Novel Outer Retinal Columnar Abnormalities (ORCA) and Non-Vasogenic Cystoid Macular Edema in Dense Deposit Disease

• Published in: RETINAL Cases & Brief Reports
• Main Authors: Peng, Claudia Liang, Olvera-Barrios, Abraham, Schwartz, Roy, Grimaldi, Gabriela, Egan, Catherine, Tufail, Adnan
• Format: Article in Journal/Newspaper
• Language: English
• Published: Ovid Technologies (Wolters Kluwer Health) 2023
• Subjects: Orca
• Online Access: http://dx.doi.org/10.1097/icb.0000000000001488; https://journals.lww.com/10.1097/ICB.0000000000001488

Abstract Purpose: To describe the occurrence of bilateral outer retinal columnar abnormalities, non-vasogenic cystoid macular edema, and drusen in the context of dense deposit disease Methods: Case report. Patient: An 18-year-old female with dense deposit disease was referred to our specialist center for diagnosis and management with findings consistent with bilateral non-vasogenic cystoid macular edema and drusen. She was followed up in our clinic for forty months and treated with acetazolamide and ketorolac drops. Results: Baseline examination revealed bilateral visual acuity (VA) 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 microns), and outer retinal columnar abnormalities (ORCAs). 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 250mg BD PO and ketorolac 0.5% eye drops, with a partial reduction in her edema and improvement in VA. 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 non-vasogenic cystoid macular edema and ORCA in a young female patient with dense deposit disease, confirmed with multimodal imaging.