NEGATIVE VESSEL REMODELING IN STARGARDT DISEASE QUANTIFIED WITH VOLUME-RENDERED OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

To quantify retinal vasculature changes in Stargardt disease1 (STGD1) with volume-rendered optical coherence tomography angiography. Optical coherence tomography angiography volumes from healthy subjects and two subgroups of patients with STGD1 with the presence/absence of definitely decreased autofluorescence areas were compared. Optical coherence tomography angiography vessel surface area and vessel volume were measured in central zones (Z) of 1-, 2-, and 3-mm diameter. Twenty nine eyes of 15 patients with STGD1 (20/9 eyes with/without definitely decreased autofluorescence) and 30 eyes of 15 controls contributed data. An enlarged foveal avascular zone was found in patients with STGD1 without and even more with definitely decreased autofluorescence associated with a vessel rarefication in central and also paracentral zones with unnoticeable autofluorescence. Vessel surface area and vessel volume were reduced in both STGD1 subgroups for all zones (P < 0.0001). Stargardt disease 1 eyes when compared to without definitely decreased autofluorescence showed reduced vessel surface area and vessel volume in Z2+3 (both P < 0.05). Volume rendering of optical coherence tomography angiography in STGD1 shows a reduced retinal flow in the central macula. This is most likely secondary to loss of neurosensory tissue with disease progression and therefore not likely be favorably influenced by gene transfer and retinal pigment epithelial transplantation. Retinal blood flow assessed by 3D volume-rendered optical coherence tomography angiography could serve as surrogate marker for vascular changes of the central retina.

Déclaration de conflit d'intérêts

H. P. N. Scholl is a member of the Scientific Advisory Board of: Astellas Institute for Regenerative Medicine; GenSight Biologics; Ionis Pharmaceuticals, Inc.; Gyroscope Therapeutics Ltd.; Janssen Research & Development, LLC (Johnson & Johnson); and Pharma Research & Early Development (pRED) of F. Hoffmann-La Roche Ltd; Novartis Pharma AG (CORE). H. P. N. Scholl is paid consultant of: Boehringer Ingelheim Pharma GmbH & Co; Gerson Lehrman Group; and Guidepoint. H. P. N. Scholl is a member of the Data Monitoring and Safety Board/Committee of Belite Bio and ReNeuron Group Plc/Ora Inc. and a member of the Steering Committee of Novo Nordisk (FOCUS trial). H. P. N. Scholl is co-director of the Institute of Molecular and Clinical Ophthalmology Basel (IOB) that is constituted as a non-profit foundation and receives funding from the University of Basel, the University Hospital Basel, Novartis, and the government of Basel-Stadt. These arrangements have been reviewed and approved by the University of Basel (Universitätsspital Basel, USB) in accordance with its conflict of interest policies. H. P. N. Scholl is principal investigator of grants at the USB sponsored by the following entities: IVERIC bio (Ophthotech Corporation); Kinarus AG; and Novartis Pharma AG. Grants at USB are negotiated and administered by the institution (USB), which receives them on its proper accounts. Individual investigators who participate in the sponsored project(s) are not directly compensated by the sponsor but may receive support from the institution for their project(s). R. F. Spaide has received personal compensation from Topcon Medical Systems, Roche, Genentech, Bayer, Regeneron, Heidelberg Engineering, Adverum Biotechnologies, and DORC. P. M. Maloca is Group Leader Ophthalmic Imaging, Institute of Molecular and Clinical Ophthalmology (IOB), Basel, Switzerland, and is co-director, OCTlab, University Basel, Mittlere Strasse 91, CH-4056 Basel, Switzerland & Belegarzt Hirslanden St. Anna im Bahnhof, Luzern. Furthermore, he is a Honorary Research Fellow, Moorfields Eye Hospital, 162 City Rd, London EC1V 2PD, London, United Kingdom. P. M. Maloca is a consultant of Zeiss and Roche. The remaining authors have no conflicting interests to disclose.