July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
In vivo vessel volume quantification in Leber's hereditary optic neuropathy (LHON) during the disease conversion and progression
Author Affiliations & Notes
  • Neringa Jurkute
    NIHR Biomedical Research Centre at Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
  • Adnan Tufail
    Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
    Institute of Ophthalmology, University College London, London, United Kingdom
  • Pearse Andrew Keane
    Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
    Institute of Ophthalmology, University College London, London, United Kingdom
  • Andrew Webster
    Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
    Institute of Ophthalmology, University College London, London, United Kingdom
  • Patrick Yu-Wai-Man
    NIHR Biomedical Research Centre at Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
    Department of Clinical Neurosciences, University of Cambridge, Cambridge, United Kingdom
  • Peter Maloca
    Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
    OCTlab, University of Basel, Basel, Switzerland
  • Footnotes
    Commercial Relationships   Neringa Jurkute, None; Adnan Tufail, Heidelberg Engineering (C), Optovue (C); Pearse Keane, Allergan (S), Bayer (S), Bayer (R), DeepMind (C), Haag Streit (S), Heidelberg Engineering (S), Novartis (S), Optos (C), Topcon (S), Zeiss (S); Andrew Webster, None; Patrick Yu-Wai-Man, None; Peter Maloca, Bayer (C), Heidelberg Engineering (C), MIMO AG (C), Novartis (S), Optos (S), Roche (C), Topcon (C), Zeiss Forum (C)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 3345. doi:
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      Neringa Jurkute, Adnan Tufail, Pearse Andrew Keane, Andrew Webster, Patrick Yu-Wai-Man, Peter Maloca; In vivo vessel volume quantification in Leber's hereditary optic neuropathy (LHON) during the disease conversion and progression. Invest. Ophthalmol. Vis. Sci. 2018;59(9):3345.

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

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Abstract

Purpose : Vascular changes are seen to occur in LHON. These include telangiectactic microangiopathy in the acute phase and extensive papillomacular capillary drop out in the later phase, suggesting a vascular component in the pathophysiology of this disorder. Here we analyse vascular changes using a novel imaging technique of the optic disc and peripapillary area in a patient with LHON.

Methods : A novel imaging technique characterised by three-dimensional volume rendering of optical coherence tomography angiography (3D OCTA) was used to analyse changes in the optic disc and peripapillary area during the progression and conversion of the disease. Optical coherence tomography (Cirrus™ HD-OCT Model 5000 with AngioPlex, Carl ZeissMeditec, Inc., Dublin,USA) volume scans were obtained from the optic nerve head of a 26-year-old female with acute LHON. Consequently, three-dimensional structural OCT and OCTA were analysed, enabling segmentation and spatial characterization of vascular changes seen in different stages of the disease. Clinical findings were collected during each visit.

Results : The first set of OCTA images were obtained after 106 days of rapid central vision loss in the right eye and 49 days in the left eye followed by images recorded approximately 1.5 and 4.5 months later. At the first visit we measured vessel volume at 343,49 x 106 µm3 and area at 23.21 x 106 µm2 in the right eye; the decline in vessel measurements was noticed during the follow up visits and counted at 206,14 x 106 µm3 and 13,31 x 106 µm2 respectively during the last visit (246 days after onset). The patient presented with a shorter disease duration in the left eye (49 days after onset) and vessel volume was 406,15 x 106 µm3 and area was 28,45 x 106 µm2 at the first visit, followed by 421,79 x 106 µm3 and 28,75 x 106 µm2 during the second and 187,84 x 106 µm3 and 13,67 x 106 µm2 the last follow up visits.

Conclusions : This is the first description of 3D OCTA visualization and quantification of vascular changes in LHON patient during the disease progression. This method allows for in vivo imaging and provides crucial information and new insight on the spatio-anatomical features to better understand of the vascular component of the disease.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

 

3D OCTA follow up images in a patient with LHON. Right eye is on the left side of the image, left eye on the right.

3D OCTA follow up images in a patient with LHON. Right eye is on the left side of the image, left eye on the right.

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