Optical coherence tomography and optical coherence tomography angiography imaging in Bietti crystalline dystrophy
Ophthalmic Genetics, cilt.41, sa.2, ss.194-197, 2020 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 41 Sayı: 2
- Basım Tarihi: 2020
- Doi Numarası: 10.1080/13816810.2020.1750036
- Dergi Adı: Ophthalmic Genetics
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, MEDLINE
- Sayfa Sayıları: ss.194-197
- Anahtar Kelimeler: Choriocapillaris blood flow, crystalline deposit, outher retinal tubulation, vessel density
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Background: Bietti crystalline dystrophy (BCD) is a rare autosomal recessive disorder due to genetic defect in the CYP4V2 gene. BCD is a disease characterized by shiny yellow crystalline deposits in the retina with progressive atrophy of the retinal pigment epithelium and choriocapillaris. Our aim is to present ocular imaging findings of a patient with BCD.Materials and Metods: A 38-year-old female patient with BCD was evaluated and the findings of optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) were examined.Results: OCT imaging revealed multiple outer retinal tubulations (ORTs) with a few hyperreflective crystalline deposits. OCTA imaging showed that the vessel density of superficial anddeep capillary plexus and choriocapillaris blood flow were significantly decreased. ORTs were composedof multiple microtubules as finger-like protrusions that joined the macrotubules.Conclusion: In BCD, crystallinedeposits, ORTs and retinal vascular morphology can be shown in detail using OCT and OCTA.