Thinner temporal peripapillary retinal nerve fibre layer in Stargardt disease detected by optical coherence tomography
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RETINAL DISORDERS
Thinner temporal peripapillary retinal nerve fibre layer in Stargardt disease detected by optical coherence tomography Michael Reich 1 & Jan Lübke 1 & Lutz Joachimsen 1 & Julia Stifter 1 & Sebastian Küchlin 1 & Daniel Böhringer 1 & Clemens Lange 1 & Wolf A. Lagrèze 1 Received: 6 February 2020 / Revised: 12 October 2020 / Accepted: 23 October 2020 # The Author(s) 2020
Abstract Purpose To evaluate peripapillary retinal nerve fibre layer (RNFL) thickness measured by spectral domain optical coherence tomography (OCT) in patients with Stargardt disease (STGD). Methods A cross-sectional, monocentric, observational case-control study. Twenty patients (39 eyes) with ABCA4 mutations graded according to the Fishman STGD classification were included. RNFL measurement was performed using Heidelberg Spectralis SD-OCT. RNFL thickness in STGD patients was compared to age-matched data of healthy individuals provided by the device’s manufacturer. A manual readjustment of the optic disc-fovea angle was performed when needed. Results The mean age at first diagnosis of STGD was 22.9 years (range 9 to 50) and 39.1 years (range 18 to 74) at the time of examination. Thirty-nine percent of eyes (15 eyes) needed manual adjustment of the optic disc-fovea angle due to malfixation of the patients during OCT. The temporal quadrant corresponding to the macula showed a RNFL 16% thinner than controls (mean − 12 μm, 95%CI − 9 to −15 μm). However, global RNFL thickness did not differ from controls due to increased RNFL thickness of 12% in the nasal sectors. Duration and stage of STGD were not correlated to thinner RNFL. Conclusion STGD seems to be associated with thinner peripapillary RNFL in the sector of axons projecting to the degenerated macular area. It is yet unclear as to whether this results from anterograde transneuronal degeneration of direct injury to retinal ganglion cells.
KEY MESSAGES -
Peripapillary retinal nerve fibre layer changes have been described in different hereditary retinal dystrophies.
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Stargardt disease is accompanied by a thinner temporal and a thicker nasal peripapillary retinal nerve fibre layer.
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The influence of macular comorbidities should be taken into account when interpreting retinal nerve fibre layer measurements.
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Before interpreting the peripapillary retinal nerve fibre layer, the correct detection of the fovea must be assured in case of maculopathies and, if necessary, the angle between the optic discand fovea must be corrected.
Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s00417-02004992-2. * Wolf A. Lagrèze [email protected] 1
Eye Center, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Killianstrasse 5, D-79106 Freiburg, Germany
Graefes Arch Clin Exp Ophthalmol
Keywords Anterograde transneuronal degeneration . ABCA4 . Stargardt disease . Retinal nerve fibre layer . Optic disc
Introduction Axonal loss in the peripapillary retinal nerve fibre layer (R
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