CHOROIDAL ALTERATIONS IN ABCA4-RELATED RETINOPATHY
Philipp L Müller1, Rolf Fimmers, Martin Gliem
1*Department of Ophthalmology, University of Bonn, Bonn, Germany; †Center for Rare Diseases Bonn (ZSEB), University Hospital of Bonn, Bonn, Germany; and ‡Institute of Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
Retina (Philadelphia, Pa.)
|July 15, 2016
Summary
ABCA4-related retinopathy is linked to reduced choroidal thickness, particularly with retinal pigment epithelium (RPE) atrophy. This suggests a widespread effect, not just localized thinning, in the choroid of affected eyes.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Choroidal Imaging
Background:
- ABCA4-related retinopathy is a genetic disorder affecting vision.
- Choroidal structure and function are crucial for retinal health.
- Previous research has not fully elucidated choroidal changes in ABCA4-related retinopathy.
Purpose of the Study:
- To investigate choroidal alterations in patients with ABCA4-related retinopathy.
- To compare choroidal thickness in patients versus healthy controls.
- To assess the relationship between choroidal thinning and retinal pigment epithelium (RPE) atrophy.
Main Methods:
- Enhanced-depth imaging optical coherence tomography (EDI-OCT) was used.
- Mean and subfoveal choroidal thickness were measured in 40 patients and 65 controls.
- Full-field electroretinography (ffERG) assessed retinal function.
Main Results:
- Patients with ABCA4-related retinopathy showed significantly reduced subfoveal and mean choroidal thickness compared to controls.
- Choroidal thinning was associated with reduced ffERG amplitudes and RPE atrophy.
- Choroidal thickness was similar to controls when RPE atrophy and functional loss were limited to the central retina.
Conclusions:
- Widespread choroidal alterations occur in ABCA4-related retinopathy, especially with RPE atrophy.
- The findings suggest a diffusible factor from the RPE supports choroidal structure.
- Choroidal thinning is not confined to areas of RPE atrophy, indicating a systemic effect.
