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Optical coherence tomography angiography findings in pigmented paravenous chorioretinal atrophy

Battaglia Parodi, M; Arrigo, A; Chowers, I; Jarc-Vidmar, M; Shpigel, M; Bandello, F; Michaelidis, M; (2022) Optical coherence tomography angiography findings in pigmented paravenous chorioretinal atrophy. Retina , 42 (5) pp. 915-922. 10.1097/IAE.0000000000003407. Green open access

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Abstract

Purpose:To analyze the retino-choroidal vascular characteristics of patients affected by pigmented paravenous chorio-retinal atrophy by means of optical coherence tomography (OCT) angiography.Methods:This study was designed as an observational, cross-sectional case series. Multimodal imaging included fundus autofluorescence, structural OCT, and OCT angiography. The quantitative OCT angiography analyses included the calculation of the vessel density and choriocapillaris porosity.Results:Overall, 12 patients (24 eyes) affected by pigmented paravenous chorio-retinal atrophy were recruited. Structural OCT of the areas involved by pigmented paravenous chorio-retinal atrophy as visualized on the fundus autofluorescence showed a complete ellipsoid zone and external limiting membrane absence, with thinning of ganglion cell complex, outer nuclear layer, and outer plexiform layer, but associated with the optical partial preservation of the retinal pigment epithelium. Optical coherence tomography angiography quantitative assessment of the retinal regions affected by PPRCA, as visualized by fundus autofluorescence, was characterized by normal vessel density at the level of superficial capillary plexus but significantly altered vessel density of deep capillary plexus and choriocapillaris, with higher choriocapillaris porosity. The presence of macular atrophy was significantly correlated with worse deep capillary plexus and choriocapillaris vessel density values. Furthermore, a statistically significant correlation between the fundus autofluorescence patterns and the retinal vascular status was found.Conclusion:Optical coherence tomography angiography quantitative analyses in pigmented paravenous chorio-retinal atrophy demonstrate a specific impairment at the level of the deep capillary plexus, which could in turn bring about a thinning of ganglion cell complex and outer nuclear layer. The alterations at the level of the choriocapillaris and the choroid, in general, could then represent a secondary effect.

Type: Article
Title: Optical coherence tomography angiography findings in pigmented paravenous chorioretinal atrophy
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1097/IAE.0000000000003407
Publisher version: http://dx.doi.org/10.1097/IAE.0000000000003407
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Atrophy, Cross-Sectional Studies, Eye Diseases, Hereditary, Fluorescein Angiography, Humans, Retinal Degeneration, Retinal Pigments, Tomography, Optical Coherence
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
URI: https://discovery.ucl.ac.uk/id/eprint/10150902
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