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Published on: May 26, 2023
Clinicopathologic findings in Best vitelliform macular dystrophy.
Qing Zhang1, Kent W Small, Hans E Grossniklaus
1Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA, USA.
Summary
Histopathologic mapping of Best vitelliform macular dystrophy (BVMD) eyes revealed retinal edema, RPE abnormalities, drusen, and scars. These findings correlated with clinical imaging, enhancing our understanding of BVMD pathology.
Area of Science:
- Ophthalmology
- Pathology
- Medical Imaging
Background:
- Best vitelliform macular dystrophy (BVMD) is a genetic condition affecting central vision.
- Understanding the histopathologic basis of BVMD is crucial for diagnosis and management.
Observation:
- Postmortem examination of two eyes from a BVMD patient was conducted.
- Histopathologic sections were stained and analyzed, including electron microscopy.
- Findings were mapped in 2D and correlated with fundus photography, FAF, FFA, and OCT.
Findings:
- Right eye showed RPE hyperplasia, lipofuscin accumulation, photoreceptor material, macrophages, and drusen.
- Left eye presented RPE changes and fibrocellular proliferation, forming a chorioretinal scar.
- Histopathologic mapping confirmed retinal edema, RPE abnormalities, drusen, and scars.
Implications:
- This study provides a detailed histopathologic correlation of BVMD features with multimodal imaging.
- Understanding these correlations can improve the interpretation of diagnostic imaging in BVMD patients.
- The findings contribute to a deeper comprehension of the disease's structural changes.
