Pseudoxanthoma elasticum-associated angioid streaks near a scleral buckle

Karen E Lee; Tobin B T Thuma; Mirataollah Salabati; Meera D Sivalingam; Jose S Pulido; Kammi B Gunton

doi:10.1016/j.ajoc.2023.101970

Pseudoxanthoma elasticum-associated angioid streaks near a scleral buckle

Am J Ophthalmol Case Rep. 2023 Dec 7:34:101970. doi: 10.1016/j.ajoc.2023.101970. eCollection 2024 Jun.

Authors

Karen E Lee¹, Tobin B T Thuma¹, Mirataollah Salabati², Meera D Sivalingam², Jose S Pulido², Kammi B Gunton¹

Affiliations

¹ Department of Pediatric Ophthalmology and Strabismus, Wills Eye Hospital, Philadelphia, PA, USA.
² Retina Service, Wills Eye Hospital, Philadelphia, PA, USA.

Abstract

Purpose: We report a patient with pseudoxanthoma elasticum (PXE) with angioid streaks near a scleral buckle site.

Observations: A 46-year-old male with PXE presented for evaluation of blurry vision and was found to have classic PXE findings in both eyes and angioid streaks adjacent to the site of a scleral buckle in his left eye. He underwent multimodal imaging, genetic testing, and intravitreal aflibercept in the right eye.

Conclusions and importance: Bruch's membrane is known to be fragile in PXE, and patients are often counseled about the heightened risk of playing contact sports. This report raises the question of whether tension from a scleral buckle in the setting of a calcified and brittle BM may increase the likelihood of angioid streaks near the buckle site. In the setting of retinal detachment, it may be worthwhile to carefully weigh the pros and cons of vitrectomy versus buckle for PXE patients.

Keywords: Angioid streaks; Bruch's membrane; Pseudoxanthoma elasticum; Scleral buckle.

Publication types

Case Reports