Macular choroidal thickness in patients with pseudoxanthoma elasticum measured by enhanced-depth imaging spectral-domain optical coherence tomography

• Published in: International ophthalmology Vol. 40; no. 7; pp. 1749 - 1758
• Main Authors: Hidalgo-Díaz, Tania, Morillo-Sánchez, María José, Kamal-Salah, Radua, Rius-Díaz, Francisca, García-Fernandez, María, García-Campos, Jose Manuel
• Format: Journal Article
• Language: English
• Published: Dordrecht Springer Netherlands 01.07.2020; Springer Nature B.V
• Subjects: Atrophy; Choroid; Computed tomography; Cross-Sectional Studies; Domains; Humans; Medical imaging; Medicine; Medicine & Public Health; Ophthalmology; Optical Coherence Tomography; Original Paper; Oxidative stress; Prospective Studies; Pseudoxanthoma Elasticum - complications; Pseudoxanthoma Elasticum - diagnosis; Quadrants; Subgroups; Thickness; Tomography; Tomography, Optical Coherence; Vascularization; Optical coherence tomography; Pseudoxanthoma elasticum; Macular choroidal thickness
• ISSN: 0165-5701; 1573-2630; 1573-2630
• DOI: 10.1007/s10792-020-01343-2

Background/objectives To analyze macular choroidal thickness in patients with pseudoxanthoma elasticum (PXE) by enhanced depth imaging optical coherence tomography (EDI-OCT). Subjects/methods This is a prospective cross-sectional study. Sixty-eight eyes of 34 patients with PXE and 68 normal eyes of 34 controls were included to study the macular area with enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT). Eyes with PXE were classified in three groups: those without choroidal neovascularization (CNV) or chorioretinal macular atrophy macular (Group 1); those with active CNV (Group 2) and those with macular atrophy secondary to inactive CNV (Group 3). Results Mean subfoveal choroidal thickness (CT) was 266.70 ± 46.93 µm in control group, 304.24 ± 65.52 µm in group 1, 198.55 ± 66.33 µm in group 2, and 119.45 ± 63.89 µm in group 3 ( p  = 0.00). Comparison between PXE subgroups showed that subfoveal CT was significantly decreased in group 2 and 3 compared to group 1 ( p  < 0.0001 for both groups). The CT in the different quadrants (superior, inferior, temporal and nasal) was significantly thinner in group 3, followed by group 2 and 1 in ascendant order. Group 1 showed significant increased thickness compared to the other groups. Conclusion To the best of our knowledge, this is the first report suggesting thicker macular choroid in patients with PXE without active or inactive CNV than in normal eyes. Initial changes in Bruch membrane (MB) and choroid, in addition to the increased oxidative stress, would lead to hyperpermeability of the choroid and alterations of the barrier BM-RPE causing a thick choroid in early stages.