Ten-Year Follow-Up after Bilateral Submacular Neovascular Membrane Removal in a Case of Autosomal Recessive Bestrophinopathy

Abstract

Herein, we report the case of an 8-year-old girl who presented in December 2000 with a submacular neovascular membrane in the right eye, with a clinical diagnosis of Best disease. At that time, she underwent pars plana vitrectomy (PPV) with removal of the subretinal choroidal neovascularization (CNV). Her vision improved from 20/200 to 20/25. Four years later, a new CNV developed in the other eye. Initially, she underwent unsuccessful photodynamic therapy. As her vision worsened, she underwent a second, this time successful, PPV with membrane removal in the left eye, with vision improving to 20/30. Ten years later, she returned complaining of vision loss over the last year. Her vision was 20/200 OU, and optical coherence tomography demonstrated very large intraretinal cystoid spaces resembling bilateral macular schisis. Four ranibizumab injections as well as dorzolamide eye drops were tried, both without success. Finally, she underwent PPV with internal limiting membrane peeling and gas-fluid exchange in the left eye. One month later, the macula appeared flat and vision had improved to 20/60. The same procedure was performed 1 year later for the right eye, with vision improving to 20/80. One year later, mild cystic spaces developed again in both eyes, although much smaller than previously observed. Her vision remained stable.

Keywords

• Bestrophinopathy
• Best dystrophy
• Retina
• Vitrectomy