Authors: Grayson W. Armstrong, MD1, Vivian P Douglas, MD, DVM, MBA1, Meghan J. Smith, MD2, George N. Papaliodis, MD1, John B. Miller, MD1

Affiliations:

1Ophthalmology Department, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, USA

2Faculty of Medicine & Dentistry, University of Alberta, Edmonton, Alberta, Canada

Clinical Presentation: A 19-year-old female of Indian and Italian descent presented to the Massachusetts Eye & Ear Emergency Ward for a second opinion of her one-week history of headache, bilateral visual loss, and metamorphopsia. Visual acuity was 20/40 OD and 20/25-1 OS, with normal pupils and intraocular pressures. Amsler grid demonstrated bilateral metamorphopsia with central minification. Anterior segment examination demonstrated trace conjunctival injection, endothelial keratoprecipitates, and 2+ cell without hypopyon bilaterally. Posterior examination and fundus photography demonstrated 1+ vitritis with white dots in bilateral posterior poles as well as serous macular detachments (Fig. 1A).

Imaging findings: Fundus autofluorescence demonstrated mild hyperfluorescence in the macula at sites of serous retinal detachment (Fig. 1B). Fluorescein angiography demonstrated delayed choroidal filling with optic disc leakage as well as macular and peripapillary focal staining resembling a ‘starry sky’ pattern in early and late frames (Fig. 1C,D). Spectral-domain optical coherence tomography showed bilateral macular serous retinal detachments with a subretinal fibrinous septae in the right eye (Fig 1E) . Optical coherence tomography angiography (OCTA) demonstrated diffuse areas of decreased choriocapillaris flow signal associated with areas of serious retinal detachments (dashed line, Fig. 2C,D) as well as smaller focal areas of choriocapillaris flow void (solid line, Fig. 2C,D).

Discussion points: VKH is a severe autoimmune inflammatory disease more commonly seen in young patients with a recent viral infection and from specific ethnic backgrounds (Asian, Hispanic, Indian, Mediterranean) affecting the choriocapillaris as well as other ocular, neurologic, auditory, and integumentary structures. We present two OCTA imaging findings in the acute phase of VKH, the first being small focal areas of choriocapillaris flow void, and the second being large areas of flow void associated with serous macular detachments. All OCTA findings resolved in our patient after the use of corticosteroids (Fig 2E,F). These findings support the conclusion that OCTA imaging may be beneficial in early diagnosis, assessing for response to treatment, and monitoring for relapse of disease in VKH. Prevention of relapses through evaluation for choriocapillaris dropout on OCTA, instead of evaluating for later clinical signs, could help support the use of early corticosteroid therapy.