Quantitative OCT angiography of the retinal and choroidal vascular circulation in pediatric patients with neurofibromatosis type 1

Abstract

To investigate retinal and choroidal microvascular changes in neurofibromatosis type 1 (NF1) pediatric patients using optical coherence tomography angiography (OCTA). This study was performed on 21 eyes of 21 NF1 patients and 30 eyes of 30 healthy subjects. Foveal avascular zone (FAZ) parameters, including area, perimeter, acircularity index, and foveal density, were measured using OCTA. Superficial (SCP) and deep (DCP) capillary plexus and radial peripapillary capillary (RPC) vessel densities and choriocapillaris flow were also evaluated. Choroidal nodules and thickness were detected using infrared reflectance and enhanced-depth imaging OCT. No significant differences were observed for FAZ parameters or SCP and RPC vessel densities between groups. DCP vessel densities in the perifovea were significantly lower in the NF1 group than in the control group (P = 0.001). A significantly lower choriocapillaris flow area (P < 0.001) and increased subfoveal and nasal perifoveal choroidal thickness (P < 0.001) were observed in the NF1 group. Choriocapillaris flow area tended to be inversely related to the number of choroidal nodules in the NF1 group. Perifoveal DCP vessel density and choriocapillaris flow significantly differed between NF1 patients and controls, suggesting that retinal and choroidal vascular microcirculation is affected in the former, potentially by choroidal nodules. Further longitudinal studies are required to determine the effects of these differences on circulation in the posterior segment of the eye.