J Korean Ophthalmol Soc.  2019 Aug;60(8):808-815. 10.3341/jkos.2019.60.8.808.

Choroidal Neovascularization in a Patient with Best Disease

• Jung JU
• Kim YM
• Kang YK
• Park DH
• Shin JP

• KMID: 2455466
• DOI: http://doi.org/10.3341/jkos.2019.60.8.808

Abstract

PURPOSE
To report a case of choroidal neovascularization in a Best disease patient treated with intravitreal bevacizumab injection and followed up with optical coherence tomography angiography (OCTA).
CASE SUMMARY
A 20-year-old female visited our clinic with decreased visual acuity of the left eye for 6 months. On optical coherence tomography (OCT), subretinal fluid and hyperreflective subretinal clumps were observed in the macula of the right eye. Subretinal hemorrhage and subretinal fluid were observed in the left eye. Choroidal neovascularization in the left eye was observed using OCTA, fluorescein angiography, and indocyanine green angiography. A full-field electroretinogram was normal in both eyes, but an electrooculogram revealed that the Arden ratio was 1.564 in the right eye and 1.081 in the left eye. Intravitreal bevacizumab injection was performed in the left eye. At 6 months after the intravitreal injection, the best-corrected visual acuity of the left eye had recovered to 20/20. OCT revealed that subretinal fluid reduced and choroidal neovascularization was stable. After 12 months, visual acuity of the left eye was maintained at 20/20, but OCTA revealed that choroidal neovascularization had increased.
CONCLUSIONS
Choroidal neovascularization associated with Best disease can improve by intravitreal bevacizumab injection, and the changes in choroidal neovascularization can be followed using OCTA.

Keyword

Bevacizumab; Best disease; Choroidal neovascularization; Optical coherence tomography angiography

MeSH Terms

Angiography
Bevacizumab
Choroid*
Choroidal Neovascularization*
Electrooculography
Female
Fluorescein Angiography
Hemorrhage
Humans
Indocyanine Green
Intravitreal Injections
Subretinal Fluid
Tomography, Optical Coherence
Visual Acuity
Vitelliform Macular Dystrophy*
Young Adult
Bevacizumab
Indocyanine Green

Figure

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  Figure 1 Multimodal imaging findings of the right eye. (A) Fundus photography shows a pale yellowish lesion surrounding the foveola and dark brown spot on the parafovea. (B) Fundus autofluorescence shows ring-shaped hyperautofluorescence around the fovea and hypoautofluorescent spot in the inferior macula. (C) Optical coherence tomography shows subretinal fluid and hyper-reflective subretinal clumps, and retinal pigment epithelium hyperplasia at the dark brown spot.

• 

  Figure 2 Multimodal imaging findings of the left eye. (A) Fundus photography shows juxtafoveal retinal hemorrhage, whitish subfoveal fibrous membrane and yellowish white-colored spots around fovea. (B) Fundus autofluorescence shows hyperautofluorescent ring around the hypoautofluorescence at the fovea. (C) Optical coherence tomography shows subretinal fluid, choroidal neovascular membrane and subretinal hyperreflective material due to subretinal hemorrhage (yellow arrow).

• 

  Figure 3 Multimodal imaging findings of the left eye. (A) Fluorescein angiography and indocyanine green angiography of the left eye shows choroidal neovascularization. (B) Optical coherence tomography angiography shows lacy choroidal neovascularization in the outer retina and choriocapillaris slab. (C) Electrooculography shows that the Arden ratio is 1.564 in the right eye and 1.081 in the left eye.

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  Figure 4 Multimodal imaging findings of the left eye. Fundus photography of left eye at 1 month (A), at 3 months (C), at 6 months (E), and at 12 months (G) after intravitreal bevacizumab injection show disappeared juxtafoveal retinal hemorrhage and whitish fibrotic scar. Optical coherence tomography at 1 month (B) and at 3 months (D) show that the subretinal fluid and choroidal neovascularization membrane decreased. (F) Optical coherence tomography at 6 months shows more decreased subretinal fluid and stable choroidal neovascular membrane. (H) Optical coherence tomography at 12 months shows increased subretinal fluid and choroidal neovascular membrane. (I) Optical coherence tomography angiography at 12 months shows fan shaped choroidal neovascularization in the outer retina slab. Choroidal neovascularization is also seen in the choriocapillaris slab.

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