Optical Coherence Tomography Angiography of a Choroidal Neovascularization in Adult Onset Foveomacular Vitelliform Dystrophy: Pearls and Pitfalls

PURPOSE. The purpose of this study was to determine the sensitivity and specificity of optical coherence tomography angiography (OCT-A) in detecting choroidal neovascularization (CNV)-complicating adult onset foveomacular vitelliform dystrophy (AOFVD) and to highlight the possible pitfalls related to the heterogeneous spectrum of acquired vitelliform maculopathies. METHODS. Twenty-five eyes of 22 consecutive AOFVD patients with suspected CNV were enrolled. Conventional multimodal imaging findings, based on fluorescein angiography (FA), indocyanine green angiography (ICGA) and B-Scan OCT, were used as a basis and were compared with those obtained from OCT-A to define its sensitivity and specificity for detecting CNV in the case of AOFVD. A qualitative and quantitative analysis of the CNV appearance and of the associated OCT-A findings were also performed with the aim of defining features and elucidating possible diagnostic pitfalls. RESULTS. Conventional multimodal imaging allowed diagnosis of a CNV in 5 of 25 eyes (20%), whereas a CNV lesion was clearly observed on OCT-A in 4 of 25 cases (16%). The sensitivity and specificity of CNV detection by OCT-A in cases of AOFVD was 4 of 5 cases (80%) and 20 of 20 cases (100%), respectively. Optical coherence tomography angiography in 10 cases (40%) showed a focal hyperintense signal, without vascular aspects, at the level of the outer nuclear layer or immediately above the subretinal material accumulation. CONCLUSIONS. Our study demonstrates the capability of OCT-A to allow diagnosis of the presence of a CNV in AOFVD patients. Although FA remains the gold standard for determining the presence of a neovascular network, OCT-A offers noninvasive monitoring of the retinal and choroidal microvasculature, aiding in diagnosis and treatment decisions during follow-up.