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

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.

Automated summary

This article presents a systematic literature review on orbital Rosai-Dorfman Disease (RDD), including 88 reported cases. It also reports two cases of isolated bilateral extra-nodal orbital RDD associated with G-6PD deficiency. The disease was characterized by extensive orbital and paranasal sinus infiltration, as well as orbital bone destruction. Surgical debulking and steroid treatment improved the condition in both patients.