NOVEL OUTER RETINAL COLUMNAR ABNORMALITIES AND NONVASOGENIC CYSTOID MACULAR EDEMA IN DENSE DEPOSIT DISEASE.

Abstract

Purpose: To describe the occurrence of bilateral outer retinal columnar abnormalities, nonvasogenic cystoid macular edema, and drusen in the context of dense deposit disease.

Methods: Case report.

Patient: An 18-year-old girl with dense deposit disease was referred to our specialist center for diagnosis and management with findings consistent with bilateral nonvasogenic cystoid macular edema and drusen. She was followed-up in our clinic for 40 months and treated with acetazolamide and ketorolac drops.

Results: Baseline examination revealed bilateral visual acuity reduction and macular elevation with peripapillary drusen on fundus biomicroscopy. Optical coherence tomography revealed bilateral hyporeflective cystoid central macula changes, microcystoid changes with increased central subfield thickness (>450 μm), and outer retinal columnar abnormalities. Fluorescein angiography showed no evidence of macular leakage. Electrodiagnostic testing was within normal limits. Over the course of follow-up, she received treatment with acetazolamide 250 mg twice a day by mouth and ketorolac 0.5% eye drops, with a partial reduction in her edema and improvement in visual acuity.

Conclusion: Dense deposit disease is a rare disease secondary to complement cascade dysregulation, associated with drusen. To the best of our knowledge, this is the first report of bilateral nonvasogenic cystoid macular edema and outer retinal columnar abnormalities in a young female patient with dense deposit disease, confirmed with multimodal imaging.