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Altered ellipsoid zone reflectivity and deep capillary plexus rarefaction correlate with progression in Best disease
  1. Francesco Romano1,2,
  2. Alessandro Arrigo3,
  3. Pier Pasquale Leone4,
  4. Andrea Saladino1,
  5. Francesco Bandello1,
  6. Maurizio Battaglia Parodi1
  1. 1 Department of Ophthalmology, University Vita Salute Hospital San Raffaele, Milano, Italy
  2. 2 Eye Clinic, Department of Biomedical Science, Luigi Sacco University Hospital, Milan, Italy
  3. 3 Department of Ophthalmology, IRCCS Ospedale San Raffaele, University Vita-Salute, Milano, Italy
  4. 4 University Vita Salute, IRCCS Ospedale San Raffaele, Milano, Italy
  1. Correspondence to Dr Francesco Romano, Department of Ophthalmology, University Vita Salute Hospital San Raffaele, Milano 20132, Italy; f.romano{at}


Aims To evaluate the effects of neurovascular damage in patients with the typical vitelliform lesion of Best vitelliform macular dystrophy (BVMD) in the attempt to identify different progression patterns.

Methods Prospective, observational case series. Patients in the vitelliform stage of BVMD and healthy controls underwent complete ophthalmological examination on a yearly basis, including best-corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT) and OCT angiography (OCT-A). 4.5×4.5 mm OCT-A slabs were imported into ImageJ software and their vessel density (VD) was calculated. Similarly, the ellipsoid zone (EZ) was manually outlined and the reflectivity was measured above the vitelliform lesion and in the 500 µm external to it. Retinal pigment epithelium–Bruch’s membrane complex was taken as internal reference.

Results 34 eyes (24 patients) and 34 matched controls were included in the study. Mean follow-up was of 28.4±5.8 months, with 12 eyes showing signs of stage progression at the end follow-up. The EZ overlying the vitelliform lesion and in the peri-lesional area disclosed a significant reduction in reflectivity when compared with the foveal and para-foveal EZ of controls, respectively. VD resulted meaningfully decreased only at the deep capillary plexus. Of notice, more extensive EZ (reflectivity <0.7) and vascular alterations (VD <0.4) at baseline strongly correlated with worse BCVA and were associated with a more rapid progression at follow-up.

Conclusions Both EZ reflectivity and VD at deep capillary plexus may prove valuable biomarkers to assess BVMD severity and detect progression. In this view, ‘rapid progressors’ might benefit the most from timely genetic therapies in the future.

  • Best disease
  • progression biomarkers
  • OCT-A
  • neurovascular damage
  • ellipsoid zone
  • vessel density

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  • Contributors FR, AA, PPL, AS, FB and MBP have equally contributed to the planning of the study, analysis of the data, drafting and 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 None declared.

  • Patient consent for publication Written informed consent was obtained from both patients and controls included in the study.

  • Ethics approval The study was designed as a prospective and observational case series. The protocol was approved by the Institutional Review Board of Ospedale San Raffaele (Milan, Italy).

  • Provenance and peer review Not commissioned; externally 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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