Peripapillary choroidal neovascularization in best disease

Area of Science:

• Ophthalmology
• Genetics
• Retinal Diseases

Background:

• Best disease is an autosomal dominant retinal dystrophy characterized by macular vitelliform lesions.
• It involves lipofuscin deposition within and beneath the retinal pigment epithelium.
• Choroidal neovascularization (CNV) can complicate Best disease, typically in the macula.

Purpose of the Study:

• To report a case of peripapillary CNV in a patient with Best disease.
• To highlight the potential for successful treatment of this complication.

Main Methods:

• Interventional case report.
• Treatment with reduced-fluence photodynamic therapy for subfoveal and peripapillary CNV.

Main Results:

• A 12-year-old boy with Best disease developed subfoveal CNV treated with photodynamic therapy.
• Subsequently, he developed peripapillary CNV in the other eye, also successfully treated with repeated photodynamic therapy sessions.

Conclusions:

• Peripapillary CNV is a rare but possible complication of Best disease.
• Reduced-fluence photodynamic therapy is an effective treatment option for peripapillary CNV in Best disease.