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

Hamad M Alsulaiman, Reem Alanazi, Sahar Elkhamary, Azza Maktabi, Hind Alkatan, Rawan N Althaqib, Osama Sheik, Silvana A Schellini
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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.

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影响两兄弟的孤立性双侧眼眶和鼻旁罗赛-多夫曼病:病例报告和系统文献综述。
系统性文献回顾显示,有88例罗赛-多夫曼病(RDD)病例影响到眼眶。我们对有关眼眶 RDD 的文献进行了回顾,并介绍了两兄弟与 G-6PD 缺乏症相关的孤立性双侧结节外眼眶 RDD 病例。该病表现为不对称的广泛眼眶和副鼻窦浸润,伴有眼眶骨质破坏。眼眶肿块切除术和类固醇治疗改善了两名患者的病情。这是目前报道的最广泛的眼眶 RDD 病例系列,包括临床表现、影像学检查、病理结果、治疗和预后。警惕随访对监测潜在的恶性转化、全身表现、潜在的视力丧失或危及生命的复发至关重要。
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来源期刊
CiteScore
2.40
自引率
9.10%
发文量
136
期刊介绍: Orbit is the international medium covering developments and results from the variety of medical disciplines that overlap and converge in the field of orbital disorders: ophthalmology, otolaryngology, reconstructive and maxillofacial surgery, medicine and endocrinology, radiology, radiotherapy and oncology, neurology, neuroophthalmology and neurosurgery, pathology and immunology, haematology.
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