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Raghu C Mudumbai, Qinqin Zhang, Chieh-Li Chen, Yanping Huang, Ruikang K Wang; Optical Microangiography Imaging in a Patient with Retinal Vasculopathy from Susac’s Syndrome. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):3357.
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© ARVO (1962-2015); The Authors (2016-present)
To investigate the retinal perfusion differences in a patent found to have a focal retinal vasculopathy from Susac’s syndrome using optical microangiography (OMAG).
A 22 year old woman was diagnosed with Susac’s syndrome based on a triad of intracranial lesions including involvement of the corpus callosum on MRI, hearing loss, and vision loss. Ophthalmic examination showing 2 cotton wool spots along the inferotemporal arcade OS. HVF indicated an area of a deep defect superiorly OS that was symptomatic to the patient. Fluoroscein angiography (FA) indicated a corresponding filling defect in the arterioles without signs of pseudo-emboli consistent with Susac’s syndrome.<br /> The patient’s retina was scanned with a 67 kHz Cirrus 5000 HD-OCT based angiography prototype system with motion tracking (Zeiss, Dublin, CA) with a 6 mm x 6 mm field of view to provide 3D OMAG retinal microvascular maps. Using proprietary semi-automatic segmentation software, retinal OMAG images were segmented into 3 layers including Inner retinal layer (ganglion cell + inner plexiform layer), middle retinal layer (inner nuclear + outer plexiform layer) and outer retinal layer (outer nuclear + photoreceptor layer). Enface maximum projection was used to represent angiograms at different layers (coded with different colors for visual purpose).
Results showed strong correlation between OMAG and the area of retinal non-perfusion identified by FA. Quantitative analysis of the retinal vessel density (the ratio of retinal vessel area over the area evaluated) indicated a sharp drop in the retinal perfusion of the effected zone compared to the surrounding retina. Comparison of the retinal hemifields also indicated a significant difference between the effected zone and the corresponding area in the opposite hemifield. Cross sectional analysis of retinal perfusion zones indicated diffuse loss of both the inner and outer retinal layers, slightly more pronounced in the middle retinal vessels. See figure.
OMAG is able to provide additional information that cannot be obtained with FA that quantifies the severity of non-perfusion as well as cross sectional analysis of retinal layer vasculature involvement. OMAG may be a useful modality to characterize the retinal vasculopathy of Susac’s syndrome.
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