Adaptive optics imaging of the outer retinal tubules in Bietti's crystalline dystrophy

R. Battu, M.C. Akkali, D. Bhanushali, P. Srinivasan, R. Shetty, T.T.J.M. Berendschot, J.S.A.G. Schouten, Carroll Webers

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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.Eye advance online publication, 26 February 2016; doi:10.1038/eye.2016.22.
Original languageEnglish
Pages (from-to)705-712
Number of pages8
JournalEye
Volume30
Issue number5
DOIs
Publication statusPublished - May 2016

Keywords

  • COHERENCE-TOMOGRAPHY
  • TUBULATION
  • ECCENTRICITY
  • DENSITY
  • CYP4V2

Cite this

Battu, R., Akkali, M. C., Bhanushali, D., Srinivasan, P., Shetty, R., Berendschot, T. T. J. M., Schouten, J. S. A. G., & Webers, C. (2016). Adaptive optics imaging of the outer retinal tubules in Bietti's crystalline dystrophy. Eye, 30(5), 705-712. https://doi.org/10.1038/eye.2016.22