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Elisabetta Pilotto, Elisabetta Beatrice Nacci, Alfonso Massimiliano Ferrara, Gilda De Mojà, Raffaele Parrozzani, Stefania Zovato, Edoardo Midena; Macular flow in von Hippel-Lindau disease. Invest. Ophthalmol. Vis. Sci. 2020;61(7):2563.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate, using optical coherence tomography angiography (OCT-A), macular retinal capillary flow in patients with Von Hippel–Lindau (VHL) disease without or with peripheral retinal hemangioblastomas.
Patients with VHL disease were consecutively enrolled in this cross-sectional study. Eyes with retinal hemangioblastomas at the posterior pole, macular pucker on any other macular disease were excluded. Age-matched healthy subjects were enrolled as controls. All included eyes were evaluated with OCT-A (Spectralis HRA+OCTA, Heidelberg Engineering). Using OCT en face angiograms, automatically segmented, macular perfusion was analyzed in the superficial (SCP), intermediate (ICP) and deep retinal capillary plexuses (DCP). Quantitative analysis of the vascular plexuses was performed using an open-source available ImageJ software (National Institutes of Health, Bethesda, Maryland, USA). The following OCT-A vascular parameters were measured: vessel area density (VAD); vessel length fraction (VLF); vessel diameter index (VDI) and fractal dimension (FD).
Forty-four VHL patients (81 eyes) and 20 healthy controls (40 eyes) were enrolled. In VHL eyes, macular perfusion was significantly reduced compared to controls (p< 0.005 for all OCT-A parameters) in all capillary plexuses. No differences were detected between eyes without or with retinal hemangioblastomas.
Macular flow is reduced in patients with VHL disease, even in absence of retinal hemangioblastomas. These results could be explained considering the pathophysiology of VHL: the disruption of the hypoxia-induced factors (HIF)/vascular endothelium growth factor (VEGF) signaling, that leads to abnormal vascular remodeling, and the aberrant Notch signaling, which plays an important role in both vasculogenesis and angiogenesis. These results seem to validate the hypothesis of the VHL disease- associated hemangioblastoma as a developmental, rather than a neoplastic, phenomenon.
This is a 2020 ARVO Annual Meeting abstract.
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