Optical Coherence Tomography Angiography Assessment in Congenital Aniridia

& BULL; PURPOSE: This study aims to characterize foveal vasculature assessed by optical coherence tomography angiography (OCT-A) in congenital aniridia which is hallmarked by foveal hypoplasia (FH).& BULL; METHODS: At the National Referral Center for congenital aniridia, patients with confirmed PAX6 -related aniridia and FH diagnosed on spectral-domain OCT (SDOCT) with available OCT-A and matched control subjects were included. OCT-A was performed in patients with aniridia and control subjects. Foveal avascular zone (FAZ) and vessel density (VD) were collected. VD in the foveal and parafoveal areas at the level of the superficial and deep capillary plexi (SCP and DCP, respectively) were compared between the 2 groups. In patients with congenital aniridia, correlation between VD and the grading of FH was assessed.& BULL; RESULTS: Among 230 patients with confirmed PAX6 related aniridia, high-quality macular B-scans and OCTA were available in 10 patients. On the foveal area, mean VD was higher in aniridia patients (41.10%, n = 10) than in control subjects (22.65%, n = 10) at the level of the SCP and the DCP ( P = .0020 and P = .0273, respectively). On the parafoveal area, mean VD was lower in patients with aniridia (42.34%, n = 10) than in healthy subjects (49.24%, n = 10) at the level of both plexi ( P = .0098 and P = .0371, respectively). In patients with congenital aniridia, a positive correlation was found between the grading of FH and the foveal VD at the SCP ( r = 0.77, P = .0106).& BULL; CONCLUSIONS: Vasculature is altered in PAX6 related congenital aniridia, higher in foveal and lower in parafoveal areas, especially when FH is severe, which is consistent with the concept that the absence of reti -nal blood vessels is essential for foveal pit development. (Am J Ophthalmol 2023;253: 44-48.& COPY; 2023 Elsevier Inc. All rights reserved.)

Automated summary

This study aimed to characterize foveal vasculature in patients with congenital aniridia using optical coherence tomography angiography (OCT-A). The results showed higher vessel density in the foveal area but lower vessel density in the parafoveal area in patients with aniridia. There was a positive correlation between the grading of foveal hypoplasia and foveal vessel density.