Isolated bilateral orbital and paranasal Rosai-Dorfman disease affecting two brothers: a case report and a systematic literature review

ABSTRACT

A systematic literature review revealed 88 Rosai–Dorfman Disease (RDD) cases affecting the orbit. We present a review of the literature on orbital RDD in addition to two brothers with isolated bilateral extra-nodal orbital RDD cases associated with G-6PD deficiency. The disease manifested as asymmetric extensive orbital and paranasal sinus infiltration, with orbital bone destruction. Orbital mass debulking and steroids improved the condition in both patients. This is the most extensive case series of orbital RDD reported, including clinical manifestation, imaging, pathologic results, management, and outcome. Vigilant follow-up is essential to monitor for potential malignant transformation, systemic manifestations, potential vision loss, or life-threatening recurrences.

KEYWORDS:

• Rosai-Dorfman
• orbit
• bilateral orbital disease
• case report
• systematic review

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

Supplementary materials

Supplemental data for this article can be accessed online at https://doi.org/10.1080/01676830.2023.2291791

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Funding

The author(s) reported there is no funding associated with the work featured in this article.