Ultra-widefield imaging in patients with angioid streaks secondary to pseudoxanthoma elasticum

Abstract

Purpose : To investigate the retinal features on non-invasive ultra-widefield (UWF) imaging in patients with angioid streaks secondary to pseudoxanthoma elasticum (PXE).

Methods : Cross-sectional, observational study. Consecutive patients with angioid streaks secondary to PXE, presenting at the Medical Retina & Imaging Unit of the Department of Ophthalmology, University Vita-Salute San Raffaele, underwent UWF imaging (California, Optos PLC, Dunfermline, Scotland, UK). UWF color images and fundus autofluorescence (AF) were evaluated. Main outcome measures were types and location of retinal lesions secondary to PXE.

Results : Identified lesion included peau d’orange (72.3% of the eyes), coquille d’oeuf (72.3%), cracked eggshell (4.0%), comet lesions (29.8%), peripheral retinal degenerations (19.2%), parastreaks atrophies (15.0%), peripheral hemorrhage (5.0%), drusen of the optic disc (8.5%) and pattern-like dystrophies (9.2%)

Conclusions : Peripheral retinal lesions are often present in patients with PXE and their fundus periphery should be examined carefully. UWF color and fundus autofluorescence showed valuable utility in patients with PXE, by providing in a single image the entire spectrum of retinal alterations associated with this disease.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.