J Korean Ophthalmol Soc.  2009 Aug;50(8):1275-1281. 10.3341/jkos.2009.50.8.1275.

Best Disease With Old-age Onset Misdiagnosed as Chronic Central Serous Chorioretinopathy: A Case Report

• Cho WB
• Shin HJ
• Moon JW
• Kim HC

• KMID: 2212554
• DOI: http://doi.org/10.3341/jkos.2009.50.8.1275

Abstract

PURPOSE
To report a case of Best's disease with old-age-onset with unusual clinical features. CASE SUMMARY: A 68-year-old woman with a six-month history of using oral steroids complained of decreased vision in both eyes. Fundus examination revealed a circular area of macular elevation measuring approximately 1.5 disc diameter size in both eyes. Optical coherence tomography (OCT) showed serous retinal detachment, but pigment epithelial detachment was seen only on fluorescein angiography and indocyanine green angiography. The patient received a diagnosis of chronic central chorioretinopathy with choroidal neovascularization. Photodynamic therapy (PDT) and intravitreal bevacizumab (IVB) injections were prescribed as treatment, but were ineffective. For a definitive diagnosis, we performed an electro-oculogram (EOG) and the result was abnormal with an Arden ratio below 1.5 in both eyes. A final diagnosis of Best's disease was established. Spectral domain OCT findings at the last visit showed a clearly visible RPE split and a low reflective space between the split RPE layers, as well as a high reflectivity corresponding to the subretinal material. CONCLUSIONS: We report a case of Best's disease with old-age onset with unusual clinical features and abnormal EOG findings. Spectral domain OCT was helpful in evaluating the disease. Treatment with PDT and IVB was not effective.

Keyword

Best disease; Bevacizumab; Photodynamic therapy

MeSH Terms

Aged
Angiography
Antibodies, Monoclonal, Humanized
Choroidal Neovascularization
Electrooculography
Eye
Female
Fluorescein Angiography
Humans
Indocyanine Green
Photochemotherapy
Retinal Detachment
Steroids
Tomography, Optical Coherence
Triazenes
Vision, Ocular
Vitelliform Macular Dystrophy
Bevacizumab
Antibodies, Monoclonal, Humanized
Indocyanine Green
Steroids
Triazenes

Figure

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  Figure 1. Fundus findings show a well-circumscribed, circular area of macula elevation in both eyes. Subretinal exudates with fluid levels in the inferior part of the lesion are seen in both eyes, more marked in the left eye (arrows).

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  Figure 2. Optical coherenece tomography findings of both eyes show serous retinal detachment. High reflectivity areas corresponding to the outer retinal pigment epithelium band (arrows) are visible under the low reflective space. High reflectivity areas corresponding to subretinal exudates are also seen (arrow heads).

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  Figure 3. Fluorescein angiography findings of both eyes reveal well-defined hyperfluorescence in the early phase and persistent hyperfluoresence in the late phase, corresponding to pigment epithelial detachment. Blocked fluorescence due to subretinal exudates are also seen in both eyes, especially in the left eye (arrows).

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  Figure 4. Indocyanine green angiography findings of both eyes reveal well-demarcated early hyperfluorescence and persistent hyperfluorescence in the late phase, corresponding to pigment epithelial detachment. Large choroidal vessels (arrows) are visible under pigment epithelial detachment due to relatively clear fluid.

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  Figure 5. Optical coherence tomography findings of the left eye at 1 month after photodynamic therapy plus intravitreal bevacizumab shows the same features as in the previous one.

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  Figure 6. Optical coherence tomography findings of the left eye at the last visit show the same features as in the previous ones.

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  Figure 7. An electro-oculogram is abnormal with an Arden ratio of 1.463, 1.159 in both eyes, respectively. A diagnosis of Best's disease is established.

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  Figure 8. Spectral domain optical coherence tomography findings of the left eye at the last visit show a clearly visible retinal pigment epithelium split and a low reflective space between split retinal pigment epithelium layers. High reflectivity can be seen between split retinal pigment epithelium layers, corresponding to subretinal exudates (arrows).

Reference

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