Optic atrophy due to Rosai-Dorfman disease

case report

Abstract

A 59-year-old man presented with decreased visual acuity and progressive hearing loss. He had a history of hypertension, poorly controlled diabetes, and nasal polyp surgery. The ophthalmologic examination showed severely reduced visual acuity in both eyes, normal intraocular pressure, and pale optic discs. The studies revealed generalized scotomas, thinning of the nerve fiber layer and ganglion cells, rhinorrhea, anosmia, and ventilatory insufficiency. Rhinoscopy identified a papillomatous lesion in both nasal cavities, and computed tomography showed soft tissue densities in several paranasal sinuses. Magnetic resonance imaging revealed diffuse tissue in the nasal cavities and paranasal sinuses, with pachymeningeal thickening near the optic nerves. A nasal sinus biopsy confirmed the diagnosis of Rosai-Dorfman disease, a rare condition characterized by benign proliferation of histiocytes in lymph nodes and extranodal tissues. This disease can manifest with massive lymphadenopathy, fever, and systemic symptoms. Ocular involvement can lead to the impairment of adjacent structures, such as the optic nerves, resulting in vision loss. PET-CT showed metabolic activity in the skeleton, suggesting active disease. Due to the rarity and severity of Rosai-Dorfman disease, a multidisciplinary team considered decompressive surgery, along with systemic anti-inflammatory and immunomodulatory treatment, to address the progression of this condition.