|Year : 2019 | Volume
| Issue : 1 | Page : 1-6
Inflammatory chorioretinopathies (White Dot Syndromes), diagnosis and management: A review of the literature
Bassey Fiebai, Safinatu Wada Mohammed
Department of Ophthalmology, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria
|Date of Submission||04-Nov-2018|
|Date of Acceptance||13-Feb-2019|
|Date of Web Publication||14-Jun-2019|
Department of Ophthalmology, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State
Source of Support: None, Conflict of Interest: None
Background: The white dot syndromes or inflammatory chorioretinopathies are a heterogenous group of diseases of unknown aetiology, characterized by the appearance of white dots on the fundus. These group of disorders include, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis, multiple evanescent white dot syndrome (MEWDS), multifocal choroiditis and panuveitis (MCP), punctate inner choroidopathy (PIC), and diffuse subretinal fibrosis (DSF). They appear to have similar modes of presentation, but subtle differences noted help in their diagnosis coupled with and imaging techniques aids in the management of these disorders.
Aim: This study aims to review relevant literature available on inflammatory chorioretinopathies, their diagnosis and management.
Methods: Review of pertinent literature and available publications using the terms 'White Dot Syndrome (WDS)', 'inflammatory chorioretinopathies' acute multifocal placoid punctate epitheliopathy, birdshot chorioretinopathy, serpiginous choroiditis, multifocal choroiditis and panuveitis and punctate inner choroiditis were sought for using a comprehensive literature search of PubMed and MEDLINE. All relevant articles, full length and abstract that had information on clinical presentations, investigations and available treatment modalities were included. Additional papers were also selected from reference lists of papers identified by the electronic database search.
Results: Reviewed information shows that the WDS though similar in presentation are still considered to be separate disease entities and not really a spectrum of the same disease as some postulate. Most are self-limiting and visual prognosis is generally good. Newer treatment modalities uncovered in this review include the use of intravitreal anti-vascular endothelial growth factors in the treatment of sight-threatening complications such as choroidal neovascularisation.
Conclusion: This article has reviewed inflammatory chorioretinopathies or WDS as reported in literature over 4 decades. An appreciable data exist and reviewed information reveals that WDS are a heterogeneous group of disorders with similar aetiology and modes of presentation but with some subtle distinct characteristics. Further studies on predictors of foveal involvement would inform what prophylactic treatments maybe beneficiary in preventing visual loss.
Keywords: Birdshot chorioretinopathy, inflammatory chorioretinopathies, White Dot Syndrome
|How to cite this article:|
Fiebai B, Mohammed SW. Inflammatory chorioretinopathies (White Dot Syndromes), diagnosis and management: A review of the literature. Port Harcourt Med J 2019;13:1-6
|How to cite this URL:|
Fiebai B, Mohammed SW. Inflammatory chorioretinopathies (White Dot Syndromes), diagnosis and management: A review of the literature. Port Harcourt Med J [serial online] 2019 [cited 2019 Nov 21];13:1-6. Available from: http://www.phmj.org/text.asp?2019/13/1/1/260228
| Introduction|| |
The White Dot Syndromes (WDS) or inflammatory chorioretinopathies are a heterogeneous group of diseases of unknown aetiology.
They are characterised by the appearance of white dots on the fundus.
These group of disorders include acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), multiple evanescent WDS (MEWDS), Birdshot chorioretinopathy (BSC), multifocal choroiditis and panuveitis (MCP), punctate inner choroidopathy (PIC) and diffuse subretinal fibrosis.,
Most are believed to be autoimmune in origin and are seen in individuals below the age of 50 years except in BSC and serpiginous choroidopathy. Others are thought to be infectious in origin as they are associated with a viral prodrome [Figure 1].
A female preponderance is seen in BSC, MEWDS, MCP and PIC.
With the exception of MEWDS, the presentation is usually bilateral and asymmetric.
An update in the literature on WDS is reviewed in this article, emphasising on the diagnosis and current trends in management.
| Methods|| |
Available publications from 1968 to 2017 (49 years) using the terms 'WDS', 'inflammatory chorioretinopathies' acute multifocal placoid punctate epitheliopathy, birdshot choroidopathy, SC, MCP and punctate inner choroiditis were sought for using a comprehensive literature search of PubMed and MEDLINE. All relevant articles, full length and abstract that had information on clinical presentations, investigations and available treatment modalities were included. Additional papers were also selected from reference lists of papers identified by the electronic database search.
| Results|| |
Reviewed information shows that the WDS though similar in presentation are still considered to be separate disease entities and not really a spectrum of the same disease as some postulate. Most are self-limiting, and visual prognosis is generally good except when there is foveal involvement. Newer treatment modalities uncovered in this review include the use of intravitreal anti-vascular endothelial growth factors (Anti-Vegfs) in the treatment of sight-threatening complications such as choroidal neovascularisation.
| Discussion|| |
WDS are a rare group of idiopathic multifocal inflammatory conditions involving the choroid and retina, characterised by appearance of white dots on the fundus.,,,,, These entities have overlapping features with most presenting in an acute way but with minimal or no permanent visual impairment in the long term. Some have postulated that WDS is a clinical spectrum of the same disease entity rather than separate disease entities.
Acute posterior multifocal placoid pigment epitheliopathy
APMPEE is a rare inflammatory condition that occurs in young healthy adults. Aetiology is unknown but it has been associated with human leucocyte antigen-B7 (HLA-B7) and HLA-DR2. Patients usually have a viral prodrome and the disease has also been linked to adenovirus. Other infections also reported in association with APMPEE including Lyme disease and psoriasis. A community-based population study found it to be more common in men.
Its presentation is usually bilateral but asymmetric, starting in one eye first then the second one within a few days. It is still unclear if APMPEE is primarily a disease of the retina pigment epithelium (RPE) or an abnormality in the choroid/choriocapillary perfusion.
Fundus examination reveals multifocal placoid yellowish-white lesions at the level of the RPE and choroid from the posterior pole to the equator, varying in size from 1 to 2 disc areas [Figure 2]. Lesions resolve over 2–6 weeks leaving an area of RPE alteration of depigmentation and clumping.
Fluorescein angiography usually reveals lobular hypofluorescence in the early frames and even diffuse staining in the same areas in the late phase.
APMPPE is usually self-limiting with a generally good long-term prognosis. However, some studies have reported poor visual prognosis in older patients, likely from failure of the RPE to recover from the inflammatory insult resulting in atrophy of the RPE and overlying receptors.,,,
There is no evidence that corticosteroids or any other medications are beneficial; however, systemic steroids may be used as well as intravitreal Anti-Vegfs, if there is foveal involvement.
SC is also known as geographic helicoid peripapillary choroidopathy. It occurs in the second to seventh decade of life and has an equal sex distribution.,,
The aetiology is unknown but has been associated with immune-related occlusive vasculitis and HLA B7.,, It has also been linked with herpes viruses although the evidence is inconclusive.
The presentation is usually unilateral and characterised by painless visual loss associated with photopsia and scotomata., Anterior uveitis and vitritis are mild.
Fundus examination shows asymmetric bilateral lesions seen at the level of the inner choroid or RPE projecting in a geographic or polypoidal manner in the posterior pole.,,, Active lesions may occur in more than one area, and skipped areas are common. Macular serpiginous or peripheral SC is less common., The disease typically starts from the optic nerve and progresses in a centrifugal pattern. Visual loss is usually as a result of the development of choroidal neovascular membrane (CNVM) or macular involvement.,,
Early frames of fundus fluorescein angiogram show blockage of the choroidal flush and staining of the active edge in the late phase.,, However, early hyperfluorescence with late leakage reveals the presence of CNVM. Indocyanine green shows hyperfluorescence in all phases of the study for active and old lesions., Fundus autofluorescence aids not only in detecting RPE damage but also in following the clinical course of the disease as areas of regressed disease activity show hypo- and hyper-autofluorescence in areas with newer active lesions.
Treatment is usually with corticosteroids which could be periocular, systemic or intravitreal. However, these are not usually effective for long-term remission and immunomodulators such as azathioprine and cyclophosphamide, and other biological modulators such as cyclosporin have been used., CNVM is treated with Anti-Vegfs or application of laser.
Multiple evanescent White Dot Syndrome
This WDS is predominantly seen in young adult females usually preceded by a viral-like illness.,,
Presentation is with a painless monocular blurring of vision with associated photopsia and mild vitritis. Lesions are usually at the level of the outer neurosensory retina and retinal pigment epithelium. Numerous small (100–300 micrometer) grey white patches sparing the fovea are seen in the posterior pole [Figure 3]. They may be replaced by mild pigment mottling. Foveal granularity is common and optic disc oedema is occasionally present.
Fluorescein angiography shows early punctate hyperfluorescence in wreath-like configuration with late staining. Electroretinography shows reduced a-waves and reduced early receptor potential amplitudes that would suggest a primary involvement of the outer segments of photoreceptors., Variable visual field changes have been reported with the most common being an enlargement of the blind spot; others are temporal or paracentral scotoma., Optical coherence tomography may show inner segment/outer segment disruption.,,
Treatment is usually not required as the disorder is self-limiting and spontaneously resolves with good prognosis in uncomplicated cases as most eyes improve to 6/9 or better within 2–3 months.,
If complicated with a CNVM, Anti-Vegfs or laser photocoagulation can be given.
BSC is also known as vitiliginous choroiditis. It is a rare idiopathic autoimmune intraocular inflammatory condition, commonly seen in the fourth and fifth decades of life in females.,, BSC has an unknown aetiology but associated with HLA-A29.,
The presentation is with an insidious onset of impaired central vision associated with floaters and photopsia., There is usually mild anterior uveitis with moderate vitritis and retinal vascular leakage., The fundus shows typical multiple hypopigmented coloured lesions at the level of the retinal pigment epithelium or deeper scattered throughout the fundus [Figure 4]. Other features include cystoid macular oedema and disc oedema.,,
Fundus fluorescein angiogram is useful in demonstrating the presence of macular oedema and retina vascular leakage.
HLA typing aids in the diagnosis.
Treatment includes the use of systemic steroids, immunosuppressants and cyclosporine.,
Multifocal choroiditis and panuveitis
This condition is an intraocular inflammation characterised by panuveitis of unknown aetiology with retinochoroidal lesions., It is commonly seen between the second and sixth decades and occurs more commonly in females than males., It is a chronic disorder with multiple recurrences.
The presentation is usually bilateral with an acute onset of blurred vision associated with floaters and photopsia., There is usually marked anterior uveitis and vitritis. Fundus examination reveals multiple yellow or grey lesion at the level of choroid and RPE which are mainly located at the mid periphery [Figure 5]. These active lesions may become atrophic with pigmentation with chronicity., Linear chorioretinal streaks may be seen in the fundus. There may also be swelling or hyperaemia of the optic nerve head, cystoid macular oedema, epiretinal membrane and CNVMs which are commonly responsible for the loss of vision.,,
Visual field may reveal scotoma corresponding to areas of lesion and enlargement of blind spot. Fundus fluorescein angiogram shows early hypofluorescence and late hyperfluorescence at early presentation; however, the presence of a CNVM will show early hyperfluorescence.
Treatment involves the use of systemic steroids and immunosuppressive therapy (cyclosporine, azathioprine, methotrexate, chlorambucil and cyclophosphamide).,
MCP is a chronic disorder with multiple recurrences. Up to 66% of eyes achieve visual acuity of 20/40 or better. Those with worse visual acuity are because of the development of CNVM or cystoid macula oedema.
Punctate inner choroidopathy
PIC is a rare inflammatory ocular disease which affects mostly young women who are myopes.,, Similar to MCP, however, involvement is predominantly of the posterior pole or mid periphery with no vitritis, and patients present with blurring of vision, floaters and photopsia.,,
Fundus examination at the acute phase shows deep, yellow cylindrical lesions at the level of the RPE and inner choroid, measuring about 100–300 μm, and when healed, lesions become pigmented, punched out and manifest as atrophic scars [Figure 6].,,,,
Visual field examination shows central or paracentral scotomas. Fundus fluorescein shows filling and staining at late phase.,
Treatment is with systemic steroids for lesions involving the fovea.,
Visual prognosis is good unless there is CNVM. If CNVM is present, laser photocoagulation and photodynamic therapy can be administered.
| Conclusion|| |
This article has reviewed inflammatory chorioretinopathies or WDS as reported in literature over 4 decades. An appreciable data exist, and reviewed information reveals that WDS are a heterogeneous group of disorders with similar aetiology and modes of presentation but with some subtle distinct characteristics. Careful fundus examination backed up by multimodal imaging aids in the diagnosis. Management is based on the ability to make a good diagnosis as some are self-limiting with good prognosis such as APMPEE, MEWDS and PIC while others such as SC may develop complications which compromise vision.
Further studies on predictors of foveal involvement would inform what prophylactic treatments maybe beneficiary in preventing visual loss.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]