Purpose
To describe abnormalities of the optic nerve microvasculature in patients with persistent fetal vasculature (PFV) and their fellow eyes using optical coherence tomography angiography (OCTA).

Design
Cross-sectional study.

Subjects
۵۰ eyes of 25 patients with PFV who underwent prospective imaging using supine OCTA during examination under anesthesia (EUA) at Bascom Palmer Eye Institute from March 1, 2019, to December 31, 2022.

Methods
OCTA images of the optic nerve of the included patients were analyzed with a primary focus on blood flow. Demographic, clinical, and treatment factors were compared to morphologic changes in the optic disc microvasculature.

Main Outcome Measures
Prevalence of optic nerve microvascular abnormalities on OCTA in the affected and fellow eyes of patients with PFV.

Results
A total of 50 eyes from 25 patients were reviewed, and 28% (7/25) met image quality criteria for OCTA analysis. Optic nerve OCTA showed a persistent hyaloid artery in all (7/7) PFV eyes analyzed. Of these, flow on OCTA was detectable in 57% (4/7).

A Bergmeister’s papilla was evident in ۱۰۰% (۲۵/۲۵) fellow eyes, of which flow was detected in 68% (۱۷/۲۵).

Fluorescein Angiography (FA) demonstrated blood flow within the stalk in 40% (10/25) of PFV eyes and within the Bergmeister’s papilla in 25% (6/25) of fellow eyes. Similar findings of abnormal blood flow and presence of fibrovascular stalk were seen in both treatment naïve and treated groups.

Conclusions
OCTA allows for high-resolution visualization of subtle vascular abnormalities that are not readily apparent using RetCam FA and may serve as a useful noninvasive test to confirm the patency of the PHA and Bergmeister’s papilla in children. Results of the present study suggest PFV may be a bilateral and asymmetric process.

This is the largest report of pediatric patients with affected and unaffected PFV eyes imaged using OCTA. OCT angiography allows for high-resolution visualization of subtle vascular abnormalities that are not readily apparent using RetCam FA and may serve as a useful noninvasive test to confirm the patency of the PHA and Bergmeister’s papilla in children. Results of the present study and prior literature suggest PFV may be reconsidered as a bilateral, asymmetric process. Continued monitoring with detailed retinal vascular imaging and genetic testing is needed to further elucidate the impact of these findings and the pathophysiology of the disease.

Figure – Representative fundus, FA and OCTA studies in a 7-year-old patient with PFV on the right eye treated with PPL + PPV and Bergmeister papilla on the left eye. A-C correspond to PFV (right) eye. A represents a fundus photography after PPL + PPV treatment. B represents a fluorescein angiogram showing temporal peripheral avascularity (yellow arrowhead). C corresponds to an optical coherence tomography angiography (OCTA) image of the optic nerve. OCTA shows hyporeflective tubular structure of the persistent hyaloid artery (white asteristic) and an elevated tissue structure corresponding to the remaining fibrovascular stalk (white arrow). The persistent hyaloid artery with blood flow is also evidenced (white arrowhead). D-F correspond to fellow (left) eye. D represents a fundus photography without any evidence of Bergmeister papilla. E represents a fluorescein angiogram showing temporal peripheral avascularity (yellow arrowhead). C corresponds to an optical coherence tomography angiography (OCTA) image of the optic nerve. OCTA shows hyporeflective tubular structure of the persistent hyaloid artery (white asteristic) and an elevated tissue structure corresponding to the Bergmeister’s papilla (white arrow). The Bergmeister papilla with blood flow is also evidenced (white arrowhead).