title: Adaptive optics imaging of the outer retinal tubules in Bietti's crystalline dystrophy.
abstract: PurposeTo study the outer retinal tubules using spectral domain optical coherence tomography and adaptive optics and in patients with Bietti's crystalline dystrophy.MethodsTen eyes of five subjects from five independent families with Bietti's crystalline Dystrophy (BCD) were characterized with best-corrected visual acuity (BCVA), full-field electroretinography, and fundus autofluorescence (FAF). High-resolution images were obtained with the spectral domain optical coherence tomography (SD-OCT) and adaptive optics (AO).ResultsSD-OCT showed prominent outer retinal layer loss and outer retinal tubulations at the margin of outer retinal loss. AO images displayed prominent macrotubules and microtubules with characteristic features in eight out of the 10 eyes. Crystals were present in all ten eyes. There was a reduction in the cone count in all eyes in the area outside the outer retinal tubules (ORT).ConclusionsThis study describes the morphology of the outer retinal tubules when imaged enface on the adaptive optics in patients with BCD. These findings provide insight into the macular structure of these patients. This may have prognostic implications and refine the study on the pathogenesis of BCD.
authors.affiliation: Department of Vitreoretina, Narayana Nethralaya, Bangalore, India.
authors.forename: R
authors.initials: R
authors.lastname: Battu
authors.affiliation: Department of Vitreoretina, Narayana Nethralaya, Bangalore, India.
authors.forename: M C
authors.initials: MC
authors.lastname: Akkali
authors.affiliation: Department of Vitreoretina, Narayana Nethralaya, Bangalore, India.
authors.forename: D
authors.initials: D
authors.lastname: Bhanushali
authors.affiliation: Department of Vitreoretina, Narayana Nethralaya, Bangalore, India.
authors.forename: P
authors.initials: P
authors.lastname: Srinivasan
authors.affiliation: Department of Vitreoretina, Narayana Nethralaya, Bangalore, India.
authors.forename: R
authors.initials: R
authors.lastname: Shetty
authors.affiliation: Department of Imaging, University Eye Clinic Maastricht, Maastricht, The Netherlands.
authors.forename: T T J M
authors.initials: TT
authors.lastname: Berendschot
authors.affiliation: Department of Imaging, University Eye Clinic Maastricht, Maastricht, The Netherlands.
authors.forename: J S A G
authors.initials: JS
authors.lastname: Schouten
authors.affiliation: Department of Imaging, University Eye Clinic Maastricht, Maastricht, The Netherlands.
authors.forename: C A
authors.initials: CA
authors.lastname: Webers
mesh_terms: D000328:Adult; D003317:Corneal Dystrophies, Hereditary / Q000000981:diagnostic imaging* / Q000473:pathology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology*; D061848:Optical Imaging; D020419:Photoreceptor Cells, Vertebrate / Q000473:pathology*; D012164:Retinal Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D012165:Retinal Ganglion Cells / Q000473:pathology*; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult