Enrico Borrelli1, Siva Balasubramanian2, Giacinto Triolo3, Piero Barboni4, SriniVas R Sadda2, Alfredo A Sadun2. 1. Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, California, USA; Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles, California, USA; Ophthalmology Clinic, Department of Medicine and Science of Ageing, University G. D'Annunzio Chieti-Pescara, Chieti, Italy. Electronic address: borrelli.enrico@yahoo.com. 2. Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, California, USA; Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles, California, USA. 3. Department of Ophthalmology, San Raffaele Scientific Institute, Milan, Italy. 4. Department of Ophthalmology, San Raffaele Scientific Institute, Milan, Italy; Studio Oculistico d'Azeglio, Bologna, Italy.
Abstract
PURPOSE: To study the macular microvascular networks in patients affected by chronic Leber hereditary optic neuropathy (LHON) using optical coherence tomography angiography (OCTA), and to quantify these changes in different macular sectors. DESIGN: Prospective cross-sectional study. METHODS: Patients with a clinical and molecularly confirmed diagnosis of LHON (affected patients in the chronic stage) were enrolled from the neuro-ophthalmology clinic at the Doheny-UCLA. Patients and controls underwent a complete ophthalmologic evaluation, including imaging with OCTA. RESULTS: Twenty-nine eyes from 15 LHON patients (14 male) and 20 eyes from 20 healthy subjects (13 male) were included in the analysis. Mean age was 32.0 ± 14.2 years (range 16-49 years) in the LHON group and 34.2 ± 10.1 years (range 23-48 years) in the control group (P = .552). In the parafoveal region, the vessel length density was lower in LHON patients, at both the SCP (9.1% ± 0.5% and 9.3% ± 0.4%, P = .041) and DCP (9.4% ± 0.5% and 9.8% ± 0.3%, P = .008) levels. In the sectorial analysis, vascular changes remained significant only in the parafoveal nasal and inferior regions. Univariate linear regression analysis demonstrated that the strongest associations with visual acuity were with parafoveal SCP perfusion density (R2 = .276, P = .045) and parafoveal SCP vessel length density (R2 = .277, P = .044). CONCLUSIONS: LHON eyes have SCP and DCP changes that are mainly confined to the nasal and inferior parafoveal sectors that correspond to the papillomacular bundle. Furthermore, visual loss is associated with the SCP flow impairment, but not with the OCT-detectable structural damage.
PURPOSE: To study the macular microvascular networks in patients affected by chronic Leber hereditary optic neuropathy (LHON) using optical coherence tomography angiography (OCTA), and to quantify these changes in different macular sectors. DESIGN: Prospective cross-sectional study. METHODS:Patients with a clinical and molecularly confirmed diagnosis of LHON (affected patients in the chronic stage) were enrolled from the neuro-ophthalmology clinic at the Doheny-UCLA. Patients and controls underwent a complete ophthalmologic evaluation, including imaging with OCTA. RESULTS: Twenty-nine eyes from 15 LHON patients (14 male) and 20 eyes from 20 healthy subjects (13 male) were included in the analysis. Mean age was 32.0 ± 14.2 years (range 16-49 years) in the LHON group and 34.2 ± 10.1 years (range 23-48 years) in the control group (P = .552). In the parafoveal region, the vessel length density was lower in LHON patients, at both the SCP (9.1% ± 0.5% and 9.3% ± 0.4%, P = .041) and DCP (9.4% ± 0.5% and 9.8% ± 0.3%, P = .008) levels. In the sectorial analysis, vascular changes remained significant only in the parafoveal nasal and inferior regions. Univariate linear regression analysis demonstrated that the strongest associations with visual acuity were with parafoveal SCP perfusion density (R2 = .276, P = .045) and parafoveal SCP vessel length density (R2 = .277, P = .044). CONCLUSIONS: LHON eyes have SCP and DCP changes that are mainly confined to the nasal and inferior parafoveal sectors that correspond to the papillomacular bundle. Furthermore, visual loss is associated with the SCP flow impairment, but not with the OCT-detectable structural damage.
Authors: Samuel Asanad; Starleen Frousiakis; Michelle Y Wang; Michele Fantini; William Sultan; Terry Wood; Francis U Nwako; Rustum Karanjia; Alfredo A Sadun Journal: J Curr Ophthalmol Date: 2019-06-06