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Graefe's Archive for Clinical and Experimental Ophthalmology
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology 9/2018

01-09-2018 | Retinal Disorders

Peripapillary comet lesions and comet rain in PXE-related retinopathy

Authors: Vittoria Murro, Dario Pasquale Mucciolo, Andrea Sodi, Federica Boraldi, Daniela Quaglino, Gianni Virgili, Stanislao Rizzo

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 9/2018

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Abstract

Purpose

To study peripapillary comet lesions (PCL) in Italian patients affected with pseudoxanthoma elasticum (PXE).

Methods

Retrospective review of fundoscopic and swept-source (SS) optical coherence tomography (OCT) images of patients with PXE examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Careggi Teaching Hospital of Florence from 2012 to 2017.

Results

From 148 eyes of 74 patients affected with PXE, we identified 24 eyes of 14 patients (11 were female) with a mean age of 39 years (range, 20–58 years) characterized by peripapillary comet lesions. Of these 24 eyes, 15 eyes (of 10 patients) were characterized by comet rain. The smallest comet lesion at the OCT examination appeared as a focal roundish hyper-reflective alteration at the level of the outer retinal segments and RPE-Bruch’s membrane complex; the larger lesions appeared as circular and ovoid structures with hyper-reflective borders in the outer nuclear layer.

Conclusion

The comet lesion formation process involves the outer layers of the retina and RPE/Bruch’s membrane complex. It consists of a degenerative/rearrangement process of the photoreceptors which occurs in an area of focal altered RPE/Bruch’s membrane resembling the outer retinal tubulation.
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Metadata
Title
Peripapillary comet lesions and comet rain in PXE-related retinopathy
Authors
Vittoria Murro
Dario Pasquale Mucciolo
Andrea Sodi
Federica Boraldi
Daniela Quaglino
Gianni Virgili
Stanislao Rizzo
Publication date
01-09-2018
Publisher
Springer Berlin Heidelberg
Published in
Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 9/2018
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI
https://doi.org/10.1007/s00417-018-4037-2

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