Orbital and adnexal Rosai-Dorfman disease

To report the clinico-radiological findings, clinical course, and treatment outcomes in five patients with orbital and adnexal Rosai-Dorfman (R-D) disease.

Analysis of case records of patients with Rosai-Dorfman disease seen at four orbital units between January 2000 and December 2006.

Five patients (3 Caucasian males, 1 Hispanic female, and 1 African female), mean age 41.1 years, (range 18 months to 75 years) with orbital or adnexal Rosai-Dorfman disease were seen during the study period. Four of the patients had orbital involvement and one had eyelid involvement. Presenting features were proptosis (4 patients), diplopia (1 patient), epiphora (1 patient), and eyelid thickening (1 patient). Three of the patients with orbital involvement also had adjacent paranasal sinus involvement, and the nasolacrimal duct was involved in one patient. The patient with eyelid involvement had evidence of cutaneous R-D disease elsewhere in the body. The follow-up period (since initial diagnosis of R-D disease) ranged from 1 month to 15 years, and 2 of the patients had a history of recurrent growth despite treatment. Surgical debulking was employed in 2 patients with good results.

Orbital and adnexal Rosai-Dorfman disease is a condition with protean manifestations that may show indolent but unremitting growth despite treatment. The disease may remain extranodal and localized for many years. Adjacent paranasal sinus involvement is commonly seen in conjunction with orbital disease, simulating midline destructive lesions. Surgical debulking gives good results in patients with functional or significant cosmetic problems.