Ophthalmic Rosai-Dorfman disease: a multi-centre comprehensive study

Area of Science:

• Ophthalmology
• Pathology
• Oncology

Background:

• Rosai-Dorfman disease (RDD) is a rare, benign proliferative disorder.
• Ophthalmic involvement in RDD is uncommon but can significantly impact vision.
• Understanding the demographic and clinicopathologic features of ophthalmic RDD is crucial for diagnosis and management.

Purpose of the Study:

• To present demographic and clinicopathologic data of ophthalmic Rosai-Dorfman disease.
• To review the existing literature on ophthalmic RDD.
• To highlight key features and management considerations for this condition.

Main Methods:

• A multi-center retrospective case series.
• Inclusion of patients with histopathologically confirmed ophthalmic RDD.
• Data collection spanning January 1993 to December 2018 from three tertiary eye care centers.

Main Results:

• Eleven eyes from eight patients (50% male/female) with ophthalmic RDD were analyzed.
• The median age was 40.25 years; two patients had familial RDD.
• The orbit was the most frequent site (90.9%), with varied visual acuity and some cases presenting with extra-nodal or nodal RDD.

Conclusions:

• Ophthalmic RDD can be an isolated finding or part of systemic disease, frequently involving the orbit with potential for bone destruction and vision loss.
• Familial RDD is aggressive and may require multimodal therapy, as steroids alone may be insufficient for orbital RDD.
• A comprehensive assessment and integrated management strategy are recommended for ophthalmic RDD.