White Dot Syndromes

White Dot Syndromes
SYMPTOMS Decreased central vision, Metamorphopsia, Visual field loss (especially an enlarged blind spot), Floaters, Flashes, Photophobia, Dyschromatopsia
SIGNS Acute Retinal Pigment Epitheliitis (ARPE/Krill Disease): Typically unilateral / Symptoms usually start 1-2 weeks before actual fundus signs / Findings in the fundus include Small hyperpigmented lesions in a yellowish halo in the macula region / The retinal lesions typically resolve in 6-12 weeks with maintenance of good vision
Multiple Evanescent White Dot Syndrome (MEWDS): Typically unilateral / Usually affects young females who are moderate myopes / Typically involves a viral prodrome / Vision can be anywhere from 20/20-20/400 / Ocular signs include a Mild APD, Mild vitritis, Mild optic nerve edema, Multiple white spots at the level of the RPE and outer retina throughout the posterior pole and can extend to the mid-periphery, Granular appearance at the fovea / Typically resolves in 7-10 weeks
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Typically bilateral / Associated with an influenza-like illness / Ocular signs include a Mild vitritis, Multiple cream-colored placoid lesions at level of the RPE and outer retina throughout the posterior pole / After 1-2 weeks, these cream-colored placoid lesions will turn into areas of RPE atrophy and hyperpigmentation
Multifocal Choroiditis and Panuveitis (MCP): Typically bilateral / Usually affects young females / Ocular signs include a Mild anterior uveitis, Mild vitritis, 50-100 micron punched out chorioretinal scars throughout the posterior pole (acute lesions are yellow-white) / Choroidal neovascular membranes and Cystoid macular edema are potential complications
Punctate Inner Choroidopathy (PIC): Typically bilateral / Usually affects young females / Findings in the fundus include Small well-defined chorioretinal scars throughout the posterior pole (acute lesions are yellow) / There should be no signs of ocular inflammation / Choroidal neovascular membranes and Cystoid macular edema are potential complications
Acute Zonal Occult Outer Retinopathy (AZOOR): Typically unilateral / Usually affects young females who are moderate myopes / Ocular signs include a Mild vitritis with a Normal fundus initially (eventually there will be areas of RPE atrophy and hyperpigmentation along with retinal arteriole narrowing) / Areas of retina with RPE atrophy and hyperpigmentation tends to correlate to location of patient's symptoms such as visual field loss and flashes / 33% of patients develop recurrence of signs and symptoms
Birdshot Retinochoroidopathy: Typically bilateral / Usually affects females / Initially, severity of symptoms outweigh severity of signs / Ocular signs include a Mild vitritis, Yellowish lesions at the level of the RPE and outer retina that are more prominent nasally and radiate from the optic nerve in a shotgun pattern, Narrowing of arterioles, Cystoid macular edema, Optic nerve pallor
Serpiginous Choroidopathy: Typically bilateral / Ocular signs include a Mild vitritis with Geographic chorioretinal scarring of the macula and peripapillary region / Choroidal neovascular membranes are a potential complication / There is a high risk of recurrence which usually occurs at the border of atrophic retinal lesions
WORK-UP Pupils, Full eye exam with dilated retinal exam, OCT analysis of the macula, OCT analysis of the optic nerve, OCT-Angiography, Visual field, Fluorescein Angiography, Indocyanine Green Angiography, Fundus Autofluorescence, Fundus photos, Infrared photos, Electrodiagnostic testing (multifocal ERG, EOG), Watzke-Allen test, Macular photostress test, Amsler grid
TREATMENT Give take home Amsler grid in order to monitor for change
Acute Retinal Pigment Epitheliitis (ARPE/Krill Disease): Needs to be monitored as no treatment is usually needed
Multiple Evanescent White Dot Syndrome (MEWDS): Needs to be monitored as no treatment is usually needed
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Refer to retinal specialist for further evaluation and treatment (systemic steroids are recommended especially if there is foveal involvement). Refer to PCP to rule out CNS vasculitis and meningitis especially if patient is having symptoms such as severe headaches
Multifocal Choroiditis and Panuveitis (MCP): Refer to retinal specialist for further evaluation and treatment (systemic steroids are recommended especially early on in the disease and intravitreal anti-VEGF injections may be needed for treatment of CME and/or CNVMs)
Punctate Inner Choroidopathy (PIC): Refer to retinal specialist for further evaluation and treatment (systemic steroids are recommended especially early on in the disease and intravitreal anti-VEGF injections may be needed for treatment of CME and/or CNVMs)
Acute Zonal Occult Outer Retinopathy (AZOOR): Needs to be monitored as no treatment has shown to be effective. Refer to PCP to rule out Hashimoto’s thyroiditis, Multiple sclerosis, Addison’s disease, Myasthenia gravis, CREST syndrome, Sjogren’s disease, and Crohn’s disease
Birdshot Retinochoroidopathy: Refer to retinal specialist for further evaluation and treatment (cyclosporine, methotrexate, intravitreal steroid injections, and intravitreal steroid implants have shown to be effective)
Serpiginous Choroidopathy: Refer to retinal specialist for further evaluation and treatment (cyclosporine and cyclophosphamide have shown to be effective and intravitreal anti-VEGF injections may be needed for treatment of CNVMs)
FOLLOW-UP Acute Retinal Pigment Epitheliitis (ARPE/Krill Disease): See patient back once a month until fully resolved
Multiple Evanescent White Dot Syndrome (MEWDS): See patient back once a month until fully resolved
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Once the patient is is treated by the retinal specialist and ocular inflammation is no longer active with a stable retina and macula, the patient should be seen back every 4-6 months
Multifocal Choroiditis and Panuveitis (MCP): Once the patient is is treated by the retinal specialist and ocular inflammation is no longer active with a stable retina and macula, the patient should be seen back every 4-6 months
Punctate Inner Choroidopathy (PIC): Once the patient is is treated by the retinal specialist and ocular inflammation is no longer active with a stable retina and macula, the patient should be seen back every 4-6 months
Acute Zonal Occult Outer Retinopathy (AZOOR): See patient back once a month until fully resolved
Birdshot Retinochoroidopathy: Once the patient is is treated by the retinal specialist and ocular inflammation is no longer active with a stable retina/macula/optic nerve, the patient should be seen back every 4-6 months
Serpiginous Choroidopathy: Once the patient is is treated by the retinal specialist and ocular inflammation is no longer active with a stable retina/macula/optic nerve, the patient should be seen back every 4-6 months
ADDITIONAL LAB | TESTS Acute Retinal Pigment Epitheliitis (ARPE/Krill Disease): None
Multiple Evanescent White Dot Syndrome (MEWDS): None
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Neurological work-up with neuroimaging
Multifocal Choroiditis and Panuveitis (MCP): None
Punctate Inner Choroidopathy (PIC): None
Acute Zonal Occult Outer Retinopathy (AZOOR): Thyroid panel, Thyroid peroxidase antibodies, Thyroglobulin antibodies, CBC with differential, Renal function tests, Liver function tests, Erythrocyte sedimentation rate, C-reactive protein, Lipid panel, Viral serology, ANA, Comprehensive metabolic panel, Anticentromere antibodies, SS-A antibodies, SS-B antibodies, Rapid ACTH stimulation test, Tensilon test, Single-fiber electromyography, MRI of the brain with and without contrast, CT of the abdomen, Colonoscopy, Biopsy of salivary glands
Birdshot Retinochoroidopathy: HLA-A29
Serpiginous Choroidopathy: HLA-B7
ETIOLOGY Inflammatory chorioretinopathies of unknown etiology
DIFFERENTIAL DX Presumed ocular histoplasmosis, Ocular toxoplamosis, Ocular toxocariasis, Ocular syphillis
NOTES White dot syndromes typically affect young, healthy adults
White Dot Syndromes: Fundus photo demonstrating multiple evanescent white dot syndrome (MEWDS) https://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/MEWDS/index.htm
Posted in Ocular Conditions, Retina-Inflammatory