients by Bietti in 1937 with crystalline deposits in the retina and limbal cornea.1 Progressive retinal degeneration and sclerosis of the choroidal vessels ultimately result in progressive night blindness and constriction of the visual field approximately in the third and fourth decade of life. Histopathological studies disclosed evidence of advanced panchoroidal atrophy characterised by a marked loss of the RPE and choriocapillaris and mild retinal gliosis. Focal hypertrophy, hyperplasia and intraretinal migration of RPE cells were observed.2
To the best of our knowledge, the optical coherence tomography (OCT) features of BCD have been described in a single case only.3 Herein, we report the OCT, fundus autofluorescence (FAF) and near-infrared autofluorescence (NIR AF) findings in three patients with Bietti’s crystalline retinopathy. …
