Purpose: To describe a patient with clinically documented autosomal dominant vitreoretinochoroidopathy who has had 11 years of progression from initial description and now demonstrates evidence of central cone dysfunction.
Design: Case report.
Methods: The patient is a member of a pedigree described in the literature. This is a case report format that follows standard clinical studies.
Results: The patient had normal full field electroretinography results but focally reduced multifocal electroretinography results and evidence of macular atrophy on optical coherence tomography.
Conclusion: Autosomal dominant vitreoretinochoroidopathy may result in central cone dysfunction because of macular atrophy late in the course of the disease, although electroretinography and visual fields remain stable over extended follow-up periods.