Title : Vogt-koyanagi-harada presenting with papilledema in a 5-year-old – Case report
Abstract:
Introduction: Vogt-Koyanagi-Harada (VKH) disease is rarely observed in preschool-aged children, and when it does occur, it typically presents with exudative retinal detachment during the acute phase. Treatment in this population commonly involves corticosteroids, immunosuppressants, biologic agents, or combination therapy. Here, we report a case of a 5-year-old patient with VKH presenting atypically with papilledema and refractory inflammation, leading to uveitic glaucoma and necessitating an escalation of adalimumab to 40 mg biweekly.
Case Presentation: A 5-year-old girl presented with a 3-week history of eye redness, excessive lacrimation, and photophobia. Her medical history was unremarkable. On examination, her best-corrected visual acuity (BCVA) was 20/80 in the right eye and 20/100 in the left eye, with normal intraocular pressure (IOP) in both eyes. Anterior segment examination revealed fine keratic precipitates, significant anterior chamber inflammation (+4 cells, +4 flare), and semi-dilated pupils with posterior synechiae. Posterior segment evaluation was limited by severe vitritis. Laboratory investigations were unremarkable except for HLA typing, which was positive for HLA-DR4, DR52, and DR53. Optical coherence tomography (OCT) and retinal nerve fiber layer (RNFL) analysis demonstrated papilledema and increased retinal nerve thickness, respectively. Initial treatment with corticosteroids and methotrexate failed to achieve remission. Attempts to taper corticosteroids resulted in recurrence of anterior chamber flare, prompting the introduction of adalimumab at 20 mg biweekly. Despite relative stability, persistent anterior chamber inflammation and subsequent corticosteroid tapering led to the development of uncontrolled uveitic glaucoma, which required surgical peripheral iridectomy. Following glaucoma management, adalimumab was escalated to 40 mg biweekly, enabling successful tapering of corticosteroids. Over a 9-month follow-up period, the patient remained flare-free, with BCVA improving to 20/20 in both eyes.
Conclusion: This case highlights an atypical presentation of VKH in a preschool-aged child, characterized by papilledema without exudative retinal detachment. Escalation of adalimumab to 40 mg biweekly effectively controlled inflammation, facilitated corticosteroid tapering, and preserved visual acuity.
