Ultra-Widefield Imaging in Patients with Angioid Streaks Secondary to Pseudoxanthoma Elasticum

Purpose To investigate the application of noninvasive ultra-widefield (UWF) imaging in patients with angioid streaks secondary to pseudoxanthoma elasticum (PXE) and to compare detected findings with those obtainable with 7 standard 30° fields (7SF) imaging. Design Cross-sectional, observational study. Participants Forty eyes of 20 consecutive patients with angioid streaks secondary to PXE (8 women and 12 men; mean age, 56.9 ± 12.3 years). Methods Consecutive patients with angioid streaks secondary to PXE seeking treatment between January and June 2016 at the Medical Retina & Imaging Unit of the Department of Ophthalmology, University Vita-Salute San Raffaele, underwent UWF imaging (California; Optos PLC, Dunfermline, UK). Ultra-widefield color images and fundus autofluorescence (FAF) were evaluated. Ultra-widefield findings then were compared with those obtainable with 7SF. Main Outcome Measures Types and location of retinal lesions secondary to PXE. Results Peripheral lesions not entirely visible with 7SF were identified in 29 of 40 eyes (72.5%; P < 0.0001). These peripheral lesions included peau d'orange (52.5% of the eyes), coquille d'oeuf (52.5%), cracked eggshell (5.0%), comet lesions (27.5%), peripheral retinal degenerations (17.5%), parastreak atrophies (10.0%), and peripheral hemorrhage (5.0%). Furthermore, chorioretinal atrophies, drusen of the optic disc, cracked eggshell, pattern-like dystrophies, and retinal hemorrhages associated with angioid streaks were observed on digital color or FAF images, or both, and described. Conclusions Ultra-widefield imaging showed valuable usefulness in patients with angioid streaks by providing in a single image the entire spectrum of retinal alterations associated with PXE. Peripheral lesions often are present in patients with angioid streaks and may be missed with 7SF imaging. A careful examination of fundus periphery should be performed during screening and follow-up visits.