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A 39-year-old woman with unilateral metamorphosias
Digital Journal of Ophthalmology 2011
Volume 17, Number 4
November 20, 2011
DOI: 10.5693/djo.03.2011.11.001
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Lígia Ribeiro | Department of Ophthalmology, Centro Hospitalar Vila Nova de Gaia, Portugal
Sidnei Barge | Department of Ophthalmology, Centro Hospitalar Vila Nova de Gaia, Portugal
Luís Silva | Department of Ophthalmology, Centro Hospitalar Vila Nova de Gaia, Portugal
Arnaldo Brandão | Department of Ophthalmology, Centro Hospitalar Vila Nova de Gaia, Portugal
Dália Meira | Department of Ophthalmology, Centro Hospitalar Vila Nova de Gaia, Portugal
Differential Diagnosis
The differential diagnosis for inflammatory choroidal neovascularization includes infectious (toxoplasmosis, tuberculosis, and Lyme disease) and inflammatory etiologies (sarcoidosis and inflammatory multifocal chorioretinopathies).(1) Our patient underwent extensive evaluation to define the underlying etiology. Her work-up was negative for infectious and inflammatory conditions.

Inflammatory chorioretinopathies are often difficult to differentiate. Punctate inner choroidopathy (PIC), multifocal choroiditis and panuveitis (MCP), and presumed ocular histoplasmosis syndrome (POHS) are the subtypes most frequently associated with choroidal neovascularization; however, any other form of posterior uveitis, for instance, acute posterior multifocal placoid pigment epitheliopathy, birdshot retinochoroidopathy, multiple evanescent white dot syndrome, Vogt-Koyanagi-Harada syndrome, and sympathetic ophthalmia, can be complicated by choroidal neovascularization.(1)

PIC is an idiopathic inflammatory disorder that typically occurs in young (15-55 years), white, myopic women. Presenting symptoms are blurred vision and scotomas with or without flashes of light.(2) The symptoms are usually unilateral, but most patients show bilateral fundus involvement.(2,3) PIC is characterized by multifocal choroidal lesions (yellow-white lesions of the inner choroid and retinal pigment epithelium of approximately 100–300 ìm in size), usually distributed throughout the posterior pole but sparing the peripapillary region.(2,4) There are no other signs of ocular inflammation elsewhere in the eye. Visual prognosis is generally good since the lesions usually evolve into atrophic scars; however, choroidal neovascularization may develop in 17% to 40% of patients, potentially threatening central vision if untreated.(4-7)

MCP is an inflammatory disease that occurs in healthy young patients in their fourth decade, predominantly in white women. MCP is characterized by the presence of multiple small, yellow-gray choroidal lesions that, as the disease becomes inactive, are replaced by well-demarcated chorioretinal scars.(4) Patients with MCP typically have inflammatory cells in the anterior chamber or vitreous cavity at the time of presentation.(1) MCP may be complicated by choroidal neovascularization in 32% to 46% of the patients.(1) Choroidal neovascularization can occur more frequently in inflamed areas, although it may originate from an old chorioretinal scar.(4)

POHS is a bilateral immune reaction to an infectious agent, Histoplasma capsulatum, that affects the choroid and retina. It usually occurs in young patients. Men and women are equally affected. POHS is characterized by discrete atrophic choroidal scars in the macula and midperiphery associated with peripapillary atrophy and choroidal neovascularization—the classical triad of this disease (1). This triad is accompanied by complete absence of any signs of anterior chamber or vitreal inflammation. Choroidal neovascularization is associated with inactive choroidal scars (“histo-spots”); they do not demonstrate active inflammation.(1,4) The primary cause of visual impairment in POHS is the occurrence of choroidal neovascularization in the macula, which results in exudation and subsequent scarring.
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