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.

Peripapillary comet lesions and comet rain in PXE-related retinopathy / Murro, Vittoria; Mucciolo, Dario Pasquale; Sodi, Andrea; Boraldi, Federica; Quaglino, Daniela; Virgili, Gianni; Rizzo, Stanislao. - In: GRAEFE'S ARCHIVE FOR CLINICAL AND EXPERIMENTAL OPHTHALMOLOGY. - ISSN 0721-832X. - (2018), pp. 1605-1614. [10.1007/s00417-018-4037-2]

Peripapillary comet lesions and comet rain in PXE-related retinopathy

Boraldi, Federica;Quaglino, Daniela;
2018

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.
12-giu-2018
1605
1614
Peripapillary comet lesions and comet rain in PXE-related retinopathy / Murro, Vittoria; Mucciolo, Dario Pasquale; Sodi, Andrea; Boraldi, Federica; Quaglino, Daniela; Virgili, Gianni; Rizzo, Stanislao. - In: GRAEFE'S ARCHIVE FOR CLINICAL AND EXPERIMENTAL OPHTHALMOLOGY. - ISSN 0721-832X. - (2018), pp. 1605-1614. [10.1007/s00417-018-4037-2]
Murro, Vittoria; Mucciolo, Dario Pasquale; Sodi, Andrea; Boraldi, Federica; Quaglino, Daniela; Virgili, Gianni; Rizzo, Stanislao
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11380/1176081
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