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Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells: A Disease-in-a-dish Model for BEST1 Mutations
Published on: August 24, 2018
[Best's vitelliform macular dystrophy associated with choroidal neovascularization].
A Céspedes1, M Pérez-De-Arcelus, J García-Arumí
1Servicio de Oftalmología, Hospital Vall d'Hebron, Barcelona, España.
Archivos De La Sociedad Espanola De Oftalmologia
|October 2, 2012
Summary
Sudden vision loss in a child indicated Best's disease, a macular dystrophy. Complications like choroidal neovascularization can cause rapid vision decline.
Area of Science:
- Ophthalmology
- Genetics
- Medical Case Reports
Background:
- Best's disease, or vitelliform macular dystrophy, is an inherited eye condition.
- It typically affects the macula, leading to gradual vision loss.
- Diagnosis is often incidental, with stable visual acuity over time.
Observation:
- A pediatric case presented with acute left eye vision loss.
- Ophthalmoscopy revealed bilateral foveal vitelliform lesions and a left-eye hemorrhage.
- Fluorescein angiography identified a neovascular membrane in the affected eye.
Findings:
- Electrooculography confirmed the disease process.
- The patient was diagnosed with Best's disease complicated by choroidal neovascularization.
- This case highlights a rare presentation of rapid visual deterioration.
Implications:
- Sudden vision loss in suspected Best's disease warrants investigation for complications.
- Choroidal neovascularization is a significant complication that can lead to severe vision impairment.
- Early detection and management of neovascularization are crucial for preserving vision in Best's disease patients.
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