# Choroidal measurements in patients affected by PXE-related retinopathy

## TL;DR

This study compares choroidal measurements in pseudoxanthoma elasticum (PXE) patients and controls, finding age-related choroidal impairment in PXE-related retinopathy.

## Contribution

The study reveals that choroidal changes in PXE-related retinopathy progress with age and affect both vascular and stromal components proportionally.

## Key findings

- PXE patients had significantly lower subfoveal choroidal thickness, luminal area, stromal area, and total choroidal area compared to controls.
- Choroidal vascularity index did not differ between PXE patients and controls.
- Younger PXE patients showed no significant differences in choroidal measurements compared to healthy controls.

## Abstract

To investigate the choroid in patients affected by pseudoxanthoma elasticum (PXE)-related retinopathy using the choroidal vascularity index (CVI).

PXE patients and controls were recruited at the Eye Clinic in Florence. High-resolution imaging optical coherence tomography (OCT) scans (12 × 9 mm) of 32 PXE patients and 20 age-matched controls were examined. Images were binarized using the ImageJ software, and subfoveal choroidal thickness (SFCT), luminal area (LA), stromal area (SA), total choroidal area (TCA), and CVI were measured.

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. The 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, differences regarding SFCT, LA, SA, TCA, and CVI were not detected between PXE patients and healthy controls (p = 0.170, p = 0.990, p = 0.264, p = 0.351, and p = 0.487, respectively).

In PXE-related retinopathy, choroidal impairment appears progressive with age, and there is a simultaneous, proportional impairment of both the stromal and vascular components of the choroid.