Adaptive Optics Findings in Vogt-Koyanagi-Harada Disease

Abstract

Purpose : To describe the adaptive optics (AO) findings in different stages of Vogt-Koyanagi-Harada (VKH) disease and compare them to the anatomical changes seen on spectral domain optical coherence tomography (SD-OCT).

Methods : A 32-year-old Japanese-American female presented to clinic with a 2-week history of blurriness of vision in both eyes (OU) and worsening headache. Patient was previously diagnosed with VKH disease and managed by oral prednisone (60mg/day). The dose of prednisone was tapered, and patient was started on mycophenolate mofetil. Clinical examination and comprehensive retinal imaging, including SD-OCT (Heidelberg Spectralis), and simultaneous confocal and non-confocal split detection AOSLO, were performed on first visit and follow-up visits 1, 3, and 6 months later (Fig 1).

Results : At the time of presentation, visual acuity (VA) was 20/25 (OD) and 20/30 (OS). Slit lamp examination showed 0.5+ cells and 1+ flare in OU. Fundus examination revealed mildly hyperemic optic disc with blurred margins (OD>OS), loss of foveal reflex secondary to residual serous detachment OU, and areas of retinal pigment epithelial atrophy in the posterior pole OU. SD-OCT showed presence of residual subretinal fluid with disruption of the ellipsoid zone (EZ) OU. Confocal AOSLO demonstrated photoreceptor outer segment loss in fovea. However, non-confocal split detection AOSLO at the time of presentation revealed photoreceptor inner segments in areas that appeared devoid of outer segments on confocal imaging, possibly due to subretinal fluid affecting orientation of photoreceptor outer segments. 3 months later,patient’s VA improved to 20/20 OU. SD-OCT demonstrated resolution of subretinal fluid with significant improvement of the EZ, OU. Confocal AOSLO imaging showed presence of normal looking photoreceptors in foveal region (Fig 2). During subsequent visits, photoreceptors appeared normal on the SD-OCT and AOSLO, as shown by normal ellipsoid zone on SD-OCT and confocal AO imaging.

Conclusions : VKH patients may demonstrate transient photoreceptor abnormalities on SD-OCT. High-resolution confocal and non-confocal AO imaging enables sequential documentation and monitoring of transient photoreceptor non-visualization and subsequent improvement on therapy, emphasizing the Stiles-Crawford effect.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

Figure 1: SD-OCT Images of the Patient with VKH at Presentation and Follow-up

 

Figure 2: Adaptive Optics Images of the Patient with VKH at Presentation and Follow-up