Central cone dysfunction in autosomal dominant vitreoretino choroidopathy (ADVIRC)
1University of North Carolina Department of Ophthalmology, Chapel Hill, NC, USA. dawnandkean@mac.com
American Journal of Ophthalmology
|May 9, 2006
Summary
Autosomal dominant vitreoretinochoroidopathy can lead to central cone dysfunction due to macular atrophy over time. This progression may occur even as electroretinography and visual fields remain stable.
Area of Science:
- Ophthalmology
- Medical Genetics
Background:
- Autosomal dominant vitreoretinochoroidopathy is a rare inherited retinal disorder.
- Understanding its long-term progression and clinical manifestations is crucial for patient management.
Observation:
- A case report details an 11-year progression of a patient with autosomal dominant vitreoretinochoroidopathy.
- The patient exhibited evolving central cone dysfunction.
Findings:
- Despite stable full-field electroretinography and visual fields, focally reduced multifocal electroretinography was observed.
- Optical coherence tomography revealed macular atrophy, correlating with cone dysfunction.
Implications:
- Macular atrophy can cause late-onset central cone dysfunction in autosomal dominant vitreoretinochoroidopathy.
- This highlights the importance of detailed macular assessment in long-term follow-up of inherited retinal diseases.
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