Subclinical Angioid Streaks with Pseudodrusen: A New Phenotype of Age-Related Macular Degeneration

Riccardo Sacconi; Beatrice Tombolini; Ilaria Zucchiatti; Andrea Servillo; Matteo Menean; Giulio Falco Alessandrini; Lea Querques; Francesco Prascina; Peter Charbel Issa; Francesco Bandello; Giuseppe Querques

doi:10.1007/s40123-023-00778-x

Subclinical Angioid Streaks with Pseudodrusen: A New Phenotype of Age-Related Macular Degeneration

Ophthalmol Ther. 2023 Oct;12(5):2729-2743. doi: 10.1007/s40123-023-00778-x. Epub 2023 Aug 5.

Authors

Riccardo Sacconi^{1

2}, Beatrice Tombolini^{1

2}, Ilaria Zucchiatti^{1

2}, Andrea Servillo^{1

2}, Matteo Menean^{1

2}, Giulio Falco Alessandrini¹, Lea Querques², Francesco Prascina², Peter Charbel Issa^{3

4}, Francesco Bandello^{1

2}, Giuseppe Querques^{5

6

7}

Affiliations

¹ School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
² Division of Head and Neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
³ Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
⁴ Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
⁵ School of Medicine, Vita-Salute San Raffaele University, Milan, Italy. giuseppe.querques@hotmail.it.
⁶ Division of Head and Neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy. giuseppe.querques@hotmail.it.
⁷ Department of Ophthalmology, University Vita-Salute-IRCCS Ospedale San Raffaele, Via Olgettina 60, 20132, Milan, Italy. giuseppe.querques@hotmail.it.

Abstract

Introduction: To describe subclinical angioid streaks (AS) as a frequent, peculiar age-related macular degeneration (AMD) phenotype, comparing features of eyes with subclinical AS with those of eyes with AMD without AS.

Methods: This was a retrospective, observational study. Among a patient cohort with AMD, we selected patients without known causes for AS whose eyes showed signs of angioid streaks (AS) on structural optical coherence tomography (OCT) but not on fundus examination. Selected OCT features of AS were Bruch's membrane (BM) breaks and large BM dehiscences.

Results: Among 543 eyes of 274 patients with AMD (mean ± standard deviation: 82 ± 7 years), 73 eyes of 46 patients (81 ± 7 years; p = 0.432) showed AS features on OCT (OCT AS) that were not visible on fundus examination. Estimated prevalence of subclinical age-related AS was 13.4% (95% confidence interval 10.3-16.3%) in this AMD population. Fifty-three eyes (73%) with AS features were affected by peripapillary atrophy, often with a "petaloid-like" pattern, similar to typical features of AS disease. Almost all cases (97%) presented reticular pseudodrusen (RPD), with (41%) or without (59%) drusen showing a significant difference in RPD prevalence in OCT AS eyes in comparison to AMD eyes without subclinical AS using generalized estimating equations (P < 0.001). Among the 73 subclinical AS cases, 71 were affected by late AMD (57 with macular neovascularization, 14 with geographic atrophy), showing a more advanced AMD stage in comparison with AMD eyes without subclinical AS (P < 0.001). The following OCT features were disclosed: BM breaks in 100% of cases and BM dehiscences in 37%.

Conclusions: Subclinical AS in eyes with AMD is a peculiar phenotype of the disease, with features suggesting a primary involvement of Bruch's membrane and clinical similarities with mild, late-onset pseudoxanthoma elasticum.

Keywords: Age-related macular degeneration; Angioid streaks; Biomarker; Multimodal imaging; OCT; Reticular pseudodrusen; Subclinical angioid streaks.