July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Rapid non-mydriatic assessment of panretinal cone system function using a hand-held electroretinography device in ABCA4 retinopathy
Author Affiliations & Notes
  • diana-maria butu
    Moorfields Eye Hospital, London, London, United Kingdom
    Royal Free Hospital, London, London, United Kingdom
  • Ana Fakin
    Moorfields Eye Hospital, London, London, United Kingdom
  • Talha Soorma
    Moorfields Eye Hospital, London, London, United Kingdom
    Department of Ophthalmology, King's College London, United Kingdom
  • April Q Neville
    Moorfields Eye Hospital, London, London, United Kingdom
  • Christopher J Hammond
    Department of Ophthalmology, King's College London, United Kingdom
  • Michel Michaelides
    Moorfields Eye Hospital, London, London, United Kingdom
    UCL Institute of Ophthalmolgy, University College London, United Kingdom
  • Andrew Webster
    Moorfields Eye Hospital, London, London, United Kingdom
    UCL Institute of Ophthalmolgy, University College London, United Kingdom
  • Omar Abdul Rahman Mahroo
    Moorfields Eye Hospital, London, London, United Kingdom
    UCL Institute of Ophthalmolgy, University College London, United Kingdom
  • Footnotes
    Commercial Relationships   diana-maria butu, None; Ana Fakin, None; Talha Soorma, None; April Q Neville, None; Christopher Hammond, None; Michel Michaelides, None; Andrew Webster, None; Omar Mahroo, None
  • Footnotes
    Support  Wellcome Trust Grant 206619/Z/17/Z; NIHR Biomedical Research Centre at Moorfields Eye Hospital and the UCL Institute of Ophthalmology
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 5952. doi:
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      diana-maria butu, Ana Fakin, Talha Soorma, April Q Neville, Christopher J Hammond, Michel Michaelides, Andrew Webster, Omar Abdul Rahman Mahroo; Rapid non-mydriatic assessment of panretinal cone system function using a hand-held electroretinography device in ABCA4 retinopathy. Invest. Ophthalmol. Vis. Sci. 2019;60(9):5952.

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

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Abstract

Purpose : Patients with ABCA4 retinopathy (Stargardt disease) can be grouped by electrophysiology, with prognostic implications: those with a normal photopic full-field electroretinogram (ERG) are less likely to suffer deterioration in generalised retinal function over subsequent years. We used a portable device to obtain photopic ERGs, investigating tolerability, correlation with ultra-widefield autofluorescence (AF) and with prior conventional ERG testing.

Methods : Adults with ABCA4 retinopathy underwent photopic recordings with the portable device (RETeval, LKC Technologies Inc., Gaithersburg, MD, USA) using skin electrodes. Pupils were undilated; the device adjusts stimulus strength according to pupil diameter to deliver a retinal illuminance equivalent to international standards. Right eye recordings were analysed. ERG parameters were compared with control recordings from >500 healthy participants from the TwinsUK cohort. Amplitudes <5th centile, and peak times >95th centile were deemed abnormal. Patients underwent ultra-widefield AF (Optos plc, Dunfermline, UK) imaging the same day; images were grouped by presence of far peripheral involvement. The majority of patients had prior conventional ERG testing (in some cases several years previously).

Results : 65 patients were included (mean age 46.4 years; 33 females). Recordings were well-tolerated, lasting <5 min. 40 patients (60%) had peripheral abnormal AF; the proportion of patients with abnormal ERG parameters was higher in these patients (95% vs 23%, p<0.01), and mean ERG b-wave and flicker amplitudes were lower, and peak times more delayed, compared with those without abnormal peripheral AF (p<0.05). Of 32 patients with previously abnormal conventional ERGs, 31 (97%) had abnormal parameters with the hand-held device. Of 24 patients with previous normal ERGs, 18 (75%) had normal hand-held device ERGs.

Conclusions : Recordings were well-tolerated and complete within minutes. The majority (95%) of patients with abnormal far peripheral AF had abnormal ERGs, demonstrating a strong structure-function correlation. Portable ERG findings broadly agreed with prior conventional testing, although the duration between the two tests is likely to mean that in some patients disease progression occurred in the intervening period.

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

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