Case of the Month | March 2021

Case of the Month
March 30, 2021

The Case

The patient was a 64-year-old woman who first presented our practice 15 years ago with a choroidal neovascular membrane in the left eye that was successfully treated with Avastin.  Five years ago, she was successfully treated with Lucentis for a recurrent CNVM in the left eye. The fundus photos and OCTs are from a recent vision, when her vision was 20/40 OD and 20/30 OS.

What is the most likely diagnosis?

A. Macular degeneration

B. Sarcoidosis

C. Vitiliginous chorioretinitis (a.k.a. birdshot retinochoroidopathy)

D. Myopic maculopathy

C. Vitiliginous chorioretinitis (birdshot retinochoroidopathy)

This condition is characterized by bilateral, largely symmetrical round or oval spots in post-equatorial the fundus that tend to be somewhat oval in shape and point toward the disc. The spots are orange or yellow ill-defined patches initially when involving the choroid. Eventual atrophy of the overlying RPE atrophy results in depigmented spots. The lack of hyperpigmentation is remarkable and distinguishes this condition from most chorioretinopathies. Our patient manifested focal areas of disruption of the ellipsoid and interdigitation zones and focal atrophy.

Patients typically present in the 5th to 7th decade of life with floaters, photopsias, and blurred vision. Later, night blindness and color blindness often develop. Patients can have macular edema and disc edema, narrowed retinal blood vessels, and mild disc pallor. Though the disease is progressive, most patients retain good central vision in at least one eye. A distinguishing feature is a strong association with HLA-A29. Given the prevalence of this antigen, a positive HLA-A29 does not confirm a diagnosis of vitiliginous chorioretinitis, but a negative HLA-A29 effectively rules it out. There have been rare reports of associations with other conditions such as hearing loss or Lyme disease, but these might be coincidental.

Patients can experience central visual loss of atrophy, cystoid macular edema, and choroidal neovascularization. Our patient had a choroidal neovascular membrane in the left eye treated twice with anti-VEGF agents.

Vitiliginous choroidopathy has periods of quiescence and exacerbation. Treatment includes corticosteroids with or without other immunosuppressives such as methotrexate, mycophenolate, or cyclosporine.

Case Photos

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C. Vitiliginous chorioretinitis (birdshot retinochoroidopathy)

This condition is characterized by bilateral, largely symmetrical round or oval spots in post-equatorial the fundus that tend to be somewhat oval in shape and point toward the disc. The spots are orange or yellow ill-defined patches initially when involving the choroid. Eventual atrophy of the overlying RPE atrophy results in depigmented spots. The lack of hyperpigmentation is remarkable and distinguishes this condition from most chorioretinopathies. Our patient manifested focal areas of disruption of the ellipsoid and interdigitation zones and focal atrophy.

Patients typically present in the 5th to 7th decade of life with floaters, photopsias, and blurred vision. Later, night blindness and color blindness often develop. Patients can have macular edema and disc edema, narrowed retinal blood vessels, and mild disc pallor. Though the disease is progressive, most patients retain good central vision in at least one eye. A distinguishing feature is a strong association with HLA-A29. Given the prevalence of this antigen, a positive HLA-A29 does not confirm a diagnosis of vitiliginous chorioretinitis, but a negative HLA-A29 effectively rules it out. There have been rare reports of associations with other conditions such as hearing loss or Lyme disease, but these might be coincidental.

Patients can experience central visual loss of atrophy, cystoid macular edema, and choroidal neovascularization. Our patient had a choroidal neovascular membrane in the left eye treated twice with anti-VEGF agents.

Vitiliginous choroidopathy has periods of quiescence and exacerbation. Treatment includes corticosteroids with or without other immunosuppressives such as methotrexate, mycophenolate, or cyclosporine.

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