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

Affiliations
  • 1Department of Ophthalmology, Kyungpook National University School of Medicin, Daegu, Korea. Jps11@hanmail.net

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

  • 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.

  • 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.


Reference

1. Marmorstein AD, Marmorstein LY, Rayborn M, et al. Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium. Proc Natl Acad Sci U S A. 2000; 97:12758–12763.
Article
2. Deutman AF, Hoyng CB, van Lith-Verhoeven JJC. Macular dystrophies. In : Ryan SJ, Schachat AP, Wilkinson C, editors. Retina. 4th ed. Baltimore: Elsevier Mosby;2006. v. 2:chap. 63.
3. Shahzad R, Siddiqui MA. Choroidal neovascularization secondary to Best vitelliform macular dystrophy detected by optical coherence tomography angiography. J AAPOS. 2017; 21:68–70.
Article
4. Patel RC, Gao SS, Zhang M, et al. Optical coherence tomography angiography of choroidal neovascularization in four inherited retinal dystrophies. Retina. 2016; 36:2339–2347.
Article
5. Stattin M, Ahmed D, Glittenberg C, et al. Optical coherence tomography angiography for the detection of secondary choroidal neovascularization in vitelliform macular dystrophy. Retin Cases Brief Rep. 2017; 08. 16. DOI: 10.1097/ICB.0000000000000626.
Article
6. Guduru A, Gupta A, Tyagi M, et al. Optical coherence tomography angiography characterisation of Best disease and associated choroidal neovascularisation. Br J Ophthalmol. 2018; 102:444–447.
Article
7. Wang XN, You QS, Li Q, et al. Findings of optical coherence tomography angiography in Best vitelliform macular dystrophy. Ophthalmic Res. 2018; 60:214–220.
Article
8. Andrade RE, Farah ME, Cardillo JA, et al. Optical coherence tomography in choroidal neovascular membrane associated with Best's vitelliform dystrophy. Acta Ophthalmol Scand. 2002; 80:216–218.
Article
9. Sodi A, Murro V, Caporossi O, et al. Long-term results of photodynamic therapy for choroidal neovascularization in pediatric patients with Best vitelliform macular dystrophy. Ophthalmic Genet. 2015; 36:168–174.
Article
10. Leu J, Schrage NF, Degenring RF. Choroidal neovascularisation secondary to Best's disease in a 13-year-old boy treated by intravitreal bevacizumab. Graefes Arch Clin Exp Ophthalmol. 2007; 245:1723–1725.
Article
11. Heidary F, Hitam WH, Ngah NF, et al. Intravitreal ranibizumab for choroidal neovascularization in Best's vitelliform macular dystrophy in a 6-year-old boy. J Pediatr Ophthalmol Strabismus. 2011; 48:e19–e22.
Article
12. Ruiz-Moreno O, Calvo P, Ferrández B, Torrón C. Long-term outcomes of intravitreal ranibizumab for choroidal neovascularization secondary to Best's disease: 3-year follow-up. Acta Ophthalmol. 2012; 90:e574–e575.
Article
13. Khan KN, Mahroo OA, Islam F, et al. Functional and anatomical outcomes of choroidal neovascularization complicating BEST1-related retinopathy. Retina. 2017; 37:1360–1370.
Article
14. O'Gorman S, Flaherty WA, Fishman GA, Berson EL. Histopathologic findings in Best's vitelliform macular dystrophy. Arch Ophthalmol. 1988; 106:1261–1268.
15. Kim HK, Rho CR, Lee WK, Kim KS. Optical coherence tomography findings in Best disease. J Korean Ophthalmol Soc. 2008; 49:845–852.
Article
Full Text Links
  • JKOS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr