Ultra-Widefield Imaging in Patients with Angioid Streaks Secondary to Pseudoxanthoma Elasticum.
Objective: 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.
Methods: Cross-sectional, observational study. Methods: 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. Methods: 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.