Multi-modal imaging and anatomic classification of the white dot syndromes

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Abstract

The white dot syndromes (WDS) are a diverse group of posterior uveitidies that share similar clinical findings but are unique from one another. Multimodal imaging has allowed us to better understand the morphology, the activity and age of lesions, and whether there is CNV associated with these different ocular pathologies. The “white dot syndromes” and their uveitic masqueraders can now be anatomically categorized based on lesion localization. The categories include local uveitic syndromes with choroidal pathology, systemic uveitic syndromes with choroidal pathology, and multifocal choroiditis with outer retinal/choriocapillaris pathology with uveitis and without uveitis. Neoplastic and infectious etiologies are also discussed given their ability to masquerade as WDS.

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Most cited references 123

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Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group symposium.

Nathalie Cassoux, J. Rubenstein, Sarah E Coupland … (2010)

Primary vitreoretinal lymphoma (PVRL), also known as primary intraocular lymphoma, is a rare malignancy typically classified as a diffuse large B-cell lymphoma and most frequently develops in elderly populations. PVRL commonly masquerades as posterior uveitis and has a unique tropism for the retina and central nervous system (CNS). Over 15% of primary CNS lymphoma patients develop intraocular lymphoma, usually occurring in the retina and/or vitreous. Conversely, 65%-90% of PVRL patients develop CNS lymphoma. Consequently, PVRL is often fatal because of ultimate CNS association. Current PVRL animal models are limited and require further development. Typical clinical findings include vitreous cellular infiltration (lymphoma and inflammatory cells) and subretinal tumor infiltration as determined using dilated fundoscopy, fluorescent angiography, and optical coherent tomography. Currently, PVRL is most often diagnosed using both histology to identify lymphoma cells in the vitreous or retina and immunohistochemistry to indicate monoclonality. Additional adjuncts in diagnosing PVRL exist, including elevation of interleukin-10 levels in ocular fluids and detection of Ig(H) or T-cell receptor gene rearrangements in malignant cells. The optimal therapy for PVRL is not defined and requires the combined effort of oncologists and ophthalmologists. PVRL is sensitive to radiation therapy and exhibits high responsiveness to intravitreal methotrexate or rituximab. Although systemic chemotherapy alone can result in high response rates in patients with PVRL, there is a high relapse rate. Because of the disease rarity, international, multicenter, collaborative efforts are required to better understand the biology and pathogenesis of PVRL as well as to define both diagnostic markers and optimal therapies.

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Vogt-Koyanagi-Harada syndrome.

R S Moorthy, H Inomata, N A Rao (2015)

The Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. These manifestations are variable and race dependent. This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. On histopathologic examination typical cases show nonnecrotizing diffuse granulomatous panuveitis with initial sparing and late involvement of the choriocapillaris and formation of Dalen-Fuchs' nodules. Fluorescein angiography, lumbar puncture, and echography are useful adjuncts in the diagnosis and management of VKH syndrome. Patients with this syndrome are treated generally with high dose systemic corticosteroids or, when necessary, with cyclosporine or cytotoxic agents. The prognosis of patients with VKH syndrome is fair, with nearly 60% of patients retaining vision of 20/30 or better. The complications of VKH syndrome that lead to visual loss include cataracts in about 25% of patients, glaucoma in 33%, and subretinal neovascular membranes (SRNVMs) in about 10%; the latter, however, are an important cause of late visual loss. These complications usually require medical and/or surgical intervention, including photocoagulation. The major risk factor for the development of cataracts, SRNVMs, and, to some extent, glaucoma, is chronic recurrent intraocular inflammation that may be resistant to corticosteroid therapy. It appears that initial treatment with high dose corticosteroids, combined with prolonged corticosteroid therapy at appropriate dosage, may minimize these complications and may improve visual prognosis.

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Presumed tubercular serpiginouslike choroiditis: clinical presentations and management.

Vishali Gupta, Amod Gupta, Sunil Arora … (2003)

Choroiditis, choroidal tubercles, and tuberculomas are well known ocular manifestations of systemic tuberculosis. The present series aimed to report the occurrence of serpiginouslike choroiditis of presumed tubercular origin. Retrospective, noncomparative, interventional case series. Eleven eyes in seven consecutive patients with a diagnosis of choroidal tuberculosis simulating serpiginous choroiditis were studied between 1997 and 2000. TESTING AND INTERVENTION: All patients had their fundus photographs taken at the time of initial presentation as well as during follow-up. All patients underwent a Mantoux skin test and chest radiography. In addition, five patients had their aqueous or vitreous humor subjected to polymerase chain reaction (PCR) for Mycobacterium tuberculosis. Sputum examination, biopsy, or both were carried out whenever recommended by the pulmonologist. Systemic antituberculosis chemotherapy was instituted in combination with treatment for ocular inflammation. Therapeutic response and visual improvement. There were five men and two women ranging in age from 17 to 32 years. Clinical presentations included three morphologic variants; multifocal progressive choroiditis showing wavelike progression to confluent, diffuse lesions resembling serpiginous choroiditis (three eyes); diffuse choroiditis characterized by diffuse plaquelike choroiditis with an amoeboid pattern suggestive of serpiginous choroiditis at initial presentation (four eyes); and mixed variety where opposite eyes had mixed features (four eyes). All patients had strongly positive Mantoux skin test results and positive chest radiograph results. The PCR results from aqueous and vitreous humor in four samples was positive for Mycobacterium tuberculosis; one had sputum positive for acid-fast bacilli, whereas two had histopathologic evidence of tuberculosis from cervical or parahilar lymph nodes. Treatment was associated with resolution of choroidal lesions and visual improvement. Final visual acuity of 20/30 or better was achieved in five eyes. Choroidal tuberculosis may present as multifocal progressive or diffuse choroiditis resembling serpiginous choroiditis. It is important to recognize these presentations because these eyes show good response to systemic antituberculosis chemotherapy.

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Author and article information

Contributors

Meisha L. Raven: mraven@wisc.edu

Alexander L. Ringeisen: aringeisen@gmail.com

Yoshihiro Yonekawa: yonekawa@gmail.com

Maxwell S. Stem: maxstem@gmail.com

Lisa J. Faia: lfaia@arcpc.net

Justin L. Gottlieb: (608) 263-7290 , jlgottlieb@wisc.edu

Journal

Journal ID (nlm-ta): Int J Retina Vitreous

Journal ID (iso-abbrev): Int J Retina Vitreous

Title: International Journal of Retina and Vitreous

Publisher: BioMed Central (London )

ISSN (Electronic): 2056-9920

Publication date (Electronic): 20 March 2017

Publication date PMC-release: 20 March 2017

Publication date Collection: 2017

Volume: 3

Electronic Location Identifier: 12

Affiliations

[1 ]ISNI 0000 0001 0701 8607, GRID grid.28803.31, Department of Ophthalmology and Visual Sciences, , University of Wisconsin, ; 600 Highland Ave, Madison, WI 53705 USA

[2 ]McPherson Eye Research Institute, Madison, WI USA

[3 ]ISNI 0000 0004 0435 1924, GRID grid.417118.a, Associated Retinal Consultants, , William Beaumont Hospital, ; Royal Oak, MI USA

[4 ]ISNI 0000 0001 0701 8607, GRID grid.28803.31, Department of Ophthalmology and Visual Sciences, , University of Wisconsin, ; 2870 University Ave, Room 206, Madison, WI 53705 USA

Article

Publisher ID: 69

DOI: 10.1186/s40942-017-0069-8

PMC ID: 5357819

PubMed ID: 28331634

SO-VID: 4637c857-daae-4e8b-a39a-77c96fd43459

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 17 June 2016

Date accepted : 27 February 2017

Funding

Funded by: Unrestricted Grant from Research to Prevent Blindness, Inc

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Keywords: white dot syndromes,birdshot chorioretinopathy,multiple evanescent white dot syndrome,acute posterior multifocal placoid pigment epitheliopathy,multifocal choroiditis with panuveitis,serpiginous choroiditis,relentless placoid chorioretinitis,punctate inner choroidopathy,sympathetic ophthalmia,vogt–koyanagi–harada disease

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Keywords: white dot syndromes, birdshot chorioretinopathy, multiple evanescent white dot syndrome, acute posterior multifocal placoid pigment epitheliopathy, multifocal choroiditis with panuveitis, serpiginous choroiditis, relentless placoid chorioretinitis, punctate inner choroidopathy, sympathetic ophthalmia, vogt–koyanagi–harada disease

Multi-modal imaging and anatomic classification of the white dot syndromes

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Most cited references 123

Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group symposium.

Vogt-Koyanagi-Harada syndrome.

Presumed tubercular serpiginouslike choroiditis: clinical presentations and management.

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