Rosai-Dorfman-Destombes disease in adolescence with hearing and vision loss involvement: A multidisciplinary approach

Abstract

Purpose. To report a rare case of Rosai-Dorfman-Destombes (RDD) disease in a teenage girl exhibiting mild fever, night chills, vertigo, and progressive bilateral vision and hearing loss. Method. Case report of a 15-year-old girl who underwent extensive ophthalmic multimodal imaging and a comprehensive workup evaluation. Results. Multiple choroid lesion masses in both eyes associated with systemic multifocal lymphadenopathy, involvement of the paranasal sinuses, and unusual intracranial manifestations well-shown by magnetic resonance imaging (MRI) and whole-body PET-scan. The PET-Scan and MRI imaging showed dural-based intracranial lesions, choroid-plexus signaling, and meningeal spread, as well as a focus on the paranasal sinuses, which helped determine the best biopsy location for a definitive diagnosis through immunohistochemistry. Conclusions. Here we describe a rare condition of RDD disease with bilateral visual and hearing loss associated with bilateral multiple choroid masses and neurological symptoms in an adolescent female patient. A definitive diagnosis relied on histological assessment, emphasizing the importance of interdisciplinary evaluation. RDD should be considered in the differential diagnosis of choroidal metastasis, tuberculosis, and other lymphoproliferative diseases.