History of present illness
A 31-year-old otherwise healthy male patient with nonspecific upper respiratory illness 2 months earlier presents with blurry vision in the left eye with distortion. This has persisted for about 1 month and is associated with occasional photopsia and minimal floaters.
Ocular examination findings
Best-corrected visual acuity was 20/20 in the right eye and 20/40 in the left eye. Intraocular pressures were normal in both eyes, and slit-lamp biomicroscopy was notable for 2+ anterior vitreous cell in the left eye. Dilated fundus examination revealed creamy white lesions involving the fovea of the left eye at the level of the retinal pigment epithelium ( Fig. 33.1 ). The right eye was notable for mild vitritis and inactive pigmented epithelial scars in the macula and midperiphery.
Imaging
Optical coherence tomography (OCT) with raster through the fovea demonstrates subretinal fluid and pigment epithelial detachment. Fluorescein angiography (FA) showed early hypofluorescence and late hyperfluorescence of active lesions.
Questions to ask
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What associated systemic medical problems does the patient have? Autoimmune diseases such as sarcoidosis can cause bilateral panuveitis with inflammatory retinal changes.
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None
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Does the patient have history of sexually transmitted infections or exposure to tuberculosis (TB)? Infectious etiologies including syphilis and TB can cause ocular inflammation and placoid and subretinal granulomatous lesions, respectively.
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No
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Assessment
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This is a case of a 31-year-old male patient with no past ocular, medical, or surgical history with relapsing, remitting decreased vision involving both eyes with panocular inflammation and multifocal lesions deep to the retina.
Differential diagnosis
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Inflammatory placoid diseases
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Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)
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Serpiginous choroiditis (SC)
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Persistent placoid maculopathy
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Relentless placoid chorioretinitis (RPC)
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Other inflammatory etiologies
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Multifocal choroiditis with panuveitis (MCP)
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Sarcoidosis
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Infectious inflammatory masqueraders
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Tuberculosis (TB)
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Acute syphilitic posterior placoid chorioretinopathy
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Multimodal testing and results
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Fundus photographs
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Fundus examination reveals multiple creamy yellow to white lesions deep to the retina in the posterior pole and midperiphery with older inactive lesions demonstrating pigmentation and scarring.
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FA
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FA shows early hypofluorescence with late hyperfluorescence of active lesions. There is also staining of chorioretinal scars in the late phase with blockage from older hyperpigmented lesions.
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Indocyanine green angiography (ICG)
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ICG shows hypofluorescence in the areas corresponding to clinical lesions.
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Management
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Given the relatively good vision at presentation, the decision was made to observe and follow closely. At 2-month follow-up, the active lesions in the left eye began to pigment, and the fluid under the fovea resolved. However, the patient began to notice decreased vision in the right eye with best-corrected visual acuity dropping to 20/100. An active creamy lesion was noted in the fovea of the right eye with subretinal fluid.
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The patient was started on 60 mg of oral prednisone. However, the vision in the right eye continued to deteriorate and measured 20/400. Intravitreal triamcinolone acetonide was injected. Oral prednisone was tapered slowly by 10 mg every month.
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Six months after starting steroids, visual acuity improved to 20/50 in the right eye and 20/20 in the left eye.
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New active lesions were seen after tapering the oral steroids. Prednisone was increased to 40 mg daily, and cyclosporine 150 mg daily was added while the steroids were tapered. The prednisone was then tapered 10 mg per month while cyclosporine was maintained ( Fig. 33.2 ).