June 2023
Volume 64, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2023
A morphometric analysis with adaptive optics imaging of the retinal arterial vascular architecture in RPE65-associated RP patients undergone therapy with voretigene neparvovec
Author Affiliations & Notes
  • Friederike Charlotte Kortuem
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • Melanie Kempf
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
    Department of Ophthalmology, Center for rare diseases, University of Tubingen, Tubingen, Germany
  • Constanze Kortuem
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • laura kuehlewein
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • Krunoslav Stingl
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • Katarina Stingl
    Department of Ophthalmology, Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
    Department of Ophthalmology, Center for rare diseases, University of Tubingen, Tubingen, Germany
  • Footnotes
    Commercial Relationships   Friederike Kortuem Novartis, Code R (Recipient); Melanie Kempf None; Constanze Kortuem None; laura kuehlewein None; Krunoslav Stingl None; Katarina Stingl ProQR, Santen, Janssen, Novartis, ViGeneron, Code C (Consultant/Contractor)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2023, Vol.64, 1053. doi:
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      Friederike Charlotte Kortuem, Melanie Kempf, Constanze Kortuem, laura kuehlewein, Krunoslav Stingl, Katarina Stingl; A morphometric analysis with adaptive optics imaging of the retinal arterial vascular architecture in RPE65-associated RP patients undergone therapy with voretigene neparvovec. Invest. Ophthalmol. Vis. Sci. 2023;64(8):1053.

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

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Abstract

Purpose : To investigate the change of retinal arterial architecture after treatment with voretigene neparvovec in patients with Leber congenital amaurosis type 2 (LCA2) and early-onset severe retinal dystrophy (EOSRD) cause by bi-allelic mutations in the RPE65 gene.

Methods : All eyes treated at the university clinic of Tubingen, Germany, with voretigene neparovevec received imaging with adaptive optics ophthalmoscopy (AO). However, only six eyes of four patients were able to be follow-up over time due to vast atrophy or malfixation. For each eye five different positions at arterial vessels were selected and the lumen diameter (LD), the wall-to-lumen ratio (WLR) and the wall crosssectional area (WCSA) were measured over an observational period of 12 months with AO.

Results : AO imaging of the retinal vessels in RPE65-associated retintis pigmentosa patients is challenging. A vast atrophy dominated all gained AO images. In this study, there were no considerable changes in retinal arterial architecture after treatment with voretigene neparvovec. WLR was constant throughout the observation period. LD and WCSA changed significantly after 2 weeks from the baseline examination. In the follow-up examinations over the period of 12 months LD and WCSA remained stable. There were no signs of inflammation such as macrophages or accumulated fluid visible.

Conclusions : There was no observable inflammatory change of the retinal arterial vasculature over the observation period of 12 months. AO can be used as a diagnostic module to monitor the disease and effects of genetic treatments.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.

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