Ophthalmic Manifestations of Rosai-Dorfman Disease in 5 Patients

Purpose: To report clinical features, pathology characteristics and treatment outcomes of five patients with Rosai- Dorfman disease (RRD). Methods: A retrospective case series of all cases with RRD seen in oncology service of Wills Eye Hospital from 1974 to 2018 (n=5). Results: There were five patients (3 male and 2 female) and 6 eyes with ophthalmic manifestations of RRD. The mean age at initial presentation was 32.8 (range 10-52) years. All patients had unilateral disease except one. The mean duration of symptoms was 9 (range 5-24) months. The ocular tissue affected by RRD included the conjunctiva (n=4), cornea (n=2), orbit (n=2) and uvea (n=2). Palpable lymphadenopathy was detectable in 2 patients. Patient with bilateral disease and uveitis had diffuse lymphadenopathy and multiorgan involvement. Patients with orbital masses had no systemic involvement (n=1) or lymphadenopathy with paranasal sinusitis (n=1). None of the patients with perilimbal lesions had lymphadenopathy (n=2) but, paranasal sinusitis was detected in one of them. Of six eyes with a mean follow-up of 31 months (range, 10-76 months) after surgery, tumor regression was achieved in all cases without recurrence. Conclusion: Rosai-Dorfman Disease can occur in and around the ocular tissues. Patients with intra-ocular manifestations and/or bilateral disease usually have multiorgan systemic involvements while, most patients with conjunctival or orbital lesions have limited disease