Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
Ophthalmology Department, College of Medicine, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.
BMC Ophthalmol. 2021 Nov 23;21(1):404. doi: 10.1186/s12886-021-02173-1.
To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review.
A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018.
Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6-72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD).
Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.
通过文献复习，为眼科罗萨达-多夫曼病（RDD）提供基本的人口统计学信息和临床病理特征。
对 1993 年 1 月至 2018 年 12 月在三家三级眼科中心经组织病理学证实的眼科 RDD 患者进行多中心回顾性病例系列研究。
纳入了 8 例患者的 11 只眼，均为经组织病理学证实的眼科 RDD，男女患者数量相等。中位年龄为 40.25 岁（范围：26.6-72.4）。2 例为家族性 RDD。眼眶是最常见的受累部位（90.9%的眼）。1 例（1 只眼）表现为巩膜结节、前葡萄膜炎和类囊样黄斑水肿。视力从 20/25 到光感不等。6 例患者有眶外眼科疾病，其余 2 例患者有伴发的下颌下淋巴结病（结内 RDD）。
眼科 RDD 可能是这种全身性疾病的唯一表现，最常累及眼眶，表现为骨质破坏、颅内和/或鼻窦受累以及不同程度的视力丧失。眼科家族性 RDD 表现为严重形式，具有恶性病程。单独使用类固醇可能不足以控制眼眶 RDD，因此通常需要联合治疗。建议采用全面的评估和管理方法。
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