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Multimodal evaluation of central and peripheral alterations in Stargardt disease: a pilot study
  1. Alessandro Arrigo1,
  2. Alessio Grazioli1,
  3. Francesco Romano2,
  4. Emanuela Aragona1,
  5. Alessandro Marchese1,
  6. Alessandro Bordato1,
  7. Carlo Di Nunzio1,
  8. Andrea Sperti1,
  9. Francesco Bandello1,
  10. Maurizio Battaglia Parodi1
  1. 1Department of Ophthalmology, IRCCS San Raffaele Hospital, University Vita-Salute, Milan, Italy
  2. 2Eye Clinic, Department of Biomedical and Clinical Science, Luigi Sacco University Hospital, Milan, Italy
  1. Correspondence to Dr Alessandro Arrigo, Department of Ophthalmology, IRCCS Ospedale San Raffaele, University Vita-Salute, Milano 20132, Italy; alessandro.arrigo{at}hotmail.com

Abstract

Background The clinical phenotype of Stargardt disease (STGD) can be extremely heterogeneous, with variable macular and peripheral retinal involvement. The study aim was to correlate peripheral ultrawide field (UWF) involvement with macular alterations, as assessed by structural optical coherence tomography (OCT) and OCT angiography (OCTA), in order to identify potentially different phenotypes.

Methods The study involved patients with STGD and healthy controls. We performed a complete ophthalmologic assessment and multimodal imaging, including OCT, OCTA, fundus autofluorescence and UWF imaging. Patients with STGD were subdivided according to the peripheral involvement. OCT and OCTA quantitative parameters were analysed. The main outcome of the study was the classification of UWF subtypes and the correlation between UWF subtypes and macular involvement.

Results Seventy STGD eyes (19 male; mean age 41.3±13.2 years) and 70 healthy eyes (35 male; 50%; mean age 41.2±9.8 years) were included in the analyses. Mean best-corrected visual acuity was 0.60±0.45 LogMAR for the STGD group and 0.0±0.0 LogMAR for controls (p<0.01). All clinical and imaging findings proved to be statistically worse in patients with STGD than in the control subjects (p<0.01). UWF types were distributed as follows: type I (49%), type II (34%), type III (17%). Type III patients proved to be significantly worse in terms of visual function and OCT and OCTA imaging parameters.

Conclusions The UWF autofluorescence performed in the present study suggests that there exist three different STGD phenotypes. Each phenotype is associated with variable OCT and OCTA impairment. Further studies providing a better assessment of the peripheral retinal involvement in STGD are warranted.

  • retina
  • imaging
  • anatomy
  • dystrophy
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Footnotes

  • Contributors AA, AG and MBP were responsible for the study design, study conduction and manuscript preparation. Data acquisition and analysis were performed by FR, AM, AB, CdN and AS. EA and FB did the data interpretation and critical revision of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests Francesco Bandello consultant for Allergan (Irvine, California, USA), Bayer Shering-Pharma (Berlin, Germany), Hoffmann-La-Roche (Basel, Switzerland), NTC Pharma, Novartis (Basel, Switzerland), SIFI, SOOFT, Thrombogenics (Heverlee, Belgium), Zeiss (Dublin, USA).

  • Patient consent for publication Not required.

  • Ethics approval The study was conducted in accordance with the Declaration of Helsinki and was approved by the Ethical Committee of the Vita-Salute San Raffaele University in Milan.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.

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