Unusual Association of Inverse Retinitis Pigmentosa, Scleromalacia, and Neovascular Glaucoma

Abstract

A 31-year-old woman with inverse retinitis pigmentosa presented with severe ocular pain and ingrained visual loss. Biomicroscopy revealed a large scleromalacia area above the superior limbus, minimal Descemet’s membrane folds, aqueous flare, rubeosis iridis, and mature cataract. Intraocular pressure was 39 mmHg, and the clinical picture was consistent with neovascular glaucoma. After immediate medication to reduce ocular discomfort, an anterior chamber bevacizumab injection was performed. At 1 week post-injection, the rubeosis iridis had largely regressed and intraocular pressure was 21 mmHg. At post-injection 1 month, antiglaucomatous medication was discontinued because intraocular pressure was stable. Clear cornea, normal anterior chamber depth, and mature cataract were seen via biomicroscopy, and increased axial length with no significant change in posterior segment echogenicity were observed on ultrasonography. Three years after the single dose of bevacizumab, neovascularization was not seen in either the anterior chamber angle or on the iris surface, and intraocular pressure remained within normal range. The most important aspect of this case report is that it is the first to show an unusual association between neovascular glaucoma, scleromalacia, and inverse retinitis pigmentosa.

Keywords

• anti-vascular endothelial growth factor
• inverse retinitis pigmentosa
• intracameral bevacizumab
• intraocular inflammation
• neovascular glaucoma