Peripapillary comet lesions and comet rain in PXE-related retinopathy
- 74 Downloads
To study peripapillary comet lesions (PCL) in Italian patients affected with pseudoxanthoma elasticum (PXE).
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.
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.
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.
KeywordsPXE Comet lesion Comet rain Peripapillary comet lesion OCT ORT Outer retinal tubulation
Authors gratefully acknowledge the collaborative support of PXE Italia Onlus and the technical contribution of Sonia Costa.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (name of institute/committee) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
For this type of study, formal consent is not required.
- 15.Litts KM, Ach T, Hammack KM et al (2016) Quantitative analysis of outer retinal tubulation in age-related macular degeneration from spectral-domain optical coherence tomography and histology. Invest Ophthalmol Vis Sci 1;57(6):2647–56Google Scholar
- 17.Wolff B, Matet A, Vasseur V et al (2012) En face OCT imaging for the diagnosis of outer retinal tubulations in age-related macular degeneration J Ophthalmol 542417Google Scholar
- 18.Preti RC, Govetto A, Filho RGA et al (2017) Optical coherence tomography analysis of outer retinal tubulations: sequential evolution and pathophysiological insights. Retina. https://doi.org/10.1097/IAE.0000000000001810
- 27.Xue K, Oldani M, Jolly JK et al (2016) Correlation of optical coherence tomography and autofluorescence in the outer retina and choroid of patients with choroideremia. Invest Ophthalmol Vis Sci 1;57(8):3674–84Google Scholar