Choroidal measurements in patients affected by PXE-related retinopathy

Dario Pasquale Mucciolo^1*Vittoria Murro¹Dario Giorgio²Federica Boraldi³Laura Pavese⁴Daniela Quaglino³Andrea Sodi¹Marco Branchetti¹Liliana Pollazzi¹Gianni Virgili¹Fabrizio Giansanti¹

• ¹Universita degli Studi di Firenze, Florence, Italy
• ²Azienda USL Toscana centro, Florence, Italy
• ³Universita degli Studi di Modena e Reggio Emilia, Modena, Italy
• ⁴Azienda Ospedaliero-Universitaria di Modena, Modena, Italy

Purpose: To investigate the choroid in patients affected by Pseudoxanthoma Elasticum (PXE)-related retinopathy using the Choroidal Vascularity Index (CVI). Methods: PXE patients and controls were recruited at the Eye Clinic in Florence. High-resolution imaging OCT scans (12x9 mm) of 32 PXE patients and 20 age-matched controls were examined. Images were binarized using ImageJ software and Subfoveal Choroidal Thickness (SFCT), Luminal Area (LA), Stromal Area (SA), Total Choroidal Area (TCA) and CVI were measured. Results: Sixty-four eyes of 32 PXE patients (mean age 45.65 ± 16.12; range 14-69) and 40 eyes of 20 controls (mean age 47.3 ± 13.7; range 18-71) were included in the study. SFCT was significantly lower in PXE patients compared to controls. LA, SA and TCA of the PXE patients were significantly reduced in comparison with those obtained for controls (p = 0.012; p < 0.001; and p = 0.001, respectively). On the contrary, the CVI did not significantly differ between patients and controls (p=0.744). In young subjects, We did not detect differences regarding SFCT, LA, SA, TCA and CVI between PXE patients and healthy controls (p=0.170; p= 0.990; p=0.264; p=0.351; p=0.487, respectively). Conclusion: In PXE-related retinopathy the choroidal impairment appears progressive with age, and there is a simultaneous, proportional impairment of both the stromal and the vascular components of the choroid.