American Journal of Ophthalmology Case Reports (Mar 2022)

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome

  • Fabiola Ramos Nieves,
  • Victor M. Villegas,
  • Nimesh A. Patel,
  • Audina M. Berrocal,
  • Timothy G. Murray

Journal volume & issue
Vol. 25
p. 101362

Abstract

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Purpose: To report a Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome. Observations: A 64 year-old woman with prior diagnosis of retinal dystrophy presented with decreased vision in the right eye (OD). Ophthalmologic examination was remarkable for bilateral arteriolar attenuation, mid-peripheral bony-spicules, and waxy disc pallor. Coats-like exudative vitreoretinopathy and cystoid macular edema were present OD. Genetic testing showed a homozygous pathogenic mutation in gene NR2E3, variant c.932G>A (p.Arg311Gln), consistent with Goldmann-Favre syndrome. Targeted laser ablation and combination intravitreal therapy were effective in decreasing macular edema. Conclusions and Importance: A Coats-like exudative vitreoretinopathy may occur in the setting of Goldmann-Favre syndrome. Targeted laser ablation in combination with intravitreal therapy can be efficacious in select patients.

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