Widefield Swept-Source Optical Coherence Tomography Angiography Findings in Wagner Syndrome.

Abstract

PURPOSE:

To describe novel clinical and angiographic findings in Wagner syndrome.

METHODS:

A retrospective case series of three related patients with Wagner syndrome. Patients underwent standard OCT, B-scan ultrasonography, and fluorescein angiography in addition to wide field swept-source OCT angiography (WF SS-OCTA) (PLEX® Elite 9000, Carl Zeiss Meditec Inc.). Patients underwent genetic testing for a panel of hereditary vitreoretinopathies.

RESULTS:

Three related patients with Wagner syndrome were identified. All were found to have prominent vitreous strands, abnormal vitreoretinal adhesions, peripheral retinal holes, and varying degrees of myopia. A mid-peripheral tractional ridge was identified in all 6 eyes. All patients were positive for a known pathologic intron variant in the VCAN gene (4004-5T-A). WF SS-OCTA (12 mm x 12 mm) was performed in two patients and demonstrated perivascular capillary loss in the superficial capillary plexus along the arcades bilaterally. One patient demonstrated associated retinal atrophy within the area of capillary loss. The capillary loss extended beyond the margin of retinal atrophy.

CONCLUSION:

The unusual finding of a mid-peripheral tractional ridge of the retina associated with myopia led to a genetic diagnosis of Wagner Syndrome. WF SS-OCTA demonstrated a novel feature of perivascular loss of the superficial retinal capillary plexus. This result suggests that vitreous traction may cause localized microvasculature dysfunction and subsequent retinal atrophy in Wagner syndrome. This is the first known evaluation of Wagner syndrome utilizing OCTA.

• Authors: Isaac D Bleicher, Itika Garg, Sandra Hoyek, Emily Place, John B Miller, Nimesh A Patel
• Journal: Retinal cases & brief reports (Retin Cases Brief Rep) (Aug 11 2022) ISSN: 1937-1578 [Electronic] United States
• PMID: 36007184 (Publication Type: Journal Article)