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

<b>Purpose.</b> 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. <b>Method.</b> Case report of a 15-year-old girl who underwent extensive ophthalmic multimodal imaging and a comprehensive workup evaluation. <b>Results.</b> 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. <b>Conclusions.</b> 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.

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Publication Details

Journal
European Journal of Ophthalmology
Published
2024-11-18
DOI
https://doi.org/10.1177/11206721241300634
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

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

Marina Gaglianone Teodoro da Silva, Murilo Wendeborn Rodrigues, Eduardo Cunha de Souza, Ana Rodrigues et al.
European Journal of Ophthalmology
Histiocytic Disorders and Treatments
article

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

Marina Gaglianone Teodoro da Silva, Murilo Wendeborn Rodrigues, Eduardo Cunha de Souza, Ana Rodrigues, Maria Alice Altenburg de Assis, Inara Figueiredo Jacobsen
article en

Abstract

<b>Purpose.</b> 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. <b>Method.</b> Case report of a 15-year-old girl who underwent extensive ophthalmic multimodal imaging and a comprehensive workup evaluation. <b>Results.</b> 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. <b>Conclusions.</b> 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.

European Journal of OphthalmologyVol. 35(2)
Faculdade de Medicina de São José do Rio Preto (BR), Universidade de São Paulo (BR)
Good health and well-being
Openalex Percentile: Top 71%
Histiocytic Disorders and Treatments
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