Ophthalmic Rosai\u2013Dorfman disease: a multi-centre comprehensive study

Tariq A Alzahem,Fernando Chahud,Azza M Y Maktabi,Hind Alkatan,Antonio Augusto Cruz

doi:10.1186/s12886-021-02173-1

Abstract

BackgroundTo provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review.MethodsA multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018.ResultsEleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6–72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD).ConclusionsOphthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.

Highlights

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder first described by the French pathologist Destombes in 1965 and characterized by Juan Rosai and Ronald Dorfman in 1969 [1, 2]
Denoted as sinus histiocytosis with massive lymphadenopathy, this name was replaced by Rosai–Dorfman disease (RDD) due to the variety of extra-nodal disease signs and occasional lack of lymphadenopathy [3]
All diagnoses were established by tissue biopsy performed at the study centres. This multi-centre, retrospective, noncomparative study was approved by the Human Ethics Committee/Institutional Review Board at King Khaled Eye Specialist Hospital (KKESH) with a collaborative agreement among KKESH, King Abdulaziz University Hospital (KAUH) and HCFMRP which adheres to the tenets of the Declaration of Helsinki

Summary

Introduction

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder first described by the French pathologist Destombes in 1965 and characterized by Juan Rosai and Ronald Dorfman in 1969 [1, 2]. Denoted as sinus histiocytosis with massive lymphadenopathy, this name was replaced by RDD due to the variety of extra-nodal disease signs and occasional lack of lymphadenopathy [3]. As the previous name implies, RDD features massive, painless. Extra-nodal involvement sites in sporadic RDD have been documented in 43% of cases [6]. The skin, paranasal sinuses, bone and orbital tissues are the most frequent extra-nodal sites [6]. Central nervous system (CNS) involvement usually occurs without extracranial lesions [7]. To provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review

Methods

Results

Discussion

Conclusion