Vogt-Koyanagi-Harada Disease: Current Diagnosis and Management

Vogt-Koyanagi-Harada (VKH) disease is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin, and hair. This disease is mainly a T1 lymphocyte-mediated aggression to melanocytes. The availability of new investigational methods has improved our knowledge of the pathogenesis, clinical approach, diagnosis, and management of VKH disease. The disease has an acute onset of bilateral blurred vision with hyperemia in the absence of prior ocular trauma or any previous intraocular surgery. The chronic phase comprises of ocular and integumentary system pigmentary changes. Ocular findings may be accompanied by meningismus, hearing impairment, and skin lesions in a variable proportion of patients. Prompt diagnosis with early, aggressive, and long-term treatment of high-dose corticosteroids ensures good visual outcomes. The aim of this chapter is to present the clinicopathology, classification, recent imaging, investigations, and management of VKH disease

Long-term efficacy and safety of verteporfin photodynamic therapy in combination with anti-vascular endothelial growth factor for polypoidal choroidal vasculopathy

Purpose: The aim of the study was to analyze the outcomes of photodynamic therapy (PDT) with intravitreal anti-vascular endothelial growth factor (anti-VEGF) for patients with polypoidal choroidal vasculopathy (PCV) having visual acuity (VA) better than 20/60 in a real-world scenario in India. Methods: Retrospective review of 42 eyes of 40 patients (mean age 64.3 years) with best-corrected VA (BCVA) 20/60 or better and mean follow-up of 40 months (median 38 months; range 12–71 months) treated with PDT and anti-VEGF or triamcinolone for indocyanine green angiography (ICGA)-proven subfoveal PCV. Results: Mean BCVA improved from 0.22 logMAR at baseline to 0.15 at last visit (P < 0.001). On ICGA, polyp was observed in 42 eyes (100%) and branching vascular network (BVN) in 37 eyes (88.1%). Polyp regressed in 33 (78.6%) of 42 eyes and BVN in 26 (70.3%) of 37 eyes after combined therapy at 3 months. Mean greatest linear diameter reduced significantly (P < 0.001) from 7.22 mm to 4.11 mm. Standard-fluence PDT was performed in 35 eyes and reduced-fluence in 7 eyes. The mean number of PDT was 1.17 with mean number of injections being 6.38 at the end of follow-up. In five eyes, more than one PDT was administered. Of 42 eyes, 40 showed complete resolution of serous macular detachment (SMD) after the combined therapy at 3 months; 17 (42.5%) of the 40 eyes showed no recurrence of fluid on spectral domain optical coherence tomography till the last visit with a mean follow-up of 27 months. On long-term follow-up, SMD reoccurred in 23 eyes with a mean follow-up period of 9.64 ± 5.24 months. Of 38 eyes having a double-layer sign (DLS) on optical coherence tomography at baseline, 37 eyes were having regression of the DLS, that is, it either reduced or resolved at the final visit. At the final visit, 66.7% (P < 0.001) eyes were having fluid-free retina. No complication of subretinal hemorrhage was noted. Of the 42 eyes, only one eye had BCVA worse than 20/60 on the final visit. Conclusion: To the best of our knowledge, this is the first study to look into the long-term effect of combined PDT with anti-VEGF in PCV in eyes having good VA. Long-term effect of combined PDT appears to be a safe and effective treatment for PCV in eyes having good VA with better outcomes in real-world setting. This study further strengthens the superiority of the combined treatment modality for treatment of subfoveal PCV with no or minimal risk of complication on long-term follow-up

Retrospective diagnosis of COVID-19 following the detection of central retinal artery occlusion

Thromboembolic complications are being increasingly reported in patients with COVID-19 due to the associated hypercoagulability and are an important cause for morbidity and mortality. Retinal vascular occlusions especially arterial occlusions are one of the gravest ocular complications reported. This complication may occur in severe cases with cytokine storm or even in mild or asymptomatic patients and presentation can be anytime from few days to weeks after the onset of symptoms. Ophthalmologists should be aware of this new etiology when dealing with patients having features of retinal vascular occlusions and should investigate for the same in this pandemic situation. Although reverse transcriptase polymerase chain reaction is the diagnostic test for COVID-19, serological assays have a role in patients with delayed presentation. We describe the clinical features and multimodal imaging findings in a patient who presented with features of central retinal artery occlusion with cilioretinal artery sparing wherein his ophthalmic condition led to the diagnosis of previously undetected COVID-19 through serology. To the best of our knowledge, this is the first documentation of a case of isolated central retinal artery occlusion leading to a retrospective diagnosis of COVID-19

Real-time in vivo micromorphology and histopathology of choroidal osteoma using enhanced depth imaging

Choroidal osteoma is a usually unilateral benign tumor of the choroid composed of mature bone. Optical coherence tomography (OCT) has been used to image osteoma for several years. With the advent of enhanced depth imaging (EDI) feature of spectral-domain OCT (SD-OCT), better visualization of the morphology of choroidal lesions has been possible. Herein we present a case of choroidal osteoma in a 45-year-old woman, wherein in vivo morphology of the choroidal osteoma had been visualized using EDI technique of SD-OCT before and after performing photodynamic therapy. EDI OCT has proven to be a valuable noninvasive imaging modality, almost comparable to histopathological examination, for diagnosing choroidal osteomas and for providing an insight into the in vivo micromorphological changes occurring during the course of the disease

”Double-layer sign” on spectral domain optical coherence tomography in pachychoroid spectrum disease

Purpose: The “double-layer sign (DLS)” describes the shallow and irregular elevation of the retinal pigment epithelium from the underlying intact Bruch's membrane visualized on the spectral domain optical coherence tomography. In this study, we evaluated the frequency, characteristics of the space within the double layer and other features in the pachychoroid spectrum to aid the clinical diagnosis of these variants. Methods: This retrospective study evaluated the features of the DLS on multimodal imaging in consecutive patients with a clinical diagnosis of one of the four variants of pachychoroid: pachychoroid pigment epitheliopathy (PPE), pachychoroid neovasculopathy (PCN), chronic central serous chorioretinopathy (CCSCR), and polypoidal choroidal vasculopathy (PCV). The features of the DLS were graded by two masked graders. Results: Overall, 102 eyes of 79 consecutive patients with pachychoroid spectrum were identified for grading. Sixteen eyes with PPE did not show any evidence of DLS. The DLS was identified in 15/16 (93.75%) eyes with PCN, 11/35 (31.43%) with CCSCR, and 32/35 (91.43%) with PCV (P < 0.001). The space within the DLS showed moderate hyperreflectivity in all eyes with PCV and PCN, while the space in the DLS in CCSCR showed uniform hyporeflectivity in 10/11 (%) eyes. Conclusion: The DLS sign was most frequent in polypoidal vasculopathy and PCN. A hyporeflective gap within the DLS favored the diagnosis of CCSCR