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.
摘要：
系统的文献综述揭示了88例影响眼眶的Rosai-Dorfman病（RDD）病例。除了两个兄弟与G-6PD缺乏症相关的双侧结外轨道RDD病例外，我们还对轨道RDD的文献进行了综述。该疾病表现为不对称的广泛的眼眶和鼻旁窦浸润，眶骨破坏。眼眶肿块缩小和类固醇改善了两名患者的病情。这是报道的最广泛的轨道RDD病例系列，包括临床表现，成像,病理结果,管理,和结果。警惕的随访对于监测潜在的恶性转化至关重要，系统性表现，潜在的视力丧失,或危及生命的复发。
