En Face Optical Coherence Tomography Imaging of the Choroid in a Case with Central Serous Chorioretinopathy during the Course of Vogt-Koyanagi-Harada Disease: A Case Report

Abstract

Vogt-Koyanagi-Harada (VKH) disease and central serous chorioretinopathy (CSC) develop serous retinal detachment; however, the treatment of each disease is totally different. Steroids treat VKH but worsen CSC; therefore, it is important to distinguish these diseases. Here, we report a case with CSC which was diagnosed by en face optical coherence tomography (OCT) imaging during the course of VKH disease. A 50-year-old man was referred with blurring of vision in his right eye. Fundus examination showed bilateral optic disc swelling and macular fluid in the right eye. OCT showed thick choroid, and en face OCT images depicted blurry choroid without clear delineation of choroidal vessels. Combined with angiography findings, this patient was diagnosed with VKH disease and treated with steroids. Promptly, fundus abnormalities resolved with the reduction of the choroidal thickness and the choroidal vessels became visible on the en face images. During the tapering of the steroid, serous macular detachment in the right eye recurred several times. Steroid treatment was effective at first; however, at the fourth appearance of submacular fluid, the patient did not respond. At that time, the choroidal vessels on the en face OCT images were clear, which significantly differed from the images at the time of recurrence of VKH. Angiography also suggested CSC-like leakage. The tapering of the steroids was effective in resolving the fluid. Secondary CSC may develop in the eye with VKH after steroid treatment. En face OCT observation of the choroid may be helpful to distinguish each condition.

Vogt–Koyanagi–Harada syndrome – current perspectives

“…However, in CSCR, visual acuity remains good, patients do not complain of ocular pain and there is no evidence of granulomatous ocular inflammation or a hot optic disc on FFA 51,130. Infectious choroiditis caused by syphilis,131 tuberculosis,132 and Bartonella henselae infection133,134 should also been considered in the differential diagnosis of VKHD.…”

Shallow Anterior Chamber in a Severe Case of Unilateral Acute Central Serous Retinal Detachment

“…Thickened choroid may be present, representing the anatomical substrate supporting abnormal choroidal circulation in CSC [ 6 ]. How the enlarged choroid relates to CSC is unclear [ 7 , 8 ].…”

“…Both VKH disease and CSC lead to serous retinal detachment. Although the pathogenesis of each disease is different, a thickened choroid can be seen in OCT imaging [ 8 ]. Bilateral involvement has been reported to be as high as 40% in CSC patients, although at the time of diagnosis, it has been reported as just 4% [ 9 ].…”

Outcomes of Retinal Pigment Epithelial Detachment in Vogt-Koyanagi-Harada Disease

“…The typical FFA manifestations of VKH are numerous punctate hyper uorescent dots at the RPE level in the early stage, followed by staining and pooling of the dye in the subretinal space, optic disc hyper uorescence and leakage in the late stage [3]. Nonetheless, in classic CSC, unifocal leakage at RPE level with "ink-blot'' or "smokestack" pattern is typical, but in eyes with steroid-induced CSC, the intense leakage from multiple regions is often seen [15,20]. Typical ICGA signs of VKH include early hyper uorescent stromal vessels, which indicates severe choroidal stromal in ammatory vasculopathy, and late hypo uorescent dark dots, which are thought to correspond to choroidal granulomas, and fuzzy or lost vascular patterns of large stromal vessels due to choroidal in ammation [1][2][3]21].…”