Bilateral Complete Loss and Partial Regeneration of Photoreceptor Layers in a Pediatric Vogt-Koyanagi-Harada (VKH) Case

Abstract

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disease affecting melanocytecontaining tissues in the eyes, meninges, ear, and skin. As far as we are aware, this is a unique case report documenting the regeneration of the photoreceptor layer after bilateral complete loss of the photoreceptor layer in a child with VKH. We report a case of a 12-year-old male with no significant past medical history who presented during the chronic stage of incomplete VKH. He was found to have a complete loss of photoreceptor layer in both eyes during a work-up to confirm the aforementioned disease. Upon receiving his first pulse dose of 500 mg IV methylprednisolone as a treatment course, he developed severe steroidinduced hyperglycemia (random blood glucose of 17.6 mmol/L). Additionally, a brain MRI revealed pituitary gland changes compatible with diabetes insipidus, which is a combination that was mentioned once in the literature before. A review of the systems did not suggest any other systemic diseases. The patient's elevated blood sugar level was managed by a pediatrician, and it normalized. At his last visit, optical coherence tomography (OCT) showed hypertrophy/regeneration of the photoreceptor layer. This case report indicates that retinal photoreceptor layer regeneration can be sometimes observed with follow-up after the resolution of inflammation and uveitis.