Abstract
Purpose :
Best vitelliform macular dystrophy (BVMD) is one of the most common causes of inherited macular dystrophy. Although choroidal neovascularization (CNV) is usually associated with advanced stages of the disease, the detection of CNV in the context of a vitelliform lesion can be difficult using older imaging modalities such as fluorescein angiography. In this study, we use swept-source optical coherence tomography angiography (SS-OCTA) to evaluate the presence of CNV and to correlate structural features that may be useful for CNV detection.
Methods :
This was an IRB-approved retrospective review of consecutive patients with molecularly-confirmed BVMD imaged with SS-OCTA (PLEX Elite 9000, Carl-Zeiss Meditec Inc, Dublin, California) at the University of Iowa from September 2017 to July 2020. Clinical data including age, gender, best-corrected visual acuity (BCVA), and prior clinical diagnosis of CNV (i.e., determined without SS-OCTA) were recorded. Structural features including the presence of intraretinal fluid, subretinal fluid, nodular subretinal pillar, focal choroidal excavation, or outer retinal atrophy were identified, and the presence of CNV was also assessed using 6 mm x 6 mm fovea-centered scans by expert graders. Structural features were correlated with the presence or absence of CNV on SS-OCTA using Pearson correlation with p-value <0.05 considered statistically significant.
Results :
A total of 53 eyes from 27 patients (13 female; 48.1%) were included for analysis. The average age was 45 years old (range 8-79), and the mean LogMAR BCVA was 0.38 (range 0-1). A total of 14 eyes (26.4%) had a clinical diagnosis of CNV, whereas evaluation by SS-OCTA revealed the presence of CNV in 27 eyes (50.9%). Structural features potentially associated with CNV including interstitial fluid (14 eyes; 26.4%), focal choroidal excavation (8 eyes, 15.1%), nodular pillar (8 eyes; 15.1%) and outer retinal atrophy (21 eyes; 39.6%) were seen relatively frequently. Of these, the presence of focal choroidal excavation and nodular pillar were both independently correlated with the presence of CNV on SS-OCTA (p<0.01).
Conclusions :
CNV is relatively common in eyes of patients with BVMD. SS-OCTA is a useful tool for CNV detection, and the presence of focal choroidal excavation or nodular pillars should heighten clinical suspicion for the presence of CNV.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.