Multifocal Vitelliform Paravascular Retinopathy (MVPR): A New Disorder in the Vitelliform Spectrum.

Scott D Walter, Weilin Song, H Richard McDonald, Mark W Johnson, Peter Charbel Issa, Yoshihiro Yonekawa, Anita Agarwal, Ehsan Rahimy, Sandeep Randhawa, Omar A Mahroo, SriniVas Sadda, K Bailey Freund, David Sarraf, Marcela Bohn, Kenneth J Taubenslag, Jacques Bijon

Journal: American journal of ophthalmology 2024;269():362-372

PMID: 39237051

Full Text on Elsevier Science View on PubMed

Abstract

PURPOSE

To describe a new retinal phenotype characterized by bilateral, multifocal, subretinal vitelliform lesions along the vascular arcades that we refer to as multifocal vitelliform paravascular retinopathy (MVPR).

DESIGN

Observational case series.

METHODS

Multimodal retinal imaging including color fundus photography, fundus autofluorescence and cross sectional and en-face optical coherence tomography was performed to evaluate and characterize the lesions of MVPR.

RESULTS

Thirteen asymptomatic patients aged 10 to 78 [mean 49 ± 24, 49% under 50] were evaluated for bilateral retinal lesions. Initial visual acuity was 20/30 or better in 22 (85%) eyes. Of the 20 eyes with follow-up, 14 (70%) exhibited visual acuity 20/30 or better at final follow-up. Multifocal small round yellow lesions with distinct borders were identified along the vascular arcades in all patients. The vitelliform lesions were brightly hyperautofluorescent and consisted of focal areas of subretinal hyperreflective material on optical coherence tomography (OCT) that in some cases evolved to hyporeflective spaces (or retinal pigment epithelium atrophy) with associated hypoautofluorescence. When performed, electroretinography (ERG) and electrooculography (EOG) testing were normal and genetic testing was negative for variants in BEST1 and other genes associated with vitelliform retinopathies.

CONCLUSIONS

MVPR may represent a novel entity of vitelliform disorders with a distinct clinical presentation and phenotype and generally favorable prognosis.

Published by Elsevier Inc.

Address: From the Stein Eye Institute (W.S., D.S.), University of California, Los Angeles, California, USA.; Associated Retinal Consultants (S.R.), Royal Oak, Michigan, USA.; Kellogg Eye Center (M.W.J.), University of Michigan, Ann Arbor, Michigan, USA.; Moorfields Eye Hospital NHS Foundation Trust (M.B.), London, UK; West Hertfordshire Teaching Hospital NHS Trust (M.B.), London, UK.; West Coast Retina Medical Group (A.A., H.R.M.), San Francisco, California, USA.; Byers Eye Institute at Stanford (E.R.), Palo Alto, California, USA.; VA Maryland Healthcare System (K.J.T.), Baltimore VA Medical Center, Baltimore, Maryland, USA; Department of Ophthalmology (K.J.T.), University of Maryland, Baltimore, Maryland, USA.; Oxford Eye Hospital (P.C.I.), Oxford University Hospitals NHS Foundation Trust, Oxford, UK; Nuffield Department of Clinical Neurosciences (P.C.I.), University of Oxford, Oxford, UK; Department of Ophthalmology (P.C.I.), Technical University Munich, Munich, Germany.; NIHR Biomedical Research Centre at Moorfields Eye Hospital and the UCL Institute of Ophthalmology (O.A.M.), London, UK; Department of Ophthalmology (O.A.M.), St Thomas' Hospital, London, UK.; Vitreous Retina Macular Consultants of New York (J.B., K.B.F.), New York, USA.; Retina Consultants P.C. (S.D.W.), Hartford, Connecticut, USA.; Wills Eye Hospital, Mid Atlantic Retina (Y.Y.), Thomas Jefferson University, Philadelphia, Pennsylvania, USA.; Doheny Eye Institute (S.V.D), Pasadena, California, USA; Department of Ophthalmology (S.V.D.), University of California, Los Angeles, Los Angeles, California, USA.; Vitreous Retina Macular Consultants of New York (J.B., K.B.F.), New York, USA; Department of Ophthalmology (K.B.F.), NYU Grossman School of Medicine, New York, New York, USA.; From the Stein Eye Institute (W.S., D.S.), University of California, Los Angeles, California, USA; Greater Los Angeles VA Healthcare Center (D.S.), Los Angeles, California, USA. Electronic address: [email protected].

DOI: 10.1016/j.ajo.2024.08.028

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MeSH Terms: Humans, Tomography, Optical Coherence, Female, Male, Fluorescein Angiography, Visual Acuity, Adolescent, Middle Aged, Adult, Aged, Electroretinography, Child, Vitelliform Macular Dystrophy, Young Adult, Multimodal Imaging, Retinal Pigment Epithelium