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

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Abstract

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.

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Funding

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Author notes

  1. María García-Fernandez and Jose Manuel García-Campos have contributed equally to this work.

Authors and Affiliations

  1. Department of Ophthalmology, University Hospital Virgen de la Victoria, Malaga, Spain

    Tania Hidalgo-Díaz

  2. Department of Ophthalmology, University Hospital Virgen Macarena, Seville, Spain

    María José Morillo-Sánchez

  3. Eye Clinic, Torbay Hospital, South Devon Healthcare NHS Foundation Trust, Torquay, UK

    Radua Kamal-Salah

  4. Department of Biostatistics, Málaga University Medical School, Malaga, Spain

    Francisca Rius-Díaz

  5. Department of Human Physiology, Málaga University Medical School, Malaga, Spain

    María García-Fernandez

  6. Department of Ophthalmology, Málaga University Medical School, Malaga, Spain

    Jose Manuel García-Campos

Authors
  1. Tania Hidalgo-Díaz
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  2. María José Morillo-Sánchez
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  3. Radua Kamal-Salah
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  4. Francisca Rius-Díaz
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Correspondence to Radua Kamal-Salah.

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Hidalgo-Díaz, T., Morillo-Sánchez, M.J., Kamal-Salah, R. et al. Macular choroidal thickness in patients with pseudoxanthoma elasticum measured by enhanced-depth imaging spectral-domain optical coherence tomography. Int Ophthalmol 40, 1749–1758 (2020). https://doi.org/10.1007/s10792-020-01343-2

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  • DOI: https://doi.org/10.1007/s10792-020-01343-2

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