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Open AccessArticle

Cone Dystrophies: An Optical Coherence Tomography Angiography Study

Ophthalmology Clinic, Department of Medicine and Science of Ageing, University G. D’Annunzio Chieti-Pescara, 66100 Chieti, Italy
Scientific Institute San Raffaele, University Vita-Salute, IRCCS, 20131 Milano, Italy
Department of Medical, Oral and Biotechnological Sciences, Laboratory of Biostatistics, University “G. d’Annunzio” Chieti-Pescara, 66100 Chieti, Italy
Institute of Ophthalmology, University of Modena and Reggio Emilia, 41121 Modena, Italy
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(5), 1500;
Received: 9 April 2020 / Revised: 11 May 2020 / Accepted: 12 May 2020 / Published: 16 May 2020
Background: This study investigates the relationship between retinal vascularization and macular function in patients with cone dystrophies (CDs). Methods: Twenty CD patients (40 eyes) and 20 healthy controls (20 eyes) were enrolled in this prospective case-control study. Patients underwent full ophthalmological examination, microperimetry, full-field, pattern and multifocal electroretinogram (ERG, PERG, mfERG) and optical coherence tomography angiography (OCTA). Main outcome measures were as follows: foveal and parafoveal inner and outer retinal thickness; microperimetry sensitivity in the central 4° and 8°, ERG b wave amplitudes and peak times, PERG P50 and N95 amplitudes and latencies, and mfERG N1 to P1 amplitudes; and superficial capillary plexus (SCP), deep capillary plexus (DCP) and choriocapillary (CC) plexus vessel densities, divided into foveal and parafoveal region. Results: Retinal thickness, SCP and DCP densities were significantly related to PERG. A significant relationship was found between foveal and parafoveal retinal thicknesses and foveal SCP density (p < 0.001 and p = 0.018, respectively) and between parafoveal retinal thickness and parafoveal SCP density (p = 0.002). Foveal and parafoveal retinal thicknesses were significantly related to parafoveal DCP density (p = 0.007 and p < 0.001). Foveal and parafoveal retinal thicknesses, foveal SCP and parafoveal DPC densities were significantly reduced in CD patients compared to controls (p < 0.001; p = 0.010 and p = 0.008, respectively). PERG and mfERG amplitudes were significantly reduced in CD patients compared to controls (p < 0.01). Conclusions: CD eyes showed reduced retinal thickness significantly related to reduced vessel density, possibly caused by a decreased metabolic demand. In addition, vessel density significantly correlated with loss of function. View Full-Text
Keywords: cone dystrophies; optical coherence tomography angiography; retinal vessel density cone dystrophies; optical coherence tomography angiography; retinal vessel density
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Toto, L.; Battaglia Parodi, M.; D’Aloisio, R.; Mercuri, S.; Senatore, A.; Di Antonio, L.; Di Marzio, G.; Di Nicola, M.; Mastropasqua, R. Cone Dystrophies: An Optical Coherence Tomography Angiography Study. J. Clin. Med. 2020, 9, 1500.

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