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

Abstract Background To provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review. Methods A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary...

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Autores principales:	Tariq A. Alzahem, Antonio Augusto Cruz, Azza M. Y. Maktabi, Fernando Chahud, Hind Alkatan
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Rosai–Dorfman disease Orbit Familial Histopathology Lymphadenopathy Ophthalmology RE1-994
Acceso en línea:	https://doaj.org/article/b68b8218af734c1f8f9d60defe58acfd
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spelling	oai:doaj.org-article:b68b8218af734c1f8f9d60defe58acfd2021-11-28T12:42:18ZOphthalmic Rosai–Dorfman disease: a multi-centre comprehensive study10.1186/s12886-021-02173-11471-2415https://doaj.org/article/b68b8218af734c1f8f9d60defe58acfd2021-11-01T00:00:00Zhttps://doi.org/10.1186/s12886-021-02173-1https://doaj.org/toc/1471-2415Abstract Background To provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review. Methods A 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. Results Eleven 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). Conclusions Ophthalmic 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.Tariq A. AlzahemAntonio Augusto CruzAzza M. Y. MaktabiFernando ChahudHind AlkatanBMCarticleRosai–Dorfman diseaseOrbitFamilialHistopathologyLymphadenopathyOphthalmologyRE1-994ENBMC Ophthalmology, Vol 21, Iss 1, Pp 1-8 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Rosai–Dorfman disease Orbit Familial Histopathology Lymphadenopathy Ophthalmology RE1-994
spellingShingle	Rosai–Dorfman disease Orbit Familial Histopathology Lymphadenopathy Ophthalmology RE1-994 Tariq A. Alzahem Antonio Augusto Cruz Azza M. Y. Maktabi Fernando Chahud Hind Alkatan Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
description	Abstract Background To provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review. Methods A 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. Results Eleven 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). Conclusions Ophthalmic 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.
format	article
author	Tariq A. Alzahem Antonio Augusto Cruz Azza M. Y. Maktabi Fernando Chahud Hind Alkatan
author_facet	Tariq A. Alzahem Antonio Augusto Cruz Azza M. Y. Maktabi Fernando Chahud Hind Alkatan
author_sort	Tariq A. Alzahem
title	Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
title_short	Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
title_full	Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
title_fullStr	Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
title_full_unstemmed	Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
title_sort	ophthalmic rosai–dorfman disease: a multi-centre comprehensive study
publisher	BMC
publishDate	2021
url	https://doaj.org/article/b68b8218af734c1f8f9d60defe58acfd
work_keys_str_mv	AT tariqaalzahem ophthalmicrosaidorfmandiseaseamulticentrecomprehensivestudy AT antonioaugustocruz ophthalmicrosaidorfmandiseaseamulticentrecomprehensivestudy AT azzamymaktabi ophthalmicrosaidorfmandiseaseamulticentrecomprehensivestudy AT fernandochahud ophthalmicrosaidorfmandiseaseamulticentrecomprehensivestudy AT hindalkatan ophthalmicrosaidorfmandiseaseamulticentrecomprehensivestudy
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Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study

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