Case of the Month | October 2023

Case of the Month
October 31, 2023

The Case

The patient was a 60-year-old man who presented with a history of a sudden, painless loss of vision with some photopsias in the right eye for two weeks. The past medical history was remarkable for hypertension and a spider bite on a finger in 2011. The visual acuity was 20/200 OD and 20/20-1 OS. Anterior segment examination was remarkable for mild nuclear sclerosis in each eye. The posterior segment examination in the right eye was remarkable for a few vitreous cells and a light yellow, oval lesion superotemporal to the disc involving the central macula as well as yellowish spots above the disc. The posterior segment of the left eye was unremarkable. The visual acuity was 20/200 in the right eye, which improved to 20/30 six weeks after initiation of treatment. The visual acuity in the right eye was 20/20 one year after treatment, and clinically the was mild RPE mottling in the right macula. What was the most likely diagnosis?

The initial fundus photograph of the right eye reveals a deep yellowish, placoid lesion, and the fundus photograph three weeks later shows significant enlargement. The area of involvement shows hyperautofluorescence, indicating stress on the retinal pigment epithelium with tiny spots of more intense hyperautofluorescence. Fluorescein angiography at initial presentation shows early hyperfluorescence indicating loss of RPE pigmentation as well as tiny spots of hyper- and hypofluorescence. The late-phase picture shows well-defined enhanced hyperfluorescence at the edge of the lesion, indicating uptake of fluorescein dye by inflamed RPE at the border of the lesion.

The different diagnosis includes the following:

Acute posterior multifocal placoid pigment epitheliopathy. APMPPE typically presents as an acute loss of vision with multifocal placoid lesions following a flu-like illness. It is usually bilateral, and it typically has an age of onset between 16-40. Our patient had a single, large, oval lesion, which is atypical of APMPPE. In contrast to our patient, APMPPE patients have early hypofluorescence on fluorescein angiography followed by late hyperfluorescence. Patients with APMPPE often recover vision, but there can be vision loss due to RPE atrophy or choroidal neovascular membrane formation.

Serpiginous choroiditis. This is a progressive, much more destructive chorioretinitis in which there are creamy deep deposits followed by atrophy and, if the central macula is involved, significant visual loss.

Acute zonal occult outer retinopathy. AZOOR is a rare condition that typically occurs in young or middle-aged women and presents with photopsias and progressive visual field loss in one or both eyes. Though gradual improvement sometimes occurs, patients are left with pigmentary atrophy and persistent visual field defects.

Syphilitic outer retinopathy. Syphilis, which has a range of chorioretinal manifestations, can present as a placoid outer retinopathy, as in our patient. Our patient had a history of sex with women and men. He did not have a rash on his palms, but there was a recent history of significant peeling of skin on his feet. He had a positive RPR and FTA-Abs. Following a spinal tap, the details of which we do not have, he was treated for neurosyphilis, and the placoid lesion resolved with restoration of vision.

Amaral C, Joy L, Jimenez H, et al. Syphilitic outer retinopathy: A case report and review of the literature. J VitreoRetinal Dis 2022;6:63-70.

Soares RR, Pandit RR, Dunn JP. Acute syphilitic placoid posterior chorioretinopathy. Ophthalmol Retina 2019;3:1090.

Case Photos

Click the Images below to enlarge
Photo OD 08-26-22
Photo OS 08-26-22
Autofluorescence OD 08-26-22
Autofluorescence OD 09-13-22
Photo OD 09-13-22
FA OD 08-26-22 0:32
FA OD 08-26-22 7:10
FA OS 09-13-22 06:22
Photo OD 10-10-23

The initial fundus photograph of the right eye reveals a deep yellowish, placoid lesion, and the fundus photograph three weeks later shows significant enlargement. The area of involvement shows hyperautofluorescence, indicating stress on the retinal pigment epithelium with tiny spots of more intense hyperautofluorescence. Fluorescein angiography at initial presentation shows early hyperfluorescence indicating loss of RPE pigmentation as well as tiny spots of hyper- and hypofluorescence. The late-phase picture shows well-defined enhanced hyperfluorescence at the edge of the lesion, indicating uptake of fluorescein dye by inflamed RPE at the border of the lesion.

The different diagnosis includes the following:

Acute posterior multifocal placoid pigment epitheliopathy. APMPPE typically presents as an acute loss of vision with multifocal placoid lesions following a flu-like illness. It is usually bilateral, and it typically has an age of onset between 16-40. Our patient had a single, large, oval lesion, which is atypical of APMPPE. In contrast to our patient, APMPPE patients have early hypofluorescence on fluorescein angiography followed by late hyperfluorescence. Patients with APMPPE often recover vision, but there can be vision loss due to RPE atrophy or choroidal neovascular membrane formation.

Serpiginous choroiditis. This is a progressive, much more destructive chorioretinitis in which there are creamy deep deposits followed by atrophy and, if the central macula is involved, significant visual loss.

Acute zonal occult outer retinopathy. AZOOR is a rare condition that typically occurs in young or middle-aged women and presents with photopsias and progressive visual field loss in one or both eyes. Though gradual improvement sometimes occurs, patients are left with pigmentary atrophy and persistent visual field defects.

Syphilitic outer retinopathy. Syphilis, which has a range of chorioretinal manifestations, can present as a placoid outer retinopathy, as in our patient. Our patient had a history of sex with women and men. He did not have a rash on his palms, but there was a recent history of significant peeling of skin on his feet. He had a positive RPR and FTA-Abs. Following a spinal tap, the details of which we do not have, he was treated for neurosyphilis, and the placoid lesion resolved with restoration of vision.

Amaral C, Joy L, Jimenez H, et al. Syphilitic outer retinopathy: A case report and review of the literature. J VitreoRetinal Dis 2022;6:63-70.

Soares RR, Pandit RR, Dunn JP. Acute syphilitic placoid posterior chorioretinopathy. Ophthalmol Retina 2019;3:1090.

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