ObjectiveTo observe the OCT angiography (OCTA) features of adult-onset foveomacular vitelliform dystrophy (AFVD).MethodsRetrospective clinical observational study. Twelve patients (22 eyes) diagnosed as AFVD by multi-modal imaging in Ophthalmology Department of Yunnan Second People’s Hospital from March 2018 to May 2019 were included in this study. There were 8 males (16 eyes) and 4 females (6 eyes). The patients aged from 33 to 62 years, with the mean age of 48.7±8.9 years. Ten patients were binocular, 2 patients were monocular. The visual acuity was 0.08-0.6. In 22 eyes, the vitelloid-like substance was relatively complete in 8 eyes, the vitelloid-like substance had different degrees of rupture in 14 eyes, secondary choroidal neovascularization (CNV) was observed in 10 eyes. The Heidelberg OCTA instrument was used for OCTA examination. The central wavelength was 840 nm, the acquisition speed was 85,000 times/s. A 3 mm × 3 mm scan was obtained. In the scanning process, eye-tracking technology was adopted to select images with better image quality and position for marking and saving. The image characteristics of vitelloid-like substance, fundus vascular changes and secondary CNV in OCTA were analyzed.ResultsIn 8 eyes with a relatively complete vitelloid-like substance, B-scan images showed dense vitelloid-like substance under the retinal neurocortical layer, which was located between the RPE layer and the ellipsoid zone and had a uniform density. Blood flow signals at the vitelloid-like substance can be seen in the en-face image, which was the artifact of the vitelloid-like substance reflecting the blood vessels above. In the 14 eyes with different degrees of vitellin-like material rupture, the signal of vitellin-like substance between the ellipsoid zone and the RPE layer in the B-scan image was not uniform, and some weak reflected signal lacunae could be seen. In the image of en-face, the relatively intact areas of vitelloid-like substance still showed the artifact of the blood vessels above the reflection, while there was no blood flow signal at the rupture of vitelloid-like substance. In 22 eyes, the morphology of retinal small blood vessels in the superficial and deep capillary arch ring region of retina was abnormal in 10 eyes. Some small blood vessels could be seen to have branch and shape changes, and the anastomosis failed to show a complete arch ring structure.No significant structural changes in retinal capillaries were observed in 12 eyes. Among the 10 eyes with secondary CNV, 8 eyes showed the non-active CNV which was as thick as "wild branches", and 2 eyes showed the active CNV which was composed of dense and small vascular branches.ConclusionAFVD in OCTA can be manifested as abnormal retinal vascular morphology caused by the vitelliform material pushing, vascular artifacts reflected by the vitelliform material itself, and the presence of CNV under the vitelliform material.
ObjectiveTo observe the imaging characteristics of fundus choroidal nodules in patients with neurofibromatosis type 1 (NF1). MethodsA retrospective clinical study. From January 2018 to August 2022, 20 eyes of 10 patients with NF1 combined with choroidal nodules who were diagnosed by ophthalmology examination at the Affiliated Hospital of Yunnan University were included in the study. Among them, there were 6 male cases with 12 eyes and 4 female cases with 8 eyes; both eyes were affected. Age was (28.0±6.9) years old. Both eyes were involved. All patients underwent color fundus photography, infrared fundus photography (IR), fundus autofluorescence (FAF), fluorescein fundus angiography (FFA), and optical coherence tomography (OCT). Nine eyes underwent multi-wavelength color imaging (MC) and 5 eyes underwent OCT angiography (OCTA). ResultsIn 20 eyes, fundus color photography showed "spiral-like" changes in the small retinal blood vessels on the surface of the choroidal nodules in 1 eye. FAF and FFA examination showed no abnormalities in all affected eyes. On IR examination, choroidal nodules appeared as strong reflective lesions of varying sizes and numbers, in the form of spots and/or sheets, and were partially fused. In the 9 eyes that underwent MC examination, patchy red signals was observed in standard MC images. OCT examination showed that all affected eyes had strong choroidal reflective mass lesions under the retinal pigment epithelium, which were flat patchy or slightly raised “dome-like”, corresponding to IR strong reflective lesions. The choriocapillaris layer was squeezed and thinned, and the large choroidal vessels show weak reflection. Five eyes underwent OCTA examination, there was no loss of blood flow density at the choroidal nodules and the of the superficial an deep retinal capillary plexus in 3 eyes. The choroidal capillary blood flow density was reduced in 2 eyes. ConclusionIR of choroidal nodules is characterized by strong reflection lesions of varying sizes and numbers, which appear in spots and/or sheets. OCT shows enhanced reflection of the choriocapillaris layer corresponding to the strong IR reflection lesions.