Massive submacular hemorrhage in pseudoxanthoma elasticum

Abstract

A 58-year-old woman with a history of pseudoxanthoma elasticum (PXE) presented for ophthalmological examination at Hôpital Universitaire Pitié-Salpêtrière with acute vision loss in her right eye. On examination, best-corrected visual acuity was 20/63 in the right eye and 20/160 in the left eye because of extensive subretinal fibrosis (not shown). Fundus examination and optical coherence tomography revealed a submacular hemorrhage with an accompanying pigment epithelial detachment (A-B). Distinctive signs of ocular PXE were noted, including angioid streaks (white arrow), peau d’orange (asterisk), and macular atrophy (black arrow). The patient was treated by pars plana vitrectomy with subretinal injection of tissue plasminogen activator and sulfur hexafluoride gas tamponade. At 8 months’ follow-up, best-corrected visual acuity in her right eye remained limited at 20/63 due to subfoveal fibrovascular scarring (C-D).