Pediatric orbital Rosai-Dorfman disease: An unusual case].

Rosai-Dorfman disease (RDD) is a sinus histiocytosis with massive lymphadenopathy. This rare condition is a benign disease of unknown etiology. Bilateral orbital localization of RDD is rare. The authors report the case of a 6-year-old child who presented with bilateral orbital-palpebral masses associated with chronic cervical lymphadenopathy. There were no laboratory signs of inflammation. Serological and tuberculosis screening tests were negative. Histopathological examination of a lymph node biopsy established a diagnosis of Rosai-Dorfman disease. The patient underwent surgical excision of the orbital lesions followed by long-term corticosteroid therapy. A favorable course was observed, with no sign of recurrence after one year of follow-up. Rosai-Dorfman disease is very rare in its bilateral orbital presentation. Histopathological diagnosis remains challenging. In Africa, the presence of chronic oculo-palpebral tumor associated with or without cervical lymphadenopathy must raise the suspicion of Rosai-Dorfman disease after ruling out tuberculosis and lymphoma. Copyright © 2024 Elsevier Masson SAS. All rights reserved.

Citation

B Wade, C A Lame, M N Ndiaye Sow, A Ndiaye, F Diédhiou, M Diakhaté, E L H S Sarr, S M Seck, A D Faye, N N Gueye. Pediatric orbital Rosai-Dorfman disease: An unusual case]. Journal francais d'ophtalmologie. 2024 Feb;47(2):104070

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PMID: 38310692

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