July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
Annual change in ellipsoid zone (EZ) length in patients with choroideremia
Author Affiliations & Notes
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
    Ophthalmology, UT Southwestern Medical Center, Dallas, Texas, United States
  • Kirsten G Locke
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Yuhui Qui
    4D Molecular Therapeutics, Emeryville, California, United States
  • Jenny Holt
    4D Molecular Therapeutics, Emeryville, California, United States
  • David Kim
    4D Molecular Therapeutics, Emeryville, California, United States
  • Peter Francis
    4D Molecular Therapeutics, Emeryville, California, United States
  • Footnotes
    Commercial Relationships   David Birch, 4D Molecular Therapeutics (F); Kirsten Locke, None; Yuhui Qui, 4D Molecular Therapeutics (E); Jenny Holt, 4D Molecular Therapeutics (E); David Kim, 4D Molecular Therapeutics (E); Peter Francis, 4D Molecular Therapeutics (E)
  • Footnotes
    Support  NH Grant EY09076
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4476. doi:
  • Views
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      David G Birch, Kirsten G Locke, Yuhui Qui, Jenny Holt, David Kim, Peter Francis; Annual change in ellipsoid zone (EZ) length in patients with choroideremia. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4476.

      Download citation file:


      © ARVO (1962-2015); The Authors (2016-present)

      ×
  • Supplements
Abstract

Purpose : Choroideremia is an x-linked recessive progressive retinal degeneration caused by null mutations in the CHM gene (Xq21). Here we use spectral domain optical coherence tomography (SD-OCT) to measure annual rates of change in parameters reflecting photoreceptor structural preservation.

Methods : Patients with choroideremia and confirmed CHM mutations were enrolled in a multicenter natural history study. Inclusion criteria included intermediate disease with ≥ 30° visual field diameter (III4e) and preserved EZ within the central 10°. We report 12 months of follow-up from 20 patients (median age=35 yrs) from a single site. SD-OCT was obtained with a Heidelberg Spectralis. Linear b-scans were obtained in four meridians and measured by an experienced grader. Multiple steered OCT scans were obtained when necessary to cover the EZ. Rate of change was derived from a Linear Mixed Effect model (random intercept and slope) with time, eye, and interaction of eye and time as independent variables.

Results : All patients had preserved central vision (median letters OD=84; OS=83.5).
Two eyes had edges of preserved EZ that extended beyond the area imaged. For the remaining 38 eyes, the annual change in EZ length for each meridian is shown in the Table. The decrease in EZ at 1 year was significant for all but the vertical meridian. Differences in rates between eyes were not significant for any parameter.

Conclusions : The irregular EZ area in choroideremia can be sampled by obtaining b-scans from four meridians. The majority of meridians show a significant decrease over one year.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×